DIAGNOSTIC IMAGING

ABDOMEN
11
DIAGNOSTIC IMAGING
ABDOMEN
Michael R Federle, MD, FACR
Professor of Radiology
Chief Abdominal Imaging
University of Pittsburgh Medical Center

R. Brooke Jeffrey, MD
Professor of Radiology
Chief of Abdominal Imaging
Stanford University Medical Center

Terry S. Desser, MD
Assistant Professor of Radiology
Stanford University School of Medicine

Venkata Sridhar Anne, MD

Clinical Research Fellow
Radiology Department
Abdominal Imaging Division
University of Pittsburgh Medical Center

Andres Eraso, MD
Assistant Clinical Professor of Radiology
Louisiana State University Health Sciences Center
Radiologist
Veterans Affairs Medical Center
New Orleans, Louisiana

Joseph Jen-Sho Chen, BA

Medical Student; Research Fellow
Radiology Department
Abdominal Imaging Division
University of Pittsburgh Medical Center

Shalini Guliani-Chabra, MD
Radiology Resident
Radiology Department - Abdominal Imaging Division
University of Pittsburgh Medical Center

Karen M. Pealer, BA, CCRC

Clinical Research Coordinator
Radiology Department - Abdominal Imaging Division

.-..
University of Pittsburgh Medical Center

'"

11
AMIRSYS'"
Names you know, content you trust®

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AMIRSYS$
Names you know, content you trust®

First Edition
Text - Copyright Michael P. Federle, MD 2004

Drawings - Copyright Amirsys Ine. 2004

Compilation - Copyright Amirsys Ine. 2004

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or media
or by any means, electronic, mechanical, photocopying, recording, or otherwise, without prior written permission from Amirsys Ine.

Composition by Amirsys Inc, Salt Lake City, Utah

Printed by Friesens, Altona, Manitoba, Canada

ISBN: 1-4160-2541-3
ISBN: 0-8089-2316-1 (International English Edition)

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Library of Congress Cataloging-in-Publication Data

Diagnostic imaging. Abdomen / Michael P. Federle ... let al.].- 1st ed.
p.; cm.
Includes bibliographical references and index.
ISBN 1-4160-2541-3
1. Abdomen-Imaging.
[DNLM: 1. Radiography, Abdominal-methods. 2. Diagnostic Imaging-methods. 3.
Digestive System Diseases-radiography. WI 900 D536 2004] I. Title: Abdomen. II. Federle,
Michael P.

RC944.D526 2004
617.5'50754-dc22
2004022701

IV
 This book is dedicated to my wife, Lynne, who graciously endured many
months of late dinners during its writing, and to my colleagues and trainees at
the University of Pittsburgh Medical Center, who enthusiastically joined in my
quest for the "great cases" to include in the book.

v
VI
 DIAGNOSTIC IMAGING: ABDOMEN

We at Amirsys and Elsevier are proud to present Abdomen, the fifth volume in our acclaimed Diagnostic Imaging
series. This precedent-setting, image- and graphic-packed series began with David Stoller's Orthopaedics. The next
three books, Brain, Head and Neck, and Spine are now joined by the first of three volumes that will focus on
abdominal imaging issues. Mike Federle and his team lead off with Abdomen. Subsequent topics will include
Obstetrics and Gynecology.

The unique bulleted format of the Diagnostic Imaging series allows our authors to present approximately twice the
information and four times the images per diagnosis compared to the old-fashioned traditional prose textbook. All
the DI books follow the same format, which means the same information is in the same place: Every time! In every
organ system. The innovative visual differential diagnosis "thumbnail" that provides an at-a-glance look at entities
that can mimic the diagnosis in question has been highly popular. "Key Facts" boxes provide a succinct summary for
quick, easy review.

In summary, Diagnostic Imaging is a product designed with you, the reader, in mind. Today's typical practice
settings demand efficiency in both image interpretation and learning. We think you'll find the new Abdomen
volume a highly efficient and wonderfully rich resource that will be the core of your reference collection in
abdominal imaging. Enjoy!

Anne G. Osborn, MD
Executive Vice President and Editor-in-Chief, Amirsys Ine.

H. Ric Harnsberger, MD
CEO, Amirsys Ine.

VB
V111
 FOREWORD

Those of us who are abdominal imagers have been eagerly awaiting the Diagnostic Imaging: Abdomen book,
having admired the prior volumes in this remarkable series of imaging texts. We are also pleased to see that the editor
of this volume is Michael Federle, MD, who is Professor of Radiology and Chief of Abdominal Imaging at the
University of Pittsburgh Medical Center.

I have been privileged to know Michael for over 20 years. I share his passion for abdominal imaging, but I have
always been in awe of his clinical and teaching skills. He has used all his experience, judgment and expertise to
produce a teaching tool that will be useful at multiple levels of training, from medical student to practicing
radiologist. The format of the Diagnostic Imaging series makes it easy to learn the relevant imaging and clinical
aspects of every major disease or lesion confronting the radiologist, and should facilitate meaningful communication
with referring physicians.

A unique feature of these texts is the abundance of beautiful medical illustrations that virtually animate the
disease process and the accompanying radiographs. The imaging studies are remarkably excellent, carefully selected
and cropped to highlight every major manifestation of every Diagnosis. The illustrated Differential Diagnosis series
and the accompanying text are other unique features, making it easy to distinguish among several diseases that may
have similar clinical or imaging features.

This text covers all of the major gastrointestinal and genitourinary organs and disease processes from congenital
through traumatic, inflammatory and neoplastic. Between the individual "Diagnoses" (chapters) and the Differential
Diagnoses listed for each, you will find a comprehensive coverage of the factual material covered in the multi-volume
encyclopedic prose textbooks. The Anatomic Overview and.Imaging Issues for each section are written by Dr. Federle
to provide the "glue" to pull the individual Diagnoses together, to suggest optimal imaging protocols, and to provide
extensive tables of Custom Differential Diagnoses that the reader will be referring to on a regular basis.

I have often instructed my residents that the successful radiologist understands three aspects of a disease process:
What does the lesion look like on imaging studies? What does the lesion do on a histopathological and clinical level? What
do these pathological results look like on imaging? Apparently, Michael takes the same approach, and this book will
provide the understanding of these aspects of every GI and GU process likely to be encountered in clinical practice.

Modern imaging is very powerful in revealing the source of a patient's symptoms or signs. This book, as with the
others in the Diagnostic Imaging series, succeeds magnificently in conveying the understanding necessary to make
optimal use of our imaging tools.

Alec]. Megibow, MD, MPH, FACR

Professor and Vice Chairman for Education
New York University School of Medicine

IX
x
 PREFACE
This is a book that almost did not happen. For many years I have wanted to write a comprehensive text of
abdominal imaging that would serve as a "readable reference" for radiology residents and practicing physicians.
Increasing clinical demands and the frustrations inherent in writing and editing a traditional multivolume-
multi author text seemed to preclude this. Ric Harnsberger approached me several years ago with his vision of an
entirely new type of textbook and method of writing, with senior authors in all fields of radiology being the driving
force. The text and images would be entered into a proprietary computer program (the Amirsys authoring tool) that
would help organize the data into easily accessible, bulleted text rather than traditional prose, saving an estimated
50% of unnecessary verbiage. The reader would find the key facts, not just in imaging, but in clinical, pathological
and treatment options of every important diagnosis, and he would find it in the same place for every diagnosis and
every book in the series. Moreover, we would ignore the usual publishers' discouragement of the use of color images,
and would use color images and original artwork lavishly. I signed on with some trepidation.

The use of the Amirsys "authoring tool" provided and additional benefit of allowing for a certain uniformity of
style and depth of coverage that is usually impossible to achieve in a multi author text. At the University of Pittsburgh,
we assembled a team of bright young people who performed much of the literature review and data entry, freeing me
to concentrate on editing, overview and selecting the best images and illustrations for maximum teaching value. Our
proprietary authoring tool even facilitated the updating and entry of references; you will note many references from
within the past year, previously impossible to achieve with the lag time to publication of a traditional textbook.

The Pittsburgh team of contributors includes Venkata Anne, MD, who also assisted with the research and writing
of our first Amirsys book, the Pocket Radiologist, Top 100 Diagnoses in Abdominal Imaging. Dr. Anne has augmented
his residency in radiology with a thorough review of available texts and journal articles to acquire an encyclopedic
knowledge of abdominal imaging, and he has contributed substantially to the majority of diagnoses included in our
Pocket Radiologist and current text.

This comprehensive work would not have been accomplished without the dedication and efforts of many people:

Andres Eraso, MD, a former resident and fellow at Pitt, was my main assistant in reviewing our teaching files and
medial records to select the most compelling and informative images for the book. Joe Chen is a medical student and
future radiologist who was part of the research and writing team, along with Shalini Guliani, MD a radiologist trained
in India who is gaining additional training in our radiology residency. Their co-authorship of diagnoses is noted in
the table of contents, and their excellent work is both acknowledged and appreciated.

Karen Pealer has been my research assistant for many years and was absolutely instrumental in the success of this
project. Karen used all of her computer and communication skills to help me organize, edit, enter and illustrate each
of the diagnostic "chapters" and introductory sections. She was also the conduit of information and problem solving
between Pittsburgh and Amirsys headquarters in Salt Lake City. I suppose it would have been possible for an old dog
like me to learn all the new computer tricks necessary to complete this project, but it would have been much more
difficult and time-consuming.

The Stanford team was led by my career-long colleague and friend, Brooke Jeffrey, MD, himself the author of
several excellent textbooks and innumerable articles and other contributions. Brooke has always been one of the top
radiologists and educators in the world, and we are proud to have him as part of this project. Terry Desser, MD is the
other Stanford faculty radiologist who contributed dozens of diagnoses that are all beautifully researched, written and
illustrated.

The Amirsys team all contributed their own expertise. I particularly want to acknowledge Ric Harnsberger whose
unfailing vision and enthusiasm have seen this through some difficult times. The medical illustrations, so key to
understanding and such an Amirsys "trademark", are the work of Rich Coombs, MS and James Cooper, MD I
absolutely love what they have done and you will be seeing them in my lectures for many years!

Michael P. Federle, MD, FACR

Xl
XlI
ACKNOWLEDGMENTS

Illustrations
Richard Coombs, MS
James Cooper, MD

Art Direction and Design

Lane R. Bennion, MS
Richard Coombs, MS

Image/Text Editing
Angie D. Mascarenaz
Cassie 1. Dearth
Kaerli Main
Roth LaFleur
David Harnsberger

Medical Text Editing

Andre Macdonald, MD
Doug Green, MD
David Avrin, MD
Akram Shaaban, MD

Production lead
Melissa A. Morris

X111
XIV
 SECTIONS

PART I
GI Tract and Abdominal Cavity

Peritoneum, Mesentery, and Abdominal Wall [1]

Esophagus [I]
Gastroduodenal [l]
Small Intestine [!]
Colon ~
Spleen ffiJ
PART II
Hepatobiliary and Pancreas

Liver [1]
Biliary System [l]
Pancreas [l]

PART III
Genitourinary and Retroperitoneum

Retroperitoneum [1]
Adrenal ~
Kidney and Urinary Tract ~
Ureter [!]
Bladder ~
Genital Tract (Male) [[]
xv
 TABLE OF CONTENTS
Transmensenteric Post-Operative Hernia 1-1-40
PART I Michael P. Federle, MD, FACR & Venkata S. Anne, MD

GI Tract and Abdominal Cavity Trauma

Traumatic Abdominal Wall Hernia 1-1-44
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
SECTION 1 Mesenteric Trauma 1-1-46
Peritoneum, Mesentery, and Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Abdominal Wall Traumatic Diaphragmatic Rupture 1-1-48
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Introduction and Overview
Peritoneum, Mesentery, and Abdominal Wall 1-1-2 Neoplasm, Benign
Michael P. Federle, MD, FACR Mesentric Cyst 1-1-52
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Infection Desmoid 1-1-54
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Abdominal Abscess 1-1-6
R. Brooke Jeffrey, MD
Peritonitis 1-1-10 Neoplasm, Malignant
R. Brooke Jeffrey, MD Mesothelioma 1-1-58
Terry S. Desser, MD
Inflammation Peritoneal Metastases 1-1-62
R. Brooke Jeffrey, MD
Sclerosing Mesenteritis 1-1-14
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Pseudomyxoma Peritonei 1-1-66
R. Brooke Jeffrey, MD
Ascites 1-1-18
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Omental Infarct 1-1-22
Michael P. Federle, MD, FACR & Venkata S. Anne, MD SECTION 2
Esophagus
External Hernias
Inguinal Hernia 1-1-24 Introduction and Overview
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Esophagus Anatomy and Imaging Issues 1-2-2
Femoral Hernia 1-1-28 Michael P. Federle, MD, FACR
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Ventral Hernia 1-1-30 Congenital
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Esophageal Webs 1-2-6
Spigelian Hernia 1-1-32 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Obturator Hernia 1-1-34 Infection
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Candida Esophagitis 1-2-8
Terry S. Desser, MD
Internal Hernias Viral Esophagitis 1-2-12
Paraduodenal Hernia 1-1-36 Terry S. Desser, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD

XVI
 Inflammation SECTION 3
Reflux Esophagitis 1-2-16 Gastroduodenal
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Barrett Esophagus 1-2-20 Introduction and Overview
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Gastroduodenal Anatomy and Imaging Issues 1-3-2
Caustic Esophagitis 1-2-24 Michael P. Federle, MD, FACR
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Drug-Induced Esophagitis 1-2-28
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Congenital
Gastric Diverticulum 1-3-6
Radiation Esophagitis 1-2-30
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Duodenal Diverticulum 1-3-8
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Degenerative
Achalasia, Cricopharyngeal 1-2-32
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Inflammation
Gastritis 1-3-10
Achalasia, Esophagus 1-2-34
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Gastric Ulcer 1-3-14
Esophageal Motility Disturbances 1-2-38
Michael P. Federle, MD, FACR
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Scleroderma, Esophagus 1-2-42
Duodenal Ulcer 1-3-18
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR
Esophageal Varices 1-2-46 Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Zollinger-Ellison Syndrome 1-3-22
Schatzki Ring 1-2-50 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Menetrier Disease 1-3-26
Hiatal Hernia 1-2-52 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Caustic Gastroduodenal Injury 1-3-28
Michael P. Federle, MD, FACR
Esophageal Diverticula
Zenker Diverticulum 1-2-56 Trauma
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Duodenal Hematoma and Laceration 1-3-30
Traction Diverticulum 1-2-60 R. Brooke Jeffrey, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Pulsion Diverticulum 1-2-62 Neoplasm, Benign
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Gastric Polyps 1-3-32
Intramural Pseudodiverticulosis 1-2-66 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Duodenal Polyps 1-3-36
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Trauma Intramural Benign Gastric Tumors 1-3-38
Esophageal Foreign Body 1-2-68 Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Esophageal Perforation 1-2-70 Neoplasm, Malignant
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Gastric Stromal Tumor 1-3-42
Boerhaave Syndrome 1-2-74 R. Brooke Jeffrey, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Gastric Carcinoma 1-3-46
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Neoplasm, Benign Gastric Lymphoma and Metastases 1-3-50
Intramural Benign Esophageal Tumors 1-2-78 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Duodenal Carcinoma 1-3-54
Fibrovascular Polyp 1-2-80 Terry S. Desser, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Duodenal Metastases and Lymphoma 1-3-58
R. Brooke Jeffrey, MD
Neoplasm, Malignant
Esophageal Carcinoma 1-2-82 Treatment Related
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Fundoplication Complications 1-3-60
Esophageal Metastases and Lymphoma 1-2-86 Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD

XVll
 Gastric Bypass Complications 1-3-64
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Neoplasm, Benign
Intramural Benign Intestinal Tumors 1-4-54
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Miscellaneous
Hamartomatous Polyposis (P-J) 1-4-56
Gastric Bezoar 1-3-68
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Small Bowel Carcinoma 1-4-58
Gastric Volvulus 1-3-72
Michael P. Federle, MD, FACR
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Shalini Guliani-Chabra, MD & Venkata S. Anne, MD
Aorto-Enteric Fistula 1-3-76
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Neoplasm, Malignant
Carcinoid Tumor 1-4-60
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
SECTION 4
Intestinal Metastases and Lymphoma 1-4-64
Small Intestine Michael P. Federle, MD, FACR & Venkata S. Anne, MD

Introduction and Overview Miscellaneous

Small Intestine Anatomy and Imaging Issues 1-4-2 Small Bowel Obstruction 1-4-68
Michael P. Federle, MD, FACR Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Gallstone Ileus 1-4-72
Congenital Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Malrotation 1-4-6 Malabsorption Conditions 1-4-74
Michael P. Federle, MD, FACR Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Intussusception 1-4-78
Meckel Diverticulum 1-4-8 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Radiation Enteritis 1-4-82
Michael P. Federle, MD, FACR
Infection Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Intestinal Parasitic Disease 1-4-12
R. Brooke Jeffrey, MD
Opportunistic Intestinal Infections 1-4-14 SECTION 5
R. Brooke Jeffrey, MD Colon
Inflam mation Introduction and Overvjew
Sprue-Celiac Disease 1-4-18 Colon Anatomy and Imaging Issues 1-5-2
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR
Whipple Disease 1-4-22
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Infection
Mastocytosis 1-4-24 Infection Colitis 1-5-6
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Crohn Disease 1-4-26 Pseudomembranous Colitis 1-5-10
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Scleroderma, Intestinal 1-4-30 Typhlitis 1-5-14
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR & Venkata S. Anne, MD

Vascular Disease Inflammation and Ischemia

Pneumatosis of the Intestine 1-4-34 Ulcerative Colitis 1-5-16
Michael P. Federle, MD, FACR Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Toxic Megacolon 1-5-20
Ischemic Enteritis 1-4-38 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Appendicitis 1-5-22
Vasculitis, Small Intestine 1-4-42 R. Brooke Jeffrey, MD
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Mucocele of the Appendix 1-5-26
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Trauma
Diverticulitis 1-5-28
Intestinal Trauma 1-4-46 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Epiploic Appendagitis 1-5-32
Gastrointestinal Bleeding 1-4-50 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
XV111
Ischemic Colitis 1-5-36 Splenic Tumors 1-6-26
Michael P. Federle, MD, FACR & Venkata S. Anne,· MD Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Splenic Metastases and Lymphoma 1-6-30
Neoplasm Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Venkata S. Anne, MD
Colonic Polyps 1-5-40
Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA Miscellaneous
Colon Carcinoma 1-5-44 Splenomegaly and Hypersplenism 1-6~34
Michael P. Federle, MD, FACR Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA Shalini Guliani-Chabra, MD & Venkata S. Anne, MD
Rectal Carcinoma 1-5-48
Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Villous Adenoma
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
1-5-52 PART II
Hepatobiliary and Pancreas
Familial Polyposis 1-5-56
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Gardner Syndrome 1-5-60
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA SECTION 1
Liver
Miscellaneous
Sigmoid Volvulus 1-5-62 Introduction and Overview
Michael P. Federle, MD, FACR
Liver Anatomy and Imaging Issues 11-1-2
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Michael P. Federle, MD, FACR
Cecal Volvulus 1-5-66
Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA Congenital
Congenital Absence of Hepatic Segments 11-1-6
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
SECTION 6 Congenital Hepatic Fibrosis 11-1-8
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Spleen
AD Polycystic Disease, Liver 11-1-12
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Introduction and Overview
Spleen Anatomy and Imaging Issues 1-6-2 Infection
Michael P. Federle, MD, FACR
Hepatitis 11-1-16
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Congenital
Hepatic Candidiasis 11-1-20
Asplenia and Polysplenia 1-6-6 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Venkata S. Anne, MD Hepatic Pyogenic Abscess 11-1-24
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Accessory Spleen 1-6-10
Michael P. Federle, MD, FACR Hepatic Amebic Abscess 11-1-28
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Hepatic Hydatid Cyst 11-1-32
Infection Michael P. Federle, MD, FACR & Venkata S. Anne, MD

Splenic Infection and Abscess 1-6-12

R. Brooke Jeffrey, MD Inflammation
Steatosis (Fatty Liver) 11-1-36
Vascular Michael P. Federle, MD, FACR & Venkata S. Anne, MD

Splenic Infarction 1-6-16 Cirrhosis 11-1-40

R. Brooke Jeffrey, MD Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Focal Confluent Fibrosis 11-1-44
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Trauma
Splenic Trauma 1-6-20 Primary Biliary Cirrhosis 11-1-48
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
R. Brooke Jeffrey, MD
Nodular Regenerative Hyperplasia 11-1-52
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Neoplasm
Hepatic Sarcoidosis 11-1-56
Splenic Cyst 1-6-22 Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
XIX
 Vascular Disorder Treatment Related
Hepatic AVMalformation (O-W-R) 11-1-60 Radiation Hepatitis 11-1-148
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Budd-Chiari Syndrome 11-1-64
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Passive Hepatic Congestion 11-1-68 SECTION 2
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Biliary System
HELLPSyndrome 11-1-72
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Introduction and Overview
Hepatic Infarction 11-1-76
Biliary System Anatomy and Imaging Issues 11-2-2
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Michael P. Federle, MD, FACR
Peliosis Hepatis 11-1-80
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Congenital
Caroli Disease 11-2-6
Metabolic - Inherited Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Hemochromatosis 11-1-84
Choledochal Cyst 11-2-10
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Wilson Disease 11-1-88
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Infection
Accending Cholangitis 11-2-14
Trauma Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Hepatic Trauma 11-1-90
Recurrent Pyogenic Cholangitis 11-2-16
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
R. Brooke Jeffrey, MD
Biliary Trauma 11-1-94
Pancreato-Biliary Parasites 11-2-20
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
R. Brooke Jeffrey, MD
AIDSCholangiopathy 11-2-22
Neoplasm, Benign R. Brooke Jeffrey, MD
Hepatic Cyst 11-1-98
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Inflammation
Hepatic Cavernous Hemangioma 11-1-102 Choledocholithiasis 11-2-26
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Focal Nodular Hyperplasia 11-1-106 Mirizzi Syndrome 11-2-30
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
R. Brooke Jeffrey, MD
Hepatic Adenoma 11-1-110
Hyperplastic Cholecystoses 11-2-32
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
R. Brooke Jeffrey, MD
Biliary Hamartoma 11-1-114 Cholecystitis 11-2-34
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
R. Brooke Jeffrey, MD
Hepatic Angiomyolipoma 11-1-118 Porcelain Gallbladder 11-2-38
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Terry S. Desser, MD
Milk of Calcium Bile 11-2-40
Neoplasm, Malignant R. Brooke Jeffrey, MD
Hepatocellular Carcinoma 11-1-120 Primary Sclerosing Cholangitis 11-2-42
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Fibrolamellar HCC 11-1-124
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Neoplasm, Primary
Cholangiocarcinoma (Peripheral) 11-1-128 Gallbladder Carcinoma 11-2-46
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
R. Brooke Jeffrey, MD, FACR
Epithelioid Hemangioendothelioma 11-1-132 Cholangiocarcinoma 11-2-50
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Biliary Cystadneocarcinoma 11-1-136 Ampullary Carcinoma 11-2-54
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
R. Brooke Jeffrey, MD
Angiosarcoma, Liver 11-1-140 IPMT, Biliary 11-2-58
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
R. Brooke Jeffrey, MD
Hepatic Metastases and Lymphoma 11-1-144
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Treatment Related
Chemotherapy Cholangitis 11-2-60
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
xx
 SECTION 3
Pancreas
PART III
Genitourinary and
Introduction and Overview Retroperitoneum
Pancreas Anatomy and Imaging Issues 11-3-2
Michael P. Federle, MD, FACR
SECTION 1
Normal Variants and Congenital Retroperitoneum
Agenesis of Dorsal Pancreas 11-3-6
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Annular Pancreas 11-3-8
Introduction and Overview
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Retroperitoneum Anatomy and Imaging Issues 11I-1-2
Michael P. Federle, MD, FACR
Pancreas Divisum 11-3-12
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Ectopic Pancreatic Tissue 11-3-16 Normal Variants
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Duplications and Anomalies of IVC 11I-1-6
Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Inflammation
Cystic Fibrosis, Pancreas 11-3-18
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Inflammation
Acute Pancreatitis 11-3-20 Retroperitoneal Fibrosis 11I-1-10
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR & Venkata S. Anne, MD

Pancreatic Pseudocyst 11-3-24 Pelvic Lipomatosis 11I-1-14

Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Chronic Pancreatitis 11-3-28
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Trauma
Trauma Retroperitoneal Hemorrhage 11I-1-16
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Traumatic Pancreatitis 11-3-32
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Neoplasm
Neoplasm, Benign Retroperitoneal Sarcoma 11I-1-20
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Serous Cystadenoma of Pancreas 11-3-36
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Retroperitoneal Lymphoma 11I-1-24
R. Brooke Jeffrey, MD
Pancreatic Cysts 11-3-40
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD
Retroperitoneal Metastases 11I-1-28
Terry S. Desser, MD

Neoplasm, Malignant
Treatment Related
Mucinous Cystic Pancreatic Tumor 11-3-42
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Retroperitoneal Lymphocele 11I-1-32
Terry S. Desser, MD
IPMT, Pancreas 11-3-46
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Pancreatic Ductal Carcinoma 11-3-50 SECTION 2
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Pancreatic Islet Cell Tumors 11-3-54
Adrenal
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Solid and Papillary Neoplasm 11-3-58
Introduction and Overview
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA Adrenal Anatomy and Imaging Issues 11I-2-2
Michael P. Federle, MD, FACR
Pancreatic Metastases and Lymphoma 11-3-60
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Infection
Adrenal TBand Fungal Infection 11I-2-6
Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA

XXI
 Vascular - Traumatic Infection
Adrenal Hemorrhage 11I-2-8 Pyelonephritis 11I-3-28
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Renal Abscess 11I-3-32
Metabolic Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Adrenal Hyperplasia 11I-2-12
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Xanthogranulomatous Pyelonephritis 11I-3-36
Michael P. Federle, MD, FACR
Adrenal Insufficiency 11I-2-16 Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Michael P. Federle, MD
Shalini Guliani-Chabra, MD, FACR & Joseph Jen-Sho Chen, BA Emphysematous Pyelonephritis 11I-3-40
Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Neoplasm, Benign
HIV Nephropathy 11I-3-42
Adrenal Cyst 11I-2-18 R. Brooke Jeffrey, MD
Michael P. Federle, MD, FACR
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Inflammation
Adrenal Adenoma 11I-2-20
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Glomerulonephritis 11I-3-44
Terry S. Desser, MD
Adrenal Myelolipoma 11I-2-24
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Renal Papillary Necrosis 11I-3-46
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Pheochromocytoma 11I-2-26
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Urolithiasis 11I-3-48
Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Neoplasm, Malignant
Nephrocalcinosis 11I-3-52
Adrenal Carcinoma 11I-2-30 Terry S. Desser, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Acquired Cystic Disease of Uremia 11I-3-56
Adrenal Metastases and Lymphoma 11I-2-34 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Medullary Sponge Kidney 11I-3-60
Adrenal Collision Tumor 11I-2-38 Michael P. Federle, MD, FACR
Michael P. Federle, MD, FACR & Shalini Guliani-Chabra, MD Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA

Vascular
SECTION 3 Renal Artery Stenosis 11I-3-64
Kidney and Urinary Tract Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Renal Infarction 11I-3-68
Introduction and Overview Michael P. Federle, MD, FACR & Venkata S. Anne, MD

Kidney and Urinary Tract Anatomy and Imaging 11I-3-2 Renal Vein Thrombosis 11I-3-72
Michael P. Federle, MD, FACR Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA

Normal Variants and Pseudolesions Trauma

Renal Ectopia 11I-3-6 Renal Trauma 11I-3-76
Michael P. Federle, MD, FACR Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Shalini Guliani-Chabra, MD & Venkata S. Anne, MD
Horseshoe Kidney 11I-3-10 Neoplasm, Benign
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA Renal Cyst 11I-3-80
Column of Bertin 11I-3-14 Michael P. Federle, MD, FACR
Michael P. Federle, MD, FACR Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA
Shalini Guliani-Chabra, MD & Joseph Jen-Sho Chen, BA
Renal Oncocytoma 11I-3-84
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Congenital Renal Angiomyolipoma 11I-3-88
AD Polycystic Disease, Kidney 11I-3-16 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD Multilocular Cystic Nephroma 11I-3-92
von Hippel Lindau Disease 11I-3-20 Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Ureteropelvic Junction Obstruction 11I-3-24 Neoplasm, Malignant
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA Renal Cell Carcinoma 11I-3-96
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA

XXll
Renal Medullary Carcinoma 11I-3-100
Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Neoplasm
Transitional Cell Carcinoma 11I-3-102
Michael P. Federle, MD, FACR
Bladder Carcinoma 11I-5-22
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA

Renal Metastases and Lymphoma 11I-3-106

Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
SECTION 6
Genital Tract (Male)
SECTION 4
Ureter Introduction and Overview
Genital Tract Anatomy and Imaging Issues 11I-6-2
Congenital Michael P. Federle, MD, FACR

Ureteral Duplication 11I-4-2

Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA Congenital
Ectopic Ureter 11I-4-4 Cryptorchidism 11I-6-6
Terry S. Desser, MD Terry S. Desser, MD

Ureterocele 11I-4-8
Terry S. Desser, MD Infection
Epididymitis 11I-6-8
Infection R. Brooke Jeffrey, MD

Ureteritis Cystica 11I-4-10 Prostatitis 11I-6-10

Terry S. Desser, MD Terry S. Desser, MD

Inflammation
SECTION 5 Urethral Stricture 11I-6-12
Bladder Michael P. Federle, MD, FACR & Joseph Jen-Sho Chen, BA
Hydrocele 11I-6-14
Introduction and Overview R. Brooke Jeffrey, MD

Bladder Anatomy and Imaging Issues 11I-5-2 Varicocele 11I-6-16

R. Brooke Jeffrey, MD
Michael P. Federle, MD, FACR
Testicular Torsion 11I-6-18
R. Brooke Jeffrey, MD
Congenital
Benign Prostatic Hypertrophy 11I-6-20
Urachal Remnant 11I-5-6
R. Brooke Jeffrey, MD
Terry S. Desser, MD

Infection Trauma
Scrotal Trauma 11I-6-22
Cystitis 11I-5-8 R. Brooke Jeffrey, MD
Teny S. Desser, MD

Inflammation Neoplasm
Gonadal Stromal Tumors 11I-6-24
Bladder Calculi 11I-5-10 Terry S. Desser, MD
Terry S. Desser, MD
Bladder Diverticulum Testicular Carcinoma 11I-6-26
11I-5-12 R. Brooke Jeffrey, MD
Terry S. Desser, MD
Prostate Carcinoma 11I-6-30
Bladder Fistulas 11I-5-14
Michael P. Federle, MD, FACR & Venkata S. Anne, MD
Teny S. Desser, MD
Neurogenic Bladder 11I-5-16
Michael P. Federle, MD, FACR
Venkata S. Anne, MD & Joseph Jen-Sho Chen, BA

Trauma
Bladder Trauma 11I-5-18
Michael P. Federle, MD, FACR
Venkata S. (Anne, MD & Joseph Jen-Sho Chen, BA

XX11l
XXIV
 ABBREVIATIONS
ACKD: Acquired Cystic Kidney Disease
ADPKD: Autosomal Dominant Polycystic Kidney Disease LUQ: Left Upper Quadrant
ADPLD: Autosomal Dominant Polycystic Liver Disease MAl: Atypical Myobacterial Infection
AFB: Acid Fast Bacilli MALT: Mucosa-Asociated Lymphoid Tissue
AML: Angiomyolipoma MEN: Multiple Endocrine Neoplasia
AML: Renal Angiomyolipoma Mets: Metastasis
ANA: Antinuclear Antibodies MIP: Maximum Intensity Projection
APUD: Amine Precursor Uptake & Decarboxylation MPD: Main Pancreatic Duct
ARPKD: Autosomal Recessive Polycystic Kidney Disease MRCP: MR Cholangiopancreatography
ATN: Acute Tubular Necrosis MRS: MR Spectroscopy
AVM: Arterio-Venous Malformation MVA: Motor Vehicle Accidents
BE: Barium Enema NASH: Nonalcoholic Steatohepatis
BPH: Benign Prostatic Hypertrophy NECT: Non-enhanced Computed Tomography
CA: Cancer NET: Neuroendocrine Tumor
CBD: Common Bile Duct NG: Nasogastric
CEA: Carcinomembryonic Antigen NHL: Non-Hodgkin Lymphoma
CECT: Contrast Enhanced Computed Tomography NSAID: Non-steroidal Anti-inflammatory Drug
CMV: Cytomegalovirus O-W-R: Osler-Weber-Rendu Disease
DIC: Disseminated Intravascular Coagulation PBC: Primary Biliary Cirrhosis
DPL: Diagnostic Peritoneal Lavage PD: Pancreatic Duct
DVT: Deep Venous Thrombosis PET: Positron Emission Tomograpghy
EBV: Ebstein-Barr Virus PJS: Peutz-Jeghers Syndrome
EHE: Epitheloid Hemangioendothelioma PMN: Polymorphonuclear Leukocytes
ERCP: Endoscopic Retrograde Cholangiapancreatogram PMP: Psuedomyxoma Peritonei
ESR: Erythrocyte Sedimentation Rate PPD: Positive Purified Protein Derivative
EUS: Endoscopic Ultrasound PSC: Primary Sclerosing Cholangitis
FAPS: Familial Adenomatous Polyposis Syndrome PSS: Progressive Systematic Sclerosis
FDG: 18F Flourodeoxyglucose PTC: Percutaneous Transhepatic Cholangiography
FNA: Fine Needle Aspiration PUD: Peptic Ulcer Disease
FUDR: Floxuridine PVP: Portal Venous Phase
GB: Gallbladder RAO: Right Anterior Oblique
GBM: Glomerular Basement Membrane RARE: Rapid Acquisition with Relaxation Enhancement
GE: Gastroesophageal RCC: Renal Cell Carcinoma
GERD: Gastroesophageal Reflux Disease RES: Reticuloendothelial System
GIST: Gastrointestinal Stromal Tumor RLQ: Right Lower Quadrant
GU: Genitourinary RUQ: Right Upper Quadrant
HAP: Hepatic Arterial Phase RYGB: Roux-enY Gastric Bypass
HAV: Hepatis A SB: Small Bowel
HBV: Hepatis B SBFT: Small Bowel Follow Through
HCC: Hepatocellular Carcinoma SBO: Small Bowel Obstruction, Closed Loop Obstruction
HCV: Hepatis C SMA: Superior Mesenteric Artery
HELLP: Hemolysis, Elevated Liver Enzymes, Low Platelets SMV: Superior Mesenteric Vein
HHT: Hereditary Hemorrhagic Telangiectasia SSFSE: Single Shot Fast Spin Echo
HIV: Human Immunodeficiency Virus SVC: Superior Vena Cava
HLA: Human Leukocyte Antigen TB: Tuberculosis
HNPCC: Hereditary Nonpolyposis Colorectal Cancer TCC: Transitional Cell Carcinoma
HPV: Human Papilloma Virus TI: Terminal Ileum
HSV: Herpes Simplex Virus TNM: Tumor, Nodal, Metastasis
HU: Hounsfield Units TURP: Transurethral Prostatectomy
IHBD: Intrahepatic Bile Ducts UC: Ulcerative Colitis
IMA: Inferior Mesenteric Artery UGI: Upper GI Series
IMV: Inferior Mesenteric Vein UP}: Ureteropelvic Junction
IPMT: Intraductal Papillary Mucinous Tumor US: Ultrasound
ITP: Idiopathic Thrombocytopenic Purpura UTI: Urinary Tract Infection
IVC: Inferior Vena Cava UVJ: Ureterovesical Junction
IVU: Excretory Urography VZV: Varicella-Zoster Virus
Lap. Port: Laproscopy Port WBC Scan: White Blood Cell Scan
LES: Lower Esophageal Sphincter XPGN: Xanthogranulomatous Pyelonephritis
LLQ: Left Lower Quadrant ZES: Zollinger-Ellison Syndrome
XXV
XXVI
DIAGNOSTIC IMAGING
ABDOMEN

XXVll
 PART I
GI Trad and Abdominal Cavity

Peritoneum, Mesentary, and Abdominal Wall III

Esophagus [I]
Gastroduodenal [!]
Small Intestine [!]
6
Colon []]

Spleen ~
I SECTION 1: Peritoneum, Mesentel'); and Abdominal Wall I

Introduction and Overview

Peritoneum, Mesentery, and Abdominal Wall 1-1-2

Infection
Abdominal Abscess 1-1-6
Peritonitis 1-1-10

Inflammation
Sclerosing Mesenteritis 1-1-14
Ascites 1-1-18
Omental Infarct 1-1-22

External Hernias
Inguinal Hernia 1-1-24
Femoral Hernia 1-1-28
Ventral Hernia 1-1-30
Spigelian Hernia 1-1-32
Obturator Hernia 1-1-34

Internal Hernias
Paraduodenal Hernia 1-1-36
Transmensenteric Post-Operative Hernia 1-1-40

Trauma
Traumatic Abdominal Wall Hernia 1-1-44
Mesenteric Trauma 1-1-46
Traumatic Diaphragmatic Rupture 1-1-48

Neoplasm, Benign
Mesenteric Cyst 1-1-52
Desmoid 1-1-54

Neoplasm, Malignant
Mesothelioma 1-1-58
Peritoneal Metastases 1-1-62
Pseudomyxoma Peritonei 1-1-66
 PERITONEUM, MESENTERY, AND ABDOMINAL WALL
1
2

Graphic shows pleural fluid (green) outside the confines Graphic shows pleural fluid & ascites relative to the
of the diaphragm, and ascites (yellow) within. diaphragm. Pleural fluid extends to "touch" the spine.
Ascites is held medially by diaphragm; excluded from
bare area of liver (arrow).

o Abnormal opening in abdominal wall due to

I TERMINOLOGY trauma, surgery, or weakness of supporting
Definitions structures
• Peritoneal cavity o May be inapparent on clinical exam (obesity, deep
o The potential space within the abdomen and pelvis location) or mistaken for an abdominal mass
that is lined with peritoneum o Easily demonstrated on CT, including complications
• Peritoneum (e.g., bowel obstruction)
o Single layer of mesothelium that covers a thin layer o Ventral hernia - through linea alba
of connective tissue o Spigelian hernia - along lateral margin of rectus
o Parietal peritoneum lines the abdominal and pelvic muscle, through defect in aponeuroses of internal
muscular walls oblique and transverse muscles
o Visceral peritoneum covers the bowel (serosa) and o Umbilical
abdominal viscera o Lumbar - through posterolateral abdominal wall
• Greater omentum • Inferior lumbar triangle, just above iliac crest
o Four-layered fold of peritoneum that includes fat, • Superior lumbar (Grynfelt triangle), between 12th
blood vessels, nerves, and lymph nodes rib and erector spinae muscles
o Drapes over bowel, separating it from anterior o Inguinal
abdominal wall • Indirect through deep inguinal ring
o Joins stomach and transverse colon (as the o Femoral - adjacent to femoral vessels
gastro-colic ligament), then continues inferiorly o Obturator - between pectineus and obturator
• Lesser omentum muscles
o Connects the lesser curvature of stomach and o Diaphragmatic
duodenum to undersurface of liver • Esophageal - hiatal
• Omental bursa • Traumatic
o Synonymous with lesser sac • Morgagni - congential, anterior
• Mesentery • Bochdalek - congential, posterior
o Two layers of peritoneum (plus vessels, nerves, etc.)
connecting bowel to posterior abdominal wall
o Covers and suspends jejunum and ileum I ANATOMY-BASED IMAGING ISSUES I
o Transverse and sigmoid mesocolon are analogous in Key Concepts or Questions
structure and function (root of transverse mesocolon
• Peritoneum and pleura are almost identical in
separates upper and lower compartments of the
structure and function
peritoneal cavity
o Are subject to the same inflammatory and neoplastic
• Ligament
processes
o Fold of peritoneum that connects and supports
• E.g., empyema and abdominal abscess;
structures within peritoneal cavity (e.g., gastro-colic
mesothelium (pleural and peritoneal)
ligament)
o Imaging manifestations are similar
• Hernias
• E.g., smooth thickening with infection; nodular
thickening with tumor
 PERITONEUM, MESENTERY, AND ABDOMINAL
DIFFERENTIAL DIAGNOSIS
Mesenteric or omental tissue mass
Common
• Hematoma
• Lymphoma
• ~ (Especially non-Hodgkin lymphoma)
• Lymphadenopathy
• Pancreatitis
• Carcinomatosis, metastases
Uncommon
• Mesothelioma
• Desmoid
• Mesenchymal (benign and malignant)
• ~ (E.g., lipoma, liposarcoma)
• Carcinoid
• Mesothelioma and peritoneal (or pleural)
metastases are indistinguishable by imaging
• How do you determine on CT or MR whether a
peridiaphragmatic fluid collection is intrathoracic (Le.,
pleural) or intra-abdominal (Le., ascites, abscess)?
o Relationship to diaphragm
• Outside the confines of the diaphragm =
intrathoracic
• Inside (medial to) the diaphragm = abdominal
o Pleural fluid "touches" the body wall and paraspinal
region
• Ascites is held medially by the diaphragm
o Pleural fluid has a "fuzzy" (indistinct) margin as it
abuts the top of liver and spleen
• Ascites has "sharp" interface
o Do not mistake atelectatic lung for diaphragm
• Atelectatic lower lobe appears as a tapering
curvilinear structure that is wider medially and on
more cephalic sections
• Diaphragm is a continuous curvilinear "line" of
uniform width that moves toward the body wall
on more caudal sections
o Pleural fluid displaces lung from body wall
• Ascites displaces abdominal viscera medially
• Fluid in peritoneal cavity accumulates and spreads
predictably
o First, near site of origin
• E.g., cirrhosis with ascites in right subphrenic and
subhepatic spaces)
o Influence of diaphragmatic motion
• Creates "suction", favoring collection in
subphrenic spaces
o Influence of gravity
• Pelvis is most dependent recess
• Morison pouch (posterior subhepatic space); most
dependent in upper abdomen
• Right paracolic gutter wider and more dependent
than left
• Paracolic gutters are a common pathway for
spread of fluid between upper abdomen and pelvis
o Fluid between mesenteric leaves
• Usually of bowel/mesenteric origin
WALL
1
Cystic mesenteric or omental mass 3
Common
• Loculated ascites
• Abscess
• Metastases
• ~ (E.g., ovarian, cystadenocarcinoma
• Pseudocyst (pancreatitis)
Uncommon
• Pseudomyxoma peritonei
• Mesenteric cyst, lymphangioma
• Cystic mesothelioma
• Urachal cyst
• "Cystic" (caseated) lymph nodes
• ~ Mycobacterial (TB, MAl); Whipple disease
• Has triangular, sharp wedge shape due to
peritoneal reflections
o Lesser sac
• Communicates with greater peritoneal cavity
through an opening in the hepatoduodenal
ligament (foramen of Winslow)
• Most patients with benign transudative ascites will
not have fluid in the lesser sac (unless ascites is
massive)
• Extensive fluid in lesser sac suggests a local source
(such as pancreatitis) or disseminated peritoneal
tumor or infection
o Pouch of Douglas
• = Rectovaginal space (women), rectovesical space
(men)
• Most dependent portion of peritoneal cavity
• Common site for pooling of infected or malignant
ascites (= abscess or peritoneal pelvic implant or
tumor)
Imaging Approaches
• CT is best overall
• Ultrasound can detect small peritoneal implants in the
presence of extensive ascites
• Laparoscopy detects many seed-like tumor implants
missed by imaging
I CLINICAL IMPLICATIONS
Clinical Importance
• Greater omentum
o Functions as "nature's bandage" (adheres to, and
limits the spread of hemorrhage and inflammation
o Usually prevents bowel perforations (e.g.,
diverticulitis, appendicitis) from leading to
generalized peritonitis
o Common site for metastatic disease
• Gastrointestinal tract and ovarian tumors most
common
• Usually accompanied by ascites
o Used by surgeons to cover "raw" surface of cut or
traumatized liver
 PERITONEUM, MESENTERY, AND ABDOMINAL WALL
1 Right Triangular Left Triangular
Ligament of Liver
4 ug,m\OfL'"" .. /
Transverse
Mesocolon

J-Left
Paracolic
~~gr~~olic / Gutter
Gutter--
Sigmoid
Small Bo~wel -- Mesocolon
Mesentary
;

Rectum I
Bladder

Graphic shows eviscerated abdomen and the major Graphic shows fluid in paracolic gutters. Morison pouch
peritoneal reflections. The root of the transverse is the cephalic continuation of the right paracolic gutter
mesocolon separates the upper & lower portions of the & the most dependent recess in the the upper
peritoneal cavity abdomen.

• Ascites • Rounded mass (usually in mesentery, due to NHL)

o Accumulation of fluid in the peritoneal cavity, • "Cystic" masses (usually ovarian
usually due to increased production cystadenocarcinoma)
o Common etiologies: Heart failure, cirrhosis, venous • Pseudomyxoma peritonei (massive, septated
or lymphatic obstruction, peritoneal infection or collections of mucinous fluid; scalloped surface of
malignancy liver and spleen); appendiceal or ovarian cancer
o Attenuation (density) of ascites not very helpful in
determining etiology
• Abscess (intraperitoneal) I CUSTOM DIFFERENTIAL DIAGNOSISI
o Approximately 1/3 contain gas, usually as small
bubbles mixed with fluid Misty (infiltrated) mesentery
o Enhancing wall, rounded margins, and mass effect • Mesenteric edema
are usually seen o Portal hypertension, heart failure, hypoalbuminemia
o Common etiologies: Diverticulitis, Crohn disease, • Lymphedema
surgery (especially involving colon, stomach, biliary o Surgery, congenital, radiation therapy
tree) • Inflammation
• Hemorrhage o Pancreatitis, inflammatory and infectious bowel
o Common etiologies: Trauma, anticoagulation disease
o Appearance varies by age and location o Fibrosing (sclerosing) mesenteritis
o Acute hemorrhage with active extravasation = • Hemorrhage
extravasation of blood isodense to opacified vessels o Trauma, anticoagulation, bowel ischemia
o Clotted blood: Usually 4S to 70 HU, heterogeneous, • Neoplasms
first accumulates near site of hemorrhage (= o Lymphoma, carcinoid, mesothelioma
"sentinel clot" sign) o Ovarian, colon, pancreatic carcinoma
o Lysed clot, free blood in abdomen: Usually 20 to 4S
HU
o Hematocrit effect: Settling of cellular elements; fluid I SELECTED REFERENCES
level; sign of anticoagulation 1. Diihnert W: Radiology Review Manual (4th ed),
• Tumor Philadelphia, Lippincott, Williams and Wilkins. 615-721,
o Common etiologies: Non-Hodgkin lymphoma 2000
(NHL), carcinomas of the GI tract (including 2. Ghahremani GG: Abdominal and Pelvic HHernias: In Gore
pancreas and biliary), ovary and uterus RM, Levine MS (eds) Textbook of Gastrointestinal
Radiology (2nd ed), Philadelphia, WB Saunders.
o Appearance
1993-2009,2000
• Infiltration and thickening of mesentery or 3. Heiken JP, et al: Peritoneal Cavity and Retroperitoneum:
omentum; mesentery may appear "pleated" and Normal Anatomy and Examination Techniques. In Gore
stiff, like a fan RM, Levine MS (eds) Textbook of Gastrointestinal
• Nodular or strand-like soft tissue densities Radiology (2nd ed), Philadelphia, WB Saunders.
• Omental "cake" (soft tissue mass separating bowel 1930-1947, 2000
from anterior abdominal wall)
• Calcified foci (usually ovarian carcinoma or
malignant teratoma)
_P_E_R_I"Ji_O_N_EU_M_, _M_E_S_E_N_T_ER_~_,_A_N_D_A_B_D_O_M_IN_A_L_W_J\_LL_II

IMAGE GALLERY
1
5
Typical
(Left) Axial CECT shows
pleural fluid (arrow) and
ascites (open arrow); note
interface with surface of
liver, spine, and diaphragm
(curved arrow). (Right) Axial
CECT shows "sentinel clot",
high density blood near
splenic laceration, lower
density blood adjacent to
liver.

Typical
(Left) Axial CECT shows
peritoneal and omental
metastases (arrows) from
gallbladder carcinoma.
(Right) Axial CECT shows
"omental cake" (arrow)
between transverse colon
and abdominal wall; ovarian
carcinoma.

Typical
(Left) Graphic shows typical
anterior abdominal wall
hernias; A = ventral,
epigastric; B = umbilical; C =
incisional; 0 = Spigelian.
(Right) Axial NECT shows
bilateral Bochdalek hernias
with cephalic displacement
of kidneys toward the thorax.
 ABDOMINAL ABSCESS
1
6

Axial CECT shows pyogenic post-op abscess (arrows) Axial CECT shows pyogenic post-op abscess after bowel
after bowel resection. Note muldple fluid collections resection. Note muldple fluid collections with enhancing
with enhancing rims; gas is seen only in pelvic abscess rims. Cas noted in pelvic abscess (arrow), but not in
(open arrow). other collections.

o Subphrenic abscess: Pleural effusion and lower lobe

ITERMINOLOGY atelectasis
Definitions • Fluoroscopy
o Abscess sinogram
• Localized abdominal collection of pus
• Useful after percutaneous drainage
• Defines catheter position in dependent portion of
abscess
I IMAGING FINDINGS • Detection of fistulas to bowel, pancreas or biliary
General Features duct
• Best diagnostic clue: Fluid collection with mass effect CT Findings
& enhancing rim with or without gas bubbles or
• NECT: Low attenuation fluid collection, mass effect,
air-fluid level on CECT
gas in 50% of cases
• Location: Anywhere within abdominal cavity;
• CECT: Peripheral rim-enhancement
intraparenchyma; within intra- or extraperitoneal
spaces MR Findings
• Size: Highly variable; 2-15 cm in diameter; • T1WI: Low signal
microabscesses < 2 cm • T2WI: Intermediate to high signal fluid collection
• Morphology: Low density fluid collection with • T1 C+
peripheral enhancing rim o Similar to CECT
o Low signal fluid collection with enhancing rim
Radiographic Findings
• Radiography Ultrasonographic Findings
o Ectopic gas (50% of cases) • Real Time
o Air-fluid level o Complex fluid collection with internal low level
o Soft tissue "mass" echoes, membranes or septations on US
o Focal ileus o Dependent echoes representing debris
o Loss of soft tissue-fat interface o Fluid-fluid level

DDx: Spectrum of Cystic Abdominal lesions Mimicking Abscess

Lymphocele Loculated Ascites Pane Pseudocyst

 ABDOMINAL ABSCESS

Key Facts
1
Terminology Pathology 7
• Localized abdominal collection of pus • General path comments: Pus collection; peripheral
fibrocapillary "capsule"; often polymicrobial from
Imaging Findings enteric organisms
• Best diagnostic clue: Fluid collection with mass effect • Enteric perforation
& enhancing rim with or without gas bubbles or
air-fluid level on CECT Clinical Issues
• Location: Anywhere within abdominal cavity; • Most common signs/symptoms: Fever, chills;
intra parenchyma; within intra- or extraperitoneal abdomen pain; increased heart rate, decreased blood
spaces pressure if septic
• NECT: Low attenuation fluid collection, mass effect, • Variable depending on extent of abscess, patient's
gas in 50% of cases immune system status; excellent prognosis
• CECT: Peripheral rim-enhancement • Percutaneous abscess drainage (PAD)
• Complex fluid collection with internal low level
echoes, membranes or septations on US Diagnostic Checklist
• Best imaging tool: CECT • Diagnostic mimics: Biloma, lymphocele, pseudocyst

o High amplitude linear echoes with reverberation • May contain septations on US

artifacts representing gas bubbles
o Inflamed fat adjacent to abscess: Echogenic mass Pancreatic fluid collection/pseudocyst
• Color Doppler • History of pancreatitis
o Hypervascular periphery • Associated pancreatic necrosis on CECT
o Avascular center of abscess • Location: Highly variable but most often within
o Hyperemic inflamed fat pancreatic parenchyma, lesser sac, anterior pararenal
space, transverse mesocolon
Nuclear Medicine Findings • Pseudocyst requires several weeks to develop
• Gallium scan peripheral pseudocapsule
o Useful for fever of unknown origin
o Nonspecific: Positive with tumor such as lymphoma
and granulomatous lesions !PATHOlOGY
• WBC scan
o 73-83% sensitivity General Features
o False positives with bowel infarct or hematoma • General path comments: Pus collection; peripheral
• Newer agents fibrocapillary "capsule"; often polymicrobial from
o Indium-labeled polyclonal IgG enteric organisms
o Tc99m-labeled monoclonal antibody • Genetics
o Increased risk if genetically altered immune response
Imaging Recommendations o Diabetics have increased incidence. of gas-forming
• Best imaging tool: CECT abscesses
• Protocol advice: Oral & LV. contrast, 150 ml LV. • Etiology
contrast at 2.5 ml/sec o Enteric perforation
• Appendicitis
• Diverticulitis
I DIFFERENTIAL DIAGNOSIS • Crohn disease
o Post-operative
lymphocele • Typically intraperitoneal spaces such as cul-de-sac,
• History of lymph node dissection Morison pouch and subphrenic spaces
• Fluid collections with mass (often bilateral) along o Bacteremia
lymphatic drainage o Trauma
• Attenuation values -10 HU to +10 HU • Epidemiology
Biloma o Most commonly due to post-operative complication
• History of biliary or hepatic surgery o Microabscesses due to fungal infections in
immunocompromised patients
• Perihepatic fluid collection commonly in gallbladder
fossa or Morison pouch o Higher incidence in diabetics, immunocompromised
• Attenuation value 0-15 HU patients and posto-perative patients

loculated ascites Gross Pathologic & Surgical Features

• Evidence for cirrhosis or chronic liver disease • Often adherent omentum or bowel loops; pus
collection
• Minimal or no mass effect
• Often passively conforms to peritoneal space • Mayor may not have "capsule"
 ABDOMINAL ABSCESS
1 Microscopic Features Image Interpretation Pearls
8 • PMN and white cell debris • Half of abscesses don't contain gas or air-fluid levels;
• Bacteria, fungi detected mass effect & enhancing rim highly suggestive in
appropriate clinical context
Staging, Grading or Classification Criteria
• Organism: Bacterial, fungal amebic
• Related to organ of origin (i.e., liver abscess) I SELECTED REFERENCES
• Intraperitoneal
1. Men S et al: Percutaneous drainage of abdominal abcess.
• Extraperitoneal
Eur] Radiol. 43(3):204-18, 2002
• Communicating 2. Benoist S et al: Can failure of percutaneous drainage of
o Underlying fistula to GI tract postoperative abdominal abscesses be predicted? Am] Surg.
o Connection to biliary tract or pancreatic duct 184(2):148-53, 2002
3. Cinat ME et al: Determinants for successful percutaneous
image-guided drainage of intra-abdominal abscess. Arch
I CLINICAL ISSUES Surg. 137(7):845-9, 2002
4. Betsch A et al: CT-guided percutaneous drainage of
Presentation intra-abdominal abscesses: APACHEIII score stratification
• Most common signs/symptoms: Fever, chills; of I-year results. Acute Physiology, Age, Chronic Health
Evaluation. Eur Radiol. 12(12):2883-9,2002
abdomen pain; increased heart rate, decreased blood
5. Ralls PW: Inflammatory disease of the liver. Clin Liver Dis.
pressure if septic 6(1):203-25, 2002
• Clinical profile: Leukocytosis, + blood cultures and 6. Harisinghani MG et al: CT-guided transgluteal drainage of
elevated ESR deep pelvic abscesses: indications, technique,
procedure-related complications, and clinical outcome.
Demographics Radiographics. 22(6):1353-67, 2002
• Age: Any age 7. Lohela P: Ultrasound-guided drainages and sclerotherapy.
• Gender: M = F Eur Radiol. 12(2):288-95, 2002
8. Maggard MA et al: Surgical diverticulitis: treatment
Natural History & Prognosis options. Am Surg. 67(12):1185-9, 2001
• Variable depending on extent of abscess, patient's 9. vanSonnenberg E et al: Percutaneous abscess drainage:
immune system status; excellent prognosis update. World] Surg. 25(3):362-9; discussion 370-2,2001
10. Green BT:Splenic abscess: report of six cases and review of
Treatment the literature. Am Surg. 67(1):80-5, 2001
• Options, risks, complications 11. Deck A] et al: Perinephric abscesses in the neurologically
impaired. Spinal Cord. 39(9):477-81, 2001
o Percutaneous abscess drainage (PAD)
12. ]acobs]E et al: Computed tomography evaluation of acute
• 80% success rate of percutaneous drainage pancreatitis. Semin Roentgenol. 36(2):92-8, 2001
• Patient selection critical for success 13. Krige]E et al: ABC of diseases of liver, pancreas, and biliary
• Best candidates for PAD have well-localized, system. BMJ. 322(7285):537-40, 2001
fluid-filled abscesses> 3 cm with safe catheter 14. Zibari GB et al: Pyogenic liver abscess. Surg Infect
access route (Larchmt). 1(1):15-21,2000
o Contraindications for PAD related to patient 15. Sirinek KR:Diagnosis and treatment of intra-abdominal
• Coagulopathy with prothrombin time> 3 sec abscesses. Surg Infect (Larchmt). 1(1):31-8,2000
16. Barakate MS et al: Pyogenic liver abscess: a review of 10
• International normalized ratio> 1.5 years' experience in management. Aust N Z] Surg.
• Platelets < 50,000 uL 69(3):205-9, 1999
o Contraindications for PAD related to abscess 17. Barakate MS et al: Pyogenic liver abscess: a review of 10
• Infected necrosis (i.e., pancreatic abscess) years' experience in management. Aust N Z] Surg.
• Gas-forming infection such as emphysematous 69(3):205-9, 1999
pancreatitis 18. Kimura K et al: Amebiasis: modern diagnostic imaging with
• Soft tissue infection (i.e., phlegmon) pathological and clinical correlation. Semin Roentgenol.
• No safe access route for catheter insertion 32(4):250-75, 1997
19. Montgomery RS et al: Intraabdominal abscesses:
o Surgery indications
image-guided diagnosis and therapy. Clin Infect Dis.
• Extensive intraperitoneal abscesses 23(1):28-36, 1996
• Debridement of necrotic infected tissue 20. Snyder SK et al: Diagnosis and treatment of
• Failed PAD intra-abdominal abscess in critically ill patients. Surg Clin
o Antibiotic therapy North Am. 62(2):229-39, 1982
• Abscesses < 3 cm

I DIAGNOSTIC CHECKLIST
Consider
• Diagnostic mimics: Biloma, lymphocele, pseudocyst
 ABDOMINAL ABSCESS
I IMAGE GALLERY 1
9
Typical
(Left) Axial CECT
demonstrates gas-forming
pyogenic liver abscess in
diabetic patient. Note
air-fluid level within abscess
cavity (arrow). (Right) Axial
US shows liver abscess
demonstrating linear high
amplitude echoes with
"dirty" distal acoustic
shadowing representing gas
(arrow).

Typical
(Left) Axial CECT of amebic
abscess. Note peripheral low
attenuation zone of edema
(arrow) surrounding abscess.
(Right) Sagittal US of amebic
abscess. Note low level
echoes (arrow) within
hypoechoic mass and lack of
distal acoustic enhancement.

Typical
(Left) Sagittal US of fungal
microabscesses due to
systemic Candidiasis. Note
multiple "target" lesions
(arrows). (Right) Sagittal US
of spleen demonstrates
abscess with low-level
echoes (arrow).
 PERITONITIS
1
10

Axial CECT of perforated appendicitis with peritonitis. Axial CECT of perforated appendicitis with peritonitis
Note linear appendicolith (arrow), symmetric shows multiple appendicoliths (arrow) with
thickening of the peritoneum (open arrow) and nonenhancing necrotic tip of appendix (open arrow) &
adjacent low attenuation pus. surrounding soft tissue infiltration.

• Medial displacement of cecum and ascending

ITERMINOLOGY colon; present in 90% of patients with significant
Definitions ascites
o +/- Free air
• Infectious or inflammatory process involving
o Hydropneumoperitoneum
peritoneum or peritoneal cavity
o Air in lesser sac with perforated gastric ulcer
• Contrast studies
o Perforated ulcer with contrast leak on UGI
IIMAGING FINDINGS o Perforation of diverticulum in diverticulitis on
General Features contrast enema
• Best diagnostic clue: Ascites, symmetric enhancement CT Findings
of peritoneum with fat stranding of abdominal fat
• CECT
• Location: Peritoneal surface, mesentery, omentum o Ascites, enhancing peritoneum with smooth
• Size: Variable, may be focal or diffuse thickening, infiltration and soft tissue stranding of
• Morphology: Symmetric thickening of peritoneum fat within mesentery on CECT
Radiographic Findings o +/- Gas bubbles, low attenuation nodes in TB
• Radiography peritonitis
o Evidence of ascites: More than 500 ml of fluid MR Findings
required for plain film diagnosis • Tl WI: Low attenuation peritoneal fluid
• Bulging of flanks • T2WI: High attenuation peritoneal fluid
• Indistinct psoas margin
• Tl C+
• Small bowel loops floating centrally o Thickened enhancing peritoneum
• Lateral edge of liver displaced medially (Hellmer o Low attenuation peritoneal fluid
sign); visible in 80% of patients with significant
ascites Ultrasonographic Findings
• Pelvic "dog's ear"; present in 90% of patients with • Real Time
significant ascites

DDx: Spectrum of Intraperitoneal Fluid

Carcinomatosis Benign Ascites Pseudomyxoma Hemoperitoneum

 PERITONITIS

Key Facts
1
Terminology Top Differential Diagnoses 11
• Infectious or inflammatory process involving • Peritoneal carcinomatosis
peritoneum or peritoneal cavity • Benign ascites
• Pseudo myxoma peritonei
Imaging Findings
• Hemoperitoneum
• Best diagnostic clue: Ascites, symmetric enhancement
of peritoneum with fat stranding of abdominal fat Pathology
• Ascites, enhancing peritoneum with smooth • General path comments: Pus in peritoneal cavity,
thickening, infiltration and soft tissue stranding of fat thickened peritoneum or mesentery
within mesentery on CECT
• Tl WI: Low attenuation peritoneal fluid Clinical Issues
• T2WI: High attenuation peritoneal fluid • Most common signs/symptoms: Fever, abdominal
• Peritoneal fluid, septations, thickened echogenic pain and distension
mesentery on US Diagnostic Checklist
• Best imaging tool: CECT
• Peritoneal carcinomatosis
• Symmetric enhancement of thickened peritoneum

o Peritoneal fluid, septations, thickened echogenic • Scalloping of liver and spleen contour
mesentery on US • Rupture of mucinous tumor of appendix
o Dilated fallopian tube with fluid-debris level • Calcified cystic implants
(pyosalpinx) in pelvic inflammatory disease (pm) • Cystic masses attached to ligaments such as falciform
o Complex adnexal cystic masses in PID or gastrohepatic ligament
• Tubo-ovarian abscesses (TOA)
• Color Doppler
Hemoperitoneum
o Hyperemic thickened echo genic fat • High attenuation intraperitoneal fluid
• Associated with gastrointestinal source of • Free lysed blood measuring 30-45 Hounsfield units
inflammation (HU)
• Clotted blood measuring 60 HU
Imaging Recommendations • Active arterial extravasation
• Best imaging tool: CECT o Isodense with adjacent major arterial structures
• Protocol advice o Large surrounding hematoma
o Oral and IV contrast (150 ml injected at 2.5ml/sec)
o Rectal contrast to distinguish colon from pelvic
infection I PATHOLOGY
o 5 mm collimation with 5 mm reconstruction
interval General Features
• General path comments: Pus in peritoneal cavity,
thickened peritoneum or mesentery
I DIFFERENTIAL DIAGNOSIS • Etiology
o Spontaneous
Peritoneal carcinomatosis • Secondary bacterial infection of chronic ascites
• Nodular implants on peritoneum • Younger patients have higher incidence of
• Omental caking pneumococcal or hemolytic streptococcal
• Ascites infection
• Mesenteric nodules and adenopathy o Bacterial
• Bowel perforation
Benign ascites • Pelvic inflammatory disease (pm)
• Cirrhosis • Infected intrauterine device (IUD)
• Bile leak • Ruptured tubo-ovarian abscess
o Caused by trauma, surgery, liver biopsy, biliary • Gastric or duodenal ulcer
drainage • Ruptured appendicitis
• Pancreatic ascites • Ruptured diverticulitis
o Caused by pancreatic duct leakage o TB
• Chylous ascites • Ingestion of tuberculous sputum with
• Urine ascites development of TB peritonitis
o Caused by bladder perforation o Traumatic
• Congestive heart failure (CHF), fluid overload • Duodenum, jejunum, distal ileum most common
Pseudomyxoma peritonei sites
.' Small bowel injury may present 4-6 weeks
• Massive accumulation of gelatinous ascites in
post-trauma
peritoneal cavity
 PERITONITIS
1 • Bowel injury from deceleration injury
• Colonic injuries rare, but have rapid clinical onset
I DIAGNOSTIC CHECKLIST
12 of peritonitis Consider
o Iatrogenic • Peritoneal carcinomatosis
• Inadvertent bowel perforation during laparotomy • Causes of water-attenuation ascitic fluid
or diagnostic/therapeutic paracentesis o Transudate (e.g., cirrhosis)
• Post-operative anastomotic leak o Urine
• Retained foreign body during surgery o Chyle
• Dropped gallstones during laparoscopic o Bile
cholecystectomy o Pancreatic juice
• Epidemiology
o Increased incidence in patients with chronic ascites Image Interpretation Pearls
• Cirrhosis • Symmetric enhancement of thickened peritoneum
• Peritoneal dialysis • Inflammatory changes with adjacent fat of mesentery
o Increased incidence in patients with risk factors for and omentum
PID • Enlarged fallopian tube (pyosalpinx) with fluid-fluid
• IUD level & complex adnexal mass (TOA) in PID
• Multiple sexual partners
Gross Pathologic & Surgical Features
I SELECTED REFERENCES
• Pus in peritoneal cavity
• Inflammatory changes in mesentery 1. Shetty H et al: Treatment of infections in peritoneal
dialysis. Contrib Nephrol. (140):187-94, 2003
• Inflammatory adhesions
2. Alberti LE et al: Spontaneous bacterial peritonitis in a
• Hyperemia of adherent omentum or mesentery patient with myxedema ascites. Digestion. 68(2-3):91-3,
Microscopic Features 2003
3. Cheadle WG et al: The continuing challenge of
• > 500 leukocytes per mm3 indicates infected ascites intra-abdominal infection. Am] Surg. 186(5A):15S-22S;
discussion 31S-34S, 2003
Staging, Grading or Classification Criteria
4. Troidle L et al: Continuous peritoneal dialysis-associated
• Localized peritonitis: a review and current concepts. Semin Dial.
o Walled off infection 16(6):428-37, 2003
• Diffuse 5. Yao V et al: Role of peritoneal mesothelial cells in
o Multiple peritoneal compartments involved peritonitis. Br] Surg. 90(10):1187-94, 2003
6. Marshall]C et al: Intensive care unit management of
intra-abdominal infection. Crit Care Med. 31(8):2228-37,
I CLINICAL ISSUES 7.
2003
Witte MB et al: Repair of full-thickness bowel injury. Crit
Care Med. 31(8 Suppl):S538-46, 2003
Presentation
8. Malangoni MA: Current concepts in peritonitis. Curr
• Most common signs/symptoms: Fever, abdominal pain Gastroenterol Rep. 5(4):295-301, 2003
and distension 9. Chow KM et al: Indication for peritoneal biopsy in
tuberculous peritonitis. Am] Surg. 185(6):567-73,2003
Demographics 10. Sabri M et al: Pathophysiology and management of
• Age: All ages pediatric ascites. Curr Gastroenterol Rep. 5(3):240-6, 2003
• Gender: No predilection for male or female 11. Veroux M et al: A rare surgical complication of Crohn's
diseases: free peritoneal perforation. Minerva Chir.
Natural History & Prognosis 58(3):351-4,2003
• Sepsis if not treated promptly 12. Reijnen MM et al: Pathophysiology of intra-abdominal
• Prognosis determined by primary etiology adhesion and abscess formation, and the effect of
o Excellent if localized and no evidence of septicemia hyaluronan. Br] Surg. 90(5):533-41, 2003
o Poor if generalized peritonitis and gram-negative 13. Hanbidge AE et al: US of the peritoneum. Radiographies.
23(3):663-84; discussion 684-5, 2003
septicemia 14. Runyon BA:Strips and tubes: improving the diagnosis of
Treatment spontaneous bacterial peritonitis. Hepatology. 37(4):745-7,
2003
• Options, risks, complications 15. Brook I: Microbiology and management of intra-abdominal
o Etiology of peritonitis determines treatment infections in children. Pediatr Int. 45(2):123-9, 2003
o Correct underlying cause (Le., perforated ulcer) 16. Sivit CJ et al: Imaging of acute appendicitis in children.
o Antibiotic therapy Semin Ultrasound CT MR. 24(2):74-82, 2003
• Early PID 17. Nishie A et al: Fitz-Hugh-Curtis syndrome. Radiologic
• Soft tissue inflammation (phlegmon) from manifestation.] Comput Assist Tomogr. 27(5):786-91, 2003
appendicitis or diverticulitis
o Surgery for failed antibiotic therapy
o Surgery for perforated viscus
• Appendicitis
• Duodenal ulcer
• Diverticulitis
 PERITONITIS
I IMAGE GALLERY 1
13
Typical
(Left) Axial GCT of
spontaneous bacterial
peritonitis. Note marked
ascites. (Right) Axial GCT of
spontaneous bacterial
peritonitis. Note symmetric
thickening of parietal
peritoneum in left flank
(arrow).

Typical
(Left) Axial GCT of TB
peritonitis demonstrates
ascites, nodular thickening of
omentum (arrow) and
peritoneum. (Right) Axial
CECT of traumatic bowel
perforation with serosal
inflammation and peritonitis.
Note pneumoperitoneum
(arrow) and hyperemic
thickened small bowel (open
arrow).

Typical
(Left) Axial CECT of
gallbladder perforation with
bile peritonitis and
hemoperitoneum. Note c/ot
in gallbladder (arrow) and
massive perihepatic low
density fluid. (Right) Axial
CECT of gallbladder
perforation w/bile peritonitis
& hemoperitoneum. Note
interruption of gallbladder
wall (arrow) w/adjacent c/ot
(open arrow), and large
amount of bile in peritoneal
cavity.
 SCLEROSING MESENTERITIS
1
14

Axial CECT shows infiltrative mesenteric mass that Axial CECT shows mildly thickened bowel wall and
encases blood vessels. Note engorgement of mesenteric mesenteric venous congestion due to fibrosing
veins. mediastinitis.

• Key concepts
ITERMINOLOGY o Rare, benign, nonspecific process involving
Abbreviations and Synonyms mesenteric fat
• Retractile mesenteritis, fibrosing mesenteritis, o Histologically: Classified into three types based on
mesenteric panniculitis, mesenteric lipodystrophy, predominant tissue type in the mass
liposclerotic mesenteritis, systemic nodular • Mesenteric panniculitis: Chronic/acute
panniculitis inflammation & fat necrosis more than fibrosis
• Mesenteric lipodystrophy: Fat necrosis more than
Definitions inflammation & fibrosis
• Complex mesenteric inflammatory disorder of • Retractile mesenteritis: Fibrosis/retraction more
unknown etiology than inflammation/fat necrosis
o Retractile mesenteritis
• Considered as final, more invasive/chronic form
IIMAGING FINDINGS o Often associated with other idiopathic inflammatory
disorders (more than one condition may be present)
General Features o May coexist with malignancy (e.g., lymphoma,
• Best diagnostic clue: Fibrofatty mesenteric mass that breast/lung/colon cancer & melanoma)
encases mesenteric vessels but preserves a fat halo
around vessels Radiographic Findings
• Location • Fluoroscopic guided barium study
o Most common site: Root of small bowel mesentery o Involved bowel loops
o Occasionally: Colon (transverse/rectosigmoid) • Dilated/ displaced/ fixed/narrowed/tethering
o Rarely: Peripancreatic, omentum & retroperitoneum o Fold thickening: Submucosal infiltration/edema
• Morphology o Thumbprinting of bowel wall
o Mostly characterized by a mixture of mesenteric of: • Submucosal edema due to ischemia/lymphedema
• Chronic inflammation o Preservation of mucosal pattern
• Fat necrosis & fibrosis • Clue in differentiating from carcinoma
o Mass that is often obscured in leaves of mesentery o Luminal narrowing
• Common finding in retractile mesenteritis

DDx: Infiltrated Mesentery +/- Mass

Treated Lymphoma Carcinoid Cirrhosis Oesmoid Tumor

 SCLEROSING
Key Facts
Terminology
• Retractile mesenteritis, fibrosing mesenteritis,
mesenteric panniculitis, mesenteric lipodystrophy,
liposclerotic mesenteritis, systemic nodular
panniculitis
• Complex mesenteric inflammatory disorder of
unknown etiology
Imaging Findings
• Best diagnostic clue: Fibrofatty mesenteric mass that
encases mesenteric vessels but preserves a fat halo
around vessels
• Area of subtle increased attenuation in mesentery
(inflamed fat)
• Calcification; enlarged mesenteric lymph nodes
• Fatty necrotic cystic mass may be seen
CT Findings
• Findings vary depending on predominant tissue
o Area of subtle increased attenuation in mesentery
(inflamed fat)
• May be solitary/multiple, well-Jill-defined
o Calcification; enlarged mesenteric lymph nodes
o Fatty necrotic cystic mass may be seen
o May show Infiltration of pancreas or portahepatis
o Encasement of mesenteric vessels & collateral vessels
• Narrowing/occlusion seen on contrast study
o "Fat ring" sign: Preservation of fat around vessels
• Hypodense fatty halo surrounding mesenteric
vessels & nodules
• Predominantly seen in mesenteric panniculitis
• Differentiates sclerosing mesenteritis from other
mesenteric processes (e.g., lymphoma, carcinoid
tumor, carcinomatosis)
o Pseudocapsule: Peripheral band of soft tissue
attenuation that limits normal mesentery from
inflammatory process
• Seen in mesenteric panniculitis phase
• Enhancement of pseudocapsule may be seen
o "Misty mesentery": Nonspecific sign
• Increased attenuation of mesentery
• Evidence of small mesenteric nodes seen
• No discrete soft tissue mass
• Seen in any pathology that infiltrates mesentery
o Thickening/infiltration/displacement/narrowing of
bowel loops
o Solid soft tissue mass usually in root of small bowel
mesentery (fibrous tissue)
• Single/large/lobulated or ill-defined increased
density mass with linear radiating strands
(fibroma-rare)
• Small mesenteric soft tissue nodules of increased
density (fibromatosis)
• CTA
o Delineates relationship of mass to mesenteric
vasculature
MESENTERITIS
1
• "Fat ring" sign: Preservation of fat around vessels 15
• Pseudocapsule: Peripheral band of soft tissue
attenuation that limits normal mesentery from
inflammatory process
Top Differential Diagnoses
• Non-Hodgkin lymphoma
• Carcinoid tumor
• Mesenteric edema
• Desmoid tumor (fibromatosis)
• Carcinomatosis (mesenteric metastases)
Diagnostic Checklist
• "Fat ring" sign: Differentiates sclerosing mesenteritis
from lymphoma, carcinoid & mesenteric metastases
• Check for other idiopathic inflammatory disorders &
malignancies (breast/lung/colon cancers; melanoma)
MR Findings
• Variable signal intensity: Due to
inflammation/ fat/ fibrosis/vascular/ Ca ++
• Mesenteric panniculitis & lipodystrophy
o Tl WI: Mixed signal intensity
o T2WI: Mixed signal intensity
• Retractile mesenteritis: In mature fibrotic reaction
o Tl WI: Decreased signal intensity
o T2WI: Very low signal intensity
o Gradient-echo MR image with flip angle 30°
• Narrowing/occlusion of flow in mesenteric vessels
• Collateral vessels are seen
Imaging Recommendations
• CT with 3D volume rendering is optimal study
I DIFFERENTIAL DIAGNOSIS
Non-Hodgkin lymphoma
• Large discrete/confluent mesenteric nodes
• Lymphoma, no calcification unless treated
• Nodal mass in root of mesentery may mimic sclerosing
mesenteritis
• Preservation of fat ring sign favors a diagnosis of
sclerosing mesenteritis
Carcinoid tumor
• Discrete enhancing mass in bowel wall
• Hypervascular liver metastases
• Increased urinary S-HIAA
• Mimic sclerosing mesenteritis due to
o Ill-defined, infiltrating soft tissue mass in root of
mesentery with associated calcification &
desmoplastic reaction
o Preservation of fat ring sign favors a diagnosis of
sclerosing mesenteritis
Mesenteric edema
• Fluid infiltrates mesentery & increases attenuation of
mesenteric fat, simulating sclerosing mesenteritis
• Seen in conditions like
o Cirrhosis, hypoalbuminemia, heart failure
 SCLEROSING MESENTERITIS
1 o Portal or mesenteric vein thrombosis,
o Pancreatitis
vasculitis
Demographics
16 • Age: 2nd-8th decade of lifei average age 60-70 years
Desmoid tumor (fibromatosis) • Gender: M:F = 1.8:1
• Usually discrete solid mass
• Associated with Gardner syndrome
Natural History & Prognosis
• Occur in traumatized sites • Complications
• Diagnosis: Biopsy & histologic analysis o Bowel or ureteral obstruction
o Ischemia due to mesenteric vessels narrowing
Carcinomatosis (mesenteric metastases) • Prognosis
• Mesenteric implants can mimic CT appearance of o Partial or complete resolution
sclerosing mesenteritis o Nonprogressive course or aggressive course
• Implants: Not only root of mesentery, but also seen in
omentum, surface of liver, spleen or bowel Treatment
• Calcification: In case of mucinous adenocarcinoma • Steroids, colchicine, immunosuppressive agents
o Example: Ovarian or colon cancer o Effective before fibrotic change
• Ascites is common in carcinomatosis • Surgical excision
o Fibrosis & retraction with obstructive symptoms

I PATHOLOGY
General Features
• General path comments
o Excessive fat deposition ~ inflammation ~ fatty
degeneration/necrosis
o Fibrosis ~ thickened mesentery contracts ~
adhesions ~ nodular changes
• Etiology
o Exact etiology remains unknown
o Possible causative factors
• Autoimmune, infection, trauma, ischemia
• Prior abdominal surgery, neoplastic
• Epidemiology
o Prevalence: 0.6%
o Peak incidence in 6th & 7th decades
• Associated abnormalities
o Idiopathic inflammatory disorders
• Retroperitoneal fibrosis, sclerosing cholangitis
• Riedel thyroiditis, orbital pseudotumor
Gross Pathologic & Surgical Features
• Encapsulated firm/hard masses
• Nodules of fat, areas of necrosis & fibrosis
• Thickened mesentery/adhesions/displaced bowel loops
Microscopic Features
• Fat with lipid-laden macrophages & fibrous septa
• Lymphocytes/plasma cells/eosinophilsi calcifications
• Invasion of bowel muscle/submucosa
• Mucosa is spared
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Abdominal pain, fever, nausea, vomiting, weight
loss, diarrhea
o Abdominal tenderness & palpable mass
o Incidental finding in an asymptomatic patient
• Lab data
o Increased ESR & decreased hematocrit (anemia)
o PAS positive (histological DDx-Whipple)
• Diagnosis
o Percutaneous or surgical excisional biopsy
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other pathologies which can mimic sclerosing
mesenteritis
Image Interpretation Pearls
• CT appearances vary depending on predominant
tissue component (fat, inflammation, or fibrosis)
• "Fat ring" sign: Differentiates sclerosing mesenteritis
from lymphoma, carcinoid & mesenteric metastases
• Check for other idiopathic inflammatory disorders &
malignancies (breast/lung/colon cancerSi melanoma)
I SELECTED REFERENCES
1. Horton KM et al: CT findings in sclerosing mesenteritis
(panniculitis): spectrum of disease. Radiographies. 23(6):
1561-7, 2003
2. Seo BK et al: Segmental misty mesentery: analysis of CT
features and primary causes. Radiology. 226(1): 86-94, 2003
3. Lawler LP et al: Sclerosing mesenteritis: depiction by
multi detector CT and three-dimensional volume
rendering. A]RAm] Roentgenol. 178(1): 97-9, 2002
4. Horton KM et al: Volume-rendered 3D CT of the
mesenteric vasculature: normal anatomy, anatomic
variants, and pathologic conditions. Radiographies. 22(1):
161-72, 2002
5. Daskalogiannaki M et al: CT evaluation of mesenteric
panniculitis: prevalence and associated diseases. A]RAm]
Roentgenol. 174(2): 427-31, 2000
6. Sabate ]M et al: Sclerosing mesenteritis: imaging findings
in 17 patients. A]R Am] Roentgenol. 172(3): 625-9, 1999
7. Fujiyoshi F et al: Retractile mesenteritis: small-bowel
radiography, CT, and MR imaging. A]R Am] Roentgenol.
169(3): 791-3, 1997
8. Mindelzun RE et al: The misty mesentery on CT:
differential diagnosis. A]R Am] Roentgenol. 167(1): 61-5,
1996
9. Kronthal A] et al: MR imaging in sclerosing mesenteritis.
AjR Am] Roentgenol. 156(3): 517-9, 1991
 SCLEROSING MESENTERITIS
I IMAGE GALLERY 1
17
Typical
(Left) Axial CECT shows
mesenteric mass encasing
vessels, causing mesenteric
venous distention. (Right)
Axial CECT shows focal
calcification within
mesenteric mass (arrow) and
vascular engorgement.

Typical
(Left) Axial CECT in a 59
year old woman with "misty
mesentery" and mesenteric
adenopathy due to
mesenteritis. (Right) Axial
CECT in a 59 year old
woman with mesenteritis
and subtle infiltration of
~

/.'.'
',:
'./;:.
. .'
J ..•••..
~;,. : It ,.
mesenteric fat.

I)
•• y
,.1,' '0: .

Typical
(Left) Axial CECT of an 84
year old patient with
retractile mesenteritis. Note
calcified, spiculated mass
that encases vessels and
distorts bowel. (Right) Axial
CECT in a patient with
dermatomyositis (note
subcutaneous calcification,
arrow) along with sclerosing
mesenteritis .
 ASCITES
1
18

Axial CECT shows ascites due to hepatic cirrhosis, with Axial CECT shows massive ascites due to right heart
large varices and splenomegaly. failure.

• Exceptions: Tense ascites, local source (gastric

ITERMINOlOGY ulcer or pancreatitis)
Abbreviations and Synonyms • Otherwise, usually due to carcinomatosis or
• Intraperitoneal fluid collection infected ascites
• Morphology
Definitions o Free-flowing fluid: Shaped by surrounding structures
• Pathologic accumulation of fluid within peritoneal & does not deform normal shape of adjacent organs
cavity • Fluid insinuates itself between organs
o Loculated: Rounded, bulging contour, encapsulated
• Does not conform to organ margins
jlMAGING FINDINGS • Key concepts
o Transudate, exudate, hemorrhagic, pus
General Features o Chylous, bile, pancreatic, urine, cerebrospinal fluid
• Best diagnostic clue: Diagnostic paracentesis o Pseudomyxoma peritonei, neonatal ascites
• Location
o In uncomplicated cases, fluid flows to most Radiographic Findings
dependent position • Plain abdominal film: Insensitive for diagnosis
o Morison pouch (hepatorenal fossa) o Hellmer sign; lateral edge of liver medially displaced
• Most dependent upper abdominal recess from adjacent thoracoabdominal wall
o Pelvis o Obliteration of hepatic & splenic angle
• Most dependent space o Symmetric densities on sides of bladder ("dog's ear")
o Paracolic gutters o Medial displacement of ascending & descending
• Lateral to ascending & descending colon colon; lateral displacement of properitoneal fat line
o Subphrenic spaces o Indirect signs: Diffuse abdominal haziness; bulging
• Not dependent, but fill due to suction effect of of flanks; poor visualization of psoas & renal outline
diaphragmatic motion • Separation of small bowel loops; centralization of
o Lesser sac floating gas-containing small bowel
• Usually does not fill with ascites o Chest film: Elevation of diaphragm, sympathetic
pleural effusion; with massive ascites

DDx: Fluid in Peritoneal Cavity

Hemoperitoneum pseudonmyx. Peril. Ovarian Mets Ovarian Tumor

 ASCITES
Key Facts
Terminology
• Pathologic accumulation of fluid within peritoneal
cavity
Imaging Findings
• Simple ascites: Low density free fluid collection
• 0-30 Hounsfield units (HU); transudate fluid
• Small amounts seen in right perihepatic space,
Morison pouch, pouch of Douglas
• Larger amounts of fluid in paracolic gutters
• Massive ascites; distends peritoneal spaces
• Associated evidence of liver, heart, kidney failure
• Complicated ascites: Exudates; infection,
inflammation, malignancy
• Loculated ascites: Adhesions, malignancy, or
infection
CT Findings
• Simple ascites: Low density free fluid collection
o 0-30 Hounsfield units (HU); transudate fluid
o Small amounts seen in right perihepatic space,
Morison pouch, pouch of Douglas
o Larger amounts of fluid in paracolic gutters
• Centralization of bowel loops; triangular
configuration within leaves of mesentery
o Massive ascites; distends peritoneal spaces
o Associated evidence of liver, heart, kidney failure
• Liver diseases; around liver (92%), pelvis (77%),
para colic gutters (69%), Morison pouch (63%)
• CT findings in other intraperitoneal collections
o Exudates: Density of ascitic fluid increases with
increasing protein content
o pseudomyxoma peritonei: Large low attenuation
collection; multiseptate, loculations
• Multiple cystic-appearing masses; calcification
• Thickening of peritoneal & omental surface
• Scalloping of liver & spleen contour
o Chylous ascites: Less than 0 HU; intraperitoneal &
extra peritoneal water density fluid (in trauma)
o Bile ascites: Less than 20 HU; typically in right or
left supramesocolic spaces
• Located adjacent to liver or biliary structures
• Bilomas have sharp margins
o Urine ascites: Nonspecific CT appearance
• Intravenously administered contrast material
accumulates after renal concentration & excretion
o Cerebrospinal fluid ascites: Small amounts of free
fluid normal with ventriculoperitoneal shunt
• Localized collection in association with tip of
shunt tube; pathologic, implies malfunction
o Pancreatic ascites: Peripancreatic, lesser sac, anterior
pararenal space
• Disruption of pancreatic duct or severe
pancreatitis
MR Findings
• Transudate: Hypointense on T1WI
o Hyperintense on T2WI
• Exudate: Intermediate to short T1 & long T2 values
1
Top Differential Diagnoses 19
• Hemoperitoneum
• Malignant ascites
• Infectious ascites
• Cystic peritoneal metastases
Clinical Issues
• Diagnosis: Paracentesis; US guidance or blind tap
• Complication: Spontaneous bacterial peritonitis,
respiratory compromise, anorexia
Diagnostic Checklist
• Difficult to characterize nature & underlying cause of
peritoneal fluid collections on basis of imaging alone
o T1 relaxation time decreases with increased protein
• Ascites hyperintense on gradient-echo images (fluid
motion; transmitted pulsations)
Ultrasonographic Findings
• Uncomplicated ascites: Homogeneous, freely mobile,
anechoic collection; deep acoustic enhancement
o Free fluid: Acute angles where fluid borders organs
• Shifts with change in patient position
• Compresses with increased transducer pressure
• Complicated ascites: Exudates; infection,
inflammation, malignancy
o Internal echoes: Coarse (blood); fine (chyle)
o Loculation; atypical fluid distribution
o Multiple septa (tuberculous peritonitis,
pseudomyxoma peri tone i)
o Matted or clumped; infiltrated bowel loops
o Thickened interfaces between fluid & adjacent
structures; peritoneal lining, omental thickening
• Loculated ascites: Adhesions, malignancy, or infection
o No movement with change in patient position
o Non compressible with increased probe pressure
• Thickening of gall bladder wall; more than 3 mm in
benign ascites; in carcinomatosis less than 3 mm thick
• Small free fluid in cul-de-sac; physiologic in women
• Sonolucent band; small amounts of fluid in Morison
pouch, around liver
• Polycyclic, "lollipop", arcuate appearance
o Small bowel loops arrayed on either side of vertically
floating mesentry; seen with massive ascites
• Transverse & sigmoid colon usually float on top of
fluid (non dependent gas content when patient supine)
o Ascending & descending colon do not float
• Right kidney may be displaced anteriorly & laterally
• Triangular fluid cap; overdistended bladder obscures
o Fluid displaced to peritoneal reflection adjacent to
uterine fundus
Imaging Recommendations
• us: Simplest, sensitive, small volume (5-10 ml)
visualized, cost -effective
• CT: If US equivocal; as part of diagnostic work-up for
cause, nature of ascites
 ASCITES
1 I DIFFERENTIAL DIAGNOSIS
20
Hemoperitoneum
• High attenuation fluid; > 30 HU (30-60 HU)
o Unless present for> 48 hours or diluted by ascites,
urine, bile, bowel contents
o Active hemorrhage: Fluid collection isodense to
contrast-enhanced blood vessels
• Sentinal clot: Focal heterogeneous collection of high
density (> 60 HU); accumulates near site of bleed
Malignant ascites
• Loculated collections; fluid in greater & lesser sac
• Bowel loops tethered along posterior abdominal wall
• Thickening of peritoneum; peritoneal seeding
• Hepatic, splenic, lymph node lesions; mass arising
from ovary, gut, pancreas
Infectious ascites
• Higher attenuation fluid (20-30 HU)
• Partialloculations; multiple septa
• Peritoneal enhancement; frank abscess
• Tuberculosis, acquired immunodeficiency syndrome,
fungal infections
Cystic peritoneal metastases
• Massive ascites; loculated fluid collections
• Thickening of greater omentum
• Small nodules along peritoneal surface
• Apparent thickening of mesenteric vessels (fluid
within leaves of mesentery)
• Increased density of linear network in mesenteric fat
• Adnexal mass of cystic density (ovarian, Krukenberg)
I PATHOLOGY
General Features
• General path comments: Diminished effective volume
(hydrostatic vs. colloid osmotic pressure); overflow
• Etiology
o Hepatic: Cirrhosis, portal hypertension
• Budd-Chiari, portal vein thrombosis, alcoholic
hepatitis, fulminant hepatic failure
o Cardiac: Congestive heart failure, constrictive
pericarditis, cardiac tamponade
o Renal: Nephrotic syndrome, chronic renal failure
o Neoplasm: Colon, gastric, pancreatic, hepatic,
ovarian; metastatic disease (breast/lung etc.)
• Meig syndrome, mesothelioma
o Infections: Tuberculosis
• Acquired immunodeficiency syndrome
• Bacterial, fungal or parasitic infections
o Trauma: Blunt, penetrating or iatrogenic
• Diagnostic/therapeutic peritoneal lavage
• Bile ascites: Trauma, cholecystectomy, biliary or
hepatic surgery, biopsy, percutaneous drainage
• Urine ascites: Direct or seat belt trauma to bladder
or collecting system; instrumentation
• Cerebrospinal fluid: Ventriculoperitoneal shunts
• Chylous: Blunt, penetrating, surgical trauma
o Hypoalbuminemia; protein-losing enteropathy
o Miscellaneous: Myxedema, marked fluid overload
Gross Pathologic & Surgical Features
• Transudate; clear, colorless or straw colored
• Exudate; yellowish or hemorrhagic
• Neoplasm; bloody, clear or chylous
• Pyogenic; turbid, chylous; yellowish white, milky
• Pseudomyxoma peritonei; gelatinous, mucinous
Microscopic Features
• Ascitic fluid may contain blood cells, colloids, protein
molecules, or crystalloids (such as glucose) & water
o Protein content: Less than 2.5 g/dl transudate;
greater than 2.5 g/dl exudate
o Polymorphonuclear leukocyte count greater than
SOO/cu.mm suggests infection or pancreatic ascites
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Asymptomatic,
abdominal discomfort & distension, weight gain
• Physical examination: Bulging flanks, flank dullness,
fluid wave, umbilical hernia, penile or scrotal edema
• Diagnosis: Paracentesis; US guidance or blind tap
o Indications: All patients with new onset ascites
• Chronic ascites with fever, abdominal pain, renal
insufficiency, or encephalopathy
o Fluid analysis: Protein, lactate dehydrogenase
• Amylase, blood cell count with differential
bacteriology, cytology, pH, triglycerides
• Serum-ascites albumin gradient; greater than 1.1
g/dL indicates portal hypertension
Natural History & Prognosis
• Complication: Spontaneous bacterial peritonitis,
respiratory compromise, anorexia
Treatment
• Sodium restriction & diuretics
• Therapeutic paracentesis
• Refractory cases: Large volume paracentesis
o Peritoneovenous shunting; LeVeen, Denver
o Transjugular intrahepatic porto systemic shunting
o Liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Difficult to characterize nature & underlying cause of
peritoneal fluid collections on basis of imaging alone
I SELECTED REFERENCES
1. Hanbidge AEet al: us of the peritoneum. Radiographies.
23(3):663-84; discussion 684-5, 2003
2. Jeffery J et al: Ascitic fluid analysis: the role of biochemistry
and haematology. Hosp Med. 62(5): 282-6, 2001
3. Heneghan MA et al: Pathogenesis of ascites in cirrhosis and
portal hypertension. Med Sci Monit. 6(4):807-16, 2000
4. Habeeb KSet al: Management of ascites. Paracentesis as a
guide. Postgrad Med. 101(1): 191-2, 195-200,1997
5. Henriksen JH et al: Ascites formation in liver cirrhosis: the
how and the why. Dig Dis. 8(3):152-62, 1990
 ASCITES
I IMAGE GALLERY 1
21
Typical
(Left) Axial CECT shows
loculated ascites due to
peritoneal dialysis. Note

. ",~i~\
mass effect and

.. ". ~' contrast-enhancing wall.

(Right) Axial CECT in a
cirrhotic patient with
. V.,
I ,a
....
••.•
;;:;
•
,
spontaneous bacterial
" . peritonitis, loculation,

••• enhancing rim and gas

(arrows).

-'0
·Y.
Typical
(Left) Axial NECT in patient
with pancreatitis. Note
prominent lesser sac
collection of fluid (arrow).
(Right) Axial CECT shows
fluid in lesser sac and
"scalloped" surface of liver
and spleen. Peritoneal
carcinomatosis.

Typical
(Left) Axial CECT shows
ascites, including lesser sac,
along with thickened,
"smudged" omentum
(arrow). Malignant ascites.
(Right) Axial CECT shows
loculated ascites, peritoneal
thickening (arrows), nodular
omentum. Ovarian
carcinoma metastases.
 OMENTAL INFARCT
1
22

Axial CECT shows infiltration and mass effect in the Axial CECT shows increased attenuation stranding in the
omentum adjacent to the sigmoid colon. Note staple omental fat (arrow) adjacent to the ascending colon
line (arrow) from prior sigmoid resection 2 months (Courtesy O. Green, MO).
previously

ITERMINOlOGY Ultrasonographic Findings

• Real Time
Abbreviations and Synonyms
o Hyperechoic, non-mobile/compressible, fixed mass
• Omental infarction o Free fluid may be seen
Definitions Imaging Recommendations
• Necrosis caused by interruption of arterial blood • Helical CECT with oral contrast
supply to omentum

IIMAGING FINDINGS I DIFFERENTIALDIAGNOSIS

General Features
• Best diagnostic clue: Focal mass of heterogeneous
omental fat ± a capsule in right lower quadrant
• Size: Varies from 3.5-15.0 cm
• Morphology
o Inflamed ± hemorrhagic omental fat
o Triangular, ovoid or cake-like in shape
CT Findings
• Heterogeneous fatty mass + hyperattenuated streaks
• Located between anterior abdominal wall & colon
• Pericolonic inflammatory changes
• Adherence to colon or parietal peritoneum
• Thickening of overlying abdominal wall (rare)
• Free fluid may be visualized
Acute appendicitis
• Appendicolith may be seen
• Periappendiceal soft tissue stranding
• Wall thickening of cecum or terminal ileum
• Right lower quadrant fluid or abscess
Epiploic appendagitis
• Paracolic fatty mass + hyperattenuating ring
• Location: More common in LLQ (rectosigmoid)
• Pericolonic fat stranding; thickened peritoneum/bowel
Omental torsion
• Due to omental cysts, hernias, tumors, adhesions
• Fibrous + fatty folds converging towards torsion
Pancreatitis
• Focal or diffuse enlargement of pancreas
• Fluid collections, infiltration of peripancreatic fat

DDx: Heterogeneous Fat in Omentum or Mesentery

Appy. Abscess Epiploic Append. Pancreatitis Fibrosing Mesent.

 OMENTAL INFARCT

Key Facts 1
Imaging Findings • Epiploic appendagitis 23
• Best diagnostic clue: Focal mass of heterogeneous • Omental torsion
omental fat ± a capsule in right lower quadrant • Pancreatitis
• Located between anterior abdominal wall & colon • Fibrosing sclerosing mesenteritis
• Pericolonic inflammatory changes Diagnostic Checklist
• Adherence to colon or parietal peritoneum
• Omental infarct in case of acute abdominal pain with
Top Differential Diagnoses absence of constitutional symptoms or i WBC
• Acute appendicitis • CT: Fatty mass with hyperattenuated streaks between
abdominal wall & colon on right side

• Abscess, pseudocyst, gallstones

• Chest: Pleural effusion & basal atelectasis Treatment
• Conservative management
Fibrosing sclerosing mesenteritis • Laparoscopic excision
• Soft tissue mass usually in root of small bowel
mesentery (fibrous tissue)
• Thickening, infiltration, displacement, narrowing of I DIAGNOSTIC CHECKLIST
bowel loops
Consider
• Omental infarct in case of acute abdominal pain with
[PATHOLOGY absence of constitutional symptoms or i WBC

General Features Image Interpretation Pearls

• General path comments • CT: Fatty mass with hyperattenuated streaks between
o Hemorrhagic infarction with fat necrosis abdominal wall & colon on right side
o Followed by inflammatory infiltrate
o Fibrosis & retraction ~ healing or autoamputation
• Etiology I SELECTED REFERENCES
o Unclear, abnormal omental vascular system 1. Macari M et al: The acute right lower quadrant: CT
• Right epiploic vessels involved in 90% of cases evaluation. Radiologic clinics of North America.
o Precipitating factors: Obesity, vascular congestion, 14(6):1117-36,2003
kinking of vessels, i in intra-abdominal pressure 2. Grattan-Smith JD et al: Omental infarction in pediatric
• Epidemiology: Adults (85%), children (15%) patients: sonographic and CT findings. AJRAm J
Roentgenol. 178(6):1537-9, 2002
Gross Pathologic & Surgical Features 3. McClure MJ et al: Radiological features of epiploic
• Infarcted omentum often adherent to parietal appendagitis and segmental omental infarction. Clin
Radiol. 56(10):819-27, 2001
peritoneum or colon
4. Puylaert JB: Right-sided segmental infarction of the
• Serosanguineous fluid in peritoneal cavity omentum: clinical, US, and CT findings. Radiology.
185(1):169-72, 1992
Microscopic Features
• Inflammatory infiltrate: Predominantly plasmocytic,
lymphocytic and histiocytic cells
I IMAGE GALLERY
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute abdominal pain, right-sided in 90% of cases
o Feveri ± palpable mass in RLQ
• Lab-data: WBC & ESRnormal or mildly elevated
Demographics
• Age
o Elderly obese people (85% of cases)
o Less common in children (15% of cases)
• Gender: M > F (Left) Axial NECT shows infiltration of omental fat (arrow) (Courtesy
O. Avrin, MO). (Right) Axial CECT shows heterogeneous "mass" of
Natural History & Prognosis mixed fat density in the omentum (arrow), displacing the anterior
• Complications: Abscess, adhesions, bowel obstruction abdominal wall.
• Prognosis
o Usually self limiting and will resolve spontaneously
 INGUINAL HERNIA
1
24

Axial CECT shows dilated small bowel entering inguinal Axial CECT shows "knuckle" of fluid-filled small
canal, collapsed bowel leaving it (arrow). intestine strangulated within right inguinal hernia
(arrow).

o Direct IH: Occurs in floor of inguinal canal, through

ITERMINOlOGY Hesselbach triangle
Abbreviations and Synonyms • Protrudes medial to inferior epigastric vessels (lEV)
• It is not contained in spermatic cord & it generally
• Inguinal hernia (IH)
does not pass into scrotum
• Pelvic & groin hernia
• Medial umbilical fold divides Hesselbach triangle
Definitions into medial & lateral parts
• External hernia: Abnormal protrusion of • Medial & lateral direct IH
intra-abdominal tissue • Morphology
o Through defect in abdominal or pelvic wall & o Indirect IH in males within spermatic cord has
extending outside abdominal cavity smooth contour & elongated oblique course
o IH: Inguinal location of hernia orifice • ]uxtafunicular hernias: Have a more irregular
contour; do not protrude into a preformed sac
• Dissect through subcutaneous fat & fibrous tissue
I IMAGING FINDINGS o Direct IH: Broad & dome-shaped; appears as a small
bulge in groin; short & blunt aperture
General Features
Radiographic Findings
• Location
o Indirect IH: Passes through internal inguinal ring, • Radiography
down the inguinal canal & emerges at external ring o Films of abdomen with patient supine can indicate
• Can extend along spermatic cord into scrotum; incarceration or strangulation
complete hernia • Convergence of distended intestinal loops toward
• In females, hernia follows course of round inguinal region
ligament of uterus into labium majus • Soft tissue density or gas-containing mass
• Passes lateral to epigastric vessels (lateral umbilical overlying obturator foramen on affected side
fold) & is also known as lateral IH o Barium examination of small or large bowel
• ]uxtafunicular: Indirect hernia passes outside • Tapered narrowing or obstruction of intestinal
spermatic cord segments as it enters hernia orifice

DDx: Mass Near Inguinal ligament

Femoral Hernia Hematoma Hematoma Lymphadenopathy

 INGUINAL HERNIA
Key Facts
Imaging Findings
• Indirect IH: Passes through internal inguinal ring,
down the inguinal canal & emerges at external ring
• Direct IH: Occurs in floor of inguinal canal, through
Hesselbach triangle
• Indirect IH in males within spermatic cord has
smooth contour & elongated oblique course
• Direct IH: Broad & dome-shaped; appears as a small
bulge in groin; short & blunt aperture
• Indirect IH: May see well-defined ovoid mass in groin
• Collapsed bowel loops & mesenteric fat in hernia sac
• Neck of indirect IH can be demonstrated at deep
inguinal ring lateral to lEV
• Whereas direct IH remain medial to lEV throughout
o Attempt should be made to reduce hernia manually
under fluoroscopy
o Visualize afferent & efferent loops of protruding
intestine
• Herniography: Indirect IH; emerges from lateral
inguinal fossa & protrudes medially
o Roughly parallel to superior pubic ramus
o Persistent processus vaginalis has a width of 1 to 2
mm; may extend into scrotum
• Communicating hydrocele
o When length of sac exceeds 4 cm, it usually widens
abruptly beyond external opening of inguinal canal
o Widened internal opening of inguinal canal is seen
as triangular outpouching of lateral inguinal fossa
• Its acute apex is directed inferomedially & its
medial border is concave
o No plicae or indentations visualized lateral to an
indirect hernia
o Open Nuck's canal in women; same herniographic
appearance as patent processus vaginalis in men
• More lateral direct hernia, protruding from medial
inguinal fossa; usually dome-shaped with wide neck
• More medially located direct IH; protrude from
supravesical fossa & are usually smaller
CT Findings
• Indirect IH: May see well-defined ovoid mass in groin
o Collapsed bowel loops & mesenteric fat in hernia sac
• Neck of indirect IH can be demonstrated at deep
inguinal ring lateral to lEV
o Whereas direct IH remain medial to lEV throughout
• CT useful when there is suspicion that another disease
process is either mimicking or precipitating hernia
MR Findings
• In obese patients; physical examination difficult
• Dynamic evaluation in multiple imaging planes may
have advantages
Ultrasonographic Findings
• Real Time
o May see bowel loops peristalse within hernia
o Useful when patient presents non-urgently with
history suggesting reducible hernia
1
Top Differential Diagnoses 25
• Femoral hernia
• Iatrogenic
• Lymphadenopathy
Pathology
• 75-80% of all hernias occur in inguinal region
• Indirect IH are 5 times more common than direct IH
• Contents: Include small bowel loops or mobile colon
segments such as sigmoid, cecum & appendix
Clinical Issues
• Diagnosis: History & physical examination
• Indirect IH five to ten times more common in men
• Complications: Incarceration; strangulation
• US real time examination allows patient to stand
upright & perform Valsalva maneuver
o Valsalva maneuver: In direct herniation distended
pampiniform plexus displaced by hernia sac
• In indirect hernia impaired swelling of
pampiniform plexus seen
• Color Doppler
o To distinguish among types of groin hernias
• Demonstrate inferior epigastric artery (origin &/or
trunk segment) & its relationship with hernia sac
Imaging Recommendations
• Best imaging tool
o US; CT; MRI for demonstrating acutely strangulated
hernia in obese patients
• In problem cases where there is clinical diagnostic
uncertainty
• Protocol advice
oCT: Oral + intravenous CECT; axial plane; thinner
slice collimation (e.g., 5 mm)
• Frequent image reconstructions to show lEV
I DIFFERENTIAL DIAGNOSIS
Femoral hernia
• Medial position within femoral canal posterior to line
of inguinal ligament; caudal & posterior to IH
• Frequently has a narrow neck; neck remains below
inguinal ligament & lateral to pubic tubercle
• More common in women
Iatrogenic
• Arterial puncture following arteriography; needle
biopsy or aspiration
o Hematoma formed may extend into rectus muscle or
lateral abdominal wall muscles
o Blood can track directly from groin, along
transversalis fascia & transversus abdominis muscle
oCT, US, MR: Appearance of blood; extent of lesion;
changes over time
o Pseudoaneurysm: Perivascular, rounded mass; neck +
track connecting it with injured artery
 INGUINAL
1 Lymphadenopathy
26 • Appears as mass near inguinal ligament
• CT, US: Help differentiate hernia contents from other
masses involving groin & scrotum
o Such as hydrocele, varix, lipoma of spermatic cord,
undescended testicle, abscess, tumor
I PATHOLOGY
General Features
• Etiology
o Indirect IH considered to be congenital defect
• Patency of processus vaginalis; weakness of crus
lateralis at lateral aspect of inguinal canal
o Direct IH considered acquired lesion
• Weakness in transversalis fascia of posterior wall
of inguinal canal in Hesselbach triangle
• Epidemiology
o 75-80% of all hernias occur in inguinal region
o Indirect IH are 5 times more common than direct IH
o Incidence: IH occurs in 1-3% of all children
• In premature infants, incidence is one-half to two
times greater
o Approximately 5% of men develop IH during their
lifetime & require an operation
o Bilateral patent processus vaginalis occurs in up to
10% of patients with indirect IH
Gross Pathologic & Surgical Features
• Contents: Include small bowel loops or mobile colon
segments such as sigmoid, cecum & appendix
• Sliding IH: Partially retroperitoneal organs
o Urinary bladder, distal ureters or ascending or
descending colon, included in herniation
o Retroperitoneal structures constitute wall of sac
o Blood vessels supplying herniated segments, may be
injured during surgical repair or trauma
• Littre hernia: Meckel diverticulum in hernia sac
• Richter hernia: Only portion of bowel circumference
in sac (antimesenteric)
• Incomplete IH: Sac not extended through external
inguinal ring
• Diverticular direct IH: Protrudes from either medial
inguinal or supravesical fossa
o Small opening in otherwise normal transverse fascia
o Distinct circumscribed neck & usually protrudes
more in anterior than inferior direction
• Potential indirect hernias are associated with an
undescended testis; a testis in inguinal canal
o Testicular or spermatic cord hydrocele
I CLINICAL ISSUES
Presentation
• Asymptomatic; sudden appearance of lump in groin;
intermittently present; ± groin pain; palpable bulge
• Physical examination: Recumbent & upright position;
may be reducible; bowel sounds audible; ± tender
o Indirect hernia lightly touches tip of finger
• Examining finger placed along spermatic cord at
scrotum & passed into external ring along canal
HERNIA
• During maneuvers that 1 intra-abdominal pressure
o Direct hernia causes bulge forward low in canal
• Incarcerated or strangulated hernia: Bowel distension;
painful & often tense swelling in groin or scrotum
• Diagnosis: History & physical examination
Demographics
• Age
o Indirect IH may occur from infancy to old age but
generally occur by fifth decade of life
o Direct IH increases in occurrence with age
• Gender
o Indirect'lH five to ten times more common in men
o Direct IH occurs mostly in men & seldom in women
Natural History & Prognosis
• Pediatric IH: Almost always indirect; higher risk of
incarceration
o Usually on right (60-75%); often bilateral (10-15%)
• Recurrent hernia: Groin hernias recur after
herniorrhaphy in up to 20% of patients
o Direct IH may develop after repair of an indirect
hernia
o Diverticular hernias are a form of recurrence
• Multiple hernias: One is usually a direct type
o May obscure smaller hernias that can be clinically
significant
• Saddlebag, pantaloon, combined IH: Simultaneous
occurrence of direct & indirect IH in same groin
o Separation of two adjacent hernia sacs by lEV creates
bilocular appearance
• Indirect IH accounts for 15% of intestinal obstructions
• Diverticulitis; appendicitis; primary or metastatic
tumor may occur within hernia sac
• Complications: Incarceration; strangulation
o Direct IH rarely becomes incarcerated & less often
associated with strangulation
Treatment
• Laparoscopic or open hernia repair
I DIAGNOSTIC CHECKLIST
Consider
• Hernias that protrude from lateral inguinal fossa are
indirect
• Those from medial & supravesical fossae are direct
I SELECTED REFERENCES
1. van den Berg JC: Inguinal hernias: MRI and ultrasound.
Semin Ultrasound CT MR. 23(2): 156-73, 2002
2. Zhang GQ et al: Groin hernias in adults: value of color
Doppler sonography in their classification. J Clin
Ultrasound. 29(8): 429-34, 2001
3. Shadbolt CL et al: Imaging of groin masses: inguinal
anatomy and pathologic conditions revisited.
Radiographies. 21 Spec No: S261-71, 2001
4. Toms AP et al: Illustrated review of new imaging
techniques in the diagnosis of abdominal wall hernias. Br J
Surg. 86(10): 1243-9, 1999
5. Hahn-Pedersen J et al: Evaluation of direct and indirect
inguinal hernia by computed tomography. Br J Surg. 81(4):
569-72, 1994
 INGUINAL HERNIA
I IMAGE GALLERY 1
Typical 27

(Left) Axial CECT shows left

inguinal hernia containing
only fat and spermatic cord.
(Right) Small bowel follow
through (S8FT) shows
almost entire small intestine
within scrotum due to right
inguinal hernia.

Typical
(Left) Axial CECT shows left
inguinal hernia at upper end
of inguinal canal. There is
mass effect due to herniated
fat and bowel. (Right) Axial
CECT shows left inguinal
hernia containing sigmoid
colon. Also note right thigh
hematoma.

Typical
(Left) Axial CECT shows right
inguinal hernia with colon in
upper scrotum. (Right) Axial
CECT in elderly woman
shows left inguinal hernia,
right obturator hernia
(arrow) and pessary (curved
arrow).
 FEMORAL HERNIA
1
28

Axial CECT shows left femoral hernia containing Axial CECT shows small bowel obstruction due to
"knuckle" of strangulated bowel. strangulated femoral hernia.

ITERMINOlOGY Radiographic Findings

• Herniography: Characteristic posterior "inbulging"
Abbreviations and Synonyms o Due to proximity of symphysis pubis to posterior &
• Femoral hernia (FH) medial aspect of hernia
• Crural hernia; Enteromerocele; Femorocele
CT Findings
Definitions • Anatomic localization; relationship to femoral
• FH occurs when intra-abdominal contents protrude triangle; medial to femoral vein
along femoral sheath in femoral canal • Identify contents of sac; differentiating hernia from
other causes of groin swelling

jlMAGING FINDINGS Ultrasonographic Findings

General Features
• Best diagnostic clue: History + clinical examination
• Location
o Hernia contents protrude posteriorly to inguinal
ligament
• Anteriorly to pubic ramus periosteum (Cooper
ligament), medially to femoral vessels
o FH traverses femoral canal & presents as mass at
level of foramen ovale
o Neck of FH always remains below inguinal ligament
& lateral to pubic tubercle
• Morphology
o FH protrudes at right angle to inguinal canal
o Has a narrow neck & characteristic pear shape
• In patients with undiagnosed pain; mass in groin
• Questionable inguinal or femoral region hernias
• Post-operative complaints after herniorrhaphy
• Possible femoral aneurysms or pseudoaneurysms
I DIFFERENTIALDIAGNOSIS
Inguinal hernia
• FH differentiated from inguinal hernia by its medial
position within femoral canal
o Posterior to line of inguinal ligament
• Like direct inguinal hernia, FH arises from medial
inguinal fossa
o But FH protrudes in caudal rather than anterior
direction

DDx: Mass Near Femoral Artery

Inguinal Hernia Inguinal Hernia Hematoma Lymphadenopathy

 FEMORAL HERNIA
Key Facts
Terminology
• FH occurs when intra-abdominal contents protrude
along femoral sheath in femoral canal
Imaging Findings
• FH traverses femoral canal & presents as mass at level
of foramen ovale
• Neck of FH always remains below inguinal ligament
& lateral to pubic tubercle
Iatrogenic
• Hematoma; following arterial puncture in femoral
sheath during arteriography; needle biopsy etc.
• CTi US: Appearance of blood + extent of lesion
lymphadenopathy
I PATHOLOGY
General Features
• Etiology
o There may be congenital defect in insertion of
transversalis fascia to ileopubic tract
• Forms superior & anterior limit of lacuna vasorum
• If it is weakened, femoral canal dilates, permitting
formation of FH
o Associated with 1 intra-abdominal pressure
• Epidemiology
o FH accounts for less than 1% of all groin hernias in
children
o In adults accounts for approximately 5-10% of groin
hernias
o Accounts for about one third of groin hernias in
women
Gross Pathologic & Surgical Features
• Hernia contents: Usually properitoneal fat, edge of
omentum or loop of small bowel
• Richter hernia: Usually in older women with FH
ICLINICAL ISSUES
Presentation
• Swellingi ± pain; dragging sensation in groin
• Lump usually felt in top of thigh, below groin crease
o Large FH may bulge over inguinal ligament
• Neck palpable lateral & inferior to pubic tubercle
• Nausea, vomiting, severe abdominal pain may occur
with strangulated hernia
• Often difficult to diagnose clinicallYi deep location of
femoral canal & abundance of overlying adipose tissue
Demographics
• Age
o Incidence: 36% in over 80 years age group
• 16% among patients in their seventies
o Children are rarely affected
• Gender: Occurs predominantly in women (M:F = 1:3)
1
• Has a narrow neck & characteristic pear shape 29
Top Differential Diagnoses
• Inguinal hernia
• Iatrogenic
• Lymphadenopathy
Clinical Issues
• Gender: Occurs predominantly in women (M:F = 1:3)
• 25-40% are incarcerated; because of a narrow neck
Natural History & Prognosis
• FH may displace or narrow femoral veini may descend
along saphenous vein
• Complications: Incarceration; strangulation
o 25-40% are incarcerated; because of a narrow neck
o FH is 8-12 times more prone to incarceration &
strangulation than inguinal hernia
o Due to firm & unyielding margins of femoral ring
• Morbidity: Significantly related to intestinal
obstruction
• Mortality: 1% in 70-79 age group, 5% in 80-90 years
age grouPi associated with intestinal obstruction
Treatment
• Femoral sheath defect closed by apposing Cooper
ligament & posterior reflection of inguinal ligament
• Repair using a mini-incision, "tension free" technique,
utilizing mesh
• Often floor of inguinal canal is also reinforced, using
transversalis fascia
I SELECTED REFERENCES
1. Toms AP et al: Illustrated review of new imaging
techniques in the diagnosis of abdominal wall hernias. Br J
Surg. 86(10): 1243-9, 1999
2. Radcliffe G et al: Reappraisal of femoral hernia in children.
Br J Surg. 84(1): 58-60, 1997
3. Chamary VL: Femoral hernia: intestinal obstruction is an
unrecognized source of morbidity and mortality. Br J Surg.
80(2): 230-2, 1993
I IMAGE GALLERY
(Left)Axial CECT in elderly woman shows right femoral hernia
(arrow) and pessary (curved arrow). (Right) Axial CECT shows right
femoral hernia containing small bowel that caused obstruction.
 VENTRAL HERNIA
1
30

Axial CECT shows herniation of small bowel through Axial CECT shows ventral hernia containing ascites and
wide ventral hernia at site of prior paramedian surgical varices in a patient with cirrhosis (Umbilicus at a lower
incision. section).

ITERMINOlOGY
Definitions
• Term "ventral hernia" (VH) encompasses herniations
through anterior & lateral aspects of abdominal wall
o Incisional Hernia (IH): Hernia resulting from
abdominal wall incisions
I IMAGING FINDINGS
General Features
• Location
o Majority occur in midline; emerge through
aponeurosis forming linea alba
o Epigastric hernia: Above umbilicus
o Hypogastric hernia: Below umbilicus
o IH: Along midline, paramedian incisions; although
any surgical scar may be a potential site
Radiographic Findings
• Fluoroscopic evaluation of gastrointestinal tract with
barium; may reveal clinically occult, unsuspected IH
o Areas of old surgical scars may be viewed in profile
while patient strains; detect reducible hernias
• Distended bowel loops proximal to obstruction in
relationship to locally tender part of abdominal wall
CT Findings
• Defect in peritoneal & fascial layers of ventral
abdominal wall
o Through which omentum or knuckle of intestines
protrude into subcutaneous fat
• Early dehiscence of muscle layer in anterior abdominal
wall closure; without disruption of overlying skin
• Useful when contents & orifice not palpable;
interstitial or interparietal location
o Herniated segments dissect & "hide" between
muscular & fascial layers of abdominal wall
• Differentiate incarcerated hernia & post-operative
hematoma
Ultrasonographic Findings
• Actual defect & herniation in ventral wall of abdomen
I DIFFERENTIAL DIAGNOSIS
Parastomal hernia
• Following stoma formation of an ileostomy or
colostomy
Spigelian hernia
• Anterolateral; defect involving lateral border of rectus
sheath

DDx: Anterior Abdominal Wall Defect or Mass

Parastomal Hernia Spigelian Hernia Umbilical Hernia Pannus

 VENTRAL HERNIA

Key Facts
1
Terminology • Defect in peritoneal & fascial layers of ventral 31
• Term "ventral hernia" (VH) encompasses herniations abdominal wall
through anterior & lateral aspects of abdominal wall Top Differential Diagnoses
• Incisional Hernia (IH): Hernia resulting from
• Parastomal hernia
abdominal wall incisions
• Spigelian hernia
Imaging Findings • Umbilical hernia
• Majority occur in midline; emerge through • Hematoma or abscess
aponeurosis forming linea alba • Pannus

• Symptoms out of proportion to objective findings if

Umbilical hernia
• Umbilical defect; patent umbilical ring
Hematoma or abscess
• No defect in abdominal wall
• Distinguish gas in abscess from gas in bowel
Pannus
• Mid-abdomen may protrude over lower, simulating
hernia
I PATHOLOGY
General Features
• Etiology
o Congenital; spontaneous; primary defect
o Acquired: Latrogenic; previous abdominal surgery,
laparoscopy, peritoneal dialysis, stab wound etc.
o Factors which 1 likelihood of hernia occurrence
• Patient-related: Collagen biochemistry, obesity,
age over 65, pulmonary disease, uremia, diabetes,
steroids, malignancy, trauma
• Technical factors: Wound infection, suture
material, types of incisions & closures
• Epidemiology
o More than 80% of VH result from prior surgery
• IH reported to occur after 0.2-26% of abdominal
procedures
o Majority of incisional hernias are ventral
Gross Pathologic & Surgical Features
• Portions of greater omentum, properitoneal fat, or a
bowel loop protrude anteriorly
incarceration or strangulation occurs
• 10% of IH not detected on physical examination
o Diagnosis difficult in obese patients, severe pain or
distension, keloid, thick panniculus
Natural History & Prognosis
• Complications: Incarceration & strangulation of
contents; may occur frequently
Treatment
• Repair techniques: Open suture; open mesh;
laparoscopic mesh
• Laparoscopic repair: Lower recurrence rate (0-9%)
• With development of prosthetic mesh safe to place
intraperitoneally, recurrence rate has I to under 5%
I DIAGNOSTIC CHECKLIST
Consider
• Accurate demonstration of size & site of hernial
orifice, along with contents of sac
o May be useful in assessing potential risk of
strangulation or likely success of hernia repair
I SELECTED REFERENCES
1. Millikan KW: lncisional hernia repair. Surg Clin North Am.
83(5):1223-34, 2003
2. Thoman DS et al: Current status of laparoscopic ventral
hernia repair. Surg Endosc. 16(6):939-42, 2002
3. Yahchouchy-Chouillard E et al: lncisional hernias. I.
Related risk factors. Dig Surg. 20(1):3-9, 2003;20(1):3-9.

I CLINICAL ISSUES I IMAGE GALLERY

Presentation
• Bulge or swelling on abdominal wall; can become
larger & pain aggravated with exertion
• IH: Tend to occur during first 4 months after surgery
o Progressive enlargement usually manifested within
first post-operative year
o 5-10% remain clinically silent for several years
o Most IH are incidental findings at imaging
o Initially, vague abdominal discomfort; localized
tenderness of healed scar
o Advanced stage: Persistent bulging mass resulting
from incarcerated bowel loops may be seen
(Left) Axial CECT shows a lateral ventral hernia. (Right) Axial CECT
shows a lateral incisional hernia containing colon.
 SPIGELIAN HERNIA
1
32

Radiograph shows dilated colon due to left Spigelian Axial CECT shows Spigelian hernia just lateral to rectus
hernia containing descending colon (arrow). muscle, with obstructed, herniated descending colon.

o Clinically: In fascia below level of umbilicus, lateral

ITERMINOlOGY
Abbreviations and Synonyms
• Spigelian hernia (SH)
Definitions
• Spigelian hernia is a protrusion of intra-abdominal fat
or bowel through a defect in "'Spigelian aponeurosis"
I IMAGING FINDINGS
General Features
• Location
o Spigelian aponeurosis: Part of anterior abdominal
wall aponeurosis
• Lies between linea semilunaris laterally & lateral
edge of rectus muscle medially
o SH is classically found at position cranial to junction
of inferior epigastric vessels & Spigelian aponeurosis
• Caudal to this point it is termed "low SH"
o Anatomically: Hernia sac extends through defect
• In transversalis fascia, transversus abdominis, &
internal oblique aponeurosis
• Lies deep to external oblique aponeurosis
• Classic SH: External oblique aponeurosis remains
intact & hernial sac is intermuscular
to junction of semilunar line & arcuate lines
• Morphology
o Defect size range: 1-7.5 cm diameter
• Most commonly; 1-3 cm
Radiographic Findings
• Barium enema or small bowel examination: Position &
contents of hernia
o May show distended bowel loops proximal to a
narrowing or obstruction
CTFindings
• Hernia defect involving lateral border of rectus sheath
• Interstitial protrusion of omentum or intestinal loops
I DIFFERENTIAL DIAGNOSIS
Ventral hernia
• Majority occur in midline; emerge through
aponeurosis forming linea alba
• Epigastric or hypogastric: Above or below umbilicus
o Most SH are caudal & lateral to umbilical level
Umbilical hernia
• CT; us: "Knuckle" of bowel, fat, ascites protruding
through umbilical defect in midline

DDx: Anterior Abdominal Wall Defect

Ventrai Hernia Umbilicai Hernia Lap. Port Hernia Rectus Hematoma

 SPIGELIAN HERNIA

Key Facts
1
Terminology Top Differential Diagnoses 33
• Spigelian hernia is a protrusion of intra-abdominal fat • Ventral hernia
or bowel through a defect in '''Spigelian aponeurosis" • Umbilical hernia
• Hernia through laparoscopy port (lap. port)
Imaging Findings • Rectus sheath hematoma
• Classic SH: External oblique aponeurosis remains
intact & hernial sac is intermuscular Pathology
• Hernia defect involving lateral border of rectus sheath • Frequency: 2% of anterior abdominal hernias
• Interstitial protrusion of omentum or intestinal loops

• Because hernial ring is usually small, irreducibility &

Hernia through laparoscopy port (lap. port)
• Several potential sites
• Usually medial or lateral to Spigelian site
Rectus sheath hematoma
• Cylindrical, heterogeneous, encapsulated
strangulation are not uncommon
• Presence of congenital SH predisposes to development
of ipsilateral undescended testis
Treatment
mass • Tension free repair using a prosthetic mesh
• Repair of pediatric SH utilizes endogenous tissue

I PATHOLOGY
General Features
I DIAGNOSTIC CHECKLIST
• Etiology: Variations in SHs site, size, age suggest Consider
multifactorial etiology; including congenital • Intra-operative US is a valid option for accurate
• Epidemiology localization of SH, especially in obese patients
o Frequency: 2% of anterior abdominal hernias o Extensive intra-operative dissection, distortion of
o SH is considered rare; but more likely underreported tissue planes, & morbidity risks may be avoided
o 37 reported cases in children (newborn-17 y)
• Bilateral hernia: 15% incidence in children
• Associated abnormalities I SELECTED REFERENCES
o Undescended testis (17%) in children; ipsilateral
1. Losanoff JE et al: Spigelian hernia in a child: case report
o Anterior wall defects: Gastroschisis, omphalocele,
and review of the literature. Hernia. 6(4): 191-3, 2002
bladder or cloacal extrophy, prune belly syndrome 2. Losanoff JE et al: Recurrent Spigelian hernia: a rare cause of
o Coexisting ventral, inguinal, umbilical hernia colonic obstruction. Hernia. 5(2): 101-4,2001
3. Losanoff JE et aI: Incarcerated Spigelian hernia in morbidly
Gross Pathologic & Surgical Features obese patients: the role of intraoperative ultrasonography
• Interparietal or interstitial herniation for hernia localization. Obes Surg. 7(3): 211-4, 1997
• Contents: Omentum & short segment of small or large
intestine (may contain empty sac, testis, ovary)
I IMAGE GALLERY
ICLINICALISSUES
Presentation
• Asymptomatic; intestinal obstruction
• Prolonged history of intermittent pain
• Slight swelling or vanishing anterolateral mass
• Difficult to diagnose clinically; due to deep anatomic
location & insidious development
• Patients tend to be obese; making firm diagnosis on
physical examination difficult
o May result in surgical exploration undertaken on
suspicion raised by history alone
(Left) Axial CfCr shows left Spigelian hernia. (Right) Axial CfCr
Demographics
shows partial small bowel (58) obstruction due to Spigelian hernia.
• Gender
o In adult: M = F
o In children: M:F = 2.1:1
Natural History & Prognosis
• Omentum within SH may infarct & cause symptoms
 OBTURATOR HERNIA
1
34

Graphic shows bowel obstruction due to obturator Axial CECT shows knuckle of bowel (curved arrow)
hernia (curved arrow) with strangulated bowel lying lying between pectineus (arrow) and obturator muscles
deep to pectineus muscle (arrow) and superficial to (open arrow).
obturator externus (open arrow).

ITERMINOlOGY CT Findings
• Soft tissue mass or opacified loop that protrudes
Abbreviations and Synonyms through obturator foramen
• Obturator hernia (OH) o Extends between pectineus & obturator muscles
Definitions o Sac exits pelvis near obturator vessels & nerve
• Pelvic hernia; protruding through obturator foramen Imaging Recommendations
• Best imaging tool: CT; pelvis & upper aspect of thigh
IIMAGING FINDINGS
General Features I DIFFERENTIALDIAGNOSIS
• Best diagnostic clue: CT evidence of herniated bowel Inguinal hernia
lying between pectineus & obturator lI).uscles in • Indirect: Through inguinal canal ~ external ring
elderly woman o Females; course of round ligament into labium
• Location majus
o Site of herniation is obturator canal in superolateral o Males; along spermatic cord ~ scrotum
aspect of obturator foramen • Direct: Weak area medial to inferior epigastric vessels
o More common on right
• Morphology Sciatic hernia
o Hernia between: Pectineus & obturator externus • Through greater sciatic foramen ~ laterally into
muscles (externus, most common) subgluteal region
• Superior & middle fasciculi of obturator externus
muscle; external & internal obturator membranes Perineal hernia
• Anterior: Through urogenital diaphragm
Radiographic Findings • Posterior: Between levator ani & coccygeus muscle
• Abdominal radiographs or barium studies
o Small bowel obstruction; fixed loop containing gas
or contrast medium in obturator region

DDx: Defect in Pelvic Floor

Inguinal + Obturator Femoral Hernia Femoral Hernia

 OBTURATOR HERNIA
Key Facts
Imaging Findings
• Site of herniation is obturator canal in superolateral
aspect of obturator foramen
• Extends between pectineus & obturator muscles
• Sac exits pelvis near obturator vessels & nerve
Top Differential Diagnoses
• Inguinal hernia
• Sciatic hernia
I PATHOLOGY
General Features
• General path comments
o Anatomy: Obturator canal; obliquely oriented
fibro-osseous tunnel; 2-3 cm long & 1 cm in
diameter
• Obturator nerves & vessels course through it
• Etiology
o Defect in pelvic noor or laxity
o Chronic lung disease, constipation, kyphoscoliosis,
pregnancy; predispose by 1 intra-abdominal pressure
• Epidemiology
o Accounts for 0.05-0.14% of all hernias
• 0.2-1.6% of all small bowel obstruction
o Bilateral OH rare; 6% incidence
o May have inguinal and femoral hernia also
Gross Pathologic & Surgical Features
• Usually contains an ileal loop
o May involve other viscera & pelvic adnexa (large
bowel, omentum, fallopian tube, appendix)
I CLINICAL ISSUES
Presentation
• Most patients present with acute or recurrent small
bowel obstruction; partial> complete
• May present as tender mass in obturator region on
rectal or vaginal examination
• Howship-Romberg sign; pain in medial aspect of thigh
with abduction, extension, internal rotation of knee
o Positive in 20-50% of cases
o Obturator nerve irritation; compression by hernia
• Absent adductor renex in thigh
• Rarity & non-specific signs contribute to late
diagnosis; & is made correctly in 10-30% of cases
Demographics
• Age: Mean age: 82; range: 65-95 years
• Gender
o M:F = 1:6-9
o 80-90% of OH occur in elderly women
• Owing to enlargement of obturator canal after
pregnancies & aging; broader pelvis in women
• Elderly emaciated women with chronic disease
1
• Perineal hernia 35
Pathology
• Accounts for 0.05-0.14% of all hernias
Clinical Issues
• Most patients present with acute or recurrent small
bowel obstruction; partial> complete
• Elderly emaciated women with chronic disease
• Mortality rate: 13-40%
Natural History & Prognosis
• Protruding structures often incarcerated in canal or
space between pectineus & obturator muscles
• Morbidity & mortality rates significantly high
o Group of debilitated patients with chronic disease;
undergo late operation for this elusive diagnosis
o Mortality rate: 13-40%
Treatment
• Majority require resection of strangulated small bowel
• Abdominal/inguinal approach for reduction & repair
• Contralateral side exploration is recommended
I DIAGNOSTIC CHECKLIST
Consider
• In any elderly, debilitated, chronically ill woman
o Symptoms & signs of recurrent small-bowel
obstruction (no history of surgery or hernias)
o With pain along ipsilateral thigh & knee
o High index of suspicion must be maintained
I SELECTED REFERENCES
1. Lo CYet al: Obturator hernia presenting as small bowel
obstruction. Am] Surg. 167(4): 396-8,1994
2. Chan MYet al: Obturator hernia--case reports. Ann Acad
Med Singapore. 23(6): 911-3, 1994
3. Zerbey AL3rd et al: Bilateral obturator hernias: case report,
radiographic characteristics, and brief review of literature.
Comput Med Imaging Graph. 17(6): 465-8,1993
I IMAG E GALLERY
(Left) Axia! CECT shows obturator hernia with small bowel
strangulated (curved arrow) between pectineus + obturator extern us
muscles. (Right) Axial CECT shows right obturator hernia (arrow).
 PARADUODENAL HERNIA
1
36

Graphic shows left paraduodenal hernia containing Small bowel follow through (58FT) shows cluster of
dilated, proximal jejunal loops in a peritoneal "sac". jejunal segments that seem to lie within a confining sac.

• Transmesenteric post-operative hernia

ITERMINOlOGY
Definitions
• Protrusion of bowel loops through a congenital or
acquired defect of mesentery within abdominal cavity
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Cluster of dilated bowel loops
with distorted mesenteric vessels on CECT
• Location
o Left para duodenal hernia (75%)
• Via paraduodenal (lateral to 4th part) mesenteric
fossa of Landzert close to ligament of Treitz
o Right paraduodenal hernia (25%)
• Via jejunal mesentericoparietal fossa of Waldeyer
• Key concepts
o Classification of hernias based on anatomic location
• Internal or intra-abdominal: Herniation of bowel
loops via defect within abdominal cavity
• External: Prolapse of bowel loops via defect in wall
of abdomen or pelvis
• Diaphragmatic: Protrusion of bowel loops via
hiatus or congenital defect
o Subclassification of internal hernias
• Paraduodenal hernia
• Foramen of Winslow, pericecal hernias
• Intersigmoid & transomental hernias
o Subclassification of paraduodenal based on location
• Left paraduodenal hernia (75%)
• Right paraduodenal hernia (25%)
o Second most common subtype of internal hernia
after transmesenteric post-operative hernia
o Usually congenital or rarely acquired
o Rare cause of small-bowel obstruction
o 0.6-5.8% of small-bowel obstructions (SBO)are due
to internal hernias
• Paraduodenal accounts 1% or less of all SBO cases
Radiographic Findings
• Radiography
o Plain x-ray abdomen (supine)
• "Closed loop": Markedly distended segment of
small bowel (indicates SBO)
• Fluoroscopic guided small-bowel follow through
o Crowding of bowel loops in an abnormal location
• Location: Displaced to left or right side of colon
• Small-bowel is often absent from pelvis
o Left paraduodenal hernia
• A circumscribed ovoid mass of jejunal loops in left
upper quadrant lateral to ascending duodenum
o Right paraduodenal hernia
• Ovoid mass of small-bowel loops lateral & inferior
to descending duodenum

DDx: Cluster of Dilated Bowel loops

Closed Loop SBa Closed Loop SBa SBa TMI Hernia

 PARADUODENAL
Key Facts
Terminology
• Protrusion of bowel loops through a congenital or
acquired defect of mesentery within abdominal cavity
Imaging Findings
• Best diagnostic clue: Cluster of dilated bowel loops
with distorted mesenteric vessels on CECT
• Left paraduodenal hernia (75%)
• Right paraduodenal hernia (25%)
• Crowding of bowel loops in an abnormal location
• A circumscribed ovoid mass of jejunal loops in left
upper quadrant lateral to ascending duodenum
• Ovoid mass of small-bowel loops lateral & inferior to
descending duodenum
• Varying degrees of small bowel obstruction
• Mass effect: Displacement of bowel loops
o Appear as they are contained in a sac or confining
border
o Varying degrees of small bowel obstruction
o Point of transition between dilated & nondilated
bowel (common)
o Some degree of fixation, stasis & delayed flow of
contrast seen in herniated bowel
o Inability to displace clustered loops by manual
palpation or change in position
• Right side herniated loops more fixed than left
o Lateral films: Useful in demonstrating
retroperitoneal displacement of herniated bowel
loops
CT Findings
• Left-sided paraduodenal hernia
o Evidence of small bowel obstruction (SBO)
o Encapsulated, cluster or sac-like mass of small bowel
loops
• Location: Between pancreatic body/tail &
stomach, to left of ligament of Treitz
o Mass effect: Displacement of bowel loops
• Posterior stomach wall & duodeno-jejunal
junction (inferior & medially)
• Transverse colon inferiorly
o Mesenteric vessels: Crowded & engorged
• Right-sided paraduodenal hernia
o Cluster or encapsulated small bowel loops
• Location: Lateral & inferior to descending
duodenum
o Superior mesenteric vein: Rotated anteriorly & to
left side
o Twisting of vascular jejunal branches behind SMA &
into hernial sac
o Ascending colon: Lies lateral to hernia sac
o Cecum: Normal site
o Right ureter: Laterally displaced
Ultrasonographic Findings
• Real Time: Dilated small-bowel loops
Angiographic Findings
• Conventional
o Superior mesenteric arteriogram
HERNIA
1
Top Differential Diagnoses 37
• Closed loop obstruction (SBO)
• Transmesenteric (internal) hernia (TMI hernia)
Pathology
• Congenital or developmental mesenteric anomalies
• Acquired: As a complication of surgery or trauma
• Herniation via abnormal mesenteric fossa of Landzert
(seen in 2% of autopsies)
• Herniation via abnormal mesentericoparietal fossa of
Waldeyer (seen in 1% of autopsies)
Diagnostic Checklist
• Rule out other causes of dilated small-bowel loops
• Cluster of dilated small-bowel loops lateral to
ascending or descending duodenum with
crowded/twisted mesenteric vessels
• Normal jejunal branches arise from left margin of
main trunk & abruptly turn to right & pass behind
it to supply herniated loops
Imaging Recommendations
• Helical CECT; small-bowel follow through
I DIFFERENTIAL DIAGNOSIS
Closed loop obstruction (5BO)
• Obstruction of small-bowel at two points
• Tends to involve mesentery & prone to produce a
volvulus
o Represents most common cause of strangulation
• Etiology
o Most often caused by an adhesive band
o Occasionally by an internal or external hernia
• Imaging features
o Markedly distended segment of fluid-filled
small-bowel
o Volvulus: C- or U-shaped or "coffee bean"
configuration of bowel loop
o Stretched mesenteric vessels converging toward site
of torsion
o Adjacent collapsed loops at site of obstruction
• Round, oval or triangular in shape
o "Beak sign"
• Fusiform tapering at point of torsion/obstruction
o "Whirl sign"
• Due to tightly twisted mesentery with volvulus
• May be indistinguishable from paraduodenal hernia
o Especially if associated with volvulus
Transmesenteric (internal) hernia (TMI
hernia)
• Iatrogenic (post-operative)
o Abdominal surgery: Roux-en-Y gastric bypass (73%)
o Most common subtype of internal hernia in adults
• Congenital (mesenteric defect)
o Most common subtype in pediatric age group
o Location: Close to ligament of Treitz/ileocecal valve
o Mesenteric defect size: Usually 2 to 5 em in diameter
 PARADUODENAL
1 • Herniating loops: Small-bowel (jejunum/ileum)
• Imaging findings
38 o Cluster of dilated small-bowel loops
• Right side abdomen more common
• Small-bowel obstruction (100%)
o Crowding or twisting of mesenteric vessels
o Hepatic flexure displacement: Inferiorly/posteriorly
o May be indistinguishable from para duodenal hernia
without surgical history
I PATHOLOGY
General Features
• Etiology
o Congenital or developmental mesenteric anomalies
o Acquired: As a complication of surgery or trauma
o Pathogenesis & mechanism
• Congenital: Anomalies in mesenteric fixation of
ascending or descending colon lead to abnormal
openings through which hernia may occur
• Acquired: Abnormal mesenteric defects are created
• Abnormal mesenteric fixation may lead to
abnormal mobility of small bowel & right colon,
which facilitates herniation
o Left paraduodenal hernia
• Approximately 75% occur on left
• Herniation via abnormal mesenteric fossa of
Landzert (seen in 2% of autopsies)
• Location: Lateral to ascending part of duodenum
• Herniation of bowel loops into a pocket of distal
transverse & descending mesocolon, posterior to
superior mesenteric artery (SMA)
• Herniating segment: Proximal loops of jejunum
o Right para duodenal hernia
• Approximately 25% occur on right
• Herniation via abnormal mesentericoparietal fossa
of Waldeyer (seen in 1% of autopsies)
• Location: Jejunal mesentery, immediately behind
SMA & inferior to transverse part of duodenum
• Herniation of bowel loops into a pocket of
ascending mesocolon
• Herniating segment: Proximal loops of jejunum
• Epidemiology
o Autopsy incidence
• Internal hernias: 0.2-0.9%
• Paraduodenal: One half of internal hernias
Gross Pathologic & Surgical Features
• Dilated bowel loops herniating via a mesenteric defect
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Smaller ones: Easily reducible & clinically silent
o Larger ones
• Vague discomfort, abdominal distension
• Periumbilical colicky pain, bowel obstruction
• Palpable mass, localized tenderness
o Small-bowel obstruction
• Low grade, chronic & recurrent
• May be high grade & acute
HERNIA
Demographics
• Age
o Both children & adult age group
o Typically present between 4th & 6th decade
• Gender: M:F = 3:1
Natural History & Prognosis
• Complications
o Volvulus, ischemia, strangulation
o Bowel gangrene, shock & death
• Prognosis
o Early surgical correction: Good
o Delayed surgical correction & complications: Poor
Treatment
• Laparotomy, incise enclosing mesentery
• Bowel decompression
• Avoid injury to superior & inferior mesenteric vessels
• Surgical correction of mesenteric defect
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of dilated small-bowel loops
Image Interpretation Pearls
• Cluster of dilated small-bowel loops lateral to
ascending or descending duodenum with
crowded/twisted mesenteric vessels
I SELECTED REFERENCES
1. Catalano OA et al: Internal hernia with volvulus and
intussusception: case report. Abdom Imaging, 2004
2. Blachar A et al: Gastrointestinal complications of
laparoscopic Roux-en-Y gastric bypass surgery: clinical and
imaging findings. Radiology. 223(3): 625-32, 2002
3. Blachar A et al: Internal hernia: an increasingly common
cause of small bowel obstruction. Semin Ultrasound CT
MR. 23(2): 174-83,2002
4. Blachar A et al: Radiologist performance in the diagnosis of
internal hernia by using specific CT findings with
emphasis on transmesenteric hernia. Radiology. 221(2):
422-8,2001
5. Blachar A et al: Bowel obstruction following liver
transplantation: clinical and ct findings in 48 cases with
emphasis on internal hernia. Radiology. 218(2): 384-8,
2001
6. Blachar A et al: Internal hernia: clinical and imaging
findings in 17 patients with emphasis on CT criteria.
Radiology. 218(1): 68-74, 2001
7. Khan MA et al: Paraduodenal hernia. Am Surg. 64(12):
1218-22, 1998
8. Suchato C et al: CT findings in symptomatic left
paraduodenal hernia. Abdom Imaging. 21(2): 148-9, 1996
9. Zarvan NP et al: Abdominal hernias: CT findings. AJRAm J
Roentgenol. 164:1391-1395, 1995
10. Hamy A et al: Left-sided paraduodenal internal hernia
containing sigmoid colon: diagnosis based on findings on
barium examinations. AJRAm J Roentgenol.
162:1500-1501, 1994
11. Lee GH et al: CT imaging of abdominal hernias. AJRAm J
Roentgenol. 161:1209-1213, 1993
12. Meyers MA: Paraduodenal hernias. Radiologic and
arteriographic diagnosis. Radiology. 95: 29-37, 1970
 PARADUODENAL HERNIA
I IMAGE GALLERY 1
39
Typical
(Left) Axial CECT shows left
paraduodenal hernia with
cluster of bowel between
pancreas and stomach.
(Right) Axial CECT shows left
paraduodenal hernia with
inward-directed mesenteric
vessels.

Typical
(Left) Axial CECT shows left
paraduodenal hernia with
sac of bowel behind
stomach and
inward-directed, engorged
mesenteric vessels. (Right)
Axial CECT shows sac of
dilated bowel with dilated,
distorted mesenteric vessels.

Typical
(Left) Axial CECT shows
cluster of dilated bowel
media to ascending colon
with distorted, engorged
vessels (Courtesy O. Meyers,
MO). (Right) Axial CECT
shows right paraduodenal
hernia with dilated jejunum
and its mesenteric vessels
twisted and displaced
(Courtesy O. Meyers, MOJ.
 TRANSMESENTERIC POST-OPERATIVE HERNIA
1
40

Graphic shows dilated small bowel (58) that has Axial CECT shows dilated 58 with no overlying omental
herniated through a mesenteric defect. Note peripheral fat, displacing transverse colon medially and inferiorly.
position of 58 and medial displacement of colon,
displaced mesenteric vessels.

• External: Prolapse of bowel loops via defect in wall

ITERMINOlOGY
Definitions
• Protrusion of bowel loops through an acquired defect
of mesentery within abdominal cavity
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Cluster of dilated small bowel
loops with distorted mesenteric vessels
• Location
o Abnormal opening in mesentery of small bowel or
colon
o Hernia post Roux-en-Y gastric bypass surgery via
• Transverse mesocolon (80%)
• Small bowel mesentery (14%)
• Behind Roux loop (6%): Peterson type hernia
o Hernia post liver transplant via
• Transverse mesocolon (more common)
• Small bowel mesentery
• Size: Mesenteric defect varies from few mm to few cm
• Key concepts
o Classification of hernias based on anatomic location
• Internal or intra-abdominal: Herniation of bowel
loops via defect within abdominal cavity
of abdomen or pelvis
• Diaphragmatic: Protrusion of bowel loops via
hiatus, congenital or acquired defect
o Subclassification of internal hernias
• Transmesenteric hernia
• Para duodenal hernia
• Foramen of Winslow, pericecal hernias
• Intersigmoid & transomental hernias
o Transmesenteric hernia two types based on etiology
• Transmesenteric post-operative hernia (most
common subtype of all internal hernias in adults)
• Transmesenteric congenital hernia (most common
subtype of internal hernias in pediatric age group)
o 0.6-5.8% cases of small bowel obstruction (SBO) are
due to internal hernias in selected populations
• Transmesenteric post-operative hernia accounts
more than half of these cases of SBO
• Examples: Roux-en-Y gastric bypass surgery, liver
transplantatibn, small & large bowel surgery
Radiographic Findings
• Radiography
o Plain x-ray abdomen (supine)
• "Closed loop": Markedly distended segment of
small bowel (indicates SBO)
• Crowded & dilated small bowel loops in an
abnormal location
• Multiple air fluid levels

DDx: Cluster of Dilated Bowel loops

SBO Adhesions Closed Loop SBO SB Volvulus SB Volvulus

 TRANSMESENTERIC POST-OPERATIVE
Key Facts
Terminology
• Protrusion of bowel loops through an acquired defect
of mesentery within abdominal cavity
...
Imagmg Fmdmgs
• Best diagnostic clue: Cluster of dilated small bowel
loops with distorted mesenteric vessels
• Evidence of small bowel obstruction (100%)
• Transition point dilated-nondilated (100%)
• Location: Usually adjacent to abdominal wall
• Hernia usually not encapsulated or enveloped in a sac
• Mesenteric vessels: Engorged, crowded or twisted
• "Whirl sign": Small bowel volvulus with twisted
mesenteric vessels
Top Differential Diagnoses
• Closed loop obstruction (SBO)
• Fluoroscopic guided small bowel follow through
o Crowding of bowel loops in an abnormal location
• Location: Right side abdomen (more common)
o Do not appear as they are contained in a sac or
confining border
o Varying degrees of small bowel obstruction (SBO)
o Point of transition between dilated & nondilated
bowel (common)
o Some degree of fixation, stasis & delayed flow of
contrast seen in herniated bowel
o Inability to displace clustered loops by manual
palpation or change in position
o Lateral films: Useful in demonstrating displacement
of herniated bowel loops
CT Findings
• Cluster of dilated small bowel loops
o Evidence of small bowel obstruction (100%)
o Transition point dilated-nondilated (100%)
o Location: Usually adjacent to abdominal wall
• Hernia usually not encapsulated or enveloped in a sac
• Displacement of overlying omental fat of herniated
bowel loop (74%)
• Mesenteric vessels: Engorged, crowded or twisted
• Main mesenteric trunk: Right or left displacement
• Colon displacement
o Hepatic flexure displaced inferiorly & posteriorly
(more common)
o Ascending & descending colon displaced medially
(less common)
• Thick bowel wall & ascites (more common in cases
with bowel ischemia)
• "Whirl sign": Small bowel volvulus with twisted
mesenteric vessels
• Transmesenteric smaller hernias after Roux-en-Y
surgery (via transverse mesocolon)
o Small retrogastric cluster of small bowel loops
o Mass effect on posterior stomach wall
o Redundant dilated Roux loop
o No colon or fat displacement
HERNIA
1
Pathology . 41
• Roux-en- Y gastnc bypass
• Liver transplantation, sm.all or larg~ bowel surgery
• Post-operative: Due to pnor abdommal surgery
abnormal spaces or mesenteric defects are created
Clinical Issues
• Smaller: Easily reducible & clinically silent
• Periumbilical pain, symptoms of bowel obstruction
• Gender: Females more than males
Diagnostic Checklist
• Differentiate from other types of internal hernias
• Cluster of dilated small bowel loops which are not
encapsulated, not enveloped in a sac located adjacent
to abdominal wall usually on right side with crowded
or twisted mesenteric vessels
Ultrasonographic Findings
• Real Time: Dilated small bowel loops in an abnormal
location
Angiographic Findings
• Conventional
o Superior mesenteric arteriogram
• Abrupt angulation & displacement of visceral
branches as they pass through mesenteric defect
to supply herniated loops
Imaging Recommendations
• Helical CECT; small bowel follow through
I DIFFERENTIAL DIAGNOSIS
Closed loop obstruction (5BO)
• Obstruction of small bowel at two points
• Tends to involve mesentery & prone to produce a
volvulus
o Represents most common cause of strangulation
• Etiology
o Most often caused by an adhesive band
o Occasionally by an internal or external hernia
• Imaging features
o Markedly distended segment of fluid-filled small
bowel
o Volvulus
• Cor U-shaped or "coffee bean" configuration of
bowel loop
o Stretched mesenteric vessels converging toward site
of torsion
o Adjacent collapsed loops at site of obstruction
• Round, oval or triangular in shape
o "Beak sign"
• Fusiform tapering at point of torsion or
obstruction
o "Whirl sign"
• Due to tightly twisted mesentery with volvulus
o May be indistinguishable from trans mesenteric
hernia especially if associated with volvulus
 TRANSMESENTERIC
1 !PATHOLOGY
42 General Features
• Etiology
o Transmesenteric post-operative type
• Roux-en-Y gastric bypass
• Liver transplantation, small or large bowel surgery
o Transmesenteric congenital type
• Mesenteric defect located close to ligament of
Treitz or ileocecal valve
• Size of mesenteric defect: Usually 2-5 cm in
diameter
o Pathogenesis & mechanism
• Post-operative: Due to prior abdominal surgery
abnormal spaces or mesenteric defects are created
• Congenital: Developmental mesenteric anomalies
• Abnormal mesenteric fixation or defects may lead
to abnormal mobility of small bowel loops which
facilitates herniation
• Herniating segment: Small bowel loops
(jejunum/ileum)
• Herniation: Transient or intermittent
• Epidemiology
o Autopsy incidence
• Internal hernias: 0.2-0.9%
o Transmesenteric post-operative hernia
• Accounts more than 50% of internal hernias
• Associated abnormalities
o Roux-en-Y gastric bypass loop
o Liver transplant
o Evidence of small or large bowel surgery
Gross Pathologic & Surgical Features
• Abnormal opening in mesentery of small bowel/colon
• Dilated small bowel loops herniating via a mesenteric
defect
• Distorted or twisted mesenteric vessels
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Smaller: Easily reducible & clinically silent
o Larger
• Vague discomfort, abdominal distension
• Periumbilical pain, symptoms of bowel
obstruction
• Palpable mass, localized tenderness
o Small bowel obstruction
• Low grade, chronic & recurrent
• May be high grade & acute
o Onset usually months after original surgery
Demographics
• Age
o Transmesenteric post-operative
• Usually obese adult age group who have
undergone Roux-en-Y gastric bypass surgery
• Typically between 4th & 6th decade
• Gender: Females more than males
Natural History & Prognosis
• Complications
POST-OPERATIVE HERNIA
o Volvulus, ischemia, strangulation
o Bowel gangrene, shock & death
• Prognosis
o Early surgical correction: Good
o Delayed surgical correction & complications: Poor
Treatment
• Laparotomy, bowel decompression
• Avoid injury to superior & inferior mesenteric vessels
• Surgical correction of mesenteric defect
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other types of internal hernias
Image Interpretation Pearls
• Cluster of dilated small bowel loops which are not
encapsulated, not enveloped in a sac located adjacent
to abdominal wall usually on right side with crowded
or twisted mesenteric vessels
I SELECTED REFERENCES
1. Catalano OA et al: Internal hernia with volvulus and
intussusception: case report. Abdom Imaging, 2004
2. Filip ]E et al: Internal hernia formation after laparoscopic
Roux-en-Y gastric bypass for morbid obesity. Am Surg.
68(7): 640-3, 2002
3. Blachar A et al: Gastrointestinal complications of
laparoscopic Roux-en-Y gastric bypass surgery: clinical and
imaging findings. Radiology. 223(3): 625-32, 2002
4. Blachar A et al: Internal hernia: an increasingly common
cause of small bowel obstruction. Semin Ultrasound CT
MR. 23(2): 174-83,2002
5. Blachar A et al: Radiologist performance in the diagnosis of
internal hernia by using specifie CT findings with
emphasis on transmesenteric hernia. Radiology. 221(2):
422-8,2001
6. Huang YC et al: Left paraduodenal hernia presenting as
intestinal obstruction: report of one case. Acta Paediatr
Taiwan. 42(3): 172-4, 2001
7. Blachar A et al: Bowel obstruction following liver
transplantation: clinical and ct findings in 48 cases with
emphasis on internal hernia. Radiology. 218(2): 384-8,
2001
8. Delabrousse E et al: Strangulated transomental hernia: CT
findings. Abdom Imaging. 26(1): 86-8, 2001
9. Blachar A et al: Internal hernia: clinical and imaging
findings in 17 patients with emphasis on CT criteria.
Radiology. 218(1): 68-74, 2001
10. Rha SE et al: CT and MR imaging findings of bowel
ischemia from various primary causes. Radiographies.
20(1): 29-42, 2000
11. Ha HK et al: Usefulness of CT in patients with intestinal
obstruction who have undergone abdominal surgery for
malignancy. A]R Am] Roentgenol. 171(6): 1587-93, 1998
12. Khanna A et al: Internal hernia and volvulus of the small
bowel following liver transplantation. Transpl Int. 10(2):
133-6, 1997
 TRANSMESENTERIC POST-OPERATIVE HERNIA
I IMAGE GALLERY 1
43
Typical
(Left) Axial CECT shows
herniation of bowel through
mesenteric defect, causing
twisting of mesenteric vessels
(arrow) and SB obstruction.
(Right) Surgical photograph
shows shows mesenteric
defect through which all the
visualized bowel had
herniated and obstructed.

Typical
(Left) SBFT following
Roux-en-Y gastric bypass
surgery (RYCB) shows
herniation, dilation and
twisting of Roux limb due to
internal hernia. (Right) Axial
CECT following RYCB shows
dilated, twisted Roux limb
(arrow) lying behind
bypassed stomach (open
arrow).

Typical
(Left) Axial CECT shows
cluster of dilated SB lateral to
and displacing colon (open
arrow). Focal ascites and
engorged vessels. (Right)
Axial CECT shows
transmesenteric internal
hernia with displaced,
crowded mesenteric vessels
converging at the mesenteric
defect (curved arrow).
 TRAUMATIC ABDOMINAL WALL HERNIA
1
44

Axial CECT shows traumatic avulsion of abdominal wall Axial CECT in patient with traumatic abdominal wall
muscles from pelvic insertion (arrow). hernia shows renal laceration and fractured ribs.

o Region of iliac crest; in seat belt injury (site of

ITERMINOLOGY junction of lap & shoulder strap)
Abbreviations and Synonyms o Through rent in retroperitoneum (rare)
• Traumatic abdominal wall hernia (TAWH) • Size: Anatomical defects vary from small tears to large
• Handlebar hernia disruptions
• Morphology
Definitions o Differing patterns of muscular & fascial disruption
• Traumatic disruption of musculature & fascia of • Due to different types of forces involved & tensile
anterior abdominal wall properties of various areas in abdominal wall
o With herniation of intestinal loops into • Most elastic structure, skin, remains intact
subcutaneous space
CT Findings
• Handlebar hernia (HH) is a localized abdominal wall
hernia caused by handlebar(or similar) injury • Size & site of defect
• Identify nature of herniated contents
• Rent in abdominal wall through which intestinal
I IMAGING FINDINGS loops protrude into subcutaneous space
• Avulsion of abdominal wall muscles; all layers of
General Features abdominal wall may be disrupted
• Best diagnostic clue • Associated visceral injury
o History of recent trauma, without skin penetration Imaging Recommendations
• No evidence of previous hernia defect at site of
• Best imaging tool: CT
injury
• Location
o Focal; lower abdominal quadrant defect; lateral to
rectus sheath; inguinal region; in blunt trauma
I DIFFERENTIAL DIAGNOSIS
o Larger, diffuse abdominal wall defects; sustained in Spigelian hernia
motor vehicle accidents • Through semilunar line at lateral border of rectus
abdominis muscle

DDx: Anterior Abdominal

... -..
Wall Defect or Mass
,-
,~

•-~ ,

Spigelian Hernia
v Ventral Hernia Lap. Port Hernia Rectus Hematoma
 TRAUMATIC ABDOMINAL WALL HERNIA

Key Facts
1
Terminology Imaging Findings 45
• Traumatic disruption of musculature & fascia of • Most elastic structure, skin, remains intact
anterior abdominal wall
• With herniation of intestinal loops into subcutaneous Top Differential Diagnoses
space • Spigelian hernia
• Handlebar hernia (HH) is a localized abdominal wall • Ventral hernia
hernia caused by handlebar (or similar) injury • Rectus hematoma

• Traumatic hernia may sometimes occur at typical

Spigelian hernia site
Ventral hernia
• Site of previous surgery, laparoscopy (lap port), or stab
wounds
• Midline; paramedian; small hernial aperture
Rectus hematoma
I PATHOLOGY
General Features
• General path comments
o Produced by sudden application of blunt force
• Insufficient to penetrate skin but strong enough to
disrupt muscle & fascia
o Sudden 1 in intra-abdominal pressure in
combination with direct impact
o Persistent & severe cough ("internal trauma")
• Acting over a weak abdominal wall subjected to
stress due to repetitive muscular contractions
o Shearing force applied across bony prominences
• Etiology
o Low energy injuries: Impact on small blunt object
• HH: Produced by impaction of a bicycle handlebar
on abdominal wall
o High energy injuries: Motor vehicle accidents
o Even after relatively minor trauma; in children
• Epidemiology
o TAWH remains a rare clinical entity
o Diffuse defects are 1.4 to 1.6 times more frequent
than localized defects
• Associated abnormalities
o Duodenal or mesenteric hematoma, pancreatic
injuries with HH
o Intra-abdominal injuries with high energy TAWH
• Both bowel & solid viscera are at high risk
Natural History & Prognosis
• May not be diagnosed at initial presentation; present
as delayed consequence of trauma
• Complications: Bowel strangulation; ischemia
Treatment
• Low energy injuries: Local exploration; incision
overlying defect; laparoscopic; mesh technique
• High energy injuries: Immediate exploratory
laparotomy; midline incision; primary repair
I DIAGNOSTIC CHECKLIST
Consider
• Poor correlation between site of impact & abdominal
wall defect
• Can be easily missed in presence of major pelvic &
abdominal lesions
• Careful review of associated injuries to bowel &
mesentery should be undertaken
o CT findings may correlate poorly with severity of
injury in these areas
I SELECTED REFERENCES
1. Losanoff JE et al: Handlebar hernia: ultrasonography-aided
diagnosis. Hernia. 6(1):36-8, 2002
2. Vasquez JC et al: Traumatic abdominal wall hernia caused
by persistent cough. South Med]. 92(9): 907-8, 1999
3. Damschen DD et al: Acute traumatic abdominal hernia:
case reports. J Trauma. 36(2): 273-6, 1994
I IMAGE GALLERY

I CLINICAL ISSUES
Presentation
• Varying degrees of abdominal tenderness
• Abdominal skin ecchymosis or abrasions ("seat belt
ecchymosis")
• Reducible swelling or a cough impulse

(Left) Axial. CECT shows traumatic avulsion of muscles from pelvis

(arrow). (Right) Axial CECT shows pelvic fractures and mesenteric
bleeding in patient with traumatic abdominal wall hernia.
 MESENTERIC TRAUMA
1
46
Axial CECT shows large omental/mesenteric
with active bleeding (arrow).
ITERMINOlOGY
Definitions
• Injury to mesentery; often with bowel injury
I IMAGING FINDINGS
General Features
• Best diagnostic clue: CT; mesenteric hemorrhage, focal
bowel wall thickening, free peritoneal fluid
• Location: Mesentery of small bowel injured five times
more frequently than colonic mesentery
CT Findings
• Free intraperitoneal fluid: Hemoperitoneum in absence
of detectable solid visceral injury
o Fluid that is less dense than blood may represent
bowel contents; ominous finding
o Interloop fluid or trapped between mesenteric leaves
• Mesenteric stranding: heterogeneous attenuation
o Hemorrhage or edema; diffuse or focal
• Active hemorrhage: Mesenteric fluid collection that is
isodense to contrast-enhanced blood vessels
• "Sentinel clot sign": Focal heterogeneous high density
(> 60 HU) collection in mesentery
o Indicates mesenteric source of bleed
DDx: Mesenteric Edema/Blood
Pancreatic Trauma Shock Bowel
hematoma Axial CECT shows large omental/mesenteric hematoma
and active bleeding (arrow).
• Mesenteric pseudoaneurysm: Rare; indicates
substantial mesenteric + vascular injury
o High likelihood of continued, renewed hemorrhage
• CT signs of coexisting bowel injury
o Bowel wall thickening: Focal; > 3 mm thick or
disproportionate to normal bowel wall segments
• Perforation or bowel ischemia or infarction
• Enhance more than normal bowel wall segment
• Early after injury, focal + limited to area of injury;
CT finding can guide surgeon's exploration
• Delayed detection ~ inflammation generalized,
bowel wall thickening may become diffuse
o Free intraperitoneal air: Tiny bubbles of.
extraluminal gas
• Amount of air may be substantial or minimal
• Often, only minimal air seen; anteriorly near liver
• Trapped within or between folds of mesentery
o Free retroperitoneal air: Traumatic disruption of
duodenum or colon
o Extraluminal enteric contrast: Indicative of bowel
perforation; most dense near site of perforation
• Associated injuries to other organs; spleen, liver etc.
• CT helps distinguish operable from nonoperable cases
o Incidence of specific findings
• Free peritoneal fluid (96%) + mesenteric
infiltration (86%) + focal bowel-wall thickening
(61%)
after Trauma
Liver Laceration Liver Laceration
 MESENTERIC TRAUMA

Key Facts
1
Imaging Findings Pathology 47
• Best diagnostic clue: CT; mesenteric hemorrhage, • Blunt abdominal trauma: Motor vehicle accidents
focal bowel wall thickening, free peritoneal fluid • Assault; fall from height; lap belt; child abuse
• Isolated injuries of mesentery are rare
Top Differential Diagnoses
• Traumatic pancreatitis Clinical Issues
• Shock bowel • Delay in diagnosis: 1 Morbidity & mortality (5-65%)
• Hemoperitoneum

• + Associated abdominal injuries (43%) + free air

(32%) in surgical candidates
o In nonoperable cases: Bowel thickening (84%)
• Less frequently peritoneal fluid (21%), mesenteric
infiltration (26%) or associated injuries (5%)
Imaging Recommendations
• Best imaging tool: CT; high negative predictive value
Natural History & Prognosis
• Delay in diagnosis: 1 Morbidity & mortality (5-65%)
Treatment
• Surgery: Active bleeding; most bowel injuries
• Non-operative: Isolated mesenteric or bowel wall
hematoma

I DIFFERENTIAL DIAGNOSIS I DIAGNOSTIC CHECKLIST

Traumatic pancreatitis Consider
• Peripancreatic infiltration; edema, fluid collections, • High index of suspicion required
peri pancreatic hematoma, loss of fat planes o Findings can be subtle
• Thickening of anterior perirenal fascia • Active mesenteric bleeding or combination of
mesenteric hematoma + bowel wall thickening
Shock bowel o Usually require surgery
• Extensive infiltration of mesenteric fat planes
• Diffuse bowel wall thickening + abnormally intense
contrast enhancement of bowel mucosa I SELECTED REFERENCES
• Resolves soon after adequate fluid resuscitation 1. Hanks PW et al: Blunt injury to mesentery and small
Hemoperitoneum bowel: CT evaluation. Radiol Clin North Am.
41(6):1171-82,2003
• Hyperattenuating fluid in peritoneum; typically not as 2. Strouse P] et al: CT of bowel and mesenterie trauma in
hyperattenuating as extravasated contrast material children. Radiographies. 19(5):1237-50, 1999
• 30-50 HU; less dense if ascites or bowel contents 3. Rizzo M] et al: Bowel and mesenteric injury following
• Source of bleed: Viscerallaceration/"sentinel clot" sign blunt abdominal trauma: evaluation with CT. Radiology.
173(1): 143-8, 1989

I PATHOLOGY
I IMAGE GALLERY
General Features
• Etiology
o Blunt abdominal trauma: Motor vehicle accidents
o Assault; fall from height; lap belt; child abuse
• Epidemiology
o Isolated injuries of mesentery are rare
o Incidence: 5% following blunt abdominal trauma

I CLINICAL ISSUES
Presentation
• Triad of abdominal tenderness, rigidity, absent bowel
sounds (present in 1/3 of patients) (Left) Axial CECT shows mesenteric hematoma + active bleeding
• Serial abdominal examinations; may be unreliable (arrows). Also note "triangular" and inter/oop collections of blood
• Diagnostic peritoneal lavage: Insensitive to (open arrows). (Right) Axial CECT shows sentinel clot and active
retroperitoneal injuries; 1 non therapeutic laparotomies bleeding (arrow) in mesentery and blood in left paracolic gutter.
• Fluid or gas from lavage makes CT diagnosis difficult
 TRAUMATIC DIAPHRAGMATIC RUPTURE
1
48

Chest radiograph shows "elevated" and indistinct left Axial CECT shows "dependent viscera" sign with spleen
hemidiaphragm, tip of NC tube pointed up (arrow). and bowel abutting posterior ribs.

o Associated injuries (52-100%): Rib fractures; bowel,

ITERMINOLOGY splenic, liver & renal injuries
Definitions o Posterolateral defects diagnosed at CT in 6% of
• Diaphragmatic rupture with or without herniation of nontraumatic, asymptomatic adults
abdominal contents into thorax • May mimic diaphragmatic tears
• Seen more commonly on left side
• Represent congenital asymptomatic Bochdalek
I IMAGING FINDINGS hernias
Radiographic Findings
General Features
• Radiography
• Best diagnostic clue: Discontinuity of hemidiaphragm
o Nonvisualization of diaphragmatic contour
with air-filled bowel loops in thorax
o Abnormally elevated hemidiaphragm contour
• Location o Contralateral shift of mediastinum, rib fractures
o 90-98% on left side in location
o Lower lobe soft tissue density mass: Herniated solid
• Posterolateral part of diaphragm medial to spleen
organ, omentum or airless bowel loop
o 2-4% on right side
o Intrathoracic herniation of a hollow viscus
• Size (stomach, colon, small bowel with air-fluid levels)
o Blunt trauma: Most tears> 10 cm in length
o Site of diaphragmatic tear: Focal constricted gas
o Penetrating trauma
filled bowel loop (collar sign)
• Gun shot wounds (large blast injuries)
o Hydropneumothorax: Strangulation & lung injury
• Stab wounds (smaller injuries)
o Visualization of nasogastric (NG) tube above left
• Key concepts hemidiaphragm
o Due to blunt & penetrating trauma
o Lung effusion, contusion, atelectasis & phrenic
o Occur in 0.8-5.0% of all trauma patients
nerve palsy can mask diaphragmatic injury
o Accounts for 5% of all diaphragmatic hernias
• Fluoroscopic guided contrast studies
o 90% of all strangulated diaphragmatic hernias
o "Collar sign": Focal constricted (site of'tear) contrast
o Herniated organs: Stomach, colon, small bowel,
filled bowel loop partly in thorax & abdomen
omentum, spleen, liver

DDx: Elevate or Deformed Diaphragm

\
, " 'j "
r~.~.'r' 'f~

.~,''Jr'AJ
_t.W" .
Paralyzed Diaphragm ..
". ~.,-..J
, .; Eventration Bochdalek Hernia
 TRAUMATIC DIAPHRAGMATIC RUPTURE
I IMAGE GALLERY 1
51
Typical
(Left) Coronal reformation
from axial CECT shows
herniation of abdominal
contents into left thorax.
Note constriction of stomach
(arrow) at site of tear. (Right)
Axial CECT shows
dependent viscus sign as
stomach abuts posterior ribs.

Typical
(Left) Chest radiograph
shows "elevated", distorted
diaphragm and high position
of NC tube (arrow). (Right)
Axial CECT shows
dependent viscera, plus
colon and abdominal fat
lying lateral to diaphragm
(arrow), indicating thoracic
position.

Typical
(Left) Axial CECT shows
spleen in dependent position
and abdominal fat lateral to
diaphragm (arrow). (Right)
Axial CECT at "lung
windows" shows splenic
flexure of colon (arrow)
abutting lung and
pneumothorax.
 MESENTERIC CYST
1
52

Axial CECT shows water density mass with very thin Axial CECT shows water density, thin-walled mass,
wall, "indented" by a mesenteric vessel. Cystic "indented" by a mesenteric vessel. Cystic
lymphangioma. lymphangioma.

o Fluid-filled cystic structure with thin internal septa

ITERMINOlOGY o ± Internal echoes (debris, hemorrhage or infection)
Definitions Imaging Recommendations
• Generic descriptive term for a cystic mass arising in • Best imaging tool: CECT or CEMR
the mesentery or omentum, not from an
abdominopelvic viscus
• In this and other reviews, may refer to cystic I DIFFERENTIAL DIAGNOSIS
lymphangioma unless otherwise specified
Cystic lymphangioma
• Most common type of mesenteric cyst
I IMAGING FINDINGS • Endothelial cell lining the cyst with foam cells and
thin walls that contain lymphatic spaces, lymphoid
General Features tissue and smooth muscle
• Best diagnostic clue: Fluid-filled mass in the mesentery • Frequent attachment to bowel
• Location: Occur anywhere in the mesentery or • Predominantly in male children
omentum
• Size: Few mm to 40 cm in diameter Loculated ascites
• Morphology • Significant size collection may be indistinguishable
o May extend to retroperitoneum from mesenteric cyst
o Simple or multiple, unilocular or multilocular • Evaluate other causes (e.g., neoplasm, cirrhosis)
CT Findings Enteric duplication cyst
• Well-circumscribed mass with varying attenuation, • Unilocular or multilocular mass with thick walls (has
depends on the fluid (usually water density, (serous) or enteric lining and muscular wall)
less (chylous), rarely hemorrhagic) • Contrast-enhancement of cystic walls
• ± Fine calcifications along cyst wall
Enteric cyst
Ultrasonographic Findings • Hypoechoic mass with few, thin septa and without a
• Real Time visible wall

DDx: Mesenteric Mass - Cystic

Loculated Ascites Pseudomyxoma Pseudocyst Cystic Teratoma

 MESENTERIC CYST
Key Facts
1
Imaging Findings • Enteric duplication cyst 53
• Best diagnostic clue: Fluid-filled mass in the • Enteric cyst
mesentery • Pancreatic or non-pancreatic pseudocyst
• ± Internal echoes (debris, hemorrhage or infection) • Cystic mesothelioma
• Best imaging tool: CECT or CEMR • Cystic teratoma

Top Differential Diagnoses Diagnostic Checklist

• Cystic lymphangioma • First exclude visceral origin of "cyst"
• Loculated ascites • Histologic analysis is necessary to establish diagnosis
• Fluid-filled cyst; thin internal septa

o Good after surgery, 0-13.6% recurrence rate

Pancreatic or non-pancreatic pseudocyst
• Unilocular or multilocular, visible wall Treatment
• Sequela of pancreatitis or mesenteric hematoma • Enucleation of cyst ± bowel resection
Cystic mesothelioma
• Unilocular, anechoic thin-walled mass with acoustic
enhancement; rare, benign tumor
I DIAGNOSTIC CHECKLIST
Consider
Cystic teratoma
• First exclude visceral origin of "cyst"
• Cystic mass with septa and calcification, contains
• Histologic analysis is necessary to establish diagnosis
fat-density fluid
Image Interpretation Pearls
• Fluid-filled cyst; thin internal septa
I PATHOLOGY
General Features
• Epidemiology
I SELECTED REFERENCES
o Cystic lymphangioma 1. de Perrot M et al: Mesenteric cysts. Toward less confusion?
Dig Surg. 17(4):323-8,2000
• Rare; 1/140,000 in general admission, 1/20,000 in
2. Stoupis C et al: Bubbles in the belly: imaging of cystic
pediatric admission
mesenteric or omental masses. Radiographics.
Gross Pathologic & Surgical Features 14(4):729-37, 1994
3. Ros PR et al: Mesenteric and omental cysts: histologic
• Thin-walled, multi septated, serous or chylous fluid classification with imaging correlation. Radiology.
contents 164(2):327-32, 1987
• Often attached to bowel wall 4. Vanek VW et al: Retroperitoneal, mesenteric, and omental
cysts. Arch Surg. 119(7):838-42, 1984
Microscopic Features
• Cuboidal or columnar cell lining the cyst without
smooth muscle or lymphatic spaces within the walls I IMAGE GALLERY

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Abdominal distention, vague abdominal pain
o Vomiting, palpable abdominal mass
o Acute abdomen (10% of cases)
o Small bowel obstruction (most common in children)
Demographics
• Age
o Cystic lymphangioma
(Left) Axial CECT shows cystic lymphangioma, a thin-walled water
• Children and young adults; 33% < 15 years of age
density mesenteric mass, with scattered calcifications in septa. (Right)
Natural History & Prognosis Coronal T2WI MR shows large, mu/tiloculated water intensity cystic
lymphangioma.
• Complications
o Intestinal obstruction, volvulus, hemorrhage,
rupture, infection, sepsis, cystic torsion and
obstruction of the urinary and biliary tract
• Prognosis
 DESMOID
1
54

Axial CECT in a 36 year old man 72 months following Axial CECT 20 months following colectomy for Gardner
colectomy for Gardner syndrome shows solid syndrome shows rapid growth of mesenteric mass;
mesenteric desmoid (arrow). desmoid tumor.

o Locally aggressive mesenteric primary tumor

ITERMINOLOGY o Tend to arise in musculoaponeurotic planes
Abbreviations and Synonyms • Desmoid tumor of abdominal wall
o Tendency to invade locally & recur + grow very
• Aggressive fibromatosis
rapidly, especially in Gardner syndrome
Definitions o May involve bowel loops, bladder, ribs, pelvic bones
• Rare, benign, locally aggressive, non encapsulated o Sometimes classified as a low grade fibrosarcoma, or
tumor of connective or fibrous tissue as a subgroup of fibromatosis
o Usually associated with Gardner syndrome
• Familial polyposis coli, osteomas, dental defects,
I IMAGING FINDINGS congenital pigmented lesions of retina
• Desmoid tumor, mesenteric fibromatosis
General Features • Epidermoid (sebaceous) cyst & fibromas of skin
• Best diagnostic clue: Small bowel mesentery or • Periampullary, adrenal, thyroid & liver carcinomas
abdominal wall mass arising from scar of prior surgery o 75% of desmoid tumors: Previous abdominal surgery
• Location o 18-20% Gardner syndrome cases develop desmoids
o Abdominal o Accounts for 45% of fibrous lesions in Gardner
• Mesentery: Small bowel (most common)
• Musculature: Rectus, internal/external oblique, Radiographic Findings
psoas, pelvic (rare) • When associated with Gardner syndrome
• Retroperitoneum o Fluoroscopic guided double contrast studies
o Extra-abdominal • Familial polyposis: Innumerable varied sized
• Bladder, ribs & pelvic bones radiolucent filling defects
• Size: Mass may range from 5-20 cm CT Findings
• Morphology: Well-Jill-defined, tan or white, hard
• NECT
fibrous tissue mass o Abdominal desmoids can be solitary or multiple
• Key concepts o Mesenteric desmoids
o Abdominal desmoids can be solitary or multiple
• Soft tissue mass with well or ill-defined margins

DDx: Mesenteric Mass - Solid

Lymphoma Omental Metastases Carcinoid Fibros. Mesenteritis

 DESMOID
Key Facts
Terminology
• Rare, benign, locally aggressive, non encapsulated
tumor of connective or fibrous tissue
Imaging Findings
• Best diagnostic clue: Small bowel mesentery or
abdominal wall mass arising from scar of prior
surgery
• Tendency to invade locally & recur + grow very
rapidly, especially in Gardner syndrome
• Soft tissue mass with well or ill-defined margins
• "Whorled appearance": Radiating fibrotic strands into
adjacent mesenteric fat
• ± Displacement, retraction, compression: Bowel loops
Top Differential Diagnoses
• Lymphoma
• Isodense relative to muscle
• Usually involves small bowel mesentery
• "Whorled appearance": Radiating fibrotic strands
into adjacent mesenteric fat
• ± Displacement, retraction, compression: Bowel
loops
• ± Infiltration into adjacent organs & musculature
• ± Small bowel obstruction
o Abdominal wall desmoids
• Usually solid with well or partially well-defined
margins
• Homo-/heterogeneous density (higher HU than
muscle)
• Involves rectus or oblique muscles
o Large tumors with necrosis: Hypodense
• CECT
o Mesenteric desmoid: Minimal enhancement
o Abdominal wall desmoid: May enhance
o Show extent of mesenteric & small bowel invasion
o ± Encased or displaced mesenteric vasculature
• CT colonography after colonic air insufflation
(endoluminal images)
o Small or large, sessile or pedunculated polyps
MR Findings
• TlWI
o Poorly or well-circumscribed mass
o Hypointense to muscle
• T2WI: Hypointense or variable signal intensity
• T1 C+: Variable enhancement
• Multiplanar MR imaging
o Show origin, extent & invasion of tumor
Ultrasonographic Findings
• Real Time: Well-defined mesenteric mass with variable
echogenicity
Imaging Recommendations
• Helical NE + CECT
• Multiplanar MR imaging & Tl C+
1
• Omental or Mesenteric metastases 55
• Carcinoid
• Mesothelioma
• Fibrosing mesenteritis
• Hematoma
Pathology
• Previous abdominal surgery (75% of cases)
• 18-20% of patients with Gardner syndrome develop
desmoid tumor
• May be "rock hard", resistant to percutaneous biopsy
Diagnostic Checklist
• Check history of abdominal surgery & colonic polyps
• Rule out other solid mesenteric masses
• Soft tissue density mesenteric mass ± invasion,
displacement or encasement of bowel loops & vessels
I DIFFERENTIAL DIAGNOSIS
Lymphoma
• Median age 60 years, predominantly non-Hodgkin
• Imaging
o Tiny, round soft tissue densities to large, bulky
masses
o "Sandwich" sign: Lobulated, confluent mesenteric
masses surrounding superior mesenteric artery/vein
o Associated retroperitoneal adenopathy confirms
Omental or Mesenteric metastases
• Usually multiple; less well-defined
Carcinoid
• Desmoplastic reaction (not found in desmoids)
• Most common primary tumor of small bowel
o Usually seen in ileum
• Soft tissue mass in mesentery + radiating strands
o Strands represent thickened neurovascular bundles
• Segmental thickening of adjacent bowel loops
• 50-85% of cases have nodal metastases
• Liver metastases seen in 80% of lesions> 2 em
• Encasement of mesenteric vessels ~ bowel ischemia
Pseudocyst (pancreatic)
• Cystic mass with infiltration of peripancreatic fat
• More common in body & tail + changes of pancreatitis
• NECT
o Round or oval; hypodense (near water HU)
o Lobulated, mixed HU lesion (hemorrhagic/infected)
o Acute pancreatitis>Enlarged pancreas
o Chronic pancreatitis: Gland atrophy, dilated MPD &
intraductal calculi
• CECT: Enhancement of wall, not contents
• MRCP: Hyperintense pseudocyst
Mesothelioma
• Arises from serosal lining of pleural & peritoneal cavity
• Mostly affects males exposed to asbestos
• Peritoneal cavity is involved alone or in association
with pleural disease
• Imaging
o Malignant peritoneal mesothelioma
 DESMOID
1 • Peritoneal thickening, irregularity & nodularity
• ± Omental & mesenteric involvement; ascites
56 • Adhesions & fixation of bowel loops
• Rarely present as large solitary mesenteric mass
o Benign cystic peritoneal mesothelioma
• Multiloculated cystic mass lesion
• Usually seen in women of reproductive age
• Strong predilection for pelvic viscera
Fibrosing mesenteritis
• Usually less mass-like than desmoid
• Infiltrative, can be desmoplastic mimicking carcinoid
Hematoma
• Cause: Blunt trauma, excessive anticoagulation,
thrombocytopenia
• Acute hematoma
o Typically quite dense (50-60 HU)
o Focal/dispersed between leaves of mesentery
o t In density attaining of water HU by 2 weeks
I PATHOLOGY
General Features
• General path comments
o Fibroproliferative lesion resembling scar tissue
o Desmoid tumors may be an intermediate step
between a reparative process and a true malignancy
• Genetics: May be due to somatic mutation in APC
gene 5q
• Etiology
o Exact cause is unknown
o Previous abdominal surgery (75% of cases)
• Pre-existing surgical scar
o Most often in women of childbearing age
o Gardner syndrome patients
• Due to mutation in APC gene (5q)
• After surgery in mesentery or abdominal wall
o May be sporadic
• Epidemiology
o Usually rare
o 18-20% of patients with Gardner syndrome develop
desmoid tumor
• Mesenteric more than abdominal wall desmoid
o Increased incidence in child bearing age women
• Associated abnormalities: Components of Gardner
syndrome
Gross Pathologic & Surgical Features
• Tan/white, well or poorly defined, firm mass
• May be "rock hard", resistant to percutaneous biopsy
Microscopic Features
• Well-differentiated fibroblasts invading surrounding
tissues
• Elongated spindle-shaped cells of uniform appearance
with dense bands of collagen
ICLINICALISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic; abdominal pain, palpable mass
o Acute abdominal findings
• Due to bowel obstruction, ischemia
• Most common sign: Mass in abdominal wall at site of
prior surgery
Demographics
• Age: 70% of cases seen between 20-40 years old
• Gender: M:F = 1:3
Natural History & Prognosis
• Complications
o Locally aggressive growth pattern
o Necrosis, cystic degeneration ~ abscess
o Rarely metastasize
o High recurrence rate: 25-65%
• Prognosis
o Poor prognostic signs
• Size of tumor (> 10 cm), multiplicity
• Extensive infiltration & tethering of bowel loops
• Encasement of mesenteric vessels & ureters
Treatment
• Wide surgical resection is treatment of choice
• Radiation therapy: Successful in abdominal wall rather
than mesentery
• Steroids, NSAIDs (e.g., sulindac)
• Antiestrogen (e.g., tamoxifen) therapy
• Low-dose chemotherapy & interferon therapy
• Complete resection may require small bowel
transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Check history of abdominal surgery & colonic polyps
• Look for other components of Gardner syndrome
• Rule out other solid mesenteric masses
Image Interpretation Pearls
• Soft tissue density mesenteric mass ± invasion,
displacement or encasement of bowel loops & vessels
I SELECTED REFERENCES
1. Sheth S et al: Mesenteric neoplasms: CT appearances of
primary and secondary tumors and differential diagnosis.
Radiographies. 23(2):457-73; quiz 535-6,2003
2. Healy]C et al: MR appearances of desmoid tumors in
familial adenomatous polyposis. A]R Am] Roentgenol.
169(2):465-72, 1997
3. Mindelzun RE et al: The misty mesentery on CT:
differential diagnosis. A]R Am] Roentgenol. 167(1):61-5,
1996
4. Kawashima A et al: CT of intraabdominal desmoid tumors:
is the tumor different in patients with Gardner's disease?
A]R Am] Roentgenol. 162(2):339-42, 1994
5. Ichikawa T et al: Abdominal wall desmoid mimicking
intra-abdominal mass: MR features. Magn Reson Imaging.
12(3):541-4, 1994
6. Einstein DM et al: Abdominal desmoids: CT findings in 25
patients. A]RAm] Roentgenol. 157(2):275-9, 1991
7. Casillas] et al: Imaging of intra- and extra abdominal
desmoid tumors. Radiographics. 11(6):959-68, 1991
 DESMOID
I IMAGE GALLERY 1
57
Typical
(Left) Axial CECT shows
desmoid in subcutaneous
tissue (arrow) adjacent to
scar from prior paramedian
incision. (Right) Axial CECT
shows multiple omental
masses (arrows) near site of
prior colon surgery. Note
surgical clip (open arrow).

Typical
(Left) Axial CECT in patient
with Gardner syndrome
shows bilobed large
mesenteric desmoid tumor.
(Right) Axial NECT in patient
with Gardner syndrome
shows desmoid tumors filling
the abdomen, obstructing
kidneys, and deforming the
abdominal wall.

Typical
(Left) Axial CECT in a 79
year old woman with a
homogeneous omental mass;
sporadic form of desmoid.
(Right) Axial NECT in a 79
year old woman. CT-guided
biopsy showed "rock hard"
mass, but enough tissue to
confirm desmoid tumor.
 MESOTHELIOMA
1
58

Axial CECT shows stellate thickened mesentery and Axial CECT shows irregular, thickened peritoneum
diffusely thickened bowel loops with serosalimplants. (arrow). Note bilateralcalcified pleural plaques (open
arrows).

o Two primary forms

ITERMINOLOGY • Desmoplastic form: Diffuse disease thickening
Abbreviations and Synonyms peritoneal surfaces and enveloping viscera
• Malignant mesothelioma (MM), peritoneal • Focal form: Large tumor mass in upper abdomen
mesothelioma (PM) with scattered peritoneal nodules
• Variant: Benign cystic mesothelioma = multilocular Radiographic Findings
peritoneal inclusion cyst = cystic mesothelioma of the • Radiography
peritoneum = multicystic peritoneal mesothelioma o Calcified pleural plaques in 50% of peritoneal
Definitions mesotheliomas versus 20% of pleural mesotheliomas
• Primary neoplasm arising from serosal lining of • Signifies heavier asbestos exposure in patients
peritoneum with peritoneal mesothelioma
• Fluoroscopy
o Separation and fixation of bowel loops
o Spiculation of loops when bowel wall invaded
I IMAGING FINDINGS o Segmental stenoses with circumferential bowel
General Features invasion
• Best diagnostic clue: Peritoneal masses or omental cake CT Findings
associated with calcified pleural plaques
• NECT
• Location o Calcified pleural plaques
o 60% of malignant mesotheliomas arise in pleura
o Calcification in peritoneal masses very uncommon
o 20-30% of malignant mesotheliomas arise in
peritoneum • CECT
o Stellate, thickened mesentery secondary to
o May also arise in pericardium, tunica vaginalis
encasement and straightening of mesenteric vessels
• Size o Omental and peritoneal-based masses
o Involves peritoneal surfaces diffusely
o Spreads along serosal surfaces and directly invades
o Focal masses may be several mm to several cm
adjacent viscera, especially colon and liver
• Morphology

DDx: Peritoneal and Omental Masses

\
••..•
... :
-
'"
~.
:."
••••
.•. \
,.. w:.
'1'"-
.

, ".~. • ",.
~«~.
d
\' .-'" ' /
\

Carcinomatosis Pseudomyxoma Lymphomatosis Tuberculosis

 MESOTHELIOMA
Key Facts
1
Terminology Pathology 59
• Primary neoplasm arising from serosal lining of • Malignant mesothelioma: Associated with asbestos
peritoneum exposure
• Rare: 1-2 cases/million
Imaging Findings • Open biopsy rather than FNA often needed for
• Best diagnostic clue: Peritoneal masses or omental diagnosis
cake associated with calcified pleural plaques
• Stellate, thickened mesentery secondary to Clinical Issues
encasement and straightening of mesenteric vessels • Extremely poor prognosis
• Omental and peritoneal-based masses • Remains confined to abdominal cavity and invades
• Spreads along serosal surfaces and directly invades locally
adjacent viscera, especially colon and liver
• Variable amount of ascites; massive ascites Diagnostic Checklist
uncommon • Presence of distant metastases outside abdominal
• Benign cystic mesothelioma: Multiloculated cavity makes PM unlikely
thin-walled cystic pelvic mass with anechoic spaces

o Variable amount of ascites; massive ascites

uncommon
I DIFFERENTIAL DIAGNOSIS
o Benign cystic mesothelioma Neoplastic
• Low density multiloculated cystic mass in pelvis • Peritoneal carcinomatosis
• May have calcifications o Most common cause of omental cake and peritoneal
MR Findings implants
o Serosal spread of adenocarcinoma, especially
• TlWI
o Low-intermediate signal intensity omental and ovarian, stomach, colon and pancreas
peritoneal masses with encasement of viscera • Pseudomyxoma peritonei
o Benign cystic mesothelioma: Hypointense o Low attenuation peritoneal masses
multicystic mass o Implants cause scalloping of visceral serosal surfaces
• T2WI • Lymphoma
o High signal intensity peritoneal nodules o Concomitant lymphadenopathy
o Fluid-fluid levels secondary to hemorrhage o "Sandwich sign" = confluent mesenteric nodal
o Benign cystic mesothelioma: Intermediate to high masses surrounding mesenteric vessels
signal intensity multiloculated cystic mass o Ascites without loculation
• T1 C+ Infectious
o Enhancing peritoneal thickening and nodules
• Tuberculosis
o Improved conspicuity of enhancing masses with o Low attenuation lymphadenopathy in 40% of cases
fat-saturation pulses o High attenuation ascites (25-45 HU)
Ultrasonographic Findings o Lymphadenopathy most often mesenteric, omental
• Real Time and peripancreatic rather than retroperitoneal
o Hypoechoic, sheet-like peritoneal masses o Thickened cecum and terminal ileum
o Echogenic areas within hypoechoic masses o Smooth peritoneal thickening with pronounced
representing entrapped mesenteric or omental fat enhancement
o Thickened omentum o Transperitoneal permeation
o Benign cystic mesothelioma: Multiloculated Inflammatory
thin-walled cystic pelvic mass with anechoic spaces • Retractile mesenteritis
Nuclear Medicine Findings
• Gallium scan
o Diffuse uptake I PATHOLOGY
Imaging Recommendations General Features
• Best imaging tool: Contrast-enhanced CT • General path comments
• Protocol advice o Multifocal origin from mesothelial lining of
o Use water or oral contrast to distend small bowel abdomen and pelvis
loops o Three histologic types
o Coronal reformations useful for detecting implants • Epithelial 54%
near diaphragm • Sarcomatoid 21%
• Biphasic (mixed epithelial-sarcomatoid) 25%
• Genetics
 MESOTHELIOMA
1 o Complex karyotypes • Benign cystic mesothelioma: Non-lethal but locally
• Deletions in 1p, 3p, 6q, 9p, 15q and 22q in recurrent
60 various combinations
Treatment
• Etiology
o Malignant mesothelioma: Associated with asbestos • Options, risks, complications
exposure o Cytoreductive surgery and peritonectomy combined
• 20-40 year latency between exposure and with intra-peritoneal chemotherapy
diagnosis • Heated intra-operative intraperitoneal
o Simian virus-40 may be co-carcinogen chemotherapy often used
o Benign cystic mesothelioma: Not asbestos related
• Epidemiology
o Rare: 1-2 cases/million I DIAGNOSTIC CHECKLIST
o 200-400 cases diagnosed annually in U.S.
Consider
o Disease clusters around shipyards, docks, asbestos
mines and factories • Consider PM in patients with diffuse peritoneal tumor
on CT and stigmata of asbestos exposure
o Non occupational exposure to asbestos and zeolites
common in Turkey • Peritoneal carcinomatosis is much more common than
mesothelioma
• Associated abnormalities: Asbestos-related pleural and
parenchymal lung disease Image Interpretation Pearls
Gross Pathologic & Surgical Features • Presence of distant metastases outside abdominal
cavity makes PM unlikely
• Solid tumor masses growing along serosal surfaces
• Encasement and invasion of adjacent viscera
• Can recur along surgical and laparoscopy tracts
I SELECTED REFERENCES
Microscopic Features 1. Wong WL et al: Best Cases from the AFIP: Multicystic
• Variable histologic appearance of tumor cells Mesothelioma. Radiographies. 24(1): 247-250, 2004
o Open biopsy rather than FNA often needed for 2. Sheth S et al: Mesenteric neoplasms: CT appearances of
diagnosis primary and secondary tumors and differential diagnosis.
• Positive immunostaining for calretinin, keratin, Radiographies. 23(2):457-73; quiz 535-6, 2003
3. Hanbidge AE et al: US of the peritoneum. Radiographies.
vimentin and thrombomodulin
23(3):663-84; discussion 684-5, 2003
4. Sugarbaker PH et al: A review of peritoneal mesothelioma
at the Washington Cancer Institute. Surg Oncol Clin N
I CLINICAL ISSUES Am. 12(3):605-21, xi, 2003
5. Puvaneswary M et al: Peritoneal mesothelioma: CT and
Presentation MRI findings. Australas Radiol. 46(1): 91-6, 2002
• Most common signs/symptoms 6. Mohamed F et al: Peritoneal mesothelioma. Curr Treat
o "Pain-predominant" type: Dominant tumor mass Options Oncol. 3(5):375-86, 2002
with little ascites 7. DeVita V et al: Cancer: Principles and practice of
o "Ascites-predominant" type: Abdominal distention Oncology. 6th ed. Baltimore, Lippincott Williams and
Wilkins. 1937-1969,2001
o Other signs/symptoms Haliloglu M et al: Malignant peritoneal mesothelioma in
8.
• Weight loss, malaise, cramping, new-onset hernia two pediatric patients: MR imaging findings. Pediatr
• Clinical profile: History of asbestos exposure for Radiol. 30(4):251-5, 2000
malignant mesothelioma 9. Gore R et al: Textbook of Gastrointestinal Radiology. 2nd
ed. Philadelphia, W.B. Saunders, 1980-1992,2000
Demographics 10. Kim Y et al: Peritoneal lymphomatosis: CT findings.
• Age Abdom Imaging. 23(1):87-90, 1998
o Malignant mesothelioma: Usually 6th-7th decade, 11. Ozgen A et al: Giant benign cystic peritoneal
but can occur at any age mesothelioma: US, CT, and MRI findings. Abdom Imaging.
o Benign cystic mesothelioma: 3rd-4th decade 23(5): 502-4, 1998
12. ]advar H et al: Still the great mimicker: abdominal
• Gender tuberculosis. A]R Am] Roentgenol. 168(6):1455-60, 1997
o Malignant mesothelioma: M > > F 13. Smith TR: Malignant peritoneal mesothelioma: marked
o Benign cystic mesothelioma: M < < F variability of CT findings. Abdom Imaging. 19(1):27-9,
1994
Natural History & Prognosis 14. Stoupis C et al: Bubbles in the belly: imaging of cystic
• Extremely poor prognosis mesenteric or omental masses. Radiographies.
o Median survival 6 months; death usually within 1 14(4):729-37, 1994
year 15. Akhan 0 et al: Peritoneal mesothelioma: sonographic
• Solitary tumors have better prognosis than diffuse findings in nine cases. Abdom Imaging. 18(3): 280-2, 1993
intra-abdominal disease 16. Ros PR et al: Peritoneal mesothelioma. Radiologic
• Remains confined to abdominal cavity and invades appearances correlated with histology. Acta Radiol.
32(5):355-8, 1991
locally 17. Cozzi G et al: Double contrast barium enema combined
o Does not disseminate hematogenously to brain, with non-invasive imaging in peritoneal mesothelioma.
bone, or lung Acta Radiol. 30(1): 21-4, 1989
 MESOTHELIOMA

I IMAGE GALLERY 1
61
Typical
(Left) Axial CECT shows
marked omental thickening
(arrow) and encasement of
bowel loops (curved arrow).
(Right) Axial US of left lower
quadrant shows hypoechoic
mass (open arrows) encasing
rounded, echogenic loops of
bowel and mesenteric
leaves.

Variant
(Left) Axial CECT shows
large, complex mass
invading the spleen.
Peritoneal tumor (arrow) is
also present. (Right) Axial
NECT in patient with renal
insufficiency shows lobulated
mass (arrow) along
peritoneal surface. Surgical
biopsy confirmed malignant
mesothelioma.

Other
(Left) Axial CECT shows
multiloculated cystic mass
filling the pelvis. Surgical
excision proved the mass to
be a benign cystic
mesothelioma. (Right) Axial
CECT shows mu/tiloculated
cystic mass, which proved to
be a benign cystic
mesothelioma.
 PERITONEAL METASTASES

62

Axial anatomic rendering of peritoneal metastases. Note Axial US demonstrates large omental cake (arrows)
anterior omental cake (arrow) and serosal implants from ovarian carcinoma.
(open arrows).

ITERMINOLOGY Radiographic Findings

• Radiography
Abbreviations and Synonyms o Plain film findings of ascites
• Peritoneal carcinomatosis, peritoneal implants, • Medial displacement of cecum in 90% of patients
omental caking with significant ascites
Definitions • Pelvic "dog's ear" in 90% of patients with
• Metastatic disease to the omentum, peritoneal surface, significant ascites
peritoneal ligaments and/or mesentery • Medial displacement of lateral liver edge (Hellmer
sign) in 80% of patients with significant ascites
• Bulging of flanks, central displacement of bowel
loops, indistinct psoas margin
I IMAGING FINDINGS o Plain film findings of small bowel obstruction
General Features • Dilated small bowel> 3 em
• Best diagnostic clue • Fluid-fluid levels in small bowel on upright film
o Omental caking, soft tissue implants on peritoneal • "String of pearls" sign
surface are best diagnostic clues • Collapsed gasless colon
o "Scalloped" contour of liver/spleen from • Fluoroscopy
pseudomyxoma peritonei o Barium studies
o Cystic peritoneal masses with ovarian carcinoma on • Small bowel follow through (SBFT):Dilated bowel
peritoneal surfaces with transition zone; partial small bowel
o Ascites, mesenteric stranding, bowel obstruction obstruction
• Location: Peritoneum, mesentery, peritoneal ligaments • Mural extrinsic filling defects due to serosal
• Size: Variable; 5 mm nodules to large confluent implants in small bowel
omental masses ("caking") • Spiculated extrinsic impression due to tethering of
• Morphology: Nodular, plaque-like or large omental rectosigmoid from intraperitoneal mets to pouch
mass of Douglas
• Scalloping of cecum from peritoneal implants

DDx: Peritoneal Abnormalities

TB Peritonitis Papillary Serous CA Mesothelioma Pseudomyxoma

 PERITONEAL METASTASES

Key Facts
1
Terminology Top Differential Diagnoses 63
• Peritoneal carcinomatosis, peritoneal implants, • TB peritonitis
omental caking • Papillary serous carcinoma of peritoneum
• Metastatic disease to the omentum, peritoneal • Peritoneal mesothelioma
surface, peritoneal ligaments and/or mesentery
Pathology
Imaging Findings • Peritoneal metastases indicate Stage IV disease
• Omental caking, soft tissue implants on peritoneal
surface are best diagnostic clues Clinical Issues
• Location: Peritoneum, mesentery, peritoneal • Most common signs/symptoms: Abdominal
ligaments distension and pain, weight loss; ascites mayor may
• Ascites, nodular thickening/enhancement of not be present
peritoneum, hypovascular omental masses on CECT • Gender: More common in females than males, due to
• Best imaging tool: CECT or MRI ovarian CA
• Oral and LV. contrast with CECT; Diagnostic Checklist
gadolinium-enhanced GRE or T1 sequence with MRI
• TB peritonitis

• "Omental caking" may cause invasion of • Calcification in 14% of cases

transverse mesocolon with nodularity &
spiculation of superior contour
CT Findings
• NECT: Ascites
• CECT
o Ascites, nodular thickening/enhancement of
peritoneum, hypovascular omental masses on CECT
o Spiculated mesentery
o Evidence of bowel obstruction with delineation of
transition zone from dilated to non-dilated bowel
MR Findings
• T1WI: Low signal ascites, medium signal omental
caking
• T2WI: Intermediate signal peritoneal mass and high
signal ascites
• T1 C+: Abnormal enhancement of peritoneum with
gadolinium, hypointense nodules and masses
Ultrasonographic Findings
• Real Time
o Complex ascites with septations, hypoechoic
omental masses (caking), peritoneal implants
o Not sensitive for peritoneal implants in absence of
ascites
Imaging Recommendations
• Best imaging tool: CECT or MRI
• Protocol advice
o Oral and LV. contrast with CECT;
gadolinium-enhanced GRE or T1 sequence with MRI
• 150 ml LV. contrast at 2.5 ml/sec with 5 mm
collimation and reconstruction at 5 mm intervals
I DIFFERENTIAL DIAGNOSIS
TB peritonitis
• Abnormal enhancement of peritoneum and mesentery
• Nodular or symmetric thickening of peritoneum and
mesentery
• Ascites, low attenuation mesenteric nodes
• Ileo-cecal mural thickening
• Splenomegaly
• TB mimics CT appearance of peritoneal metastases
Papillary serous carcinoma of peritoneum
• Peritoneal metastases (implants, ascites, omental
caking) without other source
• No ovarian or GI tract primary tumor
• Identical CT, US, MR findings to peritoneal metastases
from ovarian CA
Peritoneal mesothelioma
• 1/5 of all mesotheliomas are peritoneal
• Large solid omental and mesenteric masses often
infiltrating bowel and mesentery
Pseudomyxoma peritonei
• Diffuse accumulation of gelatinous masses within
peritoneum
• CECT: Scalloping of lateral contour of liver and spleen
• Etiology related to perforation of mucinous neoplasm
of appendix
• Treatment involves cytoreduction of peritoneal mass
and intraperitoneal chemotherapy
I PATHOLOGY
General Features
• General path comments
o Ascites
o Omental masses
o Mesenteric masses
o Nodular implants on peritoneal surface
• Genetics
o Colorectal and ovarian CA related to Lynch II
syndrome of hereditary nonpolypous colorectal
cancer
o GI and ovarian CA related to Lynch syndrome
o GI cancers related to polyposis syndrome
• Etiology
o Metastatic disease to peritoneal surfaces, omentum
and mesentery
 PERITONEAL METASTASES
1 o Peritoneal cavity spread of surface epithelium
tumors such as ovarian CA
o Causes symmetric thickening of peritoneum,
ileo-cecal thickening, ascites, necrotic low
64 o Ovarian and GI tract adenocarcinomas most attenuation nodes
common etiologies
o Less common causes Image Interpretation Pearls
• Metastatic lung, breast and renal CA • Omental caking
• Sarcoma, lymphoma less common causes • Peritoneal and mesenteric implants
• Epidemiology: Varies according to primary tumor • Ascites

Gross Pathologic & Surgical Features

• Infiltrating masses of peritoneal surfaces, omentum I SELECTED REFERENCES
and mesentery 1. Jayne DG: The molecular biology of peritoneal
• Omental caking carcinomatosis from gastrointestinal cancer. Ann Acad
• Ascites Med Singapore. 32(2):219-25, 2003
2. Park CM et al: Recurrent ovarian malignancy: patterns and
Microscopic Features spectrum of imaging findings. Abdom Imaging.
• Varies according to primary tumor 28(3):404-15, 2003
o Most commonly adenocarcinoma 3. Pavlidis N et al: Diagnostic and therapeutic management of
cancer of an unknown primary. Eur J Cancer.
Staging, Grading or Classification Criteria 39(14):1990-2005,2003
• Peritoneal metastases indicate Stage IV disease 4. Canis M et al: Risk of spread of ovarian cancer after
laparoscopic surgery. Curr Opin Obstet Gynecol.
13(1):9-14,2001
5. Tamsma JT et al: Pathogenesis of malignant ascites:
I CLINICAL ISSUES Starling's law of capillary hemodynamics revisited. Ann
Oncol. 12(10):1353-7,2001
Presentation 6. Sugarbaker PH: Review of a personal experience in the
• Most common signs/symptoms: Abdominal distension management of carcinomatosis and sarcomatosis. Jpn J
and pain, weight loss; ascites mayor may not be Clin Oncol. 31(12):573-83, 2001
present 7. Chorost MI et al: The management of the unknown
• Clinical profile primary. J Am Coli Surg. 193(6):666-77,2001
o No reliable lab data 8. Raptopoulos V et al: Peritoneal carcinomatosis. Eur Radiol
o Positive cytology on paracentesis 11(11):2195-2206,2001
9. Sugarbaker PH et al: Clinical pathway for the management
o Positive FNA of omental mass
of resectable gastric cancer with peritoneal seeding: best
Demographics palliation with a ray of hope for cure. Oncology.
58(2):96-107,2000
• Age 10. Seidman JD et al: Ovarian serous borderline tumors: a
o Adults generally> 40 yrs critical review of the literature with emphasis on
o Younger patients with hereditary syndromes (Le., prognostic indicators. Hum Pathol. 31(5):539-57, 2000
Lynch II) 11. Leblanc E et al: Surgical staging of early invasive epithelial
• Gender: More common in females than males, due to ovarian tumors. Semin Surg Oncol. 19(1):36-41,2000
ovarian CA 12. Canis M et al: Cancer and laparoscopy, experimental
studies: a review. Eur J Obstet Gynecol Reprod BioI.
Natural History & Prognosis 91(1):1-9,2000
• Progressive if untreated 13. Patel SV et al: Supradiaphragmatic manifestations of
papillary serous adenocarcinoma of the ovary. Clin Radiol.
o Bowel obstruction
54(11):748-54, 1999
• Pattern of peritoneal spread 14. Sugarbaker PH: Management of peritoneal-surface
o Direct seeding along mesentery and ligaments malignancy: the surgeon's role. Langenbecks Arch Surg.
o Intraperitoneal seeding along distribution of ascites 384(6):576-87, 1999
o Lymphatic 15. Ohtani T et al: Early intraperitoneal dissemination after
o Hematogenous radical resection of unsuspected gallbladder carcinoma
• Variable depending on primary tumor following laparoscopic cholecystectomy. Surg Laparosc
• Poor prognosis in general Endosc. 8(1):58-62, 1998
16. Sugarbaker PH: Intraperitoneal chemotherapy and
Treatment cytoreductive surgery for the prevention and treatment of
• Cytoreductive surgery for ovarian metastases peritoneal carcinomatosis and sarcomatosis. Semin Surg
Oncol 14(3):254-61, 1998
• All others combination of systemic and intraperitoneal
17. Fecteau AH et al: Peritoneal metastasis of intracranial
chemotherapy glioblastoma via a ventriculoperitoneal shunt preventing
organ retrieval: case report and review of the literature.
Clin Transplant. 12(4):348-50, 1998
I DIAGNOSTIC CHECKLIST 18. Conlon KC et al: Laparoscopy and laparoscopic ultrasound
in the staging of gastric cancer. Semin Oncol. 23(3):347-51,
Consider 1996
• TB peritonitis 19. Averbach AM et al: Strategies to decrease the incidence of
intra-abdominal recurrence in resectable gastric cancer. BrJ
Surg. 83(6):726-33, 1996
 PERITONEAL METASTASES
I IMAGE GALLERY 1
65
Typical
(Left) Axial CECT
demonstrates metastatic
omental nodules from
pancreatic carcinoma. Note
soft tissue nodule anterior to
hepatic flexure (arrow).
(Right) Axial CECT shows
metastatic omental nodules
from pancreatic carcinoma.
Note omental nodule
anterior to transverse colon
(arrow).

Typical
(Left) Axial US demonstrates
omental caking from ovarian
carcinoma. Note echogenic

-
mass within omentum
.•••• " ,0. (arrow), ascites and
peritoneal implants (open

.' . ••
arrow). (Right) Axial CECT
shows peritoneal spread of
lymphoma. Note diffuse

•.
infiltration of mesentery and

"• W
'l,
~
. colon by {lon-Hodgkin
lymphoma.

I
~

Typical
(Left) Axial CECT
demonstrates metastatic
melanoma to peritoneum.
Note echogenic implants on
gallbladder (arrow) and
peritoneum and massive
ascites (open arrow). (Right)
Axial CECT shows calcified
peritoneal metastases from
ovarian carcinoma. This
image demonstrates calcified
metastases (arrow) from
serous carcinoma of the
ovary adjacent to spleen .
 PSEUDOMYXOMA PERITONEI
1
66

Axial CECT of pseudomyxoma peritonei. Note Axial CECT of pseudomyxoma peritonei. Note extensive
scalloping of liver contour (arrows). low attenuation gelatinous masses involving lesser sac
surrounding stomach with more nodular 50ft tissue
infiltration laterally (arrow).

• Size: Cystic implants vary in size

ITERMINOLOGY • Morphology: Gelatinous low attenuation masses
Abbreviations and Synonyms Radiographic Findings
• Pseudomyxoma peritonei (PMP) • Radiography
Definitions o Evidence for ascites
• Diffuse intraperitoneal accumulation of gelatinous o Lateral displacement of liver margin
ascites 2° to rupture of well-differentiated mucinous o Lateral displacement of cecum
adeno CA of appendix o Pelvic "dog's ear"
o Rarely due to rupture of other mucinous tumors of o Displacement of bowel loops centrally within
colon, stomach, pancreas, gallbladder, fallopian tube abdomen
o Previously, ovary was thought to be primary site, but CT Findings
ovarian lesions now felt to be metastatic from
• CECT
appendiceal primary o Low attenuation « 20 HU) masses, mass effect on
liver & spleen ("scalloping"), centrally displaced
bowel loops on CECT
I IMAGING FINDINGS o Appendiceal primary tumor, calcified mets,
synchronous ovarian tumors in 44% of cases
General Features
• Best diagnostic clue: Scalloping of contour of liver and MR Findings
spleen by low attenuation masses • TlWI
• Location o Low signal intraperitoneal fluid collections
o Often diffuse throughout peritoneal cavity o Cystic implants on ligaments
o Along mesenteries and ligaments • T2WI
o Extensive peritoneal involvement common o High signal intraperitoneal fluid collections
o Subphrenic spaces o Cystic implants on ligaments
• Perihepatic and perisplenic locations most • Tl C+: Parenchymal scalloping of liver and spleen
common

DDx: Spectrum of low Attenuation Peritoneal Diseases

Carcinomatosis Leiomyosarcoma Bact. Peritonitis TB Peritonitis

 PSEUDOMYXOMA
Key Facts
Terminology
• Pseudomyxoma peritonei (PMP)
• Diffuse intraperitoneal accumulation of gelatinous
ascites 2° to rupture of well-differentiated mucinous
adeno CA of appendix
Imaging Findings
• Best diagnostic clue: Scalloping of contour of liver
and spleen by low attenuation masses
• Low attenuation « 20 HU) masses, mass effect on
liver & spleen ("scalloping"), centrally displaced
bowel loops on CECT
Top Differential Diagnoses
• Carcinomatosis without mucinous ascites
• Peritoneal sarcomatosis
• Bacterial peritonitis
Ultrasonographic Findings
• Real Time: Echogenic mucinous intraperitoneal
masses, multi septated peritoneal fluid on US
• Color Doppler: Cystic masses are avascular
Imaging Recommendations
• Best imaging tool: CECT
• Protocol advice
o Oral and IV contrast CECT
• 150 ml IV contrast injected at 2.5 ml/sec
• 5 mm collimation with reconstruction at 5 mm
intervals
I DIFFERENTIAL DIAGNOSIS
Carcinomatosis without mucinous ascites
• Peritoneal mets from ovarian and GI tract primary
tumors
• May occasionally cause scalloping of liver and spleen
• Omental caking
• Nodular soft tissue implants on peritoneum
• Variable amounts of ascites
Peritoneal sarcomatosis
• Solid and cystic peritoneal masses
• Hemorrhagic masses within peritoneum
• Most commonly leiomyosarcoma or GIST
Bacterial peritonitis
• Ascites and symmetrically smooth enhancing
peritoneum
• Septations within ascites
• Bowel wall thickening and enhancing serosa
• "Spontaneous" peritonitis occurs in cirrhotic patients
TB peritonitis
• Nodular thickening of peritoneum
• Mesenteric and omental nodules
• Low attenuation mesenteric nodes
• Cecal and terminal ileum (TI) mural thickening
PERITONEI
1
• TB peritonitis 67
Pathology
• Controversial etiology: Prior theory held that PMP 2°
to rupture of mucinous tumors of appendix or ovary
• More recent theory holds that PMP is always
appendiceal in origin, ovarian lesions are metastatic
Clinical Issues
• Slowly progressive, multiple bowel obstructions, 20%
5 year survival rate for very-well-differentiated adeno
CA vs. 80% for well-differentiated adeno CA
• Cytoreductive surgery with extensive debulking of all
intraperitoneal involvement (Sugarbaker procedure)
• Surgical treatment followed by infusion of heated
intraperitoneal chemotherapy
I PATHOLOGY
General Features
• General path comments: Diffuse intraperitoneal
mucinous ascites associated with mucinous tumors of
appendix or GI tract
• Genetics: No known genetic association
• Etiology
o Controversial etiology: Prior theory held that PMP
2° to rupture of mucinous tumors of appendix or
ovary
o More recent theory holds that PMP is always
appendiceal in origin, ovarian lesions are metastatic
Gross Pathologic & Surgical Features
• Gelatinous intraperitoneal masses
• 44% have synchronous tumors of ovary (likely
metastatic)
• Diffuse peritoneal involvement common
o Most common sites of involvement
• Right subphrenic space
• Liver surface
• Left subphrenic space
• Spleen surface
• Morison pouch
• Left paracolic gutter
• Pouch of Douglas or retrovesicle space
• Primary appendiceal tumor often impossible to
identify
Microscopic Features
• Spectrum
• Cytologically bland adenomucinosis
• Adenocarcinoma with mucinous back grouped
mixture of above findings
Staging, Grading or Classification Criteria
• Type I: Adenomucinous
o Cytologically bland adenomatous cells and mucin
o No frank adenocarcinoma
• Some pathologists feel that it is not truly benign,
but represents well-differentiated adenocarcinoma
o Believed to be 2° to rupture of adenoma or mucocele
 PSEUDOMYXOMA PERITONEI
1 o Better prognosis 6. Harshen R et al: Pseudomyxoma peritonei. Clin Oncol (R
Coli Radiol). 15(2):73-7,2003
• Type II: Mucinous adenocarcinoma
o Frank adenocarcinoma and mucin 7. Lo NS et al: Mucinous cystadenocarcinoma of the
68
appendix. The controversy persists: a review.
o Worse prognosis
Hepatogastroenterology. 50(50):432-7, 2003
• Type III: Intermediate 8. van Ruth S et al: Prognostic value of baseline and serial
o Mixture of types I and II carcinoembryonic antigen and carbohydrate antigen 19.9
o Combination of adenoma and adenocarcinoma cells measurements in patients with pseudomyxoma peritonei
with mucin treated with cytoreduction and hyperthermic
intraperitoneal chemotherapy. Ann Surg Oncol.
9(10):961-7, 2002
I CLINICAL ISSUES 9. Rose MG et al: Typical clinical and radiographic findings in
a patient with longstanding malignant pseudomyxoma
Presentation peritonei secondary to a mucinous adenocarcinoma. Clin
Colorectal Cancer. 2(1):59-60, 2002
• Most common signs/symptoms 10. Georgescu S et al: Mucinous digestive tumors. Case reports
o Abdominal pain and review of the literature. Rom] Gastroenterol.
o Abdominal distension 11(3):213-8, 2002
o Weight loss 11. Nakao A et al: Appendiceal mucocele of mucinous
o New onset of hernia cystadenocarcinoma with a cutaneous fistula. J Int Med
• Clinical profile: Normal lab values common Res. 30(4):452-6, 2002
12. Butterworth SA et al: Morbidity and mortality associated
Demographics with intraperitoneal chemotherapy for Pseudomyxoma
• Age: Adults, mean age S3 peritonei. Am J Surg. 183(5):529-32, 2002
13. Shappell HW et al: Diagnostic criteria and behavior of
• Gender: M < F
ovarian seromucinous (endocervical-type mucinous and
• Ethnicity: No known association mixed cell-type) tumors: atypical proliferative (borderline)
tumors, intraepithelial, micro invasive, and invasive
Natural History & Prognosis
carcinomas. Am] Surg Pathol. 26(12):1529-41, 2002
• Slowly progressive, multiple bowel obstructions, 20% 14. Sulkin TV et al: CT in pseudomyxoma peritonei: a review
S year survival rate for very-well-differentiated adeno of 17 cases. Clin Radiol. 57(7):608-13, 2002
CA vs. 80% for well-differentiated adeno CA 15. Butterworth SA et al: Morbidity and mortality associated
• Ultimately all patients die from this disease with intraperitoneal chemotherapy for Pseudomyxoma
peritonei. Am J Surg. 183(5):529-32, 2002
Treatment 16. Sherer DM et al: Pseudomyxoma peritonei: a review of
• Options, risks, complications current literature. Gynecol Obstet Invest. 51(2):73-80, 2001
o Cytoreductive surgery with extensive debulking of 17. Witkamp A] et al: Rationale and techniques of
all intraperitoneal involvement (Sugarbaker intra-operative hyperthermic intraperitoneal
chemotherapy. Cancer Treat Rev. 27(6):365-74, 2001
procedure)
18. Elias DM et al: Intraperitoneal chemohyperthermia:
o Surgical treatment followed by infusion of heated rationale, technique, indications, and results. Surg Oncol
intraperitoneal chemotherapy Clin N Am. 10(4):915-33, xi, 2001
19. Van H et al: Histopathologic analysis in 46 patients with
pseudomyxoma peritonei syndrome: failure versus success
I DIAGNOSTIC CHECKLIST with a second-look operation. Mod Pathol. 14(3):164-71,
2001
Consider 20. Esquivel] et al: Clinical presentation of the
Pseudomyxoma peritonei syndrome. Br] Surg.
• Ovarian carcinomatosis
87(10):1414-8,2000
Image Interpretation Pearls 21. Nawaz A et al: Pseudomyxoma peritonei manifesting as
• Scalloped contour of liver and spleen by low intestinal obstruction. South Med J. 93(9):891-3, 2000
22. Pestieau SR et al: Pleural extension of mucinous tumor in
attenuation masses
patients with pseudomyxoma peritonei syndrome. Ann
• Cystic implants on ligaments such as falciform and Surg Oncol. 7(3):199-203,2000
gastrohepatic ligament 23. Wirtzfeld DA et al: Disseminated peritoneal
adenomucinosis: a critical review. Ann Surg Oncol.
6(8):797-801, 1999
I SELECTED REFERENCES 24. Hosch WP et al: Therapy of pseudomyxoma peritonei of
appendiceal origin--surgical resection and intraperitoneal
1. van Ruth S et al: Pseudomyxoma peritonei: a review of 62 chemotherapy. Z Gastroenterol. 37(7):615-22, 1999
cases. Eur J Surg Oncol. 29(8):682-8, 2003 25. Hinson FL et al: Pseudomyxoma peritonei. BrJ Surg.
2. Moran BJ et al: The etiology, clinical presentation, and 85(10):1332-9, 1998
management of pseudomyxoma peritonei. Surg Oncol Clin
N Am. 12(3):585-603, 2003
3. Park CM et al: Recurrent ovarian malignancy: patterns and
spectrum of imaging findings. Abdom Imaging.
28(3):404-15,2003
4. Pickhardt PJ et al: Primary neoplasms of the appendix:
radiologic spectrum of disease with pathologic correlation.
Radiographies. 23(3):645-62, 2003
5. Hanbidge AE et al: US of the peritoneum. Radiographics.
23(3):663-84; discussion 684-5, 2003
 PSEUDOMYXOMA PERITONEI

I IMAGE GAllERY 1
69
Typical
(Left) Axial CECT shows
cystic peritoneal implants
from pseudomyxoma
peritonei. Note large cystic
mass indenting anterior
aspect of liver (arrow).
(Right) Axial CECT of cystic
peritoneal implants from
pseudomyxoma peritonei.
Note calcified perisplenic
implants (arrow).

Typical
(Left) Sagittal US findings in
pseudomyxoma peritonei
demonstrate echogenic
perihepatic implant (arrow).
(Right) Axial US of
pseudomyxoma peritonei
demonstrates cystic splenic
and perisplenic implants
(arrows).

Typical
(Left) Axial CECT
demonstrates ligamentous
involvement in
pseudomyxoma peritonei.
Note low density implants
involving gastrohepatic
(arrow) and falciform
ligaments (open arrow).
(Right) Axial CECT
demonstrates scalloping of
liver contour (arrow) and
falciform ligament implant
(open arrow) in
pseudomyxoma peritonei.
 SECTION 2: Esophagus

Introduction and Overview

Esophagus Anatomy and Imaging Issues 1-2-2

Congenital
Esophageal Webs 1-2-6

Infection
Candida Esophagitis 1-2-8
Viral Esophagitis 1-2-12

Inflammation
Reflux Esophagitis 1-2-16
Barrett Esophagus 1-2-20
Caustic Esophagitis 1-2-24
Drug-Induced Esophagitis 1-2-28
Radiation Esophagitis 1-2-30

Degenerative
Achalasia, Cricopharyngeal 1-2-32
Achalasia, Esophagus 1-2-34
Esophageal Motility Disturbances 1-2-38
Scleroderma, Esophagus 1-2-42
Esophageal Varices 1-2-46
Schatzki Ring 1-2-50
Hiatal Hernia 1-2-52

Esophageal Diverticula
Zenker Diverticulum 1-2-56
Traction Diverticulum 1-2-60
Pulsion Diverticulum 1-2-62
Intramural Pseudodiverticulosis 1-2-66

Trauma
Esophageal Foreign Body 1-2-68
Esophageal Perforation 1-2-70
Boerhaave Syndrome 1-2-74

Neoplasm, Benign
Intramural Benign Esophageal Tumors 1-2-78
Fibrovascular Polyp 1-2-80

Neoplasm, Malignant
Esophageal Carcinoma 1-2-82
Esophageal Metastases and Lymphoma 1-2-86
 ESOPHAGUS ANATOMY AND IMAGING ISSUES

2
2

Graphic shows nasopharynx (base of skull to palate); Lateral film from pharyngo-esophagram shows vallecula
oropharynx (palate to base of epiglottis); hypopharynx (arrow) and cricopharyngeal indentation (open arrow),
(epiglottis to cricopharyngeus muscle); esophagus usually located at the C5-C6 disk space.
(below cricopharyngeus).

o Essentially synonymous with "esophageal vestibule"

TERMINOLOGY or "phrenic ampulla"
Abbreviations and Synonyms o Occasionally recognized radiographically as a 2-4 cm
• Upper esophageal sphincter (UES) long luminal dilation between the esophageal "A"
o Formed primarily by cricopharyngeal muscle and "B"rings
• Lower esophageal sphincter (LES)
o Phrenic ampulla, esophageal vestibule
• "A"ring I ANATOMY-BASED IMAGING ISSUES I
o Sporadically imaged indentation of esophageal
Normal Measurements
lumen at the cephalic end of the lower esophageal
sphincter (the "tubulo-vestibular junction") • Swallowing
o Mastication and tongue motion propels food bolus
• "B"ring
into oropharynx
o Transverse mucosal fold marking the
o Sequential contraction of constrictor muscles of
esophagogastric junction and often corresponding
pharynx
to the mucosal junction between squamous +
o Upper esophageal sphincter (including
columnar epithelium
cricopharyngeus) opens to allow free passage of
• GE bolus
o Gastroesophageal
• Failure of UES to open completely
("cricopharyngeal achalasia" or "dyskinesia") is a
common cause of dysphagia and "food sticking in
IMAGING ANATOMY throat"
• Pharynx o Cricopharyngeal dyskinesia often accompanies other
o Nasopharynx malfunction of the pharynx and esophagus
• From base of skull to tip of soft palate • Primary peristalsis
o Oro (mesopharynx) o Initiated by swallowing
• From soft palate to hyoid bone o Normal is a continuous aboral esophageal
o Hypo (laryngopharynx) contraction wave, lasting 6-8 seconds, that propels
• From hyoid to bottom of cricopharyngeus muscle the bolus to the stomach
• Upper esophageal sphincter o Best evaluated by individual swallows of barium
o At pharyngoesophageal junction with patient in the prone oblique position
o Formed primarily by cricopharyngeus muscle • Secondary peristalsis
• Esophagus o Similar aboral contraction wave, but initiated by
o Muscular tube 20-24 cm in length esophageal distention or gastric reflux, rather than
o Lined by stratified squamous epithelium by swallowing
o Outer longitudinal and inner circular muscle fibers • Tertiary peristalsis
• Striated muscle in upper third o Nonperistaltic, disorganized contractions
• Smooth muscle in distal two thirds o May be intermittent, weak, asymptomatic
• Lower esophageal sphincter o May be persistent, repetitive, strong and produce
o Defined by manometric evidence of high resting dysphagia or pain
tone or pressure
 ESOPHAGUS ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
Benign masses • Malignant melanoma (primary)
• Leiomyoma • Lymphoma
• Squamous papilloma • Metastases
• Glycogenic acanthosis Mass lesions (common)
•
•
Adenoma
Fibrovascular polyps
• Carcinoma
• Leiomyoma (+ other stromal)
2
• Granular cell tumor
3
• (Duplication cyst) Mass lesions (uncommon)
• Papilloma
Malignant tumors
• Adenoma
• Squamous cell carcinoma • Metastases/lymphoma
• Adenocarcinoma
• Fibrovascular polyp
• Spindle cell carcinoma • Duplication cyst
• Small cell carcinoma • Inflammatory GE polyp
• Leiomyosarcoma
• Kaposi sarcoma

o Increase in frequency with aging
("presbyesophagus")
Key Concepts or Questions
• Symptoms of "food sticking in back of throat", choking
or concern for aspiration pneumonitis
o Modified barium swallow is technique of choice
o Videotaped monitoring while patient swallows
barium suspensions varying in consistency from
water to solid
• Symptoms of dysphagia, odynophagia,atypical chest
pain, "heartburn", concern for GE reflux
o Barium esophagram is techniques of choice
o Double contrast views in upright position following
ingestion of an effervescent agent and thick barium
o Single contrast views in upright and prone position
o Individual swallows to assess peristalsis
o Full distention views with continuos swallowing to
assess morphology (tumor, stricture, ulcer, etc.)
o Videotaped swallowing of food or radiopaque tablet
to assess stricture ("food sticking" symptoms)
• Symptoms of dyspepsia, early satiety, abdominal pain
o Upper gastrointestinal (UGI) series is technique of
choice
o Double and single contrast views of stomach and
duodenum before and after esophageal evaluation
Imaging Approaches
• Role of fluoroscopic imaging
o Complementary to manometry for most causes of
dysphagia
o Complementary to pH monitoring for
gastroesophageal reflux
o Complementary to endoscopy for esophagitis and
tumors
o Complementary to CT (+ MR) for primary
esophageal and mediastinal tumors
• CT (or MR)
o Complementary to endoscopic ultrasound in staging
esophageal cancer
• PET-CT
o Best study for staging esophageal cancer
• Endoscopic sonography
o Best study for depth of invasion by esophageal
carcinoma
Imaging Pitfalls
• Accuracy of fluoroscopic evaluation of the pharynx
and esophagus varies widely
• Depends mostly on the interest, skill, and
thoroughness of the radiologist performing the exam
I CLINICAL IMPLICATIONS
Clinical Importance
• Aspiration pneumonitis is an important cause of
morbidity + mortality in hospitalized and debilitated
patients
• "Modified barium swallow" is the most accurate means
of evaluating patient's ability to safely ingest foods of
varying consistency
• Opportunistic infections of the esophagus occur in
immune-suppressed patients, though less commonly
with newer pharmaceutical treatment protocols
• Gastroesophageal reflux is extremely common and has
contributed to a dramatic increase in the prevalence of
esophageal cancer
I CUSTOM DIFFERENTIAL DIAGNOSISI
Submucosal mass lesions
• Leiomyoma
o Smooth intramural mass
• Fibrovascular polyp
o Large pedunculated mass
o Arises in wall, but grows into lumen
o Arises in cervical esophagus
• Lipoma
o Fat density on CT
• Duplication or retention cyst
o Near water density, no enhancement
Extrinsic impressions on esophagus
• Normal
o Aortic arch
 ESOPHAGUS ANATOMY AND IMAGING ISSUES

2 "A ring
n

i /
4
/ mucosal
junction
vestibule (z-Iine)
(lower esoph. ~.
sphincter)
,,
'8' ring

Graphic shows normal esophageal landmarks and Barium esophagram shows "A" ring (arrow), "8" ring
anatomy (open arrow), esophageal vestibule (between "A & 8"
rings) and a small hiatal hernia.

o Left main bronchus o Leukoplakia

o Heart
• Abnormal
Esophageal strictures
o Enlarged left atrium or ventricle • Distal esophagus
o Tortuous aorta (distal esophagus) o Peptic strictures
o Congenital vascular (e.g., aberrant subclavian artery) o Barrett esophagus
o Thyroid masses o Carcinoma (usually adenocarcinoma)
o Mediastinal masses o Nasogastric intubation
o (Retraction to opposite side) o Crohn disease
o Alkaline reflux strictures
Esophageal ulceration • Midesophagus
• Common o Barrett esophagus
o Reflux esophagitis o Carcinoma (usually squamous cell carcinoma)
o Candida esophagitis o Radiation
o Herpes esophagitis o Caustic ingestion
o Drug-induced esophagitis o Oral medications
• Uncommon o Opportunistic infection (usually candidiasis)
o Radiation esophagitis o Epidermolysis bull osa dystrophica
o Caustic esophagitis o Benign mucous membrane pemphigoid
o Tuberculous esophagitis o Eosinophilic esophagitis
o Cytomegalovirus esophagitis o Chronic graft-versus-host disease
o HIV esophagitis o Metastatic tumor
o Crohn disease
o Nasogastric intubation
o Alkaline reflux esophagitis I SELECTED REFERENCES
o Behcet disease 1. Dibble C et al: Detection of reflux esophagitis on
o Epidermolysis bullosa dystrophica double-contrast esophagrams and endoscopy using the
o Benign mucous membrane pemphigoid histologic findings as the gold standard. Abdom Imaging.
29(4):421-5, 2004
Mucosal nodularity 2. Gupta S et al: Usefulness of barium studies for
• Common differentiating benign and malignant strictures of the
o Reflux esophagitis esophagus. AJRAm J Roentgenol. 180(3):737-44, 2003
o Candida esophagitis 3. Dahnert W: Radiology Review Manual (4th ed).
o Glycogenic acanthosis Philadelphia, Lippencott Williams + Wilkins. p615-721,
2000
• Uncommon
4. Levine MS: Gastroesophageal reflux disease. In Gore RM,
o Barrett esophagus Levine MS (eds) Textbook of Gastrointestinal Radiology
o Radiation esophagus (2nd ed.) Philadelphia, WB Saunders. 329-49, 2000
o Superficial spreading carcinoma 5. Levine MS et al: Update on esophageal radiology. AJRAm J
o Esophageal papillomatosis Roentgenol. 155(5):933-41, 1990
o Acanthosis nigricans 6. Ott DJ et al: Esophagogastric region and its rings. AJRAm J
o Cowden disease Roentgenol. 142(2):281-7, 1984
 ESOPHAGUS ANATOMY AND IMAGING ISSUES

I IMAGE GALLERY
Typical
(Left) Barium esophagram
shows normal extrinsic
indentations from aortic arch 2
and left main bronchus
(arrow). (Right) Esophagram
shows typical malignant 5
stricture (adenocarcinoma)
with irregular mucosal
surface, abrupt "shoulder" at
proximal end of stricture.

(Left) Esophagram shows

typical intramural or extrinsic
mass effect; leiomyoma.
(Right) Esophagram shows
typical benign distal stricture
with smooth taper and
nodular mucosal surface,
both due to reflux
esophagitis.

(Left) Esophagram shows

extensive ulcerations
throughout distal esophagus
due to reflux esophagitis.
(Right) Fused PET-CT(axial)
images show FOG-avid
lesion at gastroesophageal
junction (arrow) and
multiple lesions in the neck
(open arrows), representing
metastatic esophageal
adenocarcinoma.
 ESOPHAGEAL WEBS

2
6

Lateral view of barium esophagram shows thin shelf-like Frontal view of esophagram in a patient with
indentation of the esophagus (arrow). Plummer-Vinson syndrome who had glossitis, iron
deficiency anemia and dysphagia. Esophageal web
(arrow) and stricture (open arrow) are noted.

ITERMINOlOGY Radiographic Findings

• Videofluoroscopic rapid sequence esophagram
Definitions
o Frontal, lateral & oblique views
• Thin ring-like mucosal constriction projecting into • 1-2 mm wide, shelf-like filling defect along
lumen most frequently from anterior wall of proximal anterior wall of cervical esophagus
cervical esophagus • Circumferential, radiolucent ring
• Mild/moderate/severe luminal narrowing
o Partial obstruction: Suggested by
IIMAGING FINDINGS • Jet phenomenon: Barium spurting through ring
General Features • Dilatation of esophagus proximal to web
• Best diagnostic clue: A circumferential, radiolucent Imaging Recommendations
ring in proximal cervical esophagus on dynamic • Barium studies (including both frontal & lateral views)
esophagogram • Dynamic examination reveals increase in percentage
• Location of webs than does a spot film alone
o Cervical esophagus near cricopharyngeus (common) • Better demonstration achieved by large barium bolus
o Distal esophagus (uncommon) • Better visualized during maximal distention
• Morphology
o Thin band of mucosa with or without submucosa
o Usually composed of normal squamous epithelium I DIFFERENTIAL DIAGNOSIS
& lamina propria
• Other general features Esophageal stricture
o Congenital/normal variant/sequela of inflammation • Circumferential (> 2 mm) or vertical area of
o Vary from small shelf-like lesion to hemispheric bar complete/incomplete narrowing with length of> 2 cm
or circumferential ring
o Seen as isolated findings in 3-8% of patients Schatzki ring
undergoing upper gastrointestinal examinations • Lower (GE junction) esophageal mucosal or Bring
• Web-like constriction or shelf-like luminal projection

DDx: Short Segment Narrowing of Esophageal lumen

.\.
..
Peptic Stricture Crica. Achalasia Schatzki Ring Eso. Cancer
 ESOPHAGEAL WEBS

Key Facts
Terminology • Jet phenomenon: Barium spurting through ring
• Thin ring-like mucosal constriction projecting into Top Differential Diagnoses
lumen most frequently from anterior wall of
• Esophageal stricture
proximal cervical esophagus
• Schatzki ring
Imaging Findings
• Best diagnostic clue: A circumferential, radiolucent
• Cricopharyngeal achalasia 2
Diagnostic Checklist
ring in proximal cervical esophagus on dynamic 7
• Rapid sequence imaging during swallowing of a large
esophagogram
bolus of barium

• Fixed, anatomic, nondistensible, transverse ring o Spoon-shaped nails (koilonychia); ± thyroid disorder
• Symptomatic & acquired due to reflux esophagitis
• May be indistinguishable from distal esophageal web Demographics
• Age: Usually middle-age group people
Cricopharyngeal achalasia • Gender: More common in females than males
• Appears as a round, broad-based protrusion from
posterior pharyngeal wall at the level of Natural History & Prognosis
pharyngoesophageal segment (CS-6Ievel) • Complications
o Cervical esophageal web
• Cervical esophageal & hypopharyngeal carcinoma
I PATHOLOGY • Prognosis
o Usually good
General Features
Treatment
• Etiology
o Cervical esophageal webs may be • Balloon dilatation; bougienage during esophagoscopy
• Congenital
• Idiopathic (normal variant)
• Due to sequela of inflammation & scarring such as I DIAGNOSTIC CHECKLIST
epidermolysis bull osa (postcricoid) or benign Consider
mucous membrane pemphigoid
• Rapid sequence imaging during swallowing of a large
o Distal esophageal webs
bolus of barium
• Sequela of inflammation & scarring: GE reflux
• Epidemiology: One autopsy series showed 16% of
patients had incidental cervical esophageal webs
• Associated abnormalities
I SELECTED REFERENCES
o Cervical esophageal webs may be associated with 1. Taylor AJ et al: The esophageal jet phenomenon revisited.
• Plummer-Vinson (Paterson-Kelly) syndrome AJR 155: 289-90, 1990
2. Nosher JL et al: The clinical significance of cervical
• Glossitis, iron deficiency anemia, dysphagia (webs
esophageal and hypopharyngeal webs. Radiology 117:
+/- strictures) 45-7, 1975
• Rarely associated with GE reflux 3. Clements JL et al: Cervical esophageal webs: A
o Distal esophageal webs are associated with roentgen-anatomic correlation. AJR 121: 221-31, 1974
• Chronic gastroesophageal reflux
Gross Pathologic & Surgical Features
• Thin mucosal band projecting into esophageal lumen
I IMAGE GALLERY
Microscopic Features
• Normal variant: Squamous epithelium, lamina propria
• Inflammatory web: Inflammatory cells; scar tissue

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Mostly asymptomatic
o Dysphagia: With impaction of meat above web
o Odynophagia: Very rarely painful swallowing
• Plummer-Vinson syndrome findings (Left) A circumferential and relatively thick web (arrow) causes
o Esophageal webs, dysphagia significant luminal narrowing. (Right) Lateral esophagram shows tight
web-like narrowing at pharyngo-esophageal junction due to
o Iron deficiency anemia, stomatitis & glossitis
epidermolysis bullosa.
 CANDIDA ESOPHAGITIS

2
8

Illustration shows longitudinally-oriented mucosal Double-contrast barium esophagram shows

plaques characteristic of Candida esophagitis. longitudinally-oriented filling defects representing
Candida plaques (arrows).

• Cobblestone or snakeskin appearance with

ITERMINOlOGY confluent plaques
Abbreviations and Synonyms • Severe: "Shaggy" esophagus; usually AIDS patients
• Esophageal Candidiasis, moniliasis • Severe cases: Deep ulcers
• "Foamy" esophagus
Definitions • Discrete aphthoid ulcers that can mimic viral
• Infectious esophagitis caused by fungi of Candida esophagitis
species, usually Candida albicans • Nonspecific: Earliest finding is abnormal motility
(tertiary contractions, dilatation or atony)
• Nonspecific: Thickened longitudinal folds due to
I IMAGING FINDINGS submucosal edema
• Chronic: May lead to strictures (rare)
General Features • Rare: "Double-barreled esophagus" = intramural
• Best diagnostic clue: Mucosal plaques in tracts
immune-compromised patients o Single-contrast esophagram: Unreliable
• Location
o Predominantly mid or upper esophagus CT Findings
o Often spares distal esophagus • CECT
• Size: Plaques: Several mm in size; usually < 1 cm o Uniform circumferential wall thickening (> 5 mm)
• Morphology: Longitudinally oriented plaques with o Usually involves relatively long segment
intervening normal mucosa o "Target" sign
• Edematous wall shows hypodense submucosa
Radiographic Findings • Inflamed mucosal segments show avid
• Fluoroscopy enhancement
o Double-contrast esophagram: Several patterns
• Discrete plaques in mid to upper esophagus with Imaging Recommendations
longitudinal orientation and intervening normal • Best imaging tool: Double-contrast esophagram
mucosa
• Nodular or granular mucosa

DDx: Mimics of Candidiasis

Reflux Ulcer Herpes Ulcers Superficial Ca. Clyc. Acanthosis

 CANDIDA ESOPHAGITIS

Key Facts
Terminology • Viral esophagitis
• Infectious esophagitis caused by fungi of Candida • Superficial spreading carcinoma
species, usually Candida albicans • Drug-induced esophagitis

Imaging Findings Pathology

• Best diagnostic clue: Mucosal plaques in • Most common cause of infectious esophagitis 2
immune-compromised patients Clinical Issues
• Predominantly mid or upper esophagus 9
• Immunocompromised patients
• Often spares distal esophagus • Patients with physiologic or mechanical obstruction
• Discrete plaques in mid to upper esophagus with of esophagus and local stasis
longitudinal orientation and intervening normal
mucosa Diagnostic Checklist
• Cobblestone or snakeskin appearance with confluent • Odynophagia with plaques in immunocompromised
plaques patient should suggest Candidiasis
Top Differential Diagnoses
• Reflux esophagitis

• Protocol advice: Use rapid swallows instead of

effervescent granules to achieve gaseous distention Technical artifacts
without artifacts • Undissolved granules, air bubbles, debris
• Transient
Varices
I DIFFERENTIAL DIAGNOSIS • Serpiginous, longitudinal filling defects
Reflux esophagitis • Best seen on mucosal relief views
• Appearance varies depending on respiration,
• Usually extends proximally from gastroesophageal
intraluminal pressure
junction
• Nodular or granular mucosa but with poorly defined
borders
• Ulcers, strictures in distal esophagus I PATHOLOGY
• Often associated with hiatal hernia General Features
Viral esophagitis • General path comments
• Herpes esophagitis o Most common cause of infectious esophagitis
o Usually multiple small discrete ulcers o Candida species are normal oropharyngeal
o Advanced disease may have larger plaque-like commensals
lesions indistinguishable from Candida • Can have esophageal colonization without
• CMV esophagitis infection
o May have multiple small superficial ulcers • Mucosal biopsy, not brushings, necessary for
o Giant (> 1 cm), flat ulcers in mid-distal esophagus definitive diagnosis
more characteristic • Etiology
o AIDS patients o Probably caused by downward spread of Candida
albicans to esophagus
Superficial spreading carcinoma o Candida surface binding molecules ~ adhere to
• Confluent nodules without intervening areas of mucosa
normal mucosa • Protease secretion ~ tissue breakdown
• Adenosine secretion ~ blockage of neutrophil
Drug-induced esophagitis oxygen radical production ~ resistance to
• Tetracycline, KCI, quinidine, NSAIDs phagocytosis
• Focal contact esophagitis • Epidemiology: Occurs in 15-20% of patients with AIDS
o Single or multiple superficial ulcers • Associated abnormalities
o Usually in mid-esophagus near aortic arch or left o May coexist with herpes or CMV esophagitis
mainstem bronchus o Oral thrush
Glycogenic acanthosis • Oral Candidiasis + esophageal symptoms =}
71-100% positive predictive value for Candidal
• Asymptomatic; probably age-related benign
esophagitis
degenerative condition
• Absence of oral thrush does not rule out
• Mucosal plaques or nodules more rounded and less
esophageal Candidiasis
well defined than Candidiasis
• Oral thrush may be present with esophagitis of
viral etiology
 CANDIDA ESOPHAGITIS
o Esophageal intramural pseudodiverticulosis o Lung abscess
• Tiny outpouchings representing dilated excretory
ducts of esophageal submucosal glands Treatment
• Usually seen with strictures • Options, risks, complications
• Candidiasis likely represents superinfection o Oral antifungals: Ketoconazole or fluconazole
secondary to stasis in pseudodiverticula o Amphotericin-B for treatment failures or recurrences
o Chronic mucocutaneous candidiasis
2 • Defect in cell-mediated immunity
• Chronic fungal infection of skin, mucous I DIAGNOSTIC CHECKLIST
10 membranes and nails
Consider
• Esophageal strictures in this setting should suggest
Candidal involvement • Odynophagia with plaques in immunocompromised
patient should suggest Candidiasis
Gross Pathologic & Surgical Features
Image Interpretation Pearls
• Patchy, creamy white small « 1 cm) plaques on friable
erythematous mucosa • Plaques are raised mucosal lesions; if a central contrast
o Plaques represent necrotic epithelial debris and/or collection is present ~ lesion is an ulcer
Candida colonies
• Advanced cases
o Ulcerated, necrotic mucosa with pseudomembranes I SELECTED REFERENCES
o Shaggy or cobblestone mucosa 1. Luedtke P et al: Radiologic diagnosis of benign esophageal
o Narrowing strictures: a pattern approach. Radiographies. 23(4):
897-909,2003
Microscopic Features 2. Berkovich GY et al: CT findings in patients with
• Tissue invasion by fungal mycelia seen on endoscopic esophagitis. A]R Am] Roentgenol. 175(5):1431-4,2000
mucosal biopsy 3. Gore R et al: Textbook of Gastrointestinal Radiology. 2nd
ed. Philadelphia, W.B. Saunders, 1980-1992,2000
• Budding yeast cells, hyphae and pseudohyphae on
4. Wilcox CM et al: Esophageal infections: etiology,
silver stain, PASstain or Gram stain diagnosis, and management. Gastroenterologist. 2(3):
188-206, 1994
5. Laine L et al: Esophageal disease in human
ICLINICAllSSUES immunodeficiency virus infection. Arch Intern Med.
154(14): 1577-82, 1994
Presentation 6. Levine MS: Radiology of esophagitis: a pattern approach.
• Most common signs/symptoms Radiology. 179(1): 1-7, 1991
o Odynophagia 7. Levine MS et al: Update on esophageal radiology. A]R Am]
Roentgenol. 155(5): 933-41, 1990
o Other signs/symptoms
8. Levine MS: Radiology of the esophagus. Philadelphia, W.B.
• Dysphagia Saunders, 49-60, 1989
• GI bleeding 9. Raufman ]P: Odynophagiafdysphagia in AIDS.
o Oral thrush in 50-75%, but may be absent Gastroenterol Clin North Am. 17(3): 599-614, 1988
• Clinical profile 10. Mathieson R et al: Candida esophagitis. Dig Dis Sci. 28(4):
o Immunocompromised patients 365-70, 1983
• AIDS, transplant patients, diabetes, steroid use
o Patients with physiologic or mechanical obstruction
of esophagus and local stasis
• Scleroderma
• Achalasia
• Esophageal strictures
o Can occasionally occur in immunocompetent
patients
Demographics
• Age: All
• Gender: Both
Natural History & Prognosis
• Usually self-limited with rapid response to oral therapy
• Radiographic findings may lag behind clinical
response
• Chronic cases may develop strictures
• Severe cases: Hematogenously disseminated fungal
infection
• Unusual complications
o Esophageal perforation
o Aortoesophageal fistula
o Tracheoesophageal fistula
 CANDIDA ESOPHAGITIS
I IMAGE GALLERY

Typical
(Left) Barium esophagram in
a baby with AIDS shows a
"shaggy" esophagus with
areas of deep ulceration
2
(arrows). (Right)
Double-contrast esophagram 11
shows longitudinally oriented
plaques in mid-esophagus
(arrows) (Courtesy of M.
Nino-Murcia, MD).

Variant
(Left) Double-contrast
esophagram in a patient with
esophageal Candidiasis
shows small shallow
ulcerations indistinguishable
from those of herpes
esophagitis (arrows). (Right)
Sagittal fluoroscopy shows
Candida plaques (white
arrow) in a patient with
achalasia. Note dilated
esophagus tapering to a beak
(black arrow).

Typical
(Left) Anteroposterior
fluoroscopy shows Candida
plaques and associated
intramural
pseudodiverticulosis (arrow)
(Courtesy M. Federle, MD).
(Right) Anteroposterior
fluoroscopy shows extensive
intramural
pseudodiverticulosis in a
patient with Candida
esophagitis.
 VIRAL ESOPHAGITIS

2
12

Anteroposterior fluoroscopy shows punctate ulcers of Lateral fluoroscopy shows tiny ulcers surrounded by
Herpes esophagitis en face (arrows). radiolucent halo in patient with herpes esophagitis.
Ulcers are seen en face (open arrow) and in profile
(arrow).

o VZV: Esophagitis in immunocompromised children

ITERMINOLOGY with chicken pox or adults with herpes zoster
Abbreviations and Synonyms • Location: Upper to mid-esophagus
• Herpes simplex virus (HSV) esophagitis, herpetic • Size
esophagitis o Herpes: Small « 1 cm) ulcers
• Cytomegalovirus (CMV) esophagitis o CMVjHIV: Large ulcers
• Human immunodeficiency virus (HIV) esophagitis • Morphology
• Varicella-zoster virus (VZV) esophagitis o Herpes: Punctate or linear superficial ulcers with
• Ebstein-Barr virus (EBV)esophagitis surrounding radiolucent halo
• Human papilloma virus (HPV) esophagitis, squamous o CMV: Large flat ulcer with surrounding lucent
papillomatosis edema
• Alternatively, small superficial ulcers resembling
Definitions Herpetic lesions
• Inflammation of the esophagus of viral etiology o HIV: Large, flat ulcers
o EBV:Deep, linear ulcers
o HPV: Multiple papillary excrescences
I IMAGING FINDINGS Radiographic Findings
General Features • Double-contrast esophagram
• Best diagnostic clue o Herpes
o Herpes: Multiple small, discrete punched-out ulcers • Multiple small discrete punctate, linear, or stellate
on background of normal mucosa ulcers
o CMV and HIV: One or more large, flat ulcers • Surrounding radiolucent halo of edema
• Giant ulcers in HIV-positive patients most often • Background of normal mucosa
caused by HIV rather than CMV • Plaque formation not typical
o EBV:Esophagitis in patients with infectious • Advanced cases may have plaques, cobblestoning
mononucleosis and "shaggy" appearance identical to Candida
esophagitis
o CMV

DDx: Mimics of Viral Esophageal Ulcers

Candidiasis Drug Induced Lye Ingestion

 VIRAL ESOPHAGITIS

Key Facts
Imaging Findings • CMV may be transmitted via transplantation or
• Herpes: Multiple small, discrete punched-out ulcers transfusion
on background of normal mucosa • Up to 40% of AIDS patients may develop symptoms
• CMV and HIV: One or more large, flat ulcers of esophageal disease
• Best imaging tool: Double-contrast esophagram Clinical Issues
• Odynophagia is most common presenting symptom
2
Top Differential Diagnoses
• Reflux esophagitis • Herpes: Usually in immunocompromised patients but 13
• Drug-induced esophagitis can occur in otherwise healthy patients
• Crohn disease • CMV, HIV esophagitis: Only in
• Radiation esophagitis, caustic esophagitis immunocompromised patients
• Technical artifacts Diagnostic Checklist
Pathology • Small discrete shallow ulcers should suggest viral
• Viral esophagitis usually represents reactivation of esophagitis in immunocompromised patients with
latent virus rather than exogenous reinfection odynophagia

• One or more giant (> 1 cm) flat ulcers in • Solitary or multiple shallow ulcers
mid-distal esophagus
• Discrete superficial ulcers indistinguishable from Crohn disease
herpes • Discrete aphthous ulcers
o HIV • Should have Crohn elsewhere in GI tract
• Giant, flat ulcers identical to CMV Radiation esophagitis, caustic esophagitis
• May have small satellite ulcers
• Clinical history is diagnostic
• May cause fistulae
• Shallow or deep ulcers
o Squamous papillomatosis
• Usually have stricture
• Clusters of sessile polypoid lesions < 1 cm in size
Technical artifacts
CT Findings
• Gas bubbles, undissolved barium precipitates
• NECT: Thickened esophagus (> 5 mm)
• Should be transient
• CECT
o Thickened esophageal wall (> 5 mm) Differential diagnosis of giant ulcers
o "Target" sign • Nasogastric intubation
• Edematous submucosa • Sclerotherapy
• Avidly enhancing mucosa • Caustic ingestion
Imaging Recommendations • Mediastinal radiation
• Drugs
• Best imaging tool: Double-contrast esophagram
• Protocol advice
o Prone oblique double-contrast views may be helpful
o Use rapid swallows instead of effervescent granules
I PATHOLOGY
to achieve gaseous distention without artifacts General Features
• General path comments
o Viral esophagitis usually represents reactivation of
I DIFFERENTIAL DIAGNOSIS latent virus rather than exogenous reinfection
Infectious • HSV: Latency in nerve ganglion cells
• CMV: Latency in leukocytes
• Candida esophagitis may co-exist with viral
o HPV infection found in many cases of esophageal
esophagitis
papillomatosis
Reflux esophagitis • Etiology
• Usually in distal esophagus o Impaired immune surveillance, radiation and
• Often have distal stricture chemotherapy render the esophageal mucosa
• May see hiatal hernia vulnerable to infection
• Linear ulcers, fixed transverse folds ("stepladder o CMV may be transmitted via transplantation or
esophagus") transfusion
• Epidemiology
Drug-induced esophagitis o Up to 40% of AIDS patients may develop symptoms
• Focal contact esophagitis of esophageal disease
• Usually in mid-esophagus secondary to compression • Candida, CMV and HSV most common pathogens
by aortic arch or left mains tern bronchus
 VIRAL ESOPHAGITIS
• Associated abnormalities: Oral lesions in 27-37% of
HSV or HIV esophagitis Demographics
• Age: All
Gross Pathologic & Surgical Features • Gender: Both
• Herpes esophagitis
o Early disease: Esophageal blisters or vesicles rupture Natural History & Prognosis
~ discrete punched out ulcers • Herpes esophagitis
2 o Advanced disease: Fibrinous exudate covers ulcers to
form pseudomembranes
o Usually self-limited in otherwise normal patients
o Can be severe and prolonged in
14 • Mucosa in advanced disease indistinguishable immunocompromised patients
from Candida esophagitis • HIV esophagitis
• CMV: One or more shallow or deep ulcers o Usually self-limited in immunocompetent patients
• HIV: Acute ulceration o Advanced disease with deep ulcers may require
o Advanced disease: Deep penetrating ulcers treatment
• Varicella-zoster esophagitis
Microscopic Features o Usually resolves spontaneously
• Herpes o May cause necrotizing esophagitis in severely
o Cowdry type A intranuclear inclusions in epithelial immunocompromised patients
cells
o Ballooning of surface epithelial cells Treatment
o Multinucleated giant cells • Options, risks, complications
o No intracytoplasmic inclusions o Treatment
• Analgesics for odynophagia
• CMV
o "Owl's eye" inclusion bodies • Antiviral therapy for CMV, VZV and persistent
o Eosinophilic bodies within endothelial cells and herpes
fibroblasts • Deep ulcers of HIV may require steroid or
o Ground glass nuclei with viral particles thalidomide treatment
o Multinucleated giant cells o Immunocompromised patients with HSV
o Cytoplasmic inclusions esophagitis can get herpes pneumonitis or
disseminated infection
• HIV
o Viral particles morphologically similar to HIV on • Severe esophagitis may cause strictures or
electron microscopy hemorrhage

I CLINICAL ISSUES I DIAGNOSTIC CHECKLIST

Presentation Consider
• Most common signs/symptoms • Small discrete shallow ulcers should suggest viral
o Odynophagia is most common presenting symptom esophagitis in immunocompromised patients with
o Other signs/symptoms odynophagia
• Dysphagia, chest pain, upper GI bleeding Image Interpretation Pearls
o Immunocompetent patients may have an
• Careful analysis of double-contrast patterns is
influenza-type prodrome followed by acute onset
necessary to distinguish plaques from ulcers
odynophagia
o HIV esophagitis may develop during acute HIV
infection as well as after AIDS is established
• May be associated with maculopapular rash
I SELECTED REFERENCES
o CMV may present with nausea and vomiting 1. Mosca S et al: Squamous papilloma of the esophagus:
secondary to concomitant infection in stomach or long-term follow up.] Gastroenterol Hepatol. 16(8):857-61,
2001
intestine
2. Gore R et al: Textbook of Gastrointestinal Radiology. 2nd
• Clinical profile ed. Philadelphia, W.B. Saunders, 1980-1992,2000
o Risk factors for viral esophagitis 3. Berkovich GY et al: CT findings in patients with
• Immunodeficiency esophagitis. A]R Am] Roentgenol. 175(5):1431-4,2000
• Chemotherapy 4. Fenoglio-Preiser CM. Gastrointestinal pathology: An atlas
• Organ transplantation and text. 2nd ed. Philadelphia, Lippincott-Raven. 62-65,
• Diabetes 1999
5. Laine L et al: Esophageal disease in human
• Alcoholism
immunodeficiency virus infection. Arch Intern Med.
• Advanced age 154(14): 1577-82, 1994
o Herpes: Usually in immunocompromised patients 6. Wilcox CM et al: Esophageal infections: etiology,
but can occur in otherwise healthy patients diagnosis, and management. Gastroenterologist. 2(3):
• Immunocompetent patients usually have history 188-206, 1994
of exposure to sexual partners with herpetic ulcers 7. Levine MS: Radiology of esophagitis: a pattern approach.
o CMV, HIV esophagitis: Only in Radiology. 179(1): 1-7, 1991
immunocompromised patients
 VIRAL ESOPHAGITIS
I IMAGE GALLERY
Typical
(Left) Lateral fluoroscopy
shows clusters of nodules
typical of squamous 2
papillomatosis. (Right) Axial
CECT shows thickened
irregular esophageal wall in 15
patient with endoscopically
proven CMVesophagitis.

Variant
(Left) Lateral fluoroscopy
shows both ulcers and
plaques in patient with
herpes esophagitis. (Right)
Lateral fluoroscopy shows
elongated plaques (arrows)
in patient with herpes
esophagitis. Findings are
indistinguishable from
Candida esophagitis.

(Left) Lateral fluoroscopy

shows tiny ulcers (arrows)
on background of normal
mucosa, typical of herpes
esophagitis. (Right) Lateral
fluoroscopy shows small
ulcers of herpes esophagitis
in profile (open arrows) and
en face (arrows).
 REFLUX ESOPHAGITIS

2
16

Double-contrast esophagram shows small hiatal hernia Double-contrast esophagram shows small hiatal hernia,
(arrow) with shallow round + linear esophageal ulcers. distal stricture, and nodular; granular esophageal
mucosa that simulates Barrett esophagus.

• May represent an effect (complication) rather than

ITERMINOLOGY a cause of reflux esophagitis
Definitions Radiographic Findings
• Inflammation of esophageal mucosa due to • Double-contrast esophagraphy (en face/profile views)
gastroesophageal reflux o Acute reflux esophagitis
• May show increased frequency of non peristaltic
waves
IIMAGING FINDINGS • Decreased or absent primary peristalsis
General Features • Mucosal nodularity: Fine nodular, granular or
discrete plaque-like defects (pseudomembranes)
• Best diagnostic clue: Irregular ulcerated mucosa of
• Ulcers: Multiple tiny collections of barium with
distal esophagus on barium esophagram
surrounding mucosal edematous mounds,
• Location: Usually involves distal third or half of
radiating folds & puckering
thoracic esophagus
• Thickened vertical or transverse folds (more than
• Morphology
3 mm): Best seen in mucosal relief views of
o Varies from mild to severe
collapsed esophagus
o Various morphological changes of mucosa
• Inflammatory esophagogastric polyps: Smooth,
• Nodularity, ulceration, thickened folds
ovoid elevations
• Inflammatory polyps, scarring & strictures
o Chronic or advanced reflux esophagitis
• Other general features
• Decreased distal esophageal distensibility with
o Usually a complication of gastroesophageal reflux
irregular, serrated contour (due to
disease (GERD)
ulceration/ edema/ spasm)
o Based on onset, classified clinically & radiologically
• Sacculations & pseudodiverticula may be seen
• Acute reflux esophagitis
• Peptic stricture (1-4 cm length/O.2-2 cm width):
• Chronic reflux esophagitis
Concentric smooth tapered narrowing of distal
o Severity of reflux esophagitis
esophagus with proximal dilatation
• Depends on intrinsic resistance of mucosa
• "Stepladder" appearance: Transverse folds due to
o Sliding hiatal hernia
vertical scarring

DDx: Feline Esophagus

Feline Esoph. Candida Crohn Esoph.

 REFLUX ESOPHAGITIS
Key Facts
Imaging Findings
• Best diagnostic clue: Irregular ulcerated mucosa of
distal esophagus on barium esophagram
• Mucosal nodularity: Fine nodular, granular or discrete
plaque-like defects (pseudomembranes)
• Ulcers: Multiple tiny collections of barium with
surrounding mucosal edematous mounds, radiating
folds & puckering
• Thickened vertical or transverse folds (more than 3
mm): Best seen in mucosal relief views of collapsed
esophagus
• Peptic stricture (1-4 cm length/O.2-2 cm width):
Concentric smooth tapered narrowing of distal
esophagus with proximal dilatation
• Hiatal hernia: Seen in more than 95% of peptic
stricture patients
• Hiatal hernia: Seen in more than 95% of peptic
stricture patients
CT Findings
• NECT: Diffuse or focal circumferential esophageal wall
thickening (? 5 mm)
• CECT: "Target" sign: Combination of mucosal
enhancement & surrounding hypodense submucosa
Imaging Recommendations
• Videofluoroscopic double-contrast esophagram
o En face & profile views
• Biphasic examination with upright double-contrast &
prone single-contrast views of esophagus
o Best radiologic technique for evaluation of patients
with suspected reflux disease
I DIFFERENTIAL DIAGNOSIS
Fungal & viral esophagitis
• Examples
o Fungal: Candida albicans
o Viral: Herpes, CMV & HIV
• Acute or early Candida esophagitis
o "Foamy" esophagus: Multiple tiny, round lucencies
on top of barium
o Discrete linear, irregular plaque-like filling defects
o Multiple fine nodular or granular appearance
o "Cobblestone" or "snakeskin" appearance
• Severe or advanced Candidiasis
o Grossly irregular or "shaggy" esophagus
o Long tapered distal stricture due to scarring
• Indistinguishable from reflux esophagitis stricture
o Diagnosis: Endoscopy & biopsy
• Budding yeast cells, hyphae, pseudohyphae
• Herpes esophagitis
o Multiple small discrete superficial punctate, linear
ulcers (often diamond shaped)
o Extensive ulceration or plaque formation
• Cytomegalovirus (CMV) & HIV esophagitis
o Location: Usually in mid-esophagus
o CMV
Top Differential Diagnoses
• Fungal & viral esophagitis
• "Feline esophagus"
• Caustic esophagitis
•
•
Scleroderma
Crohn disease 2
Pathology 17
• Gastroesophageal reflux disease (GERD)
• Lower esophageal sphincter (LES):Incompetent plus
decreased tone leads to increased reflux
Diagnostic Checklist
• Differentiate from other types of esophagitis
• Smooth, tapered, concentric narrowing in distal
esophagus above a sliding hiatal hernia is diagnostic
of a peptic stricture due to reflux esophagitis
• Multiple discrete, superficial ulcers
• One or more giant, flat ovoid/elongated ulcers
more than 1 cm in size
o HIV: One or more giant, flat ovoid/diamond-shaped
ulcers more than 1 cm in size
o Impossible to differentiate CMV ulcers from HIV
o Diagnosis: Endoscopic biopsy
"Feline esophagus"
• Fine, barium-etched, thin transverse striations
o Striations: Due to muscularis mucosae contractions
• Seen crossing entire esophageal luminal diameter
• This pattern is termed as "feline esophagus" or
esophageal shiver; is transient
• Often associated with GE reflux
Caustic esophagitis
• Caustic agents: Liquid lye & strong acids
• Location: Usually involves entire thoracic esophagus
• CT imaging findings
o Diffuse circumferential esophageal wall thickening
(? 5 mm)
o Acute phase: May show "target sign"
• Mucosal enhancement & hypodense submucosa
o Chronic phase
• Luminal irregularity & narrowing
• Esophageal perforation: Pneumomediastinum,
pleural effusion
• Esophagography (water soluble contrast study)
o Acute phase
• Atonic dilated esophagus
• Multiple shallow, irregular ulcers
o Chronic phase
• Segmental strictures, sacculations,
pseudodiverticula
• Thread-like/filiform appearance of mid-esophagus
• Indistinguishable from reflux esophagitis especially
when lower esophageal segment is only involved
Scleroderma
• Collagen vascular disease, more common in women
• Pathogenesis
o Smooth muscle atrophy/fibrosis; incompetent LES
 REFLUX ESOPHAGITIS
o Increased risk of stasis, reflux esophagitis, Candida
esophagitis & Barrett esophagus
• Barium study findings
o Aperistalsis, dilated esophagus, patulous GE junction
o GE reflux, mucosal findings of reflux esophagitis
o Later leads to stricture + proximal dilatation
• Diagnosis: Anti-Scl 70 & anticentromere antibodies &
2 endoscopic biopsy
Crohn disease
18
• Least common site of involvement of GI tract
• Always have associated disease in small bowel or colon
• Radiographic findings
o Discrete, widely separated aphthous ulcers
o Thickened folds, pseudomembranes
o Longitudinal & transverse intramural tracks
• Due to transmural involvement
• Appearance: "Double-barreled" esophagus
o Distal esophageal involvement may be
indistinguishable from reflux esophagitis
I PATHOLOGY
General Features
• General path comments
o Sequelae of reflux esophagitis
• Hyperemia, epithelial thinning, WBC infiltrate
• Necrosis & finally ulceration
• Etiology
o Gastroesophageal reflux disease (GERD)
• Irritants: Drugs, alcohol, smoking, corrosive
chemicals, radiation
o Pathogenesis of reflux esophagitis
• Lower esophageal sphincter (LES): Incompetent
plus decreased tone leads to increased reflux
• Hydrochloric acid (HCL) & pepsin: Increased
synergistic effect producing more injury than HCL
• Epidemiology
o GERD: Most common inflammatory disease
o 15-20% of Americans have heartburn due to reflux
Gross Pathologic & Surgical Features
• Hyperemia, inflammation
• Superficial necrosis, white plaques, strictures
Microscopic Features
• Basal cell hyperplasia, edema
• Thinning of stratified squamous epithelium
• Submucosal polymorphonuclear leukocyte infiltrate
• Superficial necrosis & ulceration
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Heartburn, regurgitation, angina-like pain
o Dysphagia, odynophagia
• Lab-data
o Manometric/ambulatory pH-monitoring techniques
• Reveal increase acid production
• Assess gastroesophageal reflux
• Diagnosis
o Endoscopy, biopsy & histological studies
Demographics
• Age: Usually middle & adult age group
• Gender: M = F
Natural History & Prognosis
• Complications
o Ulceration, bleeding, stenosis
o Sliding hiatal hernia: Due to
• Severe inflammation & scarring
• Longitudinal esophageal shortening
• Disruption of ligaments surrounding GE junction
• Pulls gastric fundus into thorax
o Squamous or adeno carcinoma (Barrett mucosa)
• Prognosis
o Acute reflux esophagitis: Good
o Chronic reflux esophagitis: Poor
Treatment
• H2 receptor antagonists, proton-pump inhibitors
• Antacids, cessation of irritants
• Metaclopramide: Increases LES tone
• Surgery (fundoplication)
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other types of esophagitis
Image Interpretation Pearls
• Smooth, tapered, concentric narrowing in distal
esophagus above a sliding hiatal hernia is diagnostic of
a peptic stricture due to reflux esophagitis
I SELECTED REFERENCES
1. Dibble C et al: Detection of reflux esophagitis on
double-contrast esophagrams and endoscopy using the
histologic findings as the gold standard. Abdom Imaging.
29:421-5,2004
2. Hu C et al: Solitary ulcers in reflux esophagitis:
radiographic findings. Abdom Imaging. 22(1):5-7, 1997
3. Levine MS: Reflux esophagitis and Barrett's esophagus.
Semin Roentgenol. 29(4):332-40, 1994
4. Thompson JK et al: Detection of gastroesophageal reflux:
Value of barium studies compared with 24-hr pH
monitoring. AJR. 162: 621-6, 1994
5. Levine MS: Radiology of esophagitis: a pattern approach.
Radiology. 179(1):1-7, 1991
6. Levine MS et al: Update on esophageal radiology. AJRAm J
Roentgenol. 155(5):933-41, 1990
7. Mann NS et al: Barrett's esophagus in patients with
symptomatic reflux esophagitis. Am J Gastroenterol.
84(12):1494-6, 1989
8. Levine MS et al: Pseudomembranes in reflux esophagitis.
Radiology. 159(1):43-5, 1986
9. Levine MS et al: Fixed transverse folds in the esophagus: a
sign of reflux esophagitis. AJRAm J Roentgenol.
143(2):275-8, 1984
10. Graziani L et al: Reflux esophagitis: radiologic-endoscopic
correlation in 39 symptomatic cases. Gastrointest Radiol.
8(1):1-6, 1983
11. Creteur V et al: The role of single and double-contrast
radiography in the diagnosis of reflux esophagitis.
Radiology. 147(1):71-5, 1983
 REFLUX ESOPHAGITIS
I IMAGE GALLERY

(Left) Prone esophagram

shows tight stricture (arrow)
just above the GEjunction, 2
proximal dilatation. (Right)
Endoscopic image of distal
esophagus shows 19
pseudo-membranes, mucosal
ulceration (arrow),
nodularity, and stricture.

(Left) Esophagram shows

sliding hiatal hernia and
multiple esophageal ulcers
(arrows). (Right)
Esophagram shows nodular
distal esophageal mucosa.

Typical
(Left) Esophagram shows
thickened, irregular
longitudinal folds and a large
ulcer (open arrow). (Right)
Esophagram shows hiatal
hernia, stricture at GE
junction, and obstructed
passage of food (walnuts)
within lumen (arrows).
 BARRETT ESOPHAGUS

2
20

Graphic shows sliding hiatal hernia, distal esophageal Esophagram shows hiatal hernia, strictures at GE
stricture and nodular mucosal surface. Note discrete junction (arrow) and higher. Also note discrete ulcer
ulcer (arrow)and an adenocarcinoma (open arrow). (open arrow).

• Risk of adenocarcinoma is 30-40 times more than

ITERMINOLOGY
Definitions
• Metaplasia of distal esophageal squamous epithelium
to a columnar epithelium
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Mid-esophageal stricture with
hiatal hernia & reflux is pathognomonic
• Location: Middle & distal third of esophagus
• Size: Length of esophageal stricture varies
• Morphology
o Velvety, pinkish-red columnar mucosa which
extends 3 cm or more above lower esophageal
sphincter (LES)
• Often seen as islands or tongues
o Usually associated with hiatal hernia & reflux
• Other general features
o Barrett esophagus is an acquired condition
o Most common etiology: Chronic reflux esophagitis
o Most common in adults but occasionally seen in
children & infants
o Premalignant condition associated with increased
risk of esophageal adenocarcinoma
in general population
• 90-100% adenocarcinomas found to arise from
Barrett mucosa
• Barrett esophagus is classified into two types based on
endoscopy & histopathologic findings
o Long-segment: Columnar epithelium more than 3
cm above gastroesophageal (GE) junction
• Due to more severe reflux disease
• Usually seen in mid-esophagus
• Frequency less than short-segment
• Hiatal hernia greater than 2 cm in 96% of patients
• Increase risk of cancer than short-segment
• Prevalence: Approximately 1% at endoscopy &
10% in symptomatic
o Short-segment: Columnar epithelium 3 cm or less,
above GE junction
• Due to less severe reflux disease
• Usually seen in distal esophagus
• Frequency greater than long-segment
• Hiatal hernia greater than 2 cm in 72% of patients
• Less risk of cancer than long-segment
• Prevalence: 2-12% at routine endoscopy
Radiographic Findings
• Double-contrast esophagography
o Long-segment Barrett esophagus (mid-esophagus)
• Early: Focal indentation/small concavity of wall
• Severe: Ring-like constriction/tapered narrowing

DDx: Esophageal Stricture +/- Ulceration

Reflux Stricture Esoph. Cancer Drug-Ind. Esoph. Scleroderma

 BARRETT ESOPHAGUS
Key Facts
Terminology
• Metaplasia of distal esophageal squamous epithelium
to a columnar epithelium
Imaging Findings
• Best diagnostic clue: Mid-esophageal stricture with
hiatal hernia & reflux is pathognomonic
• Long-segment Barrett esophagus (mid-esophagus)
• Mid-esophageal Barrett ulcer (deep ulcer crater)
• Short-segment Barrett esophagus (distal esophagus)
• Granular or nodular mucosa, tiny ulcers
• Short tapered stricture: Symmetrical/asymmetrical
Top Differential Diagnoses
• Esophagitis
• Esophageal carcinoma
• Drug-induced injury
• Mid-esophageal Barrett ulcer (deep ulcer crater)
• Mid-esophageal stricture: More common & early
• Sliding hiatal hernia
• Gastroesophageal (GE) reflux
o A mid-esophageal stricture/ulcer, hiatal hernia & GE
reflux: Strongly suggests a Barrett esophagus
o Short-segment Barrett esophagus (distal esophagus)
• Granular or nodular mucosa, tiny ulcers
• Vertical or transverse thickened, irregular folds
(peptic scarring)
• Short tapered stricture: Symmetrical/asymmetrical
• Sliding hiatal hernia
• Gastroesophageal reflux
• May show intramural pseudodiverticula
o Reticular mucosal pattern: Specific sign (distal to
stricture)
• Innumerable, tiny, barium-filled mucosal
grooves/ crevices
• Usually seen adjacent to distal aspect of
esophageal stricture
• This finding is seen in only 5-30% of patients
o Based on double-contrast esophagography &
endoscopy
• High risk patients: Mid-esophageal stricture, ulcer,
reticular mucosa
• Moderate risk: Distal peptic stricture & reflux
esophagitis
• Low risk: If none of above findings are present
Imaging Recommendations
• Videofluoroscopic double-contrast esophagography
• En face, profile, oblique & prone views
I DIFFERENTIAL DIAGNOSIS
Esophagitis
• Reflux esophagitis
o Mucosa: Fine nodular/granular or plaque-like
o Ulcers: Multiple tiny, radiating folds & puckering
• Barrett ulcers: Usually deep ulcer craters within
columnar mucosa at a greater distance from GE
junction
• Scleroderma
Pathology
• Recurrent GE reflux, inflammation, ulceration
• Re-epithelialization by pluripotent stem cells
•
•
Differentiate into gastric or intestinal epithelium
Increased risk: Adenocarcinoma in Barrett mucosa
2
• Velvety, pinkish-red mucosa (3 cm/more above LES) 21
• Long to mid-esophageal/short to distal esophageal
stricture
Diagnostic Checklist
• Rule out other causes of esophageal stricture ±
ulceration
• Long to mid-esophageal or short distal-esophageal
stricture/ulcer associated with hiatal hernia/GE reflux
o Folds: Thickened vertical or transverse (> 3 mm)
o Peptic stricture: Smooth tapered narrowing of short
distal segment
• Barrett stricture: Classic mid-esophageal; when
distally located indistinguishable from peptic type
o Diagnosis: Endoscopic biopsy & histology
• Infectious esophagitis
o Fungal esophagitis: Candida albicans
• "Foamy" esophagus: Multiple tiny round lucencies
• "Cobblestone" or "snakeskin" appearance
• Grossly irregular or "shaggy" esophagus
o Viral esophagitis: Herpes, CMV, HIV
• Multiple small discrete superficial punctate ulcers
• One or more giant, flat ovoid or elongated ulcers
o Diagnosis: Endoscopic biopsy & histology
• Radiation esophagitis
o Granular mucosa, decreased distensibility of
irradiated segment
o Mid-esophageal stricture indistinguishable from
Barrett stricture
o History of lung cancer with mediastinal irradiation
• Caustic esophagitis
o Location: Any esophageal segment is involved
• Usually middle & lower thirds (more common)
o Atonic dilated esophagus; multiple shallow irregular
ulcers
o Acute: Diffuse narrowing, grossly irregular contour,
ulceration
o Chronic: Diffuse thread-like or filiform appearance
o One/more segmental strictures in cervical/thoracic
esophagus highly suggestive of caustic ingestion
o Diagnosis: History & endoscopic biopsy
Esophageal carcinoma
• Asymmetric contour with abrupt proximal borders of
narrowed distal segment (rat-tail appearance)
• Mid-esophageal stricture may mimic Barrett stricture
• Diagnosis: Endoscopic biopsy & history
Drug-induced injury
• Examples: Antibiotics (doxycycline & tetracycline)
o Ulcers: Solitary, several discrete or multiple tiny
o Clustered circumferentially in mid-esophagus
 BARRETT ESOPHAGUS
• Examples: KCL, quinidine, NSAlDs
o Produce more severe esophagitis than antibiotics
o KCL, quinidine: Large areas of ulceration & edema
• Ulcer simulates ulcerated carcinoma
o NSAlDs: Giant, flat, diamond-shaped ulcers (4-6 cm)
• Diagnosis: History of drug ingestion, biopsy
2 Scleroderma
• Collagen vascular disease; more common in women
22 • Pathogenesis
o Smooth muscle atrophy/fibrosis; incompetent LES
o Reflux esophagitis & high risk of Barrett esophagus
• Fluoroscopic barium study findings
o Aperistalsis, dilated esophagus, patulous GE junction
o GE reflux, peptic stricture in distal one third
• Diagnosis: Endoscopic biopsy
I PATHOLOGY
General Features
• Genetics: Genomic instability in patients of Barrett
esophagus may increase risk of adenocarcinoma
• Etiology
o Chronic GE reflux & reflux esophagitis
• Due to acid & pepsin reflux
o Patients with total or partial gastrectomy
• Due to bile reflux esophagitis
o Contributing factors
• Decreased LES pressure, transient LES relaxation
• Hiatal hernia, decreased acid sensitivity
• Alcohol, tobacco, chemotherapy, scleroderma
o Pathogenesis
• Recurrent GE reflux, inflammation, ulceration
• Re-epithelialization by pluripotent stem cells
• Differentiate into gastric or intestinal epithelium
• Epidemiology
o Incidence: 0.3-4% in general population
o Prevalence: 8-20% (overall 10%) in patients with
reflux esophagitis
o Increased risk: Adenocarcinoma in Barrett mucosa
• Prevalence: 2.4-46% (overall about 15%)
• Incidence: 0.5-1.5% per year (in long-segment)
• Associated abnormalities: GE reflux & hiatal hernia
Gross Pathologic & Surgical Features
• Velvety, pinkish-red mucosa (3 cm/more above LES)
• Long to mid-esophageal/short to distal esophageal
stricture
• Hiatal hernia
Microscopic Features
• Proximally: Specialized columnar epithelium
o Villous architecture with goblet cells
• Distal to above: Junctional-type epithelium
o Cardiac mucous glands
• More distally: Fundic-type epithelium
o Parietal & chief cells
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Reflux symptoms
• Heartburn/regurgitation/ angina-like
pain/dysphagia
o Long-segment Barrett esophagus patients
• More severe reflux disease
o Short-segment Barrett esophagus patients
• Less severe reflux disease
o 20-40% of patients are asymptomatic
• Lab-data
o Manometric or ambulatory pH-monitoring
• Assess gastroesophageal reflux
• Diagnosis
o Endoscopy, biopsy & histopathology
Demographics
• Age: Mean: 55-65 years; prevalence increases with age
• Gender: M:F = 10:1
• Ethnicity: Caucasians more than African-Americans
(10:1)
Natural History & Prognosis
• Complications
o Ulceration, stricture, perforation, adenocarcinoma
• Prognosis
o Usually good after early detection & treatment
o Poor due to perforation or malignant transformation
Treatment
• Cessation of irritants (e.g., smoking & alcohol)
• H2 receptor antagonists; proton pump inhibitors
• Increase LES pressure: Metoclopramide
• Surgical resection in high grade dysplasia
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of esophageal stricture ±
ulceration
Image Interpretation Pearls
• Long to mid-esophageal or short distal-esophageal
stricture/ulcer associated with hiatal hernia/GE reflux
I SELECTED REFERENCES
1. Luedtke P et al: Radiologic diagnosis of benign esophageal
strictures: a pattern approach. Radiographies.
23(4):897-909,2003
Yamamoto AJ et al: Short-segment Barrett's esophagus:
2.
findings on double-contrast esophagography in 20
patients. AJRAm J Roentgenol. 176(5):1173-8, 2001
3. Rosch T: Gastroesophageal reflux disease and Barrett's
esophagus. Endoscopy. 32(11):826-35, 2000
4. Chen MY et al: Barrett esophagus and adenocarcinoma.
Radiol Clin North Am. 32(6):1167-81, 1994
5. Levine MS: Reflux esophagitis and Barrett's esophagus.
Semin Roentgenol. 29(4):332-40, 1994
6. Glick SN: Barium studies in patients with Barrett's
esophagus: importance of focal areas of esophageal
deformity. AJRAm J Roentgenol. 163(1):65-7, 1994
7. Gilchrist AM et al: Barrett's esophagus: Diagnosis by
double-contrast esophagography. AJR 150 (I): 97-102, 1988
8. Levine MS et al: Re: Reticular pattern as a radiologic sign of
the Barrett esophagus. Radiology. 156(3):843-4, 1985
 BARRETT ESOPHAGUS
I IMAGE GALLERY

(Left) Esophagram shows

subtle raised plaque (arrow)
in distal esophagus.
Adenocarcinoma on Barrett
2
mucosa. (Right) Esophagram
shows diffuse nodularity of 23
distal esophagus.

(Left) Two views from

esophagram show
mid-esophageal stricture and
ulcer in patient with small
hernia and reflux. (Right)
Endoscopic image shows
large ulcer (arrow), velvet
texture of Barrett mucosa
and stricture.

(Left) Esophagram shows

two strictures on an ulcer
(arrow) and hiatal hernia.
(Right) Two views from an
esophagram show a
polypoid mass (arrow);
adenocarcinoma arising in
Barrett mucosa.
 CAUSTIC ESOPHAGITIS

2
24

Graphic shows long stricture of esophagus and mucosal Esophagram shows long tapered high grade stricture of
necrosis. distal two-thirds of esophagus, several months after
ingestion of lye in a suicide attempt.

• Strong alkali: Liquid lye (concentrated sodium

ITERMINOlOGY hydroxide) usually used as drain cleaner
Abbreviations and Synonyms o Classification based on clinical & radiological
• Corrosive esophagitis findings
• Acute & chronic phases
Definitions • Mild & severe injury patterns
• Esophageal inflammation/injury due to alkali or acid
Radiographic Findings
• Chest PA & lateral views (acute)
I IMAGING FINDINGS o Dilated, gas-filled esophagus
o Esophageal perforation
General Features • Mediastinal widening, pneumomediastinum,
• Best diagnostic clue: Nondistensible, rigid segment pleural effusion
(stricture) of esophagus with ulcerated mucosa • Fluoroscopic guided water soluble or barium contrast
• Location studies
o Any esophageal segment is involved o Acute mild phase
o Usually middle & lower thirds • Atonic dilated esophagus
• Other general features • Multiple shallow, irregular ulcers
o Accidental or intentional ingestion of caustic agents o Acute severe phase
cause • Extensive ulceration
• Mild or severe injury to upper GI tract: • Diffusely narrowed esophagus with irregular
Esophagus/stomach/ duodenum contour
o Esophagus is most commonly injured in caustic • Double-barreled appearance: Linear or streaky
ingestion collections of barium in esophageal wall
o After esophagus, gastroduodenal injury is most o Chronic phase
common • Sacculations, pseudodiverticula
o Most commonly used caustic agent in US • Long or short segmental strictures: Smooth,
concentric & symmetric or irregular, eccentric &
asymmetric

DDx: Long Segmental Stricture

Peptic Stricture Candida Carcinoma NC Intubation

 CAUSTIC ESOPHAGITIS

Key Facts
Terminology Top Differential Diagnoses
• Corrosive esophagitis • Reflux esophagitis
• Esophageal inflammation/injury due to alkali or acid • Infectious esophagitis
• Esophageal carcinoma
Imaging Findings
• Best diagnostic clue: Nondistensible, rigid segment
•
•
Radiation esophagitis
Nasogastric intubation
2
(stricture) of esophagus with ulcerated mucosa
• Diffusely narrowed esophagus with irregular contour Diagnostic Checklist 25
• Double-barreled appearance: Linear or streaky • Rule out other inflammatory & noninflammatory
collections of barium in esophageal wall pathology which can cause long segmental strictures
• Long or short segmental strictures: Smooth, • Usually history of strong alkali or acid ingestion &
concentric & symmetric or irregular, eccentric & endoscopy with biopsy yields definitive diagnosis
asymmetric • Diffuse long segmental narrowing of thoracic
• Diffuse long stricture: Thread-like or filiform esophagus with irregular contour & extensive
appearance of entire thoracic esophagus (due to ulceration giving rise to thread-like or filiform
extensive scarring & fibrosis) appearance highly suggestive of caustic ingestion

• Diffuse long stricture: Thread-like or filiform

appearance of entire thoracic esophagus (due to Infectious esophagitis
extensive scarring & fibrosis) • Examples: Fungal & viral
o Fungal: Candida albicans
CT Findings o Viral: Herpes, CMV & HIV
• Diffuse circumferential esophageal wall thickening (:? • Acute or early Candida esophagitis
Smm) o "Foamy" esophagus
• Acute phase • Multiple tiny, round lucencies on top of barium
o "Target sign": Mucosal enhancement & hypodense o "Cobblestone" or "snakeskin" appearance
submucosa • Severe or advanced Candidiasis
o Esophageal perforation: Pneumomediastinum, o Grossly irregular or "shaggy" esophagus
pleural effusion o Long tapered distal stricture due to scarring
• Chronic phase: Luminal irregularity & narrowing • May be indistinguishable from caustic stricture
• Thread-like or filiform esophagus differentiates
Imaging Recommendations caustic from infectious stricture
• Videofluoroscopic esophagram (en face/profile views) • Viral esophagitis (herpes, CMV, HIV)
• Acute phase: Water soluble, non-ionic contrast agent o Multiple small discrete superficial punctate ulcers
o One or more giant, flat ovoid or elongated ulcers
more than 1 cm in size
I DIFFERENTIAL DIAGNOSIS o Diagnosis: Endoscopic biopsy & history
Reflux esophagitis Esophageal carcinoma
• Acute reflux esophagitis • Asymmetric contour with abrupt proximal borders of
o Mucosa narrowed distal segment
• Fine nodular or granular o Rat-tail appearance
• Pseudomembrane (plaque-like) • Lye stricture with irregular margins or abrupt borders
o Ulcers o Indistinguishable from infiltrating carcinQma
• Multiple tiny ulcers • Diagnosis: Endoscopic biopsy & history
• Mucosal edematous mounds, radiating folds,
puckering Radiation esophagitis
o Folds • Granular mucosa, decreased distensibility of irradiated
• Thickened vertical or transverse (more than 3 segment
mm) • Stricture: Usually smooth, tapered narrowing within
o Inflammatory polyps: Smooth, ovoid elevations area of radiation
• Chronic reflux esophagitis • History of lung cancer with mediastinal irradiation
o Sacculations or pseudodiverticula may be seen
Nasogastric intubation
o Peptic stricture
• Usually seen in patients with long standing intubation
• Concentric, smooth tapered narrowing of short
• More commonly seen in institutionalized people
distal segment
• Smooth narrowing of esophagus seen
• May be indistinguishable from caustic stricture
o Location: Esophageal lumen that comes in contact
• History & associated sliding hernia differentiates
with distal part of nasogastric tube
reflux from caustic esophagitis
 CAUSTIC ESOPHAGITIS
I PATHOLOGY
General Features
• Etiology
o Strong alkaline agents: Liquid lye
• Liquid lye is concentrated sodium hydroxide
2 • Pathogenesis: Injury by liquefaction necrosis
o Strong acids: Hydrochloric, sulfuric, acetic, oxalic,
carbolic/nitric
26
• Pathogenesis: Injury by coagulative necrosis
o Ammonium chloride, phenols, silver nitrate
o Degree of injury depends on
• Nature/concentration/volume of agent & duration
o Adults: Intentional (to commit suicide)
o Children: Accidental
• Epidemiology: Increased incidence of caustic
esophagitis by liquid lye in US
• Associated abnormalities: Associated gastroduodenal
injuries seen in 5-10% cases
Gross Pathologic & Surgical Features
• Hyperemia/ inflammation/necrosis/ulcera tion/ strictures
Microscopic Features
• Thinning of stratified epithelium, inflammatory
basal cell hyperplasia
Staging, Grading or Classification Criteria
• Classification based on pathology
o Stage I: Acute necrotic phase (1-4 days)
o Stage II: Ulceration-granulation phase (5-28 days)
o Stage III: Cicatrization & scarring (3-4 weeks)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Pain, drooling, vomiting, hematemesis
o Odynophagia, fever, shock
• Clinical profile: Patient with history of caustic
ingestion & painful swallowing
• Diagnosis
o History of alkali or acid ingestion
o Endoscopy & water soluble contrast esophagraphy
• Detects extent & severity of esophageal injury
Demogr<!phics
• Age: Any age group
Natural History & Prognosis
• Complications
o Perforation, mediastinitis, peritonitis, fistulas, shock
o Increased risk of cancer after 20-40 yrs
• Prognosis
o Acute mild phase with early treatment: Good
o Acute severe & chronic phases: Poor
Treatment
• Medical
o Steroids, antibiotics, parenteral feedings
o Esophageal bougienage
• Surgical
o Esophageal bypass
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other inflammatory & noninflammatory
pathology which can cause long segmental strictures
• Usually history of strong alkali or acid ingestion &
endoscopy with biopsy yields definitive diagnosis
Image Interpretation Pearls
• Diffuse long segmental narrowing of thoracic
esophagus with irregular contour & extensive
ulceration giving rise to thread-like or filiform
appearance highly suggestive of caustic ingestion
I SELECTED REFERENCES
1. Dibble C et al: Detection of reflux esophagitis on
double-contrast esophagrams and endoscopy using the
histologic findings as the gold standard. Abdom Imaging.
29:421-5,2004
2. Sam JW et al: The "foamy" esophagus: a radiographic sign
of Candida esophagitis. AJRAm J Roentgenol.
174(4):999-1002,2000
3. Berkovich GY et al: CT findings in patients with
cells, esophagitis. AJRAmJ Roentgenol. 175(5):1431-4,2000
4. Catalano 0 et al: Radiologic findings in chronic esophagitis
dissecans. AJRAm J Roentgenol. 170(6):1671-2, 1998
5. Collazzo LA et al: Acute radiation esophagitis: radiographic
findings. AJRAm J Roentgenol. 169(4):1067-70, 1997
6. Glick SN: Barium studies in patients with Candida
esophagitis: pseudoulcerations simulating viral esophagitis.
AJRAm J Roentgenol. 163(2):349-52, 1994
7. Thompson JK et al: Detection of gastroesophageal reflux:
Value of barium studies compared with 24-hr pH
monitoring. AJR. 162: 621-6, 1994
8. Levine MS: Radiology of esophagitis: a pattern approach.
Radiology. 179(1):1-7, 1991
9. Levine MS et al: Update on esophageal radiology. AJRAm J
Roentgenol. 155(5):933-41, 1990
10. Mann NS et al: Barrett's esophagus in patients with
symptomatic reflux esophagitis. Am J Gastroenterol.
84(12):1494-6, 1989
11. Levine MS et al: Herpes esophagitis: sensitivity of
double-contrast esophagography. AJRAm J Roentgenol.
151(1):57-62, 1988
12. Williams JM: Medication-induced esophagitis: diagnosis by
double-contrast esophagography. AJRAm J Roentgenol.
149(3):646, 1987
13. Goldman LP et al: Corrosive substance ingestion: a review.
American Journal of Gastroenterol. 79: 85-90, 1984
14. Creteur V et al: The role of single and double-contrast
radiography in the diagnosis of reflux esophagitis.
Radiology. 147(1):71-5, 1983
15. Graziani L et al: Reflux esophagitis: radiologic-endoscopic
correlation in 39 symptomatic cases. Gastrointest Radiol.
8(1):1-6,1983
16. Creteur V et al: Drug-induced esophagitis detected by
double-contrast radiography. Radiology. 147(2):365-8,
1983
17. Muhletaler CA et al: Acid corrosive esophagitis:
radiographic findings. AJR. 134: 1137-1140, 1980
18. Franken EA: Caustic damage of the gastrointestinal tract:
Roentgen features. AJR. 118: 77-85, 1973
19. Martel W: Radiologic features of esophagogastritis
secondary to extremely caustic agents. Radiology. 103:
31-36, 1972
 CAUSTIC ESOPHAGITIS

I IMAGE GALLERY

(Left) Lateral view

esophagram hours after
caustic ingestion. Complete 2
obstruction of mid-distal
esophagus. (Right) Frontal
chest radiograph following 27
water soluble esophagram.
Complete obstruction of
esophagus with stasis. Acute
injury.

Typical
(Left) Axial CECT of patient 2
hours after caustic ingestion.
Marked thickening of
esophageal wall. (Right)
Axial CECT shows of patient
following suicide attempt by
caustic ingestion. Marked
thickening of gastric wall
with submucosal edema
(arrow).

Typical
(Left) Esophagram one week
following a lye ingestion.
Long stricture with deep
ulcerations. (Right)
Esophagram one month after
caustic ingestion shows short
esophagus, long stricture,
and hiatal hernia. Reflux
esophagitis may have similar
appearance.
 DRUG-INDUCED ESOPHAGITIS

2
28

Graphic shows medication pills "stuck" at level of aortic Esophagram shows broad shallow ulceration (arrow) at
arch with focal stricture and ulceration. aortic arch level. Patient had odynophagia and recent
tetracycline ingestion. Spontaneously resolved.

• Superficial ulceration
ITERMINOlOGY o Solitary or localized cluster of tiny ulcers distributed
Abbreviations and Synonyms circumferentially on normal background mucosa
• Drug-induced esophagitis (DIE) o En face: Punctate, linear, stellate, serpiginous, or
• Pill-induced; medication-induced esophagitis ovoid; collections of barium on esophageal mucosa
o In profile; seen as shallow depressions
Definitions o Thickening or distortion of adjacent esophageal
• Iatrogenic esophageal injury induced by oral folds; surrounding nodular mucosa
medication and direct contact o Usually with ingestion of doxycycline, tetracycline
• Giant, flat ulcers
o Several centimeters or more in length
I IMAGING FINDINGS o Larger areas of ulceration; with potassium chloride
& quinidine; in patients with cardiomegaly
General Features o Mass effect surrounding ulcer; due to edema &
• Best diagnostic clue inflammation; mimicking ulcerated carcinoma
o Definite temporal relationship between ingestion of o Smooth, re-epithelialized depressions; seen with
offending drug & onset of esophagitis healing of these ulcers; mimicking active ulcer crater
• Healing of lesions after withdrawal of drug • Strictures: Short segmental; concentric narrowing
• Location o Usually with potassium chloride & quinidine
o Most common site; mid-esophagus o Above level of enlarged left atrium, compressing
• Near level of aortic arch or left main bronchus distal esophagus; passage of pill impeded
• Morphology • Patients taking these pills often have
o Superficial ulcers cardiomegaly, mitral-valvular disease
o Solitary ring ulcer; several discrete ulcers; • Severe esophagitis; may see stricture
diamond-shaped ulcer • Repeat esophagram 7-10 days after withdrawal of drug
o Deep ulcers & strictures o May show healing or disappearance of lesions
Radiographic Findings Imaging Recommendations
• Double-contrast esophagography (en face & in profile) • Videofluoroscopic double-contrast esophagram

DDx: Superficial Esophageal Ulceration +/- Stricture

Reflux Esoph. Herpes Candida Barrett Esoph.

 DRUG-INDUCED
Key Facts
Terminology
• Iatrogenic esophageal injury induced by oral
medication and direct contact
Imaging Findings
• Most common site; mid-esophagus
• Superficial ulceration
• Giant, flat ulcers
• Strictures: Short segmental; concentric narrowing
I DIFFERENTIAL DIAGNOSIS
Reflux esophagitis
• Fine nodular; granular mucosa; superficial ulceration
• Peptic stricture: Concentric smooth; eccentric
narrowing of distal esophagus
• In DIE, normal appearing mucosa below focal
ulceration in mid-esophagus; unusual with reflux
Infectious esophagitis
• Herpes esophagitis; multiple, small, discrete,
superficial ulcers; in upper or mid-esophagus
o 'lmmunocompromised patients; in healthy patients
may be indistinguishable from DIE on imaging
• CMV + HIV; large superficial ulcers
• Clinical history & presentation help differentiate
Esophageal carcinoma
• Irregular narrowing; nodular, ulcerated mucosa
Barrett esophagus
• Mid-esophageal stricture; deep ulcer crater; hiatal
hernia, reflux
I PATHOLOGY
General Features
• General path comments
o Prolonged contact of drug with esophageal mucosa
o Secondary to drugs ability to alter local condition
o Pill taken at bedtime, recumbent, without water
o 22 times more frequent with capsules than with
tablets; easier mucosal adhesion; slow disintegration
• Etiology
o Doxycycline, tetracycline, potassium chloride,
quinidine, aspirin, nonsteroidal antiinflammatory
o Haloperidol, alendronate, ferrous sulfate, ascorbic
acid, clindamycin, lincomycin, alprenolol chloride
• Epidemiology
o DIE may be under-recognized
o Doxycycline & tetracycline account for half the
reported cases of DIE
Microscopic Features
• Chemical esophagitis; erosions or ulcerations,
exudative inflammation
ESOPHAGITIS
Top Differential Diagnoses
• Reflux esophagitis
• Infectious esophagitis
• Esophageal carcinoma
• Barrett esophagus
2
Pathology
• Doxycycline, tetracycline, potassium chloride, 29
quinidine, aspirin, nonsteroidal antiinflammatory
I CLINICAL ISSUES
Presentation
• Odynophagia (94%), retrosternal burning pain (75%),
dysphagia (56%), severe chest pain
• Elderly; those with pre-existing esophageal disease
• Symptoms resolve rapidly after withdrawal of
offending medication; usually within 7-10 days
• Diagnosis: Endoscopy; presence & healing of lesions
Natural History & Prognosis
• Clinical course may be relatively uneventful, without
any long term sequelae
• Complications: Persistent dysphagia due to stricture
o Rarely, hemorrhage, perforation
Treatment
• Avoided by taking pills upright & with plenty of fluids
• Withdrawal of offending medication
• Proton-pump inhibitor & prokinetic; sucralfate
I SELECTED REFERENCES
1. McCullough RW et al: Pill-induced esophagitis complicated
by multiple esophageal septa. Gastrointest Endosc.
59(1):150-2, 2004
2. O'Neill JL et al: Drug-induced esophageal injuries and
dysphagia. Ann Pharmacother. 37(11):1675-84, 2003
3. Levine MS: Drug-induced disorders of the esophagus.
Abdom Imaging. 24(1):3-8, 1999
I IMAGE GAllERY
(Left) Double-contrast esophagram shows cluster of ulcers in distal
esophagus. (Right) Elderly patient with heart disease taking Quinidine
and other medications. Stricture and ulceration at thoracic inlet, an
unusual site for drug-induced esophagitis.
 RADIATION ESOPHAGITIS

2
30

Axial CECT of a patient with dysphagia following Axial CECT at lung windows shows radiation fibrosis of
radiation therapy for lung cancer shows marked the lung in a patient with lung cancer and radiation
thickening of esophageal wall surrounding the NG tube. esophagitis.

• Due to punctate ulcers, edema, spasm

ITERMINOlOGY • With t distensibility of irradiated segment
Abbreviations and Synonyms o Thickened mucosal folds due to edema
• Radiation esophagitis (RE) o Slight luminal narrowing within radiation portal
o Disordered motility; interruption of primary
Definitions peristalsis at superior border of radiation portal
• Inflammation of esophageal mucosa + wall induced by • Numerous non peristaltic contractions distal to
curative & palliative radiotherapy (RT) point of disruption of primary wave
• Usually 4-8 weeks after completion of RT; may
present even decades after RT
I IMAGING FINDINGS • Chronic RE
o Strictures: Concentric, smooth, tapered narrowing
General Features • Upper or mid-esophagus; within radiation portal
• Location: Usually within prior radiation portal • Usually 4-8 months after completion of RT
• Morphology o Ulcers at site of extrinsic compression by
o Acute RE: Superficial ulcers, granular mucosa mediastinal lymphadenopathy, tumor
o Chronic RE: Deep ulcers, strictures, fistula o Late developing deep ulcers; ominous; may progress
to fistula formation
Radiographic Findings o Fistula: Left main bronchus; most frequent site
• Fluoroscopy: Double-contrast esophagography • Radiation necrosis; usually within first year of RT
• Acute RE • Avoid ionic; use nonionic water soluble contrast if
o Superficial ulcers; shallow, irregular collections of suspected
barium on esophageal mucosa o Retraction of esophagus; related to lung resection &
• May be seen within 7-10 days of RT regional fibrosis
• Location: Usually conforms to radiation portal;
sharp demarcation at inferior border of portal Imaging Recommendations
• Severe RE; grossly irregular, serrated contour; • Videofluoroscopic esophagram; en face + profile views
larger areas of ulcerations, mucosal sloughing o Start with nonionic water soluble
o Granular appearance of mucosa • Follow with barium if no leak or fistula

DDx: Stricture and Mucosal Irregularity

i.

...
i:
,'\ :

'

"
Peptic Stricture Lye Stricture Candida Drug-Ind. Strict.
 RADIATION ESOPHAGITIS

Key Facts
Terminology Top Differential Diagnoses
• Inflammation of esophageal mucosa + wall induced • Reflux esophagitis
by curative & palliative radiotherapy (RT) • Caustic esophagitis
• Infectious esophagitis
Imaging Findings
• Location: Usually within prior radiation portal
•
•
Nasogastric intubation
Tumor recurrence
esophagitis
2
• Acute RE: Superficial ulcers, granular mucosa
• Chronic RE: Deep ulcers, strictures, fistula Clinical Issues 31
• Substantial morbidity

• Diagnosis: Acute RE on clinical grounds; temporal

I DIFFERENTIAL DIAGNOSIS relationship between onset of RT & onset of symptoms
Reflux esophagitis • Imaging & endoscopy + biopsy; not usually performed
• Peptic stricture; concentric or eccentric smooth to confirm acute RE
narrowing of distal esophagus + proximal dilatation o In chronic RE; to detect strictures, tumor recurrence

Caustic esophagitis Natural History & Prognosis

• Acute: Atonic dilated esophagus; multiple shallow,
irregular ulcers
• Chronic: Long stricture, irregular contour
Infectious esophagitis
• Rarely causes stricture
• Candida; plaques + ulcers; shaggy surface
Nasogastric intubation esophagitis
• Unusually long, rapidly progressive stricture; long
segment extensive ulceration; mid & distal esophagus
Tumor recurrence
• Irregular, eccentric narrowing; extrinsic mass effect
• Nodularity, "shaggy" ulcerated core; abrupt junction
• Late reactions rare; might be severe, life-threatening
• Substantial morbidity
Treatment
• Early complications: Viscous lidocaine, indomethacin
• Strictures: Endoscopic dilatation, stent-implantation,
endoscopic percutaneous gastrostomy
• Radio-protective: Pretreatment with amifostine
I DIAGNOSTIC CHECKLIST
Consider
• Tumor infiltration of esophagus should be excluded
• With esophageal carcinoma, severe dysphagia before
RT; clinical evidence of RE often masked

I PATHOLOGY
I SELECTED REFERENCES
General Features
1. Collazzo LA et al: Acute radiation esophagitis: radiographic
• Etiology findings. A]R Am] Roentgenol. 169(4):1067-70, 1997
o RT for adjacent thoracic & cervical neoplasms; 2. Seeman H et al: Esophageal motor dysfunction years after
Hodgkin disease, lung, mediastinum, spine radiation therapy. Dig Dis Sci. 37(2):303-6, 1992
o RT for primary esophageal carcinoma 3. Chowhan NM: Injurious effects of radiation on the
• External beam; intraluminal brachytherapy esophagus. Am] Gastroenterol. 85(2):115-20, 1990
• Smaller doses; 2,000-4,500 rads ~ self-limited RE
without permanent damage
• Doses of 4,500-6,000 rads ~ severe RE; irreversible I IMAGE GALLERY
• Chemotherapy; Adriamycin; potentiates effect of RT
o RE occurs earlier; even at low doses of RT
Microscopic Features
• Acute: Ulceration; necrosis; sloughing of mucosa
• Chronic: Marked thickening of submucosa; edema &
fibrosis; cicatrization process; strictures

I CLINICAL ISSUES
Presentation
• Acute RE: Substernal burning, dysphagia, odynophagia
o Within 2-3 weeks of onset of RT (Left) Two views from esophagram show a mid-esophageal stricture
o Usually abates within few weeks of cessation of RT following radiation therapy for lung cancer. (Right) Sagittal
• Chronic RE: Motor dysfunction; even decades after RT reformation of CECT shows a long stricture and mural thickening
(arrow) of esophagus following radiation for breast cancer.
 ACHALASIA, CRICOPHARYNGEAL

2
32

Graphic shows hypertrophied contracted Lateral view from esophagram shows rounded,
cricopharyngeus muscle (arrow) at the hypertrophied cricopharyngeus muscle impinging on
pharyngo-esophageal junction (usually near the C5-6 the posterior lumen at the pharyngo-esophageal
cervicalleve/). junction (arrow).

ITERMINOLOGY Radiographic Findings

Definitions
• Failure of cricopharyngeal muscle (upper esophageal
sphincter) relaxation due to hypertrophy or spasm
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Prominent cricopharyngeus
muscle at pharyngoesophageal junction with retention
of barium in pharynx on lateral view
• Location: Pharyngoesophageal junction: CS-6 level
• Morphology: Prominent or hypertrophied
cricopharyngeus muscle
• Other general features
o Activity of constrictor muscles must be coordinated
with cricopharyngeal relaxation & luminal opening
o Cricopharyngeus must relax & open completely to
allow unimpeded passage of bolus
o Complete opening of lumen at pharyngoesophageal
junction is accomplished by several factors
• Cricopharyngeal relaxation
• Superior & anterior movement of larynx
• Pharyngeal constriction, producing thrust
• Intrabolus pressure
• Videofluoroscopic pharyngoesophagraphy
o Lateral view
• Smoothly outlined shelf or lip-like projection
posteriorly at level of cricoid (CS-6 level)
o Prominent cricopharyngeus muscle
o Prolonged closure of cricopharyngeus muscle
o Jet effect: Noted below narrowing simulating a
stenotic lesion
o Pharyngeal retention of barium; ± aspiration
o Distention of proximal cervical esophagus & distal
pharynx
Imaging Recommendations
• Video fluoroscopic recording: Frontal/lateral/oblique
o Rapid sequence required for demonstration
I DIFFERENTIAL DIAGNOSIS
Cervical osteophytes (indentation)
• Large anterior cervical osteophytes can impinge on
pharyngoesophageal junction simulating
cricopharyngeal achalasia
Esophageal tumor
• Tumor at pharyngoesophageal junction may constrict
the lumen concentrically or eccentrally

DDx: "Mass" at Pharyngo-Esophageal Junction

Osteophytes Carcinoma Esophageal Web

 ACHALASIA, CRICOPHARYNGEAL

Key Facts
Terminology • Smoothly outlined shelf or lip-like projection
• Failure of cricopharyngeal muscle (upper esophageal posteriorly at level of cricoid (CS-6 level)
sphincter) relaxation due to hypertrophy or spasm • Jet effect: Noted below narrowing simulating a
stenotic lesion
Imaging Findings • Distention of proximal cervical esophagus & distal
• Best diagnostic clue: Prominent cricopharyngeus pharynx 2
muscle at pharyngoesophageal junction with
retention of barium in pharynx on lateral view
Top Differential Diagnoses 33
• Cervical osteophytes (indentation)
• Esophageal tumor

o Benign tumor: Smooth & well-defined margins o Does not impede passage of fluids or food
o Malignant: Mucosal irregularity/ill-defined margins o Usually resolves within a few weeks of birth
• Complications
o Rare: Aspiration, pneumonia, lung abscess
I PATHOLOGY • Prognosis
o Usually good
General Features
• General path comments Treatment
o Embryology-anatomy • Cricopharyngeal myotomy
• May be due to incomplete maturation of • Treat underlying problem
neurologic reflexes governing swallowing o Such as reflux esophagitis with spasm
• Etiology o Neuromuscular disorder
o Usually just "poor timing" of CP contraction
o Due to "presbyesophagus" or other cause of
dysmotility I DIAGNOSTIC CHECKLIST
o Compensatory mechanism to GE reflux
o Neuromuscular dysfunction of deglutition Consider
• Primary neural disorders: Brainstem disorder • Persistent narrowing or just intermittent indentation
(bulbar poliomyelitis, amyotrophic lateral Image Interpretation Pearls
sclerosis, multiple sclerosis), cerebrovascular
• Smoothly outlined lip-like projection posteriorly at
occlusive disease, Huntington chorea
CS-6 level with jet effect seen via narrowed lumen
• Primary muscle disorder: Myotonic dystrophy,
polymyositis, dermatomyositis, sarcoidosis,
myopathies secondary to steroids, thyroid
dysfunction, oculopharyngeal myopathy
I SELECTED REFERENCES
• Myoneural junction disorder: Myasthenia gravis, 1. Curtis DJ et al: The cricopharyngeal muscle: A
diphtheria, tetanus videorecording review. AJR. 142: 497-500, 1984
2. Ekberg 0 et al: Dysfunction of the cricopharyngeal muscle.
• Epidemiology: Unusual in severe form
A cineradiographic study of patients with dysphagia.
• Associated abnormalities Radiology. 143: 481-6, 1982
o Pharyngeal paresis, gastroesophageal reflux 3. Bergman AB et al: Complete esophageal obstruction from
o Esophageal motility disturbance: Spasm/achalasia cricopharyngeal achalasia. Radiology. 123: 289-90, 1977
o Aging, Zenker diverticulum
Gross Pathologic & Surgical Features
• Hypertrophy of cricopharyngeus muscle
I IMAGE GALLERY

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Intermittent
symptoms: Dysphagia, food "sticking" in throat
Demographics
• Age: Any age group, more (10%) in elderly
• Gender: M = F
Natural History & Prognosis
• Natural history in infants (Left) Lateral esophagram shows prominent cricopharyngeus (arrow),
o Prominent cricopharyngeal impression on pharyngeal distention, and an esophageal web (open arrow). (Right)
barium-filled esophagus Two views from rapid sequence esophagram show cricopharyngeal
achalasia (arrow) and tracheal aspiration (open arrow).
 ACHALASIA, ESOPHAGUS

2
34

Upright frontal esophagram shows dilated esophagus Esophagram shows grossly dilated, tortuous esophagus
with an abrupt taper ("bird beak") just above the GE with "sigmoid" appearance.
junction. Note absent gastric air bubble.

• Vigorous: Simultaneous high-amplitude &

ITERMINOlOGY repetitive contractions
Definitions • Both variants are transitional & finally evolve into
• Primary motility disorder, esophageal smooth muscle classic achalasia
o Classic achalasia (primary): Simultaneous
low-amplitude contractions
I IMAGING FINDINGS o Motor function of pharynx & upper esophageal
sphincter are normal
General Features
Radiographic Findings
• Best diagnostic clue: "Bird-beak" deformity-dilated
esophagus with smooth, symmetric, tapered • Radiography .
o Chest x-ray AP & lateral views: Advanced achalaSIa
narrowing at esophagogastric region
• Classic mediastinal widening; double contour of
• Morphology: Grossly dilated esophagus with smooth
mediastinal borders
tapering at lower end of esophagus
• Outer borders represent dilated esophagus
• Other general features
projecting beyond shadows of aorta & heart
o Classified based on etiology
• Anterior tracheal bowing
• Primary (idiopathic)
• Air-fluid level in mediastinum; small or absent
• Secondary (pseudoachalasia)
gastric air bubble
o Manometric characteristics of achalasia
• Lower lobes: Decreased lung volume, linear
• Absence of primary peristalsis
opacities & tubular radiolucencies
• Increased or normal resting lower esophageal
• Videofluoroscopic barium study findings
sphincter (LES) pressures
o Primary achalasia
• Incomplete or absent LES relaxation on
• Markedly dilated esophagus
swallowing
• Absent primary peristalsis
o Variants of achalasia: Atypical manometric findings
• "Bird-beak" deformity: V-shaped conical &
• Early: Characterized by aperistalsis with normal
smooth, symmetric, tapered narrowing of distal
LES pressure
esophagus extending to GE junction

DDx: Dilated Nonperistaltic Esophagus

41 Scleroderma Gastric Ca. Peptic Stricture Post Vagotomy

 ACHALASIA, ESOPHAGUS
Key Facts
Terminology
• Primary motility disorder, esophageal smooth muscle
Imaging Findings
• Best diagnostic clue: "Bird-beak" deformity-dilated
esophagus with smooth, symmetric, tapered
narrowing at esophagogastric region
• Absent primary peristalsis
• Timed barium swallow studies
Top Differential Diagnoses
• Scleroderma
• Esophageal carcinoma
• Gastric carcinoma
• Esophagitis with stricture
• Neuromuscular disorders
• Post surgical change (post vagotomy)
• Hurst phenomenon: Temporary transit via cardia
when hydrostatic pressure of barium column is
above tonic LESpressure
• Length of narrowed segment: Less than 3.5 cm;
widest diameter is more than 4 cm
o Secondary achalasia (pseudoachalasia)
• Mildly dilated esophagus (less than 4 cm at its
widest point)
• Decreased or absent peristalsis
• Eccentricity, nodularity, shouldering of narrowed
distal segment or
• Smooth, symmetric, tapered narrowing of distal
esophagus
• Length of distal narrowed esophageal segment:
More than 3.5 cm (approximately)
CT Findings
• Moderate to marked dilatation of esophagus with
diameter more than 4 cm
• Decreased or normal wall thickness
• Air-fluid level within dilated esophagus
• Abrupt, smooth narrowing distal esophageal segment
near gastroesophageal (GE) junction
• Squamous cell carcinoma of esophagus in
longstanding achalasia
o Mild dilatation of esophagus; irregular wall
thickening
o Eccentric narrowing of distal esophagus extending
into GE junction
o Involvement of periesophageal soft tissues & blood
vessels
o Enlarged mediastinal lymph nodes
Imaging Recommendations
• Videofluoroscopic barium studies
• Helical CT including sagittal reconstructions
• Timed barium swallow studies
o Aid in diagnosis & management of achalasia
• Transit & emptying studies
o Quantitate esophageal retention before & after
therapy
o Standing films taken after ingestion of 200 ml
barium: At I, 2, and 5 minutes
Pathology
• Primary achalasia: Idiopathic or neurogenic disorder
• Abnormality of myenteric ganglia (decrease in
number) in Auerbach plexus
• Degenerative vagal nerve changes
• Secondary achalasia: Intrinsic/extrinsic neoplasm, 2
peptic/fungal stricture, scleroderma, Chagas disease,
35
postvagotomy effect
Clinical Issues
• Longstanding dysphagia, weight loss
• Regurgitation, foul breath
• Esophageal carcinoma (in 2-7% of cases)
Diagnostic Checklist
• Seek evidence of cancer, prior surgery, severe GERD
I DIFFERENTIAL DIAGNOSIS
Scleroderma
• Collagen vascular disease; more common in women
• Pathogenesis
o Smooth muscle atrophy/fibrosis; incompetent LES
o Reflux esophagitis & high risk of peptic stricture &
Barrett esophagus
• Uncomplicated cases: Dilated esophagus with a
patulous esophagogastric region
• Fluoroscopic barium study findings
o Aperistalsis, dilated esophagus
o Peptic stricture in distal one third
• May simulate primary achalasia
o Diagnosis
• Anti-Scl 70 & anticentromere antibodies
• Endoscopic biopsy
Esophageal carcinoma
• Asymmetric contour with abrupt proximal borders of
narrowed distal segment (rat-tail appearance)
• Mucosal irregularity, shouldering, mass effect
• Periesophageal & distal spread may be seen
• Occasionally, smooth, tapered narrowing of lower
esophagus with aperistalsis, simulating achalasia
• Diagnosis: Endoscopic biopsy & history
Gastric carcinoma
• Malignancy involving gastric cardia with extension
into distal esophagus shows
o Smooth, tapered narrowing of lower esophagus with
aperistalsis, simulating achalasia
• Growth may show mucosal irregularity & mass effect
• Diagnosis: Endoscopic biopsy & history
Esophagitis with stricture
• Peptic stricture: Smooth tapered narrowing of short
distal segment
o Almost always associated with hiatal hernia &
gastroesophageal (GE) reflux
o Rarely associated with aperistalsis or gross dilatation
• Mucosa: Fine nodular/granular or plaque-like
• Ulcers: Multiple tiny, radiating folds & puckering
 ACHALASIA, ESOPHAGUS
• Folds: Thickened vertical or transverse
• Diagnosis: Endoscopic biopsy & history
Neuromuscular disorders
• Diffuse esophageal spasm (DES)
o characterized by chest pain, radiation to shoulder
simulating angina & dysphagia
2 o Cause is unknown; may be neurogenic damage
o Intermittent disruption of primary peristalsis
36 associated with focally obliterative simultaneous
contractions
o Sometimes contractions are repetitive & esophageal
lumen may show typical "corkscrew" or "rosary
bead" appearance
o Mostly normal LESfunction with complete
sphincter relaxation during swallowing
o Diagnosis: Clinical, radiographic & manometric
Post surgical change (post vagotomy)
• Stricture and neural damage may simulate achalasia
!PATHOLOGY
General Features
• General path comments
o Progressive dilatation of esophagus above LES
o Marked esophageal wall thinning + risk of rupture
o Following mucosal changes may be seen
• Ulceroinflammatory lesions with white thickened
patches (leukoplakia)
• Superimposed infection (e.g., Candida esophagitis)
• Sites of dysplasia or neoplasia
• Etiology
o Primary achalasia: Idiopathic or neurogenic disorder
o Pathogenesis of neurogenic disorder
• Abnormality of myenteric ganglia (decrease in
number) in Auerbach plexus
• Degenerative vagal nerve changes
• Decreased cell bodies in dorsal motor vagal
nucleus: Primary defect is in extraesophageal
parasympathetic nerve supply & esophageal
changes are secondary
o Secondary achalasia: Intrinsic/extrinsic neoplasm,
peptic/fungal stricture, scleroderma, Chagas disease,
postvagotomy effect
• Epidemiology: Uncommon primary motility disorder
Gross Pathologic & Surgical Features
• Massively dilated esophagus with smooth narrowed
distal segment
Microscopic Features
• Decreased number of ganglion cells in myenteric
plexus of esophagus
\CLlNICAL ISSUES
Presentation
• Most common signs/symptoms
o Primary achalasia
• Longstanding dysphagia, weight loss
• Regurgitation, foul breath
o Secondary achalasia
• Short duration of dysphagia
• Chest pain or odynophagia
Demographics
• Age
o Primary achalasia: Younger patients (30-50 years)
o Secondary achalasia: Older patients
• Gender: Equal in both males & females
Natural History & Prognosis
• Complications
o Coughing, aspiration, pneumonia, lung abscess
o Esophageal carcinoma (in 2-7% of cases)
• Prognosis
o Treatment cannot correct abnormal esophageal
motility & LESdysfunction
o Aimed at improving esophageal emptying by
disrupting increased LESpressure
Treatment
• Calcium channel blockers; botulinum toxin injection
• Pneumatic dilatation
• Heller myotomy (partial thickness incision of LES)
• Risks of treatment
o Pneumatic dilatation: Perforation
o Myotomy: Outpouching & reflux
I DIAGNOSTIC CHECKLIST
Consider
• Seek evidence of cancer, prior surgery, severe GERD
Image Interpretation Pearls
• Markedly dilated esophagus, absence of primary
peristalsis & "bird-beak" deformity of distal esophagus
I SELECTED REFERENCES
1. Vaezi MF et al: Timed barium oesophagram: better
predictor of long term success after pneumatic dilation in
achalasia than symptom assessment. Gut. 50(6):765-70,
2002
2. Sabharwal T et al: Balloon dilation for achalasia of the
cardia: experience in 76 patients. Radiology. 224(3):719-24,
2002
3. Adler DG et al: Primary esophageal motility disorders.
Mayo Clin Proc. 76(2):195-200,2001
4. Woodfield CA et al: Diagnosis of primary versus secondary
achalasia: reassessment of clinical and radiographic criteria.
A]R Am] Roentgenol. 175(3):727-31,2000
5. de Oliveira]M et al: Timed barium swallow: a simple
technique for evaluating esophageal emptying in patients
with achalasia. A]R Am] Roentgenol. 169(2):473-9, 1997
6. Noh HM et al: CT of the esophagus: Spectrum of disease
with emphasis on esophageal carcinoma. Radiographies 15:
1113-34, 1995
7. Schima W et al: Esophageal motor disorders:
Video fluoroscopic and manometric evaluation-prospective
study in 88 symptomatic patients. Radiology 185: 487-91,
1992
 ACHALASIA, ESOPHAGUS

I IMAGE GALLERY

(Left) Typical esophagram

appearance of achalasia, pre
Heller myotomy. (Right)
Esophagram shows marked
2
reduction of esophageal
dilatation and retention 37
following Heller myotomy.

Typical
(Left) Esophagram in upright
position shows static column
of barium at thoracic inlet;
patient at a great risk for
aspiration pneumonitis.
(Right) Esophagram shows
large pulsion epiphrenic
diverticulum in a patient
with achalasia.

Typical
(Left) Esophagram shows
typical findings of achalasia
plus numerous irregular
plaques (arrow) due to
Candida esophagitis. (Right)
Esophagram in a patient with
long-standing achalasia
shows an "apple core"
irregular constricting mass;
squamous cell carcinoma.
 ESOPHAGEAL MOTILITY DISTURBANCES

2
38

Esophagram shows "corkscrew" esophagus due to Esophagram shows diffuse esophageal spasm with
diffuse esophageal spasm. corkscrew appearance. Also note pulsion diverticulum
(arrow).

• Increased or normal resting lower esophageal

ITERMINOlOGY sphincter (LES) pressures
Abbreviations and Synonyms • Incomplete or absent LES relaxation on
• Diffuse esophageal spasm (DES) swallowing
• Presbyesophagus or nonspecific esophageal motility • Normal upper esophageal sphincter
disorder (NEMD) o Diffuse esophageal spasm: Related to varying degrees
of neurogenic damage
Definitions • Simultaneous contractions & intermittent primary
• Primary & secondary motility disorders of esophageal peristalsis
smooth muscle • Repetitive or prolonged-duration contractions
• High amplitude & frequent spontaneous
contractions
I IMAGING FINDINGS • Normal LES function with complete sphincter
relaxation during swallowing
General Features • Intermittent disruption of primary peristalsis
• Best diagnostic clue associated with focally obliterative simultaneous
o Achalasia: "Bird-beak" deformity-dilated esophagus contractions
with smooth, tapered narrowing at GE junction o Presbyesophagus: Esophageal motility dysfunction
o Scleroderma: Dilated atonic esophagus, distal associated with aging
stricture • Also called nonspecific esophageal motility
• Other general features disorder (NEMD)
o Classification of esophageal motility disorders • Decreased frequency of normal peristalsis
• Primary: Achalasia; diffuse esophageal spasm; • 1 Frequency of nonperistaltic contractions
presbyesophagus • Less commonly, incomplete LES relaxation
• Secondary: Scleroderma o Scleroderma: Multisystemic disorder of small vessels
o Achalasia: Idiopathic or neurogenic disorder & connective tissue
• Absence of primary peristalsis • Decreased or absent resting LES pressure
• Simultaneous low-amplitude contractions • Absent peristalsis in lower two thirds of esophagus

DDx: Abnormal Contour or Contractions of Esophagus

Peptic Stricture Post Fundoplication Esophageal Cancer Gastric Cancer

 ESOPHAGEAL MOTILITY DISTURBANCES
Key Facts
Terminology
• Diffuse esophageal spasm (DES)
• Presbyesophagus or nonspecific esophageal motility
disorder (NEMD)
• Primary & secondary motility disorders of esophageal
smooth muscle
Imaging Findings
• Achalasia: "Bird-beak" deformity-dilated esophagus
with smooth, tapered narrowing at GE junction
• Scleroderma: Dilated atonic esophagus, distal stricture
• Intermittent absence of primary peristalsis in thoracic
esophagus + focally obliterative contractions (DES)
• Presbyesophagus or NEMD: Multiple nonperistaltic
contractions & disrupted primary peristalsis
• Scleroderma: 70% GE reflux ~ 37% Barrett esophagus
Radiographic Findings
• Videofluoroscopic barium studies
o Achalasia
• Markedly dilated esophagus
• Absent primary peristalsis
• "Bird-beak" deformity: V-shaped conical &
smooth, tapered narrowing at esophagogastric
region
o Diffuse esophageal spasm
• Primary peristalsis present in cervical esophagus
• Intermittent absence of primary peristalsis in
thoracic esophagus + focally obliterative
contractions (DES)
• Contractions are repetitive, esophageal lumen
may show "corkscrew" or "rosary bead" pattern
o Presbyesophagus or NEMD: Multiple non peristaltic
contractions & disrupted primary peristalsis
o Scleroderma
• Mild-moderate dilatation of proximal esophagus
• Absence of peristalsis in lower 2/3rd of esophagus
• Patulous GE region + reflux ~ fusiform distal
peptic stricture; ± hiatal hernia
• Erosions & superficial ulcers in distal esophagus
• ± Wide-mouthed sacculations of esophagus
• Scleroderma: 70% GE reflux ~ 37% Barrett
esophagus
Imaging Recommendations
• Videofluoroscopic barium studies
• Timed barium swallow (transit & emptying) studies
o Aid in diagnosis & management
I DIFFERENTIAL DIAGNOSIS
Esophagitis with stricture
• Mucosa: Fine nodular/granular or plaque-like
• Ulcers: Multiple tiny ulcers + thickened folds
• Peptic stricture: Smooth, narrowing of distal segment
o Usually associated with hiatal hernia & GE reflux
o Occasionally may simulate achalasia in absence of
hiatal hernia & GE reflux
Top Differential Diagnoses
• Esophagitis with stricture
• Post-fundoplication
• Esophageal carcinoma
Diagnostic Checklist 2
• Differentiate from conditions which can simulate
primary/secondary esophageal motility disorders 39
• Correlate: Clinical/radiographic/manometric findings
• Check family history of collagen vascular disorders
• Achalasia: Dilated esophagus, absence of primary
peristalsis & "bird-beak" deformity of distal esophagus
• Scleroderma: Mild-moderate dilatation of esophagus
with distal fusiform stricture + !or absent peristalsis
• DES: Disrupted primary peristalsis; "corkscrew" or
"rosary bead" pattern of esophagus
o Distinguished from motility disorders by normal
peristalsis
Post-fundoplication
• Dilated esophagus, narrowed GE junction + delayed
esophageal emptying
• Fairly common result or complication of
fundoplication, especially in elderly
Esophageal carcinoma
• Asymmetric contour with abrupt proximal borders of
narrowed distal segment (rat-tail appearance)
• Mucosal irregularity, shouldering, mass effect
• Periesophageal & distal spread may be seen
• Smooth, tapered narrowing of lower esophagus with
aperistalsis simulates achalasia or scleroderma
• Gastric cancer invading esophagus submucosa may
closely mimic achalasia
• Diagnosis: Endoscopic biopsy & history
I PATHOLOGY
General Features
• General path comments
o Achalasia
• Progressive dilatation of esophagus above LES
• Thin esophageal wall (t risk of rupture)
• May have superimposed infection (e.g., Candida)
• May show sites of dysplasia or neoplasia
o Diffuse esophageal spasm
• Esophageal muscle: Normal « 4 mm) or thickened
o Scleroderma
• Initially smooth muscle atrophy & fragmentation
• Followed by collagen deposition & fibrosis
• Genetics
o Scleroderma
• Localized: Associated with HLA-DRl, 4 & 5
• Diffuse: Associated with HLA-DRS
• Etiology
o Achalasia: Idiopathic or neurogenic disorder
• Abnormality of myenteric ganglia in Auerbach
plexus; degenerative vagal nerve changes
 ESOPHAGEAL MOTILITY DISTURBANCES
o Diffuse esophageal spasm
• Unknown
• Related to varying degrees of neurogenic damage
o Presbyesophagus: Due to aging
o Scleroderma
• Unknown; autoimmune + genetic predisposition
• Environmental antigens: Silica, L-tryptophan
2 • Epidemiology
o Primary motility disorders: Uncommon
40 o Secondary motility disorder (scleroderma)
• Incidence: 14.1 cases/million
• Prevalence: 19-75/100,000 people
• Associated abnormalities: Scleroderma may be
associated with SLE, polymyositis or dermatomyositis
Gross Pathologic & Surgical Features
• Achalasia: Massively dilated esophagus with smooth,
narrowed distal segment
• DES: Esophageal muscle, normal or hypertrophied
• Scleroderma
o Rubber-hose inflexibility: Lower 2/3rd esophagus
o Thin & ulcerated mucosa + distal stricture
Microscopic Features
• Achalasia: I Number of ganglia in myenteric plexus
• DES: Cellular hypertrophy of esophageal muscle
• Scleroderma: Atrophy + fragmentation of smooth
muscle ~ collagen deposition + fibrosis
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Longstanding dysphagia for solids or liquids
o Epigastric fullness, regurgitation, foul breath
o Sensation of food sticking in chest; weight loss
oDES
• Chest pain ± radiation to shoulder or back
simulating angina
• Acute odynophagia due to food impaction
• Lab-data: Manometric findings
• Diagnosis
o Clinical, radiographic & manometric findings
o Endoscopic biopsy & histology
Demographics
• Age
o Achalasia & scleroderma (young patients)
o Presbyesophagus: Elderly people
• Gender
o Primary motility disorders: M = F
o Scleroderma
• Young African-American females (> common);
M:F = 1:3
• Ethnicity: Scleroderma: African-Americans more than
Caucasians
Natural History & Prognosis
• Complications
o Coughing, aspiration, pneumonia, lung abscess
o Scleroderma: Barrett esophagus, adenocarcinoma
• Prognosis
o Achalasia: Fair by improving esophageal emptying
with balloon dilatation or myotomy
o Scleroderma: Limited disease (good); diffuse (poor)
Treatment
• Achalasia: Ca++ channel blockers; botulinum toxin
injection; pneumatic dilatation; Heller myotomy
• Scleroderma: Elevation of head end of bed; H-2
receptor antagonists; metoclopramide
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from conditions which can simulate
primary/secondary esophageal motility disorders
• Correlate: Clinical/radiographic/manometric findings
• Check family history of collagen vascular disorders
Image Interpretation Pearls
• Achalasia: Dilated esophagus, absence of primary
peristalsis & "bird-beak" deformity of distal esophagus
• Scleroderma: Mild-moderate dilatation of esophagus
with distal fusiform stricture + I or absent peristalsis
• DES: Disrupted primary peristalsis; "corkscrew" or
"rosary bead" pattern of esophagus
• Presbyesophagus: Nonperistaltic contractions in an
elderly person
I SELECTED REFERENCES
1. Adler DG et al: Primary esophageal motility disorders.
Mayo CUn Proc. 76(2):195-200, 2001
2. Coggins CA et al: Wide-mouthed sacculations in the
esophagus: a radiographic finding in scleroderma. AJRAm J
Roentgenol. 176(4):953-4,2001
3. Woodfield CA et al: Diagnosis of primary versus secondary
achalasia: reassessment of clinical and radiographic criteria.
AJRAmJ Roentgenol. 175(3):727-31,2000
4. 5chima W et al: Radiographic detection of achalasia:
diagnostic accuracy of videofluoroscopy. CUn Radiol.
53(5):372-5,1998
s. Ott DJ: Esophageal motility disorders. Semin Roentgenol.
29(4):321-3'1, 1994
6. Schima W et al: Esophageal motor disorders:
videofluoroscopic and manometric evaluation--prospective
study in 88 symptomatic patients. Radiology.
185(2):487-91, 1992
Levine MS et al: Update on esophageal radiology. AJR 155:
7.
933-41, 1990
8. Ott DJ et al: Radiologic evaluation of esophageal motility:
results in 170 patients with chest pain. AJRAm J
Roentgenol. 155(5):983-5, 1990
9. Ott DJ et al: Esophageal motility: assessment with
synchronous video tape fluoroscopy and manometry.
Radiology. 173(2):419-22, 1989
10. Chen YM et al: Diffuse esophageal spasm: radiographic and
manometric correlation. Radiology. 170(3 Pt 1):807-10,
1989
11. Ott DJ et al: Esophageal radiography and manometry:
correlation in 172 patients with dysphagia. AJRAm J
Roentgenol. 149(2):307-11, 1987
 ESOPHAGEAL MOTILITY DISTURBANCES
I IMAGE GALLERY

(Left) Esophagram shows

dilated atonic esophagus and
complete spasm of LES;
achalasia. (Right)
2
Esophagram shows markedly
dilated, elongated 41
("sigmoid") esophagus due
to achalasia.

(Left) Esophagram shows

dilated, atonic esophagus
with distal stricture due to
scleroderma. (Right)
Esophagram shows markedly
dilated esophagus and short
distal stricture due to
scleroderma.

(Left) Esophagram shows

prominent tertiary,
nonpropulsive contractions
in an elderly patient
("presbyesophagus ").
(Right) Barium pill (arrow) is
retained in LESafter
esophagram and swallow of
water. Note underlying
tertiary contractions/spasms.
 SCLERODERMA, ESOPHAGUS

2
42

Upper GI shows dilated, atonic esophagus with distal Axial NEeT shows "honeycomb" lung. Interstitial
stricture. Note underlying lung disease. fibrosis due to scleroderma.

• Most common sites: Esophagus> anorectal>

ITERMINOLOGY small bowel> colon
Abbreviations and Synonyms • Most frequent cause of chronic intestinal
• Progressive systemic sclerosis (PSS) pseudo-obstruction
o Scleroderma is subclassified into two types
Definitions • Diffuse scleroderma
• Multisystemic disorder of small vessels & connective • CREST syndrome (benign course)
tissue (collagen vascular disease) of unknown etiology o Diffuse scleroderma: Diffuse cutaneous + early
visceral involvement
• Severe interstitial pulmonary fibrosis
I IMAGING FINDINGS • Tends to involve women/organ failure more likely
'" Associated with antitopoisomerase 1 antibody
General Features (anti-Scl 70)
• Best diagnostic clue: Dilated atonic esophagus with o CREST syndrome: Minimal cutaneous & late visceral
distal stricture involvement
• Other general features • C: Calcinosis of skin
o Multisystemic disorder with immunologic & • R: Raynaud phenomenon
inflammatory changes • E: Esophageal dysmotility
o Characterized by: Atrophy/fibrosis/sclerosis of skin, • S: Sclerodactyly (involvement of fingers)
vessels & organs • T: Telangiectasia
o Involves skin, synovium, & parenchyma of multiple '" Associated with anticentromere antibodies
organs like
• Gastrointestinal tract (GIT), lungs, heart, kidneys Radiographic Findings
& nervous system • Fluoroscopic guided (esophagography)
o Gastrointestinal tract scleroderma o Normal peristalsis above aortic arch (striated muscle
• 3rd most common manifestation after skin in proximal 1/3)
changes & Raynaud phenomenon o Hypotonia or atony or aperistalsis: Lower 2/3
• Seen in up to 90% of patients esophagus (smooth muscle)
o Mild-moderate dilatation of esophagus

DDx: Esophageal Stricture +/- Dysmotility

Achalasia Peptic Stricture Esophageal Cancer Post Vagotomy

 SCLERODERMA, ESOPHAGUS

Key Facts
Terminology Top Differential Diagnoses
• Multisystemic disorder of small vessels & connective • Achalasia
tissue (collagen vascular disease) of unknown etiology • Peptic stricture
• Esophageal carcinoma
Imaging Findings
• Best diagnostic clue: Dilated atonic esophagus with
• Iatrogenic
2
distal stricture Pathology
• CRESTsyndrome (benign course) • Unknown; autoimmune condition with genetic 43
• C: Calcinosis of skin predisposition
• R: Raynaud phenomenon • Incidence: 14.1 cases/million
• E: Esophageal dysmotility
• S: Sclerodactyly (involvement of fingers) Diagnostic Checklist
• T: Telangiectasia • Rule out other pathologies that cause distal
• Erosions, superficial ulcers, fusiform peptic stricture esophageal stricture ± dysmotility
• Gastroesophageal reflux (70% cases) ~ 37% develop • Check for family history of collagen vascular diseases
Barrett esophagus • Mild-moderate dilatation of esophagus with distal
fusiform stricture + decreased or absent peristalsis

o Patulous lower esophageal sphincter (LES)

• Early finding of scleroderma
o Erosions, superficial ulcers, fusiform peptic stricture
• Due to reflux esophagitis
o Gastroesophageal reflux (70% cases) ~ 37% develop
Barrett esophagus
o Hiatal hernia
• Fluoroscopic guided (barium meal)
o Stomach: gastric dilatation + delayed emptying
• Fluoroscopic guided (small bowel study)
o Marked dilatation of small-bowel (particularly 2nd,
3rd parts of duodenum & jejunum)
o Pathognomonic: "Hidebound" sign
• Dilated small-bowel (jejunum) with crowded
normal circular folds
• Seen in > 60% cases of scleroderma-related
pseudo-obstruction
• Due to muscle atrophy & its uneven replacement
by collagen in longitudinal fibers + intense fibrosis
of submucosa
o Wide-mouthed sacculations (true diverticula) on
antimesenteric border
o Prolonged transit time with barium retention in
duodenum up to 24 hrs
o ± Pneumatosis intestinalis + pneumoperitoneum
o ± Transient, nonobstructive intussusceptions
• Fluoroscopic guided (barium enema)
o Sacculations on antimesenteric border (transverse &
descending colon)
o Marked dilatation (simulate Hirschsprung disease)
o Chronic phase: Complete loss of haustrations
• Simulates cathartic colon or chronic ulcerative
colitis
o Stercoral ulceration (from retained fecal material)
o ± Benign pneumoperitoneum
Imaging Recommendations
• Fluoroscopic guided esophagography
I DIFFERENTIAL DIAGNOSIS
Achalasia
• Best diagnostic clue
o "Bird-beak" deformity: Grossly dilated esophagus
with smooth beak-like tapering at lower end
o Scleroderma shows mild-moderate dilatation of
esophagus with fusiform stricture
• Absent primary peristalsis
Peptic stricture
• Concentric, smooth tapering of short distal esophageal
segment with proximal dilatation
• Distinguished from scleroderma by normal peristalsis
Esophageal carcinoma
• Asymmetric contour with abrupt proximal borders of
narrowed distal segment (rat-tail appearance)
• Mucosal irregularity, shouldering, mass effect
• Periesophageal & distal spread may be seen
• Absence of peristalsis in malignant stricture may
simulate scleroderma
• Diagnosis: Endoscopic biopsy & history
Iatrogenic
• Example: Fundoplication & vagotomy
• Tight wrap narrows esophageal lumen
• Vagotomy, scarring decrease peristalsis
I PATHOLOGY
General Features
• General path comments
o Initial smooth muscle atrophy & fragmentation
o Followed by collagen deposition & fibrosis
• Genetics
o Localized: Anticentromere antibodies associated
with HLA-DRl, 4 & 5
o Diffuse: Antitopoisomerase 1 antibodies associated
with HLA-DRS
• Etiology
 SCLERODERMA, ESOPHAGUS
o Unknowni autoimmune condition with genetic
predisposition
o May be initiated by environmental antigens like
silica & L-tryptophan
o Immunologic mechanism (delayed hypersensitivity
reaction)
• 1 Production of cytokines (TNF-(){or IL-l) ~ 1
2 collagen production
• Vascular damage & activation of fibroblasts
44 • Epidemiology
o Incidence: 14.1 cases/million
o Prevalence: 19-75/100,000 persons
• Associated abnormalities: Maybe SLE, polymyositis or
dermatomyositis
Gross Pathologic & Surgical Features
• Rubber-hose inflexibility: Lower 2/3 esophagus
• Thin & ulcerated mucosa
• Dilated gas & fluid containing small bowel loops with
sacculations
Microscopic Features
• Perivascular lymphocytic infiltrates
• Early capillary & arteriolar injury
• Atrophy + fragmentation of smooth muscle ~
collagen deposition + fibrosis
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
oEsophagus
• Dysphagia, regurgitation
• Epigastric fullness & burning pain
o Small bowel
• Bloating, abdominal pain
• Weight loss, diarrhea, anemia
o Colon
• Chronic constipation & episodes of bowel
obstruction
• Lab-data
o Increased erythrocyte sedimentation rate (ESR)
o Iron, B12 & folic acid deficiency anemias
o Increased antinuclear antibodies (ANA)
o CREST syndrome: Anticentromere antibodies
o Diffuse scleroderma
• Antitopoisomerase 1 antibody (anti-Scl 70)
Demographics
• Age: 30-50 years
• Gender: Young African-American females (>
common)i M:F = 1:3
• Ethnicity: African-Americans more than Caucasians
Natural History & Prognosis
• Complications
o Barrett esophagus ~ adenocarcinoma
o Bowel pseudoobstruction
• Prognosis
o Limited disease with ANA bodies: Good prognosis
o Diffuse disease: Poor with involvement of kidneys,
heart & lungs rather than GI tract
Treatment
• Small frequent mealsi elevation of head of the bed
• Avoid tea & coffee
• Cimetidine, ranitidine, omeprazole
• Metoclopramide, laxatives
• Patients with severe malabsorption
o Parenteral hyperalimentation
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other pathologies that cause distal esophageal
stricture ± dysmotility
• Check for family history of collagen vascular diseases
Image Interpretation Pearls
• Mild-moderate dilatation of esophagus with distal
fusiform stricture + decreased or absent peristalsis
I SELECTED REFERENCES
1. Mayes MD: Scleroderma epidemiology. Rheum Dis Clin
North Am. 29(2):239-54, 2003
2. Goldblatt F et al: Antibody-mediated gastrointestinal
dysmotility in scleroderma. Gastroenterology.
123(4):1144-50,2002
Coggins CA et al: Wide-mouthed sacculations in the
3.
esophagus: a radiographic finding in scleroderma. AJRAm J
Roentgenol. 176(4):953-4, 2001
4. Duchini A et al: Gastrointestinal hemorrhage in patients
with systemic sclerosis and CRESTsyndrome. Am J
Gastroenterol. 93(9):1453-6, 1998
5. Weston S et al: Clinical and upper gastrointestinal motility
features in systemic sclerosis and related disorders. Am J
Gastroenterol. 93(7):1085-9, 1998
6. Lock G et al: Gastrointestinal manifestations of progressive
systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
7. Young MA et al: Gastrointestinal manifestations of
scleroderma. Rheum Dis Clin North Am. 22(4):797-823,
1996
8. Ott DJ: Esophageal motility disorders. Semin Roentgenol.
29(4):321-31, 1994
9. Sjogren RW: Gastrointestinal motility disorders in
scleroderma. Arthritis Rheum. 37(9):1265-82, 1994
10. Kahan A et al: Gastrointestinal involvement in systemic
sclerosis. Clin Dermatol. 12(2):259-65, 1994
11. Levine MS et al: Update on esophageal radiology. A]R 155:
933-41, 1990
12. Marshall JB et al: Gastrointestinal manifestations of mixed
connective tissue disease. Gastroenterology. 98(5 Pt
1):1232-8, 1990
13. Rohrmann CAJr et al: Radiologic and histologic
differentiation of neuromuscular disorders of the
gastrointestinal tract: visceral myopathies, visceral
neuropathies, and progressive systemic sclerosis. A]RAm]
Roentgenol. 143(5):933-41, 1984
14. Berk RN: The radiology corner. Scleroderma of the
gastrointestinal tract. Am J Gastroenterol. 61(3):226-31,
1974
15. Horowitz AL et al: The "hide-bound" small bowel of
scleroderma: Characteristic mucosal fold pattern. AJR 119:
332-34, 1973
 SCLERODERMA, ESOPHAGUS

I IMAGE GALLERY
Typical
(Left) Two views from
air-contrast esophagram
show atonic, but not dilated
esophagus, patulous CE
2
junction. (Right)
Single-contrast esophagram 45
shows hiatal hernia,
shortening of the esophagus
and a long smooth distal
stricture.

Typical
(Left) Air-contrast
esophagram shows dilated
atonic esophagus with an
air-contrast level and a long
smooth distal stricture. Small
hiatal hernia + "short
esophagus". (Right)
Single-contrast esophagram
shows short smooth stricture
+ dilated atonic esophagus.

(Left) Air-contrast
esophagram shows markedly
dilated esophagus and distal
stricture due to scleroderma.
(Right) Single-contrast upper
CI shows dilated, atonic
esophagus with patulous
LES.Also dilated duodenum
abruptly narrowed as it
crosses spine. All due to
scleroderma.
 ESOPHAGEAL VARICES

2
46

Graphic shows dilated, tortuous, submucosal collateral Double-contrast esophagram shows tortuous, nodular
veins (varices). longitudinal "folds" typical of varices.

• Uphill varices: 1 Portal venous pressure ~ upward

ITERMINOlOGY venous flow via dilated esophageal collaterals to
Definitions superior vena cava (SVC)
• Dilated tortuous submucosal venous plexus of • Downhill varices: Obstruction of SVC ~
esophagus downward venous flow via esophageal collaterals
to portal vein & inferior vena cava (IvC)
Radiographic Findings
I IMAGING FINDINGS • Radiography
o Chest x-ray
General Features
• Retrocardiac posterior mediastinal lobulated mass
• Best diagnostic clue: Tortuous or serpiginous
• ± Mediastinal widening, abnormal
longitudinal filling defects on esophagraphy
azygoesophageal recess
• Location
• Fluoroscopic guided esophagography
o Uphill varices: Distal third or half of thoracic
o Mucosal relief views
esophagus (more common)
• Tortuous, serpiginous, longitudinal radiolucent
o Downhill varices: Upper or middle third of thoracic
filling defects in collapsed or partially collapsed
esophagus (less common)
esophagus
• Morphology: Tortuous dilated veins in long axis of
o Double-contrast study
esophagus, protruding directly beneath mucosa or in
• Multiple radiolucent filling defects etched in
periesophageal tissue
white
• Other general features o Distended views of esophagus
o Usually due to portal HTN with cirrhosis or other
• Varices may be obscured
liver diseases
o After sclerotherapy varices may appear as fixed rigid
o Idiopathic varices: Very rare in patients with no
filling defects '
portal HTN or SVC block
o Classification of esophageal varices based on CT Findings
pathophysiology • NECT
o Thickened esophageal wall, lobulated outer contour

DDx: Thickened Esophageal Folds

Varcoid Cancer Reflux Esoph. Reflux Esoph.

 ESOPHAGEAL VARICES

Key Facts
Terminology
• Dilated tortuous submucosal venous plexus of
esophagus
Imaging Findings
• Best diagnostic clue: Tortuous or serpiginous
longitudinal filling defects on esophagraphy
• Uphill varices: Distal third or half of thoracic
esophagus (more common)
• Downhill varices: Upper or middle third of thoracic
esophagus (less common)
• Multiple radiolucent filling defects etched in white
• Varices may be obscured
• Scalloped esophageal mural masses
• Homogeneous HU & enhance to same degree as
adjacent veins
Top Differential Diagnoses
• Varicoid esophageal carcinoma
• Esophagitis
• Lymphoma
Clinical Issues 2
• Asymptomatic until rupture or significant block
• Uphill varices: Hematemesis/mild bleeding (melena) 47
• Downhill varices: SVC syndrome
• Clinical profile: Patient with history of cirrhosis,
portal HTN, hematemesis/melena, facial/arm swelling
• Alcoholic cirrhosis: Most prevalent cause in USA
Diagnostic Checklist
• Lack of change for thick "folds" should suggest
esophagitis or cancer rather than varices

o Scalloped esophageal mural masses

o Uni-/bilateral soft tissue masses (paraesophageal
I DIFFERENTIAL DIAGNOSIS
varices) Varicoid esophageal carcinoma
• CECT • May simulate varices, especially "downhill"
o Well-defined round, tubular, or smooth serpentine • Varicoid carcinoma
structures o Rigid, fixed appearance; abrupt demarcation;
o Homogeneous HU & enhance to same degree as well-defined borders
adjacent veins o Produce thickened, tortuous folds in esophagus due
o Location to submucosal spread of tumor
• Esophageal, coronary ± paraumbilical o Mostly squamous cell or adenocarcinomas
• Abdominal wall, perisplenic, perigastric, • Varices tend to change in size & shape with peristalsis,
paraesophageal, retroperitoneal, omental, respiration & Valsalva maneuvers
mesenteric • Clinically varicoid may cause dysphagia, whereas this
MR Findings symptom rarely occurs in patients with varices
• Diagnosis: Imaging & endoscopic biopsy
• Tl &T2WI
o Multiple areas of flow void Esophagitis
• Tl c+ • Occasionally esophagitis may mimic varices
o Portal venous phase (PVP) • Due to associated submucosal edema & inflammation
• Enhancement of varices seen which can manifest as thickened, tortuous
Ultrasonographic Findings longitudinal folds simulating esophageal varices
• Real Time: Increased esophageal wall thickness at least • Diagnosis: Endoscopy & history
5 mm with irregular wall surface Lymphoma
• Color Doppler: Hepatofugal venous flow within • Esophagus: Least common site within GI tract
esophageal wall • Accounts for only about 1% of all cases
Angiographic Findings • Usually non-Hodgkin & less commonly Hodgkin
• Conventional • Patients almost always have generalized lymphoma
o Portal venogram • Primary esophageal lymphoma seen in AIDS cases
• Uphill varices: May show cavernous • Imaging
transformation of portal vein + reversal of blood o Usually contiguous spread from gastric
flow via splenic vein ~ coronary vein ~ cardia/fundus to distal esophagus
esophageal varices o Polypoid or ulcerated mass or infiltrating stricture
o Submucosal infiltration (less common)
Imaging Recommendations • Enlarged, tortuous longitudinal folds mimicking
• Helical NE + CECT varices
• Fluoroscopic guided esophagography • Diagnosis: Endoscopy with deep esophageal biopsy
o High density barium
o Position: Prone right anterior oblique (RAG)
o Mucosal relief views I PATHOLOGY
o Anticholinergic agents; avoid swallowing
General Features
• General path comments
 ESOPHAGEAL VARICES
o Normal esophageal venous drainage
• Upper third of esophagus: Via intercostal,
bronchial & inferior thyroid veins
• Middle third: Via azygous & hemiazygous venous
systems
• Distal third: Via periesophageal plexus of veins ~
coronary vein ~ splenic vein
2 • Etiology
o Uphill varices
48 • Cirrhosis + portal HTN
• Obstruction of hepatic veins/IVC; CHF; marked
splenomegaly
• Pathogenesis: Collateral blood flow from portal
vein ~ azygos vein ~ SVC
o Downhill varices
• Obstruction of SVC distal to entry of azygos vein
• Usually due to lung cancer, lymphoma, fibrosing
mediastinitis, retrosternal goiter, thymoma
• Pathogenesis: Collateral blood flow from SVC ~
azygos vein ~ IVC or portal system
o Idiopathic varices: Exact mechanism is unknown
• Postulated as a result of congenital weakness in
venous channels of esophagus
• Epidemiology
o Incidence: 30-70% cases of cirrhosis + portal HTN
o Mortality: 20-50% of bleeding esophageal varices
• Associated abnormalities: Cirrhosis with portal HTN
Gross Pathologic & Surgical Features
• Tortuous dilated veins in long axis of esophagus
Microscopic Features
• Tortuous, serpiginous dilated veins protruding
beneath mucosa
• ± Superficial ulceration, inflammation, blood clot
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic until rupture or significant block
o Uphill varices: Hematemesis/mild bleeding (melena)
o Downhill varices: SVC syndrome
• Facial, periorbital, neck, bilateral arm swelling
• Dilated superficial veins over chest
o Lab-data: Guaiac positive stool or iron deficiency
anemia
• Clinical profile: Patient with history of cirrhosis, portal
HTN, hematemesis/melena, facial/arm swelling
Demographics
• Age: Middle & elderly age group
• Gender: M = F
Natural History & Prognosis
• Complications
o Inflammation, ulceration, hemorrhage, hematemesis
o Esophageal variceal hemorrhage
• Common cause of acute upper GI bleeding
• Alcoholic cirrhosis: Most prevalent cause in USA
• Accounts for 20-50% of all deaths from cirrhosis
• Prognosis
o Varices without bleeding
• Usually good after treatment
o Varices with massive bleeding
• Poor with & without treatment
Treatment
• Bleeding varices
o Vasopressin infusion
o Balloon tamponade (Sengstaken-Blakemore tube)
o Endoscopic sclerotherapy or variceal ligation
o Portal shunt surgery
o TIPS: Transjugular intrahepatic porto-systemic shunt
I DIAGNOSTIC CHECKLIST
Consider
• Lack of change for thick "folds" should suggest
esophagitis or cancer rather than varices
Image Interpretation Pearls
• Mucosal relief views: Tortuous, serpiginous,
longitudinal radiolucent filling defects in collapsed
esophagus
• Double-contrast study: Multiple radiolucent filling
defects etched in white
I SELECTED REFERENCES
1. Kang HK et al: Three-dimensional multi-detector row CT
portal venography in the evaluation of porto systemic
collateral vessels in liver cirrhosis. Radiographies.
22(5):1053-61, 2002
2. Matsumoto A et al: Three-dimensional portography using
multislice helical CT is clinically useful for management of
gastric fundic varices. AJRAm J Roentgenol.
176(4):899-905,2001
3. Kishimoto R et al: Esophageal varices: evaluation with
transabdominal US.Radiology. 206(3):647-50, 1998
4. Lee SJet al: Computed radiography of the chest in patients
with paraesophageal varices: diagnostic accuracy and
characteristic findings. AJRAmJ Roentgenol.
170(6):1527-31, 1998
5. Horton KMet al: Paraumbilical vein in the cirrhotic
patient: imaging with 3D CT angiography. Abdom
Imaging. 23(4):404-8, 1998
6. Cho KC et al: Varicesin portal hypertension: evaluation
with CT.Radiographies. 15(3):609-22, 1995
7. Itai Y et al: CT and MRI in detection of intrahepatic
porto systemic shunts in patients with liver cirrhosis. ]
Comput AssistTomogr. 18(5):768-73, 1994
8. Nelson RC et al: Splenic venous flow exceeding portal
venous flow at Doppler sonography: relationship to
portosystemic varices. A]RAm] Roentgenol. 161(3):563-7,
1993
9. Balthazar EJet al: CT evaluation of esophageal varices. A]R
Am J Roentgenol. 148(1):131-5, 1987
10. McCain AH et al: Varices from portal hypertension:
correlation of CT and angiography. Radiology. 154(1):63-9,
1985
11. Ishikawa T et al: Detection of paraesophageal varices by
plain films. AJRAm J Roentgenol. 144(4):701-4, 1985
 ESOPHAGEAL VARICES

I IMAGE GALLERY
Typical
(Left) Axial CECT in cirrhotic
patient shows massive
varices as tortuous enhanced
vessels in the periesophageal
region. (Right) Axial CECT in
2
cirrhotic patient shows 49
massive varices in the gastric
fundus wall.

(Left) Endoscopic image

shows tortuous blue varices
in the submucosal region of
the distal esophagus. (Right)
Endoscopic ultrasound
shows multiple anechoic
spaces with thin walls.

(Left) Esophagram shows

serpiginous submucosal
filling defects in the proximal
half of the esophagus;
downhill varices. (Right)
Catheter venacavogram
shows obstruction of
superior vena cava. The SVC
is occluded above azygous
arch. Downhill varices
(arrow) in the
periesophageal region are
part of collateral venous
drainage.
 SCHATZKI RING
2
50
Graphic shows small hiatal hernia and an annular
ring-like narrowing at the gastroesophageal juncIJon
ITERMINOlOGY
Definitions
• Annular, inflammatory, symptomatic narrowing of
normal lower esophageal mucosal or "B"ring
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Thin, web-like or annular
constriction at GE junction above hiatal hernia
• Location: Gastroesophageal (GE) junction
• Size: Less than 13 mm in diameter up to 20 mm
• Other general features
o Schatzki ring: Symptomatic narrowing of normal
lower esophageal mucosal or "B"ring
• Most likely results from reflux esophagitis
o Normal lower esophageal mucosal or "B"ring
• Most common ring-like narrowing in distal
esophagus that marks gastroesophageal junction
o Classification based on ring diameter VS symptoms
• Ring < 13 mm in diameter: Symptomatic
• Ring 13-20 mm: Occasionally symptomatic
• Ring> 20 mm in diameter: Asymptomatic
Radiographic Findings
• Fluoroscopic guided single-contrast barium study
DDx: Short Segment Narrowing at GE Junction
Peptic Stricture Esophageal Web
Esophagram shows small hiatal hernia and a ring-like
narrowing at the gastroesophageal junction.
o Thin (2-4 mm in height), web-like constriction at GE
junction
o Margins: Smooth & symmetric
o Fixed, anatomic, nondistensible, transverse ring
with constant shape
o Sliding hiatal hernia: Seen below the ring
• Schatzki ring best visualized
o Lumen above & below ring is distended beyond
caliber of ring
o With adequate distention of GE junction
o In prone: During arrested deep inspiration with
Valsalva maneuver while barium column passes
through GE junction
o 50% of rings seen on prone single-contrast are not
visualized on double-contrast
Imaging Recommendations
• Fluoroscopic guided single-contrast esophagram
• Position: Prone right anterior oblique (RAO)
o Best position to demonstrate Schatzki ring
o Best technique for distention of distal esophagus
I DIFFERENTIAL DIAGNOSIS
Annular peptic stricture
• Irregular/asymmetric/greater height than Schatzki ring
• Accounts for 15% of peptic strictures in distal
esophagus
A + BRings
 SCHATZKI RING

Key Facts
Terminology Top Differential Diagnoses
• Annular, inflammatory, symptomatic narrowing of • Annular peptic stricture
normal lower esophageal mucosal or "B"ring • Esophageal web
• Localized esophageal cancer
Imaging Findings
• Muscular or contractile or "A"ring
• Best diagnostic clue: Thin, web-like or annular 2
constriction at GE junction above hiatal hernia Diagnostic Checklist
• Size: Less than 13 mm in diameter up to 20 mm • Need deep inspiration, Valsalva, distention to 51
• Margins: Smooth & symmetric demonstrate Schatzki ring

Esophageal web Natural History & Prognosis

• Occasionally seen in distal esophagus • Complications
• Located several centimeters above GE junction o Peptic stricture: With longstanding reflex
• More proximal location differentiates from Schatzki o Perforation: Due to bolus obstruction & dilatation
• Prognosis
Localized esophageal cancer o Decrease in caliber over 5 years (in 25-33%)
• Focal constriction may resemble a Schatzki ring
• Asymmetric, irregular borders within narrowed part Treatment
• Advise to eat more slowly & chew food more carefully
Muscular or contractile or "A" ring • Recurrent dysphagia: Mechanical disruption of ring by
• Caused by active muscular contraction o Endoscopic rupture, bougienage, pneumatic dilation
• Found much less frequently in distal esophagus o Electrocautery incision, rarely surgery
• Located at superior border of esophageal vestibule
• On esophagography
o Broad, smooth area of narrowing I DIAGNOSTIC CHECKLIST
o May vanish completely with esophageal distention
o Varies in size/position due to esophageal contraction Consider
• Need deep inspiration, Valsalva, distention to
demonstrate Schatzki ring
I PATHOLOGY
General Features I SELECTED REFERENCES
• Etiology
o Gastroesophageal reflux disease, reflux esophagitis 1. Ott DJ et al: Radiographic & endoscopic sensitivity in
detecting lower esophageal mucosal ring. AJR 147: 261-5,
o Exaggeration of lower esophageal mucosal ring ~ t
1986
mucosal fold thickening + t scarring: Schatzki ring 2. Ott DJ et al: Esophagogastric region and its rings. AJR 142:
• Epidemiology: Incidence: Seen in 6-14% of population 281-7, 1984
• Associated abnormalities: GERD, hiatal hernia 3. Schatzki R: The lower esophageal ring: Long term followup
of symptomatic/asymptomatic rings. AJR90: 805-10,1963
Gross Pathologic & Surgical Features
• Annular, ring-like mucosal structure at GE junction
Microscopic Features I IMAGE GALLERY
• Squamous epithelium superiorly & columnar inferiorly

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Episodic dysphagia for solids
o Minimal dysphagia or asymptomatic
o "Steakhouse syndrome": Due to inadequately
chewed piece of meat impacted above ring
o Severe chest pain, sticking sensation
Demographics (Left) Esophagram shows small hiatal hernia and a tight (7 mm
• Age: More common in old age than young age diameter) Schatzki ring that was symptomatic (frequent dysphagia,
• Gender: M > F food "sticking"). (Right) Esophagram shows Schatzki ring with a
piece of meat impacted (arrow) above il.
 HIATAL HERNIA

2
52

Surgical classification of hiatal hernias. (See text for Esophagram shows small sliding hiatal hernia. Note
details.) Type I = sliding hiatal hernia. Types /I - IV = thickened gastric folds (arrow) in hernia continuing into
paraesophageal hernias. abdomen. "Feline" appearance of esophageal mucosa
due to reflux.

• Prominent diagonal notch may be seen on left

ITERMINOlOGY lateral and superior aspect of hiatal hernia; due to
Abbreviations and Synonyms crossing gastric sling fibers at cardiac incisura
• Hiatal hernia (HH), hiatus hernia • ± Kink or narrowing of hiatal hernia at esophageal
hiatus; extrinsic compression by diaphragm
Definitions • Esophageal peristaltic wave stops at GE junction
• Protrusion of a part of the stomach through the • Tortuous esophagus having an eccentric junction
esophageal hiatus of the diaphragm with hernia
• Often reducible in erect position
• Numerous (> 6) longitudinal gastric folds within
I IMAGING FINDINGS hiatal hernia continue through hiatus into
abdominal part of stomach
General Features • Areae gastricae pattern demonstrated within
• Best diagnostic clue: Fluoroscopy after barium meal herniated portion of fundus
showing portion of stomach in thorax • Gastric folds converging superiorly toward a point
• 2 types several centimeters above diaphragm
o Sliding (axial) hiatal hernia: Gastroesophageal (GE) • "Riding ulcers" at hiatal orifice: Repeated trauma
junction and gastric cardia pass through esophageal of gastric mucosa on ridge riding over hiatus
hiatus of diaphragm into thorax • Intra-hernial tumor: Well demarcated sessile
o Paraesophageal (rolling) hernia: Gastric fundus ± polypoid mass with eccentric wall thickening
other parts of stomach herniate into the chest o Paraesophageal hernia
• Portion of stomach anterior or lateral to
Radiographic Findings esophagus in chest
• Fluoroscopic barium esophagram & upper GI • Frequently nonreducible
o Sliding hiatal hernia • ± Gastric ulcer of lesser curvature at level of
• Lower esophageal mucosal ("B")ring observed 2 diaphragmatic hiatus
em or more above diaphragmatic hiatus • ± Entire stomach herniated into chest and
inverted or upside-down configuration

DDx: Intrathoracic Position of Gastric Fundus

Phrenic Ampulla Post Esophagectomy Epiphrenic 'Tic Epiphrenic 'Tic

 HIATAL HERNIA
Key Facts
Imaging Findings
• Best diagnostic clue: Fluoroscopy after barium meal
showing portion of stomach in thorax
• Sliding (axial) hiatal hernia: Gastroesophageal (GE)
junction and gastric cardia pass through esophageal
hiatus of diaphragm into thorax
• Paraesophageal (rolling) hernia: Gastric fundus ±
other parts of stomach herniate into the chest
• Lower esophageal mucosal ("B")ring observed 2 cm or
more above diaphragmatic hiatus
• Prominent diagonal notch may be seen on left lateral
and superior aspect of hiatal hernia; due to crossing
gastric sling fibers at cardiac incisura
Top Differential Diagnoses
• Phrenic ampulla
CT Findings
• Widening of esophageal hiatus
o Dehiscence of diaphragmatic crura (> 15 mm);
increased distance between crura and esophageal
wall
• Pseudomass
o Sliding hiatal hernia causing soft tissue mass
involving gastric cardia
o Usually filled with contrast material
o Lying within or above esophageal hiatus
• Focal fat collection in middle compartment of lower
mediastinum
o Omentum herniates through phrenicoesophageal
ligament
o May see 1 in fat surrounding distal esophagus
• CT clearly demonstrates paraesophageal hernia
through widened esophageal hiatus; often as an
incidental finding
o Visualize size, contents, orientation of herniated
stomach within lower thoracic cavity
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided barium
esophagram and upper GI studies
• Protocol advice
o Fluoroscopic-guided single contrast barium studies
(patient prone right anterior oblique)
• Obtain fully distended views in several positions,
including upright
• Film with full inspiration and Valsalva
o Sensitivity; full-column technique 100%; mucosal
relief 52%; double-contrast techniques 34%
I DIFFERENTIAL DIAGNOSIS
Phrenic ampulla
• Saccular, slightly more distensible distal segment of
esophagus that communicates with stomach
• Phrenic ampulla or vestibule corresponds to location
of lower esophageal sphincter (LES)
• Post-surgical change
• Epiphrenic diverticulum ('tic)
Pathology
• 99% of all hiatal hernias are sliding hiatal hernia
• 1% paraesophageal hernia 2
Clinical Issues
53
• Position dependent (supine worse) substernal or
epigastric burning pain, regurgitation, dysphagia,
hoarse voice
• Paraesophageal hernia may present with acute
life-threatening complications
Diagnostic Checklist
• Esophagram and upper GI series remain best test to
demonstrate and classify hiatal hernias
o 2-4 cm high pressure zone extends up from GE
junction just into thorax
o At upper end of sphincter; muscle coalescence called
A ring; B ring at lower end of sphincter at GE
junction
o Ampulla when fully distended has bulbous
configuration
Post-surgical change
• Gastric pull-up procedure: Stomach is mobilized and
brought into chest to replace resected esophagus
Epiphrenic diverticulum ('tic)
• Usually large sac-like protrusion in epiphrenic region;
tends to remain filled after most barium emptied
• From lateral esophageal wall of distal 10 cm
• Lateral chest film: May appear as soft tissue mass often
containing air-fluid level; mimics hiatal hernia
I PATHOLOGY
General Features
• Etiology
o Complex multifactorial etiology
o Acquired
• Common cause for both types of hiatal hernia
• Rupture or stretching of phrenoesophageal
membrane; progressive wear and tear caused by
constant swallowing; abdominal pressure>
thoracic (pregnancy or obesity)
• Nerve disease
• Alteration in viscoelastic properties of distal
esophagus
• Increased strength of longitudinal muscle layers
• Scarring from reflux esophagitis may cause
longitudinal esophageal shortening; sliding hiatal
hernia may be an effect rather than cause of reflux
esophagitis; pulls gastric fundus into thorax
o Family history
• Familial occurrence of sliding hiatal hernia has
been reported in more than 20 cases
• Epidemiology
 HIATAL HERNIA
o 99% of all hiatal hernias are sliding hiatal hernia
o 1% paraesophageal hernia
o Paraesophageal hernia: Sporadic pattern of incidence
in most cases
• Associated abnormalities: Diverticulosis (25%), reflux
esophagitis (25%), duodenal ulcer (20%) or gallstones
(18%)
2 Staging, Grading or Classification Criteria
54 • Surgical classification of hiatal hernia
o Type I: GE junction and gastric cardia are
intrathoracic (sliding hiatal hernia)
o Type II: GE junction intraabdominal gastric fundus
intrathoracic (paraesophageal hernia)
o Type III: Both GE junction and fundus are in chest
(paraesophageal hernia)
o Type IV: GE junction and all of stomach in chest
(paraesophageal hernia)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Sliding hiatal hernia
• Symptoms of GE reflux disease & reflux
esophagitis
• Position dependent (supine worse) substernal or
epigastric burning pain, regurgitation, dysphagia,
hoarse voice
o Paraesophageal hernia
• Often asymptomatic; incidental finding on
imaging
• Severe anemia, abdominal pain, vomiting
• Commonly have multiple other medical problems
• GE reflux disease or reflux esophagitis are rare
versus sliding hiatal hernia
• Diagnosis: Fluoroscopic-guided barium esophagram
and upper GI studies
• Endoscopy
o Cameron ulcers and erosions
• In 5.2% of patients with hiatal hernia
• Present as acute upper GI bleeding (6.3%)
• Persistent, recurrent iron deficiency anemia (8.3%)
• Pathogenesis: Mechanical trauma, ischemia, acid
mucosal injury may play role
Demographics
• Age: Prevalence increases with age
• Gender: M < F
Natural History & Prognosis
• Complications of paraesophageal hernia
o Paraesophageal hernia may present with acute
life-threatening complications
o May gradually enlarge
o Gastric volvulus: Intrathoracic upside-down
stomach
o Traction or torsion of stomach at or near level of
hiatus
• May lead to obstruction, strangulation, infarction
or perforation of intrathoracic stomach
• Rapidly deteriorating clinical condition
• Patients often undergo emergency surgery without
pre-operative barium studies
o Complications of nonsurgical treatment may be
sudden and severe
• Associated transmural esophagitis and fibrosis of
esophageal wall may cause hiatal hernia recurrence
• Prognosis
o Laparoscopic repair: Low morbidity and mortality
rates, shorter hospital stay, faster recovery, excellent
results
o Conventional open repair: Low mortality, but
significant morbidity
Treatment
• Sliding hiatal hernia
o Medical treatment and lifestyle modification;
treatment same as GE reflux disease
o Increasing use of laparoscopic fundoplication to
treat GE reflux disease
• Paraesophageal hernia
o Surgery, even without symptoms, is warranted
• Hernial sac excision, crural closure & antireflux
procedure (fundoplication or gastropexy)
o Large or symptomatic patients or patients with giant
paraesophageal hernia need surgical treatments
I DIAGNOSTIC CHECKLIST
Consider
• Esophagram and upper GI series remain best test to
demonstrate and classify hiatal hernias
I SELECTED REFERENCES
1. Insko EK et al: Benign and malignant lesions of the
stomach: evaluation of CT criteria for differentiation.
Radiology. 228(1):166-71, 2003
2. Pierre AF et al: Results of laparoscopic repair of giant
paraesophageal hernias: 200 consecutive patients. Ann
Thorac Surg. 74(6):1909-15; discussion 1915-6, 2002
3. Luketich JD et al: Laparoscopic repair of giant
paraesophageal hernia: 100 consecutive cases. Ann Surg.
232(4):608-18, 2000
4. Schauer PR et al: Comparison of laparoscopic versus open
repair of paraesophageal hernia. Am J Surg. 176(6):659-65,
1998
5. Weston AP: Hiatal hernia with cameron ulcers and
erosions. Gastrointest Endosc Clin N Am. 6(4):671-9, 1996
6. Vas W et al: Computed tomographic evaluation of
paraesophageal hernia. Gastrointest Radiol. 14(4):291-4,
1989
7. Chen YM et al: Multiphasic examination of the
esophagogastric region for strictures, rings, and hiatal
hernia: evaluation of the individual techniques.
Gastrointest Radiol. 10(4):311-6, 1985
8. Pupols A et al: Hiatal hernia causing a cardia pseudomass
on computed tomography. J Com put Assist Tomogr.
8(4):699-700, 1984
9. Cassel DM et al: Hiatus hernia causing CT gastric mass
lesions. Comput Radiol. 7(3):177-9, 1983
 HIATAL HERNIA
I IMAGE GALLERY
Typical
(Left) Type /I paraesophageal
hernia. The gastric fundus
has herniated into the chest, 2
but the GEjunction (arrow)
remains below the
diaphragm. (Right) Axial 55
CECT shows a large type /I
paraesophageal hernia
(arrow) with the fundus lying
along side the esophagus
within the thorax.

Typical
(Left) Upright film from
barium esophagram shows
non reducible type 1/1
paraesophageal, with fundus
and GEjunction above
diaphragm. (Right) Axial
CECT shows large type 1/1
paraesophageal hernia.

Typical
(Left) Axial CECT shows
herniation of stomach and
colon through a grossly
enlarged esophageal hiatus.
(Right) Fluoroscopic upper
GI series shows a type 1/1
paraesophageal hernia with
a large fungation mass within
the herniated stomach.
Adenocarcinoma.
 ZENKER DIVERTICULUM

2
56

Graphic shows a pouch-like herniation through Killian Lateral view of barium swallow shows large pouch
dehiscence in the cricopharyngeal muscle. (arrow) arising from the posterior pharyngoesophageal
junction.

o Average maximal dimension: 2.5 em

ITERMINOLOGY
Abbreviations and Synonyms
• Pharyngoesophageal diverticulum or posterior
hypopharyngeal diverticulum/outpouching
Definitions
• Mucosal herniation through an area of anatomic
weakness in the region of cricopharyngeal muscle
(Killian dehiscence)
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Lateral view: Barium-filled sac
posterior to cervical esophagus
• Location
o Location: Killian dehiscence (triangular anatomical
area of weakness)
• Midline posterior wall of pharyngoesophageal
segment just above cricopharyngeus muscle
(C5-C6 level)
• Between oblique & horizontal fibers of
cricopharyngeal muscle (most common site) or
between thyro-/cricopharyngeal muscles
• This area of weakness occurs in 1/3 of patients
• Size
oRange: 0.5-8 em
• Morphology: Posterior hypopharyngeal saccular
outpouching with neck opening above
cricopharyngeus muscle
• General features
o Posterior hypopharyngeal pulsion diverticulum
o Usually acquired rather than congenital
o Zenker diverticulum is a false diverticulum
• Presence of mucosa & submucosa
• Lack of muscle
o Almost all patients have associated hiatal hernia &
many of them have GE reflux & reflux esophagitis
o On barium studies: Any irregularity of contour of
Zenker diverticulum suggests either an
inflammatory or a neoplastic complication
Radiographic Findings
• Radiography
o Chest x-ray
• May show air-fluid level in superior mediastinum
• Fluoroscopic guided barium study
o Frontal view
• Barium-filled sac below the level of hypopharynx
• Large diverticulum: Extends inferiorly into
mediastinum
o Lateral or oblique view
• Barium-filled sac posterior to cervical esophagus

DDx: Outpouching from Proximal Esophagus

lIP
,
K-j Diverticulum Esophageal Webs Web, Epi. Bull. Pulsion 'Tic
 ZENKER DIVERTICULUM
Key Facts
Terminology
• Pharyngoesophageal diverticulum or posterior
hypopharyngeal diverticulum/outpouching
• Mucosal herniation through an area of anatomic
weakness in the region of cricopharyngeal muscle
(Killian dehiscence)
Imaging Findings
• Best diagnostic clue: Lateral view: Barium-filled sac
posterior to cervical esophagus
• Average maximal dimension: 2.5 cm
• Barium-filled sac posterior to cervical esophagus
• Neck of diverticulum with its opening into posterior
hypopharyngeal wall above cricopharyngeus muscle
• Luminal narrowing at pharyngoesophageal junction
• ± Nasopharyngeal regurgitation
• Neck of diverticulum with its opening into
posterior hypopharyngeal wall above
cricopharyngeus muscle
• Prominent or thickened cricopharyngeal muscle
• Luminal narrowing at pharyngoesophageal
junction
• ± Nasopharyngeal regurgitation
o During swallowing
• Diverticulum appears as a posterior bulging of
distal pharyngeal wall above an anteriorly
protruding pharyngoesophageal segment
(cricopharyngeal muscle)
o At rest
• Barium-filled diverticulum extends below level of
cricopharyngeal muscle & is posterior to cervical
esophagus
• Large diverticulum may protrude laterally to left
or compress cervical esophagus
o After swallowing
• Regurgitation or emptying of barium into
hypopharynx
o Pseudo-Zenker diverticulum
• Barium trapped between peristaltic wave &
prominent cricopharyngeal muscle or early
closure of upper cervical esophagus
• Does not extend posteriorly beyond contour of
cervical esophagus
• After peristaltic wave has passed, during
suspended respiration, trapped barium is cleared &
pseudo-Zenker diverticulum is not evident
Imaging Recommendations
• Fluoroscopic guided pharyngo-esophagram
• AP, lateral & oblique views
• Lateral view during suspended respiration
o To rule out pseudo-Zenkers diverticulum
I DIFFERENTIAL DIAGNOSIS
Killian-Jamieson diverticulum (K-J)
• Transient or persistent protrusions of anterolateral
cervical esophagus into Killian-Jamieson space
Top Differential Diagnoses
• Killian-Jamieson diverticulum (K-J)
• Esophageal web
• Epidermolysis bullosa dystrophica
Pathology 2
• False diverticulum
• Cricopharyngeal dysfunction (achalasia, spasm, 57
premature closure); i intraluminal pressure
Diagnostic Checklist
• Differentiate from Killian-Jamieson diverticula which
is also an outpouch at pharyngo-esophageal junction
• Lateral view: Barium-filled outpouch posterior to
cervical esophagus with neck opening above .
cricopharyngeus muscle at C5-6 level
• Also known as lateral cervical esophageal pouches or
diverticula
o Killian-Jamieson pouches
• Morphology
o Round to oval, smooth-surfaced outpouching
• Location
o Seen in anterolateral wall of cervical esophagus
(triangular area of weakness)
• Just below cricopharyngeus muscle
• Size: 3-20 mm in diameter
• Less common & smaller than Zenker diverticulum
• Less likely to cause symptoms
• Less likely to be associated with overflow aspiration or
gastroesophageal reflux than is Zenker diverticulum
• Fluoroscopic guided barium study
o Frontal views
• Appear as shallow & broad-based protrusions
• Location: Lateral upper esophageal wall
• Fill late during swallowing & empty after
swallowing
o Lateral views
• Barium-filled saccular protrusions
• Neck of diverticulum opening below level of
cricopharyngeus muscle
• Zenker diverticulum: Neck opens into posterior
hypopharyngeal wall above cricopharyngeus
muscle (C5-6 level) & sac extends inferiorly
behind cervical esophagus
Esophageal web
• Radiolucent ring in proximal cervical esophagus near
cricopharyngeus
• On imaging
o 1-2 mm wide, shelf-like filling defect along anterior
wall of cervical esophagus
o Mild, moderate or severe luminal narrowing
Epidermolysis bullosa dystrophica
• Stricture + proximal dilatation of cervical esophagus
• Cervical esophageal webs near cricopharyngeus
• High esophageal strictures or webs in children or
young adults with clinical history suggests diagnosis
 ZENKER DIVERTICULUM
I PATHOLOGY
General Features
• General path comments
o False diverticulum
• Only mucosa & submucosa are seen
2 • No muscle tissue
• Etiology
o Cricopharyngeal dysfunction (achalasia, spasm,
58
premature closure); t intraluminal pressure
o Spasm, incoordination or abnormal relaxation of
upper esophageal sphincter (achalasia)
o Other contributing factors to development of Zenker
diverticulum
• Gastroesophageal reflux
• Reflux esophagitis
• Hiatal hernia
• Esophageal spasm & achalasia
• Epidemiology: Prevalence: 0.01-0.11 %
• Associated abnormalities
o Gastroesophageal (GE) reflux
o Reflux esophagitis, hiatal hernia
Gross Pathologic & Surgical Features
• Posterior hypopharyngeal saccular outpouching
broad or narrow neck
Microscopic Features
• Mucosal & submucosal layers of hypopharynx
• Lack of muscle
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Upper esophageal dysphagia
o Regurgitation & aspiration of undigested food
o Halitosis, choking, hoarseness, neck mass
o Some patients are asymptomatic
Demographics
• Age
o Higher in elderly people
o 50% of cases seen in 7th-8th decade
• Gender: M > F
Natural History & Prognosis
• Complications
o Aspiration pneumonia (in 30% of cases)
o Bronchitis, bronchiectasis, lung abscess
o Diverticulitis, ulceration, fistula formation
o Risk of perforation during endoscopy or placement
of nasogastric tube
o Risk of carcinoma (seen in 0.3% of cases)
• Prognosis
o Usually good after surgery
o Poor prognosis: Neoplastic complication
Treatment
• Small asymptomatic diverticula: No treatment
• Asymptomatic & symptomatic large diverticula
o Surgical diverticulectomy or endoscopic repair
• Associated motor disorder: Myotomy
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from Killian-Jamieson diverticula which
is also an outpouch at pharyngo-esophageal junction
Image Interpretation Pearls
• Lateral view: Barium-filled outpouch posterior to
cervical esophagus with neck opening above
cricopharyngeus muscle at C5-6 level
I SELECTED REFERENCES
1. Postma GN: RE: endoscopic diverticulotomy of Zenker's
diverticulum: management and complications (Dysphagia
17:34-39). Dysphagia. 18(3):227; author reply 227-8,2003
2. Ibrahim 1M: Zenker diverticulum. Arch Surg. 138(1):111,
2003
3. Veenker EA et al: Cricopharyngeal spasm and Zenker's
diverticulum. Head Neck. 25(8):681-94, 2003
4. Sasaki CT et al: Association between Zenker diverticulum
and gastroesophageal reflux disease: development of a
working hypothesis. Am] Med. 115 SuppI3A:169S-171S,
2003
5. Richtsmeier WJ: Endoscopic management of Zenker
with diverticulum: the staple-assisted approach. Am J Med. 115
SuppI3A:175S-178S, 2003
6. Rubesin SE et al: Killian-Jamieson diverticula: radiographic
findings in 16 patients. AJR Am] Roentgenol. 177(1):85-9,
2001
7. Siddiq MA et al: Pharyngeal pouch (Zenker's diverticulum).
postgrad Med]. 77(910):506-11,2001
8. Sydow BD et al: Radiographic findings and complications
after surgical or endoscopic repair of Zenker's diverticulum
in 16 patients. AJR Am J Roentgenol. 177(5):1067-71, 2001
9. DeFriend DE et al: Sonographic demonstration of a
pharyngoesophageal diverticulum. J Clin Ultrasound.
28(9):485-7,2000
10. Achkar E: Zenker's diverticulum. Dig Dis. 16(3):144-51,
1998
11. Walters ON et al: Zenker's diverticulum in the elderly: a
neurologic etiology? Am Surg. 64(9):909-11, 1998
12. Bremner CG: Zenker diverticulum. Arch Surg.
133(10):1131-3, 1998
13. Chin E et al: Zenker's diverticulum. Am J Gastroenterol.
92(4):720, 1997
14. Woodruff WW et al: Non-nodal neck masses. Semin
Ultrasound CT MR. 18(3):182-204, 1997
15. Ponette E et al: Radiological aspects of Zenker's
diverticulum. Hepatogastroenterology. 39(2):115-22, 1992
16. Fulp SR et al: Manometric aspects of Zenker's diverticulum.
Hepatogastroenterology. 39(2):123-6, 1992
17. Cook IJ et al: Pharyngeal (Zenker's) diverticulum is a
disorder of upper esophageal sphincter opening.
Gastroenterology 103: 1229-35, 1992
18. Frieling T et al: Upper esophageal sphincter function in
patients with Zenker's diverticulum. Dysphagia. 3(2):90-2,
1988
19. Bowdler DA et al: Carcinoma arising in posterior
pharyngeal pulsion diverticulum (Zenker's diverticulum).
Br J Surg. 74(7):561-3, 1987
20. Zitsch RP et al: Pharyngoesophageal diverticulum
complicated by squamous cell carcinoma. Head Neck Surg.
9(5):290-4, 1987
21. Semenkovich JW et al: Barium pharyngography:
Comparison of single and double contrast. AJR 144:
715-20, 1985
 ZENKER DIVERTICULUM
I IMAGE GALLERY

(Left) Lateral esophagram

shows small Zenker
diverticulum at the C5-C6
level. (Right) Lateral view
esophagram shows large
2
diverticulum (arrow) 59
displacing and compressing
the posterior wall of the
proximal esophagus.

Typical
(Left) Frontal and oblique
views of barium esophagram
show large Zenker
diverticulum extending into
thorax with retained barium
after the bolus has passed.
(Right) Lateral view
esophagram shows a
moderate size diverticulum
displacing and compressing
the posterior wall of the
esophagus, just above the
thoracic inlet.

Variant
(Left) Frontal view from
esophagram shows large
diverticulum with retained
barium, extending into upper
thorax. (Right) Lateral view
esophagram shows unusually
large Zenker diverticulum
(arrow) that was
symptomatic with obstructed
swallowing, halitosis, and
regurgitation of undigested
food.
 TRACTION DIVERTICULUM

2
60

Graphic shows subcarinal lymph nodes that are Esophagram shows barium-filled tented outpouching
adherent to the esophageal wall resulting in a from midesophagus. Calcified subcarinal lymph nodes
diverticulum. were evident on chest radiograph.

ITERMINOLOGY Radiographic Findings

Abbreviations and Synonyms
• Esophageal saccular protrusion or outpouching
Definitions
• Outpouching of all layers of esophageal wall due to
perihilar pathology
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Barium-filled tented or triangular
shaped outpouching from mid-esophagus
• Location: Usually mid-esophagus
• Size: Varies from few millimeters to few centimeters
• Morphology: Tented or triangular configuration
• Other general features
o Classification of esophageal diverticula based on
mechanism of formation
• Traction type (true diverticula): Herniation of all
layers (mucosa, submucosa & muscularis propria)
• Pulsion type (pseudodiverticula): Mucosal,
submucosal herniation & lack of muscle
o Traction diverticulum: Acquired condition due to
subcarinal or perihilar granulomatous lymph node
pathology
• Radiography
o Chest x-ray PAview
• Calcification Ofperihilar nodes may be seen
o Lateral view
• Thickening of posterior tracheal stripe
• Videofluoroscopic esophagogram (barium studies)
o Mid-esophagus: Traction diverticulum
• Tented or triangular in shape with pointed tip
• Diverticulum tends to empty when esophagus is
collapsed (because it contains all layers)
o Whereas pulsion diverticula tend to remain filled
after most of barium is emptied (lack of muscle)
Imaging Recommendations
• Videofluoroscopic esophagram
o Frontal, lateral & oblique views
• Better demonstrated by large bolus of barium
• Better visualized during maximal distention
I DIFFERENTIAL DIAGNOSIS
Pulsion diverticulum
• Zenker diverticulum (posterior hypopharyngeal
diverticulum)
o Lateral view: Barium-filled sac opening into
posterior hypopharynx above cricopharyngeus

. ,
DDx: Outpouching from Mid-Esophagus

• Eso. Perforation Pulsion 'Tics Pulsion 'Tics Stricture + Ulcer

 TRACTION DIVERTICULUM
Key Facts
Imaging Findings • Esophageal ulcer
• Best diagnostic clue: Barium-filled tented or Pathology
triangular shaped outpouching from mid-esophagus
• Pulmonary tuberculosis, histoplasmosis, sarcoidosis
• Diverticulum tends to empty when esophagus is
collapsed (because it contains all layers) Diagnostic Checklist
Top Differential Diagnoses • Differentiate from other outpouchings from
2
mid-esophagus (in cases of mediastinitis or chest
• Pulsion diverticulum 61
pain)
• Esophageal perforation (mid-esophageal)
• Check for prior history of granulomatous diseases

• Mid & distal esophageal pulsion diverticula tend to • Prognosis: Symptomatic-good after surgery
remain filled after most of barium is emptied (lack of
muscle) Treatment
• Large symptomatic diverticula: Diverticulectomy
Esophageal perforation (mid-esophageal)
• A sealed-off leak seen as self-contained extraluminal
collection of contrast medium that communicates I DIAGNOSTIC CHECKLIST
with adjacent esophagus
• May be indistinguishable from traction diverticulum Consider
without history • Differentiate from other outpouchings from
mid-esophagus (in cases of mediastinitis or chest pain)
Esophageal ulcer • Check for prior history of granulomatous diseases
• Solitary ring-like or stellate shaped large ulcer
• Halo of edema and associated findings (e.g., stricture) Image Interpretation Pearls
differentiate from diverticulum • Traction diverticulum: Tends to empty when
esophagus is collapsed (contain all esophageal layers )

I PATHOLOGY
General Features
• Etiology
o Pulmonary tuberculosis, histoplasmosis, sarcoidosis
o Pathogenesis: Acutely inflamed, enlarged subcarinal
nodes indent and adhere to esophageal walls
o As inflammation subsides, nodes shrink and retract
the adherent esophagus
• Epidemiology
o Less common compared to pulsion type
o Common in areas of endemic histoplasmosis & TB
Gross Pathologic & Surgical Features
• Saccular outpouchings mostly in mid-esophagus
• Adherent to subcarinallymph nodes
Microscopic Features
• All layers are seen (true diverticulum)
I SELECTED REFERENCES
1. Lopez A et al: Esophagobronchial fistula caused by traction
esophageal diverticulum. Eur J Cardiothorac Surg.
23(1):128-30, 2003
2. Raziel A et al: Sarcoidosis and giant midesophageal
diverticulum. Dis Esophagus. 13(4):317-9,2000
3. Schima W et al: Association of midoesophageal diverticula
with oesophageal motor disorders. Videofluoroscopy and
manometry. Acta Radiol. 38(1):108-14, 1997
4. Savides TJ et al: Dysphagia due to mediastinal granulomas:
diagnosis with endoscopic ultrasonography.
Gastroenterology. 109(2):366-73, 1995
5. Duda M et al: Etiopathogenesis and classification of
esophageal diverticula. Int Surg. 70(4):291-5, 1985
I IMAGE GALLERY

ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Small diverticula: Usually asymptomatic
o Large diverticula: ± Dysphagia or regurgitation
Demographics
• Age: Usually seen in elderly age group
• Gender: M = F
(Left) Esophagram shows outpouching at the subcarinallevel. (Right)
Natural History & Prognosis Esophagram shows mid-esophageal diverticulum.
• Complications
o Erosion; inflammation; perforation; fistula
 PULSION DIVERTICULUM

2
62

Esophagram shows large outpouching from esophagus Esophagram shows sac-like protrusion from distal
just above diaphragm (epiphrenic). Esophagus was esophagus.
aperistaltic with narrowed GE junction (achalasia).

o Traction type (true diverticula): Herniation of all

ITERMINOLOGY layers
Abbreviations and Synonyms • Other general features
• Esophageal saccular protrusion or outpouching or o Classification of esophageal diverticula based on
pseudodiverticulum location & mechanism of formation
• Pharyngoesophageal junction: Pulsion type
Definitions (Zenker diverticulum)
• Outpouching or sac-like protrusion of one or more • Cervical esophagus (Killian-Jamieson space):
layers of esophageal wall Triangular area of weakness in anterolateral wall
(Killian-Jamieson diverticula)
• Mid-esophagus: Pulsion & traction type
I IMAGING FINDINGS • Distal esophagus (epiphrenic): Pulsion type
• Intramural pseudodiverticulosis: Small
General Features outpouchings of mucosal glands
• Best diagnostic clue: Barium-filled outpouching from o Pulsion diverticula
esophagus on esophagram • Usually acquired rather than congenital
• Location • More common & are often associated with other
o Posterior pharyngoesophageal junction (CS-6Ievel): radiographic evidence of motor dysfunction
Killian dehiscence (triangular anatomical area of
weakness) Radiographic Findings
o Mid-esophagus • Large epiphrenic pulsion diverticulum
o Distal esophageal or epiphrenic o Chest x-ray PA view: Prominent bulge along right or
• Size: Varies from few millimeters to few centimeters left border of heart
• Morphology o Lateral view
o Saccular outpouching with broad or narrow neck • Large soft tissue mass, mimicking hiatus hernia
o Pulsion type (pseudodiverticula): Mucosal & • ± Air-fluid level
submucosal herniation through muscularis propria • ± Retained foreign body or calcified enterolith
(common) • Videofluoroscopic esophagram (barium studies)
o Pharyngoesophageal junction: Zenker diverticulum

DDx: Outpouching from Distal Esophagus

~'*
. I; . -

Traction Diverticulum Hiatal Hernia Boerhaave Syndrome Eso. Perforation

 PULSION DIVERTICULUM
Key Facts
Terminology
• Esophageal saccular protrusion or outpouching or
pseudodiverticulum
Imaging Findings
• Best diagnostic clue: Barium-filled outpouching from
esophagus on esophagram
• Lateral view: Barium-filled sac opening into posterior
hypopharyngeal wall above cricopharyngeus-Zenker
• Diverticula tend to remain filled after most of barium
is emptied (due to lack of muscle in wall)
• Midesophageal: Frequently multiple of varied sizes
• Usually large barium-filled sac in epiphrenic area
• Better visualized during maximal distention
Top Differential Diagnoses
• Traction diverticula (for mid-esophageal pulsion type)
• Lateral view: Barium-filled sac opening into
posterior hypopharyngeal wall above
cricopharyngeus-Zenker
• Size: 10-80 mm
o Cervical esophagus: Anterolateral wall
(Killian-Jamieson diverticula)
• Frontal view: Round to oval, smooth-surfaced
diverticula; 3-20 mm in size
• Lateral view: Barium-filled transient or persistent
pouches anterior to cervical esophagus opening
below level of cricopharyngeus (Killian-Jamieson)
o Mid-esophagus: Pulsion diverticula
• Best seen in profile & recognized en face as ring
shadows on double contrast studies
• Barium-filled outpouchings from esophagus
• Usually smooth, rounded contour & a wide neck
• Diverticula tend to remain filled after most of
barium is emptied (due to lack of muscle in wall)
• Small diverticula (0.5-2 cm): Seen as transient
outpouchings that develop only during peristalsis
• Midesophageal: Frequently multiple of varied sizes
• Evidence of diffuse esophageal spasm/motor
dysfunction
o Distal esophagus (epiphrenic): Pulsion diverticula
• Usually large barium-filled sac in epiphrenic area
• Usually from lateral esophageal wall (distal 10 cm)
• Right side more common than left side
• Often associated with achalasia or hiatal hernia
Imaging Recommendations
• Videofluoroscopic esophagram
o Frontal, lateral & oblique views
• Better demonstration achieved by large boluses of
barium
• Better visualized during maximal distention
I DIFFERENTIAL DIAGNOSIS
Traction diverticula (for mid-esophageal
pulsion type)
• Location: Mid-esophagus
• Etiology
• Hiatal hernia (for a large epiphrenic pulsion type)
• Esophageal perforation
Pathology
• Defect in muscular wall of esophagus
• Esophageal motility disorders
• Pathogenesis: Pulsion due to i intraluminal pressure
2
63
Diagnostic Checklist
• Differentiate mid-esophageal pulsion
pseudodiverticulum from traction (true) diverticulum
• Distal esophageal (epiphrenic) diverticulum may
simulate hiatal hernia & Boerhaave syndrome
• Pulsion diverticula tend to remain filled after most of
barium is emptied (lack of muscle/contraction)
o Tuberculosis, histoplasmosis & sarcoidosis of
perihilar/subcarinal nodes
• Pathogenesis
o Due to fibrosis & scarring in periesophageal tissues
• Pathology: All layers are seen (true diverticula)
• Esophagography
o Tented or triangular configuration with pointed tip
o Usually have no neck
o Diverticula tend to empty when esophagus is
collapsed (because traction diverticula contain all
layers of esophageal wall)
o Whereas in pulsion type, diverticulum remains filled
after most of barium is emptied from esophagus by
peristalsis
• Due to lack of muscle/contraction in diverticulum
Hiatal hernia (for a large epiphrenic pulsion
type)
• About 99% of all hiatal hernias are sliding &
remaining 1% are paraesophageal
• Phren oesophageal membrane: Firm, elastic structure
surrounding GE junction
o Tethers distal esophagus to diaphragm & prevents
herniation of proximal part of stomach via
esophageal hiatus of diaphragm into chest
• Etiology: Gastroesophageal reflux disease (GERD),
reflux esophagitis
• Pathogenesis
o Progressive wear & tear of phren oesophageal
membrane
o Stretching or rupture of membrane & axial
herniation of stomach into chest
• Chest x-ray
o Posteroanterior (PA)view
• Prominent bulge along right border of heart
o Lateral view: Large retrocardiac soft tissue mass
mimicking the appearance of a epiphrenic
diverticulum
• Esophagography
o GE junction above esophageal hiatus of diaphragm
o Lower esophageal mucosal ring or Schatzki ring
demarcates anatomic location of GE junction
 PULSION DIVERTICULUM
o Sliding hiatal hernia: Diagnosed on barium study
when a mucosal ring is seen 2 em or more above
diaphragmatic hiatus
o Gastric rugae may be seen in herniated part
o May simulate a large epiphrenic pulsion
diverticulum
o Best position for optimal demonstration
2 • Prone right anterior oblique position (RAO)
o Avoid erect position: Hernia is reduced into
64 abdomen & inadequately distended
o Barium: Thin, low density suspension
Esophageal perforation
• Iatrogenic: Post instrumentation (e.g., endoscopy)
• Boerhaave syndrome: Spontaneous perforation
o Location: Lateral wall of distal esophagus just above
GE junction
o Morphology: Vertically oriented full-thickness linear
tear measuring 1-4 em in length
o Etiology: Violent vomiting/retching (alcoholic
binge, pregnancy, etc.)
• Small esophageal perforation
o Extravasation of contrast medium from lateral wall
of esophagus into adjacent mediastinum
• A sealed-off leak seen as self-contained extraluminal
collection of contrast medium that communicates
with adjacent esophagus
o Extraluminal contrast is persistent
o Wall is less distinct than epiphrenic diverticulum
I PATHOLOGY
General Features
• General path comments
o Embryology-anatomy
• Cervical esophagus: Killian-Jamieson space is an
anatomical triangular area of weakness just below
cricopharyngeal muscle
• Etiology
o Defect in muscular wall of esophagus
o Pulsion diverticula
• Chronic wear & tear forces (for most diverticula)
• Esophageal motility disorders
• Mechanical obstruction
• Risk factors: GERD/reflux esophagitis/hiatal hernia
o Pathogenesis: Pulsion due to 1 intraluminal pressure
• Epidemiology
o Pulsion (more common)
o Traction (uncommon) except in areas with endemic
histoplasmosis or tuberculosis
• Associated abnormalities: Esophageal motility
disorders may be seen
Gross Pathologic & Surgical Features
• Saccular outpouchings with broad or narrow neck
Microscopic Features
• Pulsion diverticula (pseudodiverticula)
• Mucosa & submucosa are seen; lack of muscle
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Small diverticula: Usually asymptomatic
o Large diverticula: ± Dysphagia or regurgitation
Demographics
• Age: Usually seen in elderly age group
• Gender
o Zenker diverticulum: (M > F)
o Mid-/distal esophageal diverticula (M = F)
Natural History & Prognosis
• Complications
o Erosion with bleeding; inflammation with abscess
o Perforation; fistula formation; retained foreign body
o Neoplasm (carcinoma of epiphrenic diverticula)
• Prognosis: Usually good after surgery
Treatment
• Very large symptomatic diverticula
o Surgical diverticulectomy
• Associated motor disorders: Distal myotomy
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate mid-esophageal pulsion
pseudo diverticulum from traction (true) diverticulum
• Distal esophageal (epiphrenic) diverticulum may
simulate hiatal hernia & Boerhaave syndrome
Image Interpretation Pearls
• Zenker diverticulum: Barium-filled outpouch posterior
to cervical esophagus with neck opening above
cricopharyngeus muscle at CS-6 level
• Distal esophageal epiphrenic diverticulum: Often
associated with achalasia or hiatal hernia
• Pulsion diverticula tend to remain filled after most of
barium is emptied (lack of muscle/contraction)
I SELECTED REFERENCES
1. Fasano NC et al: Epiphrenic diverticulum: clinical and
radiographic findings in 27 patients. Dysphagia.
18(1):9-15, 2003
Sasaki CT et al: Association between Zenker diverticulum
2.
and gastroesophageal reflux disease: development of a
working hypothesis. Am] Med. 115 SuppI3A:169S-171S,
2003
3. Sydow BD et al: Radiographic findings and complications
after surgical or endoscopic repair of Zenker's diverticulum
in 16 patients. A]R Am J Roentgenol. 177(5):1067-71,2001
4. Rubesin SE et al: Killian-]amieson diverticula: Radiographic
findings in 16 patients. A]R 177: 85-9, 2001
5. Schima W et al: Association of midesophageal diverticula
with esophageal motor disorders. Videofluoroscopy and
manometry. Acta Radiol. 38(1):108-14, 1997
6. Ponette E et al: Radiological aspects of Zenker's
diverticulum. Hepatogastroenterology. 39(2):115-22, 1992
7. Bruggeman LL et al: Epiphrenic diverticula. An analysis of
80 cases. Am] Roentgenol Radium Ther Nucl Med.
119(2):266-76, 1973
 PULSION DIVERTICULUM

I IMAGE GALLERY
Typical
(Left) Esophagram shows
pulsion diverticulum (arrow)
above a large
paraesophageal hernia (open
2
arrow). (Right) Esophagram
shows severe distortion and 65
dysmotility of esophagus
("corkscrew esophagus")
and a pulsion diverticulum
(arrow).

(Left) Axial NEeT shows a

large epiphrenic diverticulum
containing contrast and
retained food. (Right)
Esophagram shows large
epiphrenic diverticulum and
esophageal dysmotility with
tertiary contractions.

Typical
(Left) Esophagram shows
large right and small left
epiphrenic diverticula and
dilated esophagus with
dysmotility. (Right) Oblique
view of esophagram shows
pulsion diverticulum near
thoracic inlet.
 INTRAMURAL PSEUDODIVERTICULOSIS

2
66

Endoscopic image shows the opening of innumerable Double contrast esophagram shows innumerable tiny
pseudodiverticula, many arranged in orderly, outpouchings from the distal two thirds of the
longitudinal rows. esophagus. Bridging between adjacent pseudodivertula
creates intramural tracks (arrow).

o Neck; 1 mm or less in diameter

ITERMINOlOGY • Incomplete filling may erroneously suggest lack of
Abbreviations and Synonyms communication with esophageal lumen
• Intramural pseudodiverticulosis (IPD) o Ductal obstruction by inflammatory material or
debris may prevent barium from entering ducts
Definitions resulting in failure to visualize pseudodiverticula
• Rare benign disorder characterized by esophageal • Intramural tracks: Bridging between adjacent
diverticulosis-like outpouchings pseudodiverticula
o Caused by dilation of excretory ducts of deep o More likely to occur with diffuse form of IPD
mucous glands o May have little or no known clinical significance
o Mistaken for large flat ulcer or extramural collection
• Large, irregular extraluminal collection of barium
I IMAGING FINDINGS o Due to massive ductal dilatation or sealed-off
perforation of duct
General Features • Stricture: Of varying length; pseudodiverticula often
• Best diagnostic clue: Radiologic examination; more extend above & below level of stricture
sensitive than endoscopy o Are not always benign; should be evaluated for
• Location radiologic signs of malignancy
o Pseudodiverticula in wall of thoracic esophagus
o Diffuse (50%) or segmental involvement CT Findings
• Size • Thick esophageal wall, narrow lumen
o Outpouchings: Tiny, usually 1-4 mm • Intramural gas collections
o Intramural tracks: Average length of 1.2 cm (length • Peridiverticulitis & abscess formation
range, 0.3-7 cm) o Due to perforation (rare)
• Morphology: Multiple; flask-shaped outpouchings Imaging Recommendations
Radiographic Findings • Esophagram: Frontal; lateral; oblique views
• Outpouchings: Barium-filled; innumerable; tiny; in
longitudinal rows parallel to long axis of esophagus

DDx: Numerous Small Outpouchings

Reflux Stricture Candida Esophagitis Candida Ulcers

 INTRAMURAL PSEUDODIVERTICULOSIS
Key Facts
Terminology Top Differential Diagnoses
• Rare benign disorder characterized by esophageal • Esophagitis
diverticulosis-like outpouchings
• Caused by dilation of excretory ducts of deep mucous Diagnostic Checklist
• Periodic surveillance of patients with IPD for
glands
esophageal carcinoma may be worthwhile 2
Imaging Findings
• Pseudodiverticula in wall of thoracic esophagus 67
• Morphology: Multiple; flask-shaped outpouchings

• Prevalence of IPD significantly higher in patients with

I DIFFERENTIAL DIAGNOSIS
Esophagitis
• Multiple discrete ulcers associated with various types
of esophagitis
• Viewed en face; pseudodiverticula mistaken for ulcers
• True ulcers communicate directly with lumen
o Pseudo diverticula seem to be floating outside;
without apparent communication with lumen
• Ulcers, strictures and pseudodiverticula can occur
together
I PATHOLOGY
General Features
• Etiology
o Its etiology & pathogenesis are largely unknown
o Inflammation, resulting in periductal fibrosis &
compression of duct orifices, may be causative factor
• Epidemiology: Rare condition; diagnosed in less than
1% of patients undergoing esophagography
• Associated abnormalities
o Chronic esophagitis; gastroesophageal reflux
o Candidiasis, motility disorders
o Esophageal web formation; may be under-reported
o Chronic alcoholism (15%), diabetes mellitus (20%)
esophageal carcinoma
o Association implies increased risk of esophageal
carcinoma in patients with IPD
Treatment
• Strictures: Balloon or bougie dilatation
I DIAGNOSTIC CHECKLIST
Consider
• Periodic surveillance of patients with IPD for
esophageal carcinoma may be worthwhile
o Endoscopic; endosonographic; histological
examination may be required to exclude malignancy
I SELECTED REFERENCES
1. Bhattacharya S et al: Intramural pseudodiverticulosis of the
esophagus. Surg Endosc. 16(4):714-5,2002
2. Canon CL et al: Intramural tracking: a feature of
esophageal intramural pseudodiverticulosis. AJR Am J
Roentgenol. 175(2):371-4,2000
3. Plavsic BM et al: Intramural pseudodiverticulosis of the
esophagus detected on barium esophagograms: increased
prevalence in patients with esophageal carcinoma. AJR Am
J Roentgenol. 165(6):1381-5, 1995

Gross Pathologic & Surgical Features

• Numerous intramural pouches; stricture I IMAGE GALLERY

I CLINICAL ISSUES
Presentation
• Intermittent or slowly progressive dysphagia
• Associated diseases are almost always present
• Diagnosis: Esophagography; findings pathognomonic
o Endoscopy; orifices of ducts difficult to visualize
Demographics
• Age: Seen in adults; most commonly 45-65 years
• Gender: M:F = 3:2
Natural History & Prognosis (Left) Two views from esophagram show innumerable tiny
• Pseudo diverticula themselves rarely cause problems flask-shaped outpouchings within the esophageal wall. (Right) Two
• Often noted to disappear after esophageal dilation, but views from esophagram show diffuse intramural pseudodiverticulosis.
Note distal esophageal stricture.
may persist asymptomatically in some patients
• Complication: Perforation; peridiverticulitis;
mediastinitis secondary to ruptured IPD
 ESOPHAGEAL FOREIGN BODY

2
68

Esophagram shows complete obstruction of esophagus Two views from esophagram show hiatal hernia with a
with distal filling defect (ingested meat). After peptic stricture (arrow). Foreign body (walnut) is
endoscopic removal of foreign body, a Schatzki ring impacted above the stricture (open arrow).
was found.

o Lateral neck radiograph: Radiopaque FB

ITERMINOLOGY • Ingested bone fragments; linear or slightly curved
Abbreviations and Synonyms densities with well-defined margins
• Radiolucent FB; indirect evidence of mucosal
• Esophageal foreign body (FB)
trauma; localized soft tissue emphysema, lump
Definitions • Diffuse widening of retropharyngeal soft tissue
• Ingested foreign body impacted within esophagus o Chest radiograph: Coins; flat objects orient in
coronal plane if within esophagus
• Fluoroscopy
IIMAGING FINDINGS o Barium swallow; performed early; to determine
presence of FB & obstruction
General Features o Animal, fish bone: Easily obscured by barium
• Best diagnostic clue: History of ingestion followed by • May be seen as linear filling defect
dysphagia or odynophagia o Large food bolus; un chewed meat
• Location • May cause complete esophageal obstruction
o Gastroesophageal junction; area of indentation by • Polypoid filling defect with irregular meniscus
aortic arch or left main bronchus • Barium outlining superior border of food bolus
o Above pre-existing stricture; Schatzki ring; tumor • With incomplete obstruction; small amount of
o Bones tend to lodge in cervical esophagus, just barium may trickle into distal esophagus, stomach
below level of cricopharyngeus muscle (C6 level) • May erroneously suggest stricture; esophagus
• Size: Smooth objects measuring less than 1-2 em in incompletely distended below level of impaction
diameter may pass uneventfully o Barium-soaked cotton ball, marshmallow; get
• Morphology entangled in FB; helps identify nonopaque, small FB
o Radiolucent: Food, plastic, wood, medication etc. o Follow up esophagram; after removal of FB
o Radiopaque: Coin, battery, pin, nail, needle etc. • Underlying disease; motor function; lumen
o Sharp or dull; pointed or blunt; toxic or nontoxic patency; mucosal injury induced by FB
Radiographic Findings CT Findings
• Radiography • May detect faintly opaque bone chip

DDx: Filling Defect in Esophageal lumen

Eso. Cancer Eso. Cancer Candida Esoph.

 ESOPHAGEAL FOREIGN BODY
Key Facts
Terminology
• Ingested foreign body impacted within esophagus
Imaging Findings
• Radiolucent: Food, plastic, wood, medication etc.
• Radiopaque: Coin, battery, pin, nail, needle etc.
• May be seen as linear filling defect
• May cause complete esophageal obstruction
• Polypoid filling defect with irregular meniscus
• Localized soft tissue emphysema, edema, hematoma,
or abscess; may see FB penetrating esophageal wall
Imaging Recommendations
• Protocol advice
o Lateral film of neck; upright; neck well extended;
phonation; more informative than anteroposterior
o Water soluble iodinated contrast if perforation
suspected; if no leakage seen, films with barium
I DIFFERENTIAL DIAGNOSIS
Esophageal tumor
• May appear as filling defect in esophageal lumen
• Benign; submucosal; leiomyoma, lipoma, fibroma
• Malignant; esophageal cancer, metastases
Esophagitis
• Candida or viral; may cause fibrinous pseudotumor
I PATHOLOGY
General Features
• Etiology
o In adults; usually animal or fish bones or unchewed
boluses of meat
o In children; coins, toy parts, jewels, batteries,
needles, pins, "large" amounts of food
• Epidemiology: 80% of FB impactions occur in children
• Associated abnormalities: FB impaction often caused
by underlying webs, rings, or strictures (adults)
I CLINICAL ISSUES
Presentation
• High risk adults; underlying esophageal disease,
prisoners, mentally retarded, psychiatric illness
o Carpenters, dressmakers; habit of holding nails,
needles with their lips
• Transient symptoms at moment of ingestion;
retrosternal pain, cyanosis, dysphasia
o Acute dysphagia, odynophagia after choking or
gagging episode; respiratory difficulty, wheezing
• Diagnosis: History; by patient or an observer
o Children, impaired adults; unable to give accurate
history; high index of suspicion must be maintained
• May erroneously suggest stricture; esophagus
incompletely distended below level of impaction
Top Differential Diagnoses
• Esophageal tumor
• Esophagitis 2
Clinical Issues
69
• High risk adults; underlying esophageal disease,
prisoners, mentally retarded, psychiatric illness
Natural History & Prognosis
• Most FB pass spontaneously through gastrointestinal
tract without difficulty
• Sharp, pointed, elongated FB; associated with greater
risk of perforation, vascular penetration, fistula
• Recurrent FB ingestion; usually seen in mentally
retarded & psychiatric patients
• Successful removal rates as high as 92-98%, with
minimal or no complications
Treatment
• Management depends on type, size, location of FB
• Remove within 24 hoursi risk of perforation later
• Conservative; natural elimination; smooth, small FB
• Endoscopic extraction; flexible fiberoptic (87%) or
rigid esophagoscope
• Fluoroscopically guided; balloon-tipped Foley or
Fogarty catheter; Dormia-type wire basket
• Surgical removal; rarely indicated; for complications
I SELECTED REFERENCES
1. Arana A et al: Management of ingested foreign bodies in
childhood and review of the literature. Eur J Pediatr.
160(8):468-72, 2001
2. Harned RK 2nd et al: Esophageal foreign bodies: safety and
efficacy of Foley catheter extraction of coins. AJRAm J
Roentgenol. 168(2):443-6, 1997
3. Brady PG: Esophageal foreign bodies. Gastroenterol Clin
North Am. 20(4):691-701, 1991
I IMAGE GALLERY
(Left) Esophagram shows complete obstruction of mid-esophagus
with a meniscus of barium outlining piece of ingested meat, impacted
above a stricture in Barrett esophagus. (Right) Esophagram shows an
intramural mass effect in distal esophagus due to an ingested chicken
bone that was imbedded in wall, causing an inflammatory reaction.
 ESOPHAGEAL PERFORATION

2
70

Water soluble esophagram shows extravasation into Axial CECT shows mediastinal collections of gas and
mediastinum due to esophageal perforation during oral contrast medium due to esophageal perforation
laparoscopic hiatal hernia repair (fundoplicationJ. during laparoscopic fundoplication. Also note pleural
effusions and atelectasis.

!TERMINOLOGY Radiographic Findings

Abbreviations and Synonyms • Radiography
o Cervical EP: Anteroposterior, lateral films of neck
• Esophageal perforation (EP)
• Subcutaneous emphysema
• Esophageal rupture or transection
• Lateral film: Widening of prevertebral space
Definitions • Retropharyngeal abscess; mottled gas, air-fluid
• Transmural esophageal tear level
• Air may dissect along fascial planes from neck into
chest; pneumomediastinum
I IMAGING FINDINGS o Thoracic EP: Chest radiograph
• May be normal in 9-12%
General Features • Pneumomediastinum; radiolucent streaks of gas
• Best diagnostic clue along left lateral border of aortic arch; descending
o Diagnosis depends on high degree of suspicion & aorta; right lateral border of ascending aorta; heart
recognition of clinical features • V-shaped radiolucency seen through heart
• Confirmed by contrast esophagography or CT • Sympathetic left pleural effusion; atelectasis in
• Location basilar segment of left lung; with distal EP
o Most endoscopic EP; occur on posterior wall of • Pleural effusion, hydropneumothorax, localized
cervical esophagus; level of cricopharyngeus muscle pneumonitis; due to esophageal-pleural fistula
o Thoracic EP; at or near gastroesophageal junction • Hydropneumothorax; 75% on left; 5% on right;
• Areas of anatomic narrowing; site of extrinsic 20% bilateral
compression by aortic arch or left main bronchus • Hydrothorax; usually unilateral; right sided with
• At or above benign or malignant strictures; with upper or mid EP; left-sided with distal EP
biopsies or dilatation procedures • EP of intra-abdominal segment of distal
• Site of ruptured anastomosis; after esophageal esophagus; rare; below diaphragmatic hiatus
surgery • Abdominal plain film: Pneumoperitoneum
• Gas in lesser sac or retroperitoneum
• Fluoroscopy

DDx: "Extraluminal" Contrast Material

Pulsion Oivertic. Boerhaave Syndrome Heller Myotomy Boerhaave Syndome

 ESOPHAGEAL PERFORATION
Key Facts
Terminology
• Transmural esophageal tear
Imaging Findings
• Diagnosis depends on high degree of suspicion &
recognition of clinical features
• Confirmed by contrast esophagography or CT
• Subcutaneous emphysema
• Pneumomediastinum; radiolucent streaks of gas
along left lateral border of aortic arch; descending
aorta; right lateral border of ascending aorta; heart
• Pleural effusion, hydropneumothorax, localized
pneumonitis; due to esophageal-pleural fistula
• Small EP: Localized extravasation of contrast medium
from esophagus into neck or mediastinum
• Start with non-ionic water soluble contrast media
o Esophagography: To determine site & extent of EP
o Small EP: Localized extravasation of contrast
medium from esophagus into neck or mediastinum
o EP near gastroesophageal junction
• Contrast seen extravasating from left lateral aspect
of distal esophagus into adjacent mediastinum
o Sealed-off EP: Self-contained extraluminal collection
of contrast medium
• Communicating with adjacent esophagus
o Larger EP: Free extravasation of contrast medium
into mediastinum
• Extension along fascial planes superiorly or
inferiorly from site of EP
o Extension into pleural space is common
CT Findings
• CT scans optimally define extraluminal manifestations
• Extraesophageal air; most useful finding (92%)
• Extraluminal oral contrast medium
• Esophageal thickening, pleural effusion, single or
multiple abscesses; acute mediastinitis
• Mediastinal fluid collections (92%); periesophageal,
pleural, pericardial
• Esophagopleural fistula; site of communication
between pleural space & esophagus may be seen
• Limitation: Inability to locate exact site of EP
Imaging Recommendations
• Protocol advice
o Videofluoroscopic esophagography
• Start with non-ionic water soluble contrast media
• If no leak or fistula; follow with barium
• Water soluble contrast agent; may fail to detect
15-25% of thoracic EP & 50% of cervical EP
• Barium may detect small leak not visible initially
I DIFFERENTIAL DIAGNOSIS
Esophageal diverticulum
• Mucosa lined pouch
• No free mediastinal gas or inflammation
• If no leak or fistula; follow with barium
Top Differential Diagnoses
• Esophageal diverticulum
• Esophageal ulceration
•
•
Boerhaave syndrome
Post-operative
2
• Tracheobronchial aspiration 71
Pathology
• Instrumentation: Most common cause of EP
• Endoscopic procedures; responsible for 75-80% of all
EP; rigid & fiberoptic endoscopy
Clinical Issues
• Mortality rates; cervical EP: 15%; thoracic EP: 25%
• Extraluminal contrast, fluid + gas in mediastinal
pocket in EP; may mimic epiphrenic (pulsion)
diverticulum
Esophageal ulceration
• Mucosal inflammatory changes
• Deep; Barrett; large, flat ulcers; ulcerative carcinoma;
ulcers with nasogastric intubation or caustic ingestion
• Extraluminal contrast; with communication with
esophageal lumen; may mimic sealed-off perforation
Boerhaave syndrome
• Spontaneous distal EP; violent retching, vomiting,
usually after an alcoholic binge
• Extraluminal gas + contrast material in lower
mediastinum surrounding esophagus
• 1-4 cm, vertically oriented, linear tears on left lateral
wall just above gastroesophageal junction
Post-operative
• Post esophagectomy anatomy can be misinterpreted
o Near site of anastomosis; irregular contour
o Post Heller myotomy-intramural linear collection
Tracheobronchial aspiration
• Contrast material in trachea or bronchi
• Differentiate esophageal-airway fistula from aspiration
o Initial swallow in lateral projection with video
recording of hypopharynx
I PATHOLOGY
General Features
• Etiology
o Iatrogenic
• Instrumentation: Most common cause of EP
• Endoscopic procedures; responsible for 75-80% of
all EP; rigid & fiberoptic endoscopy
• Biopsy, esophageal surgery, bouginage, breakdown
of surgical anastomoses
• Pneumatic balloon dilation (2-10%)
• Nasogastric or endotracheal tubes; Celestin tubes
• Sengstaken-Blakemore tubes (35%)
 ESOPHAGEAL PERFORATION
• Esophageal obturator airways (2%)
• Flexible endoluminal prosthesis (10%)
• In infants & children; during placement of feeding
tube or suctioning of oropharyngeal secretions
o Trauma
• Penetrating injuries; knife or bullet wounds
• Blunt trauma; to chest or abdomen
2 o Foreign bodies
• Impacted animal or fish bones; sharp, pointed
72 objects; caustic agents
o Spontaneous
• Boerhaave syndrome
• 1 Intrathoracic pressure; coughing, weightlifting,
childbirth, status asthmaticus, seizures
o Neoplastic
• Esophageal carcinoma
o Idiopathic
• Epidemiology: Incidence of EP has increased as use of
endoscopic procedures has become more frequent
Gross Pathologic & Surgical Features
• Necrosis of mucosa; submucosal hemorrhage;
transmural tear
ICLINICALISSUES
Presentation
• Cervical EP: Acute onset dysphagia, neck pain, fever
o Physical examination: Subcutaneous emphysema;
crepitus in neck
• Thoracic EP: Sudden onset excruciating substernal or
lower thoracic chest pain
o Crepitus in soft tissues of anterior chest wall or neck
• Rapid onset of overwhelming sepsis; fever,
tachycardia, hypotension, shock
o Severe mediastinitis; as swallowed food, saliva,
refluxed peptic acid may enter mediastinum
• Dysphagia, increased oral secretions, respiratory
distress soon after endoscopy
o Endoscopist may be unaware that EP has occurred at
time of examination
o Patient may subsequently present with neck pain,
fever, dysphagia
• Atypical chest pain; referred to shoulder or back;
epigastric pain; crepitus may not always be present
o Clinical diagnosis may be mistaken
• Perforated peptic ulcer, myocardial infarction,
aortic dissection
• Presence of "signal" hemorrhage from gastrointestinal
tract; vascular trauma caused by perforating object
• Diagnosis: Contrast esophagography
o 90% of contrast esophagrams are positive
Demographics
• Age: Any age; EP in infants & children occurs more
frequently than reported
Natural History & Prognosis
• Most serious & rapidly fatal type of perforation in
gastrointestinal tract
• Life-threatening; associated with high morbidity &
without intervention, high mortality
• 1 Risk of EP; underlying esophageal disease,
diverticulum, cervical lordosis, osteophytes
• With dilatation procedures, EP may be immediate or
delayed for several days
• Complications: Cervical EP; retropharyngeal abscess
associated with sepsis, shock
o Thoracic EP: Mediastinitis, mediastinal abscess,
pericarditis, pneumothorax, fistula
• Outcome after EP; dependent on cause; location of
injury; presence of underlying esophageal disease
• Prognosis; directly related to interval between
perforation & initiation of treatment
o After 24 hours; 70% mortality rate for thoracic EP
o Untreated thoracic EP; mortality rate nearly 100%
• Fulminant mediastinitis
• Cervical EP has better prognosis than thoracic EP
o Mortality rates; cervical EP: 15%; thoracic EP: 25%
Treatment
• Conservative: Parenteral fluids & antibiotics
o Successful for limited esophageal injuries meeting
proper selection criteria
o Small cervical EP
o Rarely; thoracic EP heal spontaneously without
surgical intervention
• Surgical: Large cervical EP; cervical mediastinotomy &
open drainage
o Thoracic EP: Immediate thoracotomy
• Primary closure of EP
• Mediastinal drainage
o Covered metallic stents for fistulas
• Nonsurgical interventional drainage techniques;
transesophageal drainage of mediastinal abscesses
I DIAGNOSTIC CHECKLIST
Consider
• Clinical & radiographic signs of EP may be subtle
o Active investigation is needed to establish diagnosis
I SELECTED REFERENCES
1. Newt C et al: Esophageal perforation after fracture of the
cervical spine: case report and review of the literature. J
Spinal Disord Tech. 15(6):513-8,2002
2. Buecker A et al: Esophageal perforation: comparison of use
of aqueous and barium-containing contrast media.
Radiology. 202(3):683-6, 1997
3. Kim 10 et al: Perforation complicating balloon dilation of
esophageal strictures in infants and children. Radiology.
189(3):741-4, 1993
4. Hoover EL:The diagnosis and management of esophageal
perforations. J Natl Med Assoc. 83(3):246-8, 1991
5. Niezgoda JA et al: Pharyngoesophageal perforation after
blunt neck trauma. Ann Thorac Surg. 50(4):615-7, 1990
6. Scholl DG et al: Esophageal perforation following the use
of esophageal obturator airway. Radiology. 122(2):315-6,
1977
 ESOPHAGEAL PERFORATION

I IMAGE GALLERY

(Left) Esophagram following

upper endoscopy shows
esophageal "dissection" with
a mucosal flap (arrow)
2
separating the true and false
lumens. Perforation was at 73
the pharyngo-esophageal
junction. (Right) Esophagram
following endoscopy shows
esophageal" dissection".
Contrast is retained within
the false lumen (open arrow)
long after the true lumen has
cleared the contrast bolus.

(Left) Esophagram shows

contained leak (arrow)
following endoscopic biopsy
of an esophageal stricture.
(Right) Esophagram shows
contained leak (arrow)
following balloon dilatation
of esophageal stricture.

(Left) Esophagram shows left

mediastinal collection of
contrast medium (arrow)
following balloon dilatation
for achalasia. (Right) Axial
NECT shows extraluminal
oral contrast medium in the
upper abdomen foiling
placement of a nasogastric
tube that perforated the
esophago-gastric junction.
 BOERHAAVESYNDROME

2
74

Graphic shows a vertically oriented laceration of the Esophagram shows irregular extraluminal contrast and
distal esophagus, just above the hiatus and GEjunction. gas (arrow) dissecting through mediastinum. Perforation
of distal esophagus (open arrow). Immediate drainage
was required.

ITERMINOLOGY o Right side is protected by descending thoracic aorta

o Rarely, cervical/upper thoracic esophagus is involved
Definitions • Cervical: Less devastating injury
• Spontaneous distal esophageal perforation following • Mortality rate: Less than 15%
vomiting or other violent straining • Better prognosis than thoracic
Radiographic Findings
• Radiography
I IMAGING FINDINGS o Chest x-ray
General Features • Mediastinal widening, pneumomediastinum
• Left side pleural effusion or hydropneumothorax
• Best diagnostic clue: Extraluminal gas & contrast
material in lower mediastinum surrounding esophagus • Atelectasis in basilar segments of left lung
• Location: Left lateral wall of distal esophagus just • Radiolucent streaks of gas along left lateral border
above gastroesophageal (GE) junction of aortic arch & descending thoracic aorta or
along right lateral border of ascending aorta &
• Morphology: Vertically oriented full-thickness linear
heart
tear of distal esophagus, 1-4 cm in length
• ± Gas streaks (supraclavicular area)
• Other general features
o Abdomen x-ray
o Most serious & rapidly fatal type of perforation in
gastrointestinal tract • Rarely when perforation is below diaphragmatic
hiatus, show collection of gas in lesser sac or
o Sudden increase in intraluminal pressure leads to
retroperitoneum
full thickness esophageal perforation
• Fluoroscopic guided esophagography
o Most cases: Violent retching/vomiting, usually after
an alcoholic binge o Distal thoracic small esophageal perforation
o Left side of distal thoracic esophagus • Extravasation of contrast medium from left lateral
wall of distal esophagus (laterally & superiorly)
• Most vulnerable (due to lack of supporting
into adjacent mediastinum
mediastinal structures)
o Distal thoracic large esophageal perforation
• Mortality rate: 70% due to fulminant mediastinitis
if untreated

DDx: "Extraluminal" Contrast at GE Junction

Epiphrenic Divertic. Post Dilation Leak Post Surgical Leak Post Surgical Leak
 BOERHAAVESYNDROME
Key Facts
Terminology
• Spontaneous distal esophageal perforation following
vomiting or other violent straining
Imaging Findings
• Best diagnostic clue: Extraluminal gas & contrast
material in lower mediastinum surrounding
esophagus
• Location: Left lateral wall of distal esophagus just
above gastroesophageal (GE) junction
• Extravasation of contrast medium from left lateral
wall of distal esophagus (laterally & superiorly) into
adjacent mediastinum
• Extraluminal gas (highly suggestive of esophageal
perforation)
• Periesophageal, pleural, pericardial fluid collections
• Free extravasation of contrast medium into
mediastinum & extension along fascial planes
superiorly & inferiorly
• Extravasation of contrast medium into pleural
space (left more than right)
o Upper cervical esophageal perforation
• Extravasation of contrast medium into neck
o Regardless of site of perforation
• A sealed-off leak seen as self-contained
extraluminal collection of contrast medium that
communicates with adjacent esophagus
CT Findings
• Extraluminal gas (highly suggestive of esophageal
perforation)
• Periesophageal, pleural, pericardial fluid collections
• Extravasation of oral contrast material, esophageal
thickening
• Major limitation: Inability to locate exact site of
perforation
Imaging Recommendations
• Helical CT, plain chest x-ray
• Esophagography with non-ionic water-soluble contrast
agent
• Initial study with water-soluble contrast medium fails
to show a leak; examination must be repeated
immediately with barium to detect subtle leaks (more
likely to be visualized with higher density contrast)
I DIFFERENTIAL DIAGNOSIS
Mallory-Weiss syndrome
• Irregular linear mucosal tear or laceration in long axis
of esophagus
• Location
o Distal esophagus near GE junction or gastric cardia
• Size: 1-4 cm in length
• Pathology
o Involves mucosa
o Does not penetrate the wall
• Etiology
Top Differential Diagnoses
• Mallory-Weiss syndrome
• Epiphrenic (pulsion) diverticulum
• Iatrogenic injury (post instrumentation)
Pathology 2
• Rapid 1 intraluminal pressure ~ spontaneous rupture
of normal esophagus 75
Diagnostic Checklist
• Differentiate from other conditions in which
"extraluminal" contrast material is seen near
gastroesophageal junction
• Check for history of violent retching or vomiting
• Extraluminal air & contrast material around distal
esophagus particularly on left side
o Violent retching or vomiting after an alcoholic
binge or by protracted vomiting for any other
reason
o Rarely: Hiccuping, coughing, seizures, childbirth
• Pathogenesis
o Sudden, rapid increase in intraesophageal pressure
• Imaging findings
o Occasionally seen on double-contrast esophagrams
• Shallow, longitudinally oriented, linear collection
of barium in distal esophagus or near GE junction
• Indistinguishable from a linear ulcer of reflux
esophagitis in distal esophagus
• Perforated Mallory-Weiss tear is Boerhaave
Epiphrenic (pulsion) diverticulum
• Synonym: Distal esophageal pulsion diverticulum
• Pulsion diverticulum is a pseudo diverticulum
o Muscular layer is not seen
• Mucosa lined pouch from distal esophagus
• No linear mucosal tear or laceration
• No free mediastinal gas or inflammation
• Etiology: Chronic wear & tear forces, motility
disorders
• Pathogenesis: Due to pulsion caused by increased
intraluminal pressure
• Imaging findings
o Chest x-ray PA view
• Large epiphrenic diverticulum seen as prominent
bulge along right or left border of heart
o Chest x-ray lateral view
• Large soft tissue mass mimicking hiatal hernia
• ± Air-fluid level
o Videofluoroscopic esophagography
• Usually large barium-filled sac (epiphrenic area)
• Location: Lateral esophageal wall of distal 10 cm
• Side: Right side more common than left
• Wide neck; fills & empties freely into esophagus
• No linear tear or laceration
• Often associated with achalasia or hiatal hernia
Iatrogenic injury (post instrumentation)
• Endoscopic procedures account for 75% of cases
• Location: Cervical (common), thoracic esophagus
 BOERHAAVESYNDROME
• Indistinguishable from Boerhaave syndrome especially
when iatrogenic perforation occurs in left lateral wall
of distal esophagus just above GE junction
I PATHOLOGY
• Small perforation
o May heal spontaneously without surgical
in terven tion
• Small, self-contained perforation
o Managed nonoperatively with broad spectrum
antibiotics & parenteral alimentation

2 General Features

76
• Etiology
o Commonly
I DIAGNOSTIC CHECKLIST
• Seen in bulimic patients Consider
o Rare causes • Differentiate from other conditions in which
• Coughing, weightlifting, childbirth, defecation "extraluminal" contrast material is seen near
• Seizures, status asthmaticus, blunt trauma to chest gastroesophageal junction
or upper abdomen • Check for history of violent retching or vomiting
o Rapid 1 intraluminal pr~ssure ~ spontaneous
rupture of normal esophagus Image Interpretation Pearls
o Emetogenic injury of esophagus from sudden 1 in • Extraluminal air & contrast material around distal
intra-abdominal pressure + relaxation of distal esophagus particularly on left side
esophageal sphincter in presence of a moderate to • Left side pleural effusion or hydropneumothorax or
large amount of gastric contents pneumomediastinum
• Epidemiology: Accounts for 15% of total esophageal
perforation cases
I SELECTED REFERENCES
Gross Pathologic & Surgical Features
1. Rubesin SE et al: Radiologic diagnosis of gastrointestinal
• Full-thickness linear tear on left lateral wall of distal perforation. Radiol Clin North Am. v, 41(6):1095-115, 2003
esophagus just above GE junction 2. Gimenez A et al: Thoracic complications of esophageal
disorders. Radiographies. 22 Spec No:S247-58, 2002
Microscopic Features 3. Nehoda H et al: Boerhaave's Syndrome. New England
• Normal mucosa, submucosa & muscularis extern a Journal of Medicine 344: 138-9, 2001
• No inflammatory pathology 4. Chang YC et al: Right-sided pleural effusion in
spontaneous esophageal perforation. Ann Thorac
Cardiovasc Surg. 6(1):73-6, 2000
I CLINICAL ISSUES 5. Isserow JA et al: Spontaneous perforation of the cervical
esophagus after an alcoholic binge: case report. Can Assoc
Presentation Radiol J. 49(4):241-3, 1998
6. Buecker A et al: Esophageal perforation: comparison of use
• Most common signs/symptoms of aqueous and barium-containing contrast media.
o Classic triad Radiology. 202(3):683-6, 1997
• Vomiting, severe substernal chest pain 7. Lee S et al: The leaking esophagus: CT patterns of
• Subcutaneous emphysema of chest wall & neck esophageal rupture, perforation, and fistulization. Crit Rev
o Dysphagia or odynophagia Diagn Imaging. 37(6):461-90, 1996
o Rapid onset of overwhelming sepsis 8. Gupta NM et al: Spontaneous esophageal perforation:
atypical presentation. Indian J Gastroenterol. 14(1):29-30,
• Fever, tachycardia, !in blood pressure, shock
1995
• Clinical profile: Patient with history of chronic 9. Ooms HW et al: Esophageal perforation: role of
alcoholism, severe vomiting, sudden severe substernal esophagography and CT. A]R Am] Roentgenol.
chest pain & fall in blood pressure 162(4):1001-2, 1994
10. White CS et al: Esophageal perforation: CT findings. AJR
Demographics Am] Roentgenol. 160(4):767-70, 1993
• Age: Usually adults 11. Ghahremani GG: Radiologic evaluation of suspected
• Gender: M = F gastrointestinal perforations. Radiol Clin North Am.
31(6):1219-34, 1993
Natural History & Prognosis 12. Backer CL et al: Computed tomography in patients with
• Complications esophageal perforation. Chest. 98(5):1078-80, 1990
o Mediastinitis, sepsis & shock 13. Henderson]A et al: Boerhaave revisited: spontaneous
• Prognosis esophageal perforation as a diagnostic masquerader. Am]
o Large perforation Med. 86(5):559-67, 1989
14. Allen KS et al: Perforation of distal esophagus with lesser
• Without treatment after 24 hrs mortality rate 70% sac extension: CT demonstration. ] Com put Assist Tomogr.
• After immediate surgical drainage: Good 10(4):612-4, 1986
o Small perforation: Good 15. Jeffrey RB et al: Value of computed tomography in
o Small, self-contained perforation: Good detecting occult gastrointestinal perforation. ] Comput
Assist Tomogr. 7(5):825-7, 1983
Treatment
• Large perforation
o Immediate thoracotomy with surgical closure of
perforation & mediastinal drainage
 BOERHAAVESYNDROME
I IMAG E GALLERY

(Left) Axial CECT shows

bilateral pleural effusions
containing high density that 2
may be extravasated oral
contrast medium (arrow) or
pleural calcification. (Right) 77
Axial CECT (lung window)
shows food particles and gas
(arrow) in mediastinum.

Typical
(Left) Esophagram shows
irregular collection of
contrast material in
mediastinum and source of
perforation (arrow) in distal
left side of esophagus.
(Right) Esophagram shows
mediastinal collection of gas
(arrow) but no apparent leak
of contrast material.

(Left) Axial CECT shows

localized perforation of distal
left side of esophagus
(arrow) with relatively mild
periesophageal inflammatory
changes. (Right) Esophagram
shows localized perforation
of distal left side of
esophagus.
 INTRAMURAL BENIGN ESOPHAGEAL TUMORS

2
78

Single-contrast esophagram shows intramural mass in Single-contrast esophagram shows intramural mass
distal esophagus with central ulceration (arrow) due to (leiomyoma) with smooth surface, central ulcer (arrow)
leiomyoma. Incidental note of traction diverticulum + right angle or obtuse angle with wall.
(open arrow).

o Leiomyoma: ± Amorphous or punctate calcifications

ITERMINOLOGY
Definitions
• Benign mass composed of one or more tissue elements
of the esophageal wall
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Intramural mass with smooth
surface & slightly obtuse borders on barium studies
• Other general features
o Types include leiomyoma (50%); granular cell;
lipoma; hemangioma (2-3%); hamartoma
Radiographic Findings
• Fluoroscopic-guided barium studies
o Discrete mass; solitary (most common) or multiple
o Round or ovoid filling defects sharply outlined by
barium on each side (en face view)
o Overlying mucosa may ulcerate
o Smooth surface lesion, with upper & lower borders
of lesion forming right or slightly obtuse angles with
adjacent esophageal wall (profile view)
o Narrowed (tangential view) or stretched and
widened (en face view) esophageal lumen
o ± Varying degree of obstruction
o Granular cell: Distal third> middle third; 0.5-2.0 em
o Hamartoma
• Large & pedunculated; mimics fibrovascular polyp
• Diffuse, nodular mucosa comprised of tiny,
innumerable, hamartomatous polyps (hereditary)
CT Findings
• Discrete mass in wall; no signs of invasion/metastases
• Helps distinguish lipoma (fat density) and other
mediastinal masses (e.g., mediastinal cyst)
Ultrasonographic Findings
• Endoscopic ultrasound
o Leiomyoma: Hypoechoic & homogeneous mass with
sharply demarcated margins in muscular layer
o Granular cell: Hypo- to isoechoic mass in submucosa
o Lipoma: Homogeneous hyperechoic mass with
smooth outer margins in submucosa
Imaging Recommendations
• Best imaging tool: Barium studies followed by CT
I DIFFERENTIAL DIAGNOSIS
Mediastinal tumor
• Extrinsically compress or indent the esophagus
• More obtuse, gently sloping borders (in profile view)

DDx: Mass Effect with Normal Mucosa

Mediastinal Tumor Aorta + Bronchus Impacted Food

 INTRAMURAL BENIGN ESOPHAGEAL TUMORS
Key Facts
Imaging Findings • Impacted food
• Best diagnostic clue: Intramural mass with smooth Diagnostic Checklist
surface & slightly obtuse borders on barium studies • Barium esophagram and CT are complementary
• Best imaging tool: Barium studies followed by CT • Most intramural masses are benign (unlike gastric
Top Differential Diagnoses tumors)
• Calcifications suggest leiomyoma, almost never
2
• Mediastinal tumor
• Normal mediastinal structures occurs in other benign/malignant esophageal tumors 79
• Esophageal carcinoma

• Use CT if barium studies are equivocal

Treatment
Normal mediastinal structures • No treatment; if symptomatic, do surgical enucleation
• Indentation by aorta, left main bronchus, aberrant or
dilated vessels
Esophageal carcinoma
I DIAGNOSTIC CHECKLIST
• Apple core or eccentric mucosal mass Consider
Impacted food • Barium esophagram and CT are complementary
• Most intramural masses are benign (unlike gastric
• Intraluminal mass, often above stricture
tumors)
Image Interpretation Pearls
I PATHOLOGY • Calcifications suggest leiomyoma, almost never occurs
in other benign/malignant esophageal tumors
General Features
• Associated abnormalities
o Leiomyoma: Uterine or vulva leiomyomas I SELECTED REFERENCES
o Hemangioma: Osler-Weber-Rendu disease
o Hamartoma: Cowden disease 1. Levine MS: Benign tumors of the esophagus: radiologic
evaluation. Semin Thorac Cardiovasc Surg. 15(1):9-19,
Gross Pathologic & Surgical Features 2003
2. Rice TW: Benign esophageal tumors: esophagoscopy and
• Leiomyoma: Firm, round, gray/yellow, unencapsulated
endoscopic esophageal ultrasound. Semin Thorac
• Granular cell: Broad based, pinkish tan mass with Cardiovasc Surg. 15(1):20-6, 2003
normal overlying mucosa & rubbery consistency 3. Noh HM et al: CT of the esophagus: spectrum of disease
• Lipoma: Smooth, yellow, encapsulated tumor with emphasis on esophageal carcinoma. Radiographies.
composed of well-differentiated adipose tissue 15(5): 1113-34, 1995
• Hemangioma: Blue to red, nodular mass
• Hamartoma: Various elements including cartilage,
bone, adipose and fibrous tissue and muscle I IMAGE GALLERY
• Mediastinal foregut cyst
o Thin-walled nonenhancing contents
o Contents are water density (50%) to calcific

ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic (most common)
o Dysphagia, retrosternal pain, pyrosis, cough,
odynophagia, weight loss and bleeding
Demographics
(Left) Axial CECT shows water density mass (arrow) displacing distal
• Age: Leiomyoma: > 40 years of age esophagus (duplication or foregut cyst). (Right) Axial T2W MR shows
• Gender: Leiomyoma: M:F = 2: 1 duplication ("foregut") cyst (arrow) as complex fluid intensity.
Natural History & Prognosis
• Complications: Hemorrhage, obstruction, ulceration
• Prognosis: Very good
 FIBROVASCULAR POLYP
2
80
Graphic shows long, smooth, sausage-like mass arising
from proximal esophageal wall.
!TERMINOLOGY
Definitions
• Rare, benign tumor-like lesion of esophagus
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Giant, smooth, sausage-shaped,
intraluminal, expansile mass
• Location: Cervical esophagus near cricopharyngeus
• Size: Variable in size (average length of 15 cm)
• Morphology
o Usually originate in loose submucosal tissue
o Pedunculated, intraluminal, gigantic mass
o Extend inferiorly into middle or distal esophagus
o Attached to cervical esophagus by a pedicle
• Other general features
o Histologic classification of fibrovascular polyps
based on predominant mesenchymal components
• Hamartomas, fibromas, lipomas, fibrolipomas
• Fibromyxomas, fibroepithelial polyps
Radiographic Findings
• Radiography: Superior mediastinal mass ± anterior
tracheal bowing
• Fluoroscopic guided esophagography
DDx: large Intramural or Mucosal Mass
Esophageal Cancer
Barium esophagram shows expansile, sausage-shaped
mass extending from cervical to distal esophagus
(Courtesy M. Levine, MOj.
o Smooth, expansile, sausage-shaped & intraluminal
o Cervical esophageal mass ± extension distally with
prolapse into gastric fundus
o Mass with lobulated contour mimics malignancy
CT Findings
• Intraluminal mass + surrounding thin rim of contrast
• Varied HU based on degree of fat, fibrovascular tissue
o Fat density: Abundance of adipose tissue
o Heterogeneous HU: Equal amount of fat, soft tissue
o Soft tissue density: Abundance of soft tissue + no fat
MR Findings
• Fibrovascular polyp with abundant adipose tissue
o Hyperintense (TlWI); hypointense (T2WI)
Ultrasonographic Findings
• Endoscopic sonography: Fibrovascular polyp with
high fat content seen as increased echo genic lesion
I DIFFERENTIAL DIAGNOSIS
Esophageal carcinoma (polypoid tumors)
• Spindle cell carcinoma
o Usually large, polypoid intraluminal mass
simulating fibrovascular polyp
o Margins irregular & more lobulated compared to
smooth contour & less lobulated fibrovascular polyp
Esophageal Cancer Leiomyoma
 FIBROVASCULAR POLYP
Key Facts
Terminology
• Rare, benign tumor-like lesion of esophagus
Imaging Findings
• Best diagnostic clue: Giant, smooth, sausage-shaped,
intraluminal, expansile mass
• Location: Cervical esophagus near cricopharyngeus
• Size: Variable in size (average length of 15 em)
• Mass with lobulated contour mimics malignancy
o Expand or dilate esophagus without obstruction
o Rarely infiltrating or annular lesions
o Location: Middle or distal third of esophagus
• Primary malignant melanoma
o Bulky, polypoid intraluminal mass
o Expand esophageal lumen without obstruction
o Distinguished from fibrovascular polyp by its
location (lower half) due to 1 # of melanocytes
• .Squamous cell & adenocarcinomas
o May be polypoid mimicking fibrovascular polyp
o Tend to narrow lumen rather than expand it
• On imaging: Abundant fat & fibrovascular tissue
distinguishes fibrovascular polyp from other lesions
• Diagnosis: Biopsy & histology
Intramural (submucosal) benign tumors
• Leiomyoma & lipoma
o En face: Round/ovoid filling defect + sharp margins
o In profile: Smooth surface with obtuse borders
o Pedunculated lipomas in cervical esophagus mimic
fibrovascular polyp with abundant adipose tissue
o Smooth extrinsic esophageal compression
distinguishes from expansile fibrovascular polyp
I PATHOLOGY
General Features
• Etiology: Unknown
• Epidemiology: Very rare
Gross Pathologic & Surgical Features
• Giant, smooth or lobulated expansile polyp with a
discrete pedicle attached to cervical esophagus
Microscopic Features
• Varying amounts of fibrovascular & adipose tissue
covered by normal squamous epithelium
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Long-standing dysphagia
o Wheezing/inspiratory stridor (tracheal compression)
o Rarely regurgitation of mass into pharynx or mouth
Demographics
• Age: Usually seen in elderly people
• Gender: Males more than females (M > F)
• Varied HU based on degree of fat, fibrovascular tissue
Top Differential Diagnoses
• Esophageal carcinoma (polypoid tumors)
• Intramural (submucosal) benign tumors
Diagnostic Checklist 2
• Rule out other esophageal polypoidal masses
81
• Giant, smooth, sausage-shaped, expansile mass in
cervical esophagus ± distal extension
Natural History & Prognosis
• Complications
o Laryngeal occlusion, asphyxia & sudden death
o Fibrovascular polyps may bleed
o Malignant degeneration: Extremely rare
• Prognosis: Usually good after resection
Treatment
• Small fibrovascular polyps: Endoscopic resection
• Gigantic fibrovascular polyps: Surgical resection
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other esophageal polypoidal masses
Image Interpretation Pearls
• Giant, smooth, sausage-shaped, expansile mass in
cervical esophagus ± distal extension
I SELECTED REFERENCES
1. Levine MS: Benign tumors of the esophagus: radiologic
evaluation. Semin Thorac Cardiovasc Surg. 15(1):9-19,
2003
2. Ascenti G et al: Giant fibrovascular polyp of the esophagus:
CT and MR findings. Abdom Imaging. 24(2):109-10, 1999
3. Levine MS et al: Fibrovascular polyps of the esophagus:
clinical, radiographic, and pathologic findings in 16
patients. AJR Am J Roentgenol. 166(4):781-7, 1996
I IMAGE GALLERY
(Left) Esophagram shows long, smooth polyp (arrow) extending from
cervical esophagus into middle third; 67 year old woman (Courtesy
M. Levine, MO). (Right) Esophagram shows large expansile mass
filling proximal two thirds of esophagus (Courtesy M. Levine, MO).
 ESOPHAGEAL CARCINOMA

2
82

Graphic shows sessile polypoid mass with irregular Esophagram shows polypoid mass of distal esophagus
surface that infiltrates the esophageal wall and narrows with irregular surface and luminal narrowing. Squamous
the lumen. cell carcinoma.

o Squamous cell carcinoma

ITERMINOlOGY • Accounts for 50-70% of all esophageal cancers
Definitions • 1% of all cancers & 7% of all gastrointestinal
• Squamous cell carcinoma: Malignant transformation cancers
of squamous epithelium • Two major risk factors in US: Tobacco & alcohol
• Adenocarcinoma: Malignant dysplasia in columnar abuse
metaplasia (Barrett mucosa) • Human papillomavirus: Synergistic increase risk
factor in China & South Africa
o Adenocarcinoma
I IMAGING FINDINGS • Accounts for 30-50% of all esophageal cancers
• 90-100% of cases arise from Barrett mucosa
General Features • Increasing in prevalence relative to squamous cell
• Best diagnostic clue: Fixed irregular narrowing of Radiographic Findings
esophageal lumen
• Radiography .
• Location: Middle 3rd (50%), lower 3rd (30%), upper
o Chest x-ray (PA & lateral view): Advanced carcmoma
3rd (20%)
• Hilar retrohilar or retrocardiac mass
• Size • Ante~ior bowing of posterior tracheal wall
o Early esophageal cancer: Less than 3.5 cm
• Retrotracheal stripe thickening more than 3 mm
o Advanced esophageal cancer: More than 3.5 cm
• Double-contrast esophagography: En face/profile views
• Morphology o Early esophageal squamous cell cancer
o Histologically: Two main types
• Plaque-like lesions; small, sessile polyps or
• Squamous cell carcinoma depressed lesions
• Adenocarcinoma (dysplasia in Barrett mucosa)
o Early adenocarcinoma in Barrett esophagus
o Classification of advanced esophageal cancer based
• Plaque-like lesions; flat, sessile polyps
on gross pathology & radiographic findings
• Localized area of flattening/stiffening in wall of
• Infiltrating, polypoid, ulcerative, varicoid lesions
peptic stricture (common in distal 1/3)
• Other general features o Advanced esophageal squamous cell cancer
o Carcinoma is the most common tumor of esophagus

DDx: Irregular luminal Narrowing

Peptic Stricture Lung Cancer Eso. Leiomyoma Foreign Body

 ESOPHAGEAL CARCINOMA
Key Facts
Terminology
• Squamous cell carcinoma: Malignant transformation
of squamous epithelium
• Adenocarcinoma: Malignant dysplasia in columnar
metaplasia (Barrett mucosa)
Imaging Findings
• Best diagnostic clue: Fixed irregular narrowing of
esophageal lumen
• Infiltrating, polypoid, ulcerative, varicoid lesions
• Plaque-like lesions; flat, sessile polyps
• Asymmetric contour with abrupt proximal borders of
narrowed distal segment (rat-tail appearance)
Top Differential Diagnoses
• Inflammatory stricture
• Intramural primary esophageal tumor
• Infiltrating lesion (most common): Irregular
narrowing/luminal constriction (stricture) with
nodular/ulcerated mucosa
• Polypoid lesion: Lobulated/fungating intraluminal
mass
• Ulcerative lesion: Well-defined meniscoid ulcers
with a radiolucent rim of tumor surrounding ulcer
in profile view
• Varicoid lesion: Thickened, tortuous, serpiginous
longitudinal folds due to submucosal spread of
tumor, mimicking varices (nonpliable)
o Advanced adenocarcinoma in Barrett esophagus
• Radiologically indistinguishable from squamous
• Long infiltrating lesion in distal esophagus
o Stricture in advanced carcinoma
• Asymmetric contour with abrupt proximal borders
of narrowed distal segment (rat-tail appearance)
CT Findings
• CT: Staging of esophageal carcinoma
o Stage I: Localized wall thickening of 3-5 mm or
intraluminal tumor
• Not as accurate as endoscopic ultrasonography
o Stage II: Localized wall thickening more than 5 mm
& no mediastinal extension
o Stage III: Tumor extends beyond esophagus into
mediastinal tissues
• Tracheobronchial invasion: Posterior wall
indentation/bowing & tracheobronchial
displacement/compression; ± collapse of lobes
• Aortic invasion: Uncommon finding (2% of cases)
• Pericardial invasion: Based on obliteration of fat
plane/mass effect
• Mediastinal adenopathy: Discrete/inseparable
from primary tumor
o Stage IV: Extends into mediastinum & distant areas
• Liver, lungs, pleura, adrenals, kidneys & nodes
• Subdiaphragmatic adenopathy: Seen in more than
2/3 of distal cancers
MR Findings
• MR imaging is limited by motion artifacts due to long
acquisition times
• Extrinsic encasement of esophagus
• Esophageal foreign body
Pathology
• Smoking, alcohol, achalasia, lye strictures
• Adenocarcinoma: Barrett esophagus (increased risk
factors-GERD, reflux esophagitis, motility disorders)
2
• Spread: Local, lymphatic, hematogenous 83
Clinical Issues
• Clinical profile: Elderly patient with history of
difficulty in swallowing for solids & weight loss
Diagnostic Checklist
• Overlap of imaging findings with inflammatory
causes of strictures and mucosal irregularity
• Endoscopic biopsy often required
• Axial cardiac-gated & sagittal scans
o Esophageal mass indenting or displacing posterior
tracheal wall
Ultrasonographic Findings
• Real Time
o Endoscopic ultrasonography (EUS)
• Useful technique for determining extent of
esophageal wall invasion
• Malignant nodes: Hypoechoic & well-defined
• Benign nodes: Hyperechoic; indistinct borders
Nuclear Medicine Findings
• PET: 18F-Fluorodeoxyglucose Positron Emission
Tomography (FDG PET): More sensitive & superior to
CT in detecting regional & distant metastases
Imaging Recommendations
• Double-contrast esophagraphy (en face/profile views)
• Helical CT & PET for metastases
• EUS for local invasion
I DIFFERENTIAL DIAGNOSIS
Inflammatory stricture
• Reflux esophagitis
o Concentric, smooth tapering of short distal segment
• Distinguished by normal peristalsis in benign type
• Lack of peristalsis in malignant stricture
• Infectious esophagitis
o Severe or advanced Candidiasis
• Grossly irregular or "shaggy" esophagus
• Long tapered distal stricture due to scarring
o Viral: Herpes, CMV, HIV
• Multiple small punctate or giant, flat ovoid ulcers
• Usually strictures are not seen in viral esophagitis
• Caustic esophagitis
o Chronic phase
• Segmental strictures, sacculations,
pseudo diverticula
• Thread-like or filiform esophagus/stricture,
differentiates from malignant stricture
• History of caustic ingestion (strong alkali/acid)
 ESOPHAGEAL CARCINOMA
• Diagnosis: Endoscopic biopsy & history
Intramural primary esophageal tumor
• Leiomyoma
o Round or ovoid filling defect, outlined by barium
o Borders form right angles/obtuse angles with wall
o Lobulation/ulceration of mass suggests malignancy
2 • Fibrovascular polyp
o Smooth, expansile, sausage-shaped mass
84
Extrinsic encasement of esophagus
• Example: Invasion by lung cancer
• History & imaging evidence of lung cancer
Esophageal foreign body
• Impacted meat bolus appears as polypoid filling defect
• Incompletely distended esophagus below impaction
may be mistaken for a pathologic narrowing
• Esophagram after foreign body removal may show
underlying normal esophagus, Schatzki ring, stricture
I PATHOLOGY
General Features
• Genetics: Genomic instability in patients with Barrett
esophagus may increase the risk of adenocarcinoma
• Etiology
o Squamous cell carcinoma
• Smoking, alcohol, achalasia, lye strictures
• Celiac disease, head & neck tumor
• Plummer-Vinson syndrome, radiation, tylosis
o Adenocarcinoma: Barrett esophagus (increased risk
factors-GERD, reflux esophagitis, motility disorders)
o Spread: Local, lymphatic, hematogenous
• Epidemiology: Increased incidence in Turkey, Iran,
India, China, S. Africa, France, Saudi Arabia
Gross Pathologic & Surgical Features
• Infiltrating, polypoid, ulcerative or varicoid lesions
Microscopic Features
• Squamous cell atypia; columnar glands
• Adeno & squamous components
Staging, Grading or Classification Criteria
• TNM Staging
o Stage 0: Carcinoma in situ
o Stage I: Lamina propria or submucosa
o Stage IIA: Muscularis propria & adventitia
o Stage IIB: Lamina propria, submucosa, muscularis
propria & regional lymph nodes
o Stage III: Adventitia, adjacent structures, regional
lymph nodes + any other nodes
o Stage IV: All layers, adjacent structures, regional
lymph nodes + any other nodes & distant metastases
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Dysphagia (solids),
odynophagia (painful swallowing), anorexia, weight
loss, retrosternal pain
• Clinical profile: Elderly patient with history of
difficulty in swallowing for solids & weight loss
• Lab-data
o ± Hypochromic, microcytic anemia
o ± Hemoccult positive stool or decreased albumin
• Diagnosis: Endoscopic biopsy & histology
Demographics
• Age: Usually above 50 years
• Gender: M:F = 4:1
• Ethnicity: African-Americans more than Caucasians
(2:1)
Natural History & Prognosis
• Complications
o Fistula to trachea (5-10%); bronchi/pericardium
• Prognosis
o Early cancer: 5 year survival is 90%
o Advanced cancer: 5 year survival is less than 10%
Treatment
• Curative treatment
o Surgery, radiation (pre & post-op-radiation)
• Palliative treatment
o Surgery, radiation, chemotherapy
o Laser treatment, indwelling prosthesis
I DIAGNOSTIC CHECKLIST
Consider
• Overlap of imaging findings with inflammatory causes
of strictures and mucosal irregularity
• Endoscopic biopsy often required
Image Interpretation Pearls
• Irregular narrowing with nodular/ulcerated mucosa
• Asymmetric contour with abrupt proximal borders of
narrowed distal segment (rat-tail appearance)
I SELECTED REFERENCES
1. Gupta S et al: Usefulness of barium studies for
differentiating benign and malignant strictures of the
esophagus. A]R Am] Roentgenol. 180(3):737-44,2003
2. Iyer RB et al: Diagnosis, staging, and follow-up of
esophageal cancer. AJRAm. J. Roentgenol. 181: 785 - 793,
2003
3. Levine MS: Esophageal cancer. Radiologic diagnosis. Radiol
Clin North Am. 35(2):265-79, 1997
4. Levine MS et al: Carcinoma of the esophagus and
esophagogastric junction: sensitivity of radiographic
diagnosis. AJRAm] Roentgenol. 168(6):1423-6, 1997
5. Levine MS et al: Fibrovascular polyps of the esophagus:
clinical, radiographic, and pathologic findings in 16
patients. AJRAm J Roentgenol. 166(4):781-7, 1996
6. Glick SN: Barium studies in patients with Barrett's
esophagus: importance of focal areas of esophageal
deformity. A]R Am] Roentgenol. 163(1):65-7, 1994
7. Vilgrain V et al: Staging of esophageal carcinoma:
Comparison of results with endoscopic sonography and
CT. AJR 155: 277-81, 1990
 ESOPHAGEAL CARCINOMA

I IMAGE GAllERY
Typical
(Left) Two views from
esophagram show a large
fungating polypoid mass
with acute angle interface
with esophageal wall. (Right)
2
Two views from esophagram 85
demonstrate delayed
passage of barium pill,
drawing attention to a
stricture with subtle mucosal
irregularity; squamous cell
carcinoma.

(Left) Axial CECT shows

mural thickening near CE
junction due to carcinoma.
Liver metastasis (arrow).
(Right) Axial CECT shows
shows extensive
lymphadenopathy in
gastrohepatic region due to
metastases from distal
esophageal carcinoma.

Typical
(Left) Endoscopic image
shows an irregular polypoid
mass in distal esophagus;
adenocarcinoma. (Right)
Endoscopic sonography
demonstrates an intraluminal
mass (arrow) that does not
penetrate the muscularis
propria (Tl A
adenocarcinoma) .
 ESOPHAGEAL METASTASES AND LYMPHOMA
2
86
Upper CI series shows mass (arrows) in gastric fundus
extending cephalad into the esophagus (open arrows).
Castric carcinoma.
ITERMINOLOGY
Definitions
• Metastases from primary cancer of other sites
• Lymphoma: Malignant tumor of lymphocytes
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Ulcerated or polypoid mass of
gastric cardia extending into distal esophagus
• Other general features
o Esophageal metastases
• Types of spread: Direct, lymphatic, hematogenous
• Direct invasion: Most common (carcinoma of
stomach accounts for 50% of cases)
o Esophageal lymphoma
• Least common site of GIT (accounts for 1% cases)
• Usually non-Hodgkin & less commonly Hodgkin
• Secondary lymphoma (90%); primary (10%)
• Primary esophageal lymphoma seen in AIDS
Radiographic Findings
• Fluoroscopic guided double contrast barium study
o Direct invasion, gastric carcinoma: Distal esophagus
• Ulcerated or polypoid mass of cardia or fundus
extending into gastroesophageal junction
DDx: Intramural Esophageal Mass
Esophageal Cancer Esophageal Cancer
Axial CECT shows gastric carcinoma in fundus (arrows)
extending cephalad into the esophagus (open arrow).
• Irregular or smooth, tapered narrowing of distal
esophagus ± discrete mass
• Cardia obliteration + subtle esophageal extension
o Direct invasion of larynx, pharynx, thyroid, lung
cancer: Cervical or thoracic esophagus
• Smooth or slightly irregular esophageal wall + soft
tissue mass in adjacent neck or mediastinum
• Serrated, scalloped, or nodular esophageal wall ~
narrowing or obstruction
• Thyroid cancer: Expansile intraluminal mass
o Contiguous involvement by mediastinal nodes
(breast & lung cancer): Mid-esophagus
• Smooth, lobulated esophageal indentation or
ulceration at level of carina
o Hematogenous spread: Mid-esophagus> common
• Breast cancer> common: Short/eccentric strictures
• Malignant melanoma (rare): Submucosal masses
or centrally ulcerated "bull's eye" lesions
o Secondary lymphoma: Spread from stomach
• Polypoid or ulcerated lesion in cardia + irregular
narrowing of distal esophagus
o Primary intrinsic esophageal lymphoma
• Polypoid mass/stricture mimicking esophageal
cancer
• Nodular "folds" mimicking varices
• Submucosal mass simulating leiomyoma
CT Findings
• Shows primary tumor + esophageal extension
Leiomyoma Varices
 ESOPHAGEAL METASTASES AND LYMPHOMA
Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Ulcerated or polypoid mass of • Intramural benign esophageal tumors
gastric cardia extending into distal esophagus • Esophageal carcinoma
• Direct invasion: Most common (carcinoma of • Esophageal varices
stomach accounts for 50% of cases)
• Breast cancer> common: Short/eccentric strictures
Diagnostic Checklist 2
• Polypoid mass/stricture mimicking esophageal cancer • Check for history of primary cancer, biopsy required
• Overlapping radiographic features of esophageal 87
• Submucosal mass simulating leiomyoma
• CT detects mediastinal lymphadenopathy + extent metastases, lymphoma & primary carcinoma

• CT detects mediastinal lymphadenopathy + extent

Natural History & Prognosis
Ultrasonographic Findings • Complications: GI bleeding; perforation; obstruction
• Real Time • Prognosis: Usually poor
o Endoscopic ultrasonography (EUS)
• Hypoechoic mass disrupting normal wall layers Treatment
• Selective/diffusely thickened echogenic wall layers • Chemotherapy & surgical resection of lesions causing
complications like obstruction & upper GI bleeding

I DIFFERENTIAL DIAGNOSIS
I DIAGNOSTIC CHECKLIST
Intramural benign esophageal tumors
• Submucosal lesions arising within esophageal wall Consider
• Leiomyoma • .Check for history of primary cancer, biopsy required
o Round or ovoid filling defect, outlined by barium Image Interpretation Pearls
o Borders form right or obtuse angles with wall
• Overlapping radiographic features of esophageal
o Extrinsic compression & no invasion of wall
metastases, lymphoma & primary carcinoma
o Lobulation/ulceration of mass suggests malignancy
• Imaging important to suggest & stage malignancy
Esophageal carcinoma
• Polypoid, ulcerated, infiltrative types simulate
esophageal metastases or lymphoma I SELECTED REFERENCES
• Narrow distal segment + abrupt borders (rat-tail) 1. Moreto M: Diagnosis of esophagogastric tumors.
• Periesophageal & distal spread may be seen Endoscopy. 35(1):36-42, 2003
• Diagnosis: Endoscopy & biopsy 2. Coppens E et al: Primary Hodgkin's lymphoma of the
esophagus. AJR Am J Roentgenol. 180(5):1335-7, 2003
Esophageal varices 3. Holyoke ED et al: Esophageal metastases and dysphagia in
• Serpiginous, longitudinal radiolucent filling defects patients with carcinoma of the breast. J Surg Oncol
mimic submucosal infiltration of lymphoma 1:97-107, 1969

I PATHOLOGY I IMAGE GALLERY

General Features
• Associated abnormalities: Primary carcinoma;
generalized adenopathy; AIDS
Gross Pathologic & Surgical Features
• Solitary/multiple; polypoid/ulcerated masses; stricture
Microscopic Features
• Metastases: Varies based on primary cancer
• Lymphoma: Lymphoepitheliallesions

I CLINICAL ISSUES (Left) Esophagram shows smooth stricture of mid esophagus due to
direct invasion by lung cancer. (Right) Esophagram shows
Presentation innumerable submucosal nodules, ]-10 mm in size due to lymphoma
• Most common signs/symptoms: Asymptomatic, (Courtesy M. Levine, MO).
dysphagia, weight loss, hematemesis
SECTION 3: Gastroduodenal

Introd~Ktionand Overview
Gastroduodenal Anatomy and Imaging Issues 1-3-2

Congenital
Gastric Diverticulum 1-3-6
Duodenal Diverticulum 1-3-8

Inflammation
Gastritis 1-3-10
Gastric Ulcer 1-3-14
Duodenal Ulcer 1-3-18
Zollinger-Ellison Syndrome 1-3-22
Menetrier Disease 1-3-26
Caustic Gastroduodenal Injury 1-3-28

Trauma
Duodenal Hematoma and Laceration 1-3-30

Neoplasm, Benign
Gastric Polyps 1-3-32
Duodenal Polyps 1-3-36
Intramural Benign Gastric Tumors 1-3-38

Neoplasm, Malignant
Gastric Stromal Tumor 1-3-42
Gastric Carcinoma 1-3-46
Gastric Lymphoma and Metastases 1-3-50
Duodenal Carcinoma 1-3-54
Duodenal Metastases and Lymphoma 1-3-58

Treatment Related
Fundoplication Complications 1-3-60
Gastric Bypass Complications 1-3-64

Miscellaneous
Gastric Bezoar 1-3-68
Gastric Volvulus 1-3-72
Aorto-Enteric Fistula 1-3-76
 GASTRODUODENAL ANATOMY AND IMAGING ISSUES

3
2

Graphic shows liver reflected up to reveal stomach and Graphic shows axial section through gastroesophageal
its ligamentous and omental attachments. Dotted line junction, which usually lies at the level of the porta
indicates plane of section through gastroesophageal hepatis, or fissureof the ligamentum venosum.
junction.

o Complementary role in diagnosing gastritis and

TERMINOLOGY ulcers, especially complicated (e.g., perforation,
Definitions abscess)
• Gastric cardia o Primary role in diagnosing inflammatory processes
o Junction of the greater and lesser curvatures that affect the stomach secondarily (e.g.,
o Site of esophageal entry into stomach pancreatitis)
o Site of mucous secreting glands • Role of endoscopy
• Gastric fundus o Most accurate means of diagnosing gastric
o Uppermost section of stomach carcinoma and primary inflammatory conditions
o Main site of pepsinogen secreting glands o May fail to detect submucosal gastric masses
• Hepatoduodenalligament (normal overlying mucosa)
o Double layer of peritoneum; attached to pylorus and
duodenum
o Contains portal vein, hepatic artery, common bile I CLINICAL IMPLICATIONS
duct Clinical Importance
o Forms margin of epiploic foramen (of Winslow);
• Gastric mucosa and submucosa normally contain
entry into lesser sac (behind stomach)
some lymphoid tissue
o "Suspends" duodenum from underside of liver
o Chronic antigenic stimulation by H. pylori infection
• Greater omentum
can result in proliferation of mucosa-associated
o Four layers of peritoneum
lymphoid tissue (MALT)
o Passes from greater curvature as an "apron" covering
o If detected and eradicated (by antibiotics), treatment
bowel
is curative
o Connects stomach to transverse colon (gastrocolic
o Otherwise may progress to gastric B cell lymphoma
ligament)
• Duodenal wall contains mucus secreting (Brunner)
glands
o These may enlarge to simulate multiple polyps
I ANATOMY-BASED IMAGING ISSUES I (Brunner gland hypertrophy)
Imaging Approaches o May develop into a benign neoplastic mass (Brunner
• Role of fluoroscopic barium studies gland adenoma)
o Complementary to endoscopy for most cases of • Blood supply
dyspepsia and abdominal pain (e.g., peptic ulcer, o Arterial blood supply to stomach and duodenum is
tumor) quite variable; numerous collateral pathways arising
o Superior to endoscopy in evaluation of functional from branches of the celiac and superior mesenteric
abnormalities (e.g., reflux, delayed emptying, arteries make these organs resistant to ischemic
submucosal masses/infiltrative processes) injury (and difficult to control by catheter
• Role of computed tomography (CT) embolotherapy in the setting of acute hemorrhage)
o Primary role in staging primary and metastatic o Gastric veins become enlarged collaterals (varices)
tumors involving stomach commonly due to portal hypertension or splenic
vein occlusion
• Post-operative stomach
 GASTRODUODENAL ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
Gastric malignant tumors Gastric benign tumors
• Adenocarcinoma • Hyperplastic polyps
• Lymphoma • Adenoma
• GI stromal tumors (GIST) • Hamartoma
• Carcinoid • Stromal tumors
• Kaposi sarcoma • Lipoma
• Metastases • Villous adenoma
Duodenal malignant tumors Duodenal benign tumors
• Carcinoma • Villous adenoma
• Ampullary carcinoma • Stromal tumors
• GI stromal tumors (GIST) • ~ Lipoma, leiomyoma, etc.
• Carcinoid 3
• Lymphoma/metastases
• Kaposi sarcoma 3

o In many radiology practices, most fluoroscopic o Idiopathic

exams of the stomach are performed following • Varioliform erosions; top of antral rugal folds
surgical procedures that alter gastric anatomy o Aspirin; nonsteroidal anti-inflammatory drugs
o Familiarity with surgical techniques, "normal" (NSAIDs)
post-operative appearance, and complications is • Linear, multiple, greater curvature body and
essential antrum
• Most common procedures o Crohn disease
o Fundoplication • Antrum and body; contraction and stricture of
• Indication: Gastroesophageal reflux stomach
• Technique: Various types of gastric fundus wraps • Ulcers
around distal esophagus o H. pylori
• Appearance: Extrinsic mass effect in fundus • Lesser curvature or posterior wall of antrum
compressing distal esophagus o Aspirin (NSAIDs)
• Complications: Perforation, esophageal • Distal body and antrum, greater curvature
obstruction, dehiscence or slip of wrap o Gastritis
o Bariatric surgery • Variable
• Indication: Morbid obesity o Zollinger-Ellison
• Technique: Reduction of gastric size ± bypass of • Gastric and duodenal
proximal small bowel o Gastric cancer
• Appearance: Vertical banding to create small • Nodular folds, mass surrounds ulcer
pouch; surgical separation of proximal pouch with • Gastric bull's eye lesions
anastomosis to Roux limb (Roux-en-Y gastric o Metastases (especially melanoma)
bypass); extrinsic prosthetic adjustable band o Lymphoma
around fundus o Kaposi sarcoma
• Complications: Obstruction (esophagus, o Carcinoid
anastomosis, bowel); perforation (leak, abscess); o Adenocarcinoma
internal hernia; ulceration (at anastomosis) o Ectopic pancreas (greater curve, antrum)
o Partial gastrectomy
• Indications: Intractable peptic ulcer disease; Thick gastric folds
gastric tumor • Common
• Technique: BiLroth I and II (+ variations) o (Normal, nondistended stomach)
• Appearance: Distal gastrectomy with duodenal o Gastritis
anastomosis (Bil I); distal gastrectomy with o Pancreatitis
anastomosis to jejunum or Roux loop (Bil II) o Portal hypertension
• Complications: Recurrent ulcer or tumor; o Neoplastic
obstruction; perforation; gastric stasis; bezoar; o Varices
dumping syndrome; intussusception • Uncommon
o Menetrier disease
o Zollinger-Ellison syndrome
o Caustic ingestion
I CUSTOM DIFFERENTIAL DIAGNOSISI o Radiation gastritis
Gastric ulcers o Eosinophilic gastritis
• Erosions o Amyloidosis
 GASTRODUODENAL ANATOMY AND IMAGING ISSUES

3
4

Barium enema shows obstruction to retrograde flow Upper CI series shows result of a Bilroth I type distal
due to gastric carcinoma that invaded transverse colon gastrectomy. Note bezoar within stomach.
via the gastrocolic ligament.

Gastric antral narrowing Duodenal filling defects

• Common • Non-neoplastic
o Gastritis o Prolapsed antral mucosa
o Carcinoma o Flexural pseudotumor
o Lymphoma/metastases o Heterotopic gastric mucosa
• Uncommon o Brunner gland hyperplasia
o Crohn disease o Benign lymphoid hyperplasia
o Tuberculous/fungal o Choledochocele
o Syphilis o Duplication cyst
o Caustic ingestion o Intramural hematoma
o Radiation o Intramural pseudocyst
o Sarcoid and amyloid • Neoplastic
o Polyps (hyperplastic, hamartomatous, and
Gastric dilation adenomatous)
• Gastric atony o Isolated or polyposis syndromes
o Vagotomy, medications, post-op
o Diabetes
o Uremia I SELECTED REFERENCES
o Scleroderma
• Outlet obstruction 1. Horton KM et al: Current role of CT in imaging of the
stomach. Radiographies. 23(1):75-87, 2003
o Peptic ulcer
2. Insko EK et al: Benign and malignant lesions of the
o Antral stricture (Crohn, TB, etc.) stomach: evaluation of CT criteria for differentiation.
o Pancreatitis Radiology. 228(1):166-71, 2003
o Tumor 3. Levine MS: Textbook of gastrointestinal radiology:
o Bezoar Stomach and duodenum: differential diagnosis. 2nd ed.
o Prolapsed antral polyp Philadelphia, WB Saunders. pp 698-702, 2000
o Volvulus 4. Pattison CP et al: Helicobacter pylori and peptic ulcer
disease: evolution to revolution to resolution. A]R Am]
Dilated (mega-) duodenum Roentgenol. 168(6):1415-20, 1997
• Mechanical obstruction 5. Eisenberg RL: Gastrointestinal radiology: a pattern
o Pancreatitis approach. 3rd ed. Philadelphia, JB Lippincott, 1996
6. Fishman EK et al: CT of the stomach: spectrum of disease.
o Tumor
Radiographies. 16(5):1035-54, 1996
o SMA syndrome 7. Levine MS et al: The Helicobacter pylori revolution:
o Crohn disease radiologic perspective. Radiology. 195(3):593-6, 1995
o Peptic ulcer 8. Diihnert W: Radiology review manual. 4th ed.
• Scleroderma Philadelphia, Lippincott, Williams, and Wilkins. p615-721,
• Acute ileus (post-op, metabolic, drugs) 2000
• Hereditary visceral myopathy 9. Reeder MM: Reeder and Felson's gamut's in radiology. 3rd
ed. New York, Springer Verlag. 1993
• Hereditary visceral neuropathy
 GASTRODUODENAL ANATOMY AND IMAGING ISSUES

I IMAGE GALLERY

(Left) Esophagram shows

laparoscopically placed band
(arrow) around gastric
fundus; an anti-obesity
procedure. Note leak of
contrast (open arrow).
(Right) Upper GI following
Nissen fundoplication shows
intact wrap (arrow) but a
leak from the fundus or
esophagus (open arrow). 3
5

(Left) Upper GI series shows

numerous aphthoid
(varioliform) erosions
(arrows) along antral folds;
gastritis. (Right) Upper GI
series shows massive nodular
thickenings of gastric folds;
lymphoma.

(Left) Upper GI series shows

a featureless, contracted
stomach with conical,
narrowed antrum; Crohn
disease. (Right) Upper GI
series shows a
"mega-duodenum ";
scleroderma.
 GASTRIC DIVERTICULUM
3
6
Upper CI series shows air-contrast level within a gastric
diverticulum, arising near the gastroesophageal junction.
ITERMINOlOGY
Definitions
• A pouch or sac opening from the stomach
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Barium-filled diverticulum from
fundus, near gastroesophageal junction
• Other general features
o 2 types of gastric diverticula
• True gastric diverticula
• Intramural or partial gastric diverticula (false)
Radiographic Findings
• Fluoroscopic-guided barium studies
o True diverticula
• Most (75%) are juxtacardiac diverticula:
Diverticula near gastroesophageal junction, on
posterior aspect of lesser curvature of the stomach
• Usually 1-3 cm, up to 10 cm in diameter
• Barium-filled diverticulum with air-fluid level
• Pooling of barium; mimics ulceration
• Large gastric diverticulum fails to fill with gas or
barium; mimics smooth submucosal mass
• In antrum (rare); mimics ulcer craters
DDx: Mass or Pseudomass in lUQ
Adrenal Mass Adrenal Mass
Axial CECT shows air-fluid level within gastric
diverticulum (arrow) which lies medial + posterior to
gastric fundus.
o Intramural or partial gastric diverticula
• Most are prepyloric diverticula: Diverticula at
greater curvature of the distal antrum
• Tiny collection of barium extending outside the
contour of the adjacent gastric wall; mimics ulcers
• Movement of barium with peristalsis
• Heaped-up area overlying diverticulum; mimics
ectopic pancreatic rest on greater curvature
CT Findings
• Abnormal rounded soft tissue shadow
o Often in suprarenal location; mimics adrenal mass
• Air-filled, fluid-filled or contrast-filled mass
• No enhancement of contents
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided barium studies
• Protocol advice
o ]uxtacardiac diverticula are best seen in lateral views
on barium studies
o Obtain CT in supine and prone position: Air will
usually fill the diverticulum
I DIFFERENTIAL DIAGNOSIS
Adrenal mass
• CT: Diverticular contents do not enhance; adrenal
masses (except cyst) do
Splenosis Polysplenia
 GASTRIC DIVERTICULUM

Key Facts
Imaging Findings • Pancreatic mass
• Best diagnostic clue: Barium-filled diverticulum from • Splenic mass
fundus, near gastroesophageal junction Clinical Issues
• Best imaging tool: Fluoroscopic-guided barium
• Asymptomatic (most common)
studies
Diagnostic Checklist
Top Differential Diagnoses
• Often mistaken for adrenal mass on CT
• Adrenal mass
• Barium studies or CT in supine/prone position
• Abscess

• Distinguished by barium studies

Demographics 3
Abscess • Age: Any age 7
• Air- or fluid-filled mass with a thick wall • Gender: M:F = 1:1
• Distinguished by clinical history (Le., fever)
Natural History & Prognosis
Pancreatic mass • Complications (rare): Bleeding, ulceration, carcinoma
• Barium studies: Heaped-up area overlying prepyloric • Prognosis: Very good
diverticulum
Treatment
Splenic mass • No treatment needed without complications
• Splenosis, accessory spleen, polysplenia • If with complications, diverticulectomy or partial
• Distinguished by barium studies; isodensity to splenic gastrectomy can be used to resect diverticulum
tissue on all phases of CT contrast-enhancement

I DIAGNOSTIC CHECKLIST
I PATHOLOGY
Consider
General Features • Often mistaken for adrenal mass on CT
• General path comments
o 0.02% of autopsy specimens Image Interpretation Pearls
o 0.04% of upper gastrointestinal series • Barium studies or CT in supine/prone position
o True gastric diverticula: 75% of gastric diverticula
are juxtacardiac diverticula
o Intramural or partial gastric diverticula: Rare I SELECTED REFERENCES
• Etiology 1. Chasse E et al: Gastric diverticulum simulating a left
o True gastric diverticula: Congenital adrenal tumor. Surgery. 133(4):447-8, 2003
o Intramural or partial gastric diverticula: Acquired 2. Schwartz AN et al: Gastric diverticulum simulating an
• Associated with peptic ulcer disease, pancreatitis, adrenal mass: CT appearance and embryogenesis. AJRAm J
cholecystitis, malignancy or outlet obstruction Roentgenol. 146(3):553-4, 1986
3. Dickinson RJ et al: Partial gastric diverticula: radiological
Gross Pathologic & Surgical Features and endoscopic features in six patients. Gut. 27(8):954-7,
• True gastric diverticula 1986
o Pouch/sac that includes 3 normal layers of bowel
wall (Le., mucosa, submucosa/muscularis propria)
• Intramural or partial gastric diverticula [IMAGE GALLERY
o Focal invagination of mucosa & submucosa into
muscular layer of gastric wall; no muscular elements

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o True gastric diverticula
• Asymptomatic (most common)
• Vague upper abdominal pain
o Intramural or partial gastric diverticula
• Asymptomatic
• Diagnosis Axial NECT shows gastric diverticulum (arrow) adjacent to
(Left)
o Fluoroscopic-guided barium studies fundus of stomach. (Right) Axial NECT shows gastric diverticulum
(arrow) seemingly separated from stomach. Only air-fluid level allows
recognition.
 DUODENAL DIVERTICULUM

3
8

Upper GI series shows two duodenal diverticula Axial CECTshows three duodenal diverticula, two with
(arrows) as rounded outpouchings from medial side of air-contrast levels (arrows) and one with
descending duodenum, one filled with air, one filled food/particulate debris (open arrow).
with barium.

• Air within the diverticulum

ITERMINOLOGY
Definitions
• A pouch or sac opening from the duodenum
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Smooth, rounded outpouching
from medial descending duodenum
• Other general features
o 3 types of duodenal diverticula
• True diverticula
• Pseudodiverticula
• Intraluminal diverticula
Radiographic Findings
• Fluoroscopic-guided barium studies
o True diverticula
• Location: Medial (70%) descending duodenum in
periampullary region, third or fourth portion
(26%), lateral (4%) descending duodenum
• Most (75%) are juxtapapillary diverticula:
Diverticula within 2 cm of ampulla
• Intradiverticular papilla: Papilla arises within
• Multiple, smooth, rounded outpouchings
• Diverticula change configuration during study
• ± Filling defects (by food, blood clots or gas)
• ± Bizarre, multiloculated or giant diverticula
o Pseudodiverticula: Outpouching at base of bulb
o Intraluminal diverticula
• "Wind sock" appearance: Barium-filled, globular
structure of variable length, originating in second
portion of duodenum, fundus extending into
third portion and filling defect in fourth portion;
outlined by a thin, radiolucent line
• "Halo" sign: Finger-like sac separated from contrast
in adjacent duodenal lumen by a radiolucent band
• Emptied of barium; mimics pedunculated polyps
CT Findings
• True diverticula: Air-fluid level within diverticulum
• Intraluminal diverticula
o Contrast-opacified 2nd portion of duodenum
o Air-filled fourth portion of duodenum
o ± Debris within diverticulum; "halo" sign
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided barium studies
I DIFFERENTIAL DIAGNOSIS
Pseudocyst in head of pancreas
• CT or us: Simulates fluid-filled duodenal diverticulum

DDx: Cystic Mass or Pseudo mass Near Pancreatic Head

.
:~.' .P!· }.~~
Ii: w'

Pseudocyst
'4!-Z-' Cystic Tumor Mucinous Cyst /PMT
 DUODENAL DIVERTICULUM

Key Facts
Imaging Findings • Perforated duodenal ulcer
• Best diagnostic clue: Smooth, rounded outpouching Clinical Issues
from medial descending duodenum
• Asymptomatic (90%)
• Best imaging tool: Fluoroscopic-guided barium
studies Diagnostic Checklist
Top Differential Diagnoses • Periampullary diverticulum makes endoscopic
sphincterotomy difficult or dangerous
• Pseudocyst in head of pancreas
• Use oral contrast and/or position changes to help
• Pancreatic cystic tumor identify

• Differentiate by history, signs of pancreatitis

Natural History & Prognosis
3
Pancreatic cystic tumor • Complications: Diverticulitis, hemorrhage, outlet 9
• Diverticulum may contain air or enteric contrast obstruction, perforation and pancreaticobiliary disease
• Prognosis: Very good
Perforated duodenal ulcer
• Differentiate by inflammation of surrounding fat (gas Treatment
and/or edema) • True diverticula: No treatment except in complications
• Intraluminal diverticula: Lateral duodenotomy and
excision with preservation of ampulla of Vater
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
• General path comments: 1-5% of upper GI series
• Etiology Consider
o True diverticula: Acquired • Periampullary diverticulum makes endoscopic
o Pseudodiverticula: Acute or chronic duodenal ulcer sphincterotomy difficult or dangerous
o Intraluminal diverticula: Congenital duodenal
diaphragm or web Image Interpretation Pearls
o Pathogenesis • Use oral contrast and/or position changes to help
• True diverticula: Area of weakness where vessel identify
penetrates the duodenal wall or where dorsal and
ventral pancreas fuse in embryologic development
• Intraluminal diverticula: Mechanical factors (Le., I SELECTED REFERENCES
forward pressure by food & peristalsis) ~ gradual 1. Macari M et al: Duodenal diverticula mimicking cystic
elongation of duodenal web or diaphragm neoplasms of the pancreas: CT and MR imaging findings in
seven patients. AJR Am J Roentgenol. 180(1):195-9, 2003
Gross Pathologic & Surgical Features 2. Lawler LP et al: Multidetector row computed tomography
• True diverticula: Sac of mucosal and submucosal layers and volume rendering of an adult duodenal intraluminal
herniated through a muscular defect "wind sock" diverticulum. J Com put Assist Tomogr.
• Pseudodiverticula: Exaggerated outpouching of 27(4):619-21, 2003
3. Afridi SA et al: Review of duodenal diverticula. Am J
inferior & superior recesses of duodenal bulb
Gastroenterol. 86(8):935-8, 1991
• Intraluminal diverticula
o Sac of duodenal mucosa originating in the second
portion of duodenum near papilla of Vater
o Connected to part of or entire circumference of wall I IMAGE GALLERY
o Projecting distally as far as fourth part of duodenum;
Often a second opening located eccentrically in sac

ICLINICALISSUES
Presentation
• Most common signs/symptoms
o True diverticula
• Asymptomatic (90%)
o Intraluminal diverticula: Nausea and vomiting
• Diagnosis: Fluoroscopic-guided barium studies
Demographics (Left) Axial CECT shows unusual location of diverticulum, extending
• Age: True: 40-60 years of age; intraluminal: Any age off lateral surface of duodenum (arrow). (Right) UCI series shows
intraluminal diverticulum (arrow) having a "windsock" appearance,
within the lumen of the duodenum.
 GASTRITIS

3
10

Graphic shows ulcer crater and numerous mucosal Upper Gl series shows contracted antrum with
erosions, mostly in antrum along the "ridges" of thickened nodular folds and numerous varioliform
hypertrophied folds. erosions (arrows).

!TERMINOLOGY Radiographic Findings

• Fluoroscopic-guided double contrast barium studies
Definitions o Erosive gastritis, complete or varioliform erosions
• Inflammation of gastric mucosa induced by a group of (most common type)
disorders that differs in their etiological, clinical, • Location: Gastric antrum on crests of rugal folds
histological and radiological findings • Multiple punctate or slit-like collections of barium
• Erosions surrounded by radiolucent halos of
edematous, elevated mucosa
I IMAGING FINDINGS • Scalloped or nodular antral folds
General Features • Epithelial nodules or polyps (chronic)
o Erosive gastritis, incomplete or "flat" erosions
• Best diagnostic clue: Ulcers and thickened folds
• Location: Antrum or body
• Other general features
• Multiple linear streaks or dots of barium
o Classification of gastritis
• No surrounding edematous mucosa
• Erosive or hemorrhagic gastritis (2 types:
o Erosive gastritis, NSAIDs induced
Complete or varioliform and incomplete or "flat")
• Linear or serpiginous erosions clustered in the
• Antral gastritis
body, on or near greater curvature
• H. pylori gastritis
• Varioliform or linear erosions in antrum
• Hypertrophic gastritis
• NSAIDs-related gastropathy: Subtle flattening and
• Atrophic gastritis (2 types: A and B)
deformity of greater curvature of antrum
• Granulomatous gastritis (Crohn disease and
o Antral gastritis
tuberculosis)
• Thickened folds, spasm or decreased distensibility
• Eosinophilic gastritis
• Scalloped or lobulated folds oriented
• Emphysematous gastritis
longitudinally or transverse folds
• Caustic ingestion gastritis
• Crenulation or irregularity of lesser curvature
• Radiation gastritis
• Hypertrophied antral-pyloric fold: Single lobulated
• AIDS-related gastritis: Viral, fungal, protozoal and
fold on lesser curvature of distal antrum extends
parasitic infections
via pylorus to base of duodenal bulb (chronic)

DDx: Thickened Gastric Folds +/- Ulceration

Antral Carcinoma Antral Carcinoma Pancreatitis Lymphoma

 GASTRITIS
Key Facts
Terminology
• Inflammation of gastric mucosa induced by a group
of disorders that differs in their etiological, clinical,
histological and radiological findings
Imaging Findings
• Best diagnostic clue: Ulcers and thickened folds
• Erosive gastritis, complete or varioliform erosions
(most common type)
• Erosive gastritis, incomplete or "flat" erosions
Top Differential Diagnoses
• Gastric carcinoma
• Zollinger-Ellison syndrome
• Pancreatitis
• Gastric metastases and lymphoma
o H. pylori gastritis
• Location: Antrum, body or occasionally fundus;
diffuse or localized
• Thickened, lobulated gastric folds (polypoid
gastritis)
• Enlarged areae gastricae (:? 3 mm in diameter)
o Hypertrophic gastritis
• Location: Fundus and body
• Markedly thickened, lobulated gastric folds
o Atrophic gastritis
• Location of type A: Fundus and body
• Location of type B: Antrum
• Narrowed or tubular stomach
• Smooth, featureless mucosa
• Decreased distensibility
• Decreased or absent mucosal folds
• Small (1-2 mm in diameter) or absent areae
gastricae
o Granulomatous gastritis, Crohn disease
• Location: Antrum and body
• Multiple aphthous ulcers
• Indistinguishable from erosive gastritis,
varioliform type
• Advanced disease - large ulcers, thickened folds,
nodular or cobblestone mucosa
• "Ram's horn" sign: tubular, narrowed,
funnel-shaped antrum
• Single, continuous tubular structure involve
antrum and duodenum; obliteration of normal
anatomy
• Severe disease - filiform polyps
o Granulomatous gastritis, tuberculosis
• Location: Lesser curvature of antrum or pylorus
• Antral narrowing - obstruction
o Eosinophilic gastritis
• Location: Antrum and body
• Mucosal nodularity, thickened folds, antral
narrowing and rigidity
o Emphysematous gastritis (use water-soluble contrast)
• Multiple streaks, bubbles, or mottled collections of
gas in the wall of stomach, silhouetting the gastric
shadow; do not alter with positional changes
o Caustic ingestion (use water-soluble contrast)
Pathology
• Erosive gastritis: Superficial acute inflammation or
focal necrosis of mucosa
• H. pylori gastritis: Lymphoid nodules or increased
neutrophils or plasma cells
Clinical Issues
• Asymptomatic
• Epigastric pain, nausea, vomiting or hematemesis
Diagnostic Checklist
• History and H. pylori infection
• H. pylori gastritis: Thickened, lobulated gastric folds 3
with enlarged areae gastricae
• Erosive gastritis: Multiple collections of barium 11
surrounded by radiolucent halos of edematous,
elevated mucosa
• Location: Lesser curvature and distal antrum
• Acute: Ulceration, thickened folds, gastric atony
or mural defects
• Chronic: Antral narrowing & deformity (scarring)
o Radiation gastritis
• Acute: Ulceration, thickened folds, gastroparesis or
spasm
• Chronic: Antral narrowing & deformity (scarring)
o AIDS-related gastritis
• Mucosal nodularity, erosions, ulcers, thickened
folds or antral narrowing
CT Findings
• Decreased wall attenuation (edema or inflammation)
• Thickened gastric folds or wall
• Target or "halo": Mucosal enhancement and decreased
HU of submucosa (edema)
• H. pylori gastritis: Circumferential antral wall
thickening or focal thickening of posterior gastric wall
along greater curvature
• Emphysematous gastritis: Thickened wall and
collections of gas within the wall with or without gas
in intrahepatic portal veins
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided double contrast
barium or water-soluble contrast studies
I DIFFERENTIAL DIAGNOSIS
Gastric carcinoma
• Differentiate from gastritis by loss of distensibility and
decreased or absent peristalsis in involved portion
• Scirrhous carcinoma (linitis plastica)
o Nodular, distorted mucosa
o Thickened, irregular folds
o Most important differential for atrophic gastritis
Zollinger-Ellison syndrome
• Thickened gastric folds in fundus and body (edema,
inflammation, and hyperplasia)
• 1 Fluids in lumen and:? 1 ulcers at unusual locations
 GASTRITIS
Pancreatitis
• Common cause of gastric wall thickening; mimics
thickened folds
Gastric metastases and lymphoma
• CT: Submucosal tumor is soft tissue density
• Metastases (e.g., malignant melanoma, breast cancer)
o Simulate gastritis by thickened folds
o Differentiate by loss of distensibility
• Gastric lymphoma
o Variably sized, rounded, often confluent nodules
o Mucosal nodularity is difficult to differentiate from
enlarged areae gastricae
3 o Markedly thickened and lobulated folds mimics
antral, H. pylori and hypertrophic gastritis
12
!PATHOLOGY
General Features
• Etiology
o Erosive: NSAIDs, alcohol, steroids, stress, trauma,
burns or infections
o Atrophic: Fundus and body: Autoimmune; antral: H.
pylori, bile or alcohol
o Antral: Alcohol, tobacco, coffee or H. pylori
o Granulomatous: Crohn disease, sarcoidosis,
tuberculosis, syphilis or candidiasis
o Emphysematous: E. coli, S. aureus, Clostridium
perfringens or Proteus vulgaris
o Caustic ingestion: Strong acids (hydrochloric,
sulfuric, acetic, oxalic, carbolic or nitric acid)
o Radiation: > 5,000 rads
o AIDS-related: Cytomegalovirus, cryptosporidiosis,
toxoplasmosis or strongyloidiasis
• Associated abnormalities
o Atrophic gastritis: Underlying 90% of pernicious
anemia patients
o Hypertrophic gastritis: 66% of patients have
duodenal ulcers
Gross Pathologic & Surgical Features
• Erosive gastritis: Areas of congested, edematous or
ulcerated mucosa
• Atrophic gastritis: Thin smooth mucosa, flattened
rugae or tubular stomach
Microscopic Features
• Erosive gastritis: Superficial acute inflammation or
focal necrosis of mucosa
• H. pylori gastritis: Lymphoid nodules or increased
neutrophils or plasma cells
• Atrophic gastritis: Thin mucosa, atrophy of mucosal
glands, loss of parietal and chief cells or intestinal
metaplasia
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Epigastric pain, nausea, vomiting or hematemesis
o Atrophic gastritis: Neurologic symptoms from
vitamin B12 deficiency
• Lab-Data
o I Leukocytes; Positive fecal occult blood test
o Atrophic gastritis: j Vitamin B12
o Positive H. pylori (endoscopy, histology, cultures,
urea breath and serologic tests)
Natural History & Prognosis
• Caustic ingestion gastritis: Acute necrotic phase (1-4
days); ulceration-granulation phase (5-28 days);
cicatrization and scarring (3-4 weeks)
• Radiation gastritis: Inflammation (1-6 months);
scarring and fibrosis (6 months)
• Complications
o Gastric or duodenal ulcer, pernicious anemia, low
grade MALT lymphoma or gastric carcinoma
o Eosinophilic gastritis: Gastric outlet obstruction
o Caustic ingestion gastritis: Gastric necrosis
• Prognosis
o Erosive, antral, H. pylori and atrophic gastritis: Good
after treatment
o Eosinophilic gastritis: Chronic, relapsing disease
with intermittent exacerbation and asymptomatic
intervals
o Emphysematous gastritis: 60-80% mortality
Treatment
• Stop offending agents: Alcohol, tobacco, NSAIDs,
steroids and coffee
• H. pylori treatment: Metronidazole, bismuth and
clarithromycin, amoxicillin or tetracycline
• Hypertrophic gastritis: Antisecretory agents
(H2-receptor antagonists or proton-pump inhibitors)
• Atrophic gastritis: Replace vitamin B12
• Eosinophilic gastritis: Steroids
• Emphysematous gastritis: IV fluids, antibiotics, but no
nasogastric tube
• Caustic ingestion gastritis: Steroids, antibiotics,
parenteral feedings and ± surgery
I DIAGNOSTIC CHECKLIST
Consider
• History and H. pylori infection
Image Interpretation Pearls
• H. pylori gastritis: Thickened, lobulated gastric folds
with enlarged areae gastricae
• Erosive gastritis: Multiple collections of barium
surrounded by radiolucent halos of edematous,
elevated mucosa
I SELECTED REFERENCES
1. Horton KM et al: Current role of CT in imaging of the
stomach. Radiographies. 23(1):75-87, 2003
2. Bender GN et al: Double-contrast barium examination of
the upper gastrointestinal tract with non endoscopic
biopsy: findings in 100 patients. Radiology. 202(2):355-9,
1997
3. Sohn J et al: Helicobacter pylori gastritis: radiographic
findings. Radiology. 195(3):763-7, 1995
 GASTRITIS
I IMAGE GALLERY
Typical
(Left) Upper CI series shows
contracted antrum with
nodular thickened folds,
some of which have
prolapsed into the
duodenum. (Right) Upper CI
series shows rows of
varioliform erosions along
the top of hypertrophied
gastric antral folds.

3
13

Typical
(Left) Upper CI series shows
almost complete absence of
gastric folds in atrophic
gastritis. (Right) Upper CI
series shows numerous
variolHorm (aphthous)
erosions in gastric antrum
(arrow). (The duodenal bulb
is collapsed and filled with
barium).

Typical
(Left) Axial CECT in renal
transplant recipient shows
massive gastric wall
thickening. The low density
process (arrow) represents
gastritis, while the soft tissue
density (open arrow) is
PTLD. (Right) Axial CECT in
a renal transplant recipient
shows gastritis (arrow) and
PTLD (open arrow)
(post-transplant
Iymphoproliferative
disorder).
 GASTRIC ULCER

3
14

Graphic shows gastric ulcer with smooth gastric folds Upper GI series shows barium pool in ulcer crater
radiating to the edge of the ulcer crater. Also note (arrow), with smooth folds radiating to the edge of the
infolding of the gastric wall "pointing" toward the ulcer. ulcer.

ITERMINOLOGY • Morphology
o Lesser or greater curvature
Abbreviations and Synonyms • Profile view: Can see size, shape, depth, Hampton
• Peptic ulcer disease line, ulcer collar, ulcer mound or radiating folds
o Anterior or posterior wall
Definitions • En face view: Best for radiating folds
• Mucosal lesion of stomach
Radiographic Findings
• Fluoroscopic-guided double contrast barium studies
I IMAGING FINDINGS o Benign gastric ulcer - profile view
• Ulcer crater: Round or ovoid collections of barium
General Features • Hampton line: Thin radiolucent line separating
• Best diagnostic clue: Sharply marginated barium barium in gastric lumen from barium in crater
collection and folds radiating to edge of ulcer crater on • Ulcer mound: Smooth, bilobed hemispheric mass
fluoroscopic-guided double contrast barium studies projecting into lumen on both sides of ulcer; outer
• Location borders form obtuse, gently-sloping angles with
o Benign gastric ulcer adjacent gastric wall (edema or inflammation)
• Usually lesser curvature or posterior wall of • Ulcer collar: Radiolucent rim of edematous
antrum or body mucosa around ulcer
• 3-11% on greater curvature; 1-7% on anterior wall • Smooth, round ulcer projecting beyond lesser
o 0 Malignant gastric ulcer curvature
• Usually greater curvature • Smooth, symmetric radiating folds to edge of ulcer
• Size crater
o > 0.5 cm to be visualized • Incisura defect: Smooth or narrow indentation on
o Most diagnosed ulcers are < 1 cm greater curvature opposite an ulcer on lesser
o Larger ulcers tend to be more proximal in stomach curvature (muscle contraction)
o Giant (> 3 cm) ulcers are mostly benign, but • Enlarged areae gastricae in adjacent mucosa
increased risk of complications (edema or inflammation)

DDx: Persistant Collection of Contrast with Mucosal Ulceration

,.
I,' .• ' ~ ...
/
~

1':1~
Gastric Cancer Gastric Lymphoma Melanoma Met. Leiomyoma
 GASTRIC ULCER
Key Facts
Terminology
• Mucosal lesion of stomach
Imaging Findings
• Best diagnostic clue: Sharply marginated barium
collection and folds radiating to edge of ulcer crater
on fluoroscopic-guided double contrast barium
studies
• Ulcer crater: Round or ovoid collections of barium
• Hampton line: Thin radiolucent line separating
barium in gastric lumen from barium in crater
• Ulcer mound: Smooth, bilobed hemispheric mass
projecting into lumen on both sides of ulceri outer
borders form obtuse, gently-sloping angles with
adjacent gastric wall (edema or inflammation)
• Sump ulcers: Distal one half of greater curvature
(NSAIDs)
• Ulcer on greater curvature ~ area of mass effect
and thickened irregular folds
• Linear barium-coated ulcer: j In depth (healing)
• Splitting of 1 ulcer to 2 smaller ulcers (healing)
• Central pit or depression, radiating folds, or
retraction of adjacent gastric wall (scarring)
• "Hourglass" stomach: Marked narrowing of body
(scarring)
o Benign gastric ulcer - en face view
• Ring shadow: Shallow ulcer on anterior or
posterior wall (barium coated rim and unfilled
crater)
o Malignant gastric ulcer - profile
• "Carman meniscus" sign: Ulcer crater and
radiolucent elevated border
• Does not project beyond expected gastric contour
• Discrete tumor mass forms acute angles
o Malignant gastric ulcer - en face
• Irregular crater eccentrically located within a
tumor mass
• Focal nodularity, distortion or obliteration of
adjacent areae gastricae (tumor infiltration)
• Nodular, clubbed, fused, or amputated folds
(tumor infiltration)
CT Findings
• CECT (use water or water-soluble oral contrast)
o Signs of complications
• Wall thickening or luminal narrowing of stomach
• Infiltration of surrounding fat or organs (pancreas)
• Free air in abdomen or lesser sac
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided
double-contrast barium studies (en face and profile
views)
• Protocol advice: In addition to standard air-contrast
views, prone compression views of gastric antrum and
body or prone Trendelenburg position should be used
to demonstrate anterior wall ulcers
• Ulcer collar: Radiolucent rim of edematous mucosa
around ulcer
• Smooth, symmetric radiating folds to edge of ulcer
crater
Top Differential Diagnoses
• Gastritis
• Gastric metastases and lymphoma
Clinical Issues
• Burning, gnawing, or aching pain at the epigastrium
• < 2 hrs after mealsi not relieved by food or antacids
Diagnostic Checklist 3
• Rule out malignant gastric ulcers 15
• Malignant gastric ulcers: "Carman meniscus" sign;
nodular, blunted folds
I DIFFERENTIAL DIAGNOSIS
Gastritis
• Markedly thickened gastric folds
• Helicobacter pylori (H. pylori) gastritis
o Thickened gastric folds in antrum or body
o Enlarged areae gastricae (2c 3 mm)
• Hypertrophic gastritis
o Glandular hyperplasia and 1 secretion of acid
o Thickened gastric folds in fundus and body
Ulcerated intramural primary tumor
• Leiomyoma or gastrointestinal stromal tumor (GIST)
o Millimeters to enormous masses
o In profile view, discrete submucosal mass with
smooth surface that is etched in white and borders
form right angles or slightly obtuse angles with
adjacent gastric wall
o Larger than 2 cm ~ ulcerated, central barium-filled
crater within a smooth or slightly lobulated
submucosal mass ("bull's eye" or "target" lesions)
Gastric metastases and lymphoma
• Malignant melanoma
o Most common hematogenous metastasis to stomach
o Necrotic; development of giant, cavitated lesion
o Amorphous collection of barium (5-15 cm in size)
that communicates with lumen
o "Bull's eye" or "target" lesions also occur
• Metastatic Kaposi sarcoma
o Increased incidence in homosexual AIDS patients
o GI involvement in 50%
o Almost always associated with cutaneous lesions
o Rarely symptomatic
o Elevated lesions; submucosal defects (0.5-3.0 cm)
o As nodules enlarge, often ulcerate, producing 1 or
more "bull's eye" or "target" lesions
• Gastric Lymphoma
o More frequent than other parts of GI tract
o 50% confined to stomach
o Majority are non-Hodgkin lymphoma (B-cell origin)
 GASTRIC ULCER
o Evidence suggests chronic H. pylori gastritis may
lead to low grade mucosa-associated lymphoid tissue
(MALT) lymphomas
o Low grade MALT lymphoma appearance is variably
sized, rounded, often confluent nodules
o Mucosal nodularity is difficult to differentiate from
enlarged areae gastricae
Artifactual
• Barium precipitates
o Resemble tiny ulcers; differentiated by lack of
projection beyond wall
o Absence of mucosal edema or radiating fold
• "Stalactites"
3 o Hanging droplets of barium (on anterior gastric wall)
o Differentiated by transient nature on
16 double-contrast barium studies
I PATHOLOGY
General Features
• General path comments
o One of two forms of peptic ulcer disease
o Unequivocal benign gastric ulcers on double
contrast studies: No further testing
o Equivocal gastric ulcers (mixed features of benign
and malignant)
• Endoscopy and biopsy to exclude malignancy
• 1£endoscopy and biopsy are negative, follow-up
with double-contrast studies until complete
healing or repeat endoscopy and biopsy
o Multiplicity
• 80% benign; most likely cause is NSAIDs
• Genetics
o Genetic syndromes
• Multiple endocrine neoplasia type 1 (MEN I)
• Systemic mastocytosis
o Greater concordance in monozygotic twins
o Increased incidence with blood type 0
• Etiology
o 2 major risk factors: H. pylori (60-80%) and NSAIDs
o Other risk factors: Steroids, tobacco, alcohol, coffee,
stress, reflux of bile, delayed gastric emptying
o Less common etiologies
• Zollinger-Ellison syndrome
• Hyperparathyroidism
• Cushing ulcer: Head injuries (stress)
• Curling ulcer: Burns
• Gastritis
o Pathogenesis
• Normal or decreased levels of gastric acid
• Breakdown in mucosal defense by H. pylori
• Epidemiology
o 95% benign, 5% malignant
o Multiplicity: 20-30% prevalence
Gross Pathologic & Surgical Features
• Round or oval; sharply punched-out and regular walls;
flat adjacent mucosa
Microscopic Features
• Necrotic debris; zone of active inflammation;
granulation and scar tissue
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Burning, gnawing, or aching pain at the epigastrium
o < 2 hrs after meals; not relieved by food or antacids
o Pain that awakens patients from sleep (33%)
o Anorexia and weight loss (50%)
• Diagnosis: Endoscopy with biopsy
Demographics
• Age: > 40 years of age
• Gender: Equal in both males and females
Natural History & Prognosis
• Complications
o Hemorrhage, perforation, obstruction and fistula
• Prognosis
o Good with medical treatment and surgery
Treatment
• Ulcer without H. pylori: H2-receptor antagonists
(cimetidine, ranitidine, or famotidine) or
proton-pump inhibitors (omeprazole or lansoprazole)
• H. pylori treatment: Metronidazole, bismuth and
clarithromycin, amoxicillin or tetracycline
• Ulcer with H. pylori: H. pylori treatment and
H2-receptor antagonists or proton-pump inhibitors
• NSAID-induced: Misoprostol and stop NSAIDs
• Other agent: Sucralfate
• Surgery required for
o Recurrent or intractable ulcers
o Ulcer complications
o Equivocal or suspicious findings on radiologic or
endoscopic examinations
• Follow-up: 6-8 weeks after medical treatment
o 1£not healed, suggests malignant gastric ulcer
I DIAGNOSTIC CHECKLIST
Consider
• Rule out malignant gastric ulcers
Image Interpretation Pearls
• Benign gastric ulcers: Ulcer crater, Hampton line, ulcer
mound and collar; smooth, radiating folds
• Malignant gastric ulcers: "Carman meniscus" sign;
nodular, blunted folds
I SELECTED REFERENCES
1. Horton KM et al: Current role of CT in imaging of the
stomach. Radiographies. 23(1):75-87, 2003
2. Pattison CP et al: Helicobacter pylori and peptie ulcer
disease: Evolution to revolution to resolution. AJR 168:
1415-20, 1997
3. Fishman EKet al: CT of the stomach: spectrum of disease.
Radiographies. 16(5):1035-54, 1996
4. Levine MS et al: The Helicobacter pylori revolution:
Radiologic perspective. Radiology 195: 593-6, 1995
5. Jacobs JM: Peptic ulcer disease: CT evaluation. Radiology
178: 745-8, 1991
 GASTRIC ULCER

I IMAGE GAllERY

(Left) Upper GI series shows

ulcer crater (arrow) with
radiating folds to the edge of
the crater. (Right) Axial
CECT shows large posterior
wall gastric ulcer (arrow).
Gastric folds are thickened.

3
17

(Left) Axial CECT shows

perforated gastric antral ulcer
resulting in intraperitoneal air
(arrow), fluid and enteric
contrast medium (open
arrow). (Right) Axial CECT
shows perforated posterior
gastric wall ulcer with enteric
(ora/) contrast medium in
lesser sac (arrow).

(Left) Axial CECT shows

posterior gastric wall ulcer
with loculated fluid and gas
in lesser sac (arrow). (Right)
Lateral view of upper GI
series shows deep posterior
wall gastric ulcer (arrow)
and a "mound" of
edematous folds.
 DUODENAL ULCER

3
18

Graphic shows duodenal ulcer with deformed bulb due Upper GI series shows ulcer crater (arrow) and
to converging folds and spasm. deformed bulb, including a pseudodiverticulum (curved
arrow).

ITERMINOLOGY Radiographic Findings

Abbreviations and Synonyms
• Peptic ulcer disease
Definitions
• Mucosal erosion of duodenum
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Sharply marginated barium
collection with folds radiating to edge of ulcer crater
on fluoroscopic-guided double-contrast barium study
• Location
o 95% duodenal bulbar ulcers; 5% postbulbar ulcers
o Bulbar ulcers are located at the apex, central portion,
or base of the bulb
o Postbulbar ulcers are located on the medial wall of
the proximal descending duodenum above papilla
of Vater
o 50% of duodenal ulcers are located on anterior wall
• Size: Most ulcers are < 1 cm at time of diagnosis
• Morphology
o Round or ovoid collections of barium
o 5% of duodenal ulcers have linear configuration
• Fluoroscopic-guided double-contrast barium studies
o Bulbar ulcers
• Persistent small round, ovoid or linear ulcer niche
(collection of barium)
• Ulcer mound: Smooth, radiolucent mound of
edematous mucosa
• Radiating folds converge centrally at the edge of
the ulcer crater
• Ring shadow: Barium coating rim of unfilled
anterior wall ulcer crater (air contrast view)
• Deformity of bulb (edema and spasm or scarring)
• Residual depression of central portion of scar
mimics active ulcer crater
• Pseudodiverticula: Ballooning out between areas
of fibrosis and spasm
• "Cloverleaf" deformity: Multiple pseudodiverticula
o Postbulbar ulcers
• Smooth or rounded indentation on lateral wall
opposite of ulcer crater (edema and spasm)
• "Ring stricture": Eccentric narrowing (scarring)
o Giant duodenal ulcers (> 2 cm)
• Always located in duodenal bulb
• Replace virtually entire bulb; mistaken for a
scarred or normal bulb
• Fixed or unchanging configuration is key clue
• Focal narrowing - outlet obstruction (edema and
spasm)

DDx: Duodenal Fixed Deformity +/- Contrast Collection

Radiation Duodenitis Crohn Duodenitis Duodenal Cancer Duodenal Diverticula

 DUODENAL
Key Facts
Terminology
• Mucosal erosion of duodenum
Imaging Findings
• Best diagnostic clue: Sharply marginated barium
collection with folds radiating to edge of ulcer crater
on fluoroscopic-guided double contrast barium study
• 95% duodenal bulbar ulcers; 5% postbulbar ulcers
• Persistent small round, ovoid or linear ulcer niche
(collection of barium)
• Ulcer mound: Smooth, radiolucent mound of
edematous mucosa
• Radiating folds converge centrally at the edge of the
ulcer crater
• Ring shadow: Barium coating rim of unfilled anterior
wall ulcer crater (air contrast view)
CT Findings
• CECT (use water or water-soluble oral contrast)
o Signs of ulcer penetration and perforation
• Wall thickening or luminal narrowing of
duodenum
• Infiltration of surrounding fat/organs (pancreas)
• Extraluminal gas intra- or retro-peritoneal
Imaging Recommendations
• Best imaging tool
o Fluoroscopic guided double contrast barium studies
o High density barium for views of duodenal bulb
o Low density barium for upright or prone
compression views
• Protocol advice: Routinely obtain prone compression
views of duodenum to observe anterior wall ulcers
I DIFFERENTIAL DIAGNOSIS
Duodenal inflammation
• Duodenitis
o Inflammation without frank ulceration
• Crohn disease
o Usually with antral involvement
o Aphthous ulcers are the earliest abnormality
observed
o Thickened, nodular folds; cobblestone appearance
o Asymmetric duodenal narrowing with outward
ballooning of duodenal wall between area of fibrosis
o Smooth, tapered areas of narrowing; extend from
apical portion of the bulb to descending duodenum
o One or more strictures in second or third portions of
duodenum ~ marked obstruction and proximal
dilatation (megaduodenum)
• Tuberculosis
o Usually with antral involvement
o Ulcers, thickened folds, narrowing or fistula
o Enlarged lymph nodes adjacent to duodenum ~
narrowing or obstruction of lumen
Duodenal stricture
• Pancreatitis
ULCER
Top Differential Diagnoses
• Duodenal inflammation
• Duodenal stricture
• Duodenal carcinoma
Pathology
• 2-3 times more frequent than gastric ulcers
Clinical Issues
• Burning, gnawing, or aching pain at the epigastrium
• 2-4 hrs after meals; relieved by antacids or food
Diagnostic Checklist 3
• Eradication of H. pylori is the first step of treatment
• Check for deformity of the duodenal bulb 19
• Prone compression views are necessary to evaluate
anterior wall duodenal ulcers
o "Inverted 3" sign of Frostberg
• Central limb of the 3: Point of fixation where
pancreatic and common bile ducts insert into the
papilla
• Above and below the point reflect edema of major
and minor papilla or smooth muscle spasm and
edema in the duodenal wall
o Thickened folds associated with medial compression
or widening of duodenal sweep
o Spiculation of mucosal folds (edema or
inflammation)
• Gallstone erosion
o Radiolucent filling defect in duodenum
o Cause mucosal inflammation, ulceration,
hemorrhage, perforation and obstruction
o Barium reflux into gallbladder and bile ducts
Duodenal carcinoma
• < 1% of all gastrointestinal cancers
• Located in the postbulbar portion at or distal to
papilla of Vater
• Polypoid, ulcerated, or annular lesions
• Narrowed lumen with thickened wall
Duodenal diverticulum
• 1-5% as incidental findings in barium studies
• Mostly located on medial border of descending
duodenum in periampullary region
• Smooth, rounded outpouching from the medial border
of the descending duodenum
• Multiple diverticula observed; configuration may
change during course of study
• Differentiate from postbulbar ulcers by lack of
inflammatory reaction and change in shape
Extrinsic invasion
• Pancreatic carcinoma
o Widening of duodenal sweep
o Mass effect ~ double contour effect on medial
border of duodenum: Differential filling with
interfold spaces along the inner aspect containing
less barium than corresponding spaces along the
outer aspect
 DUODENAL ULCER
o Displacement or frank splaying of the spikes: Tumor o 2-4 hrs after meals; relieved by antacids or food
infiltrating the duodenal wall with traction and o Pain that awakens patients from sleep (66%)
fixation of the folds o Other signs/symptoms
• Gallbladder carcinoma • Pain episodes that occur in clusters of days to
o Compression of bulb or proximal duodenum weeks followed by longer pain-free intervals
• Metastases • Rarely anorexia and weight loss; hyperphagia and
o Widening of duodenal sweep weight gain because eating relieves pain
o Multiple submucosal masses or "bull's eye" lesion • Lab-Data
o Diagnostic tests (serology or urease breath test) for
Duodenal hematoma H. pylori
• Radiolucent filling defects from blood clots • Diagnosis: Endoscopy
• Well-circumscribed intramural masses with discrete
margins ~ stenosis and obstruction Demographics
• Diffuse hemorrhage ~ thickened, spiculated folds or • Age: Adults of all ages
3 thumbprinting • Gender: Equal in both males and females

20 Natural History & Prognosis

!PATHOLOGY • Complications:
o Hemorrhage, perforation, obstruction and fistula
General Features o Giant duodenal ulcers have 1 risks of complications
• General path comments • Prognosis
o Multiplicity o Good with medical treatment and surgery
• 15% of patients with duodenal ulcers
Treatment
• Ulcers located in duodenal bulb and beyond
• Ulcer without H. pylori: H2-receptor antagonists
• Suspicious of Zollinger-Ellison syndrome
(cimetidine, ranitidine, or famotidine) or
• Genetics
proton-pump inhibitors (omeprazole or lansoprazole)
o Genetic syndromes
• H. pylori treatment: Metronidazole, bismuth and
• Multiple endocrine neoplasia type 1 (MEN 1)
clarithromycin, amoxicillin or tetracycline
• Systemic mastocytosis
o Greater concordance in monozygotic twins • Ulcer with H. pylori: H.pylori treatment and
H2-receptor antagonists or proton-pump inhibitors
o Increased incidence with blood type a
• Other agent: Sucralfate
• Etiology
o Two major risk factors: Helicobacter pylori (H. • Follow-up: Intractable ulcers and complications
pylori) (95-100%) & NSAIDs
o Other risk factors: Steroids, tobacco, alcohol, coffee,
stress, reflux of bile, delayed gastric emptying
I DIAGNOSTIC CHECKLIST
o Less common etiologies Consider
• Zollinger-Ellison syndrome • Eradication of H. pylori is the first step of treatment
• Hyperparathyroidism
• Chronic renal failure Image Interpretation Pearls
• Chronic obstructive pulmonary disease • Check for deformity of the duodenal bulb
o Pathogenesis • Prone compression views are necessary to evaluate
• H. pylori mediates or facilitates damage to gastric anterior wall duodenal ulcers
and duodenal mucosa
• 1 Gastric acid and 1 gastric emptying ~ 1 acidic
exposure in the duodenum I SELECTED REFERENCES
• Epidemiology 1. Jayaraman MV et al: CT of the duodenum: an overlooked
o Incidence: 200,000 cases per year segment gets its due. Radiographies. 21 Spec No:SI47-60,
o 2-3 times more frequent than gastric ulcers 2001
2. Pattison CP et al: Helicobacter pylori and peptic ulcer
Gross Pathologic & Surgical Features disease: Evolution to revolution to resolution. AJR168:
• Round or oval; sharply punched-out and regular walls; 1415-20, 1997
flat adjacent mucosa 3. Levine MS et al: The Helicobacter pylori revolution:
Radiologic perspective. Radiology 195: 593-6, 1995
Microscopic Features 4. Jacobs JM: Peptic ulcer disease: CT evaluation. Radiology
• Necrotic debris; zone of active inflammation; 178: 745-8, 1991
granulation and scar tissue

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Burning, gnawing, or aching pain at the epigastrium
 DUODENAL ULCER
I IMAG E GALLERY
Typical
(Left) Upper GI series shows
a duodenal ulcer (arrow)
and a large
pseudodiverticulum (curved
arrow), which changed
shape during the exam.
(Right) Upper GI series
shows a "giant" duodenal
ulcer (arrow) which did not
change shape during the
exam. 3
21

Typical
(Left) Axial NECT shows free
air (open arrow) and active
extravasation of oral contrast
medium (arrow) from a
perforated duodenal ulcer.
High density fluid
accumulated in Morison
pouch (curved arrow).
(Right) Axial NECT shows
free air (open arrow) and
high density fluid in the right
paracolic gutter (curved
arrow) and retroperitoneum
(arrow).

(Left) Axial CECT shows

retroperitoneal gas
surrounding kidney and third
portion of duodenum due to
perforated ulcer. (Right)
Upper GI series shows large
post-bulbar ulcer (arrow)
with marked narrowing of
lumen.
 ZOLLINGER-ELLISON SYNDROME

3
22

Upper GI series shows thickened gastric and duodenal Upper GI series shows gastric fold thickening and
folds and excess fluid in stomach. Several duodenal excess fluid. Duodenal and jejunal strictures are present
ulcers are present (arrows). (arrows), probably from prior ulceration.

o Islet cell tumors are neuroendocrine tumors

ITERMINOLOGY
Abbreviations and Synonyms
• Zollinger-Ellison syndrome (ZES)
Definitions
• Severe peptic ulcer disease associated with marked
increase in gastric acid due to gastrin producing islet
cell tumor (gastrinoma) of pancreas
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Hypervascular pancreatic mass
with multiple peptic ulcers & thickened folds
• Location
o Gastrinoma: Pancreas (75%); duodenum (15%); liver
& ovaries (10%)
o Common site (gastrinoma): Gastrinoma triangle
• Superiorly: Cystic & common bile ducts
• Inferiorly: 2nd & 3rd parts of duodenum
• Medially: Junction of neck & body of pancreas
o Ulcers: Stomach & duodenal bulb (75%); postbulbar
& jejunum (25%)
• Other general features
o Usually due to non-~ islet cell tumor (gastrinoma) of
pancreas
o Rare in comparison with tumors of exocrine
pancreas
o Gastrinomas are 2nd most common functioning
islet cell tumors after insulinomas
o Gastrinomas are multiple (60%); malignant (60%);
metastases (30-50%)
Radiographic Findings
• Barium studies: Gastric, duodenal & proximal jejunum
o Large volume of fluid dilutes barium & compromises
mucosal coating
• Hypersecretion of gastric acid ~ 1 fluid collection
o Markedly thickened folds
o Peptic ulcers: Round or ovoid collections of barium
surrounded by a thin or thick radiolucent rim
(edematous mucosa) & radiating folds
CT Findings
• Gastrinomas
o Heterogeneous density lesion; small or large
o ± Cystic & necrotic areas; ± calcification
o Liver metastases are common
o Arterial & portal venous phase scans
• Hypervascular (primary & secondary) lesions
• ± Local or vascular invasion
o Inflammatory changes in stomach, duodenum &
proximal small-bowel
• Thickened gastric, duodenal & jejunal folds

DDx: Gastric Wall Thickening +/- Ulceration

Gastritis Gastritis Carcinoma Lymphoma

 ZOLLINGER-ELLISON
Key Facts
Terminology
• Zollinger-Ellison syndrome (ZES)
• Severe peptic ulcer disease associated with marked
increase in gastric acid due to gastrin producing islet
cell tumor (gastrin om a) of pancreas
Imaging Findings
• Best diagnostic clue: Hypervascular pancreatic mass
with multiple peptic ulcers & thickened folds
• Common site (gastrinoma): Gastrinoma triangle
• Peptic ulcers: Round or ovoid collections of barium
surrounded by a thin or thick radiolucent rim
(edematous mucosa) & radiating folds
• Hypervascular (primary & secondary) lesions
• Thickened gastric, duodenal & jejunal folds
• Luminal narrowing of stomach & duodenum
o Shows signs of ulcer penetration
• Wall thickening
• Luminal narrowing of stomach & duodenum
o Shows signs of ulcer perforation
• Free air in abdomen (duodenal/antral ulcer)
• Lesser sac (gastric ulcer)
MR Findings
• TIWI: Fat-saturated sequence: Hypointense
• T2WI
o Spin-echo sequence: Hyperintense
• Both primary & secondaries
• Tl C+
o Fat-saturated delayed spin-echo sequence
• Hyperintense, hypervascular
Ultrasonographic Findings
• Real Time
o Endoscopic ultrasonography (EUS)
• Detects small gastrinomas, better than CT or MR
• Homogeneously hypoechoic mass
o Intraoperative ultrasonography
• Detects very small tumorsi sensitivity (75-100%)
Angiographic Findings
• Conventional
o Gastrinomas & metastases: Hypervascular
o Portal venous sampling: After intra-arterial secretin
stimulation abnormal increase in gastrin levels
Imaging Recommendations
• Helical CTi MR & T1 C+; EUSibarium studies
I DIFFERENTIAL DIAGNOSIS
H. pylori gastritis
• Helicobacter pylori: Gram-negative bacillus
• Most common cause of chronic active gastritis
• Location: Gastric antrum (most common site)
o Proximal half or entire stomach may be involved
• Double contrast barium findings
o Thickened gastric folds
o Enlarged areae gastricae (?c 3 mm in diameter)
SYNDROME
Top Differential Diagnoses
• H. pylori gastritis
• Gastric carcinoma
• Gastric metastases & lymphoma
• Extrinsic inflammation
• Other gastritides
Pathology
• Gastrinoma: 1 Gastrin levels ~ 1 gastric acid
secretions ~ peptic ulcers
Diagnostic Checklist
• Rule out other causes of gastric wall thickening & 3
ulceration
• Hypervascular pancreatic tumor, liver metastases with 23
multiple ulcers & thickened folds of stomach,
duodenum & jejunum
o Polypoid gastritis: Markedly thickened, lobulated
gastric folds
• CT findings
o Circumferential antral wall thickening
o Focal thickening of posterior gastric wall along
greater curvature
• Both barium & CT findings may mimic peptic ulcer
disease of ZES
• Diagnosis: Endoscopic biopsy, culture, urea breath test
Gastric carcinoma
• Most common primary gastric tumor
• H. pylori (3-6 fold), pernicious anemia (2-3 fold) 1 risk
• Double-contrast barium findings
o Early gastric cancer
• Superficial lesion: Mucosal nodularity, ulceration,
plaque-like or localized thickened gastric folds
• Indistinguishable from focal peptic ulcers of ZES
• CT findings
o Early gastric cancer
• Focal wall thickening with mucosal irregularity
o May simulate focal peptic ulcer disease of ZES
• Diagnosis: Endoscopic biopsy & histology
Gastric metastases & lymphoma
• Gastric metastases: Most common organs of origin
o Malignant melanoma, breast, lung, colon, pancreas
o Breast cancer: Most common metastases to stomach
• Gastric lymphoma
o Stomach most frequently involved organ in GI tract
• Accounts 50% of all GI tract lymphomas
o Majority are non-Hodgkin lymphomas (B-cell)
o Arise from mucosa associated lymphoid tissue
(MALT)in patients with chronic H. pylori gastritis
• Barium findings
o Malignant melanoma: "Bull's eye" or "target" lesions
• Centrally ulcerated submucosal masses
o Lobular breast cancer metastases
• Linitis plastica or "leather bottle" appearance: Loss
of distensibility of antrum & body with thickened
irregular folds
• Mucosal nodularity, ulceration & spiculation
(simulating peptic ulcers of ZES)
 ZOLLINGER-ELLISON
o Gastric lymphoma
• Diffusely thickened irregular folds, discrete ulcers,
ulcerated submucosal masses
• Low grade MALTlymphoma: Confluent
varying-sized nodules
• CT findings
o Markedly thickened gastric wall & mucosal folds
o "BullIseye" or "target" or giant cavitated lesions
• Thickened gastric folds & ulcers may simulate ZES
Extrinsic inflammation
• Example: Pancreatitis
• Mimic ZESdue to thickened gastric wall
3 Other gastritides
• Examples: Crohn & eosinophilic
24 • Early gastric Crohn: Multiple aphthous ulcers
• Eosinophilic: Mucosal nodularity, thickened folds
[PATHOLOGY
General Features
• General path comments
o Embryology-anatomy
• Islet cell tumor: Originate from embryonic
neuroectoderm
• Etiology
o Gastrinomas
• Arise from amine precursor uptake &
decarboxylation (APUD) cells of islet of
Langerhans
o Pathogenesis
• Gastrinoma: t Gastrin levels ~ t gastric acid
secretions ~ peptic ulcers
• Epidemiology: Accounts 0.1-1 % of pancreatic tumors
• Associated abnormalities
o 20-60% are associated with multiple endocrine
neoplasia (MEN I)
• MEN 1:Tumors of pituitary, parathyroid, adrenal
cortex & pancreas
Gross Pathologic & Surgical Features
• Tumors: Encapsulated & firm; cystic, necrotic, Ca++
• Ulcers: Round or oval; sharply punched out walls
Microscopic Features
• Gastrinoma: Sheets of small round cells with uniform
nuclei & cytoplasm
• Ulcers: Necrotic debris, zone of granulation tissue
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Pain, increased
acidity, severe reflux, diarrhea, upper GI tract ulcers
• Lab-data: Secretin injection test
o Paradoxical increase in serum gastrin to > 200 pg/ml
above base levels in 90% of cases
• Diagnosis
o Gastrinoma & peptic ulcers on imaging
o Hypergastrinemia is hallmark of ZES
• Serum gastrin levels of more than 1,000 pg/ml
(virtually diagnostic of ZES)
SYNDROME
Demographics
• Age: Any age group (more commonly 4th-5th decade)
• Gender: M > F
Natural History & Prognosis
• Complications
o Gastrinoma: t Risk of malignancy, metastases
o Peptic ulcer: Perforation
• Prognosis
o Good: After surgical resection of primary gastrinoma
& stomach
o Poor: Gastrinoma + liver metastases; post-operative
recurrent ulcers
Treatment
• Medical: Cimetidine, ranitidine, famotidine,
omeprazole
• Surgical: Gastrinoma resection; total gastrectomy
• Liver metastases: Chemotherapy & hepatic artery
embolization
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of gastric wall thickening &
ulceration
Image Interpretation Pearls
• Hypervascular pancreatic tumor, liver metastases with
multiple ulcers & thickened folds of stomach,
duodenum & jejunum
[SELECTED REFERENCES
1. Sheth S et al: Imaging of uncommon tumors of the
pancreas. Radiol Clin North Am. 40(6):1273-87, vi, 2002
2. Sheth S et al: Helical CT of islet cell tumors of the pancreas:
typical and atypical manifestations. AJRAm J Roentgenol.
179(3):725-30,2002
3. Nino-Murcia M et al: Multidetector-row CT and volumetric
imaging of pancreatic neoplasms. Gastroenterol Clin North
Am. 31(3):881-96, 2002
4. Rodallec M et al: Helical CT of pancreatic endocrine
tumors. J Comput Assist Tomogr. 26(5):728-33, 2002
5. Oshikawa 0 et al: Dynamic sonography of pancreatic
tumors: comparison with dynamic CT. AJRAm]
Roentgenol. 178(5):1133-7,2002
6. Rodallec M et al: Helical CT of pancreatic endocrine
tumors. J Comput Assist Tomogr. 26(5):728-33, 2002
7. Fidler JL et al: Imaging of neuroendocrine tumors of the
pancreas. Int J Gastrointest Cancer. 30(1-2):73-85, 2001
8. Ichikawa T et al: Islet cell tumor of the pancreas: biphasic
CT versus MR imaging in tumor detection. Radiology.
216(1):163-71, 2000
9. Buetow PC et al: Islet cell tumors of the pancreas: clinical,
radiologic, and pathologic correlation in diagnosis and
localization. Radiographics. 17(2):453-72; quiz 472A-472B,
1997
10. Van Hoe L et al: Helical CT for the preoperative
localization of islet cell tumors of the pancreas: value of
arterial and parenchymal phase images. AJRAm J
Roentgenol. 165(6):1437-9, 1995
11. Eelkema EA et al: CT features of nonfunctioning islet cell
carcinoma. AJRAmJ Roentgenol. 143(5):943-8, 1984
 ZOLLINGER-ELLISON SYNDROME

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
thickened hypervascular
gastric folds from Z-E
syndrome. (Right) Axial
CECT (arterial phase) shows
small hypervascular mass
(arrow) in pancreatic head, a
gastrinoma.

3
25

Typical
(Left) Axial CECT (portal
venous phase) shows small
hypodense mass (arrow) in
pancreatic head and multiple
subtle liver metastases.
(Right) Axial CECT shows
fluid-distended intestine due
to Zollinger-Ellison
syndrome.

Typical
(Left) Upper GI series shows
markedly thickened folds in
stomach, duodenum, and
jejunum from
Zollinger-Ellison syndrome.
(Right) Axial NECT shows
numerous hepatic metastases
and thick gastric wall due to
Zollinger-Ellison syndrome.
 MENETRIER DISEASE

3
26

Upper CI series shows massive fold thickening in gastric Upper CI series shows gross, tortuous gastric fold
fundus and body due to Menetrier disease. thickening in fundus and body with poor gastric coating
by the barium.

• Hypoproteinemia: Protein loss (gastric mucosa)

ITERMINOlOGY o Variations do occur: Mucosal hypertrophy may be
Abbreviations and Synonyms associated with
• Hyperplastic gastropathy, giant hypertrophic or cystic • Hyperproteinemia, hyperchlorhydria or normal
gastritis, giant mucosal hypertrophy protein & HCllevels
o Menetrier disease: E.g., of hypertrophic gastropathy
Definitions o Irreversible in most of adult patients, whereas in
• Characterized by large, tortuous gastric mucosal folds, children it usually resolves spontaneously
which may be localized or may involve whole stomach
Radiographic Findings
• Fluoroscopic guided double-contrast study
I IMAGING FINDINGS o Grossly thickened, lobulated folds in gastric fundus
& body with relative sparing of antrum
General Features o May show thickened gastric folds even in antrum
• Best diagnostic clue: Grossly thickened, lobulated folds o Focal area of rugal hypertrophy on greater curvature
in gastric fundus & body, with poor barium coating o Giant, mass-like elevation of folds on greater
• Location curvature of gastric body mimicking polypoid cancer
o Stomach o Stomach remains pliable & distensible
• Throughout gastric fundus (most common) o Excessive mucus may dilute barium & compromise
• Body (particularly along greater curvature) mucosal coating
• Antrum (usually spared) o Rare variant of Menetrier disease: Thickened,
• Morphology: Large, thickened, tortuous gastric folds nodular folds in proximal duodenum
• Other general features CT Findings
o Rare condition of unknown cause
• Markedly thickened gastric wall with mass-like
o Characterized by
elevations (giant, heaped-up folds)
• Marked foveolar hyperplasia in stomach
• Enlarged gastric rugae Imaging Recommendations
• Hypochlorhydria (HCl output t in 75% of cases) • Barium double-contrast studies; helical CT

DDx: Diffuse or Focal Thickening of Gastric Wall

Gastritis Lymphoma Gastric Carcinoma Pancreatitis

 MENETRIER DISEASE

Key Facts
Terminology Top Differential Diagnoses
• Hyperplastic gastropathy, giant hypertrophic or cystic • H. pylori gastritis
gastritis, giant mucosal hypertrophy • Gastric metastases & lymphoma
• Gastric carcinoma
Imaging Findings
• Extrinsic inflammation (e.g.,: Pancreatitis)
• Grossly thickened, lobulated folds in gastric fundus & • Other gastritides
body with relative sparing of antrum
• Giant, mass-like elevation of folds on greater Diagnostic Checklist
curvature of gastric body mimicking polypoid cancer • Check for hypoproteinemia & i HCI with biopsy

I DIFFERENTIAL DIAGNOSIS Natural History & Prognosis

3
• Complications 27
H. pylori gastritis
o Gastric carcinoma develops in about 10% of patients
• Thickened, lobulated folds favors antrum o Increased risk of deep venous thrombosis (DVT)
• Diagnosis: Endoscopic biopsy; culture; urease test o Risk of atrophic gastritis, gastric ulcer, GIT bleeding
Gastric metastases & lymphoma • Prognosis
• Lobular breast cancer metastases o Prolonged illness with intractable symptoms
• Gastric lymphoma o Spontaneous remission & few respond to treatment
o Thickened gastric folds with mucosal nodularity Treatment
Gastric carcinoma • Medical therapy: Anticholinergic agents, antibiotics
• May cause mass-like elevated thickened folds on • Total gastrectomy & vagotomy (unresponsive cases)
greater curvature; gastric peristalsis often absent
Extrinsic inflammation (e.g.,: Pancreatitis) I DIAGNOSTIC CHECKLIST
• Mimic Menetrier due to thickened gastric wall
• CT will show peri pancreatic inflammation Consider
• Check for hypoproteinemia & i HCI with biopsy
Other gastritides
• Examples: Advanced Crohn & eosinophilic
• Thickened folds, large ulcers, mucosal nodularity I SELECTED REFERENCES
• Usually involves antrum or antrum & body 1. Fishman EK et al: CT of the stomach: Spectrum of disease.
RadioGraphies 16: 1035-54, 1996
2. Wolfsen HC et al: Menetrier's disease: A form of
I PATHOLOGY hypertrophic gastropathy (or) gastritis. Gastroenterology
104: 1310-9, 1993
General Features 3. Reese DF et a1:Giant hypertrophy of the gastric mucosa
• Etiology (Menetrier's disease): A correlation of the
o Unknown roentgenographic, pathologic and clinical findings. AJR 88:
o Mucosal thickening (massive foveolar hyperplasia) 619-26, 1962

Gross Pathologic & Surgical Features

• Large, thickened, tortuous gastric mucosal folds I IMAGE GAllERY
Microscopic Features
• Cystic dilatation, elongated gastric mucous glands
• Atrophy of chief/parietal cells; deepening foveolar pits

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Epigastric pain, vomiting, diarrhea, weight loss
o Occasionally peripheral edema (hypoproteinemia)
• Lab: i Albumin; i or absent HCI; ± fecal occult blood
• Diagnosis: Endoscopic full-thickness biopsy (Left) Upper CI series shows massive fold thickening, sparing only the
antrum. (Right) Upper CI series shows massive gastric fold thickening
Demographics
and poor coating by barium.
• Age: Usually occur in older people (range 20-70 years)
• Gender: M > F
 CAUSTIC GASTRODUODENAL INJURY

3
28

Upper CI series shows nondistensible, small Upper CI series shows extremely small, non-distensible,
nonperistaltic, featureless stomach due to prior ingestion distorted stomach due to ingestion of hydrochloric acid.
of hydrochloric acid.

ITERMINOLOGY Radiographic Findings

• Radiography
Abbreviations and Synonyms
o Dilated & gas-filled stomach
• Corrosive gastroduodenitis o Fulminating cases
Definitions • Streaky, bubbly or mottled intramural gas
• Gastroduodenal inflammation/injury due to acid or • Fluoroscopic guided water-soluble contrast studies
alkali o Acute mild phase
• Atonic dilated stomach ± proximal duodenum
• Multiple shallow, irregular ulcers
IIMAGING FINDINGS o Acute severe phase
• Thickened folds, extensive deep ulceration
General Features • Severe pylorospasm + delayed emptying
• Best diagnostic clue: Grossly abnormal stomach with • Mural defects (due to edema & hemorrhage)
intramural dissection of contrast & mural defects • Intramural dissection of contrast or loculated
• Location: Lesser curvature & distal antrum of stomach perigastric collections
• Other general features • ± Reveal free perforation into peritoneal cavity
o Esophagus is most often injured within GI tract o Chronic phase
• Classically damaged by strong alkaline agents • Narrowing/deformity of stomach ± duodenal bulb
• Most commonly used alkali in US: Liquid lye • Antrum may be smooth + tubular configuration
o After esophagus, gastroduodenal injury> common • Gastric outlet obstruction (antral scarring/fibrosis)
• Most likely to be damaged by strong acids • Antral scarring mimics scirrhous carcinoma
• Commonly used acids: Hydrochloric & sulfuric • Duodenal bulb & sweep may appear normal due
• Caustic agents cause intense pylorospasm, so to severe pylorospasm or marked antral scarring
duodenal injury is less common • Rarely strictures between bulb-ligament of Treitz
o Classification based on clinical/radiological findings
CT Findings
• Acute & chronic phases
• Acute severe phase: Pneumoperitoneum (perforation)
• Mild & severe injury patterns
• Chronic phase: Luminal irregularity & narrowing

DDx: Small, Non-Distensible Stomach

Gastric Carcinoma Gastric Carcinoma Gastric Lymphoma Post Freezing

 CAUSTIC GASTRODUODENAL INJURY

Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Grossly abnormal stomach with • Gastric carcinoma (scirrhous type)
intramural dissection of contrast & mural defects • Gastric metastases & lymphoma
• Atonic dilated stomach ± proximal duodenum • Gastric thermal injury
• Thickened folds, extensive deep ulceration
• Severe pylorospasm + delayed emptying Diagnostic Checklist
• Narrowing/deformity of stomach ± duodenal bulb • Check for history of strong acid or alkali ingestion
• Antrum may be smooth + tubular configuration • Thickened folds, ulceration, atony, spasm & stricture
• Antral scarring mimics scirrhous carcinoma

Imaging Recommendations Natural History & Prognosis

3
• Videofluoroscopic water-soluble contrast studies • Complications 29
o Outlet obstruction, perforation, peritonitis, shock
o Increased risk of cancer after 20-40 years
I DIFFERENTIAL DIAGNOSIS • Prognosis
o Acute mild phase with early treatment: Good
Gastric carcinoma (scirrhous type) o Acute severe & chronic phases: Poor
• Usually arise near pylorus & extend up
• Diffuse linitis plastic a mimics caustic gastric injury Treatment
o Nodularity, spiculation, ulceration, thickened folds • Conservative treatment for stable patients
o Esophageal injury & history favors caustic ingestion o Antibiotics, steroids, parenteral feedings
• Gastric outlet obstruction
Gastric metastases & lymphoma o Gastroenterostomy or partial gastrectomy
• Example: Lobular breast carcinoma & non-Hodgkin
• Linitis plastica pattern simulate caustic injury
• Differentiated by breast primary, biopsy & history I DIAGNOSTIC CHECKLIST
Gastric thermal injury Consider
• Example: Post freezing, when iced saline infusions • Check for history of strong acid or alkali ingestion
used for bleeding varices
Image Interpretation Pearls
• Thickened folds, ulceration, atony, spasm & stricture
I PATHOLOGY
General Features I SELECTED REFERENCES
• General path comments 1. Muhletaler CA et al: Acid corrosive esophagitis:
o Pathologically caustic injury occurs in three phases radiographic findings. AJR 134: 1137-1140, 1980
• Acute necrotic phase (after 1-4 days) 2. Franken EA: Caustic damage of the gastrointestinal tract:
• Ulceration-granulation phase (after 5-28 days) Roentgen features. AJR 118: 77-85, 1973
• Cicatrization & scarring (after 3-4 weeks) 3. Martel W: Radiologic features of esophagogastritis
• Etiology secondary to extremely caustic agents. Radiology 103:
o Alkali: Liquid lye (concentrated sodium hydroxide) 31-36, 1972
• Pathogenesis: Injury by liquefaction necrosis
o Acids: HCI, sulfuric, acetic, oxalic, nitric, carbolic
• Pathogenesis: Injury by coagulative necrosis I IMAGE GALLERY
• Associated abnormalities: Esophageal injury
Gross Pathologic & Surgical Features
• Hyperemia/inflammation/necrosis/ulceration/ strictures
Microscopic Features
• Thinning of epithelium, inflammatory cells, cellular
hyperplasia & areas of necrosis

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms (Left) Axial CECT shows dilated esophagus with thickened wall and
o Severe abdominal pain, nausea, vomiting aspiration pneumonitis. Lye ingestion. (Right) Axial CECT shows
o Hematemesis, fever & shock gastric wall thickening + submucosal edema due to lye ingestion.
 DUODENAL HEMATOMA AND LACERATION

3
30

Axial CECT of duodenal perforation from blunt trauma. Axial CECT of duodenal traumatic perforation. Note
Note ectopic gas and fluid in right anterior pararenal large ectopic gas collection adjacent to duodenum
space (arrows). (arrow).

ITERMINOLOGY o Free air and fluid in peritoneal cavity or anterior

pararenal space
Definitions • CECT: Nonenhancing intramural hematoma; active
• Trauma to duodenum resulting in intramural extravasation from gastroduodenal artery; interruption
hematoma or laceration of duodenal wall; ectopic gas/fluid; periduodenal
stranding
MR Findings
I IMAGING FINDINGS • Tl WI: High signal intramural hematoma
General Features • T2WI
o High signal free fluid
• Best diagnostic clue: High attenuation intramural
hematoma, ectopic gas, fluid in peritoneal cavity or o High signal hematoma
anterior pararenal space • Tl C+
• Morphology: "Dumbbell-shaped" intramural o Wall thickening of duodenum
hematoma o Nonenhancing hematoma

Radiographic Findings Ultrasonographic Findings

• Radiography • Echogenic intramural mass representing hematoma
o Free air (pneumoperitoneum or ectopic Angiographic Findings
retroperitoneal gas) • Conventional: Selective if active bleeding
o Free fluid extravasation from gastroduodenal artery
• Fluoroscopy: GI series: Narrowing of duodenal lumen
by intramural hematoma; extravasation of oral Imaging Recommendations
contrast into peritoneal cavity or retroperitoneum • Best imaging tool: CECT, UGI
CT Findings
• NECT
o High attenuation intramural duodenal hematoma

DDx: lesions Mimicking Duodenal Hematoma

Perf Duodenal Ulcer Villous Adenoma Duodenal Lymphoma

 DUODENAL HEMATOMA AND LACERATION

Key Facts
Imaging Findings Clinical Issues
• Best diagnostic clue: High attenuation intramural • Clinical profile: Child with midepigastric blunt
hematoma, ectopic gas, fluid in peritoneal cavity or trauma, adult with high speed motor vehicle accident
anterior pararenal space injuries
• Best imaging tool: CECT, UGI • Non-operative management for isolated duodenal
hematoma with perforation
Pathology • Surgery for duodenal perforation and associated head
• 4th most common organ injury in children of pancreas injury

I DIFFERENTIAL DIAGNOSIS Treatment

3
• Options, risks, complications 31
Perforated duodenal ulcer
o Non-operative management for isolated duodenal
• Ectopic gas or fluid in peritoneal cavity or anterior hematoma with perforation
pararenal space o Surgery for duodenal perforation and associated
• Periduodenal inflammatory changes head of pancreas injury
• Mural thickening of duodenum
Villous adenoma
• Polypoid mucosal mass 3-9 cm; rarely causes I DIAGNOSTIC CHECKLIST
obstruction Consider
Duodenal lymphoma • Perforated duodenal ulcer
• Most often extension of gastric lymphoma Image Interpretation Pearls
• Bulky submucosal mass
• Ectopic gas/fluid in pararenal space

I PATHOLOGY I SELECTED REFERENCES

General Features 1. Desai KM et al: Blunt duodenal injuries in children. ]
• General path comments: Intramural duodenal Trauma. 54(4):640-5; discussion 645-6, 2003
hematoma 2. Zissin R et al: Pictorial review. CT of duodenal pathology.
Br] Radiol. 75(889):78-84, 2002
• Epidemiology
3. Degiannis E et al: Duodenal injuries. Br] Surg.
o 4th most common organ injury in children 87(11):1473-9,2000
o 2-10% of all blunt injuries 4. Lorente-Ramos RM et al: Sonographic diagnosis of
• Associated abnormalities intramural duodenal hematomas. ] Clin Ultrasound.
o Pancreatic laceration (47%) or fracture 27(4):213-6, 1999
o Liver or splenic laceration (16-32%) 5. Weigelt]A: Duodenal injuries. Surg Clin North Am.
70(3):529-39, 1990
Staging, Grading or Classification Criteria
• Isolated intramural hematoma
• Perforated duodenum I IMAGE GALLERY
• Combined head of pancreas and duodenal injury

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Nausea, vomiting,
abdominal pain/tenderness
• Clinical profile: Child with midepigastric blunt
trauma, adult with high speed motor vehicle accident
injuries
Natural History & Prognosis
• Isolated intramural hematoma has excellent prognosis
(Left) Axial CECT of duodenal perforation secondary to trauma. Note
with non-operative management mural thickening of duodenum (open arrow) and ectopic gas bubble
• Combined duodenal perforation with head of (arrow). (Right) Axial CECT of duodenal perforation secondary to
pancreas laceration has morbidity of 26% trauma. Note para duodenal hematoma (arrow).
 GASTRIC POLYPS

3
32

Graphic shows pedunculated polyp in gastric antrum, Upper GI series shows a polypoid mass (arrow) in the
prone to prolapse through pylorus with peristalsis. antrum that periodically prolapsed through the pylorus.
Leiomyoma.

ITERMINOlOGY • Typical & atypical (large & giant); virtually no

malignant potential
Definitions • Typical: Small, multiple, sessile « 1 cm); location
• A protruding, space-occupying, epithelial lesion (fundus & body)
within stomach • Atypical Large: Solitary, pedunculated (2-6 cm);
location (body & antrum)
• Atypical giant: Polyp (6-10 cm) multilobulated
I IMAGING FINDINGS mass; location (antrum & body)
• 8-28% associated with atrophic gastritis,
General Features pernicious anemia & cancer
• Best diagnostic clue: Radiolucent filling defects, ring • Fundic gland polyps: Variant of hyperplastic
shadows or contour defect on barium study polyps « 1 cm)
• Morphology o Adenomatous polyps
o Hyperplastic polyps: Smooth, sessile, pedunculated • Less common « 20%); dysplastic lesions
o Adenomatous polyps: Usually single with lobulated • 1 Risk of malignant change via
or cauliflower-like surface adenoma-carcinoma sequence
o Hamartomas: Cluster of broad based polyps • Usually solitary, occasionally multiple, > 1 cm;
• Other general features location (antrum)
o 85-90% of gastric neoplasms are benign • Histologically: Tubular (75%); tubulovillous
• 50% Mucosal & 50% submucosal (15%); villous (10%)
o Gastric polyps are mucosal lesions • Gastric adenomatous polyps 30 times < common
o More common in hereditary polyposis syndromes than gastric cancer
o Polyps classified into three types based on pathology • Carcinoma in situ & invasive carcinoma: Seen in
• Hyperplastic, adenomatous & hamartomatous 50% of polyps> 2 cm
o Hyperplastic polyps • 30-40% associated with: Atrophic gastritis,
• Most common benign epithelial neoplasms of pernicious anemia & cancer
stomach (80-90%) • 1 Risk of coexisting gastric cancer more than risk
of malignant change in polyp

DDx: Discrete Filling Defect(s) in Stomach

Gastric Carcinoma Met. Melanoma Carcinoid Tumors Ectopic Pancreas

 GASTRIC POLYPS
Key Facts
Terminology
• A protruding, space-occupying, epithelial lesion
within stomach
Imaging Findings
• Best diagnostic clue: Radiolucent filling defects, ring
shadows or contour defect on barium study
• Dependent (posterior wall): Radiolucent filling
defects
• Nondependent (anterior wall): Ring shadows + white
rim (barium)
• "Mexican hat" sign: Characterized by a pair of
concentric rings
Top Differential Diagnoses
• Retained food & pills
• Gastric carcinoma (polypoid type)
o Polyposis syndromes involving stomach
• Familial adenomatous polyposis syndrome (FAPS):
Seen in > 50% cases of gastric polyps
• Hamartomatous polyposis: Example: (PJS)
Peutz-Jeghers syndrome (10-15% of gastric polyp
cases)
Radiographic Findings
• Fluoroscopic guided double-contrast barium study
• Hyperplastic polyps
o Typical: Multiple, smooth, sessile, round or ovoid
lesions, < 1 cm in size
o Based on location: Dependent & nondependent wall
• Dependent (posterior wall): Radiolucent filling
defects
• Nondependent (anterior wall): Ring shadows +
white rim (barium)
o Variant: Fundic gland polyps (multiple up to 50 in
fundus, < 1 cm in size)
• Small rounded nodules, indistinguishable from
hyperplastic polyps
o Atypical: Large & giant
• Large: Solitary, conglomerated, pedunculated,
lobulated, 2-6 cm in size
• Giant polyps: Multilobulated conglomerate mass +
trapping of barium in interstices between lobules;
6-10 cm in size
• Atypical antral large & giant pedunculated polyps
may prolapse
• Polyp prolapse ~ pylorus ~ duodenum leads to
gastric outlet obstruction
• Adenomatous polyps
o Usually solitary or rarely multiple; sessile or
pedunculated; more lobulated; > 1 cm in size
o Pedunculated polyp en face: Hanging from
non dependent anterior wall
• "Mexican hat" sign: Characterized by a pair of
concentric rings
• Outer ring: Represents head of polyp
• Inner ring: Represents stalk of polyp
o Lobulated polyp with basal indentation: 1 Risk of
adenocarcinoma
• Polyposis syndromes involving stomach
• Gastric metastases & lymphoma
• Gastric stromal tumor
• Ectopic pancreas
Pathology
• General path comments: Proliferation of mucosa
• Chronic atrophic & H. pylori gastritis
• Hereditary: Autosomal dominant (FAPS& PJS)
Diagnostic Checklist
• Check for family history of GI tract polyps
• Hyperplastic polyps (typical): Multiple, smooth,
sessile, round or ovoid lesions, < 1 cm in size
• Adenomatous polyps: Solitary, sessile or
3
pedunculated, more lobulated & > than 1 cm in size 33
• Large, solitary, sessile polyp with lobulated surface &
basal indentation, highly suggests adenocarcinoma
o Familial adenomatous polyposis syndrome (FAPS)
• Fundic gland polyps & adenomas (> 50% cases)
• Seen as multiple small filling defects
o Peutz-Jeghers syndrome (hamartomatous polyposis)
• Cluster of polyps (10-15% of gastric polyp cases)
Imaging Recommendations
• Best imaging tool
o Upper gastrointestinal double-contrast barium study
o En face, profile & oblique views
I DIFFERENTIAL DIAGNOSIS
Retained food & pills
• Filling defects in barium pool simulating polyps
Gastric carcinoma (polypoid type)
• Lobulated or fungating mass
• Barium study findings
o Dependent or posterior wall: Filling defect
o Nondependent or anterior wall
• Etched in white by a thin layer of barium
• Indistinguishable from giant lobulated hyperplastic or
adenomatous polyp
• Diagnosis: Endoscopic biopsy & histology
Gastric metastases & lymphoma
• Gastric metastases: Example: Malignant melanoma &
squamous cell carcinoma
• Gastric lymphoma: Example: Low grade MALT
lymphoma
o MALT:Mucosa-associated lymphoid tissue
• Barium study findings
o Malignant melanoma metastases
• Initially: Submucosal masses seen as filling defects
may mimic polyps
• Ulcerated lesions: "Bull's eye" or "target" pattern
o Low grade MALTlymphoma
• Confluent varying-sized nodules (filling defects)
• May be indistinguishable from gastric FAPS
Gastric stromal tumor
• Submucosal lesions
 GASTRIC POLYPS
• Example: Leiomyoma
• Non-ulcerated leiomyoma
o In profile
• Smooth surface etched in white
• Borders: Right or obtuse angles with adjacent wall
o En face
• Seen as a filling defect simulating polyp
• Intraluminal surface: Abrupt well-defined borders
• Diagnosis: Endoscopic biopsy & histology
Ectopic pancreas
• Submucosal lesion
• Seen as smooth, broad-based submucosal mass
o Indistinguishable from a gastric polyp
3 • Location: Greater curvature of distal antrum
• Often contain a central umbilication or dimple
34 o Represents orifice of a primitive ductal system
• May present as a "bull's eye" appearance
• Rarely, due to barium reflux into rudimentary ducts
may produce club shaped pouches (pathognomonic)
I PATHOLOGY
General Features
• General path comments: Proliferation of mucosa
• Genetics
o FAPS: Abnormal or deletion of APC gene located on
chromosome Sq
o Hamartomatous polyposis: Peutz-]eghers syndrome
• Spontaneous gene mutation on chromosome 19
• Etiology
o Chronic atrophic & H. pylori gastritis
o Hereditary: Autosomal dominant (FAPS & P]S)
• Familial adenomatous polyposis syndrome
• Hamartomatous polyposis syndromes
• Epidemiology
o Incidence
• Gastric polyps: 1-2% of all GI tract polyps
• Giant hyperplastic polyps (2% of all hyperplastic)
• FAPS & P]S: 1 in 10,000 people
• Associated abnormalities: Polyposis syndromes
Gross Pathologic & Surgical Features
• Hyperplastic polyps: Small, sessile nodules; smooth,
dome-shaped contour
• Adenomatous polyps: Tubular (thin stalk + tufted
head); villous (broad base)
• FAPS: Innumerable small-medium sized polyps
• P]S: Carpet, cluster-like or scattered polyps
Microscopic Features
• Hyperplastic polyps: Elongated, cystically dilated
glandular structures
• Adenomatous polyps: Tubular, tubulovillous, villous
pattern; dysplastic cells
• P]S: Muscularis mucosa core extends ~ lamina propria
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Usually asymptomatic
o Ulcerated polyps: Low grade upper GI bleeding
o
FAPS: Rectal bleeding & diarrhea
o
P]S: Cramping pain, rectal bleeding or melena
o
Mostly incidental findings on imaging & endoscopy
o
Pedunculated polyps in antrum: Nausea & vomiting
• Due to outlet obstruction
• Diagnosis: Endoscopic biopsy & histology
Demographics
• Age
o Hyperplastic polyps: Middle & elderly age group
o FAPS & P]S: 10-30 years
• Gender: Equal in both males & females
Natural History & Prognosis
• Complications
o Risk of cancer in adenomatous polyp, FAPS & P]S
o Gastric outlet obstruction
• Prognosis
o Good: After removal of benign + cancer in situ polyp
o Poor: Invasive carcinoma
Treatment
• Small < 1 cm & asymptomatic: Periodic surveillance
• Large> 1 cm; sessile or pedunculated; lobulated &
symptomatic: Polypectomy
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other gastric discrete filling defects
• Check for family history of GI tract polyps
• Screen rest of GI tract to rule out associated hereditary
polyposis syndromes
Image Interpretation Pearls
• Hyperplastic polyps (typical): Multiple, smooth,
sessile, round or ovoid lesions, < 1 cm in size
• Adenomatous polyps: Solitary, sessile or pedunculated,
more lobulated & > than 1 cm in size
• Large, solitary, sessile polyp with lobulated surface &
basal indentation, highly suggests adenocarcinoma
I SELECTED REFERENCES
1. Insko EKet al: Benign and malignant lesions of the
stomach: evaluation of CT criteria for differentiation.
Radiology. 228(1):166-71, 2003
2. Ba-Ssalamah A et al: Dedicated multidetector CT of the
stomach: spectrum of diseases. Radiographies.
23(3):625-44, 2003
3. Cherukuri R et al: Giant hyperplastic polyps in the
stomach: radiographic findings in seven patients. AjR Am J
Roentgenol. 175(5):1445-8,2000
4. Cho GJ et al: Peutz-Jeghers syndrome and the
hamartomatous polyposis syndromes:
radiologic-pathologic correlation. Radiographies.
17(3):785-91, 1997
5. Harned RKet al: Extracolonic manifestations of the
familial adenomatous polyposis syndromes. AJRAm J
Roentgenol. 156(3):481-5, 1991
6. Feczko PJ et al: Gastric polyps: radiological evaluation and
clinical significance. Radiology. 155(3):581-4, 1985
7. Gordon R et al: Gastric polyps on routine double-contrast
examination of the stomach. Radiology. 134(1):27-9, 1980
 GASTRIC POLYPS

I IMAGE GALLERY
Typical
(Left) Upper CI series shows
polypoid mass in duodenal
bulb that is a prolapsed
gastric antral polyp
(adenoma). (Right)
Endoscopic photo shows
antral polyp (adenoma) that
intermittently prolapsed
through the pylorus (arrow).

3
35

Typical
(Left) Upper CI series shows
dozens of small hyperplastic
gastric polyps. (Right) Upper
CI series shows multiple
hyperplastic gastric polyps.

Typical
(Left) Upper CI series shows
large adenomatous gastric
polyp (arrow). (Right) Axial
CECT shows a large gastric
adenomatous polyp (arrow)
prolapsed into the
duodenum.
 DUODENAL POLYPS

3
36

Upper GI series shows large adenomatous polyp Upper GI series shows a polyp (arrow) in duodenal
(arrow) as a radiolucent filling defect. bulb, endoscopically resected and found to represent a
carcinoid tumor.

ITERMINOLOGY • Duodenum 2nd most common site of familial

Definitions
• Protruding, space-occupying, epithelial lesions
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Radiolucent filling defects, ring
shadows or contour defect on barium study
• Location: Usually first & second parts of duodenum
• Size: Adenomatous polyps: Few mm to 2 cm
• Morphology
o Adenomatous polyps (most common)
• Usually single with lobulated or cauliflower-like
o Hyperplastic polyps: Smooth, sessile, pedunculated
o Hamartomas: Cluster of broad based polyps
• Other general features
o Duodenal polyps are < common than gastric polyps
o Polyps classified into three types based on pathology
• Adenomatous, hyperplastic & hamartomatous
o Adenomatous polyps
• Most common polyps of duodenum
• Typically arise from medial wall of duodenum
• Occur in 47-72% of familial polyposis cases
• Increased risk of malignant change via
adenoma-carcinoma sequence
adenomatous polyposis (FAPS)after colon
• FAPScases: Clustered around periampullary region
• Non-FAPS cases: Bulbar distribution
• Tubular (75%); tubulovillous (15%); villous (10%)
• Duodenal carcinoma usually seen with adenoma
• 4% of patients develop periampullary carcinoma
in less than 5 years after colectomy
o Hyperplastic polyps
• Rare, benign epithelial neoplasms of duodenum
• Virtually no malignant potential
o Hamartomatous polyps
• Usually seen in Peutz-]egher syndrome (P]S)
• Duodenum most common after jejunum/ileum
Radiographic Findings
• Fluoroscopic guided double contrast barium study
• Adenomatous polyps
o Solitary or multiple tiny tubular adenomas
o Sessile or pedunculated, > lobulated, 5 mm or less
o Pedunculated polyp (en face)
• "Mexican hat" sign: Pair of concentric rings
o Lobulated polyp with basal indentation
• Increased risk of adenocarcinoma
o Fungating mass highly suggestive of carcinoma
• May obstruct distal common bile duct
• Hyperplastic polyps
o Typical: Small multiple, smooth, sessile, round or
ovoid lesions, less than 2 cm in size

DDx: Duodenal Polyps

,J
-- .
Brunner Glands
~.

Brunner Glands Brunner Glands Ectop. Gastric Mucosa

 DUODENAL POLYPS

Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Radiolucent filling defects, ring • Brunner gland hyperplasia
shadows or contour defect on barium study • Pseudopolyp
• Adenomatous polyps (most common) • Ectopic gastric mucosa
• FAPS cases: Clustered around periampullary region
• Non-FAPS cases: Bulbar distribution Diagnostic Checklist
• Sessile or pedunculated, > lobulated, 5 mm or less • Check for family history of GI tract polyps
• "Mexican hat" sign: Pair of concentric rings • Screen rest of GI tract to rule out polyposis
• Fungating mass highly suggestive of carcinoma syndromes

o Based on location: Dependent & nondependent

• Dependent (posterior wall): Filling defect
wall
Demographics
3
• Nondependent (anterior wall): Ring shadows + • Age: FAPS & P]S: 10-30 years 37
white rim (barium)
Natural History & Prognosis
o Atypical: Large or giant pedunculated or lobulated
• Complications: Risk of cancer in adenomatous polyps
Imaging Recommendations • Prognosis: Benign (good); invasive carcinoma (poor)
• Fluoroscopic guided double contrast barium study Treatment
o En face, profile & oblique views
• Small < 1 cm & asymptomatic: Periodic surveillance
• Large> 1 cm, lobulated, symptomatic: Polypectomy
I DIFFERENTIAL DIAGNOSIS
Brunner gland hyperplasia I DIAGNOSTIC CHECKLIST
• M,-<ftiple small, rounded nodules in duodenal bulb Consider
("cobblestone" or "Swiss cheese" appearance)
• Check for family history of GI tract polyps
• Brunner gland hamartomas
• Screen rest of GI tract to rule out polyposis syndromes
o Submucosal or sessile lesions mimicking polyps
o May also show large polypoid defects Image Interpretation Pearls
Pseudopolyp • Lobulated polyp + basal indentation (adenocarcinoma)
• Seen at apex of duodenal bulb due to acute bend
• Also seen in inflammatory & post inflammatory states
I SELECTED REFERENCES
Ectopic gastric mucosa 1. Waye JD et al: Approach to benign duodenal polyps.
• Discrete, angulated or polygonal 1-5 mm nodules Gastrointest Endosc. 55(7):962-3, 2002
(filling defects) near base of duodenal bulb 2. Harned RKet al: Extracolonic manifestations of the
familial adenomatous polyposis syndromes. AJRAm J
Roentgenol. 156(3):481-5, 1991
!PATHOlOGY
General Features I IMAGE GAllERY
• Genetics
o FAPS: Abnormal APC gene on chromosome 5q
o P]S: Spontaneous gene mutation on chromosome 19
• Etiology: Chronic duodenitis; hereditary (FAPS, P]S)
• Epidemiology: Less than 10/0of all GI tract polyps
• Associated abnormalities: Polyposis syndromes
Gross Pathologic & Surgical Features
• Adenomatous polyps: Sessile or pedunculated
Microscopic Features
• Adenomatous: Tubular, tubulovillous, villous pattern

(Left) Upper GI series in a patient with Gardner syndrome shows

ICLINICAllSSUES
Presentation
• Most common signs/symptoms: Asymptomatic,
grade upper GI bleeding, obstructive jaundice
multiple adenomatous polyps in duodenal bulb. (Right) Axial CECT in
a patient with Gardner syndrome shows a discrete ampullary tumor
(arrow). Liver metastases are from concurrent colon cancer.
low
 INTRAMURAL BENIGN GASTRIC TUMORS

3
38

Graphic shows a "generic" intramural gastric mass with Upper GI series shows a sharply defined submucosal
intact mucosa and acute to slightly obtuse angles at the mass with intact mucosa, except for a central ulceration
interface. (arrow). Benign stromal tumor.

o Borders form right angle or slightly obtuse angles

ITERMINOLOGY with adjacent gastric wall (profile view)
Definitions o Intraluminal surface of tumor has abrupt,
well-defined borders (en face view)
• Benign mass composed of one or more tissue elements
of the gastric wall o Usually intact overlying mucosai normal areae
gastricae pattern
o Focal areas of ulceration (60% of cases)
o "Bull's eye" or "target" lesions: Central barium-filled
I IMAGING FINDINGS crater within mass (ulceration)
General Features o Central dimple or spicule at apex of mass
• Best diagnostic clue: Intramural mass with smooth (exogastric)i differentiate from extrinsic mass
surface & slightly obtuse borders on barium studies o Pedunculatedi may prolapse into duodenum
• Other general features o ± Giant, cavitated lesionsi may simulate gastric
o Types of intramural benign gastric tumors lymphoma and gastric metastases from melanoma
• Gastrointestinal stromal tumor (GIST) o GIST
• Leiomyoma, leiomyoblastoma, schwan noma, • Most common; may occur anywhere in GI tract
neurofibroma, lipoma, hemangioma, • Several mm to 30 cm
lymphangioma • ± Extragastric extensions (86%): Gastrohepatic
ligament, gastrosplenic ligament, lesser sac
Radiographic Findings o Lipoma, lymphangioma: Tendency to change in size
• Radiography & shape by peristalsis or palpation
o Mass indenting gastric air shadow; ± calcifications o Schwannoma and neurofibroma: Multiple lesions
o Lipoma: Radiolucent shadow with associated abnormalities
o Hemangioma: Phleboliths (pathognomonic)
• Fluoroscopic-guided barium studies CT Findings
o Discrete mass; solitary (very common) or multiple • GIST
o Smooth surface lesion etched in white (double o Often large with central necrosis and ulceration of
contrast) (profile view) overlying mucosa

DDx: Intramural Gastric Mass

Pseudocyst Pseudocyst Splenosis Seroma

 INTRAMURAL BENIGN GASTRIC TUMORS

Key Facts
Terminology Top Differential Diagnoses
• Benign mass composed of one or more tissue • Gastric carcinoma
elements of the gastric wall • Gastric metastases and lymphoma
• Ectopic pancreatic tissues
Imaging Findings • Gastric or duodenal ulcer
• Best diagnostic clue: Intramural mass with smooth
surface & slightly obtuse borders on barium studies Pathology
• Discrete mass; solitary (very common) or multiple • Most diagnosed incidentally by imaging or autopsy
• Smooth surface lesion etched in white (double
contrast) (profile view)
Clinical Issues
• Borders form right angle or slightly obtuse angles • Asymptomatic (most common)
with adjacent gastric wall (profile view)
• "Bull's eye" or "target" lesions: Central barium-filled
Diagnostic Checklist
• GIST is most common; imaging criteria to separate
3
crater within mass (ulceration) from other intramural tumors are not well 39
• Best imaging tool: Barium studies followed by CT established, except for lipoma
• Smooth surface, right/slight obtuse angle with wall

o Hypo- or hypervascular well-circumscribed • Broad-based smooth, extra mucosal/intramural lesion

submucosal mass (arterial phase)
o Peripheral enhancement (92%)
o Central area of low attenuation (hemorrhage,
necrosis or cystic formation)
o ± Cavitation that communicates with gastric lumen
and contain air, air-fluid levels or oral contrast
o ± Homogeneous enhancement (8%)
o ± Calcification
• Lipoma
o Located commonly in gastric antrum
o Well-circumscribed areas of uniform fatty density
(-80 to -120 HU); definitive diagnosis
Imaging Recommendations
• Best imaging tool: Barium studies followed by CT
I DIFFERENTIAL DIAGNOSIS
Gastric carcinoma
• Usually appear as polypoid or circumferential mass
with irregularity of luminal surface
• Large, lobulated, mucosal, hypovascular mass, ±
ulceration
• Associated with perigastric or hepatoduodenal
ligament and celiac lymphadenopathy
• Mucinous adenocarcinomas: Punctate, granular or
finely stippled calcification
Gastric metastases and lymphoma
• Gastric metastases
o Examples: Breast (most common metastases to
stomach), colon, melanoma, lung, pancreas
o Discrete nodules to linitis plastica
• Gastric lymphoma (e.g., non-Hodgkin B-cell)
o In GI tract, stomach is the most common location
o Associated with bulky adenopathy or adenopathy
extends into lower abdomen and pelvis
• Multiple "bull's eye" lesions; unlike leiomyoma
Ectopic pancreatic tissues
• Located on greater curvature of distal antrum or
proximal duodenum
• Central barium collection present in orifice of
primitive ductal system; may simulate ulceration
• Usually small (5-10 mm)
Pancreatic pseudocyst
• Gastric compression; may simulate leiomyoma
• No central dimple or spicule at apex of mass suggests
mass is extrinsic, not intramural
• Use CT and US to help with diagnosis
Gastric or duodenal ulcer
• Gradual transition with adjacent mucosa
• Radiolucent mound of edema; may simulate ulcerated
leiomyoma
Hematoma/seroma
• May follow gastrostomy tube
I PATHOLOGY
General Features
• General path comments
o 50% of all benign tumors in stomach and
duodenum are intramural
o Most diagnosed incidentally by imaging or autopsy
o GIST
• Distinguish by immunoreactivity for c-KIT
(CD117), a tyrosine kinase growth factor receptor
• Most common intramural primary masses
• 70% of all GIST occur in stomach; 2-3% of all
gastric tumors
• Benign are 3x more common than malignant
o Leiomyoblastoma
• Predominantly in stomach, may affect small
bowel, retroperitoneum, uterus
• Also known as epithelioid leiomyomas
• Most are benign, 10% malignant (usually> 6 em)
o Lipoma
• 2-3% benign tumors in stomach, < in duodenum
• No malignant degeneration
• 5% of all GI lipomas are in stomach or duodenum
o Hemangioma
 INTRAMURAL BENIGN GASTRIC TUMORS
• < 2% benign tumors in stomach, < in duodenum • Schwannoma: Bundled spindle-shaped cells with
• Multiple hemangiomas in GI tract and/or skin distinctive lymphoid cuff that may contain germinal
• Classified as capillary or cavernous centers; stain for S-100 protein
• Sarcomatous changes rarely occur
o Lymphangioma: Affect anywhere, rare in GI tract
o Schwannoma and neurofibroma I CLINICAL ISSUES
• 5-10% of benign tumors in stomach
• Schwannoma (most common): Neurilemoma, Presentation
schwan noma or neuroma • Most common signs/symptoms
• Neurofibroma (less common): 10% undergo o Asymptomatic (most common)
malignant degeneration o Upper GI bleeding, nausea, vomiting, abdominal or
• Neurofibroma: Arise from sympathetic nerves of epigastric pain, weight loss, abdominal distention
Auerbach myenteric plexus (more common) or
Demographics
Meissner plexus
3 • Sarcomatous changes rarely occur
• Age: GIST: > 45 years of age
• Gender: GIST: M:F == 1:1
• Etiology
40 o GIST: KIT germ line mutations (52-85%) Natural History & Prognosis
o Hemangioma: Possible congenital malformation • Complications: Obstruction, intussusception,
• Associated abnormalities hemorrhage, catastrophic intraperitoneal bleeding
o GIST: Carney triad and von Recklinghausen disease • Prognosis: Good, unless patients with recurrence or
o Hemangioma: Telangiectasias of skin size> 5 cm
o Neurofibroma: von Recklinghausen disease
Treatment
Gross Pathologic & Surgical Features
• GIST
• May have central necrosis and ulceration o Surgical resection ± chemotherapy (Gleevec) for
• GIST metastatic disease
o Involves muscularis propria o Follow-up: Monitor indefinitely for recurrence
o Propensity for exogastric growth; mass arising from • Other types of tumors
gastric wall and project into abdominal cavity o No treatment if small and asymptomatic
o Mucosal ulceration on luminal surface (:0:: 50%) o Surgery if symptomatic/malignant; usually curative
o Well circumscribed mass that compresses adjacent
tissue and lacks a true capsule
o Pink, tan, or gray surface
o ±Focal areas of hemorrhage, cystic degeneration,
I DIAGNOSTIC CHECKLIST
necrosis and cavitation Consider
• Leiomyoma: Endogastric (80%), exogastric (15%) or • GIST is most common; imaging criteria to separate
"dumbbell-shaped" (5%) from other intramural tumors are not well established,
• Leiomyoblastoma: Smooth muscle tumors except for lipoma
• Lipoma: Endogastric (95%) or exogastric (5%) lesions
with superficial ulceration due to pressure necrosis Image Interpretation Pearls
• Hemangioma: Numerous tiny vascular structures • Smooth surface, right/slight obtuse angle with wall
(capillary) or large blood spaces or sinusoids lined by
endothelial tissue (cavernous)
• Lymphangioma: Cystic appearance with progressive I SELECTED REFERENCES
accumulation of fluid 1. Levy AD et al: Gastrointestinal stromal tumors: radiologic
features with pathologic correlation. Radiographies.
Microscopic Features 23(2):283-304, 456; quiz 532, 2003
• GIST 2. Pidhorecky I et al: Gastrointestinal stromal tumors: current
o Spindle cell (70-80%): Cigar-shaped cells, elongated diagnosis, biologic behavior, and management. Ann Surg
nuclei, eosinophilic to basophilic cytoplasm Oncol. 7(9):705-12, 2000
o Epithelioid (20-30%): Round polygonal cells, 3. Suster S: Gastrointestinal stromal tumors. Semin Diagn
centrally placed nuclei, cytoplasmic vacuolization Pathol. 13(4):297-313, 1996
o Variety of architectural patterns: Bundles of 4. Taylor AJ et al: Gastrointestinal lipomas: a radiologic and
pathologic review. AJRAmJ Roentgenol. 155(6):1205-10,
interlacing fascicles, nuclear palisading pattern, 1990
nesting organoid pattern and/or vascularity 5. Heiken JP et al: Computed tomography as a definitive
o Stromal portions of tumor may show extensive method for diagnosing gastrointestinal lipomas. Radiology.
perivascular or stromal hyalinization, myxoid 142(2):409-14, 1982
change or hemorrhage 6. Appleman HD et al: Gastric epithelioid leiomyoma and
o < 5 em in largest dimension with :0:: 5 mitoses per 50 leiomyosarcoma (leiomyoblastoma). Cancer. 38(2):708-28,
consecutive high power fields (HPF): Benign 1976
7. Faegenburg D et al: Leiomyoblastoma of the stomach.
o > 5 em with ~ 5 mitoses per 50 HPF: Malignant
Report of 9 cases. Radiology. 117(2):297-300, 1975
• Lipoma: Mature fat cells surrounded by fibrous capsule 8. Kerekes ES: Gastric Hemangioma: A case report. Radiology
82:468-9, 1964
 INTRAMURAL BENIGN GASTRIC TUMORS
I IMAGE GALLERY

(Left) Endoscopic
photograph shows a
submucosal benign gastric
stromal tumor with central
ulceration (arrow). (Right)
Upper GI series shows
gastric antral mass (stromal
tumor) with intact mucosa,
except for central ulcer
(arrow).

3
41

Typical
(Left) Axial CECT shows
benign gastric stromal tumor
(GIST) as an
intramural/exophytic mass
that deforms the greater
curvature/posterior wall.
(Right) Axial CECT shows a
discrete fat-density mass
(arrow) within the gastric
wall with intact, stretched
mucosa (lipoma).

Typical
(Left) Endoscopic
sonography shows
echogenic submucosal mass
(lipoma). (Right) Upper GI
series shows an antral
submucosal mass prolapsing
into the duodenum (lipoma).
 GASTRIC STROMAL TUMOR
\ \
, ~-
.
J
"",
v '\~
, 1;
//
1/
" ..• I

3
.~
42

Anatomic depiction of gastric stromal tumor. Note Axial CECT shows exophytic gastric GIST. Note
exophytic submucosal mass (arrow) with internal heterogeneous mural mass with smooth interface with
necrosis. stomach (arrow).

ITERMINOlOGY Radiographic Findings

• Fluoroscopy
Abbreviations and Synonyms
o UGI
• Gastrointestinal stromal tumor (GIST) • Rounded, exophytic, submucosal gastric mass
Definitions • Ulcerations common in larger masses
• Submucosal tumor of gastrointestinal (GI) tract CT Findings
derived from interstitial cells of Cajal
• NECT: Calcifications in 25% of cases
• CECT
o Hypo- or hypervascular well-circumscribed
I IMAGING FINDINGS submucosal mass on arterial phase images;
General Features ulceration & necrosis common on CECT
o Sensitivity 93%, specificity 100%
• Best diagnostic clue: Well-circumscribed submucosal
mass extending exophytic ally from GI tract MR Findings
• Location • Tl WI: Isointense mass
o Stomach most common site (2/3 of cases) • T2WI
o Small bowel (especially duodenum) next most o Hypo- to isointense submucosal mass
common site o Hyperintense areas of necrosis
o May occur anywhere in GI tract • T2* GRE: Hyper- or hypointense with IV gadolinium
o Rarely occurring in esophagus (leiomyoma more on GRE sequences
common) • Tl C+
• Size o Variable vascularity; may be hyper- or hypovascular
o Variable o Enhancement of solid areas
o Large mass may be > 5 cm o Nonenhancing necrotic or hemorrhagic areas
• Morphology
o Bulky, well-circumscribed and lobulated Ultrasonographic Findings
o Often exophytic, may have cystic element • Real Time: Hypoechoic mass

DDx: Spectrum of Gastric lesions Mimicking GIST

Sarcoma Carcinoma Lipoma

 GASTRIC STROMAL TUMOR

Key Facts
Imaging Findings Pathology
• Hypo- or hypervascular well-circumscribed • GIST are distinct, not synonymous with
submucosal mass on arterial phase images; ulceration leiomyoma/sarcoma, but may not be diagnosed by
& necrosis common on CECT light microscopy alone
• PET is superior to CT on predicting early response to
Gleevec; hypermetabolic foci for both primary tumor Clinical Issues
& mets • Most common signs/symptoms: Mass effect from
• Best imaging tool: CECT, PET bulky tumor, GI bleed when ulcerated, nausea,
vomiting, weight loss
Top Differential Diagnoses • Excellent prognosis for completely resected benign
• Gastric lymphoma lesions
•
•
Sarcoma invading stomach
Exophytic gastric carcinoma
• Good response to chemotherapy (Gleevec) in patients
with metastatic disease and c-KIT mutation 3
• Submucosal GI lipoma • Prognosis often depends on tumor size; poor if > 5 cm
43

• Color Doppler: Variable vascularity on color Doppler • Focal thickening of adjacent gastric wall and gastric
outlet obstruction help differentiate from GIST
Nuclear Medicine Findings • Often causes obstruction when circumferential
• PET
o PET is superior to CT on predicting early response to Submucosal Gllipoma
Gleevec; hypermetabolic foci for both primary • Fatty attenuation diagnostic
tumor & mets
o Sensitivity 86%, specificity 98%
Imaging Recommendations
I PATHOLOGY
• Best imaging tool: CECT, PET General Features
• Protocol advice • General path comments
o Prior to scanning, distend stomach with 16-32 oz o Bulky submucosal mass
water o Central ulceration common
o Use biphasic technique to cover entire liver • Genetics
• 150 ml IV contrast injected at 4-5 ml/sec o Express growth factor receptor with tyrosine kinase
• Arterial phase acquisition at 40 seconds, venous activity (c-KIT CD117)
phase at 70 seconds o Embryology-anatomy
• 2.5 mm collimation and 2.5-5 mm reconstruction • Of mesenchymal origin, not related to
interval leiomyomas or leiomyosarcomas
• Derived from interstitial cells of Cajal that help
regulate peristaltic activity (pacemaker function)
I DIFFERENTIAL DIAGNOSIS • Etiology: Unknown
• Epidemiology: Most common mesenchymal tumor of
Gastric lymphoma GI tract
• Early stage polypoid type • Associated abnormalities
• Nodular fold thickening on barium studies o Carney triad
• Exophytic mass without bowel obstruction • Malignant epithelial gastric GIST
• Associated mesenteric and retroperitoneal adenopathy • Pulmonary chondroma
• Bulky submucosal mass • Extra-adrenal paraganglioma
• May ulcerate o von Recklinghausen disease
• May be indistinguishable from GIST • Neurofibromatosis type 1
Sarcoma invading stomach Gross Pathologic & Surgical Features
• Bulky mass • Bulky submucosal mass
• Heterogeneous on CECT • Benign lesions typically small « 3 cm)
• Liposarcomas contain fat • Malignant features include invasion, size> 5 em, and
• Secondary invasion of bowel mimics GIST evidence of metastases
• Primary location in mesentery aids in differentiation
• Bowel obstruction common unlike GIST Microscopic Features
• GIST are distinct, not synonymous with
Exophytic gastric carcinoma leiomyoma/sarcoma, but may not be diagnosed by
• Hypodense mass less vascular than GIST light microscopy alone
• May be bulky and exophytic on CT/MR
 GASTRIC STROMAL TUMOR
• Benign or malignant mesenchymal spindle cell or tumor of the liver. Arch Pathol Lab Med. 127(12):1606-8,
epithelioid neoplasm without muscle differentiation 2003
• Malignant features include high mitotic rate (> 10 6. Un SC et al: Clinical manifestations and prognostic factors
in patients with gastrointestinal stromal tumors. World J
mitoses per 50 high power fields), high nuclear grade,
Gastroenterol. 9(12):2809-12, 2003
and high cellularity 7. Bechtold RE et al: Cystic changes in hepatic and peritoneal
Staging, Grading or Classification Criteria metastases from gastrointestinal stromal tumors treated
with Gleevec. Abdom Imaging. 28(6):808-14, 2003
• Four tumor subtypes 8. Kinoshita K et al: Endoscopic ultrasonography-guided fine
o Benign spindle cell GIST needle aspiration biopsy in follow-up patients with
o Malignant spindle cell GIST gastrointestinal stromal tumours. Eur J Gastroenterol
o Benign epithelial GIST Hepatol. 15(11):1189-93, 2003
o Malignant epithelial GIST 9. Rossi CR et al: Gastrointestinal stromal tumors: from a
surgical to a molecular approach. Int J Cancer.
107(2):171-6,2003
3 I CLINICAL ISSUES 10. Connolly EM et al: Gastrointestinal stromal tumours. Br J
Surg. 90(10):1178-86, 2003
11. Wu PC et al: Surgical treatment of gastrointestinal stromal
44 Presentation
tumors in the imatinib (STl-571) era. Surgery.
• Most common signs/symptoms: Mass effect from 134(4):656-65; discussion 665-6,2003
bulky tumor, GI bleed when ulcerated, nausea, 12. Tateishi U et al: Gastrointestinal stromal tumor.
vomiting, weight loss Correlation of computed tomography findings with tumor
• Clinical profile: No specific lab abnormality grade and mortality. J Comput Assist Tomogr. 27(5):792-8,
2003
Demographics 13. Reddy MP et al: F-18 FDG PET imaging in gastrointestinal
• Age: > 45 Y stromal tumor. Clin Nucl Med. 28(8):677-9, 2003
• Gender: No gender predilection 14. Dong Q et al: Epithelioid variant of gastrointestinal stromal
tumor: Diagnosis by fine-needle aspiration. Diagn
Natural History & Prognosis Cytopathol. 29(2):55-60, 2003
• Metastasizes to liver, lungs and peritoneal cavity 15. Wong NA et al: Prognostic indicators for gastrointestinal
stromal tumours: a clinicopathological and
• Excellent prognosis for completely resected benign
immunohistochemical study of 108 resected cases of the
lesions
stomach. Histopathology. 43(2):118-26, 2003
• Good response to chemotherapy (Gleevec) in patients 16. Frolov A et al: Response markers and the molecular
with metastatic disease and c-KIT mutation mechanisms of action of Gleevec in gastrointestinal
• 50-80% 5-year survival stromal tumors. Mol Cancer Ther. 2(8):699-709, 2003
• Prognosis often depends on tumor size; poor if > 5 cm 17. Besana-Ciani I et al: Outcome and long term results of
surgical resection for gastrointestinal stromal tumors
Treatment (GIST). ScandJ Surg. 92(3):195-9, 2003
• Surgery with en bloc resection 18. Ghanem N et al: Computed tomography in gastrointestinal
• Tyrosine kinase inhibitor chemotherapy (Gleevec) for stromal tumors. Eur Radiol. 13(7):1669-78, 2003
metastatic disease 19. Duffaud F et al: Gastrointestinal stromal tumors: biology
and treatment. Oncology. 65(3):187-97, 2003
20. RosaiJ: GIST: an update. Int] Surg Pathol. 11(3):177-86,
2003
I DIAGNOSTIC CHECKLIST 21. Burkill G] et al: Malignant gastrointestinal stromal tumor:
distribution, imaging features, and pattern of metastatic
Consider spread. Radiology. 226(2):527-32, 2003
• Consider lymphoma 22. Belloni M et al: Endoscopic ultrasound and Computed
Tomography in gastric stromal tumours. Radiol Med
Image Interpretation Pearls (Torino). 103(1-2):65-73,2002
• Exophytic hypervascular GI mass arising from 23. Miettinen M et al: Evaluation of malignancy and prognosis
submucosa with central ulceration of gastrointestinal stromal tumors: A review. Hum Pathol
33(5): 478-83, 2002
24. Kim C] et al: Gastrointestinal stromal tumors: Analysis of
clinical and pathologic factors. Am Surg 67(2): 135-7, 2001
I SELECTED REFERENCES 25. Shojaku H et al: Malignant gastrointestinal stromal tumor
1. Logrono R et al: Recent Advances in Cell Biology, of the small intestine: Radiologic-pathologic correlation.
Diagnosis, and Therapy of Gastrointestinal Stromal Tumor Radiat Med 5(3): 189-92, 1997
(GIST). Cancer BioI Ther. 2004
2. Antoch G et al: Comparison of PET, CT, and Dual-Modality
PET/CT Imaging for Monitoring of Imatinib (STl571)
Therapy in Patients with Gastrointestinal Stromal Tumors.
J Nucl Med. 45(3):357-365, 2004
3. Haider N et al: Gastric stromal tumors in children. Pediatr
Blood Cancer. 42(2):186-9, 2004
4. Gayed I et al: The role of 18F-FDG PET in staging and early
prediction of response to therapy of recurrent
gastrointestinal stromal tumors. J Nucl Med. 45(1):17-21,
2004
5. Hu X et al: Primary malignant gastrointestinal stromal
 GASTRIC STROMAL TUMOR

I IMAGE GALLERY
Typical
(Left) Axial CECTshows
ulcerated GIST. Note
rounded mural mass with
oral contrast extending into
area of ulceration (arrow).
(Right) Ulcerated GIST on
lateral view of UG/. Note
large accumulation of
barium within ulceration
(arrow).
3
45

(Left) GIST on AP view of

UG/. Note smooth interface
of mass with barium pool
(arrow). (Right) AP air
contrast view of GIST on
UGI demonstrates slight
lobulated contour of
submucosal mass (arrow).

Typical
(Left) Axial CECT of
contrast-filled stomach
demonstrates rounded mural
mass (arrow). (Right) Axial
CECT of GIST demonstrates
homogeneously enhancing
mural mass (arrow). The
intraluminal polypoid
component is less common
than an exophytic extension.
 GASTRIC CARCINOMA

3
46

Graphic shows large mass with broad base and irregular Upper GI series shows large mass (arrows) with abroad
surface. base and an irregular nodular surface.

!TERMINOLOGY Radiographic Findings

• Fluoroscopy
Definitions o Early gastric cancer (elevated,superficial, shallow)
• Malignancy arising from gastric mucosa • Type I: Elevated lesion-protrudes> 5 mm into
lumen (polypoid)
• Type II: Superficial lesion (plaque-like, mucosal
I IMAGING FINDINGS nodularity, ulceration)
• Type III: Shallow, irregular ulcer crater with
General Features
adjacent nodular mucosa &
• Best diagnostic clue: Polypoid or circumferential mass
clubbing/fusion/amputation of radiating folds
with no peristalsis through lesion
o Advanced gastric cancer
• Morphology: Polypoid, ulcerated, infiltrative lesions
• Polypoid cancer can be lobulated or fungating
• Other general features
• Lesion on dependent or posterior wall: Filling
o 3rd most common GI malignancy after colorectal &
defect in barium pool
pancreatic carcinoma
• Lesion on nondependent or anterior wall: Etched
o Adenocarcinoma (95%) is most common primary
in white by a thin layer of barium trapped
gastric tumor
between edge of mass & adjacent mucosa
o Environmental factors have a major role in
• Prolapsed polypoid antral carcinoma into
development of gastric cancer
duodenum: Seen as filling defect in barium pool
o Helicobacter pylori (3-6 fold 1 risk); pernicious
• Ulcerated carcinoma (penetrating cancer):
anemia (2-3 fold 1 risk)
Accounts for 70% of all gastric cancers
o Spread of gastric carcinoma
o Malignant ulcer (in profile)
• Direct spread • Malignant ulcer has an intraluminal location
• Lymphatic (left supraclavicular Virchow node)
within a tumor
• Hematogenous or transperitoneal: Krukenberg
• Tumor surrounding ulcer forms acute angle with
tumor (ovary); Blumer shelf (rectal wall)
gastric wall
• Clubbed/nodular folds seen radiating to edge of
ulcer crater

DDx: Diffuse or Focal Thickening of Gastric Wall

.': ...• -
.. :~.~"'~-';':':~,~, '
~
,.:-.. ,,;.

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. "' ..•.... -. -~:
<'.:'
h', ffL~'
!!!"'J /'

Benign Ulcer Lymphoma Gastric GIST Caustic Gastritis

 GASTRIC CARCINOMA
Key Facts
Terminology
• Malignancy arising from gastric mucosa
Imaging Findings
• Best diagnostic clue: Polypoid or circumferential mass
with no peristalsis through lesion
• Early gastric cancer (elevated,superficial, shallow)
• Polypoid cancer can be lobulated or fungating
• Ulcer: Irregular, scalloped, angular, stellate borders
• Carman-Kirkland meniscus complex (lesser curvature
antrum or body)
• Irregular narrowing of stomach + nodularity +
mucosal spiculation
• Advanced cases: May cause gastric outlet obstruction
• Linitis plastica or "leather bottle": Irregular narrowing
& rigidity (scirrhous carcinoma)
o Malignant ulcer (en face)
• Ulcer: Irregular, scalloped, angular, stellate borders
• Converging folds to ulcer: Blunted, nodular,
clubbed, fused
• Ulcer on nondependent or anterior wall:
Double-ring shadow (outer ring represents edge of
tumor & inner ring represents edge of ulcer)
• Prone compression view: Demonstrate filling of
ulcer crater within discrete tumor on anterior wall
o Carman-Kirkland meniscus complex (lesser
curvature antrum or body)
• Broad, flat lesion with central ulceration &
elevated margins
• Prone compression view (mass on anterior wall):
Radiolucent halo (filling defect) due to elevated
edges; meniscoid ulcer-convex inner border +
concave outer border
o Infiltrating Ca: 5-15% of all gastric cancers
• Irregular narrowing of stomach + nodularity +
mucosal spiculation
• Advanced cases: May cause gastric outlet
obstruction
o Scirrhous carcinoma (5-15%): Usually arise near
pylorus & extend up
• Linitis plastic a or "leather bottle": Irregular
narrowing & rigidity (scirrhous carcinoma)
• Localized scirrhous tumor: Short, annular
lesion/shelf-like proximal borders in prepyloric
region of antrum (fundus/body-40% cases)
• Diffuse linitis plastica: Diffusely infiltrated by a
scirrhous tumor (nodularity, spiculation,
ulceration, or thickened irregular folds)
CT Findings
• Demonstration of lesions facilitated by: Negative
contrast agents (water or gas)
o A polypoid mass with or without ulceration
o Focal wall thickening with mucosal irregularity or
focal infiltration of wall
o Ulceration: Gas-filled ulcer crater within mass
o Infiltrating carcinoma: Wall thickening + loss of
normal rugal fold pattern
Top Differential Diagnoses
• Benign gastric (peptic) ulcer
• Gastritis
• Gastric metastases & lymphoma
• Gastric stromal tumor
• Pancreatitis (extrinsic inflammation)
• Menetrier disease
Pathology
• Risk factors: H. pylori, atrophic gastritis, pernicious
anemia, adenomatous polyps, Menetrier, partial
gastrectomy (Billroth II), blood type-A, smoking
3
Diagnostic Checklist
• Differentiate from other pathologies that can mimic 47
gastric cancer on imaging; usually require deep
biopsy
• Wisp-like perigastric soft tissue stranding:
Perigastric fat extension
o Scirrhous carcinoma: Markedly enhancing thickened
wall on a dynamic CT scan
o Mucinous carcinoma: Decreased attenuation of
thickened wall (1 mucin); calcification seen
o Carcinoma of cardia: Irregular soft tissue thickening;
lobulated mass
Ultrasonographic Findings
• Endoscopic ultrasonography (EUS)
o Used to stage carcinoma; to assess depth of wall
invasion & perigastric lymph nodes
Imaging Recommendations
• Double-contrast barium study, NE + CECT, EUS
I DIFFERENTIAL DIAGNOSIS
Benign gastric (peptic) ulcer
• Round ulcer, smooth mound of edema, smooth
radiating folds to ulcer edge
• Classic features of benign ulcer: Hampton line, ulcer
collar, ulcer mound (diagnosis-endoscopic biopsy)
Gastritis
• Erosive gastritis
o Varioliform erosions: Multiple punctate or slit-like
collections of barium surrounded by radiolucent
halos of edematous mucosa
o Location: Typically in gastric antrum on crests of
rugal folds
o NSAID-related gastropathy: Subtle flattening &
deformity of greater curvature of antrum
• Nonerosive or atrophic gastritis
o Narrowed, tubular stomach; l/absent mucosal folds
• Antral, H. pylori & hypertrophic gastritis
o Markedly thickened, lobulated gastric folds
• Granulomatous gastritis (Crohn disease)
o Thickened nodular folds in antrum
o "Ram's horn" sign: Smooth funnel-shaped narrowing
• Eosinophilic, radiation & AIDS-related gastritis
 GASTRIC CARCINOMA
o Mucosal nodularity, erosions, ulcers, thickened folds
& antral narrowing
o Chronic radiation gastritis
• Antral narrowing may mimic scirrhous cancer
Gastric metastases & lymphoma
• Gastric metastases: Most common organs of origin
o Colon, malignant melanoma, breast, lung, pancreas
o Breast cancer: Most common metastases to stomach
• Gastric lymphoma (e.g., non-Hodgkin B-cell)
o Stomach most commonly involved organ in GIT
• Barium findings
o Colon cancer with gastric invasion: Mass effect,
nodularity, spiculated & tethered mucosal folds
3 • Transverse colon cancer invade stomach via
gastrocolic ligament (vice versa)
48 o Lobular breast cancer metastases
• Linitis plastic a or "leather bottle" appearance,
thickened irregular folds (simulating scirrhous
carcinoma)
o Gastric lymphoma: Diffuse, thickened irregular folds
• CT findings
o Markedly thickened gastric wall & mucosal folds
• Diagnosis: Endoscopic biopsy
Gastric stromal tumor
• Example: Leiomyosarcoma or GIST
o Large, lobulated submucosal mass; ± cavitation
o Intramural-50%, exogastric-35%, endogastric-15%
Caustic gastritis
• Subacute or chronic phase resembles linitis plastica
Pancreatitis (extrinsic inflammation)
• Mimic gastric cancer due to thickened gastric wall
• CT will show peri pancreatic inflammation
Menetrier disease
• Markedly thickened, lobulated folds in gastric fundus
& body usually sparing antrum may simulate cancer
• Diagnosis: Endoscopic full-thickness biopsy
[PATHOLOGY
General Features
• Etiology
o Risk factors: H. pylori, atrophic gastritis, pernicious
anemia, adenomatous polyps, Menetrier, partial
gastrectomy (Billroth II), blood type-A, smoking
o Diet: Rich in nitrites or nitrates; salted, smoked,
poorly-preserved food
• Epidemiology
o Incidence
• Uncommon & decreasing in US
• Common in Japan, Chile, Finland, Poland,
Iceland
Gross Pathologic & Surgical Features
• Polypoid, ulcerated, local or diffuse infiltrative &
rarely multiple lesions
Microscopic Features
• Well-differentiated adenocarcinoma
• Signet ring cell, papillary, tubular, mucinous
• Early Ca: Limited to mucosa & submucosa
• Advanced: Mucosa, submucosa & muscularis propria
Staging, Grading or Classification Criteria
• CT staging of gastric cancer
o I: Intraluminal mass
o II: Intraluminal mass + gastric wall thickness> 1 cm
o III: Adjacent structures + lymph nodes
o IV: Distant metastases
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, anorexia, weight loss, anemia, pain
o Melena, enlarged left supraclavicular Virchow node
• Lab-data: Hypochromic, microcytic anemia; stool
positive for occult blood
• Diagnosis: Endoscopic biopsy & histology
Demographics
• Age: Middle & elderly age group
• Gender: Males more than females (M:F = 2:1)
Natural History & Prognosis
• Complications
o Gastric outlet obstruction in antral carcinoma
• Prognosis
o 5 year survival rate
• Early gastric cancer (85-100%)
• Advanced cancer (3-21 %)
Treatment
• Radiotherapy; chemotherapy
• Surgery: Subtotal or total gastrectomy
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other pathologies that can mimic
gastric cancer on imaging; usually require deep biopsy
Image Interpretation Pearls
• Gastric carcinoma can be ulcerative, polypoid or
infiltrative (scirrhous type) + local & distant metastases
I SELECTED REFERENCES
1. Habermann CR et al: Preoperative staging of gastric
adenocarcinoma: comparison of helical CT and endoscopic
US. Radiology. 230(2):465-71, 2004
2. Insko EK et al: Benign and malignant lesions of the
stomach: evaluation of CT criteria for differentiation.
Radiology. 228(1):166-71, 2003
3. Ba-Ssalamah A et al: Dedicated multi detector CT of the
stomach: spectrum of diseases. Radiographies.
23(3):625-44, 2003
4. "Horton KM et al: Current role of CT in imaging of the
stomach, Radiographies. 23(1):75-87, 2003
5. Fishman EKet al: CT of the stomach: spectrum of disease.
Radiographies. 16(5):1035-54, 1996
6. Levine MS et al: The Helicobacter pylori revolution:
Radiologic perspective. Radiology 195: 593-6, 1995
7. Urban BAet al: Helicobacter pylori gastritis mimieking
gastric carcinoma at CT evaluation. Radiology.
179(3):689-91, 1991
 GASTRIC CARCINOMA

I IMAGE GAllERY

(Left) Upper CI series shows

mass (arrows) as a filling
defect in the barium pool on
this supine film. (Right) Axial
CECT shows large mass
(arrows) in gastric fundus.

3
49

Typical
(Left) Upper CI series shows
advanced infiltrating
carcinoma causing nodular
thickened folds and limiting
distensibility. (Right) Axial
CECT shows scirrhous
carcinoma with enhancing
thickened wall (arrows) and
malignant ascites.

Typical
(Left) Axial CECT shows
nodular thickening of the
ventral wall of the stomach
and circumferential tumor of
the antrum causing partial
outlet obstruction. (Right)
Axial CECT shows
circumferential tumor
encasing antrum. The tumor
invades the anterior
abdominal wall (arrows) and
local lymph nodes (open
arrow).
 GASTRIC LYMPHOMA AND METASTASES
3
50
Axial CECT shows diffuse homogeneous thickening of
the gastric wall and extensive
lymphadenopathy (arrows). Gastric lymphoma
ITERMINOLOGY
Definitions
• Gastric metastases from primary cancer of other sites
• Lymphoma: Malignant tumor of B-Iymphocytes
I IMAGING FINDINGS
General Features
• Best diagnostic clue: "Bull's eye" lesions on imaging
• Gastric metastases
o Seen at autopsy in < 2% patients who die of cancer
o Various forms of metastatic spread to stomach
• Hematogenous, lymphatic & direct invasion
o Hematogenous (most common): Malignant
melanoma, carcinoma (Ca) breast & lung
• Malignant melanoma: Highest % of
hematogenous metastases
• Breast cancer (most common disease): Most
common cause of metastases to stomach
• Thyroid & testes: Rarely hematogenous spread to
stomach
o Lymphatic spread: Esophageal or colon cancer
o Direct invasion or extension: Pancreatic & colon
cancer, hepatocellular carcinoma
o Direct gastric invasion via mesenteric reflections:
Transverse colon carcinoma
DDx: Diffuse or Focal Thickening of Gastric Wall
Castric Carcinoma CIST Tumor
Axial CECT shows diffuse thickening of the gastric wall
perigastric and porto-caval adenopathy (arrow). Gastric
lymphoma.
• Gastrocolic ligament, transverse mesocolon,
greater omentum
o Most patients with gastric metastases have a known
underlying cancer
o Occasionally may occur as initial manifestation of
an occult primary tumor
o Carcinoma of breast & kidney can metastasize to
stomach many years after treatment of primary
• Gastric lymphoma
o Stomach is most frequently involved part of GI tract
o Accounts for 50% of all GI tract lymphomas, 25% of
all extranodallymphomas & 3-5% of all malignant
tumors in stomach
o More than 50% cases are primary, rest are secondary
o Majority, non-Hodgkin lymphoma (B-cell) origin
o Primary classified into two types based on pathology
• Low grade MALT (mucosa-associated lymphoid
tissue) lymphoma
• High grade or advanced lymphoma
Radiographic Findings
• Fluoroscopic guided barium study
o Malignant melanoma metastases
• Solitary/multiple discrete submucosal masses
• "Bull's eye" or "target" lesions: Centrally ulcerated
submucosal masses
• "Spoke-wheel" pattern: Radiating superficial
fissures from central ulcer
Castritis Pancreatitis
 GASTRIC LYMPHOMA AND METASTASES
Key Facts
Imaging Findings
• Best diagnostic clue: "Bull's eye" lesions on imaging
• Solitary/multiple discrete submucosal masses
• Giant cavitated lesion: Large collection of barium
(5-15 cm) communicating with lumen
• Lobular breast cancer: Linitis plastica or "leather
bottle" appearance (loss of distensibility of antrum &
body + thickened irregular folds)
• Direct invasion: Spiculated mucosal folds, nodular
mass effect, ulceration, obstruction, rarely fistula
• Rounded, confluent nodules of low grade lymphoma
(mimic enlarged areae gastricae of H. pylori gastritis)
• Polypoid lymphoma: Lobulated intraluminal mass
• Lacy reticular pattern to bulky masses (omental cake)
displacing & causing gastric wall indentation
• Regional or widespread adenopathy
• Giant cavitated lesion: Large collection of barium
(5-15 cm) communicating with lumen
• Small or large lobulated masses
o Breast carcinoma metastases
• Lobular breast cancer: Linitis plastica or "leather
bottle" appearance (loss of distensibility of antrum
& body + thickened irregular folds)
• Mucosal nodularity, spiculation, ulceration
o Esophageal squamous cell metastasis
• Large submucosal masses + central ulceration
o Esophageal adenocarcinoma (from Barrett mucosa)
• Large polypoid or ulcerated mass in gastric fundus
• Subtle findings of cardia: Small ulcers & nodules
o Pancreatic carcinoma
• Carcinoma of head: Extrinsic compression of
medial border of gastric antrum
• Carcinoma of body or tail: Extrinsic compression
of posterior wall of fundus or body
• Direct invasion: Spiculated mucosal folds, nodular
mass effect, ulceration, obstruction, rarely fistula
o Omental metastases & transverse colon carcinoma:
Gastric invasion via gastrocolic ligament more
common by omental metastases than colon Ca
• Greater curvature of antrum & body: Mass effect,
nodularity, spiculation, mucosal fold tethering,
gastrocolic fistula
o Gastric low grade MALTlymphoma
• Rounded, confluent nodules of low grade
lymphoma (mimic enlarged areae gastricae of H.
pylori gastritis)
• Shallow, irregular ulcers with nodular surrounding
mucosa
o Gastric high grade or advanced lymphoma
• Infiltrative lesions: Massively enlarged folds with
distorted & nodular contour (stomach remains
pliable/distensible)
• Ulcerative lesions: Ulcers with surrounding
nodular mucosa & thickened, irregular folds; some
may appear as giant, cavitated lesions
• Polypoid lymphoma: Lobulated intraluminal mass
• Nodular lesions: Submucosal nodules or masses
often ulcerate, resulting in "bull's eye" or target
lesions
Top Differential Diagnoses
• Gastric carcinoma
• Gastric stromal tumor (leiomyosarcoma)
• Gastritis (erosive type)
• Pancreatitis (extrinsic inflammation)
Pathology
• Spread: Hematogenous, lymphatic, direct spread
• Example: Malignant melanoma; carcinoma of breast,
lung, pancreas, colon, esophagus
• Primary: Non-Hodgkin B-cell type (> common)
Diagnostic Checklist 3
• Overlapping radiographic features of gastric
metastases, lymphoma & primary carcinoma 51
• Imaging important to suggest & stage malignancy,
but biopsy often required
CT Findings
• Demonstration of lesions facilitated by negative
contrast agents (water or gas)
• Hematogenous spread of metastases to stomach
o Malignant melanoma
• "Bull's eye" or "target" lesions (also seen in
lymphoma, Kaposi sarcoma, carcinoid tumor)
• Giant cavitated lesions
• Location: Proximal stomach; antrum spared
o Breast cancer: Linitis plastica or "leather bottle"
• Markedly thickened gastric wall; folds preserved
• Enhancement of thickened gastric wall
• Mimics primary scirrhous carcinoma of stomach
• Location: Proximal stomach; antrum spared
• Lymphatic spread of metastases to stomach
oEsophageal Ca: Growth in gastric cardia or fundus
o Multiple, well-defined, I HU enlarged nodes
• Characteristic of squamous cell metastases
• Location: Paracardiac, lesser sac & celiac
• Direct invasion of stomach
o Distal esophageal adenocarcinoma: Barrett mucosa
• Polypoid, lobulated mass in gastric fundus
• Indistinguishable from primary gastric carcinoma
o Pancreatic carcinoma
• Abnormal, irregular extrinsic gastric compression
• Carcinoma of head: Greater curvature of antrum
• Body & tail: Posterior wall (gastric fundus/body)
o Transverse colon cancer ~ gastrocolic ligament ~
stomach greater curvature
• Greater curvature: Thickened wall or mass
• ± Gastrocolic fistulous tract
o Omental metastases: Ovary, uterus, pancreas, breast
• Omental masses as small as 1 cm can be seen
• Lacy reticular pattern to bulky masses (omental
cake) displacing & causing gastric wall indentation
• Gastric lymphoma
o Markedly thickened gastric wall
o Thickened rugal folds, but contour is preserved
o Regional or widespread adenopathy
o Transpyloric spread into duodenum may be seen
 GASTRIC LYMPHOMA AND METASTASES
Ultrasonographic Findings
• Real Time
o Endoscopic ultrasonography (EUS)
• Hypoechoic mass disrupting normal wall layers
• Selective/diffusely thickened echogenic wall layers
Imaging Recommendations
• Helical CT; barium (single/double) contrast studies
I DIFFERENTIAL DIAGNOSIS
Gastric carcinoma
3 • Polypoid, ulcerated, infiltrative types indistinguishable
from gastric metastases & lymphoma
52 • Linitis plastica appearance of primary scirrhous type
mimics lobular metastatic breast cancer
• Loss of distensibility in scirrhous type differentiates
from gastric non-Hodgkin lymphoma
• However gastric Hodgkin lymphoma indistinguishable
from scirrhous due to similar desmoplastic response
Gastric stromal tumor (leiomyosarcoma)
• Usually occur as solitary lesions; mostly exophytic
• Also produce giant, cavitated lesions simulating gastric
metastases of malignant melanoma & lymphoma
Gastritis (erosive type)
• Multiple punctate barium collections surrounded by
thin radiolucent halos of edematous mucosa
• Occasionally surrounded by prominent mounds of
edema resulting in "bull's eye" lesions simulating
gastric metastases & lymphoma
Pancreatitis (extrinsic inflammation)
• Changes in greater curvature or posterior wall of
stomach mimic omental metastatic invasion
• CT will show peripancreatic inflammation
I PATHOLOGY
General Features
• Etiology
o Gastric metastases
• Spread: Hematogenous, lymphatic, direct spread
• Example: Malignant melanoma; carcinoma of
breast, lung, pancreas, colon, esophagus
o Gastric lymphoma
• Primary: Non-Hodgkin B-cell type (> common)
• Arise from mucosa associated lymphoid tissue
(MALT) in patients with chronic H. pylori gastritis
containing cytotoxin-associated antigen (CagA)
• Secondary lymphoma (generalized lymphoma)
• Epidemiology
o Gastric metastases: Seen in < 2% who die of cancer
o Gastric lymphoma: 3-5% of all gastric malignancies
• Associated abnormalities
o Primary carcinoma in gastric metastases
o Generalized adenopathy in secondary lymphoma
Gross Pathologic & Surgical Features
• Solitary/multiple; polypoid, ulcerated, cavitated
masses or leather bottle appearance of stomach
Microscopic Features
• Metastases: Varies based on primary cancer
• Lymphoma: Lymphoepitheliallesions
Staging, Grading or Classification Criteria
• Ann Arbor staging of primary lymphoma
o Stage I: Lesions involve gastric wall
o Stage II: Involve regional lymph nodes in abdomen
o Stage III: Nodes above & below diaphragm
o Stage IV: Widely disseminated lymphoma
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, pain, weight loss, palpable mass
o Hematemesis, melena, acute abdomen (perforation)
Demographics
• Age: Usually middle & elderly age group
• Gender: Metastases (M = F); lymphoma (M> F)
Natural History & Prognosis
• Complications
o Upper GI bleeding & perforation in ulcerated lesions
o Antral lesion + pyloric extension: Outlet obstruction
• Prognosis: Poor
Treatment
• Chemotherapy & surgical resection of lesions causing
complications like obstruction & upper GI bleeding
I DIAGNOSTIC CHECKLIST
Consider
• Check for history of primary cancer/H. pylori gastritis
Image Interpretation Pearls
• Overlapping radiographic features of gastric
metastases, lymphoma & primary carcinoma
• Imaging important to suggest & stage malignancy, but
biopsy often required
I SELECTED REFERENCES
1. Horton KM et al: Current role of CT in imaging of the
stomach. Radiographics. 23(1):75-87, 2003
2. Ba-Ssalamah A et al: Dedicated multidetector CT of the
stomach: spectrum of diseases. Radiographies.
23(3):625-44, 2003
3. Park MS et al: Radiographic findings of primary B-cell
lymphoma of the stomach: low-grade versus high-grade
malignancy in relation to the mucosa-associated lymphoid
tissue concept. AJRAm J Roentgenol. 179(5):1297-304,
2002
4. McDermott VG et al: Malignant melanoma metastatic to
the gastrointestinal tract. AJR Am J Roentgenol.
166(4):809-13, 1996
5. Fishman EK et al: CT of the stomach: spectrum of disease.
Radiographics. 16(5):1035-54, 1996
6. Feczko PJ et al: Metastatic disease involving the
gastrointestinal tract. Radiol Clin North Am.
31(6):1359-73, 1993
 GASTRIC LYMPHOMA AND METASTASES

I IMAGE GALLERY
Typical
(Left) Upper CI series shows
"bulf's eye" lesion (arrow), a
discrete intramural polyp
with central ulceration.
Metastatic melanoma.
(Right) Upper CI series
shows a submucosal
polypoid mass (arrow) from
metastatic melanoma.

3
53

Typical
(Left) Upper CI series shows
circumferential massive
thickening of gastric folds
but no outlet obstruction.
Lymphoma. (Right) Upper
CI series shows gastric
lymphoma. The stomach is
encased by tumor with two
large ulcerations (arrows),
but no obstruction.

Typical
(Left) Axial NECT shows
massive circumferential
thickening of gastric antral
wall (arrows), but no
obstruction. Lymphoma.
(Right) Axial CECT shows
gastric lymphoma. The entire
stomach is involved with
massive mural thickening.
 DUODENAL CARCINOMA

3
54

Axial CECT shows 50ft tissue mass along the medial Single-contrast upper GI series shows ulcerated annular
border of the second portion of the duodenum, which constricting mass in the descending duodenum (arrow),
proved to be duodenal carcinoma (Courtesy M. which proved to be duodenal carcinoma (Courtesy M.
Nino-Murcia, MO). Nino-Murcia, MO).

• Ulcerated mass
!TERMINOLOGY • Polypoid mass
Abbreviations and Synonyms • Annular constricting "apple-core" lesion
• Duodenal carcinoma (CA), duodenal adenocarcinoma • "Soap-bubble" reticulated pattern for villous
tumors
Definitions
CT Findings
• Primary neoplasm arising in duodenal mucosa
• CECT
o Discrete mass or irregular thickening of duodenal
I IMAGING FINDINGS wall
o Concentric narrowing of duodenum
General Features o Polypoid intraluminal mass
• Best diagnostic clue: Irregular intraluminal mass or o Local lymphadenopathy
apple-core lesion at or distal to ampulla of Vater o Infiltration of adjacent fat
• Location o Biliary or pancreatic duct dilatation with
o 15% in first portion of duodenum periampullary tumors
o 40% in 2nd portion of duodenum MR Findings
o 45% in distal duodenum
• Size: Usually < 8 cm • MRCP
o May see pancreatic or biliary ductal dilatation with
• Morphology
periampullary duodenal carcinomas
o Polypoid, ulcerated, or annular constricting mass
o Intraluminal mass with numerous frond-like Ultrasonographic Findings
projections for carcinomas arising in villous tumors • Real Time: Hypoechoic mass in duodenum with
Radiographic Findings echogenic center: Pseudokidney sign
• Color Doppler: May see invasion of adjacent vascular
• Radiography: Proximal obstruction pattern if lumen
structures
severely narrowed
• Fluoroscopy
o May have various appearances

DDx: Duodenal Narrowing

Annular Pancreas
 DUODENAL CARCINOMA

Key Facts
Terminology
• Primary neoplasm arising in duodenal mucosa
Imaging Findings
• Best diagnostic clue: Irregular intraluminal mass or
apple-core lesion at or distal to ampulla of Vater
• Biliary or pancreatic duct dilatation with
periampullary tumors
• Best imaging tool: Thin-section CECT with water for
luminal distention and dual-phase arterial and
venous imaging
• Protocol advice: Multidetector CT with thin
collimation generates best data set for multiplanar
reformation
Pathology
• Adenocarcinomas represent 73-90% of malignant
duodenal tumors
• 45% of small bowel adenocarcinomas arise in
duodenum
• Rare: Represents < 1% of all gastrointestinal
neoplasms
• Secondary cancers far more common than primary
cancers in proximal small bowel
• Often difficult to distinguish primary duodenal CA
from secondary GI adenocarcinoma even with special
stains
• Proximal small bowel adenocarcinoma may be a
3
marker for familial or multicentric cancer syndrome 55

Imaging Recommendations Trauma

• Best imaging tool: Thin-section CECT with water for • Duodenal hematoma
luminal distention and dual-phase arterial and venous
imaging
• Protocol advice: Multidetector CT with thin I PATHOLOGY
collimation generates best data set for multiplanar
reformation General Features
• General path comments
o Adenocarcinomas represent 73-90% of malignant
I DIFFERENTIAL DIAGNOSIS duodenal tumors
o Small bowel adenocarcinomas are rare, especially in
Neoplasms relation to length of the small bowel
• Ampullary and periampullary adenocarcinomas • 45% of small bowel adenocarcinomas arise in
o Pancreatic adenocarcinoma duodenum
o Ampullary carcinoma • 25% of all malignant small bowel tumors occur in
o Primary bile duct carcinoma duodenum
• Metastases • Genetics: Alterations in oncogenes erbB2, K-ras, cyclin
o Contiguous spread from pancreatic, colon, kidney or Dl and p53
gallbladder carcinoma • Etiology
o Hematogenous metastases from melanoma, Kaposi o Adenoma-carcinoma sequence
sarcoma • Adenomatous polyps are most important risk
o Periduodenallymph node metastases from other factor
malignancies o Risk factors
• Other duodenal primary neoplasms • Familial polyposis syndromes
o Duodenal lymphoma • Crohn disease
o Malignant GI stromal tumor • Cigarette smoking
o Duodenal carcinoid • Alcoholism
• Epidemiology
Inflammatory
o Rare: Represents < 1% of all gastrointestinal
• Benign post-bulbar peptic ulcers neoplasms
• Zollinger-Ellison syndrome o Incidence rises with age
o Multiple post-bulbar ulcers, thickened folds,
hypersecretion Gross Pathologic & Surgical Features
• Crohn disease • Duodenal mass may be flat, stenosing, ulcerative,
infiltrating or polypoid in growth pattern
Infectious
• Secondary cancers far more common than primary
• Tuberculosis cancers in proximal small bowel
Congenital o Often difficult to distinguish primary duodenal CA
from secondary GI adenocarcinoma even with
• Annular pancreas
• Duodenal duplication cyst special stains
• Proximal small bowel adenocarcinoma may be a
marker for familial or multicentric cancer syndrome
 DUODENAL CARCINOMA

Microscopic Features Treatment

• Similar histology to other GI adenocarcinomas • Options, risks, complications
o Cellular and nuclear pleomorphism o Surgery for resectable lesions
o Dysplasia • Pancreaticoduodenectomy for 1st and 2nd portion
o Gland-in-gland appearance of duodenum lesions
o Invasion into adjacent normal tissues • Segmental duodenectomy and primary
• Most duodenal carcinomas are moderately reanastomosis for 3rd and 4th portion of
differentiated with variable mucin production duodenum lesions
• 20% of duodenal carcinomas are poorly differentiated o Unresectable tumors: Palliation with radiation,
chemotherapy, stenting
Staging, Grading or Classification Criteria
• American]oint Committee on Cancer (A]CC) TNM
staging system I DIAGNOSTIC CHECKLIST
3 o Primary tumor (T)
• Tl: Tumor invades lamina propria or submucosa Consider
56 • T2: Tumor invades muscularis propria • Check for vascular invasion, especially for lesions of
• T3: Tumor invades through muscularis propria 2nd and 3rd duodenum
and:s; 2 cm into adjacent tissues • Look for regional lymph nodes and liver metastases
• T4: Tumor perforates visceral peritoneum, directly
invades other organs, or extends> 2 cm into Image Interpretation Pearls
adjacent tissues • Most duodenal carcinomas cause focal stenoses or
o Regional lymph nodes (N) obstruction; large mass with cavitation often is
• NO: No regional nodes involved lymphoma
• Nl: Regional lymph node metastasis • Scrutinize duodenum when periduodenal
o Distant metastasis (M) lymphadenopathy is present on CT without obvious
• MO: No distant metastases source
• Ml: Distant metastasis
o Staging
• Stage I: Tl or T2, NO, MO I SELECTED REFERENCES
• Stage II: T3 or T4, NO, MO 1. Lawler LP et al: Peri pancreatic masses that simulate
• Stage III: Any T, Nl, MO pancreatic disease: spectrum of disease and role of CT.
• Stage IV: Any T, any N, Ml Radiographies. 23(5):1117-31, 2003
2. Kim JH et al: Differential diagnosis of periampullary
carcinomas at MR imaging. Radiographics. 22(6):1335-52,
I CLINICAL ISSUES 3.
2002
Korman MU: Radiologic evaluation and staging of small
Presentation intestine neoplasms. Eur J Radiol. 42(3):193-205, 2002
4. Nagi B et al: Primary small bowel tumors: a
• Most common signs/symptoms radiologic-pathologic correlation. Abdom Imaging.
o Upper abdominal pain secondary to obstruction 26(5):474-80,2001
o Other signs/symptoms 5. Ishida H et al: Duodenal carcinoma: sonographic findings.
• Nausea and vomiting, weight loss, anemia, upper Abdom Imaging. 26(5):469-73, 2001
GI bleed 6. Iki K et al: Primary adenocarcinoma of the duodenum
• Periampullary tumors may present with jaundice demonstrated by ultrasonography. J Gastroenterol.
36(3):195-9, 2001
• Clinical profile
7. Gore R et al: Textbook of Gastrointestinal Radiology. 2nd
o Increased incidence of duodenal CA in familial
ed. Philadelphia, W.B. Saunders, 1980-1992,2000
polyposis syndromes 8. Buckley JA et al: CT evaluation of small bowel neoplasms:
• Peutz-]egher syndrome, Gardner syndrome spectrum of disease. Radiographies. 18(2):379-92, 1998
9. Neugut AI et al: The epidemiology of cancer of the small
Demographics bowel. Cancer Epidemiol Biomarkers Prevo 7(3):243-51,
• Age 1998
o 7th decade: Median age = 60 years 10. Maglinte DT et al: Small bowel cancer. Radiologic
o Low incidence in patients younger than 30 diagnosis. Radiol Clin North Am. 35(2):361-80, 1997
• Gender: Slight male predominance 11. Buckley JA et al: The accuracy of CT staging of small bowel
adenocarcinoma: CT/pathologic correlation. J Comput
Natural History & Prognosis Assist Tomogr. 21(6):986-91, 1997
• Spreads by direct extension to adjacent organs and 12. Buckley JA et al: Small bowel cancer. Imaging features and
through serosa to peritoneal cavity staging. Radiol Clin North Am. 35(2):381-402, 1997
13. Gore RM: Small bowel cancer. Clinical and pathologic
• Metastasizes hematogenously to liver, lungs, and bone
features. Radiol Clin North Am. 35(2):351-60, 1997
• Metastasizes via lymphatics to regional nodes 14. Arber N et al: Molecular genetics of small bowel cancer.
• 22-71% of patients have positive nodes at presentation Cancer Epidemiol Biomarkers Prevo 6(9):745-8, 1997
• Prognosis depends on resectability, lymph node 15. Laurent F et al: CT of small-bowel neoplasms. Semin
involvement, and somewhat on histologic grade Ultrasound CT MR. 16(2):102-11, 1995
• Vascular invasion makes lesion unresectable
 DUODENAL CARCINOMA

I IMAGE GALLERY

(Left) Axial CECT shows

duodenal wall thickening
(curved arrow), low density
lymph node (open arrow),
and liver metastasis (arrow)
in patient with duodenal
carcinoma. (Right) Axial
CECTshows bulky duodenal
carcinoma in second portion
of duodenum. Patient has a
gastrojejunostomy (arrow)
and biliary stent (open 3
arrow) for palliation of
obstruction. 57

(Left) Coronal CECT

thin-slab-average image
shows low attenuation
annular constricting mass in
transverse duodenum
(arrows). (Right)
Double-contrast upper GI
series shows "apple-core"
lesion of second portion of
duodenum (arrows)
representing duodenal
carcinoma (Courtesy H.
Harvin, MD).

Typical
(Left) Axial CECT shows
irregular low attenuation
mass in second portion of
duodenum. Note central low
density lumen (arrow) which
shows the mass to be arising
within duodenum rather
than pancreas. (Right) Axial
CECT shows irregular mass
distorting duodenal lumen
and extending into adjacent
fat medially (open arrow).
 DUODENAL METASTASES AND LYMPHOMA

3
58

Axial CECT demonstrates submucosal soft tissue Axial CECT demonstrates bulky soft tissue mass
infiltrating mass (arrow) due to lymphoma. involving duodenum (arrow). Biopsy revealed
lymphoma.

o Lymphoma: Bulky hypovascular soft tissue mass

ITERMINOLOGY infiltrating submucosa of stomach and duodenum
Definitions on CECT
• Involvement of duodenum with malignant lymphoma o Hematogenous mets images as rounded submucosal
or metastatic disease mass; direct invasion mets shows involvement from
primary tumor of pancreas, colon, kidney,
gallbladder or retroperitoneal node on CECT
I IMAGING FINDINGS MR Findings
General Features • T1WI: Low signal duodenal mass
• T2WI: Intermediate signal mass
• Best diagnostic clue
• T1 C+: Variable enhancement: Adeno CA typically
o Lymphoma: Bulky submucosal mass extending
hypovascular, melanoma may be hypervascular
through pylorus to secondarily invade duodenum
o Mets: "Bull's eye" or "target lesion"; submucosal or Imaging Recommendations
polypoid mass • Best imaging tool: UGI, CECT
• Location: Pylorus and duodenum, submucosal lesion
• Size: 1-5 cm
• Morphology: Lymphoma: Smooth submucosal, often I DIFFERENTIAL DIAGNOSIS
bulky, mass
Villous adenoma
Radiographic Findings
• Bulky mucosal polypoid mass, 3-9 cm; rarely causes
• Fluoroscopy obstruction
o Lymphoma: Smooth or lobulated submucosal mass • Risk of CA increases with size; 30-60% of tumors have
involving distal stomach and duodenum on UGI malignant changes
o Mets: "Target" or "bull's eye" lesion with rounded
submucosal mass; ulceration common on UGI
CT Findings
• CECT

DDx: lesions Mimicking lymphoma or Metastases

Villous Adenoma Duodenal Carcinoma Secondary Invasion

 DUODENAL METASTASES AND LYMPHOMA

Key Facts
Imaging Findings
• Lymphoma: Smooth or lobulated submucosal mass
involving distal stomach and duodenum on UGI
• Mets: "Target" or "bull's eye" lesion with rounded
submucosal mass; ulceration common on UGI
• Lymphoma: Bulky hypovascular soft tissue mass
infiltrating submucosa of stomach and duodenum on
CECT
• Hematogenous mets images as rounded submucosal
mass; direct invasion mets shows involvement from
primary tumor of pancreas, colon, kidney, gallbladder
or retroperitoneal node on CECT
Clinical Issues
• Options, risks, complications: Best option for
localized lymphoma is surgery; chemotherapy best
for mets

Duodenal carcinoma Demographics

3
• Infiltrating mural mass; "apple core" annular lesion; • Age: 55-60 years 59
may be polypoid; often ulcerated; more likely to • Gender: M < F
obstruct lumen
• 1% of all GI neoplasms; increased incidence in Treatment
Gardner syndrome, celiac disease, Crohn disease, • Options, risks, complications: Best option for localized
neurofibromatosis lymphoma is surgery; chemotherapy best for mets
• Regional lymphadenopathy & pancreatic invasion
common
I DIAGNOSTIC CHECKLIST
Secondary duodenal invasion
• Most commonly due to pancreatic CA, colon CA or Consider
renal cell CA • Duodenal carcinoma
• Large extramural mass; often asymptomatic but may
Image Interpretation Pearls
lead to outlet obstruction
• Bulky submucosal mass without obstruction

I PATHOLOGY I SELECTED REFERENCES

General Features
• General path comments
o Lymphoma: Non-Hodgkin lymphoma of B-cell
origin or mucosa-associated lymphoid tissue (MALT)
o Mets: Melanoma, CA of breast, lung, colon,
pancreas, kidney
• Etiology: MALT lymphomas associated with H. pylori
infection
• Associated abnormalities: Regional lymphadenopathy;
mets may cause outlet obstruction
1. Elliott LA et al: Metastatic breast carcinoma involving the
gastric antrum and duodenum: computed tomography
appearances. Br J Radiol. 68(813):970-2, 1995
2., Cirillo M et al: Primary gastrointestinal lymphoma: a
clinicopathological study of 58 cases. Haematologica.
77(2):156-61, 1992
3. Najem AZ et al: Primary non-Hodgkin's lymphoma of the
duodenum. Case report and literature review. Cancer.
54(5):895-8, 1984
4. Balthazar EJ: Duodenal Hodgkin's disease. Am J
Gastroenterol. 68(3):306-11, 1977

Gross Pathologic & Surgical Features

• Lymphoma: Most often associated with gastric I IMAGE GALLERY
lymphoma extending through pylorus into duodenum
• Mets: Polypoid mucosal/submucosal masses
(melanoma or secondary extrinsic mass invading
duodenum)
Staging, Grading or Classification Criteria
• GI lymphoma staging
o I: Tumor confined to bowel wall
o II: Limited nodal spread to local nodes
o III: Widespread nodal mets
o IV: Spread to bone marrow, solid viscera, Le., liver

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Abd pain, nausea,
vomiting, weight loss, palpable mass, UGI bleeding-
(Left) Axial CECT of duodenal lymphoma. Note extensive infiltration
of duodenum by 50ft tissue mass (arrow). (Right) Axial CECT of
duodenal lymphoma. Bulky mass infiltrates duodenum, invades
mesentery and extends into superior mesenteric vein (arrow).
 FUNDOPLICATION COMPLICATIONS

3
60

Graphic shows Nissen fundoplication with gastric Upper GI series following surgery shows the expected
fundus wrapped around the gastroesophageal (Gf) fundoplication defect in the stomach (arrow), and
junction. extravasation of contrast material (open arrow) from the
Gf junction.

o FDP disruption or breakdown

!TERMINOLOGY
Abbreviations and Synonyms
• Fundoplication (FDP) complications
Definitions
• Complications of anti-reflux surgery for management
of gastroesophageal reflux disease (GERD)
• Nissen FDP: Complete FDP
o Approach: Laparoscopic or open FDP
o Gastric fundus wrapped 360 degrees around
intra-abdominal esophagus to create antireflux valve
o Concomitant diaphragmatic hernia reduced;
diaphragmatic esophageal hiatus sutured
• Toupet FDP: Partial FDP
o 270 degree wrap; posterior hemivalve created
• Belsey Mark IV repair: Open surgical; 240-degree FDP
wrap around left lateral aspect of distal esophagus
o Fundus sutured to intra-abdominal esophagus; acute
esophagogastric junction angle (angle of His)
o Can also be done by minimally invasive techniques
I IMAGING FINDINGS
General Features
• "Wrap" complications
o Slipped or misplaced FDP
o FDP herniation with intra thoracic migration
o Too tight or too loose, or too long FDP
o Herniation of stomach through re-opened
diaphragmatic esophageal hiatus
• "Non-wrap" complications
o Injury to intra-abdominal, intra-thoracic organs
o Leaks; intra-abdominal, thoracic fluid collections
o Fistulas; gastropericardial, gastrobronchial etc.
o Pneumothorax, pneumonia, pancreatitis, incisional
hernia, mesenteric & portal venous thrombosis
• Late complications
o Recurrent paraesophageal herniation
o Distal esophageal stricture
Radiographic Findings
• Fluoroscopy
• Normal post-operative appearance
o Nissen FDP wrap: Well-defined "mass" in gastric
fundus; smooth contour & surface
• Distal esophagus tapers smoothly through center
of symmetric compression by wrap
o Pseudotumoral defect of gastric fundus; part of
fundus wrapped around distal esophagus
• Defect more pronounced for complete wrap of
Nissen than partial wrap of Toupet; Belsey
o Belsey Mark IV repair
• Wrap produces smaller defect than Nissen FDP
• 2 distinct angles form as esophagus passes FDP

DDx: leak, Obstruction or Mass After Surgery

"

1 -

,.' 0

.. ff - ~
Normal Appearance Bezoar Splenic Laceration
 FUNDOPLICATION COMPLICATIONS
Key Facts
Terminology
• Nissen FDP: Complete FDP
• Toupet FDP: Partial FDP
Imaging Findings
• "Wrap" complications
• Slipped or misplaced FDP
• FDP disruption or breakdown
• FDP herniation with intra thoracic migration
• Too tight or too loose, or too long FDP
• Herniation of stomach through re-opened
diaphragmatic esophageal hiatus
• "Non-wrap" complications
• Injury to intra-abdominal, intra-thoracic organs
• Leaks; intra-abdominal, thoracic fluid collections
• Shallow upper angle; where esophagus, fundus, &
diaphragm sutured together
• Steep lower angle; where stomach pulled upward
toward esophagus
• "Wrap" complications
o Tight FDP wrap
• Fixed narrowing of distal esophagus
• Delayed emptying of barium into stomach
• May also be caused by excessive closure of
esophageal hiatus of diaphragm
o Complete disruption of FDP sutures
• Recurrent hiatal hernia & gastroesophageal reflux
• Gastric outpouching above diaphragm
• Expected mass of FDP wrap not present in fundus
o Partial, disruption of FDP sutures
• Partially intact wrap; does not encircle esophagus
• One or more small outpouchings from fundus
• Hourglass stomach; as fundus slips through FDP
o Slipped Nissen
• Complete wrap may slide downward over
stomach; hourglass configuration of stomach
o Intrathoracic migration of wrap
• Intact FDP wrap herniates partially or entirely
through esophageal hiatus of diaphragm
• Type I: Paraesophageal herniation of portion of
wrap through esophageal hiatus (70%)
• Type II: Herniation of entire FDP through hiatus
• Gastroesophageal (GE) junction: In type I, below
diaphragm. In type II, at or above diaphragm
• In both types, wrap intact, without disruption
o Inappropriate placement of FDP around gastric body
• Hourglass appearance of stomach
• "Non-wrap" complications
o Presence of leaks, fistula
o Persistence of gastroesophageal reflux, gastric ulcer
CT Findings
• "Wrap": Soft tissue density area surrounding
intra-abdominal esophagus at GE junction
o Extending caudally about 4cm
o Normal post surgical esophagus collapsed without
gaseous distention of its distal part; no reflux
• Pneumothorax, pneumonia, pancreatitis, incisional
hernia, mesenteric & portal venous thrombosis
• Recurrent paraesophageal herniation
Top Differential Diagnoses
• Post-op edema
• Bezoar
• Plication defect
• Extra-gastric complications
Diagnostic Checklist
• Post-operative fluoroscopic evaluation should be used
liberally or even routinely
• CT for suspected leak or bleeding
3
61
• Immediate post-operative period, gastric cardia wall
may be thickened at area of operation; due to edema
• Wrap breakdown: Gastric circumferential thickening
surrounding GE junction (due to wrap) is lacking
o Distal esophagus may be distended
o May see recurrent diaphragmatic hernia; reflux of
contrast material into esophagus
• Herniation of an intact FDP through diaphragmatic
hiatus; may be seen with coronal reformatted images
• Retraction injury to adjacent organs
o During laparoscopic procedure; retraction of left
hepatic lobe may result in liver or splenic laceration
o Right ventricular laceration; cardiac tamponade
• Trauma by liver retractor during laparoscopic FDP
o Bleeding & hematoma in gastric wall or in
peritoneal spaces adjacent to stomach & duodenum
• Fluid collections in abdomen or mediastinum
o Herniated abdominal fluid; disrupted lymphatic
drainage; hematoma; infection ± leak; abscess
o Drainage under CT guidance; obviating surgical
• Visceral perforation: Extraluminal contrast; free air
o Reported with open & laparoscopic FDP; correlates
with surgeon experience
• Superior mesenteric vein & portal vein thrombosis
o Rare; approximately 2 weeks after laparoscopic FDP
Imaging Recommendations
• Best imaging tool
o Videofluoroscopic contrast-enhanced esophagram
• Structural information; anatomical abnormalities
• "Wrap" complications; leaks; persistence of reflux
oCT; severe abdominal or chest pain; suspected
visceral injury; abscess
I DIFFERENTIAL DIAGNOSIS
Post-op edema
• Early post-operative period; edema of FDP wrap
• Large, smooth fundal mass; with smooth, tapered
narrowing of intra-abdominal esophagus
• Delayed emptying of contrast material
 FUNDOPLICATION
• Edema usually subside, less compression of esophagus
within 1-2 weeks
o Repeat esophagram shows much smaller defect
Bezoar
• Intraluminal mass; mottled or streaked appearance
• May cause partial or complete obstruction
Plication defect
• Disruption of diaphragmatic sutures (not FDP sutures)
o Recurrent hiatal hernia; above an intact FDP wrap
• Plication of diaphragm for eventration diaphragm may
be complicated by traumatic diaphragmatic hernia
o May see bowel herniating through diaphragmatic
3 defect at site of previous diaphragm plication
Extra-gastric complications
62
• Abscess, retractor injury to spleen, liver, etc.
I PATHOLOGY
General Features
• General path comments
o Indications for anti-reflux surgery
• Medical treatment ineffective
• Side effects of long term medications
• Complications of GERD; esophagitis, stricture,
recurrent aspiration pneumonia, asthma etc.
o Surgery also employs repair of large paraesophageal
hernias associated with GERD
• Etiology: Surgeon inexperience; operative technique
• Epidemiology
o Incidence of complications increasing; as many
laparoscopic FDPs performed indiscriminately
• Intrathoracic migration of wrap; seen in 30% after
laparoscopic Nissen FDP; 9% after open procedure
• Paraesophageal hernia; incidence higher after
laparoscopic than open FDP
I CLINICAL ISSUES
Presentation
• Dysphagia; transient in early post-operative period
• "Gas bloat" syndrome; upper abdominal fullness,
inability to belch, early satiety, flatulence
• Nausea, vomiting, epigastric pain, diarrhea
• Intrathoracic wrap migrations; small, asymptomatic
o 64% of radiologically visualized intrathoracic
migrations without clinical manifestations
• Intrathoracic gastric herniation after FDP; uncommon;
potentially life-threatening
o May lead to gastric volvulus; intrathoracic
incarceration of stomach; acute gastric perforation
• Too loose; disrupted FDP: Recurrent reflux symptoms
• Leaks: Pain, fever, leukocytosis
• Visceral injury: Pain, falling hematocrit
Natural History & Prognosis
• Advantages of laparoscopic FDP: Safe; effective;
reduced length of hospital stay & recovery time
o Effective even at long term follow-up; as effective as
open procedures with lower morbidity rate
• Laparoscopic Toupet vs. Nissen FDP
COMPLICATIONS
o Similar short term results
o In longer follow-up; no difference in incidence of
post FDP symptoms related to gas-bloat syndrome
o Recurrence of GERD: Nissen; 8% symptomatic
reflux; 4% by objective testing
• Toupet: 20% symptomatic; 51% objective
o Toupet FDP; higher incidence of proton pump
inhibitor resumption, overall dissatisfaction
o Superiority of total FDP over partial; even in setting
of moderate decreases in esophageal motility
• Laparoscopic FDP: 3.5-5% rate of early post-operative
complications
o Surgical failure rate requiring re-operation: 2-17%
• Outcome: Good; as long as FDP remains intact
o Keeping GE junction at hiatus, hiatus closed,
preventing recurrence of hernia
o Overall mortality rate: 0·3%
• Antireflux surgery undertaken primarily to improve
quality of life by relieving symptoms of GERD
o Small possibility of reflux symptoms becoming
worse after FDP operation; 1% to 2% of patients
o Creation of new symptoms due to side effects of
surgery; may adversely impact quality of life
Treatment
• Minimize complications: Surgeon experience; training
o Appropriate operative techniques
o Low threshold for early laparoscopic reexploration,
early radiological contrast studies
o 5-10% of time; may need to change to open
procedure while laparoscopic surgery in process
• Dilation of esophagus; reoperation to loosen wrap
around esophagus; if dysphagia persists
• Redo laparoscopic Nissen can be performed safely after
initiallaparoscopic approach; low failure rate
• Prevent recurrent hernia after laparoscopic Nissen FDP
o Appropriate closure of crura & anchoring suture
between stomach & diaphragm are helpful
o Reinforcement of hiatal crura using prosthetic mesh
I DIAGNOSTIC CHECKLIST
Consider
• Post-operative fluoroscopic evaluation should be used
liberally or even routinely
o CT for suspected leak or bleeding
I SELECTED REFERENCES
1. Graziano K et al: Recurrence after laparoscopic and open
Nissen fundoplication: a comparison of the mechanisms of
failure. Surg Endosc. 17(5):704-7,2003
2. Hainaux B et al: Intrathoracic migration of the wrap after
laparoscopic Nissen fundoplication: radiologic evaluation.
AJRAm J Roentgenol. 178(4):859-62, 2002
3. Fernand.o HC et al: Outcomes of laparoscopic Toupet
compared to laparoscopic Nissen fundoplication. Surg
Endosc. 16(6):905-8, 2002
4. Pavlidis TE: Laparoscopic Nissen fundoplication. Minerva
Chir. 56(4):421-6, 2001
5. Waring JP: Postfundoplication complications. Prevention
and management. Gastroenterol Clin North Am.
28(4):1007-19, viii-ix, 1999
 FUNDOPLICATION COMPLICATIONS
I IMAGE GALLERY

(Left) Axial NECT shows

intact fundoplication as a
soft tissue density "mass"
(arrow) in gastric fundus.
(Right) Axial CECT shows
large air-filled collection in
mediastinum (sterile)
following surgery.

3
63

(Left) Upper GI series shows

an intact "tight"
fundoplication with
persistent dilation of the
esophagus. (Right) Upper GI
series shows intrathoracic
migration of the intact
fundoplication.

(Left) Axial CECT shows

distal esophagus surrounded
by extravasated contrast
medium within the
mediastinum due to
perforation of the esophageal
wall. (Right) Axial CECT
shows intact gastric fundus
with oral contrast medium
surrounding the
fundoplication wrap.
 GASTRIC BYPASS COMPLICATIONS
3
64
Graphic shows typical procedure for a Roux-en-y gastric
bypass procedure, with a small gastric pouch
anastomosed to a Roux limb (75 to 150 cm long).
ITERMINOLOGY
Definitions
• Complications of gastric bypass surgery (GBS) for
morbid obesity
I IMAGING FINDINGS
General Features
• Laparoscopic Roux-en-Y gastric bypass (RYGB);
bariatric procedure of choice in North America
• RYGB procedure
o Gastric pouch: 15-30 ml; along lesser curvature of
proximal stomach; excluded from distal stomach
• Anastomosed end to side to Roux-en-Y limb
• Distal gastric remnant left in its normal anatomic
position
o Roux-en-Y limb; created by transection of jejunum
at 35-45 cm distal to ligament of Treitz
• 75 to 150 cm long
• Anastomosed side to side with proximal jejunum
o Roux limb may be brought through transverse
mesocolon to be placed in retrocolic position
• Or ante colic position; anterior to transverse colon
o Mesenteric defects of jejunum, transverse colon
sutured; closed with nonabsorbable sutures
• Gastrointestinal complications (> 10%)
DDx: Leak, Obstruction or Mass After Surgery
Anastomotic Edema Distended Stomach
Upper GI series shows minor anastomotic leak, evident
only as opacification of the surgical drain (arrow)
placed near the gastric pouch (open arrow) - Roux
anastomosis (curved arrow).
o Major complications; (require intervention;
potentially life threatening) (9.5%)
• Large anastomotic leak
• Small bowel obstruction
• Anastomotic stricture
• Gastro-gastric; gastroenteric fistula
• Gastrointestinal bleeding; abscess
o Minor complications (6.7%)
• Small leaks, marginal ulcers, pancreatitis,
esophagitis, cholelithiasis
• Extra-enteric complications: Pulmonary embolism,
pneumonia, lung atelectasis, wound infection
Radiographic Findings
• Fluoroscopy: Upper gastrointestinal (UGI) series
• Anastomotic leaks
o Most commonly at gastrojejunal anastomosis
o Less commonly at distal Roux anastomosis, bypassed
stomach, esophagus, hypopharynx
o Contrast material spills into peritoneal cavity
o Opacification of surgical drain placed adjacent to
anastomosis at surgery with contrast material
• May be only clue to presence of leak; especially
small leaks on first post operative day study
• Small bowel obstruction
o Most common etiology: Internal hernias &
adhesions
o Other causes: Incarcerated ventral hernia
• Gastric pouch bezoar formation
Cholecystitis Reflux into Stomach
 GASTRIC BYPASS COMPLICATIONS
Key Facts
Terminology
• Complications of gastric bypass surgery (GBS) for
morbid obesity
Imaging Findings
• Laparoscopic Roux-en-Y gastric bypass (RYGB);
bariatric procedure of choice in North America
• Major complications; (require intervention;
potentially life threatening) (9.5%)
• Large anastomotic leak
• Small bowel obstruction
• Anastomotic stricture
• Internal hernia (IH)
• Relatively fixed cluster of small bowel loops; often
seen in left upper quadrant or mid abdomen
• Cluster remaining high on erect radiographs
• Intussusception at entero-enterotomy site
o Internal hernia (IH)
o Overlap in UGI findings with adhesions & IH
• Small-bowel segments: Clustered; distended (> 2.5
em); abrupt angulation
• Transition between dilated & nondilated segments
• Stasis & delayed passage of contrast material
o Findings that favor diagnosis of IH
• Relatively fixed cluster of small bowel loops; often
seen in left upper quadrant or mid abdomen
• Cluster remaining high on erect radiographs
• Anastomotic stricture
o Stomal stenosis of gastrojejunostomy (common)
• Dilatation of gastric pouch; spherical shape;
air-fluid-contrast material levels
• Delayed passage of contrast material through
anastomosis
o Stenosis at jejuno-jejunal anastomosis is rare (0.9%)
• Less common complications
o Gastro-gastric; gastro-cutaneous fistulas may develop
rarely in cases with enteric content leak
o Marginal ulcers; rate of 0.5-1.4% after RYGB
• Result of exposure of gastrojejunal anastomosis to
gastric acid, or ischemia
• More common in "re-do" procedures
CT Findings
• Leaks: CT may demonstrate major & minor leaks; fluid
collections not evident on UGI series
o Fluid collections: Most commonly near anastomosis;
in left upper abdomen, especially perisplenic area
• May evolve into abscesses
• Infected collections: Loculation; enhancing rim;
air-fluid levels; gas bubbles
• CT guided placement of drainage catheter into
fluid collection obviates surgery in many cases
• Internal hernia: CT appearance depends on location
o Abnormal clustering of small bowel loops;
congestion & crowding of mesenteric vessels
• Seen in all IH cases
o Transmesenteric IH: Herniation through mesocolon,
small bowel mesentery; most common
Top Differential Diagnoses
• Post-op anastomotic edema
• Post-op ileus
• Extra gastric complications
• Reflux into bypassed stomach
Clinical Issues
• Major complications: Require surgical intervention
• Minor complications; usually resolve spontaneously
Diagnostic Checklist
• CT & UGI series are important & complimentary in
evaluation of these complications 3
65
• Small bowel cluster located posterior to remnant
stomach exerting mass effect on its posterior wall
o IH through small bowel mesentery
• Cluster of small bowel pressed against anterior
abdominal wall with no overlying omental fat
• Causing central displacement of cqlon
o Peterson type hernias; very difficult to diagnose
• Herniation behind Roux-en-Y loop before passing
through defect in transverse mesocolon
• May not be apparent on CT; there is neither
confining border nor characteristic location
• Engorgement, crowding of mesenteric vessels &
evidence of obstruction may be only clues
• Other less common complications
o Obstruction & perforation of distal stomach
• Markedly dilated distal stomach; free
intraperitoneal air if perforation
• Rarely seen with laparoscopic approach; fatal
o Incisional & ventral hernias; infection of abdominal
wall wound; seen with open procedure
• Uncommon with laparoscopic RYGB
Imaging Recommendations
• Best imaging tool
o CT & UGI radiography; complementary roles
• Imaging of post-operative anatomy; complications
• Allowing early diagnosis & treatment
• Protocol advice
o UGI series with water-soluble contrast material;
performed routinely; within 24 hours after surgery
• May repeat study later if leak depicted or
suspected clinically
• Barium given subsequently after gastrointestinal
extravasation excluded; delineate anatomy better
• Evaluate pouch emptying; reflux into duodenum
o CT used if small bowel obstruction or intra
abdominal abscess suspected
• In all patients with unexplained fever, pain,
abdominal distension following RYGB
 GASTRIC BYPASS COMPLICATIONS
I DIFFERENTIAL DIAGNOSIS
Post-op anastomotic edema
• Delay in passage of contrast material at anastomotic
site; early post-operative period obstruction; resolves
Post-op ileus
• Small + large bowel ± gastric distension
• Delayed but free passage of contrast material
• Usually resolves by 4th post-operative day
Extra gastric complications
• Cholecystitis, pulmonary embolism, etc.
3 Reflux into bypassed stomach
• Via retrograde passage of oral contrast through
66 duodenojejunal segment
• Can simulate a leak
I PATHOLOGY
General Features
• General path comments
o Indications for GBS: Morbid obesity
• Body mass index: 35 kg/m2 with co morbidity
• Or 40 kg/m2 without comorbidity
o Bariatric procedures: Restrictive & combination
• Restrictive: Gastric capacity reduced; early sense of
fullness after ingestion of small quantities of food
• Combination: Part of digestive tract bypassed;
causing t absorption of nutrients & calories
• Etiology
o Surgical technique
o Leaks: Noncompliance of patients; premature
ingestion of food or fluids early postoperative period
o IH: Rapid massive weight reduction, results in t
intraperitoneal fat; enlarges mesenteric defect
• Epidemiology
o During last 3 decades, incidence of overweight
American adults nearly tripled to 35%
o RYGB, combination procedure; most common
bariatric procedure in North America
ICLINICAL ISSUES
Presentation
• Leaks: Incidence of 1-6% after laparoscopic RYGB
o Most dreaded complication of GBS surgery; may
result in sepsis & even death
o Leaks usually occur within first 10 days of surgery
o May present with only tachycardia, abdominal
discomfort, with no signs of peritonitis or fever
o High index of suspicion; especially if respiratory
distress & tachycardia> 120 beats per minute
• Small bowel obstruction
o Reported in 4-5% of patients after laparoscopic GBS
o Laparoscopic approach, associated with less trauma;
fewer adhesions; higher prevalence of IH (2.8%)
o Retrocolic placement of Roux limb, more frequently
associated with IH
• Regardless of suture closure of mesenteric defects
• Antecolic approach has become more popular
o Early obstructions, within 3 days to 3 months of
surgery; more commonly due to adhesions
• IHs develop later (in 93% > 1 month after surgery)
o Clinical symptoms of IH: nonspecific, intermittent;
nausea, distension, abdominal pain
• High index of suspicion; in patients presenting
with abdominal pain after surgery
• Transmesenteric IH prone to volvulus &
strangulation of small bowel
o May result in closed loop obstruction; can be lethal
• Stenosis at gastrojejunostomy; due to relative ischemia
o Incidence; up to 27% after RYGB
o Dysphagia, vomiting, dehydration, excessive weight
loss; diagnosis usually made with endoscopy
o Late strictures; may present months after surgery
Natural History & Prognosis
• Advantages of bariatric surgery: Reliable, significant
weight loss
o Extended weight maintenance; control or reversal of
some obesity-related health problems
• RYGB: Greater weight loss than other procedures
o Good long term weight loss & patient tolerance
o Acceptable short & long term complication rates
• Laparoscopic approach to RYGB: t post-operative pain
& complications; shorter hospital stay; faster recovery
o Less invasive; especially benefits high-risk morbidly
obese patients with multiple comorbidities
• Mortality: 0.4% after laparoscopic RYGB
Treatment
• Major complications: Require surgical intervention
o Laparoscopy; excellent technique to treat these
complications
• Anastomotic strictures: Endoscopic balloon dilatation
• Minor complications; usually resolve spontaneously
I DIAGNOSTIC CHECKLIST
Consider
• Nonspecific clinical presentation of some of
gastrointestinal complications of GBS
o CT & UGI series are important & complimentary in
evaluation of these complications
I SELECTED REFERENCES
1. Champion JK et al: Small bowel obstruction and internal
hernias after laparoscopic Roux-en-Y gastric bypass. Obes
Surg. 13(4):596-600, 2003
2. Hamilton EC et al: Clinical predictors of leak after
laparoscopic Roux-en-Y gastric bypass for morbid obesity.
Surg Endosc. 17(5):679-84,2003
3. Papasavas PK et al: Laparoscopic management of
complications following laparoscopic Roux-en-Y gastric
bypass for morbid obesity. Surg Endosc. 17(4):610-4,2003
4. Blachar A et al: Gastrointestinal complications of
laparoscopic roux-en-Y gastric bypass surgery in patients
who are morbidly obese: findings on radiography and CT.
AJRAm J Roentgenol. 179(6):1437-42, 2002
5. Blachar A et al: Gastrointestinal complications of
laparoscopic Roux-en-Y gastric bypass surgery: clinical and
imaging findings. Radiology. 223(3):625-32, 2002
 GASTRIC BYPASS COMPLICATIONS

I IMAGE GAllERY

(Left) Upper GI series shows

anastomotic stricture (arrow)
between the distended
gastric pouch and the Roux
limb. (Right) Upper GI series
shows leak (arrows)
following placement of a
gastric band (open arrow)
around the gastric fundus.

3
67

Typical
(Left) Axial CECT shows
internal hernia following
Roux-en-Y gastric bypass
(RYG8). The mesenteric
vessels (arrow) to the
herniated loops are crowded
+ swirled & the herniated
bowel (open arrow) is
dilated. (Right) Axial CECT
shows major leak following
RYG8 with extravasated oral
contrast seen (arrows).

(Left) Upper GI small bowel

follow through (58FT) shows
contrast opacification of
bowel following RYG8 and
intraperitoneal collections
(arrows) due to leak at
jejuno-jejunal anastomosis.
(Right) Upper GI series
shows marginal ulcer
(arrow) within the Roux limb
just beyond the anastomosis
with the gastric pouch.
 GASTRIC BEZOAR

3
68

Upper CI series shows fixed filling defect in stomach Axial CECT shows laminated mass (arrow) in stomach
with a swirled pattern-trichobezoar. due to phytobezoar.

o Lactobezoar: Undigested milk concretions

ITERMINOlOGY o Pharmacobezoar: Bezoar comprised of medications
Definitions Radiographic Findings
• Intragastric mass composed of accumulated ingested
• Radiography
(but not digested) material
o Abdominal plain film: Soft-tissue mass floating in
stomach at air-fluid interface
• Mottled radiotransparencies in interstices of solid
I IMAGING FINDINGS matter
General Features o ± Bowel obstruction
o Insensitive test; bezoar identified in 10-18% of
• Best diagnostic clue: CT or fluoroscopy: Intraluminal
patients from radiographs alone
mass containing mottled air pattern
• Fluoroscopy
• Location
o Intraluminal filling defect
o Sites of impaction: Stomach, jejunum, ileum
• With finely lobulated, villous-like surface
• Narrowest portion of small bowel 50-75 cm from
ileocecal valve or valve itself • Without constant site of attachment to bowel wall
o Barium or iodinated contrast outline bezoar;
• Any part can be affected; especially in patients
unattached intraluminal mass
with postoperative adhesions
o Mottled or streaked appearance; contrast medium
• Morphology
entering interstices of bezoar
o Persistent concretions of foreign matter
o Filling defect may occasionally appear completely
• Classified according to materials of which they are
smooth
composed
o Phytobezoar: Undigested vegetable matter • Could be mistaken for an enormous gas bubble
that is freely movable within stomach
• Poorly digested fibers; skin + seeds of fruits &
o Coiled-spring appearance (rare)
vegetables
o Partial or complete obstruction
• Diospyrobezoar: Persimmons
• Try to distinguish obstruction due to
o Trichobezoars: Accumulated, matted mass of hair
postoperative adhesions from bezoar-induced
o Trichophytobezoar: Both hair & vegetable matter
obstruction

DDx: large Filling Defect in Stomach

Gastric Cancer Gastric Cancer Gastric GIST Gastric Lymphoma

 GASTRIC BEZOAR
Key Facts
Terminology
• Intragastric mass composed of accumulated ingested
(but not digested) material
Imaging Findings
• Phytobezoar: Undigested vegetable matter
• Trichobezoars: Accumulated, matted mass of hair
• Intraluminal filling defect
• With finely lobulated, villous-like surface
• Without constant site of attachment to bowel wall
• Mottled or streaked appearance; contrast medium
entering interstices of bezoar
• Well-defined, oval, low-density, intraluminal mass
• Mottled air pattern
• Diagnose bezoar-induced obstruction
CT Findings
• Well-defined, oval, low-density, intraluminal mass
o Mottled air pattern
o Mottled appearance is result of air bubbles retained
in interstices of mass
o Heterogeneous mass without post-contrast
enhancement
• Pockets of gas, debris, fluid scattered throughout;
with no air-fluid level within lesion
• Small bezoars are rounded or ovoid; tend to float on
water-air surface surrounded by gastric contents
o Oral contrast material may be seen surrounding
mass, establishing free intraluminal location
• Large bezoars tend to fill lumen
Ultrasonographic Findings
• Intraluminal mass with hyperechoic arc-like surface
o With marked acoustic shadowing
• With US; identification of additional intestinal or
gastric bezoars may be difficult
Imaging Recommendations
• Best imaging tool
oCT; more accurate in confirming diagnosis of gastric
bezoar suggested by other modalities
• Diagnose bezoar-induced obstruction
• Detect presence of additional gastric or intestinal
bezoars
• Protocol advice
o May go undetected if CT scan obtained at routine
abdominal soft tissue window & level settings
• Modifying window setting by reducing level to
-100 HU makes it possible to better identify
I DIFFERENTIAL DIAGNOSIS
Gastric carcinoma
• Filling defect in stomach; polypoid or fungating
• Lesion on dependent or posterior wall seen as filling
defect in barium pool
• Wall thickening; ulceration; irregular narrowing &
rigidity; amputation of folds; stenosis
• Detect presence of additional gastric or intestinal
bezoars
Top Differential Diagnoses
• Gastric carcinoma
• Post-prandial food
• Intramural mass
Clinical Issues
• In adults, bezoars are most frequently encountered
after gastric operation
• Trichobezoars seen especially in those with
schizophrenia or other mental instability
• Complications: Decubitus ulceration + pressure
3
necrosis of bowel wall, perforation, peritonitis 69
• Symptomatic, large phytobezoars or trichobezoars
require endoscopic fragmentation or surgical removal
Post-prandial food
• Fluoroscopy: Intraluminal filling defect
• Food usually less mass-like
• CT: Bezoar shows lower density than food particles
o Occasionally difficult to differentiate bezoar from
large amount of retained food
Intramural mass
• Stromal tumor (GIST); lymphoma; melanoma
metastases
o Lobulated or polypoid filling defects; arising from
gastric wall
o Infiltration of gastric wall; mucosal thinning or
ulceration; submucosal mass
I PATHOLOGY
General Features
• General path comments
o Predisposing causes
• Previous gastric surgery: Vagotomy, pyloroplasty,
antrectomy, partial gastrectomy
• Inadequate chewing, missing teeth, dentures
• Overindulgence of foods with high fiber content
• Altered gastric motility: Diabetes, mixed
connective tissue disease, hypothyroidism
• Etiology
o Material unable to exit stomach
• Accumulated due to large size; indigestibility;
gastric outlet obstruction; poor gastric motility
o Phytobezoar: Unripe persimmon fruit, oranges
• Persimmon contains tannin; coagulates on
contact with gastric acid
• Glue-like coagulum formed; traps seeds, skin, etc.
o Medications reported to cause bezoars
• Aluminum hydroxide gel, enteric-coated aspirin,
sucralfate, guar gum, cholestyramine
• Enteral feeding formulas, psyllium preparations,
nifedipine XL, meprobamate
• Epidemiology
o Incidence: 0.40/0 (large endoscopic series)
 GASTRIC BEZOAR
o Phytobezoar: 55% of all bezoars
o Phytobezoar responsible for 0.4-4% of all intestinal
obstructions
• Associated abnormalities
o Peptic ulcer; incidence high; especially with more
abrasive phytobezoars
• Trichobezoars associated with gastric ulcer in
24-70%
o Concurrent gastric bezoar found in 17-53% of
patients with small-bowel bezoar
Gross Pathologic & Surgical Features
• Conglomerates of food or fiber in alimentary tract
• Hairball
3
70 I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic; incidentally found on imaging
o Anorexia, bloating, early satiety
o Crampy epigastric pain
o Sense of dragging, heaviness in upper abdomen
o With large bezoars; symptoms of pyloric obstruction
• Can clinically simulate gastric carcinoma
o May present with small bowel obstruction
o Trichotillomania; impulse disorder to pull out hair
from scalp, eyelashes, eyebrows, other parts of body
• With trichotillomania; gastric trichobezoar may
result in failure to gain weight
• Iron deficiency anemia, painless epigastric mass
• Clinical profile
o History of recent ingestion of pulpy foods
o History of previous gastric surgery
o Physical examination: Bald patches on patient's
head or bald sibling as proof; with trichobezoar
• In adults, bezoars are most frequently encountered
after gastric operation
o In children, associated with pica, mental
retardation, coexistent psychiatric disorders
• Trichobezoars seen especially in those with
schizophrenia or other mental instability
o Primarily girls who chew & swallow their own hair
• Lactobezoar, most often found in infants
o Pre-term infants on caloric-dense formulas
o Immature mechanism of gastric emptying
Demographics
• Age: Trichobezoar: 80% are in age less than 30 years
• Gender: Trichobezoars occur predominantly in
females
Natural History & Prognosis
• Bezoars of any type most often occur in background of
altered motility or anatomy of gastrointestinal tract
• Bezoars usually form in stomach
o Fragment & enter small bowel where they absorb
water, increase in size & become impacted
• Bezoars are an uncommon cause of acute gastric outlet
obstruction
• Trichobezoar: Can enlarge to occupy entire lumen of
stomach assuming shape of organ
o Trichobezoars do not usually migrate toward small
bowel
• Rapunzel syndrome, found characteristically in girls
with varying gastrointestinal symptoms
o Rare form of gastric trichobezoar extending
throughout the bowel
o Possessing "tail" which extends to or beyond
ileo-cecal valve; causing intestinal obstruction
o High comorbidity of serious pediatric psychiatric
disorders
• Complications: Decubitus ulceration + pressure
necrosis of bowel wall, perforation, peritonitis
o Bleeding, obstructive jaundice, intussusception &
appendicitis
Treatment
• Endoscopic lavage fragmentation + extraction presents
safe method of bezoar resolution
• Symptomatic, large phytobezoars or trichobezoars
require endoscopic fragmentation or surgical removal
• Diagnosis of bezoar as cause of obstruction important
o Modifies approach to treatment; accelerating use of
surgery
o Bezoar-induced bowel obstruction rarely improves
with conservative treatment
o Early surgery required to secure definitive solution
• 9% of patients may require second operation
o Recurrent bowel obstruction; caused by presence of
residual bezoar
• Spontaneous expulsion of bezoar; uncommon
I DIAGNOSTIC CHECKLIST
Consider
• Bezoar formation may be more common than
previously thought
o High index of suspicion could help avoid costly
evaluations for obstructive symptoms
• When an intestinal bezoar is diagnosed, consider
concomitant gastric bezoar
• Discrepancy between CT & surgical localization
o May be caused by migration of bezoar during
interval between imaging & surgery
I SELECTED REFERENCES
1. Ripolles T et al: Gastrointestinal bezoars: sonographic and
CT characteristics. A]R Am] Roentgenol. 177(1):65-9,2001
2. DuBose TM 5th et al: Lactobezoars: a patient series and
literature review. Clin Pediatr (Phila). 40(11):603-6, 2001
3. Morris B et al: An intragastric trichobezoar: computerised
tomographic appearance.] Postgrad Med. 46(2):94-5, 2000
4. Gayer G et al: Bezoars in the stomach and small bowelnCT
appearance. Clin Radial. 54(4):228-32, 1999
5. West WM et al: CT appearances of the Rapunzel syndrome:
an unusual form of bezoar and gastrointestinal
obstruction. Pediatr Radiol. 28(5):315-6, 1998
6. Phillips MR et al: Gastric trichobezoar: case report and
literature review. Mayo Clin Proc. 73(7):653-6, 1998
7. Newman B et al: Gastric trichobezoars--sonographic and
computed tomographic appearance. Pediatr Radial.
20(7):526-7, 1990
 GASTRIC BEZOAR

[IMAGE GAllERY
Typical
(Left) Upper CI series in a 3
year old girl shows large
mass in stomach:
Trichobezoa~ (Righ~ Upper
CI series in edentulous adult
shows mottled filling defects
in stomach: Phytobezoars.

3
71

(Left) Upper CI series in a

patient with Bilroth 7 type
partial gastrectomy shows
bezoar in stomach. (Right)
Upper CI series in a patient
with Bilroth 7 type partial
gastrectomy shows bezoar in
stomach.

Typical
(Left) Upper CI series in a
patient with a Bilroth 2 type
partial gastrectomy shows a
large bezoar in stomach.
(Right) Upper CI series in a
patient with a Bilroth 7 type
partial gastrectomy shows a
bezoar in stomach.
 GASTRIC VOLVULUS
3
72
Upper GI series shows intrathoracic stomach
organoaxial volvulus and partial obstruction.
ITERMINOlOGY
Abbreviations and Synonyms
• Gastric volvulus (GV)
Definitions
• Uncommon acquired twist of stomach on itself
I IMAGING FINDINGS
General Features
• Morphology: Abnormal degree of rotation of one part
of stomach around another part
• Types of GV: Organoaxial (most common);
mesenteroaxial; mixed
• Organoaxial volvulus (OAV):Rotation of stomach
around its longitudinal axis (most common form)
o Around line extending from cardia to pylorus
o Stomach twists either anteriorly or posteriorly
o Antrum moves from an inferior to superior position
• Mesenteroaxial volvulus (MAV):Rotation of stomach
about mesenteric axis
o Axis running transversely across stomach at right
angles to lesser & greater curvatures
o Stomach rotates from right to left or left to right
about long axis of gastrohepatic omentum
• Mixed volvulus: Combination of OAV & MAV
DDx: Intrathoracic Stomach
Hiatal Hernia Post-Operative
with Upper GI series shows intrathoracic stomach with
organoaxial volvulus, but no obstruction.
Radiographic Findings
• Radiography
o Abdominal plain films; patient upright
• Double air-fluid level
• Large, distended stomach; seen as air & fluid-filled
spheric viscus displaced upward & to left
• Associated elevation of diaphragm
• Usually small bowel collapsed; uncommon to see
gas shadow beyond stomach
• May see radiolucent line within gastric wall;
caused by intramural emphysema
o Chest film: Intrathoracic; up-side down stomach
• Retrocardiac fluid level; two air-fluid interfaces at
different heights; suggests intrathoracic GV
• Simultaneous fluid levels above & below
diaphragm are not required to make diagnosis
• Fluoroscopy
o Massively distended stomach in left upper quadrant
extending into chest
o Inversion of stomach
• Greater curvature above level of lesser curvature
• Positioning of cardia & pylorus at same level
• Downward pointing of pylorus & duodenum
o OAV:2 points of twist; luminal obstruction
o Incomplete or absent entrance of contrast material
into +/or out of stomach; acute obstructive GV
• OAV: Failure of contrast to enter stomach;
obstruction at esophagus or proximal stomach
- ".~ ....
A~
--•
Post-Operative fpi. Diverticulum
 GASTRIC VOLVULUS
Key Facts
Terminology
• Uncommon acquired twist of stomach on itself
Imaging Findings
• Organoaxial volvulus (OAV): Rotation of stomach
around its longitudinal axis (most common form)
• Around line extending from cardia to pylorus
• Mesenteroaxial volvulus (MAV):Rotation of stomach
about mesenteric axis
• Axis running transversely across stomach at right
angles to lesser & greater curvatures
• Double air-fluid level
• Inversion of stomach
• Greater curvature above level of lesser curvature
• Positioning of cardia & pylorus at same level
• Downward pointing of pylorus & duodenum
• If contrast material does enter stomach, it may not
pass beyond obstructed pylorus
o May see "beaking" at point of twist
o MAV:Antrum & pylorus lie above gastric fundus
CT Findings
• CT appearance may be variable
o Depends upon extent of gastric herniation, points of
torsion & final positioning of stomach
o May see linear septum within gastric lumen;
corresponding to area of torsion
• CT chest & abdomen; performed pre-operatively
o To detect associated malformation or malposition &
if possible, site, size, level of diaphragmatic gap
o Presence of unattached herniated peritoneal sac
• Large hiatal hernia accompanied by partial GV; may
mimic appearance of thrombus in inferior vena cava
o "Pseudothrombosis" of inferior vena cava on CT
MR Findings
• Coronal images demonstrate 2 points of twisting
o 2 different signal intensities reflect point of torsion
Angiographic Findings
• GV may present as acute upper gastrointestinal
hemorrhage
Imaging Recommendations
• Best imaging tool
o Fluoroscopic barium studies
• Demonstrates area of twist; anatomic detail
o Fluoroscopic guidance may help in advancing
nasogastric tube into obstructed stomach
• May allow decompression; stabilize patient
oCT; complementary role
I DIFFERENTIAL DIAGNOSIS
Hiatal hernia
• Stomach entering thorax through esophageal hiatus
• Gastroesophageal (GE) junction above diaphragmatic
hiatus (type I, sliding)
• Incomplete or absent entrance of contrast material
into +/or out of stomach; acute obstructive GV
Top Differential Diagnoses
• Hiatal hernia
• Post-operative
Pathology
• Large esophageal or paraesophageal hernia
• Diaphragmatic eventration or paralysis
Clinical Issues
•
•
Complications: Intramural emphysema; perforation
Mortality rate: 30%
3
• Detorse stomach
73
• Repair of associated defects
• Prevent recurrence
• Herniation of fundus through hiatus; GE junction
below diaphragm (type II; paraesophageal)
• Giant paraesophageal hernia: At least one third of
stomach herniated into chest
o Associated herniation of other abdominal viscera
into chest; including colon or small bowel
• Traction or torsion of stomach at or near level of
hiatus (volvulus)
Post-operative
• Esophagectomy with gastric pull through procedure
• Complete mobilization of stomach, resection of lower
esophagus, pyloroplasty, transhiatal dissection
o Intrathoracic stomach
Epiphrenic diverticulum
• Epiphrenic diverticulum
I PATHOLOGY
General Features
• General path comments
o Point of anatomic fixation: Second portion of
duodenum retains retroperitoneal position
• Becomes fixed to posterior abdominal wall
o Several ligaments normally anchor stomach within
abdomen & limit free upward movement
• 4 suspensory ligaments; gastrohepatic,
gastrosplenic, gastrocolic, gastrophrenic
• Gastrolienal ligaments also contribute to fixation
of stomach
o Due to sites of anatomic fixation, torsion of stomach
may occur with significant degrees of herniation
o Predisposing factors: Bands, adhesions
• Rapid changes in intraabdominal pressure;
degenerative changes; 1 size of esophageal hiatus
• Unusually long gastrohepatic + gastrocolic
mesenteries
• Etiology
o Primary GV: Stabilizing ligaments are too lax as a
result of congenital or acquired causes
• Absence of tethering gastric ligaments
 GASTRIC VOLVULUS
• One third of cases
o Secondary GV: Paraesophageal hernia
• Congenital or acquired diaphragmatic defects
• In children; secondary to Morgagni hernia
o Idiopathic; no apparent cause
• Epidemiology
o In children, MAV most common type; associated
anatomic defects are the rule
o Five cases of combined organomesenteroaxial GV in
children reported in world literature
• Associated abnormalities
o Large esophageal or paraesophageal hernia
• Permits part or all of stomach to assume
intrathoracic position
3 o Diaphragmatic eventration or paralysis
o Wandering spleen: Absence of ligamentous
74
connections between stomach, spleen
o Hernia of colonic transverse loop with anterior OAV
• In these cases; concomitant sliding hernia
Gross Pathologic & Surgical Features
• Partial or complete volvulus
• Term "gastric volvulus" used by some to identify
abnormalities of gastric position without obstruction
o Upside-down stomach"; gastric displacement
through sliding & large paraesophageal hernias
• "True volvulus"; term used only when obstruction
I CLINICAL ISSUES
Presentation
• GV can be asymptomatic if no outlet obstruction or
vascular compromise; incidental finding on imaging
• Acute volvulus; associated interference of blood supply
o. Surgical emergency
o Classic clinical triad (Borchardt triad)
• Violent retching with production of little vomitus
• Constant severe epigastric pain
• Great difficulty in advancing nasogastric tube
beyond distal esophagus
• Chronic GV: May present in chronic or recurrent form
o Frequently not recognized early in its presentation
o Vague & nonspecific symptoms suggestive of other
abdominal processes; causing delay in diagnosis
o May be discovered unexpectantly during clinical
work-up for an unrelated condition
• CT & MR often requested as first radiographic
study during evaluation
• Symptomatic GV in infancy & childhood may not be
as rare as is commonly assumed
Demographics
• Age
o Seen in both pediatric & adult patients
o Primarily after fourth decade of life
Natural History & Prognosis
• In small herniations, proximal portion of stomach
enters hernia sac first
o Obstruction or strangulation almost never occur at
this stage
• As herniation progresses; body & variable portion of
antrum come to lie above diaphragm
o Stomach can become entirely intrathoracic organ;
prone to volvulus
• Obstruction can occur at points of torsion or twisting
o Or at points where stomach redescends through
hiatus, fills & tightens in hernial ring
o As much as 180 degrees of twisting may occur
without obstruction or strangulation
o Twisting beyond 180 degrees usually produces
complete obstruction & clinically acute abdomen
o OAV:Can obstruct; does not usually result in
strangulation
• MAV:Can occlude gastric vessels; strangulation
• "Upside-down stomach"
o In typical case, sliding hernia & stomach (180
degrees OAV) pass through same diaphragmatic gap
• An enlarged esophageal hiatus or Bochdalek defect
o Presents with bleeding & anemia; does not usually
induce obstruction or strangulation
• Vascular occlusion leads to necrosis, shock
• Complications: Intramural emphysema; perforation
o Strangulation may lead to mucosal ischemia
• Areas of focal necrosis; may permit gas to dissect
into gastric wall
o Perforation may result from full-thickness necrosis
• Prognosis: GV is potentially catastrophic condition
• Mortality rate: 30%
Treatment
• Goals: Early recognition & surgical repair
o Detorse stomach
o Repair of associated defects
• Hiatal hernia repair
• Gastropexy; may be prophylactic
o Prevent recurrence
• Laparoscopic detorsion & percutaneous endoscopic
gastropexy
• Gastric resection; for strangulation & necrosis
• Upside-down stomach: Balloon repositioning; fixation
by percutaneous endoscopic gastrostomy
I DIAGNOSTIC CHECKLIST
Consider
• Anatomical detail of stomach often better delineated
on upper gastrointestinal studies
o Identification of GV as incidental finding on CT
• Should be excluded whenever stomach is noted
not to be in normal anatomic position
I SELECTED REFERENCES
1. Shivanand G et al: Gastric volvulus: acute and chronic
presentation. Clin Imaging. 27(4):265-8, 2003
2. Tabo T et al: Balloon repositioning of intrathoracic
upside-down stomach and fixation by percutaneous
endoscopic gastrostomy. J Am ColI Surg. 197(5):868-71,
2003
3. Godshall D et al: Gastric volvulus: case report and review
of the literature. J Emerg Med. 17(5):837-40, 1999
4. Schaefer DC et al: Gastric volvulus: an old disease process
with some new twists. Gastroenterologist. 5(1):41-5, 1997
5. Chiechi MV et al: Gastric herniation and volvulus: CT and
MR appearance. Gastrointest Radiol. 17(2):99-101, 1992
 GASTRIC VOLVULUS
I IMAGE GALLERY

(Left) fA chest radiograph

shows distended
intrathoracic stomach due to
volvulus with acute
obstruction. (Right) Lateral
chest radiograph shows
distended intrathoracic
stomach due to volvulus
with acute obstruction.

3
75

(Left) Upper GI series shows

eventration or paralysis of
left diaphragm and
mesenteroaxial gastric
volvulus with obstruction.
(Right) Axial CECT shows
mesenteroaxial gastric
volvulus with the stomach
rotated left-to-right.

(Left) Upper GI series shows

intrathoracic stomach with
organoaxial volvulus and
obstruction. (Right) Upper
GI series shows mixed
organo- and mesenteroaxial
gastric volvulus with
obstruction.
 AORTO-ENTERIC FISTULA

3
76

Graphic shows fistula between transverse duodenum Axial CECT shows fluid & gas bubble between the graft
and the aorta at the site of graft-aortic suture line. lumen & aortic wall, which is wrapped around the graft.
At surgery the graft was infected & a fistula was found to
the duodenum (arrow).

• Pseudoaneurysm formation
ITERMINOlOGY • Disruption of aneurysmal wrap .
Definitions • 1 Soft tissue between graft and aneurysmal wrap
• Abnormal communication between aorta & • Contrast in pseudo aneurysm (arterial phase)
gastrointestinal (GI) tract • Increased attenuation of intestinal lumen contents
(arterial phase); decreased attenuation (delayed phase)
Nuclear Medicine Findings
I IMAGING FINDINGS • Tagged RBC within abdominal aorta & enters bowel
General Features Imaging Recommendations
• Best diagnostic clue: Inflammatory stranding and gas • Best imaging tool: CT: 94% sensitive & 85% specific
between abdominal aorta and third part of duodenum
following aneurysm repair
• Location: Duodenum (80%) > jejunum and ileum I DIFFERENTIAL DIAGNOSIS
(10-15%) > stomach and colon (5%)
Periaortitis
Radiographic Findings
• Also known as inflammatory peri aneurysmal fibrosis
• Fluoroscopic-guided barium studies • Soft tissue attenuation encasing aorta, inferior vena
o Compression or displacement of third portion of cava and other structures
duodenum by an extrinsic mass
o Contrast extravasation: Wall of abdominal aorta Retroperitoneal fibrosis
outlined by extraluminal contrast medium tracking • Mantle of soft tissue enveloping aorta, IVC, ureters
along the graft into periaortic space (rare)
Post-operation
CT Findings • "Normal" scarring with fluid between graft & aorta
• Ectopic gas: Microbubble of gas adjacent to aortic
graft; may suggest perigraft infection Post-endovascular stent
• Focal bowel wall thickening> 5 mm • Endoleak: Blood flow outside the stent, but within an
• Perigraft soft tissue thickening> 5 mm (> 20 HU) aneurysm sac or adjacent vascular segment

DDx: Periaortic Inflammation

~.~~
-
•
-
..
~"i~~" ..,-
0;
~'--
':.

Periaortitis Retrop. Fibrosis Endo/eak Endo/eak

 AORTO-ENTERIC FISTULA

Key Facts
Imaging Findings • Post-endovascular stent
• Best diagnostic clue: Inflammatory stranding and gas Clinical Issues
between abdominal aorta and third part of • "Herald" GI bleeding, followed by hours, days or
duodenum following aneurysm repair weeks by catastrophic hemorrhage (most common)
• Best imaging tool: CT: 94% sensitive & 85% specific
Diagnostic Checklist
Top Differential Diagnoses
• Clinical and past surgical history; diagnosis requires
• Periaortitis emergent surgery
• Post-operation • Perigraft infection 1 suspicion of fistula

• May have couple of gas bubbles between stent-graft +

Natural History & Prognosis
3
aortic wall soon after placement
• Prognosis: Very poor, up to 85% mortality 77
Treatment
I PATHOLOGY • Extensive reconstructive surgery
General Features
• Etiology
o Primary I DIAGNOSTIC CHECKLIST
• Abdominal aortic aneurysms Consider
• Infectious aortitis • Clinical and past surgical history; diagnosis requires
• Penetrating peptic ulcer emergent surgery
• Tumor invasion
• Radiation therapy Image Interpretation Pearls
o Secondary • Perigraft infection 1 suspicion of fistula
• Aortic reconstructive surgery (most common)
o Pathogenesis
• Third portion of duodenum is fixed & apposed to I SELECTED REFERENCES
anterior wall of aortic aneurysm ~ pressure
1. Perks F] et al: Multidetector computed tomography
necrosis imaging of aortoenteric fistula. ] Com put Assist Tomogr.
• Surgery ~ blood supply compromised 28(3):343-7,2004
• Pseudo aneurysm formation with erosion 2. Puvaneswary M et al: Detection of aortoenteric fistula with
• Graft & suture line infection ~ anastomotic helical CT. Australas Radiol. 47(1):67-9, 2003
breakdown 3. Lenzo NP et al: Aortoenteric fistula on (99m)Tc erythrocyte
• Intraoperative injury to adjacent bowel scintigraphy. A]R Am] Roentgenol. 177(2):477-8,2001
• Epidemiology 4. Orton DF et al: Aortic prosthetic graft infections: radiologic
manifestations and implications for management.
o Incidence: 0.6-1.5% after aortic surgery
Radiographies. 20(4):977-93, 2000
o Onset after surgery: 3 years; 21 days up to 14 years 5. Low RN et al: Aortoenteric fistula and perigraft infection:
• Associated abnormalities: Perigraft infection evaluation with CT. Radiology. 175(1):157-62, 1990

I CLINICAL ISSUES I IMAGE GALLERY

Presentation
• Most common signs/symptoms
o "Herald" GI bleeding, followed by hours, days or
weeks by catastrophic hemorrhage (most common)
o Abdominal or back pain, palpable and pulsatile mass
o Intermittent rectal bleeding and recurrent anemia
o Low-grade fever, fatigue, weight loss, leukocytosis
(infection of graft and perigraft area)
• Diagnosis
o Esophagogastroduodenoscopy: Exclude obvious
causes of bleeding
o Helical CT: Definitive diagnosis
Demographics (Left) Barium enema shows extravasation of contrast which outlines a
left common iliac artery graft (arrows). (Right) Axial NEeT shows
• Age: > 55 years of age mantle of soft tissue and gas surrounding abdominal aorta and graft.
• Gender: M:F = 4-5:1 Fatal aorto-duodenal fistula and hemorrhage.
SECTION 4: Small Intestine

Introduction and Overview

Small Intestine Anatomy and Imaging Issues 1-4-2

Congenital
Malrotation 1-4-6
Meckel Diverticulum 1-4-8

Infection
Intestinal Parasitic Disease 1-4-12
Opportunistic Intestinal Infections 1-4-14

Inflammation
Sprue-Celiac Disease 1-4-18
Whipple Disease 1-4-22
Mastocytosis 1-4-24
Crohn Disease 1-4-26
Scleroderma, Intestinal 1-4-30

Vascular Disease
Pneumatosis of the Intestine 1-4-34
Ischemic Enteritis 1-4-38
Vasculitis, Small Intestine 1-4-42

Trauma
Intestinal Trauma 1-4-46
Gastrointestinal Bleeding 1-4-50

Neoplasm, Benign
Intramural Benign Intestinal Tumors 1-4-54
Hamartomatous Polyposis (P-J) 1-4-56
Small Bowel Carcinoma 1-4-58

Neoplasm, Malignant
Carcinoid Tumor 1-4-60
Intestinal Metastases and Lymphoma 1-4-64

Miscellaneous
Small Bowel Obstruction 1-4-68
Gallstone Ileus 1-4-72
Malabsorption Conditions 1-4-74
Intussusception 1-4-78
Radiation Enteritis 1-4-82
 SMALL INTESTINE ANATOMY AND IMAGING ISSUES

Graphic shows schematic cross section of small intestine 58FT shows irregular segmental thickening of jejunal
wall and the effect of submucosal (5M) accumulation of folds (arrow) due to hemorrhage in a patient with
fluid or cells to broaden the valvulae (V). Henoch-5ch6nlein purpura. Compare with normal folds
4 (open arrow).

2
o Distended SB has a wall thickness of only 1 or 2 mm
I TERMINOLOGY o Luminal diameter
Abbreviations and Synonyms • At rest, usually:::; 2.5 em
• Small bowel (SB)or intestine • Distended after meal or upper GI series/SBFT,3.5
• Serosa (S) em for jejunum, 3 em for ileum
• Longitudinal muscle (LM) • Distended during enteroclysis, 4.5 em upper
• Circular muscle (CM) jejunum, 4 em mid jejunum, 3 em ileum
• Lamina propria (LP) • Ileum
• Muscularis mucosa (MM) o Distal 40% of SB
o SBfold are fewer and flatter, easily effaced by
Definitions distension, as in enteroclysis
• Small bowel follow through (SBFT) o Prominent submucosal lymphoid follicles, especially
o Examination of the SBfollowing barium ingestion in terminal ileum in younger individuals
• Enteroclysis
o Examination of SBfollowing placement of a tube
through the nose into the distal duodenum followed I ANATOMY-BASED IMAGING ISSUES I
by sustained infusion of barium suspension to
distend bowel Key Concepts or Questions
• What are the advantages and disadvantages of the
conventional barium meal, SBFTand enteroclysis?
I IMAGING ANATOMY o SBFTadvantages
• Patient preference
• Mesenteric SBbegins at the duodenojejunal junction • Ease of performance
and ends at ileocecal valve • Ability to judge transit time
• Usually 20 to 22 feet long o SBFTdisadvantages
• Suspended from posterior abdominal wall by • Length of exam (can be hours if slow gastric
fan-shaped mesentery, a double-layered fold of emptying or SBtransit)
peritoneum that carries the blood vessels, lymphatics, • Dilution of barium by gastric contents
and nerve supply to the SB • Lack of complete distention of SB
• Jejunum o Advantages of enteroclysis
o Proximal 60% of the SB • Shorter exam time
o Characterized by prominent crescentic folds of • Better distention of SB
mucosa and submucosa, the valvulae conniventes • Greater positive and negative predictive value for
(also called the plicae circulares, folds of Kerckring, wide range of SBpathology, including strictures,
or simply, small bowel folds) adhesions and intrinsic SB disease (e.g., sprue)
o Usually number 4 to 7 folds per inch in jejunum o Disadvantages of enteroclysis
and 3 to 5 per inch in ileum • More radiologist time (intubation, etc.)
o Prominence of folds and wall thickness depends on • Patient discomfort
age (more in younger) and degree of bowel • What are the indications for fluoroscopic/barium
distention, and varies in numerous systemic and SB examinations versus computed tomography (CT)?
disease states
 SMALL INTESTINE ANATOMY AND IMAGING ISSUES
DIFFERENTIAL DIAGNOSIS
Small intestine benign tumors
• Stromal tumor ("GIST",leiomyoma, etc.)
• Lipoma
• Adenoma
• Hemangioma
• Neural tumor
• Hamartomas (polyposis syndromes)
Small intestine malignant tumors
• Stromal tumor ("GIST")
• Adenocarcinoma (duodenum> distal)
• Carcinoid (ileum> proximal)
• Lymphoma
• Metastases
• Kaposi sarcoma
o Enteroclysis is more accurate than CT in detecting
SBulceration, small luminal or mural masses, and
subtle strictures or adhesions
o CT is preferable in the setting of "acute abdomen"
(abdominal pain +/or distention) and more
accurately depicts extraluminal disease (e.g., abscess,
fistulas, large masses, mesenteric and vascular
disease)
• What are the advantages/disadvantages of capsule
endoscopy versus barium studies of the SB?
o Capsule endoscopy utilizes a tablet-size camera that
is swallowed by the patient, recording images of the
luminal GI tract at intervals until it is expelled by
the patient
o Advantages
• Allows endoluminal photographic view of the SB
beyond the reach of fiberoptic endoscopes
o Disadvantages
• Requires many hours for patient preparation time,
5B transit and subsequent analysis of the images
• Often difficult to localize a pathologic process that
is visualized
Imaging Approaches
• Patient preparation for 5BFTor enteroclysis
o Preferable to clear right side of coloni to reduce 5B
transit time and filling defects in distal 5B
o Oral laxative (e.g., magnesium citrate) at noon on
day prior to the exam
o Plus 4 bisacodyl tablets (Dulcolax, Boehringer
Ingelheim) the day prior to the exam
o Low residue diet evening before; nothing by mouth
after midnight
o Metoclopramide (Reglan, AH Robbins) optional
• 10 mg LV. or 20 mg PO prior to intubation for
enteroclysis (facilitates transpyloric passage of
tube and 5B peristalsis
• Upper GI and 5BFT
o Up to 500 ml of 42% weight/volume barium
mixture is ingested
o Fluoroscopic ± overhead radiographs at 15 to 30
minute intervals
o Manually palpate each segment of 5B
Polyposis syndromes affecting S8
• Peutz-]eghers (hereditary; stomach, SB, colon;
hamartomas; mucocutaneous pigmentation)
• Cowden (hereditary; colon + SB; facial papillomas)
• Cronkhite-Canada (non-hereditary, inflammatory;
ectodermal lesions)
• Gardner/familial polyposis (hereditary; adenomas;
colon> SB)
Multiple target, "bull's eye" lesions
• Metastases (especially melanoma)
• Lymphoma
• Kaposi sarcoma
o Continue until ileocecal valve is reached 4
o Maintain a continuous column of barium in 5B by
3
having patient continue to drink
• Double contrast 5BFT
o When the barium has reached the colon, the patient
may be given effervescent gas producing agent by
mouth and positioned in a Trendelenburg, left side
down position to distend the 5B with gas: Additional
fluoroscopy and films are obtained
• Peroral pneumocolon
o After 5BFT,a rectal tube is inserted and air is
insufflated to distend the barium-filled distal 5B
loops and right colon
o Helpful to administer 1 mg glucagon LV. prior to
insufflation to facilitate reflux through ileocecal
valve and produce 5B hypotonia
o Good technique for suspected inflammatory or
infectious process in ileocecal region (e.g., Crohn
disease)
• Enteroclysis
o 5ingle contrast: Infusion of 30 to 40%
weight/volume barium suspension at rate of 60 to
90 ml/min, suffices for most indications
o "Double contrast" enteroclysis: Infusion of 60 to
95% weight/volume barium followed by infusion of
air or methylcellulose to distend lumen, leaving
only a thin coating of viscous barium
o Double contrast technique theoretically superior for
detection of subtle lesions, but prone to technical
difficulties and patient discomfort
o Reflux small bowel examination: Essentially a single
contrast barium enema with addition of glucagon (1
mg LV.) to facilitate ileocecal reflux
• Can be fast and effective means to evaluate
possible distal 5B obstruction or inflammation
Imaging Pitfalls
• The length and redundancy of the 5B and its normal
variability in fold patterns and bowel position make
radiographic visualization and characterization of 5B
pathology very challenging
 SMALL INTESTINE ANATOMY AND IMAGING ISSUES

Axial CECT shows near-water density submucosal Axial CECT shows high density submucosal thickening
edema and intense mucosal enhancement in a patient (plus luminal narrowing and mesenteric hemorrhage) in
with "shock bowel" following abdominal trauma. a patient with jejunal hematoma due to blunt trauma.
4
4
I CUSTOM DIFFERENTIAL DIAGNOSISI Non-neoplastic obstructing lesion
SB folds evenly thickened • Congenital
o Atresia: Stenosis
• Extensive o Malrotation
o Edema (e.g., portal hypertension) o Bands
o Infection (e.g., Giardiasis)
o Hernias (internal and external)
o Eosinophilic gastroenteritis
• Inflammatory and acquired
• Focal o Adhesions
o Hemorrhage (e.g., trauma, anticoagulation) o Strictures
o Ischemia
o Hernias (internal and external)
o Vasculitis
o Intussusception (may have neoplastic cause)
o Radiation enteritis
o Inflammatory fibroid polyp
Micronodular SB fold pattern • Intraluminal
• Whipple disease o Gallstone
• Mycobacterial disease o Meconium plug (or equivalent in adult cystic
• Abetalipoproteinemia fibrosis)
• Histoplasmosis o Bezoar
• Lymphangiectasia o Foreign body ingested
• Macroglobulinemia o Worms, parasites
o Inverted Meckel diverticulum
Irregular diffuse SB fold thickening • Trauma/vascular
• Lymphangiectasia o Hematoma
• Lymphatic obstruction o Vasculitis
• Amyloidosis o Ischemia
• Mastocytosis
• Lymphoma
• Celiac-sprue I SELECTED REFERENCES
Terminal Ileum (TI) ..;fold thickening, luminal 1. Rubesin SE et al: Textbook of gastrointestinal radiology:
Small bowel: differential diagnosis. 2nd ed. Philadelphia,
narrowing WB Saunders. pp 884-890, 2000
• Crohn disease 2. Eisenberg RL: Gastrointestinal radiology: a pattern
• Acute bacterial (Yersinia, Campylobacter) approach. 3rd ed. Philadelphia, JB Lippincott, 1996
• Tuberculosis 3. Diihnert W: Radiology review manual. 4th ed.
Philadelphia, Lippincott, Williams, and Wilkins. 2000
• Cecal carcinoma (retrograde spread to TI) 4. Herlinger H et al: Clinical radiology of the small intestine.
• Lymphoma New York, Springer Verlag, 1999
"Aneurysmal" SB dilation s. Reeder MM: Reeder and Felson's gamut's in radiology. 3rd
ed. New York, Springer Verlag, 1993
• Lymphoma
• Metastases (especially melanoma)
• Iatrogenic (side-to-side anastomosis)
 SMALL INTESTINE ANATOMY AND IMAGING ISSUES
IMAGE GALLERY

(Left) 58FT shows diffuse

uniform fold thickening in a
patient with Giardiasis.
(Right) 58FT shows luminal
distention and reversal of
fold pattern (atrophied
jejunal, prominent ileal) in a
patient with sprue.

4
Typical 5
(Left) 58FT shows
aneurysmal dilation of 58
and destruction of folds
(arrow), along with
mesenteric mass effect;
lymphoma. (Right) Axial
CECTshows aneurysmal
dilation of the 58 lumen and
surrounding mass (arrow);
lymphoma.

(Left) Axial CECT shows

small bowel distention,
terminating at an
intussusception (arrow);
metastatic melanoma.
(Right) Axial GCT shows a
soft tissue mass (arrow) as
the lead point of an
intussusception; melanoma.
 MALROTATION

5BFT shows all of small intestine on right side of
abdomen, colon on left, indicating nonrotation of gut.
Duodenojejunaljunction low + midline.
4
6
ITERMINOlOGY
Definitions
• Rotational abnormality of the gut secondary to arrest
of gut rotation & fixation in embryologic development
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Small bowel on right abdomen
and large bowel on left abdomen
• Other general features
o Classification of malrotation
• Nonrotation: Midgut returns to peritoneal cavity
without rotation
• Incomplete rotation: Duodenojejunalloop fails to
complete final 90° rotation
• Reversed rotation: Cecocolic loop rotates first,
thus unwinding counterclockwise rotation of
duodenojejunalloop with 90° clockwise rotation
Radiographic Findings
• Radiography
o "Double-bubble" sign: Enlarged stomach and
duodenal bulb, little gas in remainder of small bowel
o Gasless abdomen; distended & thickened bowel wall
• Fluoroscopic-guided barium studies
Barium enema shows nonrotation of gut with entire
colon on left side of abdomen.
o Nonrotation: Small bowel on right, large bowel on
left
o Incomplete rotation: Cecum inferior to pylorus
o Reversed rotation: Duodenum anterior to superior
mesenteric artery (SMA), SMA anterior to transverse
colon
o Duodenum and jejunum on right of spine
o Redundancy of duodenum to right of spine
o Z-shape configuration of duodenum and jejunum
o Duodenojejunal junction (DDJ) low and in midline
or medial to left pedicle; or over right pedicle
o Contrast ends abruptly or in corkscrew pattern
CT Findings
• Superior mesenteric vein (SMV) on left of SMA
• Pancreatic aplasia or hypoplasia of uncinate process
Ultrasonographic Findings
• Superior mesenteric vein (SMV) on left of SMA
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided barium studies
I DIFFERENTIAL DIAGNOSIS
Malposition
• E.g., extrinsic masses, indwelling nasojejunal tubes
• Distorted DDJ without malrotation

DDx: Abnormal Position of Gut

Displacement by Mass Volvulus Volvulus Internal Hernia

 MALROTATION

Key Facts
Imaging Findings • Volvulus
• Best diagnostic clue: Small bowel on right abdomen • Internal hernia
and large bowel on left abdomen Clinical Issues
• Superior mesenteric vein (SMV) on left of SMA • Acute abdomen, abdominal distension
• Best imaging tool: Fluoroscopic-guided barium
studies Diagnostic Checklist
• Any patient with small bowel obstruction
Top Differential Diagnoses
• Inversion of SMV and SMA
• Malposition

Volvulus Natural History & Prognosis

• Whirl-like pattern: Small bowel loops and adjacent • Complications: Obstruction, volvulus, Ladd bands
mesenteric fat converging to point of torsion • Prognosis: Good, if treated promptly
• "Whirl" sign: SMV winds around SMA
Treatment
Internal hernia • Ladd procedure: Reduction of volvulus, division of
• E.g., paraduodenal hernia mesenteric bands, placement of small bowel on right
and large bowel on left and appendectomy
4
7
I PATHOLOGY
General Features
I DIAGNOSTIC CHECKLIST
• General path comments Consider
o Embryology • Any patient with small bowel obstruction
• 6-10th gestational week: Duodenojejunalloop
0
rotates 180 counterclockwise, ending inferior to Image Interpretation Pearls
SMA; cecocolic loop rotates 90 counterclockwise,
0 • Inversion of SMV and SMA
ending at anatomical left of SMA
• 10th gestational week: Midgut returns to
peritoneal cavity; duodenojejunalloop rotates I SELECTED REFERENCES
additional 90 counterclockwise ~ positions DDJ
0
1. Boudiaf M et al: Ct evaluation of small bowel obstruction.
to left of spine at level of stomach, jejunum in left Radiographies. 21(3):613-24, 2001
upper quadrant (LUQ) and ileum in right lower 2. Berrocal T et al: Congenital anomalies of the small
quadrant (RLQ); cecocolic loop rotates additional intestine, colon, and rectum. Radiographies. 19(5):1219-36,
0
180 counterclockwise ~ cecum in RLQ 1999
3. Long FR et al: Intestinal malrotation in children: tutorial
• Broad mesenteric base, from LUQ to RLQ
on radiographic diagnosis in difficult cases. Radiology.
• Fixation of gut by attachment at cecal base and 198(3):775-80, 1996
ligament of Treitz at DDJ 4. Long FR et al: Radiographic patterns of intestinal
• Epidemiology: 1 in 500 live births in U.S. malrotation in children. Radiographies. 16(3):547-56;
• Associated abnormalities discussion 556-60, 1996
o Omphalocele, gastroschisis, diaphragmatic hernia
o Asplenia & polysplenia syndrome, duodenal stenosis
or atresia, annular pancreas, Hirschsprung disease I IMAGE GALLERY
o Preduodenal portal vein, inferior vena cava
anomalies

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute abdomen, abdominal distension
o Chronic vomiting, failure to thrive
Demographics
• Age: Up to 40% diagnosed by 1 week of age, 50% by 1
month, 75% by 1 year, 25% after 1 year
(Left) Axial CECT shows malrotation with intestine on right, colon on
• Gender: Overall M = F; < 1 year of age: 2:1 left. (Right) Axial CECT in patient with bowel malrotation shows
agenesis of dorsal pancreas + uncinate process.
 MECKEL DIVERTICULUM

Graphic shows blind-ended outpouching from the Small bowel follow through (S8FT) shows blind-ended
antimesenteric border of the distal ileum. outpouching from distal ileum.

4
8
o 50% of Meckel diverticula, contain ectopic gastric
ITERMINOLOGY mucosa
Abbreviations and Synonyms • Pancreatic, duodenal & colonic mucosa can also
• Meckel diverticulum (MD) be found
o 90% of cases that present with bleeding contain
Definitions gastric mucosa
• An ileal outpouching, due to persistence of the o A fibrous band, representing obliterated part of
congenital omphalomesenteric or vitelline duct vitelline duct may connect apex of diverticulum to
umbilicus
o Rule of 2s
I IMAGING FINDINGS • Seen in 2% of population (approximately)
• Located within 2 feet of ileocecal valve
General Features • Length of 2 inches (on average)
• Best diagnostic clue: Blind-ended sac or outpouching • Symptomatic usually before age 2
on antimesenteric border of ileum o Two main complications in adults: Diverticulitis
• Location (20%); intestinal obstruction (40%)
o Within 50-60 cm of ileocecal valve
• May vary from RLQ to mid abdomen Radiographic Findings
• Size: 4-10 cm in length • Radiography
• Morphology: Tubular outpouching of ileum oX-ray A-P abdomen
• Other general features • Round collection of gas & ± solitary or multiple
o Most common congenital anomaly of GI tract calcified densities (enteroliths) within it in right
o True diverticulum (contains all layers of bowel wall) lower quadrant (RLQ)
o Arises from antimesenteric border of distal ileum & • Fluoroscopic guided enteroclysis
has a separate blood supply o Superior due to maximum luminal distention
o Formed by incomplete obliteration of ileal end of o Blind-ended sac onantimesenteric border of ileum
vitelline duct with either a broad base or a narrow neck
o Usually located within 50-60 cm of ileocecal valve o Broad based diverticulum

DDx: Right lower Quadrant Inflammation

.; --.
,'• .ft-
~. .

Appendicitis Crohn Disease Mesenteric Adenitis

• Cecal Diverticulitis
 MECKEL DIVERTICULUM
Key Facts
Terminology
• An ileal outpouching, due to persistence of the
congenital omphalomesenteric or vitelline duct
Imaging Findings
• True diverticulum (contains all layers of bowel wall)
• Seen in 2% of population (approximately)
• Located within 2 feet of ileocecal valve
• Length of 2 inches (on average)
• Symptomatic usually before age 2
• Two main complications in adults: Diverticulitis
(20%); intestinal obstruction (40%)
• CT for symptomatic (pain, tenderness) adults
• Tc99m scintigraphy in bleeding cases (children)
Top Differential Diagnoses
• Appendicitis
• Enteroclysis shows distinctive triangular
junctional fold pattern at site of origin
o Narrow neck diverticulum
• Diagnosis depends on demonstration of blind end
of diverticulum & its antimesenteric origin
o Initially appears small but fills more completely with
increased distention of lumen
o Inverted Meckel diverticulum
• Seen in 20% of cases
• Solitary, elongated, smoothly marginated, often
club-shaped intraluminal mass parallel to long
axis of distal ileum; may lead to intussusception
• Double contrast barium enema
o Occasionally demonstrates MD by reflux into ileum
• Conventional small bowel follow-through
o Rarely demonstrates diverticulum
CT Findings
• Blind-ending pouch of bowel attenuation within RLQ
• Rarely, calcified enteroliths within diverticular lumen
• Communication with small-bowel present
• Meckel diverticulitis
o Blind-ending pouch + fluid, air or particulate matter
o Inflamed: No oral contrast within diverticular lumen
o Mural thickening: Diverticulum + adjacent bowel
o Mesenteric fat infiltration & fluid ± nodes
o ± Partial or complete small bowel obstruction
o Shows mural enhancement on contrast study
Ultrasonographic Findings
• Real Time
o Hypoechoic mass with ± echogenic calculi in right
lower quadrant (nonspecific)
o Inverted Meckel diverticulum: Target-like mass with
a central area of increased echogenicity
Angiographic Findings
• Conventional
o Supers elective catheterization of distal ileal arteries
o Active bleeding: Blush of contrast medium in RLQ
o Pathognomonic: Vitelline artery (anomalous end
branch of SMA)
• Crohn disease
• Mesenteric adenitis
• Cecal diverticulitis
Pathology
• Composed of all layers of GI tract (true diverticulum)
• Ectopic gastric or pancreatic mucosa may be seen
Clinical Issues
• Children: Present with GI bleeding before age 2
• Adults: Present with diverticulitis or obstruction
Diagnostic Checklist
• Enteroclysis: Blind-ended sac on antimesenteric
border of ileum with a broad base or a narrow neck
• Meckel diverticulitis: CT shows mural thickening,
perimesenteric fat infiltration & fluid ± enteroliths
4
Nuclear Medicine Findings
• Tc99m sulfur colloid (5-10 min serial images for 1 hr) 9
o Most widely used method (diagnosing bleeding MD)
o Sensitivity> 85%; specificity> 95%; accuracy>
85-88%
o Accumulation of isotope in RLQ on positive scans
o Pentagastrin used to stimulate MD gastric mucosal
uptake (initial scintigraphy is equivocal or normal)
o False-positive results: Gastric ectopia, appendicitis,
inflammatory bowel disease
o False-negative results: Absent or minimal ectopic
gastric mucosa
Imaging Recommendations
• Fluoroscopic compression during enteroclysis
• CT for symptomatic (pain, tenderness) adults
• Tc99m scintigraphy in bleeding cases (children)
• Angiography in active bleeding cases
I DIFFERENTIAL DIAGNOSIS
Appendicitis
• Best imaging clue on CT
o Appendicolith (usually calcified) within distended
tubular appendix
• Distended enhancing appendix with surrounding
inflammation (fat stranding)
• Wall thickening of cecum or terminal ileum
• Right lower quadrant (RLQ) lymphadenopathy
• In perforated cases
o Fluid collection most commonly in RLQ or
dependent pelvis (Cul-de-sac)
o Abscess, small-bowel obstruction
• Ultrasound findings
o Echogenic appendicolith with posterior shadowing
o Noncompressible blind-ending tubular structure
over 6 mm in diameter
o Fluid or abscess collection in RLQ
• Clinically & radiographically may mimic MD
• Differentiation often made only at surgery
 MECKEL DIVERTICULUM
Crohn disease
• Synonym(s)
o Terminal ileitis, regional enteritis, ileocolitis
• Chronic, recurrent, segmental, granulomatous
inflammatory bowel disease
• Location: Anywhere along gut from mouth to anus
o Most common site: Terminal ileum
• Characterized by
o Skip lesions: Segmental or discontinuous
o Transmural inflammation, noncaseating granulomas
o Cobblestone mucosa, fissures, fistulas, sinus tracts
• Clinically may be indistinguishable from Meckel
diverticulitis when disease is localized in terminal
ileum with history of RLQ pain & melena
• Diagnosis: Endoscopic mucosal biopsy
Mesenteric adenitis
• Other common cause of RLQ pain
o Predominantly in children & adolescents
• Pathology: Benign inflammation of mesenteric nodes
4 • Imaging findings
o Enlarged, clustered adenopathy in mesentery & RLQ
10 o May also have ileal wall thickening
• Usually resolves spontaneously within 2 days
Cecal diverticulitis
• Perforation + localized pericolic inflammation/abscess
o Most common complication of diverticulosis
• Location: Right lower quadrant (RLQ)
• Imaging findings
o Bowel wall thickening, fat stranding, free fluid & air
o Bowel outpouching filled with air, contrast, feces
o Acute: Enhancement of thickened colonic wall
o Pericecal changes: Abscess, sinus or fistula
o ± Gas or thrombus in mesenteric or portal veins
o ± Liver abscesses
• Clinical presentation
o RLQ pain, fever, tenderness
o More common in middle & elderly age group
• May mimic complicated MD clinically & radiologically
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Mostly asymptomatic
o 4.2% likelihood of symptomatic during lifetime
• Pain in right lower quadrant (RLQ)
o Children: Present with GI bleeding before age 2
o Adults: Present with diverticulitis or obstruction
Demographics
• Age: Any age group
• Gender: M:F = 3:1
Natural History & Prognosis
• Complications: Life time complication rate (4%)
o GI bleeding, diverticulitis, perforation, abscess
o Enteroliths, rarely malignancy
o Obstruction due to intussusception or volvulus
o Extrusion of diverticulum into an inguinal hernia
• Hernia of Littre
o Inverted MD: Intussusception ~ bowel obstruction
o Giant MD: More likely obstruction than bleeding
• Prognosis: Good after surgery
Treatment
• Asymptomatic: No treatment
• Symptomatic: Surgical resection
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other inflammatory pathologies of RLQ
Image Interpretation Pearls
• Enteroclysis: Blind-ended sac on antimesenteric border
of ileum with a broad base or a narrow neck
• Meckel diverticulitis: CT shows mural thickening,
perimesenteric fat infiltration & fluid ± enteroliths

I SELECTED REFERENCES
I PATHOLOGY 1. Bennett GL et al: CT of Meckel's diverticulitis in 11
patients. AJR Am J Roentgenol. 182(3):625-9, 2004
General Features 2. Murakami R et al: Strangulation of small bowel due to
• General path comments Meckel diverticulum: CT findings. Clin Imaging.
o Embryology-anatomy 23(3):181-3, 1999
• Persistent omphalomesenteric or vitelline duct, 3. Mitchell AW et al: Meckel's diverticulum: angiographic
which usually obliterates by 5th embryonic week findings in 16 patients. AJR Am J Roentgenol.
170(5):1329-33, 1998
• Etiology
4. Pantongrag-Brown Let al: Meckel's enteroliths: clinical,
o Early fetal life: Primitive midgut communicates with
radiologic, and pathologic findings. AJR Am J Roentgenol.
yolk sac through vitelline duct & failure of usual 167(6):1447-50, 1996
complete regression leads to anomalies 5. Pantongrag-Brown L et al: Inverted Meckel diverticulum:
o MD (> common); bands, fistula, cyst « common) clinical, radiologic, and pathologic findings. Radiology.
• Epidemiology: Incidence (0.3-3%) 199(3):693-6, 1996
6. Rossi P et al: Meckel's diverticulum: imaging diagnosis. AJR
Gross Pathologic & Surgical Features AmJ Roentgenol. 166(3):567-73, 1996
• Solitary diverticulum located approximately 50-60 em 7. Maglinte DO et al: Meckel diverticulum: radiologic
proximal to ileocecal valve on antimesenteric border demonstration by enteroclysis. AJR Am J Roentgenol.
134(5):925-32, 1980
Microscopic Features
• Composed of all layers of GI tract (true diverticulum)
• Ectopic gastric or pancreatic mucosa may be seen
 MECKEL DIVERTICULUM
I IMAGE GAllERY

(Left) Axial CECT shows

air-contrast level within a
blind-ended "sac" (arrow) in
the right lower quadrant.
(Right) 58FT shows
blind-ended sac (arrow)
arising from distal ileum.

4
Typical 11
(Left) Tc99m sulfur colloid
scan in a young child with
GI bleeding shows
accumulation of isotope in
RLQ (arrows) due to ectopic
gastric mucosa within
Meckel diverticulum. (Right)
Axial CECT shows enteroliths
11 within blind-ended sac
(arrow) in RLQ.

Typical
(Left) Axial CECT in 17 year
old girl with crampy
abdominal pain + RLQ
tenderness shows distal ileal
intussusception (arrow) due
to an inverted Meckel
diverticulum. (Right) Axial
CECT in 17 year old girl
shows distal 58
intussusception with an
inverted Meckel diverticulum
as the lead mass.
 INTESTINAL PARASITIC DISEASE

Small bowel barium study demonstrates ascaris as a Axial CECT demonstrates amebic right lobe liver abscess
longitudinal filling defect in the distal small bowel with peripheral zone of edema (arrows).
(arrows).
4
12
• Fluoroscopy
ITERMINOLOGY o Barium studies
Definitions • Ascariasis: Linear filling defects up to 3S cm in SB;
• Ascariasis: Enteric infection with roundworm ascaris giardiasis: Thickened duodenal & jejunal folds;
lumbricordes amebiasis: Colon ulcerations
• Giardiasis: Enteric protozoal infection with Giardia CT Findings
lamblia; amebiasis: Enteric protozoal infection with • CECT: Ascariasis: SBO; amebiasis: Thickened colonic
entamoeba histolytica wall, rounded right lobe liver abscess with peripheral
zone of edema; giardiasis: Thickened SB +/- excess
fluid, distension
I IMAGING FINDINGS
Ultrasonographic Findings
General Features • Real Time: Amebiasis: Rounded hypoechoic right lobe
• Best diagnostic clue liver abscess with low-level internal echoes, little distal
o Ascariasis: Linear filling defect on small bowel acoustic enhancement
follow through (SBFT)
o Giardiasis: Thickened duodenal and jejunal folds on Imaging Recommendations
SBFT • Best imaging tool: SBFTfor ascariasis and giardiasis;
o Amebiasis: Diffuse ulcerating colitis, right lobe liver barium enema and CECT for amebiasis
abscess on CECT
• Location: Ascariasis: Small bowel (SB), colon, common
bile duct (CBD) or pancreatic duct; giardiasis: I DIFFERENTIAL DIAGNOSIS
Duodenum and jejunum; amebiasis: Colon, liver
Crohn disease
Radiographic Findings • Apthous ulcers; skip areas of SB and colon; sinus tracts
• Radiography: Ascariasis: Soft tissue mass from coiled into mesentery; fibrofatty extramural masses
worms at ileocecal valve, may progress to small bowel
obstruction (SBO)

DDx: Intestinal Disorders Mimicking Parasitic Disease

,.
1
-....~
•••••
.. -, -
 INTESTINAL PARASITIC DISEASE

Key Facts
Imaging Findings Clinical Issues
• Ascariasis: Linear filling defect on small bowel follow • Ethnicity: African patients
through (SBFT)
• Giardiasis: Thickened duodenal and jejunal folds on Diagnostic Checklist
SBFT • Consider Crohn disease, ulcerative colitis
• Amebiasis: Diffuse ulcerating colitis, right lobe liver • Linear filling defects, thickened proximal small bowel
abscess on CECT folds, ileocecal ulcerations
• Best imaging tool: SBFTfor ascariasis and giardiasis;
barium enema and CECT for amebiasis

Ulcerative colitis Demographics

• Superficial ulcers; granular mucosa; long segment • Ethnicity: African patients
strictures; rectal involvement
Natural History & Prognosis
Pseudomembranous colitis • Ascariasis: SBO, appendicitis, pancreatitis
• "Accordion sign" of haustral edema • Giardiasis & amebiasis: Continued diarrhea until
effective treatment 4
I PATHOLOGY Treatment
13
• Options, risks, complications
General Features o Ascariasis: Antihelminthic chemotherapy with
• General path comments mebendazole, albendazole or pyrantel pamoate
o Ascariasis: SBO, pancreatobiliary obstruction o Giardiasis: Nitazoxanide or metronidazole
o Giardiasis: Medium to moderate blunting of SBvilli o Amebiasis: Metronidazole
o Amebiasis: Colitis with flask-shaped ulcers
• Epidemiology
o Ascariasis infests 25% of world population I DIAGNOSTIC CHECKLIST
o Giardiasis is most common protozoal disease in US
• 2-7% of US population; higher in Consider
immunocompromised patients • Consider Crohn disease, ulcerative colitis
o Amebiasis infests 10% of world population Image Interpretation Pearls
Gross Pathologic & Surgical Features • Linear filling defects, thickened proximal small bowel
• Ascariasis: SBO due to mass of worms; appendicitis, folds, ileocecal ulcerations
pancreatitis, cholangitis
• Giardiasis: Often grossly normal small bowel
• Amebiasis: Ulcerating acute colitis; toxic megacolon; I SELECTED REFERENCES
large inflammatory masses (ameboma) mimic colon 1. Ali SA et al: Giardia intestinalis. Curr Opin Infect Dis.
CA 16(5):453-60, 2003
2. McDonald V: Parasites in the gastrointestinal tract. Parasite
Microscopic Features Immuno!. 25(5):231-4, 2003
• Ascariasis: Mucosal destruction at worm attachment 3. long E: Intestinal parasites. Prim Care. 29(4):857-77, 2002
sites; inflammatory changes in appendix, pancreas,
biliary tree
• Giardiasis: Villous blunting, inflammatory cells in I IMAGE GALLERY
lamina propria
• Amebiasis: Colitis with neutrophilic infiltrate and deep
ulcers into submucosa

ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Ascariasis: Abdominal pain, diarrhea; SBO;
appendicitis, pancreatitis; growth retardation
o Giardiasis: Abdominal pain, diarrhea; nausea,
vomiting, distension; weight loss; malabsorption
o Amebiasis: Diarrhea, fever, GI bleeding (Left) Anteroposterior view of 58FT demonstrates thickened folds in
proximal jejunum (arrows) from giardiasis. (Right) Spot film of 58FT
demonstrates ascaris in distal small bowel (arrow).
 OPPORTUNISTIC INTESTINAL INFECTIONS

AP compression spot film from barium enema CMV colitis on axial CECT.Note edematous right colon
demonstrates deep cecal ulcer (arrow) from CMV (arrow) with pericolonic soft tissue stranding (open
colitis. arrow).
4
14
ITERMINOLOGY Radiographic Findings
• Fluoroscopy
Abbreviations and Synonyms o CMV: Barium enema
• Cytomegalovirus (CMV), mycobacterium • Diffuse colitis
avium-intracellulare (MAl), atypical mycobacterial • Early stage resembles ulcerative colitis
infection, cryptosporidiosis, cryptosporidium parvum • Aphthous ulcers
Definitions • Terminal ileum characteristically involved with
• Gastrointestinal infection of immune-compromised thickened folds and/or ulceration
host by virus (CMV), protozoa (cryptosporidium), or • Later stages demonstrate deep ulceration
mycobacterium (MAl) o CMV: Small bowel follow through (SBFT)
• Thickened edematous folds
• Discrete ulcerations, deep ulcers in sinus tracts
o MAl: SBFT
I IMAGING FINDINGS • Diffuse enteritis with thickened folds
General Features • Micronodular mucosal pattern
• Best diagnostic clue o Cryptosporidiosis: SBFT
o CMV: Thickened folds with deep ulcerations on • Secretory enteritis
barium studies of small bowel or colon • Thickened folds, increased fluid in bowel
o MAl: Thickened small bowel folds; mesenteric or CT Findings
peri-portal adenopathy with low attenuation nodes
o Cryptosporidiosis: Secretory enteritis with thickened • CECT
o CMV
small bowel folds and increased fluid in bowel
• Mural thickening of colon, stomach, small bowel,
• Location especially terminal ileum
o CMV: Small bowel or colon, stomach
• Mesenteric stranding due to deep ulcers
o MAl: Small bowel, nodes
o MAl
o Cryptosporidiosis: Small bowel
• Low attenuation mesenteric adenopathy due to
necrosis or caseation

DDx: Gastrointestinal lesions Mimicking Opportunistic Infections

Giardiasis TB Peritonitis Lymphoma Whipple Disease

 OPPORTUNISTIC INTESTINAL INFECTIONS

Key Facts
Terminology
• Cytomegalovirus (CMV), mycobacterium
avium-intracellulare (MAl), atypical mycobacterial
infection, cryptosporidiosis, cryptosporidium parvum
• Gastrointestinal infection of immune-compromised
host by virus (CMV), protozoa (cryptosporidium), or
mycobacterium (MAl)
Imaging Findings
• CMV: Thickened folds with deep ulcerations on
barium studies of small bowel or colon
• MAl: Thickened small bowel folds; mesenteric or
peri-portal adenopathy with low attenuation nodes
• Cryptosporidiosis: Secretory enteritis with thickened
small bowel folds and increased fluid in bowel
• Best imaging tool: SBFT,CECT
• Protocol advice: CECT: 150 mll.V. contrast (2.5
ml/sec) with 5 mm collimation
Top Differential Diagnoses
• Giardiasis
• Tuberculosis
• Gastrointestinal lymphoma
• Whipple disease
Clinical Issues
• Most common signs/symptoms: Abdominal pain;
nausea, vomiting, diarrhea; fever; GI bleeding
• Clinical profile: HIV or immunocompromised patient
• CMV and cryptosporidiosis respond to nitazoxanide
in early stage
• MAl in AIDS patients often difficult to treat

• Thickened small bowel folds • Different patterns on barium studies: Nodular (due to 4
o Cryptosporidiosis submucosal infiltration); infiltrating, polypoid, and
• Thickened folds aneurysmal 15
• Spotty lymphadenopathy without necrosis • CECT: Focal bowel wall thickening without
obstruction; "sausage-shaped" mass
Imaging Recommendations
• Best imaging tool: SBFT,CECT Whipple disease
• Protocol advice: CECT: 150 mll.Y. contrast (2.5 • Multisystem disease caused by Thropheryma whippelii
ml/sec) with 5 mm collimation bacillus
o Results in periodic-acid-Schiff positive
glycoprotein-laden macrophages infiltrating tissues
I DIFFERENTIAL DIAGNOSIS • Small bowel, central nervous system, heart valves,
joint capsules frequently involved
Giardiasis • CECT: SBfold thickening, mesenteric and
• Small bowel infection by protozoal flagellate Giardia retroperitoneal adenopathy
lamblia; water-borne infection resulting in abdominal • Enteroclysis: Submucosal fold thickening with
pain, diarrhea micronodular pattern
• Diagnosis made by stool culture, duodenal mucosal
biopsy
• Small bowel barium studies: Thickened folds, I PATHOLOGY
increased fluid in duodenum & jejunum, and sparing
of ileum General Features
• General path comments
Tuberculosis o CMV
• May be 2° to pulmonary source or ingestion of bovine • DNA herpes virus
bacillus • Causes vasculitis leading to gastrointestinal
• Most common symptoms: Chronic abdominal pain, ulceration, ischemia, bleeding, perforation
weight loss, fever, diarrhea, palpable masses o MAl
• Ileocecal involvement mimics Crohn disease • Atypical mycobacterium
• Peritonitis results in ascites, nodular thickening of • Macrophages infiltrate lamina propria of small
omentum and peritoneum bowel distending villi
• Low attenuation mesenteric and peri-portal nodes o Cryptosporidiosis
• Marked narrowing of ileocecal area = Stierlin sign; • Protozoan organisms attach between microvilli of
hypertrophy of ileocecal valve = Fleishner sign small intestine
• Skip areas of colonic involvement with ulceration of • Leads to mucosal damage and secretory enteritis
structures, inflammatory polyps • Epidemiology: HIV or other immunocompromised
• Diagnosis with endoscopic biopsy patients
Gastrointestinal lymphoma Microscopic Features
• Most commonly non-Hodgkin lymphomas that are
• CMV
high grade large cell or immunoblastic cell types; o Cytoplasmic inclusion bodies in enterocytes,
Burkitt more common in pediatric patients macrophages, fibroblasts, and endothelial cells
• Most common symptoms: Abdominal pain, weight
• MAl
loss, fever, anemia
 OPPORTUNISTIC INTESTINAL INFECTIONS
o Coarsely granular mucosa with human immunodeficiency virus infection. Rev
o Sheets of foamy macrophages infiltrate lamina Gastroenterol Disord. 2(4):176-88, 2002
propria 7. Brantsaeter AB et al: CMV disease in AIDS patients:
o Positive acid-fast or Fite stain for organisms incidence of CMV disease and relation to survival in a
• Cryptosporidiosis population-based study from Oslo. Scand J Infect Dis.
34(1):50-5, 2002
o Organisms proliferate from apex of enterocyte 8. von Reyn CF et al: Sources of disseminated Mycobacterium
o Villous atrophy avium infection in AIDS.J Infect. 44(3):166-70, 2002
o Crypt hyperplasia 9. Pollok RC: Viruses causing diarrhoea in AIDS. Novartis
o Inflammatory infiltrate Found Symp. 238:276-83; discussion 283-8,2001
10. Huh JJ et al: Mycobacterium avium complex peritonitis in
an AIDS patient. Scand J Infect Dis. 33(12):936-8, 2001
I CLINICAL ISSUES 11. Clemente CM et al: Gastric cryptosporidiosis as a clue for
the diagnosis of the acquired immunodeficiency
Presentation syndrome. Arq Gastroenterol. 37(3):180-2, 2000
12. Chamberlain RS et al: Ileal perforation caused by
• Most common signs/symptoms: Abdominal pain; cytomegalovirus infection in a critically ill adult. J Clin
nausea, vomiting, diarrhea; fever; GI bleeding Gastroenterol. 30(4):432-5, 2000
• Clinical profile: HIV or immunocompromised patient 13. Wallace MR et al: Gastrointestinal manifestations of HIV
infection. Curr Gastroenterol Rep. 2(4):283-93, 2000
Demographics 14. Sharpstone D et al: Small intestinal transit, absorption, and
• Age: Any age permeability in patients with AIDS with and without
• Gender: M = F diarrhoea. Gut. 45(1):70-6, 1999
4 Natural History & Prognosis
15. Drew WL et al: Cytomegalovirus: disease syndromes and
treatment. Curr Clin Top Infect Dis. 19:16-29, 1999
16 • CMV and cryptosporidiosis respond to nitazoxanide in 16. Sprinz E et al: AIDS-related cryptosporidial diarrhoea: an
early stage open study with roxithromycin. J Antimicrob Chemother.
41 Suppl B:85-91, 1998
• MAl in AIDS patients often difficult to treat
17. Manabe YC et al: Cryptosporidiosis in patients with AIDS:
Treatment correlates of disease and survival. Clin Infect Dis.
27(3):536-42, 1998
• Options, risks, complications
18. Rossi P et al: Gastric involvement in AIDS associated
o CMV: Antiviral therapy with acyclovir or cryptosporidiosis. Gut. 43(4):476-7, 1998
gancyclovir 19. Lumadue JA et al: A clinicopathologic analysis of
o MAl: Antituberculous chemotherapy AIDS-related cryptosporidiosis. AIDS. 12(18):2459-66, 1998
o Cryptosporidiosis: Chemotherapy with nitazoxanide 20. Sesin GP et al: New trends in the drug therapy of localized
and disseminated Mycobacterium avium complex
infection. Am J Health Syst Pharm. 53(21):2585-90, 1996
I DIAGNOSTIC CHECKLIST 21. Julander I: Clinical manifestations and treatment of
mycobacterium avium-intracellulare complex infection in
Consider HIV-infected patients. Scand J Infect Dis Suppl. 98:19-20,
1995
• Tuberculosis, giardiasis or lymphoma 22. Benson CA: Disease due to the Mycobacterium avium
Image Interpretation Pearls complex in patients with AIDS: epidemiology and clinical
syndrome. Clin Infect Dis. 18 Suppl 3:S218-22, 1994
• CMV: Deep ulcerations & focal enteritis or colitis 23. Torriani FJ et al: Autopsy findings in AIDSpatients with
• MAl: Enteritis & low attenuation nodes Mycobacterium avium complex bacteremia. J Infect Dis.
• Cryptorsporidiosis: Thickened bowel wall and 170(6):1601-5, 1994
edematous folds 24. Hellyer TJ et al: Gastro-intestinal involvement in
Mycobacterium avium-intracellulare infection of patients
with HIV.J Infect. 26(1):55-66, 1993
I SELECTED REFERENCES 25. Cappell MS et al: Gastrointestinal obstruction due to
Mycobacterium avium intracellulare associated with the
1. Ishida T et al: The management of gastrointestinal acquired immunodeficiency syndrome. Am J
infections caused by cytomegalovirus. J Gastroenterol. Gastroenterol. 87(12):1823-7, 1992
38(7):712-3, 2003
2. Streetz KLet al: Acute CMV-colitis in a patient with a
history of ulcerative colitis. Scand J Gastroenterol.
38(1):119-22, 2003
3. Hunter PR et al: Epidemiology and clinical features of
Cryptosporidium infection in immunocompromised
patients. Clin Microbiol Rev. 15(1):145-54,2002
4. Masur H et al: Guidelines for preventing opportunistic
infections among HIV-infected persons--2002.
Recommendations of the U.S. Public Health Service and
the Infectious Diseases Society of America. Ann Intern
Med. 137(5 Pt 2):435-78, 2002
5. Ukarapol N et al: Cytomegalovirus-associated
manifestations involving the digestive tract in children
with human immunodeficiency virus infection. J Pediatr
Gastroenterol Nutr. 35(5):669-73, 2002
6. Oldfield EC 3rd: Evaluation of chronic diarrhea in patients
 OPPORTUNISTIC INTESTINAL INFECTIONS

I IMAGE GALLERY

(Left) Gastrointestinal CMV.

Lateral view of stomach from
upper GI series shows
marked antral narrowing and
thickened folds (arrow) from
antral CMV gastritis. (Right)
Gastrointestinal CMV. Small
bowel follow through
demonstrates enteritis with
thickened folds (arrows).

4
Typical 17
(Left) MAlon CECT
demonstrates mural
thickening of cecum and
terminal ileum (arrow).
(Right) Axial CECT of MAl.
Note low attenuation
mesenteric lymph node
(arrow).

(Left) Early cryptosporidiosis

of the small bowel. Small
bowel follow through
demonstrates mild fold
thickening (arrows). (Right)
Advanced cryptosporidiosis
of the small bowel. Note
marked mural narrowing and
extensive fold thickening
(arrows) on small bowel
follow through.
 SPRUE-CELIAC DISEASE

Small bowel follow through (58FT) shows decreased 58FT shows dilated lumen + dilution of barium within
size + number of jejunal folds and increased number the small intestine and a reversal of the normal jejunal +
and size of ileal folds (reversal pattern). ileal fold patterns.
4
18
o Has a familial susceptibility with genetic basis
ITERMINOLOGY o Evidence suggesting existence of immune reaction
Abbreviations and Synonyms in bowel mucosa
• Nontropical sprue; celiac sprue or disease; gluten o Classic presentation: Steatorrhea & weight loss or
sensitive enteropathy failure to thrive
o Variants of celiac sprue
Definitions • Refractory; collagenous; unmasked celiac sprue
• Nontropical sprue (Celiac disease): Malabsorption due
to intolerance to gluten protein
Radiographic Findings
• Tropical sprue: Malabsorption seen in inhabitants of • Fluoroscopic guided small bowel follow through
tropical countries o Dilatation of small bowel (jejunum): > 3 em
o Valvulae conniventes: May exhibit 5 patterns
• Normal: In most patients valvulae look normal
I IMAGING FINDINGS • Squared ends: Ends at margin are squared off
rather than rounded
General Features • Reversed jejunoileal fold pattern: Decreased
• Best diagnostic clue: j # of jejunal folds « 3 inch) & I jejunal folds & increased ileal folds
# of ileal folds (4-6 inch) on enteroclysis • Absence of valvulae: "Moulage sign" (cast)
• Location characteristic of sprue
o Celiac disease: More proximal small bowel • Thickening: In severe disease & hypoproteinemia
o Tropical sprue: Entire small bowel o "Colonization of jejunum": Loss of jejunal folds ~
• Morphology: Celiac disease-dilated small bowel, colon-like haustrations
thickened wall + valvulae, reversal of jejunoileal folds o Hypersecretion-related artifacts: Due to excess fluid
• Other general features • Flocculation: Coarse granular appearance of small
o Most common small bowel disease producing clumps of disintegrated barium due to excess
malabsorption syndrome fluid; mainly with steatorrhea
o Due to sensitivity of small bowel to (X-gliadin, a • Segmentation: Breakup of normal continual
component of gluten column of barium creating large masses of barium
separated by string-like strands

DDx: Fold Thickening, Increased Fluid

Whipple Disease Crohn Disease Ischemia Waldenstrom Macro.

 SPRUE-CELIAC DISEASE
Key Facts
Terminology
• Nontropical sprue; celiac sprue or disease; gluten
sensitive enteropathy
• Nontropical sprue (Celiac cjisease): Malabsorption due
to intolerance to gluten protein
Imaging Findings
• Best diagnostic clue: I # of jejunal folds « 3 inch) & 1
# of ileal folds (4-6 inch) on enteroclysis
• Absence of valvulae: "Moulage sign" (cast)
characteristic of sprue
• "Colonization of jejunum": Loss of jejunal folds ~
colon-like haustrations
• Mosaic pattern: Due to total villous atrophy
• "Bubbly" duodenum: Nodular pattern in mucosa
• ± Lymphadenopathy
o Transit time: Long, short or normal time
o Nonpropulsive peristalsis (flaccid & poorly
contracting bowel loops)
o Normal or thickened or effaced mucosal folds: Based
on degree of hypoproteinemia
o Painless, transient intussusceptions often seen on
fluoroscopic studies
• Fluoroscopic guided enteroclysis
o Facilitates diagnosis or exclusion of celiac sprue in
higher percentage of cases
o Jejunal folds
• Decreased number of proximal jejunal folds « 3
inch); normal (S or> foldS/inch)
• Increased separation & absence of folds
• Ileal appearance of jejunum
o Ileal folds
• Increased number of folds in distal ileum (4-6
inch); normal (2-4 foldS/inch)
• Increased fold thickness 2:: 1 mm: "Jejunization" of
ileum seen in 78% cases (ileum' ability to gain
functions normally performed by jejunum)
o Jejunoileal fold pattern reversal: Sensitivity in
diagnosing disease increased to 83%
o Mosaic pattern: Due to total villous atrophy
• 1-2 mm islands of mucosa surrounded by
barium-filled grooves
o Duodenal changes
• Decreased number & irregular folds, especially in
distal duodenum
• "Bubbly" duodenum: Nodular pattern in mucosa
CT Findings
• Dilated, fluid-filled small bowel loops
• ± Ileal mucosal thickening
• ± Small bowel intussusception (classic "target" lesion)
• ± Lymphadenopathy
Ultrasonographic Findings
• Real Time
o Increased echo free intraluminal fluid; flaccid &
mildly dilated bowel loops
o Moderately thickened small-bowel wall & valvulae
conniventes
Top Differential Diagnoses
• Whipple disease
• Crohn disease
• Opportunistic infection
• Ischemia
• Cystic fibrosis
• Immunologic disorders
Clinical Issues
• 1 Risk of T-cell lymphoma & carcinoma of jejunum
Diagnostic Checklist
• Check for history of gluten diet & differentiate from
other simulating pathologies
• Reversal of jejunoileal fold pattern; jejunal
colonization; 1 separation or absence of jejunal folds
o ± Increase in caliber of superior mesenteric artery 4
o ± Enlarged mesenteric lymph nodes; ± ascites
19
Imaging Recommendations
• Enteroclysis; small bowel follow through; CT
I DIFFERENTIAL DIAGNOSIS
Whipple disease
• Thickened proximal small-bowel folds mimic sprue
• Micronodules in jejunum on enteroclysis
• Thickening of mesentery & lymphadenopathy
• Periodic acid-Schiff (PAS) stain positive
• Electron microscopy: Trophermyma whippleii
Crohn disease
• Predominantly involves distal ileum & colon
• Classic sign on barium exam: "String sign"
• Skip lesions/fistulae/fissures/transmural inflammation
• Diagnosis: Biopsy & histology
Opportunistic infection
• AIDS (e.g., cryptosporidiosis; tuberculosis; CMV)
• Cryptosporidiosis
o Most common cause of enteritis in AIDS patients
o Pathology: Mucosal damage & secretory diarrhea
o Thickening of folds & bowel wall; 1 fluid in lumen
o Diagnosis: Oocysts in stool & mucosal biopsy
• Mycobacterial tuberculosis
o Example: (Atypical) Mycobacterium
avium-intracellulare
o Small bowel shows thickened folds, fine nodularity
oCT: Shows low density (caseated) lymph nodes
o Diagnosis: Mucosal biopsy
• Cytomegalovirus (CMV)
o Causes terminal ileitis in AIDS patients
o Narrow lumen, thickened folds, spiculation, ulcers
o Diagnosis: Round intranuclear inclusion bodies
Ischemia
• Due to vascular insufficiency
o Superior mesenteric artery (SMA)clot or narrowing
• Segmental thickening of bowel wall> 3 mm
 SPRUE-CELIAC DISEASE
• ± Gas in portal vein, bowel wall
ICLINICAllSSUES
Cystic fibrosis
• Duodenum (2nd part): Thickened or flattened folds, Presentation
nodular filling defects, sacculation of lateral border • Most common signs/symptoms
• Thickened proximal jejunal folds & reticular mucosal o Malabsorption, steatorrhea, abdominal distension
pattern may simulate celiac disease o Diarrhea, weight loss, glossitis, anemia
• Clinical profile: Young patient with history of gluten
Immunologic disorders diet, steatorrhea, abdominal distension & diarrhea
• Walden strom macroglobulinemia • Sensitive predictor in asymptomatic: 1 Levels of
o Cancer of B lymphocytes antireticulin antibody (ARA)
o Overproduction of IgM • Lab-data
• IgA deficiency o Specific screening tests: IgG antigliadin & IgA
o Often accompanied by opportunistic infections (e.g., antiendomysial antibodies
Giardia) o Positive Sudan stain for fecal fat & i D-xylose
o Both can cause nodular SB folds + malabsorption absorption
pattern o i Iron, folate, Ca++, K+, albumin, cholesterol levels
• Diagnosis
o Duodenojejunal mucosal biopsy
!PATHOlOGY o Clinical & imaging response to gluten free diet

4 General Features Demographics

• General path comments • Age
20 o Nontropical sprue (Celiac disease) o Childhood by age of 2 years
• Affects proximal small bowel o 2nd peak in 3rd & 4th decades
• Atrophy of jejunal villi, crypt hyperplasia, • Gender: M = F
lengthening & flattening of mucosa, chronic
Natural History & Prognosis
inflammation of lamina propria
• Natural history
• 1 Production of IgA & IgM antigliadin antibodies
o Adult disease: Extension childhood form; new onset
• Increased number of intraepitheliallymphocytes
& activation of T-cells in lamina propria • Complications
o Ulcerative jejunoileitis
o Tropical sprue
o 1 Risk of T-cell lymphoma & carcinoma of jejunum
• Similar to celiac disease; affects entire small bowel
• Prognosis
• Genetics: Celiac disease- class II human leukocyte
o Celiac disease: Improvement within 48 hours; full
antigens (HLA) - HLA-DR3 & HLA-DQw2
remission (weeks to months)
• Etiology
o Tropical sprue: Improvement in 4-7 days; complete
o Celiac disease: Allergic, immunologic, or toxic
recovery (6-8 weeks)
reaction to gliadin component of gluten
o Tropical sprue: Unknown etiology; may be due to Treatment
enterotoxigenic E. coli • Nontropical sprue or Celiac disease
• Epidemiology o Lifelong gluten-free diet
o Celiac disease: Increased incidence in Ireland & • Tropical sprue
Northern Europe; unknown in Africa, China, Japan o Broad spectrum antibiotics (tetracycline) & folates
o Tropical sprue: Increased incidence in tropics,
especially in Vietnam & Puerto Rico
• Associated abnormalities I DIAGNOSTIC CHECKLIST
o Dermatitis herpetiformis; IgA deficiency
o Hyposplenism; benign adenopathy Consider
o Cavitary mesenteric lymph node syndrome (rare) • Check for history of gluten diet & differentiate from
• Hyposplenism, nodal cavitary masses other simulating pathologies
Gross Pathologic & Surgical Features Image Interpretation Pearls
• Dilated small bowel, thickened wall + valvulae, • Reversal of jejunoileal fold pattern; jejunal
reversal of jejunoileal folds colonization; 1 separation or absence of jejunal folds
Microscopic Features
• Villous atrophy, thickened lamina propria, increased I SELECTED REFERENCES
number of crypts + cellular infiltrate
• Immunoperoxidase shows immunocytes with IgA & 1. Bosch HCM et al: Celiac disease: Small-bowel enteroclysis
findings in adult patients treated with a gluten-free diet.
IgM antigliadin antibodies
Radiology. 201: 803-8, 1996
2. Strobl PW et al: CT diagnosis of celiac disease. Journal of
Computer Assisted Tomography. 19 (2): 319-20, 1995
3. Rubesin SEet al: Adult celiac disease and its complications.
RadioGraphies. 9: 1045-66, 1989
 SPRUE-CELIAC DISEASE

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
fluid-distended small bowel,
prominent folds in ileum,
and a short segment
intussusception (arrow).
(Right) Axial CECT shows
shows dilated fluid-distended
bowel and the bottom of an
intussusception (arrow).
Note prominent folds in
ileum.

4
Typical 21
(Left) 58FT shows dilated
bowel, dilution of barium,
reduced number + size of
jejunal folds. (Right) 58FT
shows severe loss of folds in
duodenum + jejunum; the
"moulage" pattern.

Typical
(Left) Axial CECT in patient
with sprue. CT shows
fluid-distended bowel. One
segment of jejunum has focal
thickening (arrow) of the
wall found to be lymphoma.
(Right) Axial CECT of patient
with sprue and jejunal
lymphoma. Gastric wall
thickening (arrows) was also
due to lymphoma.
 WHIPPLE DISEASE

Small bowel follow through (58FT) shows micronodular Axial CECT shows mural thickening of long segment of
pattern of jejunum in a 40 year old man with Whipple jejunum.
disease.
4
22
ITERMINOlOGY CT Findings
• Low density (near fat HU), large, mesenteric &
Abbreviations and Synonyms
retroperitoneal lymphadenopathy
• Synonym: Intestinal lipodystrophy
o Fatty material derived from digested Whipple bacilli
Definitions • Thickened proximal small bowel folds
• Rare, chronic, bacterial infectious & systemic disease o Submucosal edema due to hypoalbuminemia
leading to chronic diarrhea & malabsorption • ± Ascites, splenomegaly, pneumatosis intestinalis

Imaging Recommendations
• Enteroclysis & CECT
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Thickened proximal small bowel
I DIFFERENTIAL DIAGNOSIS
folds with micronodularity (1-2 mm) & low density Opportunistic infection in AIDS
mesenteric adenopathy (near fat HU)
• Giardiasis
• Location: Small bowel: Jejunum & duodenojejunal o Duodenum & jejunum
junction
• Thickened irregular folds with hypermotility
• Morphology: Small intestinal mucosa laden with • Luminal narrowing & increased secretions
distended macrophages in lamina propria, which o Ileum: Usually appears normal
contain rod-shaped bacilli
• Cryptosporidiosis
Radiographic Findings o Duodenum & proximal jejunum: Thickened folds
• Fluoroscopic guided enteroclysis o Distal small bowel shows areas of flocculation
o Micronodules (1-2 mm) in proximal small bowel o CT may show small lymph nodes
o Thickened folds (especially jejunum) • Mycobacterium avium-intracellulare (MAl) infection
o ± Thickened mesentery & separation of bowel loops o May have low density nodes similar to Whipple
o Small bowel lumen may be normal or mildly dilated o Wet bowel pattern may be similar, but no nodules
o Distal small bowel may be involved in severe cases

DDx: Abnormal Small Bowel Fold Pattern

Giardiasis Cryptosporidiosis Lymphoma Waldenstrom

 WHIPPLE DISEA~E
Key Facts
Terminology
• Synonym: Intestinal lipodystrophy
• Rare, chronic, bacterial infectious & systemic disease
leading to chronic diarrhea & malabsorption
Imaging Findings
• Best diagnostic clue: Thickened proximal small bowel
folds with micronodularity (1-2 mm) & low density
mesenteric adenopathy (near fat HU)
Dysgammaglobulinemia
• E.g., Walden strom macroglobulinemia
• B-cell neoplasm of older adults with 1 serum IgM spike
• 1 Monoclonal IgM protein ~ hyperviscosity syndrome
• Lymphadenopathy, hepatosplenomegaly, anemia
• Barium studies: Micronodular mucosal pattern
mimicking Whipple (massive deposition of IgM
protein in lamina propria ~ lymphatic obstruction)
lymphoma (non-Hodgkin)
• Nodular, polypoid, infiltrating, mesenteric invasive
• Focally infiltrating form of terminal ileum
I PATHOLOGY
General Features
• Genetics: May be associated with HLA-B27
• Etiology: Tropheryma whippelii gram positive bacilli
• Epidemiology: One of the causes of malabsorption in
US and Europe
• Associated abnormalities: Pleuropericarditis,
sacroiliitis, CNS abnormalities
Gross Pathologic & Surgical Features
• Duodenum & proximal jejunum
o Marked thickening of intestinal villi
o Thickened, irregular mucosal folds
Microscopic Features
• Periodic acid-Schiff (PAS)stain positive
o Villi distended with macrophages full of bacilli in
lamina propria & mesenteric lymph nodes
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Diarrhea, fever, steatorrhea, adenopathy
o Usually present with malabsorption syndrome
o Other symptoms & signs
• Arthralgia, increased skin pigmentation, anemia
• Pleuritis, pericarditis or CNS symptoms
• Diagnosis: PASpositive from small bowel biopsy
Demographics
• Age: 40-49 years old (usually middle-aged Caucasian
men)
• Gender: M:F = 10:1
Top Differential Diagnoses
• Opportunistic infection in AIDS
• Dysgammaglobulinemia
• Lymphoma (non-Hodgkin)
Pathology
• Genetics: May be associated with HLA-B27
• Etiology: Tropheryma whippelii gram positive bacilli
• Periodic acid-Schiff (PAS)stain positive
Natural History & Prognosis
• Whipple disease is fatal without therapy
• Relapses may occur, even after long term therapy
• CNS relapse is resistant to antibiotic therapy
Treatment
• Antibiotic therapy for 1 or more years (often curative)
o Parental penicillin + streptomycin initially
4
o Followed by 1 year oral treatment with Bactrim 23
• Trimethoprim/sulfamethoxazole
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of malabsorption syndrome
Image Interpretation Pearls
• Enteroclysis: Diffuse or patchy micronodules (1-2 mm
in diameter), predominantly in proximal small bowel
• CT: Mesenteric & retroperitoneal nodes of near fat HU
I SELECTED REFERENCES
1. Herlinger H et al: Whipple disease malabsorption states. In
Clinical imaging of the small intestine. Eds Herlinger H et
a1. 2nd ed. p 357-9, 1999
2. Horton KM et al: Uncommon inflammatory diseases of the
small bowel: CT findings. AJRAm J Roentgeno1. 170(2):
385-8, 1998
3. Rubesin SE et al: Small bowel malabsorption: clinical and
radiologic perspectives. How we see it. Radiology.
184(2):297-305, 1992
I IMAGE GALLERY
(Left) 58FT shows micronodular fold thickening of most of the 58,
but no excess fluid. (Right) Axial CECT shows very low density nodes
in the retroperitoneum and mesentery.
 MASTOCYTOSIS

Axial CECT shows hepatomegaly, biliary ductal dilation, Axial CECT shows sclerotic lesions in pelvic bones due
thickened bowel wall and fluid distention of lumen. to mastocytosis.

4
24
ITERMINOlOGY
Abbreviations and Synonyms
• Systemic mast cell disease (SMCD)
Definitions
• Rare disorder characterized by mast cell proliferation
in skin, bones, lymph nodes & parenchymal organs
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Multiple peptic ulcers, thickened
bowel, mucosal nodularity & "bullis-eye" lesions
• Location: GIT: Stomach, duodenum & small bowel
• Other general features
o Usually more transient & self-limited in children
compared to adults
o Systemic disease with mast cell proliferation
• Most commonly involves skin
• Urticaria pigmentosa (UP-most common form)
o Gastrointestinal tract is involved in 16% of cases
• Peptic ulcer disease & malabsorption
o SMCD can present as isolated hematologic disease or
accompanied by other hematologic malignancies
• Myelodysplastic syndrome or acute leukemia
Radiographic Findings
• Fluoroscopic guided barium studies
o Stomach
• Multiple ulcers: Round or ovoid collections of
barium & folds radiating to edge of ulcer crater
o Duodenum & small bowel
• Multiple duodenal ulcers; i barium transit time
• Mucosal nodularity (2-3 mm sandlike nodules)
• Bowel wall: Diffuse thickening
• Folds: Thickened, irregular, distorted
• "Bullis-eye" lesions; dilated bowel loops
o Colon: Diffuse inflammatory changes
• Central skeleton: Mixed sclerotic & lytic lesions
CT Findings
• Thickened bowel wall & mucosal folds
• Dilated fluid-filled small bowel loops
• Lymphadenopathy; thickened omentum & mesentery
• Hepatomegaly (seen in 40% of adult cases)
• Splenomegaly with nodular deposits (50% of cases)
• ± Esophageal varices, ascites (due to portal HTN)
• ± Budd-Chiari hepatic veno-occlusive disease
Nuclear Medicine Findings
• Bone scan: Lytic lesions, osteoporosis or osteosclerosis
Imaging Recommendations
• Best imaging tool: Double contrast barium studies

...,-, .~,
DDx: SB Fold Thickening + Fluid Distention

.. I

~
Carcinoid
,,·U/ ,,- V -
"
Zollinger-Ellison
1
Sprue Lymphangiectasia
 MASTOCYTOSIS
Key Facts
Terminology
• Systemic mast cell disease (SMCD)
Imaging Findings
• Best diagnostic clue: Multiple peptic ulcers, thickened
bowel, mucosal nodularity & "bullis-eye" lesions
• Central skeleton: Mixed sclerotic & lytic lesions
• Lymphadenopathy; thickened omentum & mesentery
• Hepatomegaly (seen in 40% of adult cases)
I DIFFERENTIAL DIAGNOSIS
Carcinoid syndrome
• Flushing, diarrhea, etc., indicated hepatic metastases
• Urticaria pigmentosa seen in SMCD, but not carcinoid
• Lab-data: Serotonin levels are increased in carcinoid
Zollinger-Ellison syndrome (ZES)
• Gastrinoma (pancreatic tumor) + peptic ulcers
• Lab-data: Elevated gastrin levels in ZES
Celiac sprue (nontropical)
• Malabsorption due to intolerance to gluten protein
• Reversed jejunoileal folds (I jejunal & t ileal folds)
• Lab-data: Increased IgA & IgM antigliadin antibodies
Malabsorption conditions
• E.g., Whipple disease, lymphangiectasia
I PATHOLOGY
General Features
• Genetics: Mutations in c-kit proto-oncogene
(Codon-8I6 c-kit)
• Etiology
o Hyperplastic response to an unknown stimulus
rather than a neoplastic condition in some cases
o Pathogenesis: Histamine, prostaglandins, proteases
• Epidemiology: Extremely rare disorder
• Associated abnormalities: Urticaria pigmentosa, bony
lesions, hematopoietic disorders
Gross Pathologic & Surgical Features
• Ulcers, thickened wall & folds, mucosal lesions
Microscopic Features
• Cells: Spindle-shaped nucleus, eosinophilic granules
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Malabsorption: Pain, diarrhea, nausea, vomiting
o Skin: Macules, papules, nodules & plaques
o Bone pain, hepatomegaly & splenomegaly
o Lab-data
• CBC: Anemia, leukopenia, thrombocytopenia
• Tryptase > 20 ng/mL; histamine twice of normal
.
• Splenomegaly with nodular deposIts (50% of cases)
Top Differential Diagnoses
• Carcinoid syndrome
• Zollinger-Ellison syndrome (ZES)
• Celiac sprue (nontropical)
Diagnostic Checklist
• Check for skin & bony lesions, hematologic disorders
• Correlate: Clinical, biochemical & imaging findings
Demographics
• Age: Up to childhood (75%); adults 30-49 years (25%)
• Gender: Equal in both males & females
• Ethnicity: Caucasians> African-Americans
Natural History & Prognosis
• Complications
o GE reflux; hemorrhage & perforation; chloroma
4
o Hematologic malignant transformation rate is 30%
25
Treatment
• Epinephrine, steroids, HI + HZ antagonists, proton
pump inhibitors, anticholinergics, chemotherapy
• Symptomatic therapy; no curative therapy exists
I DIAGNOSTIC CHECKLIST
Consider
• Check for skin & bony lesions, hematologic disorders
• Correlate: Clinical, biochemical & imaging findings
I SELECTED REFERENCES
1. Avila NA et al: Systemic mastocytosis: CT and US features
of abdominal manifestations. Radiology. 202(2):367-72,
1997
2. Metcalfe DO: The liver, spleen, and lymph nodes in
mastocytosis. J Invest Dermatol. 96(3):45S-46S, 1991
3. Johnson AC et al: Systemic mastocytosis and
mastocytosis-like syndrome: radiologic features of
gastrointestinal manifestations. South Med]. 81(6):729-33,
750, 1988
I IMAGE GALLERY
(Left) Axial CECT shows thickened bowel wall and intussusception
(arrow) due to mastocytosis. (Right) Axial CECT shows thickened
bowel wall and sclerotic bone lesions.
 CROHN DISEASE

Small bowel follow through (58FT) shows severe 58 58FT shows "string sign", severe luminal narrowing of
strictures, fistulas and ulceration with skip areas. terminal ileum and ascending colon. Also sinus tract
(arrow) + indirect evidence of mesenteric fibrofatty
4 proliferation.

26
o Idiopathic inflammatory bowel disease
ITERMINOlOGY o There is a familial disposition
Abbreviations and Synonyms o Disease with prolonged & unpredictable course
• Terminal ileitis, regional enteritis, ileocolitis o Risk factors
• Caucasian race, Jewish (8-fold 1)
Definitions • Urban, family history & smoking (4-fold 1)
• Chronic, recurrent, segmental, granulomatous o Crohn disease is less common than ulcerative colitis
inflammatory bowel disease
Radiographic Findings
• Fluoroscopic guided barium study
I IMAGING FINDINGS o Early changes
• Lymphoid hyperplasia: 1-3 mm mucosal
General Features elevations; no ring shadow
• Best diagnostic clue: Segmental areas of ileoccolonic • Aphthoid ulcerations: "Target" or "bull's eye"
ulceration + wall thickening on barium study appearance (punctate shallow central barium
• Location collections surrounded by a halo of edema)
o Anywhere along gut from mouth to anus • Cobblestone pattern: Combination of longitudinal
• Most common: Terminal ileum & proximal colon & transverse ulcers
o Distribution • Deep ulcerations (fissuring ulcers)
• Terminal ileum (95%); colon (22-55%) • Mural thickening: Transmural inflammation,
• Rectum (14-50%) fibrosis (Crohn more than ulcerative colitis)
• Morphology o Late changes
o Characterized by • Skip lesions: Segmental/normal intervening areas
• Skip lesions (segmental or discontinuous) • Sacculations: Seen on antimesenteric border (t
• Transmural, granulomas (non caseating type) luminal pressure)
• Cobblestone mucosa, fissures & fistulas • Postinflammatory pseudopolyps, loss of haustra,
• "String sign" on barium enema intramural abscess
• Other general features • "String sign": Luminal narrowing + ileal stricture
• Sinus tracts, fissures, fistulas: Hallmark of disease

DDx: Thickened Wall, Narrowed lumen at Ileocecal Junction

Ulcerative Colitis Yersinia Mesenteric Adenitis

 CROHN DISEASE
Key Facts
Terminology
• Terminal ileitis, regional enteritis, ileocolitis
• Chronic, recurrent, segmental, granulomatous
inflammatory bowel disease
Imaging Findings
• Best diagnostic clue: Segmental areas of ileo-colonic
ulceration + wall thickening on barium study
• Cobblestone pattern: Combination of longitudinal &
transverse ulcers
• Deep ulcerations (fissuring ulcers)
• Mural thickening: Transmural inflammation, fibrosis
(Crohn more than ulcerative colitis)
• Skip lesions: Segmental/normal intervening areas
• "String sign": Luminal narrowing + ileal stricture
• Sinus tracts, fissures, fistulas: Hallmark of disease
• Anorectal lesions: Ulcers, fissures, abscess,
hemorrhoids, stenosis
CT Findings
• Discontinuous & asymmetric bowel wall thickening
(more than 1 cm)
• Acute or noncicatrizing phase: Minimal narrowing
o Mural stratification: Intact
• Distinct mucosa, submucosa, muscularis propria
o Inner ring: Soft tissue density (mucosa)
o Middle ring: Low density (submucosal edema/fat)
o Outer ring: Soft tissue density (muscularis
propria-serosa)
o Proliferation of mesenteric fat ± lymphadenopathy
o "Target" or "double halo" sign on CECT
• Intense enhancement: Inner mucosa + outer
muscularis propria
• I Attenuation: Edematous thickened submucosa
• Chronic or cicatrizing phase: 1 Luminal narrowing +
no "target" sign
o Mural stratification lost (indistinct mucosa,
submucosa, muscularis propria)
o Homogeneous attenuation of thickened bowel wall
on CECT (indicating irreversible transmural fibrosis)
o Abscesses, fistulas, sinus tracts
o Mesenteric changes: Abscess, fibrofatty areas, nodes
o Perianal disease, enlarged mesenteric lymph nodes
o "Comb" sign: Mesenteric hypervascularity
(dilatation, tortuosity & wide spacing)
• Indicates active disease
MR Findings
• Breath-holding (FLASH),fat suppression & Gd-DTPA
o Show extent, mural thickening & severity of disease
o Perianal Crohn disease
• MR sensitive in detecting fistulas/sinuses/abscesses
Ultrasonographic Findings
• Real Time
o Transrectal sonography
• Mural thickening, abscesses, fistulas
• Anal sphincter heterogeneity
Top Differential Diagnoses
• Ulcerative colitis ("backwash" ileitis)
• Infection
• Ischemia
• Radiation enteritis
• Metastases & lymphoma
• Mesenteric adenitis
Pathology
• Exact etiology unknown
• Genetic, environmental, infectious & psychologic
• Immunologic: Antibody & cell-mediated types
Diagnostic Checklist
• Check for associated findings (cholangitis, arthritis)
• CT: Small-bowel wall thickening, mesenteric fat
proliferation & hyperemia very suggestive of Crohn
4
Imaging Recommendations
• Barium enema, enteroclysis 27
• Helical NE + CECT
• MR for perianal & rectal Crohn disease
I DIFFERENTIAL DIAGNOSIS
Ulcerative colitis ("backwash" ileitis)
• 25% of UC cases have terminal ileal pathology
• Widely patent ileocecal valve, slightly thickened folds
• Mucosa may have a nodular or granular pattern
• No strictures/ulcerations; adjacent colonic lesions seen
• Usually lesions of UC: Continuous, non-transmural,
pseudopolyps, 1 risk of colon cancer
Infection
• Yersiniosis: Yersinia enterocolitica (gram negative rod)
o Common location: Terminal ileum
o Thickened mucosal folds, nodules, aphthous ulcers
o Lumen narrowing: Rare; Crohn disease (common)
o Ulceration: Superficial; Crohn disease (deep)
o Resolution: Over 6-8 weeks; Crohn (almost never)
• Particularly seen in AIDS patients
o Example: Mycobacterial, cryptosporidiosis, CMV
• Typical: Mycobacterium tuberculosis
o Ileocecal (> common): Transmural, stenosis, fistulas
o Horizontal ulcers, nodular mucosal thickening
o Cecal contraction with widely patent ileocecal valve
o CT shows pericecal lymphadenopathy
• Atypical: Mycobacterium avium-intracellulare (MAl)
o Small-bowel: Most common site of infection
o Diffusely thickened folds, micronodular mucosa
oCT: Mesenteric adenopathy & abscess
• Cryptosporidiosis
o Most common cause of enteritis in AIDS patients
o Thickening of folds & bowel wall; 1 fluid in lumen
oCT: May show small lymph nodes
o Diagnosis: Oocysts in stool & mucosal biopsy
• Cytomegalovirus (CMV)
o Terminal ileitis indistinguishable from Crohn
o Diagnosis: Round intranuclear inclusion bodies
 CROHN DISEASE
Ischemia o Arthritis, gallstones, sclerosing cholangitis, uveitis
o Ankylosing spondylitis
• Due to vascular insufficiency
• Superior mesenteric artery (SMA) clot or narrowing Gross Pathologic & Surgical Features
o Pneumatosis: "Bubble" or "band-like" air in affected • Skip lesions more common in distal ileum
small-bowel wall + segmental thickening> 3 mm • Edema, inflammation, fibrosis, luminal narrowing
o ± Gas in mesenteric or portal vein • Adhesions, fistulae, fissures, strictures
Radiation enteritis Microscopic Features
• Cause: Therapeutic or excessive abdominal irradiation • Transmural inflammation, lymphoid aggregates,
• Location: Terminal ileum + adjacent colon & rectum non caseating granulomas
• Thickened bowel wall, narrow pelvic bowel loops
• ± Strictures, sinuses, fistulas simulating Crohn disease

Metastases & lymphoma

I CLINICAL ISSUES
• Metastases (small-bowel) Presentation
o E.g., from malignant melanoma, lung/breast cancer • Most common signs/symptoms
o Location: Antimesenteric border (due to rich o Diarrhea, pain, melena, weight loss, fever
vascular submucosal plexus) o Malabsorption; fissures & fistulas (perianal area)
o Malignant melanoma • Diagnosis: Mucosal biopsy
• Smoothly polypoid lesions of different sizes
4 • Polypoid lesion with ulcers & radiating folds form Demographics
• Age: Age: 15-25 years (small peak at 50-80 y)
a typical "spoke-wheel" pattern
28 o Bronchogenic carcinoma • Gender: M = F
• Single/multiple intramural lesions (flat/polypoid) • Ethnicity: More in Caucasians & Jews
• Frequently ulcerated, narrowing & obstruction
Natural History & Prognosis
o Breast carcinoma
• Highly cellular submucosal masses • Complications
• Non-Hodgkin lymphoma: More common o Fistula, sinus, toxic megacolon
o Distribution: Stomach (51%), small-bowel (33%) o Obstruction, perforation, malignancy
o Nodular, polypoid, infiltrating, mesenteric invasive • Prognosis
o Focally infiltrating form of terminal ileum o 10-20% lead symptom free lives
o Recurrence: 30-53% after surgical resection
• Widened segment devoid of folds
• Usually proximal side of anastomosis
• Sausage-shaped thickening of affected bowel wall
• May be indistinguishable from Crohn disease Treatment
Mesenteric adenitis • Medical
o Steroids, azathioprine, mesalamine
• Common cause of RLQ pain in children & adolescents
o Metronidazole, antibody treatment
• Enlarged mesenteric nodes; ileal wall thickening
• Usually resolves spontaneously within 2-4 days • Surgical
o Resection of diseased bowel
o Strictureplasty, primary fistulotomy
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
• General path comments Consider
o Classified into three stages based on pathology
• Check for associated findings (cholangitis, arthritis)
• Early stage: Hyperplasia of lymphoid tissue &
obstructive lymphedema in submucosa ~ shallow Image Interpretation Pearls
mucosal erosions (aphthoid ulcers) • CT: Small-bowel wall thickening, mesenteric fat
• Intermediate stage: Transmural extension in proliferation & hyperemia very suggestive of Crohn
mucosa & submucosa ~ marked fold thickening
• Advanced stage: Transmural extension to serosa &
beyond ~ deep linear clefts of ulceration/fissures I SELECTED REFERENCES
• Genetics
o Common in monozygotic twins & siblings 1. Wold PB et al: Assessment of small bowel Crohn disease:
noninvasive peroral CT enterography compared with other
o Polygenic inheritance pattern imaging methods and endoscopy--feasibility study.
• Etiology Radiology. 229(1):275-81, 2003
o Exact etiology unknown 2. Antes G: Inflammatory disease of the small intestine and
o Possible factors considered colon: Contrast enema and CT. Radiology. 38: 41-5, 1998
• Genetic, environmental, infectious & psychologic 3. Gore RM et al: CT features of ulcerative colitis and Crohn's
• Immunologic: Antibody & cell-mediated types disease. AJR. 167: 3-15, 1996
• Nutritional, hormonal, vascular & traumatic 4. Hizawa K et al: Crohn disease: early recognition and
• Epidemiology: Incidence, 0.6-6.3 cases/lOO,OOO people progress of aphthous lesions. Radiology. 190: 451-4, 1994
• Associated abnormalities
 CROHN DISEASE

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
mural thickening of ileum
with submucosal edema +
mesenteric hypervascularity
("comb sign") indicating
active disease. (Right) Axial
CECT shows pelvic S8
segment with submucosal
edema + "comb sign" of
active inflammation.

4
29
(Left) Air-contrast 8E shows
Crohn (granulomatous)
colitis with multiple
aphthous ulcerations
(arrows) throughout the
colon. (Right) S8FT shows
"cobblestone" appearance
of terminal ileum, due to
longitudinal, transverse
ulcerations (arrows).

Typical
(Left) Axial CECT shows
recurrent Crohn disease
several years following
resection. Mural thickening
and mesenteric fibrofatty
proliferation. (Right) Axial
CECT shows recto-vaginal
fistula (arrow) due to Crohn
disease.
 SCLERODERMA, INTESTINAL

Upper CI (UC/) series shows dilated esophagus with
patulous CE junction and dilated duodenum with
abrupt "cut off" as it crosses the midline.
4
30
ITERMINOLOGY
Abbreviations and Synonyms
• Progressive systemic sclerosis (PSS)
Definitions
• Multisystemic disorder of small vessels & connective
tissue (collagen vascular disease) of unknown etiology
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Dilated atonic small bowel with
crowded folds & wide-mouthed sacculations
• Other general features
o Multisystemic disorder with immunologic &
inflammatory changes
o Characterized by atrophy, fibrosis, sclerosis of skin,
vessels & organs
o Involves skin, synovium, & parenchyma of multiple
organs
• GI tract, lungs, heart, kidneys & nervous system
o Gastrointestinal tract (GIT) scleroderma
• 3rd most common manifestation after skin
changes & Raynaud phenomenon
• Seen in up to 90% of patients
UCI series + small bowel follow through (58FT) shows
dilated duodenum and jejunum with thin, closely
spaced valvulae;the "hidebound" pattern.
• Most common sites: Esophagus> anorectal>
small bowel> colon
• Most frequent cause of chronic intestinal
pseudo-obstruction
o Scleroderma is subclassified into two types
• Diffuse scleroderma
• CREST syndrome (benign course)
o Diffuse scleroderma: Diffuse cutaneous + early
visceral involvement
• Severe interstitial pulmonary fibrosis
• Tends to involve women; organ failure more likely
• Associated with antitopoisomerase 1 antibody
(anti-Scl 70)
o CREST syndrome: Minimal cutaneous & late visceral
involvement
• Calcinosis of skin
• Raynaud phenomenon
• Esophageal dysmotility
• Sclerodactyly (involvement of fingers)
• Telangiectasia
• Associated with anticentromere antibodies
Radiographic Findings
• Fluoroscopic guided (esophagography)
o Normal peristalsis above aortic arch (due to striated
muscle in proximal one-third)
o Hypotonia, atony, aperistalsis: Lower two-thirds
esophagus (smooth muscle)
o Mild-moderate dilatation of esophagus

DDx: Dilated Small Intestine +/- Abnormal Fold Pattern

~ l
SMA Syndrome SMA Syndrome Celiac Sprue Disease
~ .•••.•
Hemorrhage
 SCLERODERMA,
Key Facts
Terminology
• Progressive systemic sclerosis (PSS)
• Multisystemic disorder of small vessels & connective
tissue (collagen vascular disease) of unknown etiology
Imaging Findings
• Best diagnostic clue: Dilated atonic small bowel with
crowded folds & wide-mouthed sacculations
• CRESTsyndrome (benign course)
• Calcinosis of skin
• Raynaud phenomenon
• Esophageal dysmotility
• Sclerodactyly (involvement of fingers)
• Telangiectasia
• Marked dilatation of small bowel (particularly 2nd,
3rd parts of duodenum & jejunum)
o Patulous lower esophageal sphincter (LES):Early
finding
o Erosions, superficial ulcers, fusiform peptic stricture
(reflux esophagitis)
o Gastroesophageal reflux (70%) ~ (37%) develop
Barrett esophagus or peptic stricture
• Distal stricture + dilated non peristaltic esophagus
can simulate achalasia
o Hiatal hernia
• Fluoroscopic guided (barium meal)
o Stomach: Gastric dilatation + delayed emptying
• Fluoroscopic guided (small bowel study)
o Marked dilatation of small bowel (particularly 2nd,
3rd parts of duodenum & jejunum)
o Pathognomonic: "Hidebound" sign
• Dilated small bowel (jejunum) with crowded thin
circular folds ("hidebound" sign)
• Seen in > 60% cases of scleroderma-related
pseudo-obstruction
• Due to muscle atrophy & its uneven replacement
by collagen in longitudinal fibers + intense fibrosis
of submucosa
o Wide-mouthed sacculations (true diverticula) on
antimesenteric border
o Prolonged transit time with barium retention in
duodenum up to 24 hours
o ± Pneumatosis intestinalis + pneumoperitoneum
o ± Transient, nonobstructive intussusceptions
• Fluoroscopic guided (barium enema)
o Sacculations on antimesenteric border (transverse &
descending colon)
o Marked dilatation (may simulate Hirschsprung
disease)
o Chronic phase: Complete loss of haustrations
• Simulates cathartic colon or chronic ulcerative
colitis
o Stercoral ulceration (from retained fecal material)
o ± Benign pneumoperitoneum
Imaging Recommendations
• Fluoroscopic guided single & double contrast barium
studies
o Upper GI & barium enema
INTESTINAL
.
• Dilated small bowel (jejunum) with crowded thm
circular folds ("hidebound" sign)
Top Differential Diagnoses
• Superior mesenteric root syndrome or SMA
• Celiac sprue
• Small bowel ileus
Pathology
• Initially smooth muscle atrophy & fragmentation
• Followed by collagen deposition & fibrosis
• Unknown; autoimmune with genetic predisposition
Diagnostic Checklist
• Rule out other pathologies that cause dilatation of
small bowel ± abnormal fold pattern
• Check for family history of collagen vascular diseases
I DIFFERENTIAL DIAGNOSIS
4
31
Superior mesenteric root syndrome or SMA
• Superior mesenteric artery (SMA)
• Narrowing of angle between SMA & aorta may
compress 3rd part of duodenum causing proximal
dilatation
o Usually seen in asthenic persons who rapidly lose
weight & retroperitoneal fat
o Due to prolonged bed rest or immobilization
• Example: Patients with whole body burns; body
casts; spinal injury or surgery
• Barium study findings
o Marked dilatation of 1st & 2nd parts of duodenum
o Vertical, linear, extrinsic, bandlike defect in
transverse part of duodenum overlying spine
o Occasionally, obstruction is partially relieved in
prone position
• CT may demonstrate beak-like compression of 3rd part
of duodenum between SMA & aorta
• Transient delay of barium in 3rd part of duodenum
seen in normal person may also mimic SMA syndrome
Celiac sprue
• Segmental dilatation of small intestine
• Excess fluid in lumen (flocculation of barium)
• Atrophic duodenal + jejunal folds, relative
hypertrophy of ileal folds
• Transient intussusceptions
Small bowel ileus
• Markedly dilated proximal duodenum (atony) due to
acute upper abdominal inflammatory process
o Example: Acute pancreatitis, cholecystitis, peptic
ulcer
o Small bowel folds in ileus usually normal to thick,
rather than thin + crowded in scleroderma
Intramural hemorrhage
• Bleeding into the submucosa
• Etiology: Trauma, anticoagulation
• Thick valvulae with I space between folds
• "Stack of coins" pattern
 SCLERODERMA, INTESTINAL
I PATHOLOGY Natural History & Prognosis
General Features • Complications
o Barrett esophagus ~ adenocarcinoma
• General path comments
o Bowel pseudoobstruction
o Initially smooth muscle atrophy & fragmentation
• Prognosis
o Followed by collagen deposition & fibrosis
o Limited disease with ANA bodies: Good prognosis
• Genetics
o Diffuse disease: Poor with involvement of kidneys,
o Diffuse: Antitopoisomerase 1 antibodies associated
heart & lungs rather than GI tract
with HLA-DR5
o Localized: Anticentromere antibodies associated Treatment
with HLA-DR 1, 4 & 5 • Small frequent meals; elevation of head of the bed
• Etiology • Avoid tea & coffee
o Unknown; autoimmune with genetic predisposition • Cimetidine, ranitidine, omeprazole
o May be initiated by environmental antigens like • Metoclopramide, laxatives
silica & L-tryptophan • Patients with severe malabsorption
o Immunologic mechanism (delayed hypersensitivity o Parenteral hyperalimentation
reaction)
• i Production of cytokines (TNF-(){or IL-l) ~ i
collagen production I DIAGNOSTIC CHECKLIST
• Vascular damage & activation of fibroblasts
4 • Epidemiology Consider
o Incidence: 14.1 cases/million • Rule out other pathologies that cause dilatation of
32 o Prevalence: 19-75/100,000 persons small bowel ± abnormal fold pattern
• Associated abnormalities: May be associated with • Check for family history of collagen vascular diseases
systemic lupus erythematosus (SLE); polymyositis;
dermatomyositis Image Interpretation Pearls
• Markedly dilated atonic small bowel with thin,
Gross Pathologic & Surgical Features crowded circular folds & delayed barium transit time
• Rubber-hose inflexibility: Lower 2/3 esophagus
• Thin & ulcerated mucosa
• Dilated gas & fluid containing small-bowel loops with I SELECTED REFERENCES
sacculations 1. Neef B et al: Image of the month: "hide-bound" bowel sign
in scleroderma. Gastroenterology. 124(5):1179, 1567,2003
Microscopic Features 2. Mayes MD: Scleroderma epidemiology. Rheum Dis Clin
• Perivascular lymphocytic infiltrates North Am. 29(2):239-54, 2003
• Early capillary & arteriolar injury 3. Goldblatt F et al: Antibody-mediated gastrointestinal
• Atrophy + fragmentation of smooth muscle ~ dysmotility in scleroderma. Gastroenterology.
collagen deposition + fibrosis 123(4):1144-50,2002
4. Coggins CA et al: Wide-mouthed sacculations in the
esophagus: a radiographic finding in scleroderma. AIR Am J
Roentgenol. 176(4):953-4,2001
ICLINICAL ISSUES 5. Weston S et al: Clinical and upper gastrointestinal motility
features in systemic sclerosis and related disorders. Am J
Presentation Gastroenterol. 93(7):1085-9, 1998
• Most common signs/symptoms 6. Lock G et al: Gastrointestinal manifestations of progressive
oEsophagus systemic sclerosis. Am J Gastroenterol. 92(5):763-71, 1997
• Dysphagia, regurgitation 7. Ott DJ: Esophageal motility disorders. Semin Roentgenol.
• Epigastric fullness & burning pain 29(4):321-31, 1994
o Small-bowel 8. Levine MS et al: Update on esophageal radiology. AJR. 155:
• Bloating, abdominal pain 933-41, 1990
9. Rohrmann CAJr et al: Radiologic and histologic
• Weight loss, diarrhea, anemia
differentiation of neuromuscular disorders of the
o Colon gastrointestinal tract: visceral myopathies, visceral
• Chronic constipation & episodes of bowel neuropathies, and progressive systemic sclerosis. AJRAm J
obstruction Roentgenol. 143(5):933-41, 1984
• Lab-data 10. Silver TM et al: Radiological features of mixed connective
o Increased erythrocyte sedimentation rate (ESR) tissue disease and scleroderma-systemic lupus
o Iron, B12 & folic acid deficiency anemias erythematous overlap. Radiology. 120: 269-275, 1976
o Increased antinuclear antibodies (ANA) 11. Berk RN: The radiology corner. Scleroderma of the
gastrointestinal tract. Am J Gastroenterol. 61(3):226-31,
Demographics 1974
12. Horowitz ALet al: The "hide-bound" small bowel of
• Age: 30-50 years
scleroderma: Characteristic mucosal fold pattern. AJR. 119:
• Gender: Young African-American females; M:F == 1:3 332-34, 1973
• Ethnicity: African-Americans more than Caucasians 13. Miercort RD et al: Pneumatosis and pseudo-obstruction in
scleroderma. Radiology. 92(2):359-62, 1969
 SCLERODERMA, INTESTINAL

I IMAGE GALLERY

(Left) SBFT shows typical

dilated duodenum,
hidebound fold pattern of
jejunum. (Right) SBFT shows
dilated + hidebound
appearance of duodenum.

4
33
(Left) SBFT shows dilated,
atonic bowel with
hidebound fold pattern.
(Right) SBFT shows dilated
duodenum, "pseudo SMA
syndrome", + hidebound
jejunal folds.

Typical
(Left) SBFT shows
pseudosacculation +
abnormal folds in the small
bowel. (Right) Barium
enema shows
pseudosacculation of the
transverse colon due to
scleroderma.
 PNEUMATOSIS OF THE INTESTINE

Single contrast BE shows gas "cysts" in wall of colon; Post-evacuation film from single contrast BE shows
primary colonic pneumatosis in asymptomatic patient. extensive gas within the wall of the descending colon
due to primary colonic pneumatosis.
4
34
• Fluoroscopic-guided barium studies
ITERMINOlOGY o Primary
Abbreviations and Synonyms • Radiolucent clusters of cysts along contours of
• Pneumatosis cystoides intestinalis; pneumatosis colon; resemble polyps
intestinalis; intestinal gas cysts • Multiple, large gas-filed cysts with scalloped
defects in bowel wall; resemble inflammatory
Definitions pseudopolyps or thumbprinting seen with
• Cystic or linear collections of gas in subserosal or intramural hemorrhage
submucosal layers of gastrointestinal tract wall • Cysts can concentrically compress the lumen
• Differentiate from other conditions by the striking
lucency of the gas-filled cysts versus the soft tissue
I IMAGING FINDINGS density of an intramural or intraluminal lesion or
the compressibility of the cyst on palpation
General Features • Usually insignificant, asymptomatic, idiopathic
• Best diagnostic clue: Cystic or linear distribution of gas o Secondary
along bowel wall on helical CT • Mottled, bubbly or linear collection of gas in
• Other general features bowel wall (feces-like appearance)
o Classification of pneumatosis intestinalis • Dilated bowel loops ± thumbprinting
• Primary • Usually due to ischemia, medication, or other
• Secondary known etiology
Radiographic Findings CT Findings
• Radiography • CECT
o Primary o Primary
• Location: Colon • "Bubble-like": Isolated bubbles of air or clusters of
• Cystic gas collections along bowel wall cysts in the left colonic wall
o Secondary o Secondary
• Location: Small bowel or colon • "Band-like": Bands or linear distribution of air in
• Linear distribution of gas; dilated bowel loops affected bowel wall

DDx: Pneumatosis of the Small Bowel

Bowel Necrosis Autoimmune Disease Pseudopneumatosis

 PNEUMATOSIS OF THE INTESTINE
Key Facts
Terminology Pathology
• Pneumatosis cystoides intestinalis; pneumatosis • Pneumatosis intestinalis is a sign, not a disease
intestinalis; intestinal gas cysts
Clinical Issues
Imaging Findings • Abdominal pain, distension, melena, fever, vomiting
• Best diagnostic clue: Cystic or linear distribution of and cough
gas along bowel wall on helical CT • Dependent on underlying etiology
• Lung window: Detect intramural & venous gas
Diagnostic Checklist
Top Differential Diagnoses • Bowel necrosis is a surgical emergency
• Bowel necrosis • Prognosis depends on underlying cause, not imaging
• Post-endoscopy findings
• Post-operation • Other causes of pneumatosis intestinalis usually are
• Medication-induced asymptomatic; little clinical significance
• Autoimmune disease • Important to recognize pneumatosis intestinalis, but
• Pulmonary disease significance depends on etiology and clinical setting

• Linear or curvilinear shape • Associated mesenteric infiltration 4

• In ischemic etiology, bowel lumen will be dilated o Late phase of ischemia ~ diffuse or localized
(ileus), wall thickened & abnormally enhancing pneumatosis intestinalis ± mesenteric or portal 35
• ± Pneumoperitoneum or pneumoretroperitoneum venous gas
• ± Mesenteric or portal venous gas (portal venous • Pneumatosis intestinalis can occur with either
gas: Collects in periphery of liver; biliary gas: transmural or partial mural ischemia
Collects in central ducts near porta hepatis)
• ± Mesenteric arterial or venous thrombosis Post-endoscopy
• Mucosal disruption and 1 pressure ~ 1 distension ~
Imaging Recommendations air dissection into wall
• Best imaging tool
o Helical CT Post-operation
• Lung window: Detect intramural & venous gas • Example: Intestinal bypass
• Protocol advice • Mucosal disruption and 1 pressure ~ 1 distension ~
o Water for "oral contrast" air dissection into wall
• Facilitates CT angiography Medication-induced
o Helical CT at 1.S to 3 mm collimation
• Example: Steroids or immunosuppressives
• Image delay approximately 3S seconds
• 1 Mucosal permeability and! immune system ~
• Repeat venous phase after 80 seconds
bacterial gas in wall
• Intravenous contrast at 3-4 mllsecond
• Multiplanar reformation (CT angiography) Autoimmune disease
• Example: Systemic lupus erythematosus
• 1 Mucosal permeability and I immune system ~
I DIFFERENTIAL DIAGNOSIS bacterial gas in wall
Bowel necrosis Pseudopneumatosis
• Example: ischemic enteritis, volvulus or necrotizing • Gas trapped against mucosal surface of bowel by
enterocolitis semisolid feces
• Mucosal damage ~ entry of bacteria (mainly enteric • Most common in ascending colon
organisms) into bowel wall ~ gas in wall
• Necrotizing enterocolitis Pulmonary disease
o Location: Ileum and right colon • Example: Chronic obstructive pulmonary disease and
o Feces-like appearance in right bowel ventilator (barotrauma)
o Age: Premature or debilitated infants • Partial bronchial obstruction & coughing ~ alveolar
o Prognosis: Very low survival rate rupture ~ air dissection into peribronchial &
o Gas in intrahepatic branches of portal vein: perivascular tissue planes of the mediastinum ~ hiatus
Catastrophic sign of esophagus & aorta ~ retroperitoneum ~ mesentery
• Ischemic colitis or enteritis ~ subserosa and submucosa of the bowel wall
o Colonic ischemia
• Often due to hypoperfusion in elderly, debilitated
o Small bowel infarction: Often due to embolus or [PATHOLOGY
thrombus of large vessels (superior mesenteric artery
or vein) General Features
• General path comments
 PNEUMATOSIS OF THE INTESTINE
o Pneumatosis intestinalis is a sign, not a disease
o 15% of pneumatosis intestinalis is primary Demographics
o 85% of pneumatosis intestinalis is secondary • Age
o Portal venous gas o Primary: Adults
• Often accompanies pneumatosis intestinalis o Secondary: Any age
• Amount of gas does not correlate with etiology, • Gender: M = F
prognosis or need for treatment
Natural History & Prognosis
• Not always due to bowel infarction
• Complications
• Increased likelihood of transmural infarction from
o Spontaneous rupture of pneumatosis ~
ischemic enteritis versus ischemic colitis
pneumoperitoneum
• Etiology
• Prognosis
o Secondary pneumatosis intestinalis (at least 50
o Benign or catastrophic prognosis cannot be
reported causes)
distinguished by imaging
• Bowel necrosis (most common): Necrotizing
o Primary: Good
enterocolitis, bowel infarction or caustic ingestion
o Secondary: Depends on etiology of gas; not extent
• Mucosal disruption: Endoscopy, ulcers,
o Detection of pneumatosis and portomesenteric
obstruction, inflammatory bowel disease (IBD) or
venous gas
bowel anastomoses
• Positive on radiography ~ 75% mortality
• Increased mucosal permeability: Steroids,
• Positive on CT ~ 25% mortality (more sensitive)
chemotherapy, immunosuppressive therapy,
o Pneumatosis associated with pneumoperitoneum or
4 immunodeficiency states
• Auto-immune: Systemic lupus erythematosus,
portal venous gas can be "benign" and transient

36 scleroderma or other collagen vascular diseases Treatment

• Pulmonary: Asthma, chronic obstructive
pulmonary disease, positive pressure ventilation,
pneumothorax or trauma
o Portal venous gas
• Intestinal wall lesions: Ischemia or IBD
• Bowel distention: Endoscopy, obstruction or
trauma
• Sepsis: Diverticulitis, cholecystitis, appendicitis,
colitis including Clostridium difficile infection
o Pathogenesis
• Intraluminal gastrointestinal gas: Intramural
pressure or mucosal injury ~ gas entering wall
• Bacterial gas production: Bacterial invasion ~ has
high tension ~ gas diffusion
• Pulmonary gas: Air dissection
Gross Pathologic & Surgical Features
• Primary: Gas cysts in otherwise normal colon
• Secondary: Bowel is often abnormal or ischemic
Microscopic Features
• Primary
o Multiple thin-walled, non communicating, gas-filled
cysts in subserosal or submucosal layer of the bowel
o Normal muscularis and mucosa
• Secondary
o Linear streaks of gas parallel to bowel wall
o Necrotic, inflammatory, ulcerative or ischemic
features
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Primary: Asymptomatic; insignificant
o Secondary
• Abdominal pain, distension, melena, fever,
vomiting and cough
• Depends on etiology
• Primary
o No treatment; resolves spontaneously
• Secondary
o Dependent on underlying etiology
o Oxygen may be beneficial: !Gas tension in tissues
I DIAGNOSTIC CHECKLIST
Consider
• Bowel necrosis is a surgical emergency
• Prognosis depends on underlying cause, not imaging
findings
• Other causes of pneumatosis intestinalis usually are
asymptomatic; little clinical significance
Image Interpretation Pearls
• Important to recognize pneumatosis intestinalis, but
significance depends on etiology and clinical setting
I SELECTED REFERENCES
1. See C et al: Images in clinical medicine. Pneumatosis
intestinalis and portal venous gas. N Engl J Med. 350( 4):e3,
2004
2. Sherman 5C et al: Pneumatosis intestinalis and
portomesenteric venous gas. J Emerg Med. 26(2):213-5,
2004
3. 5t Peter 5D et al: The spectrum of pneumatosis intestinalis.
Arch Surg. 138(1):68-75,2003
4. Kernagis LY et al: Pneumatosis intestinalis in patients with
ischemia: correlation of CT findings with viability of the
bowel. AJR Am J Roentgenol. 180(3): 733-6, 2003
5. Sebastia C et al: Portomesenteric vein gas: Pathologic
mechanisms, CTfindings and prognosis. Radiographies.
20: 1213-24, 2000
6. Pear BL: Pneumatosis intestinalis: A review. Radiology. 207:
13-19, 1998
7. Faberman RS et al: Outcome of 17 patients with portal
venous gas detected by CT. AJR. 169: 1535-8, 1997
 PNEUMATOSIS OF THE INTESTINE
I IMAGE GALLERY
Typical
(Left) Axial NECT shows
extensive portal venous gas
in a patient with fatal bowel
infarction. (Right) Axial
NECT in a patient with fatal
bowel infarction shows
pneumatosis throughout the
small intestine and right
colon.

4
Typical 37
(Left) Axial NEeT shows
extensive portal venous gas
in a patient with bowel
infarction who recovered
completely following bowel
resection. (Right) Axial CECT
shows pneumatosis and
mesenteric venous gas
(arrow). Surgery revealed
infarcted bowel + patient
recovered completely.

Typical
(Left) Axial CECT shows
extensive pneumatosis and
ileus or obstruction. Patient
had resection for infarcted
bowel + recovered
completely. (Right) Supine
radiograph shows linear and
bubbly appearance of gas in
wall of dilated bowel. Also
note persistent nephrogram
following prior hypotensive
episode.
 ISCHEMIC ENTERITIS

Graphic shows dilated small intestine with thickened Axial CECT shows severely atherosclerotic aorta. Small
wall, ascites, + edematous mesentery; findings seen bowel is dilated with extensive gas in bowel wall
typically with occlusion of the superiormesenteric vein. (arrow) + mesenteric veins (open arrow).
4
38
o Multiple air-fluid levels; ileus pattern
ITERMINOLOGY o Thickening of valvulae conniventes
Abbreviations and Synonyms o Linear distribution of gas (pneumatosis intestinalis)
• Small bowel ischemia; mesenteric ischemia • Fluoroscopic-guided barium studies
o Thickening of valvulae conniventes
Definitions o "Thumbprinting": Intramural accumulation of blood
• Mesenteric arterial or venous narrowing or occlusion distending the submucosa ~ focally rounded
leading to inadequate supply of nutrients and oxygen mesenteric folds, especially along the mesenteric
to the small intestine border of bowel
o "Stack of coins" appearance: Enlarged, smooth,
straight, parallel folds perpendicular to longitudinal
I IMAGING FINDINGS axis of the small bowel (submucosal edema)
o Strictures can be seen with proximal bowel dilation
General Features o Mottled, frothy, bubbly or linear collections of gas
• Best diagnostic clue: Clot or narrowing of superior in bowel wall (pneumatosis intestinalis)
mesenteric artery (SMA) or superior mesenteric vein
(SMV) with bowel wall thickening CT Findings
• Other general features • CECT
o Imaging findings vary: Acute vs. chronic; arterial vs. o Clot or reduced lumen in SMA, SMV or other
venous thrombosis mesenteric vessels
o Classification of ischemic enteritis o Segmental thickening of bowel wall (> 3 mm);
• Acute occlusive ischemia (arterial or venous) average 8 mm, up to 20 mm
• Acute nonocclusive ischemia o Emboli usually observed in origin of SMA or within
• Chronic ischemia: Older "vasculopaths" 3-10 cm of SMA distal to middle colic artery
o Late phase of ischemia can lead to diffuse or o Compromised arterial blood flow ~ lack of mucosal
localized "pneumatosis intestinalis" enhancement
o "Misty mesentery": Mesenteric fat infiltrated by
Radiographic Findings edema; more with venous thrombosis
• Radiography

DDx: Thick Bowel Wall + Infiltrated Mesentery

\ .•- ~
...
.,
••
Shock Bowel Crohn Disease Fibrosing Mesenteritis Carcinoid Tumor
 ISCHEMIC ENTERITIS
Key Facts
Terminology
• Small bowel ischemia; mesenteric ischemia
Imaging Findings
• Best diagnostic clue: Clot or narrowing of superior
mesenteric artery (SMA) or superior mesenteric vein
(SMV) with bowel wall thickening
• Late phase of ischemia can lead to diffuse or localized
"pneumatosis intestinalis"
• Segmental thickening of bowel wall (> 3 mm);
average 8 mm, up to 20 mm
Top Differential Diagnoses
• "Shock bowel"
• Crohn disease
• Fibrosing mesenteritis
• Carcinoid tumor
o Submucosal hemorrhage or hyperemia ~ increased
attenuation (venous> arterial thrombosis)
o Pneumatosis intestinalis (venous> arterial
thrombus)
• "Band-like" (linear) or "bubble-like" (cystic)
appearance in the bowel wall
• Linear, curvilinear or cystic, gas-filled spaces
• ± Gas in mesenteric or portal vein
• Bowel loops are partly fluid-filled
Ultrasonographic Findings
• Duplex Doppler
o Mainly used to assess degree of narrowing or
occlusion in chronic ischemia
o Narrowed or occluded vessels ~ !blood flow
Angiographic Findings
• Conventional
o Acute arterial ischemia: Clot or stenosis of SMA or
its branches
o Acute venous ischemia: SMV occlusion with
collaterals
o Nonocclusive ischemia: Slow flow in SMA
o Chronic ischemia: Narrowing or occlusion of celiac
artery, SMA or inferior mesenteric artery
• Increased collateral arteries
Imaging Recommendations
• Best imaging tool
o Multidetector CT with CT angiography
• More sensitive in assessing strangulation of bowel
• Lung window setting: For pneumatosis intestinalis
o Angiography
• Diagnostic confirmation and treatment
I DIFFERENTIAL DIAGNOSIS
"Shock bowel"
• Ischemia ± reperfusion of small bowel, usually
following trauma or other cause of hypotension
• Intense mucosal enhancement
• Submucosal and mesenteric edema
• Reversible with resuscitation
Pathology
• Vascular occlusion: Embolic events (atrial fibrillation
or endocarditis), thrombotic events (atherosclerosis)
or mechanical obstruction (strangulation or tumor)
Clinical Issues
• Unremitting abdominal pain out of proportion to
physical exam findings
• Intestinal angina: Postprandial abdominal pain that
subsides 1-2 hours after meal
Diagnostic Checklist
• Small bowel ischemia is a clinico-radiological
diagnosis
• Prognosis depends on underlying cause, not imaging
• Gas-filled dilated intestinal loops with multiple
air-fluid levels; bowel wall thickening
4
Crohn disease
• Location: Usually distal small bowel 39
• Differentiate from ischemia
o Asymmetric, discontinuous, thickened bowel wall
with proliferation of mesenteric fat
o Abnormalities of the folds persist; changes occur
within days to weeks in ischemic enteritis
o Focal, inflammatory, mucosal ulceration with
patchy submucosal fibrosis ~ distortion and
interruption of folds
Fibrosing mesenteritis
• Idiopathic process of inflammation and fibrosis
involving fatty tissue of mesentery
• Bowel wall thickening uncommon, due to fibrotic
constriction of mesenteric veins and lymphatics
• "Misty mesentery" appearance with halo of fat
surrounding mesenteric vessels without displacing
these vessels
• Mesenteric soft tissue mass (advanced)
• Calcifications seen histologically; rare radiologically
Carcinoid tumor
• Focal bowel wall thickening with focal desmoplastic
reaction
• Encasement of mesenteric vessels ~ edema, ischemia
• Multiple hepatic metastases can usually be found
I PATHOLOGY
General Features
• General path comments
o Accounts for 1% of acute abdomen
o Arterial-to-venous occlusive ischemia: 9:1
o 60-70% of acute ischemia is from arterial occlusion
o 5-10% of acute ischemia is from venous occlusion
o 20-30% of acute ischemia is nonocclusive ischemia
• Etiology
o Vascular occlusion: Embolic events (atrial fibrillation
or endocarditis), thrombotic events (atherosclerosis)
or mechanical obstruction (strangulation or tumor)
o Closed loop obstruction is especially dangerous
 ISCHEMIC ENTERITIS
o Hypoperfusion (more common in ischemic colitis):
Low flow states, hypotension, sepsis or heart failure
o Hypercoagulable states: Oral contraceptives, protein
C deficiency, factor V Leiden deficiency
o Inflammatory: Pancreatitis, peritonitis or vasculitis
• Common cause of ischemia in younger patients
• Systemic lupus erythematosus, polyarteritis
nodosa or other collagen vascular diseases
• Vasculitis may affect kidneys and other organs
• Angiography may show microaneurysms and
occluded vessels
o Iatrogenic causes: Chemotherapy, radiation therapy,
legal drugs (digitalis, dopamine or vasopressin) and
illegal drugs (heroin or cocaine)
o Risk factors of chronic ischemia: Hypertension,
coronary artery disease or cerebrovascular disease
o Pathogenesis of pneumatosis intestinalis
• Ischemia ~ mucosa damage ~ necrosis and entry
of bacteria (mainly enteric organisms) into bowel
wall ~ gas in bowel wall
4 Gross Pathologic & Surgical Features
• Discolored (purple), infarcted small bowel
40
Microscopic Features
• Necrotic, inflammatory or ischemic features in the
small bowel wall
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute ischemia
• Clinical triad: Sudden onset of abdominal pain,
diarrhea and vomiting
• Unremitting abdominal pain out of proportion to
physical exam findings
• Abdominal distention, tenesmus and passage of
bloody stool
• Guarding and rebound (infarction or perforation)
• Venous ischemia has a more gradual onset
o Chronic ischemia
• Intestinal angina: Postprandial abdominal pain
that subsides 1-2 hours after meal
• Nausea, vomiting, diarrhea and weight loss
• Intense pain ~ fear of eating (sitophobia)
o Lab-Data
• t WBC: 75%; Acidosis: 50%; t amylase: 25%
o Diagnosis
• High clinical suspicion is key to early diagnosis
Demographics
• Age: Majority> 50 years of age
• Gender: M = F
Natural History & Prognosis
• Complications
o Stricture, infarction, necrosis and perforation
• Prognosis
o Acute ischemia
• Depends on promptness of diagnosis and the
amount of small bowel that can be saved
• After surgical resection, results of patients with
venous ischemia are generally better
• 50-90% mortality
o Chronic ischemia
• Key to survival is dependent on degree of
collateral circulation
• Diagnosis requires at least two major mesenteric
arteries to be occluded and a third artery to be
narrowed
o Infarction: 69% mortality (in recent series)
o Pneumatosis intestinalis: Poor, dependent on
etiology of gas
o Pneumatosis intestinalis with postmesenteric venous
gas
• Positive on radiography: 75% mortality
• Positive on CT: 25% mortality (more sensitive)
Treatment
• Surgical treatment
o Exploratory laparotomy, bowel resection and
mesenteric bypass (re-establish blood flow)
o Main treatment modality for acute ischemia,
chronic ischemia and complications
• Endovascular intervention
o Intra-arterial thrombolysis, percutaneous
transluminal angioplasty ± stent placement
o Thrombolytics (streptokinase or urokinase)
o Vasodilators (papaverine): Reduce vasospasm
• Systemic anticoagulation (warfarin or heparin) for
venous occlusion
I DIAGNOSTIC CHECKLIST
Consider
• Small bowel ischemia is a clinico-radiological
diagnosis
• Talk to referring physician for history, symptoms and
key lab values
Image Interpretation Pearls
• Prognosis depends on underlying cause, not imaging
• Gas-filled dilated intestinal loops with multiple
air-fluid levels; bowel wall thickening
I SELECTED REFERENCES
1. Chou CK et al: CT of small bowel ischemia. Abdom
Imaging, 24-30, 2003
2. Segatto E et al: Acute small bowel ischemia: CT imaging
findings. Semin Ultrasound CT MR. 24(5):364-76, 2003
3. Tendler DA: Acute intestinal ischemia and infarction.
Semin Gastrointest Dis. 14(2):66-76,2003
4. Burns BJet al: Intestinal ischemia. Gastroenterol Clin
North Am. 32(4):1127-43, 2003
5. Wiesner W et al: CT of acute bowel ischemia. Radiology.
226(3):635-50, 2003
6. Horton KM et al: Computed tomography evaluation of
intestinal ischemia. Semin Roentgenol. 36(2):118-25, 2001
7. Horton KM et al: Multi-detector row CT of mesenteric
ischemia: can it be done? Radiographies. 21(6):1463-73,
2001
8. Singer A et al: Acute small bowel ischemia: Spectrum of
computed tomographic findings. Emer Radiol. 7: 302-307,
2000
 ISCHEMIC ENTERITIS
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
dilated fluid-filled small
intestine + clot in portal vein
(arrow). (Right) Axial CECT
in 23 year old woman with
hypercoagulable state +
bowel ischemia. Dilated
fluid-filled small bowel +
thrombosis of the SMV
(arrow).

4
41
(Left) Aortography shows
irregular narrowing of aorta
and occluded celiac +
superior mesenteric arteries.
Dilated gas-filled ischemic
small bowel. (Right) 5BFT
shows "stack of coins" small
bowel fold pattern due to
ischemia, intramural
hemorrhage.

Typical
(Left) Axial CECT shows
dilated fluid-filled small
bowel, pneumatosis (arrow)
+ ascites. Duodenum +

. jejunum are twisted with a

"whirl sign" (open arrow)
71 . due to mid-gut volvulus.
(Right) Axial CECT shows

. -fI)' dilated small bowel with

areas of wall thickening

,. ..
(arrow). Patient had severe
acute abdominal pain +
acidosis. Bowel infarction
from atrial fibrillation .
 VASCULITIS, SMALL INTESTINE
Axial CECT shows multifocal
parenchymal lesions due to rheumatoid vasculitis.
4
42
ITERMINOlOGY
Definitions
• Inflammation of the blood vessels of small intestine
caused by a large group of rare, systemic conditions
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Straight thickened folds with
luminal dilatation of the small bowel
• Other general features
o Categories of vasculitis
• Large-vessel vasculitis: Aorta, main visceral arteries
(e.g., superior mesenteric artery)
• Medium-vessel vasculitis: Main visceral arteries
and their branches
• Small-vessel vasculitis: Arterioles, venules,
capillaries
o Different sizes of blood vessels are affected by
various systemic conditions; however, imaging
findings overlap
o Most common systemic conditions include
• Polyarteritis nodosa: Small- to medium-vessel
• Henoch-Schonlein purpura (HSP): Small-vessel
• Systemic lupus erythematosus (SLE): Small-vessel
• Beh<;et syndrome: Small-vessel
DDx: Thick Bowel Wall and Ileus
Bowe/lschemia Crohn Disease
renal ischemic Axial CECT shows marked 58 wall thickening and renal
ischemic parenchymal lesions due to rheumatoid
vasculitis.
Radiographic Findings
• Fluoroscopic-guided barium studies
o Segmental or extensive intestinal involvement
o Aphthous ulcers
o Straight, thickened folds ± dilatation of bowel
lumen
o Concentric filling defects (submucosal hemorrhage)
o Ulceration and stricture (small-vessel)
o ± Thumbprinting; pneumatosis intestinalis
o SLE
• Nodularity of small bowel folds
• Motility disorder of lower esophagus
• Esophagitis and gastritis
o Beh<;et syndrome
• Involves ileocecal area, especially terminal ileum;
may simulate Crohn disease
• Narrowing and irregularity of terminal ileum with
slight dilatation proximally
• Large ovoid or irregular ulcers with marked
mucosal thickening of surrounding intestinal wall
• Small, multiple, discrete, "punched-out" ulcers
CT Findings
• Thickened bowel wall ± target sign
• Contrast enhancement of bowel wall
• Submucosal hemorrhage and edema
• Polyarteritis nodosa
Shock Bowe/
 VASCULITIS, SMALL INTESTINE

Key Facts
Terminology Top Differential Diagnoses
• Inflammation of the blood vessels of small intestine • Ischemic enteritis
caused by a large group of rare, systemic conditions • Crohn disease
• Small bowel obstruction (SBO)
Imaging Findings • "Shock bowel"
• Best diagnostic clue: Straight thickened folds with
luminal dilatation of the small bowel Clinical Issues
• Different sizes of blood vessels are affected by various • Abdominal pain, fever, nausea, vomiting, weight loss,
systemic conditions; however, imaging findings diarrhea or constipation
overlap
• Segmental or extensive intestinal involvement Diagnostic Checklist
• Aphthous ulcers • Differentiate by associated extraintestinal pattern of
• Ulceration and stricture (small-vessel) involvement and by size of vessels affected
• Submucosal hemorrhage and edema • Biopsy and clinical findings essential for diagnosis
• Aneurysm formation • Imaging can suggest vasculitis as a cause of small
bowel disease, but angiography and other tests are
essential to diagnosis

o Lobulated renal contour and irregular thinning • Distinguish by observing clot or narrowing of superior 4
(cortical infarcts) mesenteric artery, superior mesenteric vein or other
o Multiple hypo attenuating bands of kidney (arterial mesenteric vessels 43
occlusion), or "striated nephrogram" • Imaging features usually indistinguishable from
• SLE vasculitis, especially large-vessel vasculitis
o Abnormal bowel wall enhancement; dilated bowel
o Comb sign: Engorged mesenteric vessels in a Crohn disease
comb-like arrangement • Skip lesions, transmural inflammation, non-caseating
o Ascites, lymphadenopathy granulomas, cobblestone mucosa and fistulas
o Multiple linear hypoattenuating bands of kidney • Differentiate by irregular, prominent mural
o Hepatomegaly, splenomegaly thickening, fused and distorted folds
• Behs;et syndrome Small bowel obstruction (SBO)
o Concentric bowel wall thickening or polypoid mass
o ± Perienteric or pericolonic infiltration • Closed loop ~ segmental obstruction and ischemia
• Air-fluid levels; smooth beaking
Angiographic Findings • Bowel wall thickening
• Conventional • ± Portal venous gas, pneumatosis intestinalis
o Aneurysm formation
"Shock bowel"
• May be seen in patients with polyarteritis nodosa,
• Ischemia ± reperfusion of small bowel, usually
SLE,Wegener granulomatosis" rheumatoid
following trauma or other cause of hypotension
vasculitis, Churg-Strauss syndrome, drug abuse
o Polyarteritis nodosa visceral involvement • Intense mucosal enhancement
• Renal (80-90%), GI tract (50-70%), heart (65%), • Submucosal and mesenteric edema
liver (50-60%), spleen (45%), pancreas (25-35%), • Reversible with resuscitation
central nervous system (rare)
• Small intestine is most commonly affected in GI
tract; followed by mesentery and colon I PATHOLOGY
• Multiple aneurysms (50-60% of cases), typically at General Features
branching points
• Etiology
• 1-5 mm saccular aneurysms (more common) or o Large-vessel
fusiform aneurysms
• Giant cell arteritis
• Stenoses or occlusions of arteries
• Takayasu disease
Imaging Recommendations o Medium-vessel
• Best imaging tool: Helical CECT and angiography • Polyarteritis nodosa: > 50% involves GI
• Kawasaki disease
• Primary granulomatous central nervous system
I DIFFERENTIAL DIAGNOSIS vasculitis
o Antineutrophil cytoplasmic autoantibody
Ischemic enteritis (ANCA)-associated small-vessel vasculitis
• Multiple causes including embolus, thrombosis, • Microscopic polyangiitis
volvulus • Wegener granulomatosis
• Churg-Strauss syndrome
o Immune-complex small-vessel vasculitis
 VASCULITIS, SMALL INTESTINE
• HSP: > 50% involves GI • Diagnosis: Biopsy of involved tissue may help establish
• SL£: 10-60% involves GI diagnosis
• Behs:et syndrome: 10-40% involves GI
• Cryoglobulinemic vasculitis Demographics
• Rheumatoid vasculitis • Age
• Sjogren syndrome o Polyarteritis nodosa: 18-81 years of age
• Hypocomplementemic urticarial vasculitis o HSP: 3-10 years of age (most common), > 20 years of
• Goodpasture syndrome age (up to 30% of cases)
• Serum sickness o SL£: 16-41 years of age
• Drug-induced o Behs:et syndrome: 11-30 years of age
• Infection-induced • Gender
o Paraneoplastic small-vessel vasculitis o Polyarteritis nodosa: M:F = 2:1
• Lymphoproliferative neoplasm-induced o HSP: M:F = 2:1
• Myeloproliferative neoplasm-induced o SL£: M:F = 1:10
• Carcinoma-induced vasculitis o Behs:et syndrome: M:F = 2:1
o Inflammatory bowel disease small-vessel vasculitis Natural History & Prognosis
o Risk factors: HSP: Bacterial or viral infection,
• Complications
allergies, insect sting, drugs, certain foods
o Paralytic ileus, ischemia, hemorrhage, perforation,
• Associated abnormalities
stricture, fistula, peritonitis, sepsis
o Polyarteritis nodosa: Hepatitis B infection
o Polyarteritis nodosa: Renal failure, congestive heart
4 o SL£: Hematologic, immunologic and neurologic
involvement, photosensitivity, oral ulceration
failure, myocardiac infarction, cirrhosis, hepatic
carcinoma
44 o HSP: Renal involvement
o HSP: Intussusception in children, renal failure
o Behs:et syndrome: Neurologic involvement
o SL£: Renal failure
Gross Pathologic & Surgical Features o Prognosis: Good, unless left untreated with
• Segmental fibrinoid necrotizing vasculitis complications
• Nonspecific ulceration or inflammation Treatment
• Polyarteritis nodosa: Panmural necrotizing arterial
• Polyarteritis nodosa: Corticosteroid ±
vasculitis; mucoid degeneration
cyclophosphamide
• Behs:et's syndrome: Discrete, "punched-out" ulcers and
• HSP: Spontaneous resolution
irregular perforations
• SL£: Corticosteroid, non-steroid anti-inflammatory
Microscopic Features drugs, hydroxychloroquine
• Polyarteritis nodosa • Behs:et syndrome: Corticosteroid, sulfasalazine
o Acute: Polymorphonuclear cell infiltrate in all layers
of arterial wall and perivascular tissue
o Chronic: Mononuclear cell infiltrate with intimal I DIAGNOSTIC CHECKLIST
proliferation, thrombosis and perivascular
Consider
inflammation
• SL£: Local deposition of antigen-antibody complexes • Differentiate by associated extraintestinal pattern of
involvement and by size of vessels affected
• HSP: Immunoglobulin A deposited in vessel wall
(direct immunofluorescence) • Biopsy and clinical findings essential for diagnosis
• Behs:et syndrome: Immune complexes deposited in Image Interpretation Pearls
vessel wall • Imaging can suggest vasculitis as a cause of small
bowel disease, but angiography and other tests are
essential to diagnosis
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
I SELECTED REFERENCES
o Abdominal pain, fever, nausea, vomiting, weight 1. Ha HK et al: Radiologic features of vasculitis involving the
loss, diarrhea or constipation gastrointestinal tract. Radiographics.20(3):779-94, 2000
2. Rha SE et al: CT and MR imaging findings of bowel
o Polyarteritis nodosa: Peripheral neuropathies
ischemia from various primary causes. Radiographies.
o HSP: Palpable purpura, arthritis, GI bleeding 20(1):29-42, 2000
o SL£: Cough (serositis), oral ulcers, polyarthritis, 3. Byun JY et al:CT features of systemic lupus erythematosus
malar rash, discoid rash in patients with acute abdominal pain: emphasis on
o Behs:et's syndrome: Oral and genital ulcers, arthritis, ischemic bowel disease. Radiology. 211(1):203-9, 1999
uveitis, erythema nodosum 4. Ha HK et al: Intestinal Behcet syndrome: CT features of
• Lab-Data patients with and patients without complications.
o Polyarteritis nodosa: Cryoglobulin, positive for Radiology. 209(2):449-54, 1998
5. Jeong YKet al: Gastrointestinal involvement in
hepatitis B surface antigen
Henoch-Schonlein syndrome: CT findings. AJRAm J
o HSP: Hematuria, proteinuria Roentgenol. 168(4):965-8, 1997
o SL£: Antinuclear antibody, anti-Smith antibody
 VASCULITIS, SMALL INTESTINE
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
decreased renal
enhancement, multifocal 58
mural hemorrhage, and
hemorrhagic ascites in a 24
year old man with
Henoch-Schonlein purpura
(HSP). (Right) Clinical
photograph of purpuric skin
rash in a 24 year old man
with HSP.

4
Typical 45
(Left) Axial NECT shows
multifocal 58 mural
hemorrhage due to Sjogren
syndrome. (Right) Axial
NECT shows long segmental
mural thickening in a patient
with Sjogren vasculitis.

(Left) Axial NECT shows long

segmental mwal thickening
attributed to vasculitis in this
I.V. drug abuser. (Right)
58FT shows multifocal
segmental 58 wall thickening
and luminal narrowing
attributed to vasculitis in a
patient with systemic lupus.
 INTESTINAL TRAUMA

Axial CECT shows free air + blood in perihepatic Axial CECT shows fluid (blood + bowel contents) in
location. Distal small bowel and sigmoid transecUons. para colic gutter + mesentery, along with free air
(arrow); transected small bowel.
4
46
• Transverse tears of mesentery ~ hematoma ~
ITERMINOlOGY bowel infarction
Definitions o Rapid deceleration injuries
• Injury to bowel (duodenum, small bowel, colon) • Caused by abrupt forward movement of proximal
jejunum from its fixation by ligament of Treitz
• Shearing force between restricted & mobile bowel:
IIMAGING FINDINGS Cause transection at duodenojejunal flexure
o Gastric injury
General Features • More common in children than in adults
• Best diagnostic clue: Bowel wall thickening, • t Risk: Distended stomach after eating
mesenteric infiltration ± extravasation of enteric or • Associated injuries: Rupture of spleen & left-sided
vascular contrast medium thoracic injury
• Location: Duodenum + proximal jejunum (most o Duodenal injury
common) • Common findings: Hematoma; ectopic air or
• Other general features contrast (perforation)
o Abdominal trauma: Leading cause of death in • Location: Descending 2nd & horizontal 3rd part
United States « 40 yrs old) • 3rd part compressed against spine by a direct blow
o Children: t Incidence of intramural hematoma • Associated injury: Pancreatic head, left lobe of
o Adults: t Incidence of bowel wall transection liver
o Bowel & mesenteric injuries: Seen in S% of patients o Jejunal & ileal injury
with blunt trauma at laparotomy • Common findings: Hematoma, bowel wall
o Most common causes discontinuity, thickening
• Motor vehicle accidents (MVA), falls & assault • At or near ligament of Treitz & ileocecal valve
o Impact injuries • Clinically: Symptoms & signs develop slowly (due
• Crushing of bowel against spine to neutral pH & relative absence of bacteria)
• Location: Small bowel of limited mobility o Colon injury
(duodenum, near ligament of Treitz & near • Cause: Compression of upper abdomen (steering
ileocecal valve) wheel & seat belts)

DDx: Hemorrhage or Edema in the Bowel Wall

Shock Bowel jejunal Hematoma Henoch-Schonlein Ischemic Bowel

 INTESTINAL TRAUMA
Key Facts
Terminology
• Injury to bowel (duodenum, small bowel, colon)
Imaging Findings
• Best diagnostic clue: Bowel wall thickening,
mesenteric infiltration ± extravasation of enteric or
vascular contrast medium
• Bowel discontinuity: Injury primary finding, unusual
• Extraluminal oral contrast material: 100% specific for
bowel perforation, but uncommon
• Intramural air, extraluminal air, interloop free fluid
• Bowel wall thickening: More than 3 mm (seen in 75%
of transmural injuries)
• Mesenteric infiltration or "stranding"
• Hematoma (> 60 HU)i liquefied blood (35-50 HU)
• Active bleeding: Isodense with enhanced vessels
• Location: Transverse colon, sigmoid colon, cecum
• Transverse: Intramural hematoma or serosal tear
• Ascending or descending: Mesenteric avulsion,
full-thickness laceration, transection, ischemia
• Complications: Ischemic stricture or perforation
o Mesenteric injury
• Hematoma: Most common GIT injury seen on CT
• Complications: Disruption of mesenteric
vasculature, hemorrhage & GIT perforation
• Active mesenteric bleeding requires surgery
Radiographic Findings
• Radiography
o "Flank-stripe" sign: 1 Density zone (> 800 ml
abdominal fluid) separates vertical colon segments
from properitoneal fat & peritoneal reflection
o "Dog's-ear" sign: Pelvic fluid collections displace
bowel from urinary bladder
• Fluoroscopy (water soluble contrast) study
o Fold thickening, luminal narrowing, extravasation
o Mainly for duodenal hematoma & laceration
CT Findings
• Must view at "abdominal" & "lung" windows
• Bowel discontinuity: Injury primary finding, unusual
• Extraluminal oral contrast material: 100% specific for
bowel perforation, but uncommon
• Extraluminal air: Intra-or retroperitoneal air
• Extraluminal gas not diagnostic of bowel perforation
(also seen in barotrauma & mechanical ventilation)
• Location
o Perihepatic, perisplenic regions
o Trapped in leaves of mesentery; omental interstices
o Trapped by adhesions or ligaments (e.g., falciform)
• Intramural air, extraluminal air, interloop free fluid
o Indicates full-thickness tear
• Bowel wall thickening: More than 3 mm (seen in 75%
of transmural injuries)
o Circumferential or eccentric thickening: Due to
intramural hematoma, mesenteric trauma (arterial
or venous injury)
• Bowel-wall enhancement: More than HU of psoas
muscle or equal to blood vessels
Top Differential Diagnoses
• Shock bowel
• Coagulopathy
• Vasculitis
• Ischemic enteritis
Clinical Issues
• Clinical profile: Patient with history of MVA,
abdominal pain, distension, tenderness & guarding
• Diagnostic peritoneal lavage (DPL): Positive, severe
injury
Diagnostic Checklist
• Check for MVA history or other abdominal injury
• CT evidence of extraluminal air/contrast, bowel wall
thickening, free fluids & mesenteric "stranding"
highly suggestive of bowel injury
o Enhancement + thickening + free fluid: Strongly 4
suggests perforation
47
• Mesenteric infiltration or "stranding"
o Small hemorrhages: Streaky soft tissue infiltration of
mesenteric fat
o "Sentinel clot" sign: Localized> 60 HU mesenteric
hematoma at site of bleeding
• Intra-/retroperitoneal free fluid: Hemoperitoneum or
bowel contents
o Polygonal fluid collections between folds of
mesentery & bowel loops
o Hematoma (> 60 HU)i liquefied blood (35-50 HU)
o Bowel contenti extravasated enteric contrast
o Free intraperitoneal fluids: Common in bowel &
mesenteric injuries
o Hemoperitoneum: Common in intraperitoneal
bowel or mesenteric injury
o Active bleeding: Isodense with enhanced vessels
o Bowel rupture: At sites of oral contrast extravasation
Ultrasonographic Findings
• Real Time: Free fluid in abdomen & pelvis
Angiographic Findings
• Conventional: Vascular transection, laceration,
pseudoaneurysm, arteriovenous fistula
Imaging Recommendations
• Helical CECT ± oral contrast: Modality of choice
o LV. contrast 2: 3 ml/sec
I DIFFERENTIAL DIAGNOSIS
Shock bowel
• Intense mucosal enhancement, submucosal edema
(not blood)
• Often diffuse mesenteric edema + hypovolemia signs
o Signs of hypovolemia: Collapsed IVC & renal veins
• Is a reversible sign of recent hypotension
• Resolves quickly with fluid resuscitation
Coagulopathy
• Spontaneous, or anticoagulant treatment
 INTESTINAL TRAUMA
• Spontaneous bleeding: Example: Idiopathic
thrombocytopenic purpura, leukemia, hemophilia
• Abdominal pain, melena, intestinal obstruction
• Barium studies or CT of small bowel
o Segmental, extensive, or localized changes
o Uniform, regular thickening of valvulae conniventes
with a symmetric, spike-like configuration &
reduced luminal diameter simulating a stack of coins
o Localized bleeding may be seen as intramural mass
Vasculitis
• Polyarteritis nodosa
o Bowel: Diffuse or segmental ischemia/hemorrhage
o Renal & liver involvement are frequent
o Angiography: Small aneurysms of branches of SMA
• Systemic lupus erythematosus
o Causes small vessel arteritis in 10 to 60% of cases
o Segmental bowel lesions ~ necrosis & perforation
• Henoch-Schonlein purpura
o Children, young & middle-aged; GI tract (> 50%)
o Present with clinical triad
4 • Palpable purpura, arthritis & abdominal pain
• Barium studies or CT of small bowel in vasculitis
48
o Extensive fold thickening + luminal narrowing
o May show thumbprinting on mesenteric border
o Intussusceptions may be seen in childhood purpura
Ischemic enteritis
• Cause: Superior mesenteric vessel clot or narrowing
• Barium studies of small bowel
o Markedly thickened valvulae conniventes
o Thumbprinting: Thick, rounded folds along
mesenteric border (intramural blood collection)
• CT findings
o Shows clot or reduced lumen in SMA or SMV
o Segmental bowel wall thickening (> 3 mm)
o Later phase: Pneumatosis (focal or diffuse)
• Gas within small bowel wall & venous radicles
I PATHOLOGY
General Features
• Etiology: Blunt or penetrating trauma; falls; assault
• Epidemiology: 5% of blunt trauma at laparotomy
• Associated abnormalities: Hepatic, splenic, renal &
pancreatic injuries
Gross Pathologic & Surgical Features
• Contusion, laceration, bowel discontinuity
• Wall thickening, blood clot, rupture
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Abdominal pain, distension, tenderness, guarding
o Hypotension, tachycardia
o Loss of consciousness, shock: Due to 1 loss of blood
• Clinical profile: Patient with history of MVA,
abdominal pain, distension, tenderness & guarding
• Lab-data
o Altered CBC, electrolytes, BUN, creatinine, amylase,
PT, PIT & hematocrit
• Diagnostic peritoneal lavage (DPL): Positive, severe
injury
o RBC > 150,000/mm3; WBC > 500/mm3
o Food, bile or bacteria on Gram stain from aspirate
Demographics
• Age: Any age group
• Gender: M = F
Natural History & Prognosis
• Complications
o Perforation ~ sepsis ~ abdominal abscess ~
peritonitis ~ shock ~ death
• Prognosis
o Good: In early diagnosis & treatment
o Poor: In delayed diagnosis & treatment
• Increased morbidity & mortality up to 65%
Treatment
• Minor: Airway, LV. fluids, monitor vital signs, blood
transfusion, antibiotics
• Major: Surgery (perforation or active bleeding)
I DIAGNOSTIC CHECKLIST
Consider
• Check for MVA history or other abdominal injury
Image Interpretation Pearls
• CT evidence of extraluminal air/contrast, bowel wall
thickening, free fluids & mesenteric "stranding" highly
suggestive of bowel injury
I SELECTED REFERENCES
1. Hanks PW et al: Blunt injury to mesentery and small
bowel: CT evaluation. Radiol Clin North Am.
41(6):1171-82,2003
2. Hawkins AE et al: Evaluation of bowel and mesenteric
injury: role of multidetector CT. Abdom Imaging.
28(4):505-14,2003
3. Butela ST et al: Performance of CT in detection of bowel
injury. A]R Am] Roentgenol. 176(1): 129-35, 2001
4. Brody]M et al: CT of blunt trauma bowel and mesenteric
injury: typical findings and pitfalls in diagnosis.
Radiographies. 20(6):1525-36; discussion 1536-7, 2000
5. Federle MP: Diagnosis of intestinal injuries by computed
tomography and the use of oral contrast medium. Ann
Emerg Med. 31(6):769-71, 1998
6. Levine CD et al: CT findings of bowel and mesenteric
injury.] Comput Assist Tomogr. 21(6):974-9, 1997
7. Nghiem HV et al: CT of blunt trauma to the bowel and
mesentery. A]R Am] Roentgenol. 160(1):53-8, 1993
8. Rizzo M] et al: Bowel and mesenteric injury following
blunt abdominal trauma: evaluation with CT. Radiology.
173(1):143-8,1989
9. Wing VW et al: The clinical impact of CT for blunt
abdominal trauma. A]R Am] Roentgenol. 145(6):1191-4,
1985
 INTESTINAL TRAUMA
I IMAGE GALLERY
Typical
(Left) Axial CECT shows free
air (arrow) from jejunal
transection. (Right) Axial
CECT shows seat belt
contusion (arrow),
mesenteric infiltration, bowel
wall thickening, and free air
(open arrow). Jejunal
transection.

4
Typical 49
(Left) Axial CECT shows fluid
in anterior pararenal space.
Duodenal laceration. (Right)
Axial CECT shows
thick-walled jejunum +
mesenteric blood. Surgery:
Jejunal transection, splenic +
renal lacerations.

Typical
(Left) Axial CECT shows
thick-walled distal S8,
mesenteric blood (open
arrow) and two sites of
active mesenteric bleeding
(arrows). (Right) Axial CECT
shows intramural hematoma
of jejunum.
 GASTROINTESTINAL
Technetium 99 m tagged RBe scan (selected sequential
images) shows accumulation of radiotracer within the
cecum and ascending colon (arrows) indicating active
4 bleeding.
50
ITERMINOlOGY
Definitions
• Acute or chronic bleeding from gastrointestinal tract
I IMAGING FINDINGS
General Features
• Locatiqn
o Upper GI bleed: Proximal to ligament of Treitz
o Lower GI bleed: Distal to ligament of Treitz
• Key concepts
o GI bleeding may originate anywhere from mouth to
anus & may be overt or occult
o Classification based on location & presentation
• Upper GI bleed: Hematemesis; melena (may also
seen in small bowel or right colon bleeding)
• Lower GI bleed: Hematochezia (may also result
from vigorous upper GI bleeding, duodenal ulcer)
• Bleed anywhere in GI tract: Occult bleeding
detected by chemical testing of stool
o Classification based on onset & presentation
• Acute: Hematemesis, hematochezia, melena
• Chronic: Iron deficiency anemia
o Upper GI bleed accounts for 76% of GI hemorrhage
• Most common cause: Peptic ulcers (> 50% cases)
• Usually present with hematemesis, melena
DDx: Endoscopic Manifestations of GI Bleeding
Castric Ulcer Duodenal Ulcer
BLEEDING
Selective ileocolic arteriogram shows active bleeding in
the cecum (arrow). Bleeding diverticulum.
o Lower GI bleed accounts for 24% of GI hemorrhage
• Most common cause: Diverticulosis (50% cases)
• Usually present with hematochezia
o Endoscopy
• First line of procedure in upper GI bleeding
• Elective procedure; detect any rate of bleeding
• 90-95% accurate diagnosis
o Capsule endoscopy: Pitfalls
• May fail in bowel with strictures or prior surgery
• Long study time: 8 hrs prior preparation & 8 hrs
to compile & interpret data
• Hard to localize lesions & can also miss lesions
o Uncommon site of hemorrhage: Small bowel
between 2nd part of duodenum & ileocecal valve
• Accounts for 3-5% of all GI tract bleeding
• Difficult location to diagnose bleeding source
• Present with prolonged undiagnosed iron
deficiency anemia or episodes of melena with
normal upper endoscopy & colonoscopy
o Choice of imaging varies based on availability,
expertise, severity of hemorrhage, patient condition
& clinically suspected origin of bleeding
CT Findings
• NECT: Hyperattenuating hematoma within bowel
• CECT & CTA
o Active bleeding
• Linear, pooled or swirled focal collection of
hyperdense intraluminal contrast extravasation
Esophageal Varices Colonic AVMs
 GASTROINTESTINAL
Key Facts
Imaging Findings . • Colonic angiodysplasia (AVM)
• Upper GI bleed: Proximal to ligament of Treitz
• Lower GI bleed: Distal to ligament of Treitz
• Linear, pooled or swirled focal collection of
hyperdense intraluminal contrast extravasation
• Mesenteric angiography: > 0.5 mL/min
• Non-selective aortic angiography: 6 mL/min
• Criteria for a positive study: Radiolabeled RBCs
outside normal areas of blood pool
• Extravasation of isotope at active bleeding site
Top Differential Diagnoses
• Peptic ulceration
• Erosive gastritis
• Esophageal varices
• Diverticulosis
Angiographic Findings
• Conventional
o Usually preceded by Tc99m-labeled RBC scan
o Bleeding rate (required to be detected)
• Mesenteric angiography: > 0.5 mL/min
• Non-selective aortic angiography: 6 mL/min
o Diagnostic accuracy: 70-95%
Nuclear Medicine Findings
• Tc99m-labeled red blood cell (RBC) scans
o Bleeding rate: Requires 0.2 mL/min to be detected
• Less sensitive than Tc99m sulfer colloid (SC)scan
o Minimal amount of 5-10 mL of extravasated blood
must be present to be identified
o Sensitivity (85-95%); specificity (70-85%)
• Recent new technique: t Efficiency & sensitivity
o Continuous dynamic imaging (large FOV camera)
• 15 min dynamic image sequence of 60 images
• Repeated until bleeding identified/study stopped
o Criteria for a positive study: Radiolabeled RBCs
outside normal areas of blood pool
o Advantages of RBC scan
• Ability to monitor over a prolonged period of time
• t Likelihood of detecting intermittent bleeding
o Disadvantages of RBC scan
• Origin of bleed unclear on delayed scans
• Vascular organs may interfere with detection
• Loss oftag can produce false +/-
• Technetium Tc99m SC scans
o Bleeding rate: 0.05-0.1mL/min is detected
• More sensitive than Tc99m labeled RBC scan
o Extravasation of isotope at active bleeding site
o Highly sensitive & specific test in detecting active
bleeding
o Intravascular half-life: 2.5 minutes
• By 12-15 min, injected tracer is cleared from
vascular system, producing significant contrast
between bleeding site & surrounding tissue
o Usually valid, only for localizing lower GI bleeding
Imaging Recommendations
• Angiography
o Procedure of choice
BLEEDING
Pathology
• Duodenal ulcer (24%), gastric erosions (23%)
• Gastric ulcer (21%), varices (10%)
• Diverticulosis (43%), vascular ectasia (20%)
Clinical Issues
• Hematemesis: Bloody vomitus; red, coffee grounds
• Melena: Black, tarry stools (100-200 mL of blood in
upper GI tract is required to produce melena)
• Hematochezia: Red blood per rectum
• Occult blood detected by stool chemical testing
Diagnostic Checklist
• Scintigraphy for hemodynamically stable cases
• Angiography for hemodynamically unstable cases
• Hemodynamically unstable patients 4
• In active or massive GI bleeding
51
o Upper GI bleeding
• When endoscopy inconclusive
• Anticipation of transcatheter intervention
o Lower GI bleeding
• Angiography may be the procedure of choice
• Nuclear scintigraphy
o Procedure of choice
• Hemodynamically stable patients
• In active gastrointestinal hemorrhage
• Delineate obscure sources: Small bowel,
intermittent bleeding
• Enhance the efficacy of angiography
I DIFFERENTIAL DIAGNOSIS
Peptic ulceration
• Round or ovoid collections of barium
• Giant ulcers, usually located in duodenal bulb (t risk
of hemorrhage)
• Wall thickening or luminal narrowing
Erosive gastritis
• Complete or varioliform
o Multiple punctate or slit-like collections of barium
o Scalloped or nodular antral folds
o Location: Gastric antrum
• Incomplete or flat erosions
o Location: Antrum or body
o Multiple linear streaks or dots of barium
• Linear or serpiginous erosions clustered in body or
near greater curvature (NSAIDs induced)
Esophageal varices
• Mucosal relief views
o Tortuous, serpiginous, longitudinal radiolucent
filling defects in collapsed esophagus
• Double-contrast study: Multiple radiolucent filling
defects etched in white
• CT: Scalloped esophageal mural masses
 GASTROINTESTINAL
Diverticulosis
• 75% of tics-left colon; 70% bleeding tics-right colon
• 80% resolve spontaneously
• Up to 20% of cases bleed, 5% massively
• Flask-like protrusion, long or large neck
• Diverticulum with long & narrow neck: Mimic
pedunculated polyp on air-contrast enema
• Diverticulum with large neck: Mimics sessile polyp
• En face: Ring shadow or round barium collection
• "Bowler hat" sign: Dome of hat points away from
bowel wall (diverticulum); toward lumen (polyp)
• CT imaging
o Mural thickening of colon (4-15 mm)
o Multiple air or contrast or stool-containing
outpouchings (diverticula)
Colonic angiodysplasia (AVM)
• Arteriovenous malformations-cecum, ascending colon
• Angiography
o Cluster (tangle) of small arteries in arterial phase
4 o Early filling & delayed emptying of dilated veins
52
I PATHOLOGY
General Features
• Etiology
o Upper GI bleeding
• Duodenal ulcer (24%), gastric erosions (23%)
• Gastric ulcer (21 %), varices (10%)
• Mallory-Weiss tear (7%), esophagitis (6%)
• Neoplasm (3%), other causes (11 %)
o Lower GI bleeding
• Diverticulosis (43%), vascular ectasia (20%)
• Idiopathic (12%), neoplasia (9%)
• Colitis: Radiation (6%), ischemic (2%), ulcerative
(1%)
o Risk factors of upper GI bleeding
• Alcohol, tobacco, anticoagulants
• Aspirin, non-steroidal anti-inflammatory drugs
• Epidemiology
o More than 400,000 hospitalizations annually in US
o Lower GI bleeding
• Accounts for < 1% of all hospital admissions in US
• Annual incidence: 20.5/100,000 (M > F)
Gross Pathologic & Surgical Features
• Varies based on underlying pathology
Microscopic Features
• Varies depending on underlying cause
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Upper GI bleeding
• Hematemesis: Bloody vomitus; red, coffee grounds
• Melena: Black, tarry stools (100-200 mL of blood
in upper GI tract is required to produce melena)
o Lower GI bleeding
• Hematochezia: Red blood per rectum
• May also result from upper GI bleed (> 1000 cc)
BLEEDING
o Bleed anywhere in GI tract
• Occult blood detected by stool chemical testing
o Symptoms & signs of blood loss
• Dizziness, tachycardia, hypotension, shock
o Symptoms & signs of underlying pathology
• Lab data
o Fresh blood in vomitus or stool
o Occult blood in stool, iron deficiency anemia
o t CBC count, hematocrit, serum electrolytes
o Abnormal coagulation profile (am, PT, platelet
count, bleeding time)
o Serum blood urea nitrogen to creatine ratio> 25
• Suggests upper GI hemorrhage
Demographics
• Age: More common in older age group
• Gender: Males more than females (M > F)
Natural History & Prognosis
• Complications
o Acute massive GI bleeding: Shock & death
o Complications of underlying cause
• Prognosis
o Early detection, resuscitation & treatment: Good
o Delayed detection, resuscitation & treatment: Poor
o Mortality rate
• Upper GI bleeding: Esophageal varices (30-50%) &
varies based on Rockall risk score
• Lower GI bleeding: Ranges from 0-21 %
Treatment
• Medical: Resuscitation (fluids, electrolytes, blood)
• Endoscopic therapy
o. Topical: Tissue adhesives, collagen, clotting factors
o Injection: Sclerosant agents & vasoconstrictors
o Mechanical: Clips, balloons, sutures
o Thermal: Laser photo & electrocoagulation
• Transjugular intrahepatic porto systemic shunt (TIPS)
• Interventional: Embolotherapy (Gelfoam & coils)
• Surgical treatment
I DIAGNOSTIC CHECKLIST
Consider
• Scintigraphy for hemodynamically stable cases
• Angiography for hemodynamically unstable cases
I SELECTED REFERENCES
1. Tew K et al: MDCT of acute lower gastrointestinal bleeding.
AJRAm J Roentgeno!. 182(2):427-30,2004
2. Hastings GS: Angiographic localization and transcatheter
treatment of gastrointestinal bleeding. Radiographies.
20(4):1160-8,2000
3. Maurer AH et al: Effects of in vitro versus in vivo red cell
labeling on image quality in gastrointestinal bleeding
studies. J Nucl Med Techno!. 26:87-90, 1998
4. Whitaker SC et al: The role of angiography in the
investigation of acute or chronic gastrointestinal
hemorrhage. Clin Radio!. 47:382-8, 1993
s. Bunker SR: Cine scintigraphy of gastrointestinal bleeding.
Radiology. 187:877-8, 1993
 GASTROINTESTINAL BLEEDING
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
massive thickening of the
jejunal wall due to
spontaneous bleeding in an
anticoagulated patient.
(Right) Axial CECT in a
patient with abdominal pain
and CI bleeding following
heart transplantation shows
small bowel hematoma
(arrow) resulting in partialS8
obstruction.

4
Typical 53
(Left) Superior mesenteric
artery (SMA) arteriogram
shows a tangled cluster of
small vessels (arrow) in
cecum; angiodysplasia.
(Right) SMA arteriogram
shows early draining vein
(arrow) from angiodysplasia.

Typical
(Left) SMA arteriogram
shows enlarged early
draining vein (arrow) from
angiodysplasia and
premature filling of SM vein
(open arrow). (Right) SMA
arteriogram shows early
draining vein from
angiodysplasia and
premature filling of SM vein
(open arrow).
 INTRAMURAL BENIGN INTESTINAL TUMORS

Axial CECT shows fat density intramural/luminal mass Axial CECT shows duodenal teratoma (arrow) that is
(arrow); jejunal lipoma. comprised of fat, calcification, and soft tissue.

4
54
• Luminal protrusion & displaces adjacent bowel
ITERMINOlOGY • Smooth, oval/round luminal defects (endoenteric)
Definitions o Lipoma
• Benign mass composed of one or more tissue elements • Most common in ileum; sharply demarcated,
of the small bowel wall pedunculated mass conforms to bowel lumen
• Configuration changes in peristalsis/compression
o Hemangioma
I IMAGING FINDINGS • Millimeters in size; ± calcified phleboliths
• Multiple, intraluminal/intramural nodular defects
General Features
CT Findings
• Best diagnostic clue: Intramural mass with smooth,
oval or round luminal defects on barium studies • GIST
o Hypo-/hypervascular mass; ± calcification
• Other general features
o Enhancing mass/polyp with areas of low attenuation
o Types of intramural benign intestinal tumors
from hemorrhage, necrosis or cyst formation
• Gastrointestinal stromal tumor (GIST)
o ± Extends into mesentery & encases other structures
• Leiomyoma, lipoma, hemangioma
• Leiomyoma: 1-10 em sharply defined spherical mass
Radiographic Findings with homogeneous enhancement; ± focal calcification
• Fluoroscopic-guided barium studies • Lipoma: 1-6 em solitary mass with attenuation (-80 to
o GIST -120 HU) similar to fat; ± soft tissue stranding
• Most common; intestinal dilatation Imaging Recommendations
• Circumscribed, lobulated mass; few mm to 30 em
• Best imaging tool: Barium studies followed by CT
• Sharply defined margins; mucosal surface may
show luminal irregularity/focal ulceration (s 50%)
• Extraserosal component: Mass effect often large
• ± Cavity with fistula to intestinal lumen; irregular
I DIFFERENTIAL DIAGNOSIS
gas collection Intestinal metastases and lymphoma
o Leiomyoma • E.g., maligl}ant melanoma, lung, breast cancer

DDx: Focal Wall Thickening

Lymphoma Melanoma Hematoma

 INTRAMURAL BENIGN INTESTINAL TUMORS

Key Facts
Imaging Findings • Intestinal carcinoma
• Best diagnostic clue: Intramural mass with smooth, • Intramural hematoma
oval or round luminal defects on barium studies Diagnostic Checklist
• Sharply defined margins; mucosal surface may show • GIST is most common; imaging criteria to separate
luminal irregularity/focal ulceration (::; 50%) from other intramural tumors have not been
• Best imaging tool: Barium studies followed by CT established, except lipoma
Top Differential Diagnoses • Smooth intramural mass, may be "pulled" into lumen
• Intestinal metastases and lymphoma by peristalsis

• Lymphoma (non-Hodgkin): Large masses that ulcerate,

cavitate, extend into mesentery; ± lymphadenopathy
Intestinal carcinoma
• Adenocarcinoma: "Apple core" lesion; rigid stricture
• Carcinoid tumor: Crowded folds, bowel wall kinking,
luminal narrowing (mesenteric extension)
Intramural hematoma
• Jejunum (most common) > ileum
• Large, concentric submucosal filling defects
• Anticoagulation (10-35%), bleeding disorders, trauma
I PATHOLOGY
General Features
• General path comments
o Most diagnosed incidentally by imaging or autopsy
o GIST
• Immunoreactivity to c-KIT (CDl17)
• Most common intramural primary masses
• 20-30% of all GISTs occur in small intestine
• More aggressive than gastric GISTs with same size
• Etiology: GIST: KIT germ line mutations (52-85%)
• Associated abnormalities
o GIST:von-Recklinghausen disease
o Hemangioma: Cutaneous lesions, tuberous sclerosis,
Turner syndrome, Osler-Weber-Rendu disease
Gross Pathologic & Surgical Features
• GIST:Well-circumscribed mass compressing adjacent
tissue and lacks capsule; pink, tan or gray surface
• Leiomyoma: Endoenteric, exoenteric or bidirectional
• Lipoma: Well-circumscribed proliferation of fat
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic (most common)
o GI bleeding, intestinal obstruction, intussusception
o Nausea, vomiting, weight loss, palpable mass
o Hemangioma: 80% symptomatic; acute, severe &
intermittent GI bleeding (most common)
Demographics
• Age: > 45 years of age
• Gender: M = F
Natural History & Prognosis
• Complications: Intussusception, obstruction, bleeding
• Prognosis: Good, unless with recurrence or size> 5 cm
Treatment
• GIST
o Surgical resection ± chemotherapy (Gleevec) for
metastatic disease
4
• Other types of tumors: Usually no treatment 55
I DIAGNOSTIC CHECKLIST
Consider
• GIST is most common; imaging criteria to separate
from other intramural tumors have not been
established, except lipoma
Image Interpretation Pearls
• Smooth intramural mass, may be "pulled" into lumen
by peristalsis
I SELECTED REFERENCES
1. Levy AD et al: Gastrointestinal stromal tumors: radiologic
features with pathologic correlation. Radiographies.
23(2):283-304, 456; quiz 532, 2003
2. Laurent F et al: CT of small-bowel neoplasms. Semin
Ultrasound CT MR. 16(2):102-11, 1995
3. Solomon A et al: Computed tomographic investigation of
serosal and intramural gastrointestinal pathology.
Gastrointest Radiol. 12(1):13-7, 1987
I IMAGE GALLERY
(Left) Axial CECT shows large mass in medial wall of duodenum with
large central ulceration (arrow) (GIST). (Right) Upper GI series
shows large duodenal mass with central ulceration (arrow) (GIST).
 HAMARTOMATOUS POLYPOSIS (P-J)

Upper GI (UG/) series shows multiple polyps in Air-contrast BE shows numerous polyps (arrows) in a
duodenum in a patient with Peutz-jeghers syndrome. patient with Peutz-jeghers syndrome.

4
56
• Other general features
!TERMINOlOGY o Classification: Hamartomatous polyposis syndromes
Abbreviations and Synonyms • Peutz-Jeghers syndrome; MHS; JP; CCS
• Peutz-Jeghers syndrome (PJS) • Bannayan-Riley-Ruvalcaba syndrome
• Multiple hamartoma syndrome/Cowden disease (MHS) o PJS: Autosomal dominant, characterized by
• Juvenile polyposis OP) • Hamartomatous GI tract polyps; mucocutaneous
• Cronkhite-Canada syndrome (CCS) pigmentation of lips, oral mucosa, palms & soles
• Risk for cancer (10%): Stomach, duodenum, colon
Definitions • Extra-GIT cancers: Pancreas, breast, reproductive
• Spectrum of both hereditary & nonhereditary o MHS: Autosomal dominant (AD) genodermatosis
polyposis syndromes characterized by gastrointestinal • Mucocutaneous: Facial papules, oral papillomas,
tract (GIT) polyps & other associated lesions keratosis
• Breast: Fibrocystic (50%); ductal type cancer (30%)
• Thyroid (65%): Adenomas, goiter, follicular cancer
IIMAGING FINDINGS • Clinically: Bird-like face, high arched palate
o Juvenile polyposis OP): Sub classified into 2 types
General Features • Isolated juvenile polyps of childhood
• Best diagnostic clue: Cluster of small radiolucent • Juvenile polyposis of colon or entire GIT
filling defects in small bowel (PJS) o CCS: Inflammatory polyps + ectodermal defects
• Location
o Peutz-Jeghers syndrome Radiographic Findings
• Jejunum + ileum> duodenum> colon> stomach • Fluoroscopic guided double-contrast studies
o MHS + ]P: Most polyps in rectosigmoid colon o Multiple varied-size radiolucent filling defects
o CCS: Stomach-lOO%, colon-lOO%, small bowel-50% • Polyps in Peutz-Jeghers syndrome (P]S)
• Size: Varied size (small, medium, large) o Stomach to rectum (mouth & esophagus spared)
• Morphology o Small bowel (> 95%)
o Sessile or pedunculated polypoid lesions • Usually multiple & broad-based polyps
o Pattern: Carpet-like, cluster-like or scattered • PJS polyps occur in clusters> carpeting bowel
• Large polyps: Characteristic lobulated surface

DDx: Multiple Bowel Wall lesions

Gardner Syndrome Brunner Glands Lymphoid Follicles Lymphoma

 HAMARTOMATOUS POLYPOSIS (P-J)
Key Facts
Terminology
• Spectrum of both hereditary & nonhereditary
polyposis syndromes characterized by gastrointestinal
tract (GIT) polyps & other associated lesions
Imaging Findings
• Best diagnostic clue: Cluster of small radiolucent
filling defects in small bowel (PJS)
• Jejunum + ileum> duodenum> colon> stomach
• PJS polyps occur in clusters> carpeting bowel
Top Differential Diagnoses
• Familial adenomatous polyposis
• Brunner gland hyperplasia (hamartoma)
• Lymphoid follicles (hyperplasia)
• Metastases & lymphoma (GI tract)
Pathology
• Hereditary (AD): PJS, MHS, 25% of JP

o Colorectal: (30%): Multiple scattered; no carpeting • Lab-data: Hypochromic anemia; positive stool guaiac
o Stomach + duodenum (25%): Diffuse involvement test
Imaging Recommendations Demographics
• Double-contrast barium studies (multiple views) • Age: PJS (10-30); MHS (30-40); CCS (above 60) years
• Gender: PJS (M = F); MHS (M < F); JP (M > F); CCS (M <
F)
[DIFFERENTIAL DIAGNOSIS
Natural History & Prognosis
4
Familial adenomatous polyposis • Complications: Intussusception, SBO, cancer risk (PJS) 57
• 500-2,500 polyps carpeting colonic mucosa • Prognosis: Risk of cancer, 40% by 40 years of age
• Tubular or tubulovillous; colorectal cancer risk 100%
Treatment
Brunner gland hyperplasia (hamartoma) • Follow-up & surveillance; surgery in malignant cases
• Location: Duodenal bulb & descending part
• Hyperplasia: Multiple nodules (Swiss cheese pattern)
• Hamartomas: Simulate hamartomatous polyps I DIAGNOSTIC CHECKLIST
• Associated thickened, irregular folds differentiates
Image Interpretation Pearls
lymphoid follicles (hyperplasia) • PJS: Small bowel polyps, mucocutaneous pigmentation
• Innumerable small or tiny radiolucent nodules
• Usually generalized (duodenum, small bowel, colon)
• Distinguished by clinical history & generalized pattern I SELECTED REFERENCES
Metastases & lymphoma (GI tract) 1. Cho G] et al: Peutz-]eghers syndrome and hamartomatous
polyposis syndromes: Radiologic-pathologic correlation.
• Metastases: May be polypoid mimicking polyps
RadioGraphies. 17: 785-91, 1997
• Lymphoma: Small/bulky polypoid, mimicking polyps 2. Harned RK et al: The hamartomatous polyposis syndromes:
o Thickened bowel wall & folds; adenopathy seen Clinical and radiologic features. A]R. 164: 565-71, 1995
3. Buck]L et al: From the archives of the AFIP: Peutz-]eghers
syndrome. RadioGraphies. 12: 365-78, 1992
[PATHOLOGY
General Features I IMAGE GALLERY
• Genetics: Spontaneous mutation of gene on
chromosome 19 (PJS) & 10 (MHS)
• Etiology
o Hereditary (AD): PJS, MHS, 25% of JP
o Nonhereditary: CCS & 75% ofJP
• Epidemiology: PJS incidence: 1 in 10,000 people
Gross Pathologic & Surgical Features
• Sessile/pedunculated; carpet or cluster-like or scattered
Microscopic Features
• Extensive smooth muscle arborization of polyps

[CLINICAL ISSUES (Left) Axial CECT shows markedly dilated small bowel (58) due to
intussusception. (Right) Axial CECT in a 4 year old boy shows 58
Presentation intussusception (arrow) proved at surgery to be due to
hamartomatous polyps.
• Most common signs/symptoms: PJS: Pain,
mucocutaneous pigmentation, melena
 SMALL BOWEL CARCINOMA

Spot film from small bowel follow through (58FT) Spot film from 58FT shows jejunal mass (arrow) with
shows "apple core" stricture of terminal ileum (arrow) mucosal destruction and luminal narrowing.
and mass effect on cecum.
4
58
• Short, well-demarcated, circumferential narrowing
!TERMINOLOGY • Irregular lumen, overhanging edges, ± ulceration
Abbreviations and Synonyms • Narrow, rigid stricture with prestenotic dilatation
• Small bowel carcinoma (SBC) o Polypoid sessile tumor: Small plaque-like growth
o Pedunculated polypoid adenocarcinoma (rare)
Definitions
CT Findings
• Primary adenocarcinoma of small intestine
• Annular, ulcerative lesion or discrete nodular mass
• Circumferential thickened wall ± mesenteric invasion
IIMAGING FINDINGS • Soft tissue mass ± luminal narrowing & obstruction
• Heterogeneous density; ± enlarged mesenteric nodes
General Features • Growth shows moderate enhancement on CECT
• Best diagnostic clue: Annular or plaque-like polypoid • ± Metastases: Liver, peritoneal surfaces, ovaries
mass on enteroclysis Ultrasonographic Findings
• Location • Real Time: Thickened small bowel wall
o Proximal jejunum: Most common within first 30 cm
beyond ligament of Treitz Angiographic Findings
o Ileum: Less than 15% • Tumor that displaces feeding arteries
o Duodenum: Most common (> 50%) when included
in small bowel Imaging Recommendations
• Size: Tumor size varies from 3-8 cm in diameter • Best imaging tool: Enteroclysis; upper GI series & small
• Morphology bowel follow-through
o Infiltrating tumor
o Small plaque-like polypoid adenocarcinoma
o Pedunculated polyp I DIFFERENTIAL DIAGNOSIS
Radiographic Findings Lymphoma & leiomyosarcoma
• Fluoroscopic guided enteroclysis • Lymphoma
o Infiltrating tumor: "Apple-core" or annular lesion o Non-Hodgkin lymphoma most common

DDx: Small Intestinal Mass

Jejunal Lymphoma /leal Lymphoma Metastatic Melanoma Crohn Disease

 SMALL BOWEL CARCINOMA

Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Annular or plaque-like polypoid • Lymphoma & leiomyosarcoma
mass on enteroclysis • Metastases
• Proximal jejunum: Most common within first 30 cm • Carcinoid tumor
beyond ligament of Treitz • Crohn disease
• Circumferential thickened wall ± mesenteric invasion
• Soft tissue mass ± luminal narrowing & obstruction Diagnostic Checklist
• Heterogeneous density; ± enlarged mesenteric nodes • MDCT, 3D imaging in diagnosing, surgical planning
• ± Metastases: Liver, peritoneal surfaces, ovaries • Annular, discrete nodular or ulcerative lesion on CT

o Marked luminal dilatation is characteristic

o Nodular, polypoid, infiltrating, mesenteric invasive Natural History & Prognosis
• GI stromal tumor: Sharply defined, exophytic & bulky • Complications
o SBO; intussusception; GI bleed; perforation (rarely)
Metastases • Prognosis
• Usually from carcinoma of colon or melanoma o 5 year survival rate: Jejunum carcinoma - 46%; ileal-
• Lesions often longer, more pronounced narrowing & 20%
obstruction
• Ulceration tends to produce irregular cavity Treatment
4
• Surgical resection (localized); chemotherapy (spread) 59
Carcinoid tumor
• Malignant neuroendocrine tumor of small bowel
• Hypervascular submucosal mass; mesenteric invasion I DIAGNOSTIC CHECKLIST
• Mesenteric mass: Ca++ & desmoplastic reaction
• More common in ileum & displaces bowel loops Consider
• Ill-defined homogeneous mass, rarely annular lesion • MDCT, 3D imaging in diagnosing, surgical planning
Crohn disease Image Interpretation Pearls
• Usually distal ileum with long area of wall thickening • Annular, discrete nodular or ulcerative lesion on CT
• Aphthoid ulceration, cobblestone appearance • Infiltrating annular or polypoid lesion on enteroclysis
• Skip lesions, transmural, fistulae, sinuses, fissures

I PATHOLOGY
General Features
• Etiology: Most probably arise from adenoma
(adenoma-carcinoma sequence)
• Epidemiology: Prevalence, 0.5-3/100,000 population
• Associated abnormalities: Adult celiac disease, Crohn
disease, Peutz-Jeghers syndrome (1 incidence of SBC)
I SELECTED REFERENCES
1. Horton KM et al: Multidetector-row computed tomography
and 3-dimensional computed tomography imaging of
small bowel neoplasms: current concept in diagnosis. ]
Comput Assist Tomogr. 28(1):106-16, 2004
2. Buckley]A et al: CT evaluation of small bowel neoplasms:
spectrum of disease. Radiographies. 18(2):379-92, 1998
3. Laurent F et al: CT of small-bowel neoplasms. Semin
Ultrasound CT MR. 16(2):102-11, 1995

Gross Pathologic & Surgical Features

• Infiltrating or plaque-like polypoid tumor ± stricture I IMAGE GALLERY
Microscopic Features
• Moderate to well-differentiated neoplastic cells

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Symptomatic at the time of diagnosis
• Abdominal pain, obstruction or both (90%)
• Bleeding or anemia (50%)
o Small palpable abdominal mass (30%)
Demographics (Left) Axial CECT shows jejunal mass (arrow) with thickened wall.
(Right) Axial CECT shows jejunal mass (arrow) and regional
• Age: Usually seen in elder age group lymphadenopathy (open arrow).
• Gender: M > F
 CARCINOID TUMOR
Axial CECT shows stellate mesenteric mass (arrow) and
retraction of small bowel in right lower quadrant (RLQ).
4
60
!TERMINOLOGY
Abbreviations and Synonyms
• Gastrointestinal carcinoid (neuroendocrine tumor)
Definitions
• Primary malignant neoplasm of small bowel that arises
from enterochromaffin cells of Kulchitsky
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Solitary, well or ill-defined,
enhancing distal ileal mass + mesenteric infiltration
• Size: Varies from less than 1 cm to a few cm
• Other general features
o .Most common primary small bowel tumor beyond
ligament of Treitz
o Slow-growing tumors, but are potentially malignant
o 2nd most common small bowel malignancy after
adenocarcinoma
o 85% of all carcinoid tumors arise within GI tract
• Appendix (50%), incidental at appendectomy
• Small bowel (33%); gastric, colon & rectum (2%)
• 90% of small bowel carcinoids arise in distal ileum
o 15% of all carcinoids arise from pancreas, lungs,
biliary tree, liver, genitourinary tract & thymus
DDx: Mesenteric Mass +/- Small Bowel Abnormality
Lymphoma Metastasis
Axial CECT shows innumerable liver metastases in a
patient with "carcinoid syndrome".
o 30% of small bowel carcinoids are multiple
• Associated with other malignant neoplasm,
usually within GI tract in 29-53% of patients
o 40-80% of GIT carcinoids spread to mesentery
• Key concepts
o Carcinoid syndrome (metastatic spread to liver)
• Spectrum of symptoms (flushing, diarrhea,
asthma, pain, right heart failure)
• Often misdiagnosed for years
• Indicates hepatic metastases, usually from small
bowel tumor
o Symptoms require systemic circulation of secretory
factors produced by carcinoid
• Serotonin, histamine, dopamine, somatostatin
• Vasoactive intestinal polypeptide, substance P
Radiographic Findings
• Fluoroscopic guided small bowel series or enteroclysis
o Submucosal: Solitary/multiple, smooth filling defect
o Ulcerated submucosal tumor: "Target lesion"
o Thickening of wall & mucosal folds (extension)
o Mesenteric infiltration: Small bowel loops show
angulation, tethering, fixation & retraction
o Dilated & thickened bowel loops due to ischemia
CT Findings
• Submucosal tumors
o Solitary or multiple, well-defined enhancing lesion
Oesmoid (Gardner) Fibros. Mesenteritis
 CARCINOID TUMOR
Key Facts
Terminology
• Primary malignant neoplasm of small bowel that
arises from enterochromaffin cells of Kulchitsky
Imaging Findings
• Best diagnostic clue: Solitary, well or ill-defined,
enhancing distal ileal mass + mesenteric infiltration
• 90% of small bowel carcinoids arise in distal ileum
• Calcification within mesenteric mass (up to 70% of
cases)
Top Differential Diagnoses
• Small bowel metastases & lymphoma
• Desmoid tumor
• Hematoma
• Fibrosing mesenteritis
• Small bowel carcinoma
o Visualization of enhancing mural mass is better with
enteric water as contrast agent
• Mesenteric extension of tumor
o Ill-defined, heterogeneous mesenteric mass
o Calcification within mesenteric mass (up to 70% of
cases)
o Occasionally tumor may be of cystic density
o Tumor may show spiculation with a stellate pattern
o ± Tethering, fixation, retraction of small bowel loops
• Due to mesenteric fibrosis & desmoplastic reaction
o Desmoplastic reaction: "Finger-like" projections of
mass into adjacent mesentery
o ± Encasement & narrowing of mesenteric vessels
• Liver metastases
o Arterial phase: Intense enhancement (1 vascularity)
o Delayed imaging: Lesions may be isodense with liver
• Three-dimensional CT angiography
o Detects mesenteric mass & its relationship to vessels
o Shows encasement/occlusion of mesenteric vessels
o ± Bowel wall thickening & submucosal edema
• Due to ischemia of involved small bowel loops
o Small bowel mesenteric mass with calcification &
desmoplastic reaction favors carcinoid tumor
• Must be differentiated from treated lymphoma &
retractile mesenteritis due to similar CT findings
MR Findings
• Submucosal tumor
o Tl WI: Isointense to muscle
o T2WI: Hyperintense or isointense to muscle
o Tl C+: Homogeneous enhancement
• Bowel wall thickening
o Tl WI & T2WI: Isointense to muscle
o Tl C+: Shows enhancement
• Mesenteric extension of tumor
o TlWI & T2WI
• Mass & spiculation: Isointense to muscle
• Desmoplastic strands: Hypointense
• Calcification: Cannot be detected
o Tl C+: Shows intense enhancement
• Liver metastases
o Tl WI: Hypointense
o T2WI: Mild-moderately hyperintense
Pathology
• Epidemiology: Rare (accounts 2% of GI tract tumors)
• Cardiac abnormalities (incidence 60-70%)
Clinical ISsues
• Mostly asymptomatic
• Some patients symptomatic for 2-7 years before
diagnosis made
• Carcinoid syndrome: Episodic cutaneous flushing,
wheezing & diarrhea
• 1 Blood levels of serotonin or 5 hydroxy tryptophan
• 24-hour urine: Increased 5-HIAA levels (5x normal)
Diagnostic Checklist
• Enhancing submucosal mass in distal ileum
• Mesenteric, discrete soft-tissue mass with
calcification, desmoplastic reaction ± liver metastases
o Arterial phase: Homogeneous enhancement 4
o Portal venous phase: Isointense to liver
61 .
o Larger metastases: Heterogeneous enhancement
• Due to areas of necrosis
o Occasionally enhancement may be peripheral, with
progressive fill-in or delayed
Angiographic Findings
• Conventional
o Primary bowel mass: Focal blush of enhancement
o Mesenteric vessels, due to extension of tumor:
Retracted, beading, tortuous or occluded
o Liver metastases: Hypervascular
o Hormonal assays can be done by selective portal &
systemic venous blood sampling
Nuclear Medicine Findings
• In-llloctreotide or somatostatin receptor scintigraphy
o Positive for GI tract carcinoids & liver metastases
• Whole-body fluorine-18 dopa PET
o Detects primary tumor, nodal & distant organ
metastases by increased uptake
• l3lI-labeled MIBG
o 1 Uptake by GI tract, nodal & liver metastases
Imaging Recommendations
• Helical CECT with enteric water is best imaging
o 125 mL IV contrast at 4 mL/sec: Arterial (35 see) &
venous (70 see) delay scans through liver
• In-llloctreotide or somatostatin receptor scintigraphy
o Sensitivity (75%) & specificity (100%)
I DIFFERENTIAL DIAGNOSIS
Small bowel metastases & lymphoma
• Metastases: Recurrent colon cancer, mesothelioma,
ovarian cancer mimic mesenteric carcinoid tumor
• Lymphoma
o Most common tumor to involve mesentery is NHL
o Bulky mass that encases bowel in "sandwich sign"
o Associated retroperitoneal adenopathy confirms
 CARCINOID TUMOR
Desmoid tumor
• Well or ill-defined soft tissue mesenteric mass
• When at root of mesentery, mimics carcinoid
Hematoma
• Cause: Blunt trauma, excessive anticoagulation,
thrombocytopenia
• Acute hematoma
o Typically quite dense (50-60 HU)
o Focal or dispersed between leaves of mesentery
o Density j attaining of water HU by two weeks
o Old mesenteric hematoma may calcify (rare)
Fibrosing mesenteritis
• Mesentery becomes thickened & inflamed
• "Misty" mesentery appearance seen with halo of fat
surrounding mesenteric vessels
Small bowel carcinoma
• More common in jejunum than in ileum
4 • Spread to mesenteric nodes/liver mimicking carcinoid
62
I PATHOLOGY
General Features
• General path comments: Belong to tumors called
apudomas (amine precursor uptake & decarboxylation
tumors)
• Etiology: Malignant tumor of small bowel that arises
from enterochromaffin cells of Kulchitsky in crypts of
Lieberkuhn
• Epidemiology: Rare (accounts 2% of GI tract tumors)
• Associated abnormalities
o Cardiac abnormalities (incidence 60-70%)
• Pulmonary & tricuspid stenosis or insufficiency
• Enlargement of right heart & septal irregularities
o May be associated with other malignant neoplasms
Gross Pathologic & Surgical Features
• Firm, yellow, submucosal nodules
Microscopic Features
• Small round cells, round nucleus, clear cytoplasm
• Tumor infiltration along neurovascular bundles
• 70% of mesenteric infiltrated tumors show Ca++
• Desmoplastic reaction
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Mostly asymptomatic
o Some patients symptomatic for 2-7 years before
diagnosis made
o Carcinoid syndrome: Episodic cutaneous flushing,
wheezing & diarrhea
• Implies liver metastases with subsequent systemic
venous drainage of carcinoid secretory factors
o Other signs/symptoms
• Abdominal pain: Secondary to intestinal ischemia
• Right heart failure & murmurs (valvular defects)
• Lab-data
o 1 Blood levels of serotonin or 5 hydroxy tryptophan
o 24-hour urine: Increased 5-HIAA levels (5x normal)
Demographics
• Age: Most occur in 5th or 6th decade of life
• Gender: M:F = 2:1
Natural History & Prognosis
• GI carcinoid, no lymph node or liver metastases
o Excellent prognosis with surgical resection
• 5 year survival rate for small bowel carcinoids is 90%
• 5 year survival rate with hepatic metastases is 50%
Treatment
• Distal small bowel tumors: Surgical resection of bowel
& mesentery often with right hemicolectomy
• Proximal small bowel tumors
o Pancreaticoduodenectomy
• Liver metastases
o Palliative surgery of primary tumor often completed
o Localized to single segment/lobe: Surgical resection
o Chemoembolization; radiofrequency ablation
• Somatostatin analogue: Octreotide relieve symptoms
• Chemotherapy: No role, except in bone metastases
I DIAGNOSTIC CHECKLIST
Consider
• Carcinoid syndrome indicates liver metastases
Image Interpretation Pearls
• Enhancing submucosal mass in distal ileum
• Mesenteric, discrete soft tissue mass with calcification,
desmoplastic reaction ± liver metastases
I SELECTED REFERENCES
1. Horton KM et al: Multidetector-row computed tomography
and 3-dimensional computed tomography imaging of
small bowel neoplasms: current concept in diagnosis. J
Comput Assist Tomogr. 28(1):106-16, 2004
2. Horton KM et al: Carcinoid tumors of the small bowel: a
multitechnique imaging approach. AJRAm J Roentgenol.
182(3):559-67, 2004
3. Maccioni F et al: Magnetic resonance imaging of an ileal
carcinoid tumor. Correlation with CT and US. Clin
Imaging. 27(6):403-7, 2003
4. Sheth S et al: Mesenteric neoplasms: CT appearances of
primary and secondary tumors and differential diagnosis.
Radiographies. 23(2):457-73; quiz 535-6, 2003
5. Buckley JA et al: CT evaluation of small bowel neoplasms:
spectrum of disease. Radiographies. 18(2):379-92, 1998
6. Whitfill CH et al: Primary carcinoid of the duodenum:
detection and characterization by magnetic resonance
imaging. J Magn Reson Imaging. 8(5):1175-6, 1998
7. Mindelzun RE et al: The misty mesentery on CT:
Differential diagnosis. AJR 167:61-5, 1996
8. Laurent F et al: CT of small-bowel neoplasms. Semin
Ultrasound CT MR. 16(2):102-11, 1995
9. Pantongrag-Brown L et al: Calcification and fibrosis in
mesenteric carcinoid tumor: CT findings and pathologic
correlation. AjR Am J Roentgenol. 164(2):387-91, 1995
 CARCINOID TUMOR
I IMAGE GALL.ERY

(Left) Barium enema shows

mass effect on medial cecum
due to carcinoid of ileum.
(Right) Small bowel follow
through (sBFT) shows partial
sB obstruction, plus
angulation, spiculation and
narrowing of ileal loops.

4
Typical 63
(Left) Axial CECT (arterial
phase) shows multiple
hypervascular liver
metastases (arrows) that
could not be detected on
parenchymal phase images.
(Right) Axial CECT shows
mass in ileum and mesentery
(arrow).

Typical
(Left) Axial CECT shows
stellate mesenteric mass with
central calcification. (Right)
sBFT shows partial sB
obstruction with luminal
narrowing, mesenteric mass,
and thick angled sB folds.
 INTESTINAL METASTASES AND LYMPHOMA

Axial CECT shows massive 58 wall thickening of one
ileal segment with soft tissue density, due to lymphoma.
4
64
ITERMINOLOGY
Definitions
• Intestinal metastases from other primary cancer site
• Lymphoma: Malignant tumor of B-Iymphocytes
I IMAGING FINDINGS
General Features
• Best diagnostic clue: "Bullis-eye" or "target" lesions
0
• Sprue: Causes 1 intestinal lymphoma & carcinoma
• Intestinal metastases
o Usually incidental finding with known carcinoma
o Various forms of metastatic spread to intestine
• Intraperitoneal spread or seeding
• Hematogenous & lymphatic spread
• Direct extension from contiguous neoplasms
o Intraperitoneal spread
• E.g., primary mucinous tumors of ovary,
appendix, colon & breast cancer
• Due to natural flow & accumulation of ascitic
fluid within peritoneal recesses; influences serosal
implantation of cancer cells
• Common sites: Ileocecal region, small bowel
mesentery & posterior pelvic cul-de-sac
o Hematogenous spread
• E.g., melanoma (> common), lung, breast cancer
Axial CECT shows soft ussue mass in mesentery and
wall of colon due to lymphoma (post-transplant
Iymphoproliferative disorder).
• Reaches antimesenteric border of small bowel via
small mesenteric arterial branches
• Small bowel & mesentery most common sites of
GIT metastases from melanoma after lung & liver
• Malignant melanoma: At autopsy 35-58% of cases
• Lung cancer: At autopsy 11% (39% are large cell)
• Breast cancer: Stomach, duodenum & colon are
more often involved than mesenteric small bowel
• Breast, melanoma metastases may come to clinical
attention many years after primary tumor removal
o Lymphatic spread: E.g., colon, ovarian, breast, lung
cancer, carcinoid & melanoma
o Direct invasion
• Pancreatic cancer: 2nd & 3rd parts of duodenum
• Cecal & gynecologic malignancy: Distal ileum
• Intestinal lymphoma
o Most common malignant small bowel tumor
o Lymphoma accounts for one-half of all primary
malignant small bowel tumors
o Small bowel is 2nd most frequent site of GI tract
• Stomach (51%); small bowel (33%); colon (16%) &
esophagus « 1%)
• Ileum (51%); jejunum (47%) & duodenum (2%)
o More than 50% cases are primary, rest are secondary
o Majority, non-Hodgkin lymphoma (B-cell) origin
o Most small bowel non-Hodgkin lymphomas: High
grade of large cell or immunoblastic cell types
• 30-50% harbor disease in mesenteric lymph nodes

DDx: Multifocal Bowel Wall Thickening

Hematoma Vasculitis Crohn Disease Whipple Disease

 INTESTINAL METASTASES AND LYMPHOMA
Key Facts
Imaging Findings
• Best diagnostic clue: "Bull's-eye" or "target" lesions
• Solitary or multiple discrete submucosal masses
• Giant cavitated mass: Large barium collection
contiguous with lumen (melanoma, lymphoma)
• Direct invasion: Spiculated mucosal folds, nodular
mass effect, ulceration, obstruction, rarely fistula
• Lymphoma types: Infiltrative, polypoid, nodular,
endoexoenteric, mesenteric
• Infiltrating lymphoma (most frequent type)
• Circumferential thickening & effacement of folds
• Aneurysmal dilatation on antimesenteric border
(replacement of muscularis propria by lymphoma)
• "Sandwich sign": Mildly enhancing, multiple,
rounded masses encasing mesenteric vessels
(mesenteric lymphoma)
Radiographic Findings
• Fluoroscopic guided enteroclysis
o Intraperitoneal metastatic spread
• Pelvic ileal mass with fixation, deformity &
tethering along mesenteric border
• Multiple fixed segments of pelvic ileum + shallow
nodular indentations on mesenteric border
• Dilated jejunum & proximal ileum (obstruction)
o Malignant melanoma metastases
• Solitary or multiple discrete submucosal masses
• "Bull's-eye or "target" lesions: Centrally ulcerated
submucosal masses
• "Spoke-wheel" pattern: Radiating superficial
fissures from central ulcer
• Giant cavitated mass: Large barium collection
contiguous with lumen (melanoma, lymphoma)
• Small or large lobulated masses
• Nonobstructive, large intraluminal mass favors
melanoma metastasis
o Bronchogenic carcinoma metastases
• Solitary/multiple, flat/polypoid intramural masses
• Ulceration; narrowing, obstruction (desmoplastic)
• ± Localized extravasation or free perforation (due
to marked tendency to penetrate bowel wall)
o Breast carcinoma metastases
• Submucosal masses ± ulceration
• May be seen as multiple strictures
o Direct invasion: Spiculated mucosal folds, nodular
mass effect, ulceration, obstruction, rarely fistula
o Intestinal lymphoma (types)
• Lymphoma types: Infiltrative, polypoid, nodular,
endoexoenteric, mesenteric
o Infiltrating lymphoma (most frequent type)
• Circumferential thickening & effacement of folds
• Luminal dilatation, narrowing, stricture
• Aneurysmal dilatation on antimesenteric border
(replacement of muscularis propria by lymphoma)
o Polypoid lymphoma
• Single/multiple, mucosal/submucosal masses
• "Bull's-eye" lesion: Polypoid mass + ulceration
• Rare form: Lymphomatous polyposis (follicular
mantle cell origin)
Top Differential Diagnoses
• Hemorrhage
• Vasculitis (small intestine)
• Crohn disease
• Other inflammatory (Whipple disease)
• Opportunistic infection
Pathology
• Malignant melanoma; breast, lung, ovarian cancer
• Primary: Non-Hodgkin (most common)
• Secondary: Generalized lymphoma
Diagnostic Checklist
• Check for history of primary cancer or enteropathy
• Overlapping radiographic features of intestinal
metastases, lymphoma & primary carcinoma
• Imaging important to suggest & stage malignancy
o Nodular lymphoma 4
• Multiple small submucosal nodular defects
65
o Endoexoenteric (cavitary form): Localized
perforation into a sealed-off mesenteric space
• Barium extravasates into an exoenteric space
• Barium, air & debris-filled cavity along mesenteric
border of small bowel
• ± Ulcer, fistulae, aneurysmal dilatation
o Mesenteric lymphoma
• Displace, compress & obstruct small bowel loops
CT Findings
• Demonstration of lesions facilitated by negative
contrast agents (water or gas)
• Intestinal metastases
o Intraperitoneal metastatic spread
• Mesenteric tethering of terminal ileum in RLQ
• Enhancing focal masses within mesenteric leaves
• "Stellate" appearance: Mesenteric fat infiltration
• Ovarian carcinoma: Calcified mesenteric/omental
masses + large calcified primary pelvic mass
o Malignant melanoma
• "Bull's-eye" or "target" lesions (also seen in
lymphoma, Kaposi sarcoma, carcinoid tumor)
• Enhancing mural nodules protruding into lumen
or focal thickening of intestinal wall
• Enhancing masses within small bowel mesentery
• Lobulated submucosal or giant cavitated lesions
• Location: Distal small bowel (usually ileum)
o Bronchogenic carcinoma metastases
• Flat or polypoid intramural masses
• ± Ulceration; narrowing & obstruction
o Breast carcinoma metastases
• Submucosal masses or multiple strictures
o Direct invasion of duodenum, jejunum & ileum
• Pancreatic head carcinoma: Medial wall changes
in 2nd or 3rd parts of duodenum
• Cecal, gynecologic cancer: Distal ileal changes
• Intestinal lymphoma
o Infiltrating form (most common)
• Circumferential type: Sausage-shaped mass of
homogeneous density + minimal enhancement
• Aneurysmal dilatation on antimesenteric border
 INTESTINAL METASTASES AND LYMPHOMA
o Polypoid form
• "BullIs-eye" or "target" lesion: Mass + ulceration
o Mesenteric form
• "Sandwich sign": Mildly enhancing, multiple,
rounded masses encasing mesenteric vessels
(mesenteric lymphoma)
• Large, lobulated, "cake-like" heterogeneous mass +
areas of necrosis displacing small bowel loops
• Ill-defined mesenteric fat infiltration
• Retroperitoneal adenopathy favors lymphoma
Imaging Recommendations
• Helical CECT with negative enteric contrast agents
• Fluoroscopic guided enteroclysis
I DIFFERENTIAL DIAGNOSIS
Hemorrhage
• E.g., Coumadin, trauma
4 • Localized bleeding may be seen as intramural mass
Vasculitis (small intestine)
66 • Henoch-Schonlein purpura
o Triad: Palpable purpura, arthritis, abdominal pain
• Systemic lupus erythematosus (SLE)
o Segmental bowel lesions ~ necrosis & perforation
• Barium studies or CT of small bowel in vasculitis
o Extensive fold thickening + luminal narrowing
o May show thumbprinting on mesenteric border
o Intussusceptions may be seen in childhood purpura
Crohn disease
• Predominantly involves distal ileum
• Skip lesions, transmural, fistulae, fissures
• Aphthoid ulceration, cobbles toning, "string sign"
Other inflammatory (Whipple disease)
• Thickened proximal small bowel folds
• Micronodules in jejunum on enteroclysis
• Thickened mesentery & lymphadenopathy
Opportunistic infection
• Giardiasis
o Duodenum & jejunum
• Thickened irregular folds with hypermotility
• Luminal narrowing & increased secretions
o Ileum: Usually appears normal
• Mycobacterium avium-intracellulare (MAl)
o Small bowel shows thickened folds, fine nodularity
oCT: Low density (caseated) lymph nodes
• Cytomegalovirus (CMV)
o Often causes terminal ileitis in AIDS patients
o Thickened folds, spiculation, ulcers, narrow lumen
I PATHOLOGY
General Features
• Etiology
o Intestinal metastases
• Malignant melanoma; breast, lung, ovarian cancer
• Appendix, colon, pancreatic cancer; carcinoid
o Intestinal lymphoma
• Primary: Non-Hodgkin (most common)
• Secondary: Generalized lymphoma
• Enteropathy-associated lymphoma: Celiac disease
• Mediterranean type: Arabs, Middle Eastern Jews
• Burkitt lymphoma in children involve ileocecal
areas
• Epidemiology
o Metastases: Most common in melanoma & carcinoid
o Lymphoma: Most common small bowel neoplasm
• Associated abnormalities
o Primary carcinoma in intestinal metastases
o Generalized adenopathy in secondary lymphoma
Gross Pathologic & Surgical Features
• Solitary/multiple; polypoid, ulcerated, cavitated
Microscopic Features
• Metastases: Varies based on primary cancer
• Lymphoma: Lymphoepitheliallesions
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, pain, weight loss, palpable mass
o Malabsorption, diarrhea
o Acute abdomen: Obstruction, perforation
Natural History & Prognosis
• Complications: Bleeding, perforation, obstruction
• Prognosis: Poor
Treatment
• Chemotherapy & surgical resection of lesions causing
complications like obstruction & GI bleeding
I DIAGNOSTIC CHECKLIST
Consider
• Check for history of primary cancer or enteropathy
Image Interpretation Pearls
• Overlapping radiographic features of intestinal
metastases, lymphoma & primary carcinoma
• Imaging important to suggest & stage malignancy
I SELECTED REFERENCES
1. Horton KM et al: Multidetector-row computed tomography
and 3-dimensional computed tomography imaging of
small bowel neoplasms: current concept in diagnosis. J
Comput Assist Tomogr. 28(1):106-16, 2004
2. Sheth S et al: Mesenteric neoplasms: CT appearances of
primary and secondary tumors and differential diagnosis.
Radiographies. 23(2):457-73; quiz 535-6,2003
3. Buckley JA et al: CT evaluation of small bowel neoplasms:
spectrum of disease. Radiographies. 18(2):379-92, 1998
4. Balthazar EJ et al: CT of small-bowel lymphoma in
immunocompetent patients and patients with AIDS:
comparison of findings. AJRAm J Roentgenol.
168(3):675-80, 1997
5. Rubesin SE et al: Non-Hodgkin lymphoma of the small
intestine. Radiographies. 10(6):985-98, 1990
 INTESTINAL METASTASES AND LYMPHOMA
I IMAGE GALLERY
Typical
(Left) 58FT shows
aneurysmal dilation (arrow)
of the lumen of terminal
ileum along with mesenteric
mass effect (lymphoma).
(Right) 58FT shows diffuse
nodular fold thickening of
most of the 58 (lymphoma).

4
67
(Left) Axial CECT shows
partially necrotic mass that
envelopes, but does not
obstruct, multiple 58
segments (melanoma).
(Right) 58FT shows
aneurysmal dilation (arrow)
and mucosal destruction of
distal 58 segment
(melanoma).

(Left) Fluoroscopic spot film

from 58FT shows bull's-eye
lesion (arrow) due to
metastatic melanoma.
(Right) 58FT spot film shows
intramural mass (arrow) with
distorted mucosa, due to
melanoma.
 SMALL BOWEL OBSTRUCTION

Upright radiograph shows dilated small bowel with Supine radiograph shows dilated small bowel, no
air-fluidlevels;no colonic gas. colonic gas. Note surgicalclips in pelvis as clue to prior
surgery and probable adhesions.
4
68
ITERMINOLOGY o Simple: Sub-classified based on degree of obstruction
• Intermittent, incomplete or partial or low grade
Abbreviations and Synonyms obstruction (more common)
• Small bowel obstruction (SBO) • Prolonged, complete or high grade obstruction
o Complicated: Sub-classified into two types
Definitions • Closed-loop or incarcerated obstruction: Adhesive
• Obstruction or blockage of small bowel loops bands> internal or external hernia
• Strangulation: Most common cause of closed-loop
obstruction; indicates vascular compromise
I IMAGING FINDINGS o Major predisposing causes of intestinal obstruction
• SBO: Adhesions (75%); external hernia (10%);
General Features neoplasm (5%)
• Best diagnostic clue: Dilated small bowel loops with • Adhesions: Post surgery (> 80%), inflammation
air-fluid levels on upright film (15%) & congenital (5%)
• Size: Small bowel, proximal to obstruction: > 2.5 cm • Large bowel: Carcinoma (55%); volvulus (11%);
• Key concepts diverticulitis (9%)
o Most common causes o Clinical onset of bowel obstruction
• Adhesion & hernias (> 80% of all cases) • SBO: Acute in onset
o Accounts for 20% of surgical admissions of patients • Large bowel obstruction: Subacute or chronic
with acute abdomen o Transition zone between normal and abnormal
o Classification based on mechanism of obstruction bowel critical to define site and cause of obstruction
• Mechanical: Extrinsic, intrinsic or intraluminal
lesions Radiographic Findings
• Non-mechanical: Adynamic ileus; dynamic or • Radiography
spastic ileus (due to neuromuscular disturbances) o Need supine + upright or decubitus views
o Mechanical SBO is 4-5 times more common than o Dilated proximal small bowel loops with multiple
large bowel obstruction air-fluid levels & collapsed distal bowel
o SBO classified into two types: Simple & complicated • Pneumoperitoneum is a sign of bowel perforation

DDx: Small Bowel Distension

,.
'-4
"( ..~. l".' ~.."
LJI/~
.::>.,
r"{ , .

/.
<
.. ).
,"
_.3

- .
--
., '. t .

Aerophagia Ascites + Ileus Cystic Fibrosis

 SMALL BOWEL OBSTRUCTION
Key Facts
Imaging Findings
• "Small-bowel feces" sign: Gas bubbles mixed with
particulate matter in dilated loops proximal to SBO
• Dilated fluid-filled small-bowel loops (> 2.5 cm)
proximal to collapsed loops
• Gas filled bowel loops, mesenteric fat, vessels in
inguinal canal or other external hernia
• Thickened enhancing wall & luminal narrowing at
transition zone
• Intussusception: Target sign; sausage-shaped or
reniform mass
• ± Pneumatosis intestinalis; ± portomesenteric venous
gas (strangulation)
• Mesenteric vessels: Haziness, obliteration, congestion
or hemorrhage; ascites (strangulation)
o Radiography can "miss" SBO (fluid, distended bowel
not evident)
o "String of pearls"
• On supine radiographs
• Small air bubbles within fluid, distended bowel
• Fluoroscopic guided enteroclysis or small-bowel series
o Incomplete or partial or low grade obstruction
• Sufficient flow of contrast through point of
obstruction
o Complete or high grade obstruction
• Stasis or delay in flow of contrast beyond point of
obstruction
o Transitions in contrast column can define location
& degree of obstruction
CT Findings
• Dilated small bowel loops (> 2.5 cm) ± air-fluid levels
• "Small-bowel feces" sign: Gas bubbles mixed with
particulate matter in dilated loops proximal to SBO
o Less common but reliable indicator of SBO
• Extrinsic lesions
o Adhesions
• Dilated fluid-filled small-bowel loops (> 2.5 cm)
proximal to collapsed loops
• ± Transition zone, minimal mural thickening &
enhancement
• Uncomplicated adhesive bands: Typically
unidentified on CT (diagnosis of exclusion)
o Hernia
• Gas filled bowel loops, mesenteric fat, vessels in
inguinal canal or other external hernia
• Strangulated hernia: Thickened + 1 attenuation of
bowel wall
• Internal hernia: Cluster of dilated loops +
crowding or twisting of mesenteric vessels
o Peritoneal carcinomatosis: Omental masses; dilated
bowel loops; multiple transition zones
o Appendicitis: RLQ inflammatory mass, dilated loops,
fluid collection, abscess
o Diverticulitis
• Complicated: Abscess, peritonitis, obstruction,
dilated bowel loops
Top Differential Diagnoses
• Adynamic or paralytic ileus
• Aerophagia
• Ascites
• Cystic fibrosis (CF)
Pathology
• Most common: Adhesions (- 60%), hernias (15%), +
tumors (- 15%; metastases> primary tumor)
• Pathogenesis: Obstruction of small-bowel leads to
proximal dilatation due to accumulation of GI
secretions & swallowed air
Diagnostic Checklist
• CT best to determine presence, site, & cause of SBO +
any complications
o Intrinsic lesions: Adenocarcinoma, Crohn, TB, 4
radiation enteropathy
69
• Thickened enhancing wall & luminal narrowing at
transition zone
• Fluid & gas filled dilated bowel loops proximal to
collapsed loops
• Intussusception: Target sign; sausage-shaped or
reniform mass
o Intraluminal lesions: Gallstones, foreign bodies,
bezoars, ascaris worms
• Classic triad: Ectopic calcified stone, gas in GB or
biliary tree, obstruction (gallstone ileus)
• Bezoar: Intraluminal mass + air in interstices;
dilated fluid-filled loops
o Closed-loop obstruction: Obstruction at two points
+ involves mesentery
o Relatively little dilatation of bowel proximal to
closed loop obstruction
• Fluid distended bowel, minimal gas (closed-loop)
• Volvulus: C-shaped, U-shaped or "coffee bean"
configuration of bowel loop
• Stretched mesenteric vessels converging toward
site of torsion
• "Beak sign": Fusiform tapering at point of torsion
or obstruction (closed-loop)
• "Whirl sign": Due to tightly twisted mesentery
with volvulus
o Strangulating obstruction: Blood flow to obstructed
bowel is blocked
• "Target" or "halo" sign: Circumferentially
thickened bowel wall + 1 wall attenuation
• "Serrated beak sign": Twisting of bowel, mesenteric
edema, bowel wall thickening
• ± Pneumatosis intestinalis; ± portomesenteric
venous gas (strangulation)
• Absence or I or delayed bowel wall enhancement
in affected loops
• Mesenteric vessels: Haziness, obliteration,
congestion or hemorrhage; ascites (strangulation)
Imaging Recommendations
• Helical CECT: Acutely ill; suspected ischemia; history
of cancer or inflammatory bowel disease
 SMALL BOWEL OBSTRUCTION
o Accuracy (95%), specificity (96%) in high grade SBO
• Enteroclysis: Intermittent, chronic or low grade SBO
• Suspected perforation: Water soluble contrast agent
I DIFFERENTIAL DIAGNOSIS
Adynamic or paralytic ileus
• Etiology: Post-op, medications, post injury, ischemia
• Dilated small and large bowel loops with no transition
point, fluid levels seen but aperistaltic
• CT shows absence of obstruction
Aerophagia
• Excessive air swallowing associated with prominent
belching, flatulence & abdominal distention
• Air swallowing: Independent or with eating/drinking
• Etiology: Unknown; may be functional, behavioral,
neurological or psychiatric
• Small-bowel loops are dilated simulating SBO
o Associated gastric & colonic distension without
4 air-fluid levels differentiates from SBO
70 Ascites
• Pathologic accumulation of fluid in peritoneal cavity
• Plain abdominal film
o Medial displacement + collapse of ascending &
descending colon
o Separation & centralization of gas-filled small-bowel
loops may simulate small-bowel obstruction
Cystic fibrosis (CF)
• Small-bowel may be functionally obstructed due to
thick, viscous bowel contents
• CT findings
o Shows fatty replacement of pancreas often with
small-bowel feces sign (chronic low grade SBO)
I PATHOLOGY
General Features
• Etiology
o Most common: Adhesions (- 60%), hernias (15%), +
tumors (- 15%; metastases> primary tumor)
o Extrinsic: Adhesions; external & internal hernias,
tumor, abscess, aneurysm
o Intrinsic: Tumors, inflammatory, vascular
(ischemic), metabolic, radiation enteropathy
o Intraluminal: Gallstones, bezoars, foreign bodies,
ascaris worms
o Pathogenesis: Obstruction of small-bowel leads to
proximal dilatation due to accumulation of GI
secretions & swallowed air
• Bowel dilatation stimulates secretory activity
resulting in more fluid accumulation
• Epidemiology
o Incidence: 20% of "acute abdomen" presentations
o Mortality: Simple SBO 5-8%; strangulation 20-37%
Gross Pathologic & Surgical Features
• Dilated proximal & distal collapsed loop + transition
point; (dilated small-bowl more than 2.5 cm)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Variable from mild abdominal pain to vomiting,
constipation, fever & signs of acute abdomen
• Abdominal distention, tenderness, guarding
• Bowel sounds high pitched or absent (late sign)
Natural History & Prognosis
• Complications
o Bowel strangulation, infarction, gangrene,
perforation, peritonitis & sepsis
• Prognosis
o Simple obstruction (good); complicated (poor)
o Mortality 25%: Surgery postponed beyond 36 hrs
o Mortality I to 8%: Surgery performed within 36 hrs
o Mortality 100%: Untreated strangulated obstructions
Treatment
• Nasogastric suction, decompression, LV. fluids, NPO
• Incomplete or low grade SBO: Conservative treatment
• Complete or high grade SBO: Immediate surgery
I DIAGNOSTIC CHECKLIST
Consider
• CT best to determine presence, site, & cause of SBO +
any complications
• Difficult to distinguish partial + complete obstruction
by imaging alone
Image Interpretation Pearls
• Dilated small bowel loops with "small bowel feces"
sign on CT & "string of pearls" sign on supine film
I SELECTED REFERENCES
1. Khurana B et al: Bowel obstruction revealed by
multidetector CT. AJRAm J Roentgenol. 178(5):1139-44,
2002
2. Maglinte DD et al: Small bowel obstruction: Optimizing
radiologic investigation and nonsurgical management.
Radiology. 218: 39-46, 2001
3. Furukawa A et al: Helical CT in the diagnosis of small
bowel obstruction. Radiographies. 21(2):341-55, 2001
4. Caoili EM et al: CT of small bowel obstruction: Another
perspective using multi planar reformations. AJR.174:
993-8,2000
5. Nevitt PC: The string of pearls sign. Radiology.
214(1):157-8,2000
6. Maglinte DD et al: The role of radiology in the diagnosis of
small bowel obstruction. AJR. 168: 1171-80, 1997
7. Mayo-Smith WW et al: The CT small bowel feces sign:
description and clinical significance. Clin Radiol.
50(11):765-7, 1995
 SMALL BOWEL OBSTRUCTION

I IMAGE GALLERY
Typical
(Left) 58FT shows barium
distended proximal small
bowel, collapsed distal
bowel and colon. (Right)
Coronal reconstruction of
CECT shows diluted
fluid-filled small-bowel (not
evident on supine
radiographs). Note acutely
angulated distal 58 (arrow)
due to adhesions, which
caused 580.

4
Typical 71
(Left) Axial CECT shows
abrupt transition from dilated
to non-dilated bowel
(arrow). 580 due to
adhesions. (Right) Axial
CECT shows 5pigellian
hernia (arrow) with dilated
bowel leading into the
hernia, collapsed bowel
leaving

Typical
(Left) Closed loop
obstruction due to midgut
volvulus. CECT shows
fluid-distended small-bowel,
ascites, and twisting of the
root of the mesentery
(arrow). (Right) Closed loop
obstruction CECT shows
cluster of dilated
fluid-distended loops of
bowel (arrow), with
mesenteric infiltration, and
blurred engorged blood
vessels. Plain radiographs
were IInorma/".
 GALLSTONE ILEUS

Axial CECT shows gas in collapsed gallbladder; fistula to Axial CECT shows large gallstone (arrow) within dilated
duodenum (arrow). small bowel; bowel distal to gallstone is collapsed.

4
72
• Radiopaque gallstone surrounded by intestinal gas
ITERMINOlOGY in obstructed bowel loop
Abbreviations and Synonyms • Difficult visualization: Cholesterol; located over
• Gallstone Ileus (GSI) shadow of sacrum; obscured by dilated bowel
o Rigler triad present in only 38% of cases
Definitions o Change in position of previously identified gallstone
• Mechanical intestinal obstruction caused by impaction • Fluoroscopy
of one or more gallstones in intestine o Upper gastrointestinal series or barium enema
o Well-contained localized barium collection lateral to
first portion of duodenum
I IMAGING FINDINGS • Barium filled collapsed gallbladder, biliary ducts
o Fistulous communication: Cholecystoduodenal
General Features (60%); choledochoduodenal; cholecystocolic
• Best diagnostic clue: Small bowel obstruction + gas in • Choledochocolic; cholecystogastric
biliary tree + ectopic gallstone; (Rigler triad)
• Location
CT Findings
o Gallstone may be "hung up" at narrow portions • CT better reveals gallstone as cause of obstruction
• Duodenum; ligament of Treitz; ileocecal valve; o May see stone surrounded by intestinal gas in
sigmoid colon; any area of stricture obstructed bowel loop
• Size: Large gallstone; ~ 2.5 cm o Cholesterol stones are usually low density (near
water), but often calcified rim
Radiographic Findings • Will show collapsed gallbladder, pneumobilia
• Radiography
o Plain abdominal film
o Dilated proximal bowel I DIFFERENTIAL DIAGNOSIS
o Gas in shrunken gallbladder; bile ducts or both
• Gas in biliary tree: One of three cases; branching Intussusception
pattern; gas more prominent centrally • "Coiled spring appearance"; sausage-shaped mass
o Ectopic calcified gallstone (15-25%)

DDx: Small Bowel Obstruction Plus "Mass"

Intussusception Dropped Gallstone Pseudoobstruction

 GALLSTONE ILEUS

Key Facts
Terminology Top Differential Diagnoses
• Mechanical intestinal obstruction caused by • Intussusception
impaction of one or more gallstones in intestine • Dropped gallstone
• Small bowel tumor
Imaging Findings • Pseudoobstruction
• Best diagnostic clue: Small bowel obstruction + gas in
biliary tree + ectopic gallstone; (Rigler triad) Clinical Issues
• Age: Risk i with age; average 65-75 years

• Recurrence: 5-10% (additional silent proximal calculi)

Dropped gallstone
• In peritoneal cavity, dropped during laparoscopic
cholecystectomy
Small bowel tumor
• Leiomyoma: 1-4 cm range; lipoma; adenoma
Pseudoobstruction
• Intestinal neurological dysfunction
• May have laminated enteroliths simulating gallstones
Bowel ischemia
• Thickened wall, proximal bowel dilated
!PATHOLOGY
General Features
• Etiology
o GSI occurs in setting of chronic cholecystitis
o Following multiple ERCPs for biliary calculi
o Complication of endoscopic sphincterotomy for
large common bile duct stones
• Epidemiology
o 0.4-5% of all intestinal obstructions
o In < 1% of patients with cholelithiasis
• Although called ileus; actually mechanical obstruction
I CLINICAL ISSUES
• Prognosis: High mortality; operative mortality 19%
Treatment
• Surgical therapy to relieve bowel obstruction
• Cholecystectomy & biliary fistula excision; to prevent
recurrence
• Staged laparoscopic management of GSI & associated 4
cholecystoduodenal fistula; feasible & safe
73
I DIAGNOSTIC CHECKLIST
Consider
• Middle-aged or elderly female; recurrent episodes of
RUQ pain with most recent episode being more severe
& associated with prolonged vomiting
o With most recent episode being more severe &
associated with prolonged vomiting
• If barium studies are performed; attempt should be
made to identify biliary-enteric fistula
I SELECTED REFERENCES
1. Vaidya]S et al: Gallstone ileus. Lancet. 362(9390):1105,
2003
2. Lyburn ID et al: Gall-stone ileus: imaging features. Hasp
Med. 63(7):434-5, 2002
3. Gandhi A et al: Gallstone ileus following endoscopic
sphincterotomy. Br] Hasp Med. 54(5):229-30, 1995

Presentation
• Intermittent, acute colicky abdominal pain (20-30%); I IMAGE GALLERY
nausea, vomiting, fever, distension, obstipation
• In elderly females; frequently an underlying
pathological condition at site of obstruction in colon
• Delayed complication of ERCP; up to 2 months
• Small bowel obstruction following endoscopic
sphincterotomy for very large bile duct calculi
• Diagnosis: Frequently delayed or missed
Demographics
• Age: Risk i with age; average 65-75 years
• Gender: M:F = 1:4-7
Natural History & Prognosis
• Gallstone erodes inflamed gallbladder wall; passes into (Left) Axial CECT shows gas in thick-walled collapsed gallbladder and
gastrointestinal tract; causes bowel obstruction fistula (arrow) to duodenum. (Right) Axial CECT shows small bowel
o Usually erodes directly into duodenum obstruction with large laminated gallstone (arrow) "stuck" near
• Very large stones can pass into duodenum; apparently ileocecal valve.
after "unsuccessful" sphincterotomy
 MALABSORPTION CONDITIONS

Small bowel follow through (58FT) shows mild dilation 58FT shows dilation of 58 lumen, marked dilution and
of 58 lumen, dilution of barium, and nodular fold flocculation of the barium. Malabsorption related to IgA
thickening due to congenital IgA deficiency and deficiency.
4 giardiasis.

74
• Chronic pancreatitis, cholestasis, ileal resection
ITERMINOlOGY • Disaccharidase deficiency, Zollinger-Ellison
Definitions syndrome (ZES)
• Impaired intestinal absorption of dietary constituents o Malabsorption at mucosal level
• Celiac disease, Crohn, tropical sprue
• Short bowel syndrome, cystic fibrosis
I IMAGING FINDINGS • Eosinophilic gastroenteritis, Whipple disease
• Amyloidosis, hypogammaglobulinemia,
General Features mastocytosis
• Best diagnostic clue: Segmental dilatation & spasm of o Malassimilation
small-bowel with excess fluid + abnormal fold pattern • Primary & secondary lymphangiectasia
• Location • Abeta & hypobetalipoproteinemia
o Celiac disease: Proximal small-bowel o Malabsorption caused by bacterial overgrowth
o Tropical sprue: Entire small-bowel • Idiopathic pseudo-obstruction, systemic sclerosis
o Crohn: Usually terminal ileum • Multiple, large small-bowel diverticula
• Key concepts Radiographic Findings
o Hallmark: Steatorrhea (1 fecal fat excretion)
• Enteroclysis or small bowel follow through
• Normal: Less than 6 g/24 hrs
o Luminal dilatation
o Abnormal fecal excretion of fat, fat-soluble vitamins
• Usually more than 3 cm
(ADEK),proteins, carbohydrates, minerals & water
• May be segmental or uniform
o Celiac sprue: Most common small-bowel disease
o Changes in fold pattern
producing malabsorption
• Valvulae conniventes: Thickened (more than 2
o Classification of malabsorption
mm); uniform, irregular, distorted or nodular
• Maldigestion
• Increased, decreased or absent (varies by etiology)
• Malabsorption at mucosal level
o Bowel wall thickening
• Malassimilation
• Focal or diffuse thickening (> 1 cm)
• Malabsorption caused by bacterial overgrowth
o Mucosal nodulation & ulceration
o Maldigestion
• Nodulation: Diffuse, punctate or sand-like

DDx: Dilated Bowel, Excess Fluid

Celiac Sprue Crohn Disease Whipple Disease Giardiasis

 MALABSORPTION CONDITIONS
Key Facts
Terminology
• Impaired intestinal absorption of dietary constituents
Imaging Findings
• Best diagnostic clue: Segmental dilatation & spasm of
small-bowel with excess fluid + abnormal fold pattern
• Luminal dilatation
• Changes in fold pattern
• Bowel wall thickening
• Mucosal nodulation & ulceration
• Motility changes
• Luminal narrowing
• Increased amount of intestinal fluid
• Small-bowel intussusception (target lesion on CT)
• Mesenteric or retroperitoneal lymph nodes
• Mesenteric hypervascularity on contrast study
• Ulceration: Aphthoid or cobblestoning
o Motility changes
• Normal, short or long transit time in small-bowel
• Normal time: For barium to travel through
small-bowel to cecum can range from 1-2 hours
o Luminal narrowing
• Focal or generalized
o Increased amount of intestinal fluid
• Due to diminished absorption
• Due to increased secretions
o Multiple or large SBdiverticula
CT Findings
• Dilated, fluid-filled small-bowel loops
• Thickened bowel wall & mesentery
• Mucosal fold thickening
• Small-bowel intussusception (target lesion on CT)
• Discontinuous, asymmetric thickened bowel wall
• Mesenteric or retroperitoneal lymph nodes
• Mesenteric hypervascularity on contrast study
Imaging Recommendations
• Enteroclysis or small-bowel follow through; helical CT
I DIFFERENTIAL DIAGNOSIS
Celiac sprue
• Small bowel follow through
o Small bowel dilatation> 3 cm: Mid & distal jejunum
o Valvulae conniventes: May exhibit various patterns
• Normal: In most patients
• Reversed jejunoileal pattern: Decreased jejunal
folds & increased ileal folds
• Absence of valvulae: "Moulage sign" (cast)-
characteristic of sprue
• Thickening: Severe disease & hypoproteinemia
o "Colonization" of jejunum
• Loss of jejunal folds lead to colon-like
haustrations
o Transit time: Normal, short or long
• Enteroclysis: Facilitates diagnosis/exclusion of disease
o Jejunal folds
Top Differential Diagnoses
• Celiac sprue
• Crohn disease
• Whipple disease (intestinal lipodystrophy)
• Opportunistic infection
• Pancreatic disease
Pathology
• Celiac disease: Allergic, immunologic or toxic
reaction to gluten protein
Diagnostic Checklist
• Check history of small-bowel diseases & food allergies
• Dilated small-bowel with fold thickening, mucosal
nodularity or ulceration, motility changes & t fluid
• Imaging findings often suggest malabsorption;
specific diagnosis is difficult
• Decreased number of proximal jejunal folds « 3 4
inch); normally 5 or> folds/inch
• Increased separation or absence of folds 75
• Ileal appearance of jejunum
o Ileal folds
• Increased number of folds in distal ileum (4-6
inch); normally 2-4 foldS/inch
• Increased fold thickness (::=:: 1 mm): "Jejunization"
of ileum (seen in 78% cases)
• Diagnosis
o Duodenojejunal mucosal biopsy
o t IgA & IgM antigliadin antibodies
Crohn disease
• Predominantly involves distal ileum
o Can affect from mouth to anal canal
• Aphthoid ulceration, cobblestoning
• Skip lesions, transmural, string sign
• Fistulae, fissures, sinuses
• Diagnosis: Mucosal biopsy
Whipple disease (intestinal lipodystrophy)
• Caused by Tropheryma whippelii (PASpositive bacilli)
• Presents with chronic diarrhea & malabsorption
• Duodenum & proximal jejunum
o Dilated with thickened mucosal folds
o Micronodules: 1-2 mm; separation of loops
o Mesenteric lymphadenopathy
• Diagnosis: Mucosal biopsy
Opportunistic infection
• AIDS with giardiasis & cryptosporidiosis
• Giardiasis
o Giardia lamblia: A protozoan intestinal flagellate
o Cause of travelers diarrhea & enteritis in AIDS
o Clinical: Acute self-limited diarrhea, or may develop
chronic diarrhea, malabsorption & weight loss
o Duodenum & jejunum
• Thickened irregular folds with hypermotility
• Luminal narrowing & increased secretions
o Ileum: Usually appears normal
o Diagnosis: Mucosal biopsy; stool (ova/trophozoites)
• Cryptosporidiosis
o Most common cause of enteritis in AIDS patients
 MALABSORPTION CONDITIONS
o Severe cholera-like illness & malabsorption
• Diarrhea, crampy abdominal pain, vomiting I CLINICAL ISSUES
o Pathology: Mucosal damage Presentation
o Radiographic findings
• Most common signs/symptoms
• Duodenum & proximal jejunum: Thickened folds
o Diarrhea, steatorrhea, flatulence
• Distal loops show incomplete barium coating with
o Abdominal distension, weight loss, anemia
areas of flocculation
• Lab-data
o CT may reveal small « 0.5 cm) lymph nodes
o Decreased levels of vitamins (ADEK, B12)
Pancreatic disease o Decreased levels of iron, folates, albumin, bile salts
• Example: Zollinger-Ellison syndrome (ZES); cystic o Increased fecal fat (15-30 g/24 hrs)
fibrosis o Abnormal D-Xylose breath test: Bacterial overgrowth
• Zollinger-Ellison syndrome o Hydrogen test for lactase deficiency
o Stomach o Abnormal CBC & liver function tests
• Thickened folds, enlarged areae gastricae, erosions • Diagnosis: Mucosal biopsy & histology
o Duodenum & proximal jejunum Demographics
• Dilated, thickened nodular folds, erosions & ulcers
• Age: Any age group
o Large volume of fluid dilutes barium & compromises
mucosal coating Natural History & Prognosis
oCT: Shows other associated lesions • Complications
4 • Islet cell tumor: Gastrinoma (75% in pancreas)
• Hypervascular on contrast scan
o Micro or macrocytic anemia, bony changes
o Edema, ascites, failure to thrive
• Hypervascular metastases (common in liver) • Prognosis
76
o Diagnosis: Serum gastrin levels> 1000pg/ml o Malabsorption after treatment
• Cystic fibrosis • Celiac disease: Improvement within 48 hrs
o Autosomal recessive genetic disorder • Tropical sprue: 4-7 days
o Defect in CFTR gene on chromosome 7q • Crohn: 10-20% symptom free; 30-53% post
o Small-bowel barium study surgical recurrence
• Duodenum: Thickened or flattened folds, nodular
filling defects, sacculation along lateral border of Treatment
descending duodenum • Celiac: Gluten-free diet
• Ileum: Thickened folds + reticular mucosal pattern • Tropical sprue: Antibiotics
o CT will show fatty replacement of pancreas • Crohn disease
o Medical (steroids, azathioprine, mesalamine)
o Surgical resection
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
• Genetics: Celiac sprue- class II human leukocyte
antigens (HLA-DR3/HLA-DQw2) Consider
• Etiology • Check history of small-bowel diseases & food allergies
o Celiac disease: Allergic, immunologic or toxic Image Interpretation Pearls
reaction to gluten protein
• Dilated small-bowel with fold thickening, mucosal
o Tropical sprue: Unknown etiology; may be due to
nodularity or ulceration, motility changes & 1 fluid
enterotoxigenic E. coli
• Imaging findings often suggest malabsorption; specific
o Crohn disease: Genetic, environmental, infectious,
diagnosis is difficult
immunologic, psychologic
o Whipple disease: Tropheryma whippelii (bacilli)
o ZES: Gastrinoma ~ 1 gastric acid secretion ~ severe
peptic ulcer disease
I SELECTED REFERENCES
o Eosinophilic gastroenteritis (EGE): Self-limited 1. Farrell RJet al: Celiac sprue. N Engl] Med. 346(3):180-8,
disease; affect patients with allergic disorders 2002
2. Koch J et al: Small intestine pathogens in AIDS:
• Epidemiology: Celiac- 1 incidence Ireland & N. Europe
conventional and opportunistic. Gastrointest Endosc Clin
Gross Pathologic & Surgical Features N Am. 8(4):869-88, 1998
3. Antes G: Inflammatory disease of the small intestine and
• Varies by etiology colon: contrast enema and CT. Radiology. 38: 41-45,1998
• Celiac: Dilated, thickened bowel; reversal fold pattern 4. Horton KM et al: Uncommon inflammatory diseases of the
small bowel: CT findings. AJRAm J Roentgenol.
Microscopic Features
170(2):385-8, 1998
• Varies by etiology 5. Maglinte DD et al: Current status of small bowel
radiography. Abdom Imaging. 21(3):247-57, 1996
6. Rubesin SEet al: Small bowel malabsorption: clinical and
radiologic perspectives. How we see it. Radiology.
184(2):297-305, 1992
 MALABSORPTION CONDITIONS
I IMAGE GALLERY
Typical
(Left) 58FT shows numerous
large diverticula throughout
the jejunum which can lead
to stasis, bacterial
overgrowth and
malabsorption. (Right) 58FT
in patient with sprue shows
segmental dilation + spasm
of bowel + dilution +
flocculation of barium. Also
note transient
intussusception (arrow) of
jejunum with "coiled spring"
appearance.

4
Typical 77
(Left) 58FT shows nodular
58 fold pattern + dilated
lumen. Waldenstrom
macroglobulinemia. (Right)
Axial CECT shows 58 fold
thickening, excess fluid in
lumen, mesenteric_
engorgement due to primary
lymphangiectasia.

(Left) 58FT shows distorted

nodular 58 folds, diluted
barium.
Oysgammaglobuljnemia.
(Right) Enteroclysis shows
symmetrical fold thickening
due to hypoproteinemia.
 INTUSSUSCEPTION

Graphic shows ileocolic intussusception with a tumor in Axial CECT shows ileocolic intussusception. The outer
the bowel wall as the "lead mass". Note vascular ring (arrow) is the wall of the intussuscipiens (colon,
compromise and ischemia. here) while the intussusceptum is the small intestinal
4 segment (open arrow).

78
ITERMINOLOGY
Definitions
• Invagination or telescoping of a proximal segment of
bowel (intussusceptum) into lumen of a distal segment
(intussuscipiens)
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Bowel within bowel, "coiled
spring" appearance
• Location: Ileoileal > ileocolic> colocolic
• Key concepts
o Rarely symptomatic in adults: 0.003-0.02% of all
hospital admissions
o Adults: A different entity than in children
o Accounts for 5% of all intussusceptions & 1% of all
bowel obstructions
o May be transient or persistent
o Classified into two types in adults
• Short-segment, non-obstructing intussusception:
Usually self-limited without a lead mass
(idiopathic, adhesions, bowel wall thickening)
• Long-segment, obstructing intussusception: Mass
o Small-bowel: Benign tumors more common than
malignant
o Colon: Malignant tumors more common than
benign
o Infants & children
• Accounts for 95% of all intussusceptions
• 90% of cases, cause is idiopathic (lymphoid
hyperplasia)
• Ranks 2nd to appendicitis in children as a cause of
acute abdomen
• Location: Usually small bowel in adults; ileocolic
in children
o Imaging findings (barium studies & CT) are
pathognomonic for intussusception
Radiographic Findings
• Radiography
o Findings of bowel obstruction may be seen
• Air-fluid levels; proximal bowel dilatation
• Absence of gas in distal collapsed bowel
• Fluoroscopic guided barium study
o Classic "coiled spring" appearance
• Due to trapping of contrast between folds of
intussusceptum & intussuscipiens
o Bowel obstruction, proximal dilatation & distal
collapsed loops
CT Findings
• Seen as three different patterns on axial CT scans
o "Target" sign: Earliest stage of intussusception
• Outer layer represents intussuscipiens

DDx: Bowel Obstruction with Mass

Carcinoma Melanoma Meckel Diverticulum

 INTUSSUSCEPTION
Key Facts
Terminology
• Invagination or telescoping of a proximal segment of
bowel (intussusceptum) into lumen of a distal
segment (intussuscipiens)
Imaging Findings
• Best diagnostic clue: Bowel within bowel, "coiled
spring" appearance
• Location: Ileoileal > ileocolic> colocolic
• Short-segment, non-obstructing intussusception:
Usually self-limited without a lead mass (idiopathic,
adhesions, bowel wall thickening)
• Long-segment, obstructing intussusception: Mass
• "Target" sign: Earliest stage of intussusception
• Sausage-shaped mass: A layering pattern (later phase)
• Inner layer represents intussusceptum
o Sausage-shaped mass: A layering pattern (later
phase)
• Alternating layers of low-attenuation (mesenteric
fat) & high-attenuation areas (bowel wall)
• Enhancing mesenteric vessels
o Reniform mass: Due to edema or mural thickening
(vascular compromise)
• Vascular compromise: Seen in returning wall of
intussusceptum as hypodense layer in middle of
inner part of thick bowel wall & crescent-shaped
fluid or gas collections
o Features of intestinal obstruction
• Air-fluid levels; proximal bowel distension
MR Findings
• Bowel-within-bowel or coiled-spring appearance
• Best seen on turbo spin-echo T2WI
Ultrasonographic Findings
• Real Time
o Transverse US: Target, doughnut or "bull's eye" sign
• Peripheral hypoechoic halo: Edematous wall of
intussuscipiens
• Intermediate hyperechoic area: Space between
intussuscipiens & intussusceptum
• Internal hypoechoic ring: Due to intussusceptum
o Longitudinal US: "Pseudokidney" or hay fork sign
• Multiple, thin, parallel, hypoechoic & echogenic
stripes
• Color Doppler: Shows mesenteric vessels dragged
between entering and returning wall of
intussusceptum
Imaging Recommendations
• Depends on patient age & presentation
• Helical CT; barium studies; US
I DIFFERENTIAL DIAGNOSIS
Primary bowel tumor
• Example: Carcinoid tumor, adenocarcinoma, stromal
tumor, lipoma & adenoma
• Reniform mass: Due to edema or mural thickening
(vascular compromise)
Top Differential Diagnoses
• Primary bowel tumor
• Metastases & lymphoma
• Endometrial implant
• Meckel diverticulum
Pathology
• Tumor related lead point: Benign & malignant
Diagnostic Checklist
• Short segment, non-obstructing intussusceptions are
common in adults + require no therapy
• "Coiled spring" appearance due to trapped barium
• Lead point: Lobulated mass etched in white
• Enteroclysis 4
o Best technique for detecting mass
79
Metastases & lymphoma
• Metastases (small bowel)
o Example: From malignant melanoma, lung & breast
cancer
o Location: Antimesenteric border
o Malignant melanoma
• Smoothly polypoid lesions of different sizes
• "Spoke-wheel" pattern: Polypoid lesion with ulcers
& radiating folds
o Bronchogenic carcinoma
• Single/multiple intramural lesions (flat/polypoid)
• Frequently ulcerated, narrowing & obstruction
o Breast carcinoma
• Highly cellular submucosal masses
• Multiple strictures + intervening bowel dilatation
• Intussusception of ulcerated mural lesions
• Non-Hodgkin lymphoma (more common)
o Distribution: Stomach (51%), small-bowel (33%)
o Nodular, polypoid, infiltrating, mesenteric invasive
o Focal infiltration: Sausage-shaped thickening of
affected bowel wall simulating intussusception
o May cause intussusception
Endometrial implant
• Endometrial tissue outside the myometrium
• Common location: Pelvic organs
o Bowel is involved in 37% cases: Rectosigmoid (95%);
small-bowel (7%) predominantly terminal ileum
• Crenulation of folds or plaque-like deformities
• High grade or low grade small-bowel obstruction
o Usually due to fibrosis & rarely intussusception
• Diagnosis: Laparoscopy
Meckel diverticulum
• Most frequent congenital anomaly of GI tract
• Ileal outpouching (2 feet from ileocecal valve)
• Causes of small-bowel obstruction
o Torsion associated with a persistent vitelline band
o Extrusion of diverticulum into an inguinal hernia
(hernia of Littre)
o Intussusception of an inverted diverticulum
 INTUSSUSCEPTION
I PATHOLOGY • Severe vascular compromise
• Gangrene & perforation
General Features
Treatment
• Etiology
• None for transient, non-obstructing
o Most adult intussusceptions are short segment,
• Ileocolic, ileocecocolic & colocolic: Resection
transient, non-obstructing + not associated with a
• Children
lead tumor mass
o Hydrostatic or pneumatic reduction
o Tumor related lead point: Benign & malignant
o Surgical reduction or resection
o Benign
• Polyp, leiomyoma, lipoma, adenoma of appendix
• Appendiceal stump granuloma (> common in
small bowel)
I DIAGNOSTIC CHECKLIST
o Malignant Consider
• Primary (more common in colon) • Short segment, non-obstructing intussusceptions are
• Metastases & lymphoma (> common-small bowel) common in adults + require no therapy
o Postoperative: Risk factors (> common-small bowel)
• Suture lines, ostomy closure sites Image Interpretation Pearls
• Adhesions, long intestinal tubes • Lumen of intussusceptum as a narrow, tubular
• Bypassed intestinal segments, submucosal edema structure lined by twisted mucosal folds
• Abnormal bowel motility, electrolyte imbalance • "Coiled spring" appearance due to trapped barium
4 • Chronic dilated loop • Lead point: Lobulated mass etched in white
o Miscellaneous
80 • Meckel diverticulum; celiac & Whipple disease
• Colitis (eosinophilic & pseudomembranous) I SELECTED REFERENCES
• Epiploic appendagitis 1. Huang BYet al: Adult intussusception: diagnosis and
o Idiopathic clinical relevance. Radiol Clin North Am. 41(6):1137-51,
• Epidemiology 2003
o Incidence 2. Lvoff Net al: Distinguishing features of self-limiting adult
• Adults (uncommon); children (more common) small-bowel intussusception identified at CT. Radiology.
227(1):68-72, 2003
Gross Pathologic & Surgical Features 3. Saenz De Ormijana] et al: Idiopathic enteroenteric
• Three layers are seen intussusceptions in adults. Abdom Imaging. 28(1):8-11,
o Intussusceptum: Entering or inner tube + returning 2003
or middle tube 4. Gayer G et al: Pictorial review: adult intussusception--a CT
diagnosis. Br] Radiol. 75(890):185-90, 2002
o Intussuscipiens: Sheath or outer tube 5. Fujimoto T et al: Unenhanced CT findings of vascular
Microscopic Features compromise in association with intussusceptions in adults.
A]R Am] Roentgenol. 176(5):1167-71,2001
• Early: Inflammatory changes; late-ischemic necrosis + 6. Warshauer DM et al: Adult intussusception detected at CT
mucosal sloughing or MR imaging: clinical-imaging correlation. Radiology.
212(3):853-60, 1999
7. Catalano 0: Transient small bowel intussusception: CT
I CLINICAL ISSUES findings in adults. Br] Radiol. 70(836):805-8, 1997
8. Lorigan]G et al: The computed tomographic appearances
Presentation and clinical significance of intussusception in adults with
• Most common signs/symptoms malignant neoplasms. Br] Radiol. 63(748):257-62, 1990
9. Merine D et al: Enteroenteric intussusception: CT findings
o Adults
in nine patients. A]R Am] Roentgenol. 148(6):1129-32,
• Intermittent pain, vomiting, red blood in stool 1987
o Children
• Acute pain, palpable oblong mass in abdomen
• "Red currant jelly" stools
Demographics
• Age: Any age group
• Gender: M = F
Natural History & Prognosis
• Complications
o Obstruction, infarction & necrosis
o Hemorrhage, perforation & peritonitis
• Prognosis
o Early: Good
• After reduction, surgical resection
• Recurrence very rare
o Late: Poor
 INTUSSUSCEPTION

I IMAGE GALLERY
Typical
(Left) 58FT shows "coiled
spring" + "bowel-in-bowel"
appearance of 58
intussusception. Lead mass
was melanoma metastatic to
bowel wall. (Right) Axial
NECT following 58FT in
patient with metastatic
melanoma shows
bowel-in-bowel appearance
(arrow) of intussusception.

4
81
Typical
(Left) Axial CECT in a patient
with cystic fibrosis shows a
short segment,
non-obstructing 5B
intussusception (arrow).
(Right) Axial CECT shows 5B
intussusception (arrow) with
crescent of mesenteric fat
accompanying the
intussusceptum. Bowel
lumen is dilated due to
sprue, not obstruction.

Typical
(Left) Axial CECT shows
sausage-like mass within the
lumen of the terminal ileum
due to inverted,
intussuscepting Meckel
diverticulum. (Right) Axial
CECT shows "reniform"
(kidney-shaped) small bowel
(arrow) due to jejunal
intussusception.
 RADIATION ENTERITIS

Enteroclysis shows fixed segments of 5B luminal Air-contrast BE shows stricture of sigmoid colon
narrowing + fold distortion (arrows) due to radiation following radiation therapy for prostate cancer. Mucosa
treatment for cervical cancer. is intact.
4
82
ITERMINOlOGY • Bowel loops appear spastic (j lumen diameter)
with thickened folds (edema)
Definitions o Chronic radiation enteritis
• Damage of small bowel mucosa + wall due to • Thickened valvulae conniventes and intestinal
therapeutic or excessive abdominal irradiation wall (edema or fibrosis)
• Thickened folds appear straight and parallel
• "Stack of coins" appearance: Enlarged smooth,
I IMAGING FINDINGS straight, parallel folds perpendicular to the
longitudinal axis of small bowel (submucosal
General Features hemorrhage)
• Best diagnostic clue: Mural thickening and luminal • Spiky appearance: Barium trapped between
narrowing of pelvic bowel loops thickened folds (in profile view); folds are
• Location thickened and held closely to one another, where
o Small bowel (ileum more common than jejunum) thickness exceeds the distance between folds
o Adjacent colon (radiation colitis) and rectum • Narrowed or stenotic lumen ~ small bowel
(radiation proctitis) obstruction with dilation of proximal bowel loops
• Key concepts • Single or multiple stenoses (stricture) of varying
o Permanent radiographic findings: 1 month to 2 length (up to several cm)
years after radiation therapy • Adhesions ~ angulation between adjacent loops,
o Classification of radiation enteritis fixation of loops or "mucosal tacking"
• Acute stage: Concurrent with or < 2 months after • "Mucosal tacking": Angulation, spiking and
treatment distortion of the mucosal folds on antimesenteric
• Subacute stage: 2-12 months after treatment border; usually seen in terminal ileum and
• Chronic stage: > 12 months after treatment adjacent bowel loops
• Peristaltic activity is j or absent
Radiographic Findings • ± Large, deep ulcers; difficult to detect shallow
• Fluoroscopic-guided enteroclysis ulcers
o Acute radiation enteritis

DDx: long Segment luminal Narrowing

Crohn Disease Lymphoma Ischemia Colon Carcinoma

 RADIATION
Key Facts
Terminology
• Damage of small bowel mucosa + wall due to
therapeutic or excessive abdominal irradiation
Imaging Findings
• Best diagnostic clue: Mural thickening and luminal
narrowing of pelvic bowel loops
• Permanent radiographic findings: 1 month to 2 years
after radiation therapy
• Thickened folds appear straight and parallel
• Narrowed or stenotic lumen ~ small bowel
obstruction with dilation of proximal bowel loops
• Adhesions ~ angulation between adjacent loops,
fixation of loops or "mucosal tacking"
• Peristaltic activity is ! or absent
• Sinuses and fistulas (especially at damaged,
surgical anastomotic site caused by radiation)
• Effacement of valvulae conniventes (late, atrophic
feature)
• Fluoroscopic-guided barium enema
o Acute radiation colitis and proctitis
• ± Disrupted or distorted mucosal pattern (edema
or hemorrhage)
o Chronic radiation colitis and proctitis
• Diffuse or focal narrowing with tapered margins
• Rectal stricture or rectovaginal fistula can be seen
• Presacral space widened (in lateral view)
CT Findings
• Bowel wall thickening, luminal narrowing
• Fibrosis surrounding the bowel ± strictures
• ± Small bowel obstruction with multiple air-fluid
levels and dilation of proximal bowel loops
• ± Sinuses or fistulas
MR Findings
• T2WI
o Thick, high signal intensity layer suggests
submucosal edema; not tumor invasion
o "Bull's eye" pattern: Thickened high signal intensity
submucosa surrounded by the low signal intensity
muscularis propria and muscularis mucosae
o ± Bowel fistula: Fluid in tract appears as high signal,
contrasted by soft tissue and fat
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided enteroclysis;
fluoroscopic-guided barium enema; helical CT
I DIFFERENTIAL DIAGNOSIS
Crohn disease
• Skip lesions, transmural inflammation, granulomas,
cobblestone mucosa and fistulas
• Differentiate by irregular thickening, fused and
distorted folds; more prominent mural thickening
ENTERITIS
• Best imaging tool: Fluoroscopic-guided enteroclysis;
fluoroscopic-guided barium enema; helical CT
Top Differential Diagnoses
• Crohn disease
• Metastases and lymphoma
• Ischemic enteritis
Clinical Issues
• Colicky abdominal pain, nausea, vomiting, tenesmus,
bloody diarrhea, steatorrhea (10-40 g per day) and
weight loss
Diagnostic Checklist
• History of radiation therapy
• Small bowel obstruction; wall thickening with fixed
and angulated loops of bowel; reduced peristalsis
4
Metastases and lymphoma
• Metastases (e.g., malignant melanoma, breast cancer) 83
o Cause small bowel obstruction and narrowing
o Irradiation of abdomen is a treatment modality
• Difficult to differentiate radiologically because
both can coexist
• Interval growth or biopsy diagnostic
• Non-Hodgkin lymphoma
o Distribution: Small bowel (33%) and colon (16%)
o Infiltrative form appears similar to radiation enteritis
• Circumferential infiltration involves variable
length of small intestine ~ thickening ~
effacement of folds
o However, non-Hodgkin lymphoma rarely has same
radiologic features
• Widened lumen of bowel and rare stricture
formation
• Other classic features include multiple nodular
defects, polypoid and mesenteric invasive form
• CT: Demonstration of bulky mesenteric node
involvement
Ischemic enteritis
• Ischemic changes of small bowel ~ small bowel
obstruction mimicking radiation enteritis
• Ischemia and radiation can both cause submucosal
hemorrhage ~ "stack of coins" appearance
• Distinguish by observing clot or narrowing of superior
mesenteric artery, superior mesenteric vein or other
mesenteric vessels
Primary bowel tumor
• Can cause irregular stricture
• Usually more mass effect than with radiation enteritis
I PATHOLOGY
General Features
• General path comments
o Acute stage
• Mucosa: Thinning (reduction of crypt cell mitoses)
and edema
 RADIATION ENTERITIS
• Submucosa: Edema
• Hyperemia and ulceration
o Subacute stage
• Mucosa: Process of healing
• Submucosa: Obliterative changes in arterioles and
fibrotic thickening
o Chronic stage
• Muscularis propria: Fibrosis
• Serosa: Diffuse hyaline change ~ adhesions
between bowel loops
• Ulceration and fibrosis ~ strictures
o Radiation tolerance
• Duodenum, jejunum, ileum, transverse colon,
sigmoid colon, esophagus and rectum (from
highest to lowest tolerance)
• Tolerance dose (TD 5/5): Total dose that produce
radiation damage in 5% of patients within 5 years
• TD 5/5: 4,500 cGy (1 centigray = 1 rad) in small
bowel and colon; 5,000 cGy in rectum
• Etiology
4 o Radiation therapy for cancer of cervix, uterus, ovary,
cecum, colon, rectum and bladder
84 o Risk factors of chronic radiation enteritis
• Prior abdominal surgery ~ adhesions
• Peritonitis prior to radiation treatment
• High radiation dose over a short period of time
• Hypertension, atherosclerosis or diabetes mellitus
• Chemotherapy given with radiation ~ increase
radiation damage
o Pathogenesis (2 methods)
• Direct cytotoxic effect: Radiation ~ free radicals
interact with DNA ~ elimination of replication,
transcription and protein synthesis ~ cell
disruption and death
• Ischemic changes: Medial wall thickening and
subendothelial proliferation ~ endarteritis
obliterans ~ fibrosis
• Epidemiology
o 5-15% treated with radiation will develop chronic
radiation enteritis
o Radiation enteritis: Usually within 12 years after
radiation therapy (can occur during radiation
treatment, but up to 25 years latency)
o Radiation colitis: Within 2 years after radiation
Gross Pathologic & Surgical Features
• Thickened folds, ulceration, stricture or adhesions
Microscopic Features
• Shortening of villi, inflammatory cells and
megalocytosis of epithelial cells can be seen
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute
• Abdominal cramping, nausea, vomiting, tenesmus
and watery diarrhea
• Dehydration and malabsorption (change in small
bowel flora)
• Severity of symptoms proportional to dose and
volume of irradiation
• Radiation proctitis: Mucoid rectal discharge, rectal
pain and rectal bleeding
o Chronic
• Colicky abdominal pain, nausea, vomiting,
tenesmus, bloody diarrhea, steatorrhea (10-40 g
per day) and weight loss
• Lab-data
o Hypocalcemia; I iron and vitamin B121evels
• Diagnosis
o Radiation enteritis: Radiologic appearances on
fluoroscopic-guided enteroclysis and helical CT
o Radiation colitis: Radiologic appearances on
fluoroscopic-guided barium enema and helical CT
Demographics
• Gender: M < F
Natural History & Prognosis
• Complications
o Fistula, stricture, small bowel obstruction,
hemorrhage, abscess and perforation
• Prognosis
o Good, after medical treatment with reduction or
cessation of radiation
o Poor, if patients have chronic radiation injury with
complications
Treatment
• Reduction or cessation of radiation; medical treatment
and low-residue diet
• Surgery indicated when medical treatment fails
I DIAGNOSTIC CHECKLIST
Consider
• Rule out recurrent tumors
• History of radiation therapy
o Dose rate, fractionation of therapeutic doses, total
dose, radiation portal, type of radiation and length
of time after radiation therapy
Image Interpretation Pearls
• Small bowel obstruction; wall thickening with fixed
and angulated loops of bowel; reduced peristalsis
I SELECTED REFERENCES
1. Low RN et al: Distinguishing benign from malignant bowel
obstruction in patients with malignancy: findings at MR
imaging. Radiology. 228(1):157-65, 2003
2. Bismar MM et al: Radiation enteritis. Curr Gastroenterol
Rep. 4(5):361-5, 2002
3. Horton KM et al: CT of nonneoplastic diseases of the small
bowel: spectrum of disease. J Com put Assist Tomogr.
23(3):417-28, 1999 .
4. Capps GW et al: Imaging features of radiation-induced
changes in the abdomen. Radiographies. 17(6):1455-73,
1997
5. Bluemke DA et al: Complications of radiation therapy: CT
evaluation. Radiographies. 11(4):581-600, 1991
6. Mendelson RM et al: The radiological features of chronic
radiation enteritis. Clin Radiol. 36(2):141-8, 1985
 RADIATION ENTERITIS
I IMAGE GALLERY

(Left) Small bowel follow

through (5BFT) shows
distortion, angulation +
thickening of folds with a
"spike" appearance (arrow).
(Right) 5BFT shows luminal
dilation proximal to stricture
(arrow) + indirect evidence
of bowel wall thickening,
with a "stack of coins"
appearance of the valvulae.

4
Typical 85
(Left) 5BFT shows luminal
narrowing, fold thickening
and nodularity. (Right) 5BFT
shows distal 5B stricture
(arrow) + proximal dilation
of 5B following surgery and
radiation therapy for cecal
carcinoma.

(Left) Air-contrast BE shows

radiation proctitis with
granular mucosal pattern
and luminal narrowing.
(Right) Air-contrast BE shows
long segment stricture of
rectosigmoid colon (arrow)
months after radiation
therapy for endometrial
carcinoma.
 SECTION S: Colon

Introduction and Overview

Colon Anatomy and Imaging Issues 1-5-2

Infection
Infection Colitis 1-5-6
Pseudomembranous Colitis 1-5-10
Typhlitis 1-5-14

Inflammation and Ischemia

Ulcerative Colitis 1-5-16
Toxic Megacolon 1-5-20
Appendicitis 1-5-22
Mucocele of the Appendix 1-5-26
Diverticulitis 1-5-28
Epiploic Appendagitis 1-5-32
Ischemic Colitis 1-5-36

Neoplasm
Colonic Polyps 1-5-40
Colon Carcinoma 1-5-44
Rectal Carcinoma 1-5-48
Villous Adenoma 1-5-52
Familial Polyposis 1-5-56
Gardner Syndrome 1-5-60

Miscellaneous
Sigmoid Volvulus 1-5-62
Cecal Volvulus 1-5-66
 COLON ANATOMY AND IMAGING ISSUES

Graphic shows schematic representation of various Barium enema shows malignant stricture ("apple core")
processes that may narrow the lumen of the colon (or of the colon; primary carcinoma.
any other part of the gut).

o Ulcerative colitis
I TERMINOLOGY • Presenting symptoms: Diarrhea, rectal bleeding,
Abbreviations and Synonyms pain
• Pathology: Mucosa and submucosa; crypt
• Barium Enema (BE)
5 • Small Bowel (SB)
abscesses; punctate and collar button ulcers
• Radiology: Continuous circumferential
2 involvement starting distally; shortened, ahaustral
colon; colonic strictures (late); SB involvement
I IMAGING ANATOMY only by backwash; no fistulas, sinus tracts, or
• Anatomic splenic flexure is the point atwhich the abscesses; colon cancer and toxic megacolon are
descending colon becomes retroperitoneal (distal to serious risks
radiologic splenic flexure) o Crohn disease
• Sigmoid colon • Presenting symptoms: Diarrhea, pain, weight loss,
o Intraperitoneal colonic segment bridging the palpable mass
retroperitoneal descending colon and the rectum • Pathology: Transmural; granulomas and enlarged
lymphoid follicles; aphthous ulcers; linear and
transverse ulcers; perianal fistulas
I ANATOMY-BASED IMAGING ISSUES I • Radiology: Discontinuous eccentric colonic and SB
involvement; fibrofatty proliferation in
Key Concepts or Questions mesentery; fistulas, sinus tracts, abscesses; colon
• Advantages of double-contrast BE over single-contrast cancer and toxic megacolon rare
BE • How do you distinguish among the various causes of
o Detection of small polypoid lesions colonic luminal narrowing?
o Detection of superficial ulcerations o Benign stricture: Smooth taper, both ends
o Subtle changes from endometriosis and metastases o Malignant stricture: Irregular, abrupt narrowing,
• Advantages of single-contrast BE apple core, shoulders at one or both ends
o Patient comfort o Extrinsic: Intact mucosa, whole lumen is displaced,
o Elderly or arthritic patients oblique angles for mass effect
o Detection of strictures o Submucosal: Intact mucosa, almost right angle
o Known or suspected diverticular disease interface with luminal surface
o Detection of large masses o Mucosal: Irregular mucosal surface, acute angle
o Evaluation for obstruction interface with luminal surface
o Evaluation for ischemia or other submucosal • How common are colonic polyps (detection rate is
pathology good measure of adequacy of examination technique)?
• Indication for water soluble contrast enema o Varies from 3% (age 20 to 30) , to 25% (age 80 to 90)
o Possible perforation o More than half are present in rectum and sigmoid
o Possible fistula • How do you distinguish a barium-coated polyp from a
o Pre-operative emergent study barium-lined diverticulum on an air-contrast barium
o "Therapeutic" (obstipation) enema?
• What criteria are useful to distinguish ulcerative colitis
and Crohn disease?
 COLON ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
Benign tumors POLYPOSIS SYNDROMES
• Hyperplastic polyp
• Adenomatous polyp Adenomatous polyps
• Villous adenoma ·.Familial polyposis coli
• Hamartoma ~. Gardner syndrome
• Spindle cell tumor • :1\1rcotsyndrome
• =>(Upoma, leiomyoma, etc.) • Attenuated adenomatous polyposis coli
• Carcinoid tumor Hamartomatous polyps
Malignant tumors • Peutz-Jeghers syndrome
• Carcinoma • Juvenile polyposis
• Lymphoma • Cronkhite-Canada syndrome
• Metastases • Cowden syndrome
• Kaposi sarcoma • Bannayan-Riley-Ruvalcaba syndrome
• Squamous cell carcinoma
• =>(Anal)

o Varies with location of polyp (dependent or o May develop malignant anal tumors (squamous,
non-dependent wall) and whether seen in profile or basaloid, etc.)
"en face"
o Easiest when polyp appears as filling defect in
barium pool; diverticulum fills with barium and ICUSTOM DIFFERENTIAL DIAGNOSISI
projects off surface of colon
Heredity non polyposis colon cancer
o Look for "bowler hat" (sessile polyp) or "Mexican
hat" (pedunculated polyp) signs syndrome (HNPCC)
5
o Polyp has sharp inner margins and fuzzy (indistinct) • Five times more common than familial polyposis 3
outer margins • Lynch I
o Diverticulum has sharp outer margins, fuzzy inner o Early onset « SO),right-sided, often multiple colon
• How do you distinguish colon carcinoma from cancers
diverticular disease on imaging? • Lynch II
o Carcinoma: Luminal narrowing is short « 10 cm), o Lynch I + extracolonic tumors
abrupt, irregular and eccentric, may resemble apple • Muir-Torre
core; CT may show lymphadenopathy, metastases o Similar to Lynch II + skin lesions
o Diverticulosis: Luminal narrowing is long (> 10 cm),
transverse folds are thick, irregular, resemble "cog length of colon involvement
wheel" (circular muscle hypertrophy): No • Cancer
pericolonic disease o Short « 10 cm)
o Diverticulitis: Luminal narrowing is long (> 10 cm), • Diverticulitis
asymmetric with combination of circular muscle o Segmental (> 10 cm), usually sigmoid, spares rectum
hypertrophy, spasm, pericolonic inflammation and • Ulcerative colitis
mass (abscess); CT shows pericolonic inflammation o Long segmental, usually distal, includes rectum
± pericolonic extraluminal gas, abscess, fistula • Crohn (granulomatous) colitis
• What is the current role of CT colonography? o Segmental, usually proximal, perirectal involvement
o Competitive with barium enema and endoscopy as a • Ischemia
screening procedure for colonic polyps o Segmental (90%), usually splenic flexure or sigmoid
o Must be performed and interpreted with expertise to • Infectious colitis (e.g., C. difficile)
achieve comparable results o Long segmental or pan colitis, involves rectum
o Main rationale is to provide screening for patients • Neutropenic colitis (typhlitis)
who are resistant to, or poor candidates for barium o Segmental, ascending colon + cecum
enema or colonoscopy
Aphthoid ulcers
• Amebic colitis
I CLINICAL IMPLICATIONS • Crohn disease
• CMV + herpes colitis
Clinical Importance • Salmonella + Shigella colitis
• Normal stratified squamous epithelium of the anal • Myotonic dystrophy
canal can be infected by human papilloma virus • Beh~et disease
(sexually transmitted) • Lymphoma
o May develop benign condyloma (locally invasive)
 COLON ANATOMY AND IMAGING ISSUES

Graphic shows the profile and en face appearance of Spot film from air-contrast BE shows a "bowler hat"
various polyps (A,B,D) and a diverticulum (C) on an air appearance of a small sessilepolyp (arrow).
contrast barium enema (lower row of pictures).

o Ulcerative colitis
Colonic (or small bowel) submucosal o Crohn disease
thickening • Infectious
• Air density = pneumatosis o Pseudomembranous colitis
5 o E.g., bowel infarct, "benign" pneumatosis o Neutropenic colitis (typhlitis)
o CMV colitis
• Fat density
4 o E.g., chronic inflammatory bowel disease (lED), o Other rare
cytoreductive therapy, obesity • Neoplastic
• Near-water density o Lymphoma
o E.g., acute inflammation, ischemia, "shock bowel" o Metastases
• Soft tissue density • Miscellaneous
o E.g., tumor, inflammation, ischemia o Pneumatosis cystoides coli
• Higher density o Endometriosis
o E.g., hemorrhage in bowel wall o Cirrhosis (portal hypertension)

Colonic Ileus (pseudo-obstruction) Ahaustral (smooth) colon

• Systemic acute inflammatory/traumatic • Normal (descending colon, elderly)
o Pneumonia, myocardial infarction • Ulcerative colitis (> Crohn)
o Pancreatitis, appendicitis, peritonitis • Cathartic abuse
o Trauma; spinal injury • Radiation colitis (late)
o Post-operative • Ischemic colitis (late)
• Drug effect
o Narcotics
o Antidepressants I SELECTED REFERENCES
o Antipsychotics 1. Koeller KK, et al (eds) Radiologic Pathology (2nd ed)
o Antiparkinsonian Washington, DC, Armed Forces Institute of Pathology,
o Anticholinergics 2003
• Endocrine disorder 2. Gore RM: Colon: Differential Diagnosis. In Gore RM,
o Hypothyroidism Levine MS (eds) Textbook of Gastrointestinal Radiology.
o Hypoparathyroidism 2nd ed. Philadelphia, WB Saunders. 1159-65, 2000
3. Diihnert W: Radiology Review Manual (4th ed),
o Diabetes
Philadelphia: Lippincott, 2000
• Neuromuscular disorders 4. Eisenberg RL: Gastrointestinal Radiology: A Pattern
o Parkinson disease Approach (3rd ed). Philadelphia: JB Lippincott, 1996
5. Reeder MM: Reeder and Felson's Gamuts in Radiology (3rd
Colonic thumbprinting ed) New York: Springer Verlag, 1993
• Vascular lesions
o Ischemic colitis
o Intramural hemorrhage (anticoagulation, trauma)
o Vasculitis (e.g., Henoch-Schonlein purpura)
o Hereditary angioneurotic edema
• Inflammatory
 COLON ANATOMY AND IMAGING ISSUES
IMAGE GALLERY
Typical
(Left) Air-contrast BE shows
many shallow aphthoid
ulcerations of the hepatic
flexure; Crohn
(granulomatous) colitis.
(Right) BE shows ahaustral
colon due to chronic
ulcerative colitis.

Typical
(Left) Axial CECT shows
marked water density
submucosal colonic wall
thickening;
pseudomembranous (c.
difficile) colitis. (Right) Axial
5
CECT shows fat density
5
submucosal thickening of the
rectal wall; chronic
ulcerative colitis.

Typical
(Left) BE shows
"thumbprinting" of the
colonic wall near the splenic
flexure; ischemic colitis.
(Right) Axial CECT shows
"thumbprinting" of
ascending colon; cirrhosis
and portal hypertension with
colonic edema.
 INFECTIOUS COLITIS
Axial CECT shows pancolitis with colonic wall
thickening and mesenteric hyperemia. Campylobacter
colitisin 78 year old woman.
ITERMINOlOGY
Definitions
• Inflammation of the colon caused by bacterial, viral,
5 fungal, or parasitic infections
6 granularity of rectum
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Focal or diffuse colonic wall
thickening with mucosal ulcerations
• Location: Dependent on etiology
Radiographic Findings
• Fluoroscopic-guided barium enema
o Focal or diffuse; segmental colitis or pancolitis
o Lumen narrowing & loss of haustra (edema/spasm)
o Thickened folds & colonic wall (edema)
o Ulceration - mucosal irregularity
o Superficial or deep "collar button" ulcers
o Discrete punctate, aphthous or large oval ulcers;
may simulate Crohn disease
o ± Small nodules or inflammatory polyps
o ± Diffuse, mucosal granularity; may simulate
ulcerative colitis
o ± Extrinsic mass with inflammatory changes -
distortion, short strictures; may simulate carcinoma
DDx: long Segment Wall Thickening
Pseudomem. Colitis Granulom. Colitis
Axial CECT shows mural thickening of ascending +
transverse colon plus dilated mesenteric vessels.
Campylobacter colitis.
o ± Thumbprinting; may simulate ischemic colitis
o ± Fistulas or sinus tracts
o Typhoid fever (Salmonellosis)
• Cecum or right colon; invariably in ileum
• Ileal fold thickening and ulceration
o Shigellosis: Predominantly in left colon; mucosal
o Campylobacteriosis: Small bowel and colon
o Yersinia enterocolitis: Predominantly in right colon,
occasionally in left; invariably in terminal ileum
o E. coli colitis: Transverse colon; extends to right, left
or both sides of colon
o Tuberculosis
• Right & proximal transverse colon, involves ileum
• Oval/circumferential, transverse ulcers, loss of
ileum & right colon anatomic demarcation
• Fleischner sign: Right-angle intersection between
ileum and cecum with marked hypertrophy of
ileocecal valve
• Exuberant mural thickening; > than Crohn disease
• Can cause "apple core" colonic stricture;
indistinguishable from carcinoma
o Actinomycosis: Rectosigmoid colon (intrauterine
devices) or ileocecal region (appendectomy)
o Gonorrheal, Chlamydia, Herpesvirus colitis:
Rectosigmoid colon
o Cytomegalovirus (CMV) colitis: Cecum & proximal
colon; extends to distal ileum
Ulcerative Colitis Ischemic Colitis
 INFECTIOUS COLITIS
Key Facts
Terminology
• Inflammation of the colon caused by bacterial, viral,
fungal, or parasitic infections
Imaging Findings
• Best diagnostic clue: Focal or diffuse colonic wall
thickening with mucosal ulcerations
• Lumen narrowing & loss of haustra (edema/spasm)
• Discrete punctate, aphthous or large oval ulcers; may
simulate Crohn disease
• ± Diffuse, mucosal granularity; may simulate
ulcerative colitis
• ± Thumbprinting; may simulate ischemic colitis
• ± Fistulas or sinus tracts
• Best imaging tool: Fluoroscopic-guided barium enema
o Histoplasmosis
• Ileocecal region; polyps in rectum
• Pericecal masses; may simulate appendicitis
o Mucormycosis: Right colon; polypoid mass
o Anisakiasis: Occasionally in right colon, rarely in
transverse colon
o Amebiasis
• Right colon; terminal ileum spared
• Skip lesions (in 95%); may simulate
granulomatous colitis
• Ameboma (in 10%): Marked granulation in short
segments of bowel, located in right colon
• Can produce "apple core" type colonic stricture
• Discrete ulcers appearing as marginal defects or
granularity with barium flecks
• Residual deformity and strictures after treatment
o Schistosomiasis
• Left or sigmoid colon
• Hallmark is inflammatory polyps (granulation
response to eggs deposited in bowel wall)
o Trichuriasis
• Clumping & granularity of barium (excessive
mucus)
• Wavy, linear 3-5 cm lucencies, occasionally
terminates in ring shape with central barium
collection (worm)
CT Findings
• Wall thickening and low attenuation (edema)
• Mucosal and serosal enhancement; ascites
• Multiple air-fluid levels; inflammatory pericolic fat
• Salmonellosis: ± Small bowel thickening & effacement
• Tuberculosis
o Changes in lungs, but usually from ingestion
o Low density, marked enlargement of lymph nodes
• Actinomycosis: Large inflammatory masses
• CMV colitis
o Deep ulcers & marked wall thickening (advanced)
o Enhancement of mucosa and serosa with hypodense
thickening of intervening bowel wall (edema)
o Hemorrhage causes 1 attenuation in wall
• Histoplasmosis
o Changes in lungs and skin
Top Differential Diagnoses
• Pseudomembranous colitis
• Granulomatous colitis (Crohn disease)
• Ulcerative colitis
• Ischemic colitis
Clinical Issues
• Usually acute in onset, except tuberculosis (chronic)
• Watery or bloody diarrhea
• Crampy abdominal pain and tenderness
Diagnostic Checklist
• Diagnosis by clinical presentation; lab tests
• Barium enema or CT detects colitis; need clinical
confirmation of specific type
o Mesenteric adenopathy, hepatosplenomegaly with
or without calcifications
• Mucormycosis: Changes in sinuses, lungs & central
nervous system
• Schistosomiasis
o Changes in mesenteric or hemorrhoidal vein,
urinary tract, terminal ileum
o ± Calcification of bowel wall or liver
5
7
Imaging Recommendations
• Best imaging tool: Fluoroscopic-guided barium enema
I DIFFERENTIAL DIAGNOSIS
Pseudomembranous colitis
• Radiography: Colonic wall thickening, nodularity;
may simulate infectious colitis
• CT: "Accordion sign": Trapped oral contrast between
thickened colonic haustral folds
• Usually results in more colonic wall thickening than
other colitides
Granulomatous colitis (Crohn disease)
• Concurrent small bowel (distal ileum) disease
• Barium enema
o Cobbles toning: Longitudinal & transverse
ulcerations produce a paving stone appearance
o Transmural, skip lesions, sinuses, fistulas; may
simulate infectious colitis
• Disease is chronic, compared with infectious colitis
Ulcerative colitis
• Barium enema
o Pancolitis with decreased haustration & multiple
ulcerations; may simulate infectious colitis
o "Mucosal islands" or "inflammatory pseudopolyps"
o Diffuse & symmetric wall thickening of colon
o Chronic phase ~ "lead-pipe" colon
Ischemic colitis
• Usually located in watershed areas; focal or diffuse
• Barium enema: Thumbprinting, ulcerations (1-3 weeks
after onset of disease); strictures (later)
 INFECTIOUS COLITIS
• CT
o ± Pneumatosis, portomesenteric venous gas
o ± Thrombus within splanchnic vessels
• Differentiate by clinical presentation
I PATHOLOGY
General Features
• Etiology
o Bacterial organisms (most common in Western
countries): Salmonella, Shigella, Campylobacter,
Yersinia, Staphylococcus, Escherichia coli
(0157:H7), M. tuberculosis, Actinomyces,
Chlamydia trachoma tis, C. gonorrhea
• Chlamydia is the causative agent for
"lymphogranuloma venereum"
o Viral organisms: Herpes virus, CMY, Norwalk virus,
Rotavirus
o Fungal organisms: Histoplasma, Mucor
o Parasitic organisms (most common in
underdeveloped countries): Anisakis, Amoeba,
Schistosoma, Strongyloides, Trichuriasis
o Risk factors
• Salmonella, Shigella: Outbreaks, warm weather
• E. coli: Travel, nursing homes (0157:H7)
• Tuberculosis, CMV: AIDS
5 • Actinomycosis: Intrauterine devices,
appendectomy
8 • Histoplasma, Mucor: Chronic debilitation or
immunosuppression
• Strongyloides: Severe debilitation
o Pathogenesis
• Ingestion of pathogenic organisms (often
fecal-oral route)
• Chlamydia, Gonorrhea, Herpes virus: Direct
inoculation of rectum (anal intercourse)
Gross Pathologic & Surgical Features
• Varies based on etiology
Microscopic Features
• Varies based on etiology
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Usually acute in onset, except tuberculosis (chronic)
o Watery or bloody diarrhea
o Crampy abdominal pain and tenderness
o Fever, headache, nausea, vomiting, weight loss
o Palpable abdominal mass, anemia, malaise, rash
o Arthritis, pneumonitis, seizures, peripheral
neuropathy, microangiopathy
o Varied incubation period
o E. colli colitis: Traveler's diarrhea, hemolytic-uremic
syndrome (0157:H7)
o Schistosomiasis: Hepatosplenomegaly ~ portal
hypertension
• Lab-Data
o Bacterial organisms: Increased neutrophilic count
o Viral organisms: t Lymphocytes (I in AIDS)
o Fungal, parasitic organisms: Eosinophilia
• Diagnosis
o Stool cultures, blood cultures, endoscopic biopsy,
serology studies
Demographics
• Age: Any age, but incidence t with age
• Gender: M:F = 1:1
Natural History & Prognosis
• Complications
o Toxic megacolon, bacteremia, sepsis, death
o Hemorrhage, perforation, obstruction
o Yersinia enterocolitis: Hepatic abscess
o E. coli colitis: Hemolytic-uremic syndrome
o Amebiasis: Liver and lung abscesses
• Prognosis
o Usually very good, after treatment
o Campylobacteriosis: 25% recurrence if untreated
o E. coli 0157:H7 colitis: t Morbidity, 33% mortality
o CMV colitis: Hemorrhage and ischemia can be fatal
o Mucormycosis, Strongyloidiasis: Fatal
Treatment
• Bacterial organisms: Mostly self-limiting, last 1-2
weeks; up to 1 month
o Salmonellosis: Parenteral cephalosporins if severe
o Shigellosis: Ampicillin in severe cases
o Yersinia enterocolitis: Lasts several months; no
treatment available
o E. coli 0157:H7 colitis: Supportive treatment,
isolation procedures
o Tuberculosis: Antituberculosis drugs, no steroids
• Viral organisms: Mostly self-limiting
o CMV: Treat underlying AIDS
• Parasitic organisms: Antihelminthic drugs
o Anisakiasis: Mostly self-limiting, last 7-10 days
• Fungal organisms: Antifungal drugs
I DIAGNOSTIC CHECKLIST
Consider
• Diagnosis by clinical presentation; lab tests
Image Interpretation Pearls
• Barium enema or CT detects colitis; need clinical
confirmation of specific type
I SELECTED REFERENCES
1. Thielman NM et al: Clinical practice. Acute infectious
diarrhea. N Engl J Med. 350(1):38-47, 2004
2. Horton KM et al: CT evaluation of the colon: inflammatory
disease. Radiographics. 20(2):399-418, 2000
3. Philpotts LEet al: Colitis: use of CT findings in differential
diagnosis. Radiology. 190(2):445-9, 1994
4. Schmitt SLet al: Bacterial, fungal, parasitic, and viral
colitis. Surg Clin North Am. 73(5):1055-62, 1993
5. Wall SD et al: Gastrointestinal tract in the
immunocompromised host: opportunistic infections and
other complications. Radiology. 185(2):327-35, 1992
 INFECTIOUS COLITIS

I IMAGE GALLERY

(Left) Single-contrast BE
shows rectal stricture with
mucosal irregularity due to
"lymphogranuloma
venereum" (Chlamydia
trachomatis). (Right)
Single-contrast BE shows
"apple core" lesion of
ascending colon due to
Mycobacterium tuberculosis.

Typical
(Left) Axial CECT shows
pancolitis due to
Cytomegalovirus (CMV) in a
patient with AIDS. (Right)
Axial CECT shows
proctocolitis with mural
5
thickening and mesenteric
9
hyperemia in a 32 year old
woman due to CMV colitis.

Typical
(Left) Axial CECT shows
Campylobacter pancolitis in
a previously healthy 26 year
old woman. Note
"thumbprinting" of colonic
wall (arrow). (Right) Axial
CECT of 26 year old woman
with Campylobacter colitis
shows marked mural
thickening of sigmoid colon.
 PSEUDOMEMBRANOUS COLITIS

Graphic shows pancolitis with marked mural thickening Axial CECT shows pancolitis with marked mural
with multiple elevated yellow-white plaques thickening and enteric contrast trapped between
(pseudomembranes) . haustra ("accordion sign").

o Clindamycin causes diarrhea in 20% &

ITERMINOlOGY pseudomembranous colitis in 10% of patients
Abbreviations and Synonyms o C. difficile infection of colon follows insult to gut by
• Pseudomembranous colitis (PMC) antibiotic or chemotherapy
5 • Antibiotic colitis, Clostridium difficile colitis o C. difficile infection is responsible for virtually all
cases of PMC
10 Definitions o C. difficile toxins: Cytotoxic + enterotoxic effects
• Acute inflammation of colon caused by toxins o Complications range from watery diarrhea to
produced by Clostridium difficile bacteria colonic perforation & death
Radiographic Findings
jlMAGING FINDINGS • Radiography
o Colonic ± small bowel ileus
General Features o Gaseous distension of colon + nodular haustral
• Best diagnostic clue: Marked submucosal edema over a thickening
long segment of colon o Thumbprinting: Unusual, wide transverse bands due
• Location to thickening of haustral folds
o Usually entire colon (pancolitis) • Most prominent in transverse colon
o Rectum & sigmoid colon (typically involved in o Severe cases: Polypoid mucosal thickening
80-90% of cases) • Represent pseudomembranous plaques protruding
o Confined to more proximal colon (10% of cases) into air-containing lumen
• Morphology: Plaque-like adhesions of fibrinopurulent o Fulminant cases
necrotic debris & mucus on damaged colonic mucosa • Toxic megacolon
with submucosal edema • Pneumoperitoneum
• Other general features • Fluoroscopic guided contrast enema studies
o PMC is usually associated with antibiotic use, o Enema is contraindicated in severe PMC (due to
especially clindamycin increased risk of perforation)
o Limited role in diagnosis of PMC

DDx: Other Causes of Colitis

Campylobacter CMV Colitis Ulcerative Colitis Ischemic Colitis

 PSEUDOMEMBRANOUS
Key Facts
Terminology
• Pseudomembranous colitis (PMC)
• Antibiotic colitis, Clostridium difficile colitis
• Acute inflammation of colon caused by toxins
produced by Clostridium difficile bacteria
Imaging Findings
• Best diagnostic clue: Marked submucosal edema over
a long segment of colon
• Usually entire colon (pan colitis)
• Rectum & sigmoid colon (typically involved in
80-90% of cases)
• "Accordion sign": Represents trapped enteric contrast
between thickened colonic haustral folds
• Pericolonic stranding
• Ascites common in severe PMC
o Findings vary depending on severity & extent of
disease
o Marked mural thickening & wide haustral folds due
to intramural edema
CT Findings
• CECT with oral contrast
o Colonic wall thickening & nodularity
• Thickening is more irregular & shaggy compared
to symmetric & homogeneous in Crohn
o "Accordion sign": Represents trapped enteric
contrast between thickened colonic haustral folds
• Alternating bands of high attenuation (contrast) +
low attenuation (edematous haustra)
• Usually seen in advanced cases
• Highly suggestive of PMC
o "Target sign"
• Mucosa: Intense enhancement (hyperemia)
• Submucosa: Thickened, non-enhancing, I HU
(edema)
o Extent of disease
• Pancolitis is most common
• Rectum & sigmoid colon (80-90% of cases)
• Right colon involved exclusively in some cases
o Pericolonic stranding
• Usually mild (due to primary mucosal &
submucosal nature of PMC)
• Relative paucity of pericolonic inflammation +
marked colonic wall thickening differentiates
PMC from other colitides
o Ascites common in severe PMC
• Uncommon in other inflammatory bowel diseases
o ± Pneumatosis coli or air in intrahepatic portal vein
o Small pleural effusions & subcutaneous edema
• May be due to primary disease or debilitated state
Imaging Recommendations
• Best imaging approach is CECT with oral contrast
o 125 ml LV contrast at ~ 2.5 mllsec
COLITIS
Top Differential Diagnoses
• Granulomatous colitis (Crohn disease)
• Ulcerative colitis
• Ischemic colitis
• Neutropenic enterocolitis
Pathology
• Antibiotic therapy (clindamycin most common) due
to overgrowth of resistant enteric C. difficile
Clinical Issues
• Clinical profile: Patient with history of watery
diarrhea after antibiotic use or hospitalization
Diagnostic Checklist
• Check history of antibiotic use or debilitating diseases
• Suspect in any hospitalized patient with acute colitis
I DIFFERENTIAL DIAGNOSIS
Other infectious colitis
• Campylobacter, cytomegalovirus, etc.
• May be indistinguishable from pseudomembranous
colitis
• Often have less severe colonic wall thickening 5
Granulomatous colitis (Crohn disease) 11
• Concurrent small bowel (distal ileum) disease usually
• Cobblestoning: Longitudinal & transverse ulcerations
produce a paving stone appearance
• Segmental distribution
• Transmural, skip lesions, sinuses, fissures, fistulas
• CT shows fibrofatty proliferation of mesentery &
enlarged mesenteric lymph nodes
Ulcerative colitis
• Classic imaging appearance
o Pancolitis with decreased haustration & multiple
ulcerations on barium enema
• Colorectal narrowing; 1 presacral space> 1.5 em
• "Mucosal islands" or "inflammatory pseudopolyps"
• Diffuse & symmetric wall thickening of colon
• Backwash ileitis: Distal ileum involvement (10-40%)
• Chronic phase
o "Lead-pipe" colon: Rigid colon with loss of haustra
Ischemic colitis
• Usually seen in watershed areas; focal or diffuse
o Left side colon: Typical in elderly (hypoperfusion)
• Splenic flexure: Junction of SMA & IMA
o Right-side colon: Younger patients
• Due to decreased collateral blood supply
• Barium findings
o Thumbprinting: Submucosal edema or hemorrhage
o Ulceration: 1-3 weeks after onset of disease
o Stricture: Seen in late phase
• CT findings
o Bowel wall thickening ± luminal dilatation
o ± Pneumatosis, portomesenteric venous gas
o ± Thrombus within splanchnic vessels
• Has less wall thickening than PMC
 PSEUDOMEMBRANOUS COLITIS
• Typically takes 48 hours to confirm
Neutropenic enterocolitis o Proctosigmoidoscopy or colonoscopy
• Clinical history of neutropenia & immunosuppression • Adherent yellow plaques 2-10 mm in diameter
• Usually focal disease in right colon & cecum
• Mural thickening limited to right colon & distal ileum Demographics
• Thumbprinting, luminal narrowing, ulceration • Age
• Immunocompromised with PMC mimics neutropenic o Elderly age group> young age group
colitis when localized to cecum & right colon • Elderly are at higher risk for developing PMC &
recurrent PMC than young
• Gender: Equal in both males & females (M = F)
!PATHOLOGY Natural History & Prognosis
General Features • Complications
• Etiology o Toxic megacolon, sepsis, perforation & death
o Antibiotic therapy (clindamycin most common) due • Prognosis
to overgrowth of resistant enteric C. difficile o If treated early, full recovery expected
• Antibiotic therapy usually within 2 days to 2 o Recurrence rate higher in women & elderly
weeks & rarely up to 6 months o Severe cases may need colectomy
• Ampicillin, tetracycline, erythromycin, penicillin o Untreated cases can lead to perforation, acute
(less common) abdomen & death (mortality rate 1.1-3.5%)
o Other causes
Treatment
• Abdominal surgery, colonic obstruction, uremia,
prolonged hypotension or hypoperfusion of bowel • Mild cases: Discontinue offending antibiotic therapy
• Severe debilitating diseases (e.g., lymphoma, • Severe cases
leukemia, AIDS) o Metronidazole (drug of choice) or oral vancomycin
o Pathogenesis o Fulminant & toxic megacolon: Colectomy
• Antibiotic therapy (clindamycin)
5 • Inhibits & alters normal intestinal micro flora
• Overgrowth of resistant enteric C. difficile I DIAGNOSTIC CHECKLIST
• Enterotoxin (toxin A) & cytotoxin (toxin B) ~ Consider
12
mucosal damage
• Check history of antibiotic use or debilitating diseases
• Epidemiology
• Suspect in any hospitalized patient with acute colitis
o 1-10 cases per 1,000 patient discharges from hospital
o 1 case per 10,000 antibiotic prescriptions written Image Interpretation Pearls
outside hospital • Marked submucosal edema over long segment of colon
Gross Pathologic & Surgical Features • "Accordion sign": Trapped oral contrast between
thickened colonic haustral folds
• Inflamed colon with multiple elevated, yellow-white
• Usually pancolitis; rectum & sigmoid colon involved
plaques (pseudomembranes)
in 80-90% of cases
• When removed during endoscopy, reveals
erythematous & inflamed mucosa
Microscopic Features I SELECTED REFERENCES
• Colonization of colon by clostridium difficile 1. Gore RM et al: Inflammatory conditions of the colon.
• Mild-early: Focal necrosis of surface epithelial cells in Semin Roentgenol. 36(2):126-37, 2001
glandular crypts with neutrophilic infiltration & fibrin 2. Kirkpatrick ID et al: Evaluating the CT diagnosis of
plugging of capillaries in lamina propria and mucus Clostridium difficile colitis: should CT guide therapy? A]R
Am] Roentgenol. 176(3):635-9,2001
hypersecretion in adjacent crypts
3. Horton KM et al: CT evaluation of the colon: inflammatory
• Moderate: Crypt abscesses disease. Radiographies. 20(2):399-418, 2000
• Severe-late: Necrosis & denudation of mucosa with 4. Kawamoto S et al: Pseudomembranous colitis: spectrum of
thrombosis of submucosal venules imaging findings with clinical and pathologic correlation.
Radiographies. 19(4):887-97, 1999
5. Macari M et al: The accordion sign at CT: a nonspecific
I CLINICAL ISSUES finding in patients with colonic edema. Radiology.
211(3):743-6, 1999
Presentation 6. Ros PR et al: Pseudomembranous colitis. Radiology.
198(1):1-9, 1996
• Most common signs/symptoms
7. Gore RM et al: Radiologic investigation of acute
o Mild cases: Watery diarrhea
inflammatory and infectious bowel disease. Gastroenterol
o Severe cases: Acute abdomen Clin North Am. 24(2):353-84, 1995
• Fever, abdominal pain & tenderness, tachycardia 8. Fishman EK et al: Pseudomembranous colitis: CT
• Dehydration, leukocytosis & sepsis evaluation of 26 cases. Radiology. 180(1):57-60, 1991
• Clinical profile: Patient with history of watery diarrhea 9. Rubesin SE et al: Pseudomembranous colitis with
after antibiotic use or hospitalization rectosigmoid sparing on barium studies. Radiology. 170(3
• Diagnosis Pt 1):811-3, 1989
o Demonstration of C. difficile toxins in stool
 PSEUDOMEMBRANOUS COLITIS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
massive submucosal edema
of the transverse colon with
luminal narrowing + striking
mucosal enhancement.
(Right) Axial CECT shows
transmural involvement of
entire colon, plus ascites,
raising the concern for
perforation.

(Left) Single-contrast BE
shows pan colitis, with
"thumbprinting" indicating
submucosal edema or
hemorrhage. (Right) Photo of
opened resected colon
5
shows sloughed, necrotic
13
mucosa and raised yellow
plaques or
pseudomembranes.

Typical
(Left) Axial CECT shows
marked mural thickening of
transverse colon, plus
extraluminal gas (arrow) and
enteric contrast media (open
arrow). Fatalcolonic
perforation. (Right) Axial
CECT shows marked wall
thickening of ascending
colon with high density
ascites. C. difficile colitis with
perforation.
 TYPHLITIS

Single contrast BE shows marked irregular narrowing of Axial CECT shows thickening of the wall of the cecum +
the lumen of the cecum. Small bowel is dilated. ascending colon. The lumen of the cecum is narrowed;
ascending colon dilated.

o Thumbprinting due to bowel edema

ITERMINOLOGY o ± Pneumatosis: Speckled or linear pattern
Abbreviations and Synonyms • Fluoroscopic guided water-soluble contrast enema
• Synonym(s): Neutropenic colitis, ileocecal syndrome, o Usually not recommended (possible perforation)
5 cecitis, necrotizing enteropathy o Mural thickening & mucosal thumbprinting
o Luminal narrowing or dilatation of cecum
14 Definitions o ± Dilated adjacent bowel loops (paralytic ileus)
• Inflammatory or necrotizing process involving cecum, o Shallow or deep ulcerations
ascending colon & occasionally distal ileum/appendix
CT Findings
• NECT
IIMAGING FINDINGS o Cecal luminal distention or narrowing
o Circumferential wall thickening of cecum ±
General Features ascending colon & distal ileum
• Best diagnostic clue: Massive mural thickening of cecal o I Bowel-wall attenuation (due to edema)
± ascending colon wall o Pericecal fat stranding + thickened fascial planes
• Location: Cecum + ascending colon (more common) o Pericolonic fluid collection
• Morphology: Dilated or narrow lumen, thickened wall o ± Pneumatosis, pneumoperitoneum
• Other general features o ± Dilated adjacent bowel loops (paralytic ileus)
o Usually seen in severely neutropenic patients • CECT: Heterogeneous enhancement of bowel wall
• Post chemotherapy or bone marrow transplant
Ultrasonographic Findings
o More common in children than adults
o Clinical syndrome of fever & right lower quadrant • Real Time
tenderness in immunosuppressed host o Hypoechoic or hyperechoic thickened bowel wall
o Anechoic free fluid; ± mixed echoic abscess
Radiographic Findings
Imaging Recommendations
• Radiography
o Ileocecal dilatation with air-fluid levels • Helical CT: Study of choice for diagnosis of typhlitis
o RLQ soft tissue mass • Water-soluble contrast; colonoscopy (contraindicated)

DDx: Fold Thickening, Contraction of Cecum

,,-.

Cecal Carcinoma
,.~.
•••••
App. Abscess
' ..
Diverticulitis Pseudomem. Colitis
 TYPHLITIS

Key Facts
Imaging Findings • Cecal diverticulitis
• Best diagnostic clue: Massive mural thickening of • Crohn disease
cecal ± ascending colon wall • Pseudomembranous colitis
• Pericecal fat stranding + thickened fascial planes Pathology
• CECT: Heterogeneous enhancement of bowel wall
• Hemorrhagic, thick, boggy cecum & adjacent colon
Top Differential Diagnoses Diagnostic Checklist
• Cecal carcinoma
• Check for history of chemotherapy for leukemia or
• Appendicitis bone marrow transplantation

I DIFFERENTIAL DIAGNOSIS Demographics

• Age: Children & young adults> older adults
Cecal carcinoma
• Gender: Equal in both males & females (M=F)
• "Apple core" lesion: Narrow lumen; irregular mucosa
Natural History & Prognosis
Appendicitis
• Complications: Abscess, necrosis, perforation, sepsis
• Thickened cecal wall adjacent to inflamed appendix • Prognosis: Early stage (good); late stage (poor)
Cecal diverticulitis Treatment
• Bowel wall thickening, fat stranding, free fluid or air • Medical: High doses of antibiotics & IV fluids
• Cecal outpouching differentiates from typhlitis • Complicated case
Crohn disease o CT signs of perforation: Surgical resection
• Segmental, transmural, cobblestone mucosa o Granulocyte transfusions
• Luminal narrowing, typically seen in terminal ileum 5
Pseudomembranous colitis I DIAGNOSTIC CHECKLIST 15
• Due to Clostridium difficile bacteria
• Usually affects all or most of colon Consider
• Check for history of chemotherapy for leukemia or
bone marrow transplantation
I PATHOLOGY Image Interpretation Pearls
General Features • Cecal wall thickening & pericolonic inflammation in
severely neutropenic patients
• Etiology
o Neutropenic conditions
• Leukemia, lymphoma, any malignancy, organ or
bone marrow transplant cases on chemotherapy
I SELECTED REFERENCES
• AIDS; viral, bacterial & fungal infections 1. Horton KM et al: CT evaluation of the colon: Inflammatory
• Idiopathic, aplastic anemia, ischemia, antibiotics disease. RadioGraphies. 20: 399-418, 2000
o Mechanism 2. Adams GW et al: CT detection of typhlitis. Journal of
Computed Assisted Tomography. 9: 363-5, 1985
• Chemotherapy/antibiotics ~ immunosuppression
3. Frick MP et al: Computed tomography of neutropenic
~ neutropenia ~ infection ~ typhlitis colitis. AJR. 143: 763-5, 1984
• Epidemiology: Incidence: Children> adults
• Associated abnormalities: Neutropenic diseases
Gross Pathologic & Surgical Features I IMAGE GALLERY
• Hemorrhagic, thick, boggy cecum & adjacent colon
Microscopic Features
• Inflammatory, ischemic, necrotic, ulcerative changes

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Fever, RLQ pain, watery diarrhea, ± hematochezia
o Fullness; palpable mass; RLQ tenderness (± rebound)
• Lab data: Neutropenia, leukopenia; ± blood in stool
• Diagnosis: Imaging, clinical & lab correlation (Left) Axial CECT in leukemic patient. Cecal wall is massively
thickened, lumen narrowed, with per/colonic infiltration. (Right) Axial
CECT shows cecal wall thickening, obliteration of lumen.
 ULCERATIVE COLITIS

Graphic shows innumerable "collarbutton" ulcers and Single-contrastbarium enema (BE) shows innumerable
loss of haustra throughout descending and sigmoid "collar button" ulcers and loss of haustra throughout
colon. descending colon.

ITERMINOLOGY o Begins in rectum & extends proximally to involve

part or all of the colon
Abbreviations and Synonyms o Backwash ileitis: 10-40% of chronic UC patients,
• Ulcerative colitis (UC) distal ileum is inflamed
5 Definitions
o Ulcerative colitis> common than Crohn disease
o Incidence: First-degree relatives 30-100 times>
16 • Chronic, idiopathic diffuse inflammatory disease that general population
primarily involves colorectal mucosa & submucosa o I Risk of colorectal cancer in UC than Crohn colitis
• Annual incidence: 10% after first decade of UC
• 75-80% who develop colon cancer have pan colitis
I IMAGING FINDINGS • 25% UC cases have multiple carcinomas (often
flat & scirrhous, difficult to image)
General Features
• Best diagnostic clue: Pancolitis with I haustration + Radiographic Findings
multiple ulcerations on barium enema • Fluoroscopic guided barium contrast enema
• Location: Rectum (30%); rectum + colon (40%); o Acute changes
pancolitis (30%) • Colorectal narrowing + incomplete filling (spasm
• Morphology + irritability)
o Narrow lumen, superficial ulcers, pseudopolyps • Fine mucosal granular pattern (edema/hyperemia)
o "Lead-pipe" colon & lack of haustra in chronic phase • Mucosal stippling: Punctate barium collections
• Other general features (crypt abscesses erode ~ ulcers & barium
o Chronic relapsing inflammatory bowel disease with collection)
acute features • "Collar button" ulcers (flask-like): Due to
o Disease with continuous concentric + symmetric undermining of ulcers (ulcers enlarge ~
colonic involvement configuration lost ~ mucosal islands + polyps)
o Inflammation limited to mucosa & submucosa • Haustra: Edematous & thickened
o Characterized by pseudopolyps/crypt microabscesses • Polyps: Inflammatory & postinflammatory
pseudopolyps (remnants of mucosa & submucosa)
o Chronic changes

DDx: Ulceration, Wall Thickening of Colon

Granulomatous Colitis Pseudomem. Colitis Ischemic Colitis Cathartic Colon

 ULCERATIVE COLITIS
Key Facts
Terminology
. pseudo polyps (remnants of mucosa & submucosa
• Chronic, idiopathic diffuse inflammatory dIsease that
primarily involves colorectal mucosa & submucosa
Imaging Findings
• Best diagnostic clue: Pan colitis with I haustration +
multiple ulcerations on barium enema
• Location: Rectum (30%); rectum + colon (40%);
pancolitis (30%)
• Fine mucosal granular pattern (edema/hyperemia)
• Mucosal stippling: Punctate barium collections (crypt
abscesses erode ~ ulcers & barium collection)
• "Collar button" ulcers (flask-like): Due to
undermining of ulcers (ulcers enlarge ~
configuration lost ~ mucosal islands + polyps)
• Haustra: Edematous & thickened
• Shortening of colon with depression of flexures
(reversible)
• "Lead-pipe" colon: Rigidity + luminal narrowing
• Haustrations: Blunted or complete loss
• Backwash ileitis: Distal 5-25 cm of ileum is
inflamed (seen in 10-40% cases)
• Luminal narrowing & widened presacral space
(more than 1.5 cm)
• Benign strictures: Local sequelae of UC (seen in
10% of patients)
o Rectal valve abnormalities (double contrast study)
• Lateral rectal view: Normally at least one rectal
valve should be visible
• Fold is usually seen at the level of S3 & S4 (less
than 5 mm thick)
• Proctitis: Valve thickness> 6.5 mm or absent
CT Findings
• NECT
o Colorectal narrowing
o Widening of presacral space: > 1.5 cm
• Due to perirectal fibrofatty proliferation
o Diffuse + symmetric wall thickening of colon
• Less than 10 mm (average 7.8 mm)
o Mural thickening & luminal narrowing
• Seen in subacute & chronic ulcerative colitis
• CECT
o "Target" or "halo" sign
• Enhancing inner ring of bowel wall (mucosa)
• Nonenhancing middle ring of bowel wall
(submucosa): Due to edema in acute or halo of fat
in chronic phase
• Enhancing outer ring of bowel wall (muscularis
propria)
o Enhancement of
• "Mucosal islands" or inflammatory "pseudopolyps"
• Inflammatory peri colonic stranding
Imaging Recommendations
• Barium enema (single & double contrast studies);
helical NE + CECT
• Polyps: Inflammatory & postinflammatory )
• "Lead-pipe" colon: Rigidity + luminal narrowing
• Widening of presacral space: > 1.5 cm
• Diffuse + symmetric wall thickening of colon
Top Differential Diagnoses
• Granulomatous colitis (Crohn disease)
• Pseudomembranous colitis (PMC)
• Ischemic colitis
• Neutropenic enterocolitis
• Diverticulitis
Diagnostic Checklist .
• Continuous concentric & symmetric involvement
• Consider UC in any patient with sclerosing
cholangitis
I DIFFERENTIAL DIAGNOSIS
Granulomatous colitis (Crohn disease)
• Barium enema findings
o Aphthae: Punctate central collections of barium
o Cobbles toning: Longitudinal & transverse
ulcerations produce a paving stone 5
o Segmental distribution
• Involve both colon & small bowel (60% cases) 17
• Isolated to colon (20% cases)
o Transmural, skip lesions, sinuses, fissures, fistulas
o In late stage indistinguishable from ulcerative colitis
due to haustral loss & pseudopolyps
• CT shows
o Bowel wall thickening (1-2 cm)
o "Creeping fat" or fibrofatty proliferation of
mesentery
o Enlarged mesenteric lymph nodes
o "Comb" sign: Mesenteric hypervascularity
• Indicates active disease
Pseudomembranous colitis (PMC)
• Synonym(s): Antibiotic colitis or C. difficile colitis
• Usually involves entire colon (pan colitis)
• CT findings
o Colonic wall thickening & nodularity
o "Accordion" sign: Represents trapped enteric
contrast between thickened colonic folds
o Ascites common in PMC & unusual in other lED
Ischemic colitis
• Usually seen in watershed areas; focal or diffuse
o Left side colon: Typical in elderly (hypoperfusion)
• Splenic flexure: Junction of SMA & IMA
o Right-side colon: Young patients
• Due to decreased collateral blood supply
• Barium findings
o Thumbprinting: Submucosal edema or hemorrhage
o Ulceration: 1-3 Weeks after onset of disease
o Stricture: Seen in late phase
• CT findings
o Bowel wall thickening, ± luminal dilatation
o ± Pneumatosis, portomesenteric venous gas
 ULCERATIVE COLITIS
o ± Thrombus within splanchnic vessels
Cathartic colon
• Due to long term use/abuse of laxatives + cathartics
• Appearance of ahaustral "rigid" colon simulates
ulcerative colitis
Neutropenic enterocolitis
• Clinical history: Neutropenia & immunosuppression
• Usually focal disease in right colon & cecum
• Imaging findings
o Mural thickening limited to right colon ± distal
ileum
o Thumbprinting: Due to bowel edema
o Luminal narrowing
o Shallow or deep ulcerations ± pneumatosis
Diverticulitis
• CT findings
o Location: Most common in sigmoid colon
o Bowel wall & fascial thickening; fat stranding; free
fluid & air
o Pericolic inflammatory changes
• Abscess, sinuses, fistulas
o "Arrowhead" sign: Due to diverticular orifice edema
o Focal area of eccentric luminal narrowing
o Diverticulosis uncommon in patients with ulcerative
colitis
5 • Rule out other inflammatory
18 I PATHOLOGY
General Features
• Genetics
o 1 Frequency in monozygotic twins
o HLA B5, BW52 & DR2linked to UC
• Etiology
o Genetic, familial, environmental, neural, hormonal
o Infectious, nutritional, immunological, vascular
o Traumatic, psychological & stress factors
o Smoking decreases risk factor
• Epidemiology: Incidence 2-10 cases/lOO,OOO people
• Associated abnormalities
o Primary sclerosing cholangitis (PSC), uveitis
o Ankylosing spondylitis, rheumatoid arthritis
o Pyoderma gangrenosum, sacroiliitis
Gross Pathologic & Surgical Features
• Rectum + colon involved
• Continuous involvement
• Superficial ulcers, pseudopolyps
Microscopic Features
• Inflammatory infiltrate, crypt microabscesses
• Limited to mucosa & submucosa
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Relapsing bloody mucus diarrhea
o Fever, weight loss, abdominal pain & cramps
o Systemic manifestations
• Lab-data: Blood & mucus in stool
• Diagnosis: Mucosal biopsy & histology
Demographics
• Age: 15-25 years (small peak at 55-65 years)
• Gender: Males less than females (M < F)
• Ethnicity: More common in Caucasians & Jews
Natural History & Prognosis
• Complications
o Toxic megacolon, colorectal cancer, strictures
o Increased incidence of colon carcinoma up to 50%
after 25 years of disease
• Prognosis
o Improves with diagnosis & management
o Mortality: First 2 years of UC in > 40 years old
• Males (2.1%); females (1.5%)
Treatment
• Medical
o Sulfasalazine, steroids, azathioprine
o Methotrexate, LTB4 inhibitors
• Surgical: Total or proctocolectomy + Brooke or
continent ileostomy (Kock pouch)
I DIAGNOSTIC CHECKLIST
Consider
diseases of colon
Image Interpretation Pearls
• Colorectal narrowing + punctate & collar button ulcers
• Continuous concentric & symmetric involvement
• "Lead-pipe" (rigid) colon & haustral loss (late phase)
• Consider UC in any patient with sclerosing cholangitis
I SELECTED REFERENCES
1. Carucci LR et al: Radiographic imaging of inflammatory
bowel disease. Gastroenterol Clin North Am. 31(1):93-117,
ix, 2002
2. Horton KM et al: CT evaluation of the colon: inflammatory
disease. Radiographies. 20(2):399-418, 2000
3. Kawamoto S et al: Pseudomembranous colitis: spectrum of
imaging findings with clinical and pathologic correlation.
Radiographies. 19(4):887-97, 1999
4. Balthazar EJ et al: Ischemic colitis: CT evaluation of 54
cases. Radiology. 211(2):381-8, 1999
5. Antes G: Inflammatory disease of the small intestine and
colon: Contrast enema and CT. Radiology. 38: 41-5, 1998
6. Gore RM et al: CT features of ulcerative colitis and Crohn's
disease. AJR. 167: 3-15, 1996
7. Jacobs JE et al: CT of inflammatory disease of the colon.
Semin Ultrasound CT MR. 16(2):91-101, 1995
8. Gore RM et al: CT findings in ulcerative, granulomatous,
and indeterminate colitis. AJRAm J Roentgenol.
143(2):279-84, 1984
9. Kelvin FM et al: Double contrast barium enema in Crohn's
disease and ulcerative colitis. AJRAm J Roentgenol.
131(2):207-13, 1978
10. Laufer I et al: The radiological differentiation between
ulcerative and granulomatous colitis by double contrast
radiology. Am J Gastroenterol. 66(3):259-69, 1976
 ULCERATIVE COLITIS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
narrowed lumen and
thickened wall of descending
colon. Submucosal halo of
low density (edema) and
engorged blood vessels
indicate active disease.
(Right) Axial CECT shows
narrowed lumen and
thickened wall of sigmoid
colon with submucosal
edema and engorged vessels.

(Left) Single-contrast BE
shows prominent, thickened
haustra in right colon, but
diminished haustra in left
colon. (Right) Oblique new
single-contrast BEshows
5
narrowed lumen, ahaustral
19
left colon with diffuse
ulceration (collar button +
flask-shaped) .

(Left) Double-contrast BE
shows filiform polyps in a
patient with chronic Uc,
now in remission. (Right)
Single-contrast BE shows
ahaustral colon due to
chronic uc. Apple core
stricture of transverse colon
(arrow) due to
adenocarcinoma.
 TOXIC MEGACOLON

Supine radiograph shows ahaustral colon in an acutely Supine radiograph shows diffusely dilated bowel in an
ill patient with chronicuc. The transverse colon is acutely ill patient with ulcerative colitis. The transverse
dilated and "shaggy" in appearance due to sloughed colon is dilated, ahaustral with an irregular mucosal
mucosa and pseudopolyps. surface.

ITERMINOLOGY Radiographic Findings

Definitions
• Acute transmural fulminant colitis with
5 neuromuscular degeneration & colonic dilatation
20
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Dilated ahaustral colon with
pseudopolyps & air-fluid levels
• Location: Transverse colon (least dependent part in
supine position)
• Other general features
o Most severe, life-threatening complication of
inflammatory bowel disease
o More common in ulcerative colitis (1.6-13% cases)
o May be the initial manifestation of ulcerative colitis
o Most common cause of death directly related to
ulcerative colitis
o Diagnosis: Based on clinical status of patient +
radiographic evidence
o Precipitating factors of toxic megacolon
• Endoscopy; use of opiates & anticholinergic drugs
• Progressive metabolic alkalosis; aerophagia
• Radiography
o Marked colonic dilatation is hallmark
• Transverse colon dilatation most common
(because least dependent on supine view)
• i Colon caliber on serial radiographs
• Mean diameters of dilated segments (8.2-9.2 em)
• Absolute colonic diameter: Not a diagnostic
criterion
o "Mucosal islands" or "pseudopolyps": Common
finding (indicate severe disruption of mucosa)
o Radiologically thick bowel wall (due to subserosal +
omental edema), pathologically, wall is thin
o Radiolucent stripe parallel to colon: Pericolic fat line
o Loss of haustral pattern
• Due to profound inflammation + ulceration
• Presence of normal haustra excludes diagnosis
o ± Air-fluid levels in colon; ± Small bowel distention
o Pneumatosis coli ± pneumoperitoneum
CT Findings
• Distended colon filled with air, fluid, blood
• Distorted or absent haustral pattern
• Irregular nodular contour of colonic wall
• Intramural air ± blood
• ± Mesenteric abscess or pneumoperitoneum

DDx: Dilated Transverse Colon

Sigmoid Volvulus Colon Cancer Colon Cancer

 TOXIC MEGACOLON

Key Facts
Terminology Top Differential Diagnoses
• Acute transmural fulminant colitis with • Colonic obstruction
neuromuscular degeneration & colonic dilatation • Adynamic or paralytic ileus
Imaging Findings Pathology
• Best diagnostic clue: Dilated ahaustral colon with • Ulcerative colitis (most common), other colitides
pseudopolyps & air-fluid levels
• Presence of normal haustra excludes diagnosis Diagnostic Checklist
• (Barium enema: Contraindicated, 1 risk perforation) • Check prior history of underlying colonic pathology

• Gender: Males less than females (M < F)

Imaging Recommendations
• Plain x-ray abdomen: Supine & lateral decubitus views Natural History & Prognosis
• Helical NECT • Complications: Perforation, abscess, peritonitis
• (Barium enema: Contraindicated, 1risk perforation) • Prognosis
o Good: After colectomy without complications
o Poor: With perforation & complications
I DIFFERENTIAL DIAGNOSIS Treatment
Colonic obstruction • Surgery (colectomy); treat complications
• Etiology: Carcinoma (55%); volvulus (11%)
• Usually subacute or chronic in onset
• Gas + stool-filled colon to point of obstruction I DIAGNOSTIC CHECKLIST
• Retained haustral pattern excludes toxic megacolon
Adynamic or paralytic ileus
Consider
• Check prior history of underlying colonic pathology
5
• Etiology: Post-op, medications, post injury, ischemia 21
• Dilated small & large bowel loops up to rectum Image Interpretation Pearls
• Normal haustral pattern excludes toxic megacolon • Extensive ahaustral colonic dilatation with air-fluid
levels & "mucosal islands" or "pseudopolyps"

I PATHOLOGY
I SELECTED REFERENCES
General Features 1. Halpert RD: Toxic dilatation of the colon. Radiologic
• Etiology Clinics of North America 25: 147-155, 1987
o Ulcerative colitis (most common), other colitides 2. Truelove SC et al: Toxic megacolon: Part 1. Pathogenesis,
o Pseudomembranous & ischemic colitis diagnosis & treatment. Clinical Gastroenterology 10: 107:
o Amebiasis, strongyloidiasis, bacillary dysentery 114, 1981
3. Fazio VW: Toxic megacolon in ulcerative colitis and Crohn
o Typhoid fever, cholera, Behcet's syndrome
disease. Clinical Gastroenterology 9: 389-407, 1980
• Epidemiology
o Incidence: Seen in 1.6-13% of ulcerative colitis cases
o Medical & surgical mortality: 21.5%
I IMAGE GALLERY
Gross Pathologic & Surgical Features
• Grossly dilated colon + air & fluid; mucosal ulceration
• Absence of haustral pattern (thin bowel wall 2-3 mm)
Microscopic Features
• Transmural inflammation
• Large areas of denuded mucosa + edema
• Fissuring ulcers with extension to serosa

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Fever, pain, (Left) Axial CECT shows dilated transverse colon with pneumatosis,
tenderness, abdominal distension, bloody diarrhea intraluminal bleeding, sloughed mucosa. Toxic megacolon due to C.
• Lab-data: 1 WBC; 1 ESR;+ ve fecal occult blood test difficile colitis. (Right) Axial CECT shows generalized ileus. Ascending
+ descending colon are distended with blood + sloughed mucosa. C.
Demographics difficile colitis.
• Age: 20-35 years
 APPENDICITIS

Anatomic drawing of acute appendicitis. Note enlarged, Acute appendicitis on sonography. Sagittal color
inflamed appendix (arrow). Doppler sonogram of enlarged non-compressible
appendix demonstrates abnormal mural flow (arrow)
consistent with appendicitis.

ITERMINOlOGY Radiographic Findings

• Radiography
Definitions
o Appendicolith in 5-10% of patients
• Acute appendiceal inflammation due to luminal
5 obstruction and superimposed infection
o Air-fluid levels within bowel in RLQ
o Splinting
o Loss of right psoas margin
22
o Free peritoneal air very uncommon
I IMAGING FINDINGS o With perforation
General Features • Small bowel obstruction
• RLQ extraluminal gas
• Best diagnostic clue
• Displacement of bowel loops from RLQ
o Distended non-compressible appendix (~ 7 mm) on
US or CECT • Fluoroscopy
o Barium Enema
o Abnormal mural enhancement of appendix on
• Non-filling of appendix (normal in 1/3 of
CECT
patients)
o Periappendiceal fat stranding on CECT
• Focal mural thickening of medial wall of cecum
• Location: Cecal tip
("arrowhead deformity")
• Size
o Noncompressible appendix> 6 mm has sensitivity CT Findings
of 100%, but specificity of only 64% • NECT
o Noncompressible appendix> 7 mm has sensitivity o Dilated appendix ~ 7 mm
of 94% and specificity of 88% o Periappendiceal fat stranding
o Noncompressible appendix 6-7 mm equivocal size; o Appendicolith
increased flow on color Doppler in appendix • May be incidental finding
indicates positive study • Seen much more frequently on CT than on
• Morphology: Tip of appendix is often first site of radiography
inflammation and appendiceal perforation o With perforation
• Small bowel obstruction

DDx: Mimics of Appendicitis

Mesenteric Adenitis Ileocolitis PID Diverticulitis

 APPENDICITIS

Key Facts
Terminology • Ileocolitis
• Acute appendiceal inflammation due to luminal • Pelvic inflammatory disease
obstruction and superimposed infection • Cecal diverticulitis

Imaging Findings Pathology

• Dilated appendix::: 7 mm; abnormal enhancement of
appendiceal wall on CECT; appendicolith mayor
may not be present; focal bowel wall thickening of
cecal tip
• In pediatric patients, thin young adults & pregnant
patients: US first imaging method, to avoid excessive
radiation
• CT performed for patients with inconclusive US, if
perforation suspected or if obese
Top Differential Diagnoses
• Mesenteric adenitis
• General path comments: Obstructed appendiceal
lumen: Appendicolith or hypertrophied Peyer
patches; pus-filled lumen; thickened appendiceal wall
with infiltration by inflammatory cells
Clinical Issues
• Periumbilical pain migrating to RLQ; peritoneal
irritation @ McBurney point; atypical signs in 1/3 of
patients
• Nonspecific presentation more common in young
children

• Inflammatory fluid collections demonstrating

mass effect, most commonly in RLQ or dependent
pelvis (cul-de-sac)
• CECT
o Dilated appendix::: 7 mm; abnormal enhancement
of appendiceal wall on CECT; appendicolith mayor
may not be present; focal bowel wall thickening of
cecal tip
• Sensitivity 95%, specificity 95%
Ultrasonographic Findings
• Real Time
o Non-compressible appendix::: 7mm
o Sonographic "McBurney sign" with focal pain over
appendix
o Shadowing, echogenic appendicolith
o RLQ fluid, phlegmon, abscess
• Color Doppler
o Flow within wall of appendix is abnormal,
indicating inflammation
• Sensitivity 85%, specificity 90%
Imaging Recommendations
• Best imaging tool
o In pediatric patients, thin young adults & pregnant
patients: US first imaging method, to avoid excessive
radiation
o CT performed for patients with inconclusive US, if
perforation suspected or if obese
o CT procedure of choice for
• Elderly: Consider cecal or appendiceal tumor
• Subacute symptoms or palpable mass
• Helps differentiate inflammation, abscess, tumor
• Protocol advice
o Oral contrast alone or rectal contrast alone may be
given
o NECT may be performed in patients with ample
intraperitoneal fat
o CECT
• Visualize early appendicitis (abnormal mural
enhancement)
• Diagnose perforation with non-enhancement of
appendix & surrounding inflammation or abscess
I DIFFERENTIAL DIAGNOSIS
Mesenteric adenitis
• Enlarged and clustered lymphadenopathy in
mesentery and RLQ
• Normal appendix
• May have ileal wall thickening due to GI involvement 5
• Pain when pressure applied with US transducer over
nodes 23
• Diagnosis of exclusion as appendicitis (especially
perforated appendicitis) may have enlarged mesenteric
nodes
Ileocolitis
• Crohn disease or infectious (e.g., Yersinia)
• US: Mural thickening of cecum and terminal ileum;
increased mural flow on color Doppler
• CECT: Submucosal edema of cecum and terminal
ileum; surrounding cecal inflammation
Pelvic inflammatory disease
• Complex adnexal mass
• Dilated fallopian tube with fluid-fluid level
(pyosalpinx)
• "Indefinite uterus" sign with obscuration of posterior
wall of myometrium
Cecal diverticulitis
• Cecal diverticulum with mural thickening
• Pericecal inflammatory changes
• Thickening of lateral conal fascia
• Abscess in anterior pararenal space
Appendiceal tumor
• Soft tissue density mass infiltrating and/or obstructing
appendix
• Usually little surrounding infiltration
• Carcinoma; lymphoma; carcinoid
Cecal carcinoma
• May obstruct appendiceal orifice
• Appendix is dilated but no periappendiceal
inflammation
 APPENDICITIS
• Circumferential cecal mass and lymphadenopathy • Prognosis
suggest tumor rather than appendicitis o Excellent with early surgery

I PATHOLOGY I DIAGNOSTIC CHECKLIST

General Features Consider
• General path comments: Obstructed appendiceal • Mesenteric adenitis if appendix normal and nodes
lumen: Appendicolith or hypertrophied Peyer patchesi enlarged
pus-filled lumeni thickened appendiceal wall with
infiltration by inflammatory cells Image Interpretation Pearls
• Etiology: Obstruction of appendiceal lumen by • Distended non-compressible appendix :2: 7 mm
appendicolith or Peyer patches • Mayor may not have appendicolith
• Epidemiology: 7% of all individuals in western world • Peri appendiceal fat stranding on contrast
develop appendicitis during their lifetime enhancement

Gross Pathologic & Surgical Features

• Distended appendix with or without appendicolith I SELECTED REFERENCES
• Surrounding adhesions
1. Andersson RE: Meta-analysis of the clinical and laboratory
Microscopic Features diagnosis of appendicitis. Br J Surg. 91(1):28-37, 2004
2. O'Malley ME et al: US of gastrointestinal tract
• Pus in lumen abnormalities with CT correlation. Radiographies.
• Leukocyte infiltration of appendiceal wall 23(1):59-72,2003
• Mucosal ulceration 3. Paulson EK et al: Clinical practice. Suspected appendicitis.
• Necrosis if gangrenous N Engl J Med. 348(3):236-42, 2003
4. Wijetunga R et al: The CT diagnosis of acute appendicitis.
Staging, Grading or Classification Criteria Semin Ultrasound CT MR. 24(2):101-6, 2003
• Nonperforated 5. Jacobs JE et al: CT imaging in acute appendicitis:
5 o No evidence for necrosis and/or perforation techniques and controversies. Semin Ultrasound CT MR.
24(2):96-100, 2003
• Perforated
24 o May have surrounding periappendiceal abscess or 6. Lee JH: Sonography of acute appendicitis. Semin
Ultrasound CT MR. 24(2):83-90, 2003
soft tissue inflammation of mesentery and omentum
7. Horrow MM et al: Differentiation of perforated from
nonperforated appendicitis at CT. Radiology. 227(1):46-51,
2003
[CLINICAL ISSUES 8. Morgan AC: Unveiling appendicitis. Contemp Nurse.
15(1-2):114-7,2003
Presentation 9. Puylaert JB: Ultrasonography of the acute abdomen:
• Most common signs/symptoms gastrointestinal conditions. Radiol Clin North Am.
o Periumbilical pain migrating to RLQ; peritoneal 41(6):1227-42, vii, 2003
irritation @ McBurney pointi atypical signs in 1/3 of 10. Macari M et al: The acute right lower quadrant: CT
patients evaluation. Radiol Clin North Am. 41(6):1117-36, 2003
11. Neumayer L et al: Imaging in appendicitis: a review with
o Other signs/symptoms
special emphasis on the treatment of women. Obstet
• Anorexia, nausea, vomiting, diarrhea, possible Gynecol. 102(6):1404-9,2003
fever 12. Dixon MR et al: An assessment of the severity of recurrent
• Nonspecific presentation more common in young appendicitis. Am J Surg. 186(6):718-22; discussion 722,
children 2003
• Clinical profile 13. Morris KT et al: The rational use of computed tomography
o Highly variable and not reliable scans in the diagnosis of appendicitis. Am J Surg.
183(5):547-50,2002
o WBC mayor may not be elevated
14. Raman SS et al: Accuracy of nonfocused helical CT for the
Demographics diagnosis of acute appendicitis: a 5-year review. AJR Am J
Roentgenol. 178(6):1319-25,2002
• Age: All ages affected
15. Albiston E: The role of radiological imaging in the
• Gender: M = F diagnosis of acute appendicitis. Can J Gastroenterol.
16(7):451-63, 2002
Natural History & Prognosis 16. See TC et al: Appendicitis: spectrum of appearances on
• Treatment helical CT. Br J Radiol. 75(897):775-81, 2002
o Surgery if non-perforated or if minimal perforation 17. Bendeck SE et al: Imaging for suspected appendicitis:
o Percutaneous drainage if well-localized abscess> 3 negative appendectomy and perforation rates. Radiology.
em 225(1):131-6,2002
o Antibiotic therapy if peri appendiceal soft tissue 18. van Breda Vriesman AC et al: Epiploic appendagitis and
inflammation and no abscess omental infarction. Eur J Surg. 167(10):723-7,2001
19. Jones PF: Suspected acute appendicitis: trends in
• Complications
management over 30 years. Br J Surg. 88(12):1570-7, 2001
o Gangrene and perforationi abscess formation 20. Rosendahl K et al: Imaging strategies in children with
o Peritonitis; septicemia; liver abscess suspected appendicitis. Eur Radiol. 2004
o Pyelophlebitis
 APPENDICITIS

I IMAGE GAllERY
Typical
(Left) Color Doppler
sonography in acute
appendicitis demonstrates
marked hyperemia in wall of
appendix (arrow) consistent
with acute appendicitis.
(Right) Endovaginal coronal
view of right adnexa
demonstrates hyperemia of
appendix (arrow), consistent
with pelvic appendicitis.

Typical
(Left) Longitudinal sonogram
demonstrates enlarged (10
mm) appendix (open
arrows) with adjacent
hypoechoic inflammation
(arrows). (Right) Transverse
5
sonogram of appendix
25
demonstrates focal necrosis
of appendiceal wall (arrow)
and small adjacent abscess
(open arrow).

Typical
(Left) Axial CECT of
perforated appendicitis. Note
multiple calcified
appendicoliths (arrow) and
lack of enhancement of
appendiceal tip (open
arrow). (Right) Axial CECT of
perforated appendicitis. Note
marked surrounding
periappendiceal
inflammation (arrows).
 MUCOCELE OF THE APPENDIX

Axial CECT shows oval, thin-walled, calcified mass at Axial CECT shows oval, partially calcified thin-walled
the tip of the cecum. "cyst" near tip of cecum.

• 1 Risk of perforation, forming peritoneal implants

ITERMINOLOGY o Pseudomyxoma peritonei
Definitions • Due to rupture: Malignant> benign mucocele
• Chronic cystic dilatation of appendiceal lumen by • Peritoneal cavity filled with mucus seedlings
5 mucin accumulation o Myxoglobulosis
• Rare variant with multiple small globules
26 • Calcify & produce 1-10 mm mobile calcifications
I IMAGING FINDINGS • Differentiate from phleboliths & calcified nodes
Radiographic Findings
General Features
• Fluoroscopic guided barium enema
• Best diagnostic clue: Round or oval, thin-walled, cystic
o Appendix: Fails to fill on barium enema
mass near tip of cecum
o Cecum: Indented on its medial aspect by
• Size: 3-6 cm in diameter
smooth-walled globular mass
• Other general features
o Ileum: Terminal part is displaced
o Mucocele of appendix is a rare entity
o Classified into three groups based on histology CT Findings
• Focal or diffuse mucosal hyperplasia • NECT
• Mucinous cystadenoma o Mucocele
• Mucinous cystadenocarcinoma • Well-defined cystic mass RLQ (near water HU)
o Focal or diffuse mucosal hyperplasia • Calcification (curvilinear) within wall or lumen
• Resembles hyperplastic polyp of colon o Mucinous cystadenoma
• Does not perforate • Encapsulated low attenuation cyst
o Mucinous cystadenoma • Indistinguishable from retention mucocele
• A benign neoplasm o Mucinous cystadenocarcinoma
• Most common type of mucocele • Large irregular mass with thickened nodular wall
• 20% of cases perforate with mucus seeding • Components: Solid & cystic; Ca++ in solid area
o Mucinous cystadenocarcinoma o Pseudomyxoma peritonei
• One fifth as common as cystadenomas • Massive ascites + septations (heterogeneous HU)

DDx: Dilation or Mass of Appendix; RLQ Cystic Mass

..•..•.. ~ --.
,..........••
~: ..

•••
,.~
,

Appendiceal Abscess Appendiceal Ca

.....•

~
., "._>'~'
. 'I', • '._.~.. ,,,."

Appy. Lymphoma
"
'>~""

"."""

-~i_:..' ...;....-
.. ~
'-,$'

Cecal Carcinoma
 MUCOCELE OF THE APPENDIX

Key Facts
Terminology Top Differential Diagnoses
• Chronic cystic dilatation of appendiceal lumen by • Acute appendicitis (abscess)
mucin accumulation • Appendiceal carcinoma
• Ovarian cystic mass
Imaging Findings
• Best diagnostic clue: Round or oval, thin-walled, Pathology
cystic mass near tip of cecum • Obstructing lesions can cause mucocele formation
• Calcification (curvilinear) within wall or lumen • Associated abnormalities: Colonic adenocarcinoma
(6-fold risk)

• CECT: Loculated ascites; scalloped surface of liver +

spleen Gross Pathologic & Surgical Features
• Mucocele: Thin-walled, mucin-filled cystic structure
MR Findings
• Mucocele with t fluid content: Long T1 & T2 Microscopic Features
o T1WI: Hypointense • Mucoid material; malignant cells-cystadenocarcinoma
o T2WI: Hyperintense
• Mucocele with t mucin content: Short T1 & long T2
o T1WI & T2WI: Mucocele appears hyperintense I CLINICAL ISSUES
Ultrasonographic Findings Presentation
• Real Time • Most common signs/symptoms: Asymptomatic; pain
o Anechoic or cystic + internal echoes (septations) & tenderness RLQ; palpable mass
o Increased through transmission is characteristic • Complications: Rupture, torsion, bowel obstruction
o Complex cystic mass; ± calcification
Demographics 5
o Gravity-dependent echoes (inspissated mucus)
• Age: Mean age: SS years 27
Imaging Recommendations • Gender: M:F = 1:4
• NE + CECT, MR, us Natural History & Prognosis
• Mucocele & cystadenoma (good); carcinoma (poor)
I DIFFERENTIAL DIAGNOSIS Treatment
• Surgical resection
Acute appendicitis (abscess)
• More inflammatory changes
• Thick irregular abscess wall I SELECTED REFERENCES
Appendiceal carcinoma 1. Lim HK et al: Primary mucinous cystadenocarcinoma of
• Irregular mixed density mass (solid & cystic) the appendix: CT findings. AJR. 173: 1071-4, 1999
2. Kim SH et al: Mucocele of the appendix: Ultrasonographic
Appendiceal lymphoma and CT findings. Abdominal Imaging. 23: 292-6, 1998
• Soft tissue mass near tip of cecum 3. Madwell D et al: Mucocele of the appendix: Imaging
findings. AJR. 159: 69-72, 1992
Cecal carcinoma
• May cause dilated appendix
Ovarian cystic mass
I IMAGE GAllERY
• Distinguish by relation to broad ligament vs. cecal tip

!PATHOlOGY
General Features
• Etiology
o Obstructing lesions can cause mucocele formation
• Post appendicitis scarring (most common)
• Fecalith, appendiceal carcinoma, endometrioma
• Carcinoid, polyp, volvulus, Ca of cecum & colon
• Epidemiology: Seen in 0.3% appendectomy specimens
• Associated abnormalities: Colonic adenocarcinoma (Left) Axial CECT shows complex ascites with scalloped surface of
(6-fold risk) liver + spleen. Pseudomyxoma peritonei due to ruptured mucinous
cystadenocarcinoma of appendix. (Right) Axial CECT shows
pseudomyxoma peritonei.
 DIVERTICULITIS
Craphic shows sigmoid diverticula, luminal narrowing +
wall thickening (circular muscle hypertrophy). Penco/ic
abscess due to perforated diverticulum. Rectum spared.
[TERMINOLOGY
Definitions
• Inflammation or perforation of colonic diverticula,
5 which are acquired herniations of mucosa and
submucosa through muscular layers of bowel wall
28
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Small colonic outpouchings with
irregular wall thickening & pericolic fat stranding
• Location: Most common in sigmoid colon
• Size: Diverticula: Usually about 0.5-1.0 em
• Morphology: Saccular outpouchings of colon with
perforation, inflammation & abscess formation
• Other general features
o Most common colonic disease in Western world
o Diverticula occur mainly where vasa recta vessels
pierce muscularis propria, between mesenteric &
anti mesenteric taeniae
o Colonic diverticula are pseudodiverticula
• Mucosa + submucosa; no muscularis propria
o Diverticular diseas~ of colon represents a collection
or sequence of events
• Pre diverticular phase: Circular muscular
thickening of colonic wall (myochosis)
DDx: Wall Thickening, Pericolonic Infiltration
Sigmoid Cancer Radiation Colitis
Axial CECT shows a pericolonic abscess (arrow)
adjacent to the sigmoid colon, with luminal narrowing,
gas-filled diverticula, and pericolonic fat infiltration.
• Diverticulosis: Frank outpouchings (diverticula)
• Diverticulitis: Perforation + localized pericolic
inflammation or abscess
Radiographic Findings
• Diverticulosis: Fluoroscopic guided barium enema
(single contrast preferred)
o Immature diverticula
• En face: Resemble punctate ulcer
• In profile: Conical or triangular (1-2 mm high)
o Mature: Shape varies based on angle & degree of
barium filling
• In profile: Flask-like protrusion, long or large neck
• Diverticulum with long & narrow neck: Mimic
pedunculated polyp on air-contrast enema
• Diverticulum with large neck: Mimics sessile polyp
• En face: Ring shadow or round barium collection
• "Bowler hat" sign: Dome of hat points away from
bowel wall (diverticulum); toward lumen (polyp)
o In progressive disease: Due to muscular
hypertrophy, diverticula are irregular, lumen
narrowed with serrated or "cog-wheel" appearance
• Diverticulitis: Fluoroscopic guided water soluble
contrast enema (not recommended)
o Focal area of eccentric luminal narrowing caused by
pericolic or intramural inflammatory mass (abscess)
+ mucosal tethering
o Marked thickening + distortion of haustral folds
o Extraluminal contrast (due to peridiverticulitis)
Pseudomem. Colitis Epiploic Appendagitis
 DIVERTICULITIS
Key Facts
Terminology
• Inflammation or perforation of colonic diverticula,
which are acquired herniations of mucosa and
submucosa through muscular layers of bowel wall
Imaging Findings
• Best diagnostic clue: Small colonic outpouchings with
irregular wall thickening & pericolic fat stranding
• Location: Most common in sigmoid colon
• CT is very accurate in diagnosis (> 95%)
• Helical CT: Oral & IV ± rectal contrast for acutely ill
Top Differential Diagnoses
• Colon carcinoma
• Radiation colitis
• Ischemic colitis
• Pseudomembranous colitis (PMC)
• "Double-tracking": Longitudinal intramural
fistulous tract (connecting ruptured diverticula) is
parallel to sigmoid lumen
• Pericolonic fistulous tracts: Bladder, bowel, vagina
• Pericolonic collection: Abscess compresses colon
o Sigmoid colon obstruction with zone of transition
can mimic cancer
• Tethered or saw-toothed luminal configuration
suggests diverticular disease
CT Findings
• Diverticulosis
o Mural thickening of colon (4 to 15 mm)
o Multiple air or contrast or stool-containing
outpouchings (diverticula)
• Diverticulitis
o CT is very accurate in diagnosis (> 95%)
o Bowel wall thickening, fat stranding, thickened base
of sigmoid mesocolon, free fluid
o Long segment (> 10 em) of colonic involvement
o Pericolic abscess, sinus tracts, fistulas
o Intramural or abdomino-pelvic abscess
o "Arrowhead" sign: Due to edema at orifice of
inflamed diverticulum
o Inflammation usually localized to pericolonic area
o Free air + peritonitis (less common)
• Omentum acts as "band-aid" to limit spread
• Immunocompromised at 1 risk: Peritonitis/sepsis
o ± Gas or thrombus in mesenteric & portal veins
• Follow course of inferior mesenteric vein
o ± Liver abscesses
Ultrasonographic Findings
• Real Time
o Diverticulosis
• Thickened bowel wall (> 4 mm)
• Diverticula: Round or oval hypo-/hyperechoic foci
protruding from colonic wall with focal disruption
of normal layer ± acoustic shadows
o Diverticulitis
• Pericolic inflammation: 1 Echogenicity ±
ill-defined hypoechoic areas
• Pericolic abscess: Hypoechoic ± internal echoes
Pathology
• Most common complication of diverticulosis, in 30%
of patients with moderate diverticulosis
• Very common in Western society, rare in less
developed countries due to more processed food &
less fiber in diet
Clinical Issues
• Percutaneous abscess drainage can eliminate surgery
or allow elective one-step procedure in most cases
Diagnostic Checklist
• Check whether patient has signs & symptoms of
diverticulitis
• Long segment colonic involvement, extensive
inflammatory changes & absence of nodes or
metastases favors diverticulitis over colon cancer
Imaging Recommendations
• Helical CT: Oral & IV ± rectal contrast for acutely ill
• Diverticulosis: Single contrast barium enema
• Double contrast barium enema: Hard to distinguish
polyps from diverticula
5
I DIFFERENTIAL DIAGNOSIS 29
Colon carcinoma
• Asymmetric bowel wall thickening ± irregular surface
• Wall thickening, fat stranding & pericolonic
infiltration mimics diverticulitis
• CT findings favoring cancer
o Short segment involvement « 10 em)
o Wall thickness: More than 2 em
o Mesenteric lymphadenopathy
o Metastases
Radiation colitis
• Barium enema findings
o Acute radiation colitis & proctitis
• Disrupted or distorted mucosal pattern (due to
edema or hemorrhage)
o Chronic radiation colitis & proctitis
• Diffuse or focal narrowing with tapered margins
• Colonic stricture or fistula may be seen
• Widened presacral space (in profile view)
• CT findings
o More uniform wall thickening + luminal narrowing,
less peri colonic inflammation than diverticulitis
o Colonic luminal narrowing or stricture
o ± Sinuses or fistulas
• Diagnosis: History of radiation therapy
Ischemic colitis
• Usual sites
o Splenic flexure> recto-sigmoid junction
• Barium enema findings
o Thumbprinting (usually within 24 hrs after insult)
• Due to submucosal edema or hemorrhage
o Ulceration: Sloughing of mucosa (46-60% cases)
• Usually develops 1-3 weeks after onset of disease
 DIVERTICULITIS
• CT findings
o More uniform, extensive wall thickening & less
pericolonic infiltration than diverticulitis
o Bowel wall attenuation
• Hypoattenuation: Submucosal or diffuse edema
• Hyperattenuation: Submucosal or diffuse bleeding
o ± Pneumatosis & portomesenteric venous gas
• Diagnosis
o History of non occlusive vascular disease
• Hypoperfusion in elderly people
• Example: CHF, arrhythmia, shock & drugs
Pseudomembranous colitis (PMC)
• Synonym(s): Antibiotic colitis or C. difficile colitis
• CT findings
o Massive wall thickening, usually pancolonic
o Often transmural with pericolonic infiltration
o "Accordion" sign: Represents trapped enteric
contrast between thickened colonic folds
o Full recovery with early diagnosis, discontinuation
of offending antibiotic & metronidazole treatment
I PATHOLOGY
General Features
• General path comments
5 o Diverticulitis
• Most common complication of diverticulosis, in
30% of patients with moderate diverticulosis
30
• Etiology
o Diverticulitis: Due to fecal impaction at mouth of
diverticulum with subsequent perforation
o Contributing factors to development of diverticula
• Pressure gradient between lumen & serosa
(sigmoid): Narrowest of colon + 1 pressure +
dehydrated stool
• Bowel wall weakness: Between mesenteric &
antimesenteric taeniae
• Epidemiology
o Incidence
• 33-50% cases, over 50 years old have diverticulosis
• More than 50% have diverticulosis after 80 years
• Can occur in young adults « 30 years old)
o Very common in Western society, rare in less
developed countries due to more processed food &
less fiber in diet
• Associated abnormalities: Liver abscesses
Gross Pathologic & Surgical Features
• Outpouchings from sigmoid colon between taenia coli
Microscopic Features
• Diverticula: Mucosal herniation through a defect in
circular layer of muscle
• Diverticulitis: Perforation with inflammation &
micro-/macroabscess
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Diverticulosis
• Asymptomatic; pain & rectal bleeding (30% cases)
• Alternating constipation + diarrhea due to luminal
narrowing (circular muscle hypertrophy)
o Diverticulitis
• LLQ colicky pain, tenderness & palpable mass
• Fever, altered bowel habits
o Lab-data
• 1 WBC count; anemia; ± blood in stool
Demographics
• Age: 5th to 8th decade (peak); not rare in younger
• Gender: Equal in both males & females (M = F)
Natural History & Prognosis
• Complications
o Perforation, pericolonic abscess, fistula, sinus
o Obstruction, hemorrhage
o Peritonitis & sepsis (uncommon)
o Pylephlebitis (portal vein thrombus); liver abscesses
• Via mesenteric + portal vein ~ liver abscesses
• Prognosis: Early stages & after surgery (good)
Treatment
• High-fiber diet (preventive)
• Antibiotics, IV fluids, bowel rest
• Percutaneous abscess drainage can eliminate surgery or
allow elective one-step procedure in most cases
I DIAGNOSTIC CHECKLIST
Consider
• Check whether patient has signs & symptoms of
diverticulitis
Image Interpretation Pearls
• Bowel wall thickening, pericolic infiltration & fat
stranding affecting sigmoid colon
• Long segment colonic involvement, extensive
inflammatory changes & absence of nodes or
metastases favors diverticulitis over colon cancer
I SELECTED REFERENCES
1. Jang HJ et al: Acute diverticulitis of the cecum and
ascending colon: the value of thin-section helical CT
findings in excluding colonic carcinoma. AJRAm J
Roentgenol. 174(5):1397-402,2000
2. Horton KM et al: CT evaluation of the colon: inflammatory
disease. Radiographies. 20(2):399-418, 2000
3. Gore RM et al: Helical CT in the evaluation of the acute
abdomen. AJR 174: 901-13, 2000
4. Chin tap alii KN et al: Diverticulitis versus colon cancer:
differentiation with helical CT findings. Radiology.
210(2):429-35, 1999
5. Rao PM et al: Colonic diverticulitis: evaluation of the
arrowhead sign and the inflamed diverticulum for CT
diagnosis. Radiology. 209(3):775-9, 1998
6. Padidar AM et al: Differentiating sigmoid diverticulitis
from carcinoma on CT scans: mesenteric inflammation
suggests diverticulitis. AJRAm J Roentgenol. 163(1):81-3,
1994
7. Balthazar EJ et al: Limitations in the CT diagnosis of acute
diverticulitis: comparison of CT, contrast enema, and
pathologic findings in 16 patients. AJRAm J Roentgenol.
154(2):281-5, 1990
 DIVERTICULITIS
I IMAGE GALLERY

(Left) Single contrast BE

shows an intramural track of
barium (arrow) paralleling
the sigmoid lumen, due to
submucosal spread of
infection from perforated
diverticulum. (Right) Single
contrast water soluble
enema shows marked
distortion of sigmoid lumen
mostly due to circular
muscle hypertrophy.
Broad-based intramural +
extrinsic mass effect (arrows)
due to diverticulitis.

Typical
(Left) Axial CECT shows
shows extensive infiltration of
pelvic/pericolic fat. Bladder
has gas-fluid level and a
fistula (arrow) to the sigmoid
colon. (Right) Axial CECT
5
shows diverticulosis of
31
descending colon. Perforated
diverticulitis resulted in
extensive abscess in
retroperitoneum (arrow),
with dissection throughout
the abdominal wall (open
arrow).

(Left) Axial CECT shows

sigmoid diverticulitis with
irregular luminal narrowing +
mild pericolic infiltration;
subacute diverticulitis.
(Right) Axial CECT in patient
with subacute diverticulitis
shows a pyogenic liver
abscess due to bacterial
seeding from inferior
mesenteric-portal vein.
 EPIPLOIC APPENDAGITIS

Graphic shows two normal epiploic appendages and Axial CECT shows oval pericolonic fatty nodule (arrow)
one that is twisted and infarcted (arrow). with hyperdense ring and surrounding inflammation.

ITERMINOLOGY o Typically seen in obese people in 2nd-5th decades of

life, can occur in children
Abbreviations and Synonyms o Benign self-limiting disease
• Epiploic appendagitis (EA) o Torsion of epiploic appendage
5 Definitions
o Spontaneous venous thrombosis of draining
appendageal vein
32 • Acute inflammation or infarction of epiploic o Rarely diagnosed clinically but has highly
appendages characteristic CT features
o Radiological (CT) potential misdiagnosis of EA as:
Diverticulitis or appendicitis
I IMAGING FINDINGS CT Findings
General Features • Normal epiploic appendages
• Best diagnostic clue: Small oval pericolonic fatty o Small lobulated masses of pericolonic fat
nodule with hyperdense ring + surrounding • Rectosigmoid most evident
inflammation o Seen on CT scans only when outlined by ascites
• Location • 1-4 cm, oval-shaped, fat density paracolic lesion with
o Left lower quadrant> right lower quadrant adjacent fat stranding
• Rectosigmoid junction (57%); ileocecal (26%); • Thickened & compressed bowel wall
ascending colon (9%) • Thickened visceral & parietal peritoneum
• Transverse colon (6%); descending colon (2%); • ± Central increased attenuation "dot" within inflamed
occasionally appendix appendage (indicates thrombosed vein)
• Morphology: Epiploic appendages: Small adipose • Hyperattenuating ring sign: Characteristic finding of
structures protruding from serosal surface of colon EA on postcontrast
• Other general features o Pericolonic round fat-containing mass + thin
o Uncommon inflammatory & ischemic condition hyperattenuating ring
o Uncommon cause of acute abdomen o Ring: Thickened visceral peritoneum of inflamed
epiploic appendage
o May calcify when infarcted

DDx: Pericolonic Infiltration

Appendicitis Pseudomem. Colitis Ulcerative Colitis

 EPIPLOIC APPENDAGITIS
Key Facts
Terminology
• Acute inflammation or infarction of epiploic
appendages
Imaging Findings
• Best diagnostic clue: Small oval pericolonic fatty
nodule with hyperdense ring + surrounding
inflammation
• Left lower quadrant> right lower quadrant
• ± Central increased attenuation "dot" within inflamed
appendage (indicates thrombosed vein)
• Pericolonic round fat-containing mass + thin
hyperattenuating ring
• Infarcted EA: Probably accounts for otherwise
unexplained smooth calcified "stones" occasionally
found in dependent peritoneal recesses
• Infarcted EA: Probably accounts for otherwise
unexplained smooth calcified "stones" occasionally
found in dependent peritoneal recesses
MR Findings
• T1 & T2WI breath-hold spoiled gradient echo (SGE)
images
o Increased signal lesion + hypointense central dot +
thin hypointense ring
• T1 C+ fat suppressed gradient echo image
o Increased enhancement of ring
Ultrasonographic Findings
• Real Time
o Solid hyperechoic noncom pres sible ovoid mass
adherent to colonic wall
o Surrounded by a hypoechoic ring (corresponds to t
HU ring on CT scan)
Imaging Recommendations
• Helical CECT
I DIFFERENTIAL DIAGNOSIS
Diverticulitis
• Most common complication of diverticulosis
• Barium enema findings
o Focal eccentric luminal narrowing
o Marked thickening & distortion of haustral folds
o Colonic obstruction with zone of transition
o "Double-track": Intramural fistulous tract
• CT findings
o Location: Most common in sigmoid colon
o Bowel wall & fascial thickening, luminal narrowing
o Pericolonic fat stranding, free fluid & air
o Pericolic inflammatory changes
• Abscess, sinuses, fistulas
o "Arrowhead" sign: Due to diverticular orifice edema
• Clinically simulates epiploic appendagitis
Appendicitis
• Best imaging clue on CT
Top Differential Diagnoses
• Diverticulitis
• Appendicitis
• Pseudomembranous colitis (PMC)
• Ulcerative colitis
Pathology
• Appendages: Small pouches of peritoneum
protruding from serosal surface of colon filled with
fat + small vessels
Diagnostic Checklist
• Differentiate epiploic appendagitis especially from
diverticulitis (LLQ) & appendicitis (RLQ)
• Pericolonic round fatty mass (1-4 em) with
hyperdense rim (most common in rectosigmoid area)
• Not limited to left colon or elderly
o Appendicolith (usually calcified) within distended
tubular appendix
• Distended enhancing appendix with surrounding
inflammation (fat stranding)
• Wall thickening of cecum or terminal ileum
• Right lower quadrant (RLQ) lymphadenopathy
• In perforated cases
o Fluid collection most commonly in RLQ or in
5
dependent pelvis (Cul-de-sac)
33
o Abscess, small-bowel obstruction
• Ultrasound findings
o Echogenic appendicolith with posterior shadowing
o Noncompressible blind-ending tubular structure
over 7 mm in diameter
o Fluid or abscess collection in RLQ
• Right colonic EA clinically may simulate appendicitis
Pseudomembranous colitis (PMC)
• Synonym(s): Antibiotic colitis or C. difficile colitis
• Usually involves entire colon (pancolitis)
• CT findings
o Colonic wall thickening, nodularity, thumbprinting
o "Accordion" sign: Represents trapped enteric
contrast between thickened colonic folds
o Ascites common in PMC
o Full recovery with early diagnosis, discontinuation
of offending antibiotic & treatment with
metronidazole
Ulcerative colitis
• Pathology: Continuous, not transmural, pseudopolyps,
crypt microabscesses
• Classic imaging appearance
o Pancolitis with decreased haustration & multiple
ulcerations on barium enema
• Colorectal narrowing; 1 presacral space> 1.S em
• "Mucosal islands" or "inflammatory pseudopolyps"
• Diffuse & symmetric wall thickening of colon
• Backwash ileitis: Distal ileum involvement (10-40%)
• Chronic phase
o "Lead-pipe" colon: Rigid colon with loss of haustra
 EPIPLOIC APPENDAGITIS
I PATHOLOGY
General Features
• General path comments
o Appendages: Small pouches of peritoneum
protruding from serosal surface of colon filled with
fat + small vessels
• Seen along free tenia & tenia omentalis between
cecum & sigmoid colon
• Etiology
. 0 Torsion & venous thrombosis of appendages
o Predisposing factors for torsion & infarction of
epiploic appendages
• Precarious blood supply from colic arterial
branches
• Pedunculated morphologyi 1 mobility & obesity
• Epidemiology
o Though uncommon, not as rare as assumed
o Seen in 2.3-7.1 % of clinically suspected colonic
diverticulitis
o Reported in 1.0% of suspected appendicitis cases
Gross Pathologic & Surgical Features
• Round fat containing paracolic lesion, fat stranding,
thickened wall
Microscopic Features
5 • Visceral peritoneal lining of inflamed epiploic
appendage covered with a fibrinoleukocytic exudates
• Fat necrosis within appendage
34
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Sudden onset of focal abdominal pain
• Usually left or right lower quadrant
o Pain worsening with: Coughing, deep breathing,
abdominal stretching
o Symptoms usually subside within one week of onset
o Physical exam
• Localized tenderness, some guarding, no rigidity
o Lab-data
• WBC count (normal or slightly 1 in most cases)
Demographics
• Age: 2nd-Sth decades (obese people)
• Gender: Equal in both males & females (M = F)
Natural History & Prognosis
• Complications of epiploic appendages
o Recurrent episodes of inflammation (unusual)
o Intraperitoneal loose bodies
o Infarction
• Prognosis
o Benign self-limiting process with spontaneous
resolution within 1 week
o Good: After medical or surgical treatment
Treatment
• Medical: Conservative treatment with analgesics
• Surgical: Simple ligation & excision of infarcted
epiploic appendage
o Rarely required if accurately diagnosed
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate epiploic appendagitis especially from
diverticulitis (LLQ) & appendicitis (RLQ)
Image Interpretation Pearls
• Pericolonic round fatty mass (1-4 cm) with hyperdense
rim (most common in rectosigmoid area)
• Not limited to left colon or elderly
I SELECTED REFERENCES
1. van Breda Vriesman AC: The hyperattenuating ring sign.
Radiology. 226(2):556-7, 2003
2. Ghosh BC et al: Primary epiploic appendagitis: diagnosis,
management, and natural course of the disease. Mil Med .
168(4):346-7, 2003
3. Chowbey PK et al: Torsion of appendices epiploicae
presenting as acute abdomen: laparoscopic diagnosis and
therapy. Indian] Gastroenterol. 22(2):68-9, 2003
4. Hollerweger A et al: Primary epiploic appendagitis:
sonographic findings with CT correlation. ] Clin
Ultrasound. 30(8):481-95, 2002
5. Son H] et al: Clinical diagnosis of primary epiploic
appendagitis: differentiation from acute diverticulitis. ]
Clin Gastroenterol. 34(4):435-8, 2002
6. van Breda Vriesman AC et al: Epiploic appendagitis and
omental infarction: pitfalls and look-alikes. Abdom
Imaging. 27(1):20-8, 2002
7. Chung SP et al: Primary epiploic appendagitis. Am] Emerg
Med. 20(1):62, 2002
8. Sirvanci M et al: Primary epiploic appendagitis: MRI
findings. Magn Reson Imaging. 20(1):137-9, 2002
9. Legome EL et al: Epiploic appendagitis: the emergency
department presentation.] Emerg Med. 22(1):9-13, 2002
10. Horton KM et al: CT evaluation of the colon: inflammatory
disease. Radiographies. 20(2):399-418, 2000
11. Rao PM et al: Case 6: primary epiploic appendagitis.
Radiology. 210(1):145-8, 1999
12. Habib FA et al: Laparoscopic approach to the management
of incarcerated hernia of appendices epiploicae: report of
two cases and review of the literature. Surg Laparosc
Endosc. 8(6):425-8, 1998
13. Rao PM et al: Misdiagnosis of primary epiploic
appendagitis. Am] Surg. 176(1):81-5, 1998
14. Rao PM et al: Primary epiploic appendagitis: evolutionary
changes in CT appearance. Radiology. 204(3):713-7, 1997
15. Rioux M et al: Primary epiploic appendagitis: clinical, US,
and CT findings in 14 cases. Radiology. 191(2):523-6, 1994
16. Ghahremani GG et al: Appendices epiploicae of the colon:
radiologic and pathologic features. Radiographics.
12(1):59-77, 1992
17. Derchi LE et al: Appendices epiploicae of the large bowel.
Sonographic appearance and differentiation from
peritoneal seeding.] Ultrasound Med. 7(1):11-4, 1988
 EPIPLOIC APPENDAGITIS

I IMAGE GALLERY
(Left) Axial CECT shows
ascites outlining the fat
density of normal epiploic
appendages (arrows) of the
sigmoid colon. (Right) Axial
CECT in a patient with
suspected diverticulitis
shows an oval pericolonic fat
density nodule (arrow) with
a hyperdense ring; epiploic
appendagitis.

(Left) Axial CECT shows a fat

density nodule (arrow) with
a hyperdense ring and
surrounding inflammation.
(Right) Axial NECT shows
calcified epiploic appendage
5
(arrow) of the descending
35
colon. Such infarcted
appendages may detach
from the colon and result in
loose bodies in the
peritoneal cavity.

(Left) Axial CECT shows

epiploic appendagitis of the
hepatic flexure (arrow) with
typical findings. (Right) Axial
CECT shows infiltrated fat
near the hepatic flexure just
cephalan to the inflamed or
infarcted epiploic
appendage.
 ISCHEMIC COLITIS

Graphic shows luminal narrowing and wall thickening Single-contrast BE shows narrowed lumen of the splenic
neilr the splenic flexure, the "watershed" area between flexure with "thumbprinting" (thickened haustral folds)
the vascular distribution of the SMA and IMA. due to submucosal edema or hemorrhage. Elderly
patient with heart disease.

• Hemorrhagic, septic or hypovolemic shock

ITERMINOLOGY • Congestive heart failure (CHF); drugs like digitalis
Definitions o 20% Colonic ischemia are proximal to obstruction
• Compromise of mesenteric blood supply leading to • Colon cancer, volvulus, closed loop obstruction
5 colonic injury o Common cause of abdominal pain in elderly with
history of heart disease
36 o Most common form is segmental (90%) or pancolitis
I IMAGING FINDINGS o Usually a partial mural (nontransmural)
superficial mucosal ischemia
&

General Features o Spectrum of diseases caused by colonic ischemia

• Best diagnostic clue: Evidence of pneumatosis, • Reversible or transient ischemic colitis (> frequent)
mesenteric venous gas, symmetric bowel wall • Colonic stricture, gangrene of colon & perforation
thickening or thumb printing on CT
Radiographic Findings
• Location
o Commonly watershed segments of colon • Radiography
o Plain x-ray abdomen (supine view)
• Splenic flexure: Junction of SMA & IMA (Griffith
point) • Normal or nonspecific ileus
• Thumbprinting (submucosal edema or bleeding)
• Rectosigmoid: Junction of IMA & hypogastric
• Luminal narrowing or transverse ridging (spasm)
artery (Sudeck point)
• Ahaustralloops (rare)
• Left-side colon: Typical in elderly with I perfusion
• Fluoroscopic guided barium enema
• Right-side: Young patients (I collateral blood
o Hallmark: Serial change on studies performed over
supply); chronic renal failure
days, weeks or months
• Other general features
o Thumbprinting (usually within 24 hrs after insult)
o Most common vascular disorder of GI tract
• Smooth, round, polypoid scalloped filling defects
6 Most common cause of colitis in elderly & is often
along lumen (submucosal edema or hemorrhage)
self limiting
• Most consistent & characteristic finding-7S% cases
o Major predisposing cause in elderly: Nonocclusive
• Occurs within first 24 hrs, resorbs in less than a
vascular disease (hypoperfusion)
week or may persist for weeks

DDx: Wall Thickening; Pericolonic Infiltration

. __ t •••

,..
, 'T_ I -..
••.. ).if

••• ,~. or J

~
Diverticulitis Pseudomem. Colitis Ulcerative Colitis Colon Cancer
 ISCHEMIC COLITIS
Key Facts
Terminology
• Compromise of mesenteric blood supply leading to
colonic injury
Imaging Findings
• Best diagnostic clue: Evidence of pneumatosis,
mesenteric venous gas, symmetric bowel wall
thickening or thumbprinting on CT
• Commonly watershed segments of colon
• Thumbprinting (usually within 24 hrs after insult)
• Ulceration: Sloughing of mucosa (46-60% cases)
• Bowel wall thickening (normal range 3-5 mm)
• Hypoattenuation: Submucosal or diffuse edema
• Hyperattenuation: Submucosal or diffuse bleeding
• ± Pneumatosis
• ± Portomesenteric venous gas
• May also seen in other inflammatory bowel
diseases or infectious colitides
o Transverse ridging: Less common finding-13% cases
• Parallel, symmetric thickened folds running
perpendicular to bowel lumen
• Caused by edema or spasm; early finding &
o
usually resolves rapidly
Ulceration: Sloughing of mucosa (46-60% cases)
• Longitudinal/discrete; superficial/deep; small/large
• Usually develop 1-3 weeks after onset of disease
o Intramural barium: Unusual (sloughing of necrotic
portion of wall ~ tracking of barium intramurally)
o Stricture: 12% cases heal with stricture formation
CT Findings
• NECT
o Bowel wall thickening (normal range 3-5 mm)
• Circumferential, symmetric wall thickening ±
thumbprinting
• Due to submucosal edema or hemorrhage
o Bowel wall attenuation
• Hypoattenuation: Submucosal or diffuse edema
• Hyperattenuation: Submucosal or diffuse bleeding
• Heterogeneous: Outer serosa & muscular layers
o ± Luminal narrowing or dilatation & air-fluid levels
o Loss of haustral pattern (rare); pericolic streakiness;
paracolic fluid collections
o ± Pneumatosis
• Small gas bubbles within ischemic bowel wall
• Circumferential or band like pneumatosis
o ± Portomesenteric venous gas
• Portal venous gas collects in periphery of liver
• CECT
o Double halo or target sign: Concentric layers of low
& high attenuation
• Enhancement of mucosa & serosa (hyperemia or
hyperperfusion during recovery)
• Nonenhancement of submucosa (due to
submucosal edema or hemorrhage)
o ± Thrombus within splanchnic vessels
Ultrasonographic Findings
• Color Doppler
Top Differential Diagnoses
• Diverticulitis
• Pseudomembranous colitis (PMC)
• Ulcerative colitis (UC)
• Granulomatous colitis (Crohn disease)
• Colon carcinoma
Pathology
• Nonocclusive vascular disease (in elderly people)
• Hypoperfusion: Predisposing factors
• Hypotensive episodes: Hemorrhagic, cardiogenic or
septic shock
• CHF, arrhythmia, drugs, trauma
Diagnostic Checklist
• Check for history of cardiac, bowel, renal problems &
hypotensive medication use in elderly people
o Hypoechoic thickening of bowel wall
o Absence of arterial flow in wall of ischemic colon
Angiographic Findings
• Usually not helpful in diagnosis
o Ischemic colitis: Usually nonocclusive ischemia
Imaging Recommendations 5
• Helical NE + CECT; plain x-ray abdomen
• Single contrast barium enema (for chronic disease) 37
I DIFFERENTIAL DIAGNOSIS
Diverticulitis
• Most common complication of diverticulosis
• Barium enema findings
o Focal eccentric luminal narrowing
o Marked thickening & distortion of haustral folds
o Colonic obstruction with zone of transition
o "Double-tracking": Longitudinal intramural fistulous
tract
• CT findings
o Location: Most common in sigmoid colon
o Bowel wall & fascial thickening; fat stranding; free
fluid & air
o Pericolic inflammatory changes
• Abscess, sinuses, fistulas
o "Arrowhead" sign: Due to diverticular orifice edema
o Focal area of eccentric luminal narrowing
Pseudomembranous colitis (PMC)
• Synonym(s): Antibiotic colitis or C. difficile colitis
• Usually involves entire colon (pancolitis)
• Barium enema (contraindicated in acutely ill)
o Small, irregular plaques on mucosal surface
• Represent pseudomembranes
o Small, subtle elevated, round nodules
• Single contrast study: Shows thumb printing
indistinguishable from ischemic colitis
• CT findings
o Colic wall thickening & nodularity
o "Accordion" sign: Represents trapped enteric
contrast between thickened colonic folds
 ISCHEMIC COLITIS
o Ascites common in PMC
• Full recovery with early diagnosis, discontinuation of
offending antibiotic & treatment with metronidazole
Ulcerative colitis (UC)
• Pathology: Continuous, not transmural, pseudopolyps,
crypt microabscesses
• Classic imaging appearance
o Pancolitis with decreased haustration & multiple
ulcerations on barium enema
• Colorectal narrowing; i presacral space> 1.5 em
• "Mucosal islands" or "inflammatory pseudopolyps"
• Diffuse & symmetric wall thickening of colon
o Ischemic colitis usually shows segmental (watershed
areas) bowel wall thickening & thumbprinting
• Backwash ileitis: Distal ileum involvement (10-40%)
• Chronic phase
o "Lead-pipe" colon: Rigid colon with loss of haustra
Granulomatous colitis (Crohn disease)
• Barium enema findings
o Cobblestoning: Longitudinal & transverse ulceration
produce a paving stone appearance
o Segmental in distribution
• Involve both colon & small-bowel (60% cases)
• Isolated to colon (20% cases)
o Transmural, skip lesions, sinuses, fissures, fistulas
5 • CT findings
o Bowel wall thickening (1-2 em)
o "Creeping fat" or mesenteric fibrofatty proliferation
38
o Enlarged mesenteric lymph nodes
o "Comb" sign: Hypervascularity (active disease)
Colon carcinoma
• Asymmetric mural thickening with irregular surface
• Classic annular "apple core" lesion
o Circumferential bowel narrowing + mucosal
destruction with shelf-like, overhanging borders
o High grade obstruction + ischemia shows proximal
bowel dilatation with thumbprinting
• Extracolonic tumor extension
o Strands of soft tissue: Serosal surface ~ pericolic fat
o Loss of fat planes between colon & adjacent muscles
I PATHOLOGY
General Features
• General path comments
o Normal mesenteric vascular anatomy
• Superior mesenteric artery (SMA):Vascular supply
from 3rd part of duodenum to splenic flexure
• Inferior mesenteric artery (IMA): Splenic flexure to
rectum
• Etiology
o Nonocclusive vascular disease (in elderly people)
o Hypoperfusion: Predisposing factors
• Hypotensive episodes: Hemorrhagic, cardiogenic
or septic shock
• CHF, arrhythmia, drugs, trauma
• Arteriosclerotic disease, chronic renal failure
• Vasculitis, colonic obstruction
• Epidemiology: Mortality rate: 7% of cases
Gross Pathologic & Surgical Features
• Segmental or focal; localized or diffuse
• Thick bowel wall; dark red or purple
o Edematous, hemorrhagic, ulcerated
Microscopic Features
• Mucosal erosions, ulceration, necrosis
• Submucosal edema, hemorrhage
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Mild or severe abdominal pain
o Rectal bleeding, bloody diarrhea, hypotension
• Lab-data
o i Leukocytosis; positive guaiac stool test
o Negative blood cultures; EKG changes may be seen
Demographics
• Age: Usually elderly age group (> 50 years)
• Gender: Equal in both males & females (M = F)
Natural History & Prognosis
• Complications
o Transmural bowel infarction ~ perforation ~ death
• Prognosis
o Partial mural ischemia: Good prognosis
o Transmural infarction: Poor prognosis
Treatment
• Partial mural ischemia (nonocclusive type)
o Conservative medical treatment
• Transmural infarction: Surgical resection
I DIAGNOSTIC CHECKLIST
Consider
• Check for history of cardiac, bowel, renal problems &
hypotensive medication use in elderly people
Image Interpretation Pearls
• Segmental bowel wall thickening in watershed areas,
thumbprinting, pneumatosis, portal venous gas
I SELECTED REFERENCES
1. Wiesner W et al: CT of acute bowel ischemia. Radiology.
226(3):635-50, 2003
2. Horton KMet al: Volume-rendered 3D CTof the
mesenteric vasculature:normal anatomy, anatomic
variants, and pathologic conditions. Radiographies.
22(1):161-72, 2002
3. Horton KMet al: Multi-detector row CTof mesenteric
ischemia: can it be done? Radiographies.21(6):1463-73,
2001
4. Horton KMet al: CTevaluation of the colon: inflammatory
disease.Radiographies.20(2):399-418, 2000
5. BalthazarEJet al: Ischemic colitis: CTevaluation of 54
cases.Radiology.211(2):381-8, 1999
6. Iida M et al: Ischemic colitis: serial changes in
double-contrast barium enema examination. Radiology.
159(2):337-41, 1986
 ISCHEMIC COLITIS

IIMAGE GALLERY

(Left) Single-contrast BE in a
60 year old man with
chronic heart disease, shows
strictures of distal transverse
+ proximal descending colon
due to subacute colonic
ischemia. (Right) Axial CECT
in a 60 year old paUent with
subacute colonic ischemia
shows wall thickening
. (arrows), submucosal
edema, and luminal
narrowing of the colon.

Variant
(Left) Axial CECT of a patient
24 hours post abdominal
trauma (motor vehicle crash)
shows portal venous gas
(arrow). (Right) Axial CECT 5
shows intramural and
mesenteric venous gas. At 39
surgery, patient had
"degloving" injury (serosal
tear + devascularization)
with cecal infarction.

Variant
(Left) Axial CECT shows a
mass in the pancreatic head
with a biliary stent (arrow).
The superior mesenteric
artery + vein (open arrow)
are encased and narrowed.
Gas is present in the colon
wall. (Right) Axial CECT in
patient with pancreatic
cancer. Intramural +
mesenteric venous (arrow)
gas are present due to colon
infarction.
 COLONIC POLYPS

Graphic shows tubulovil/ous adenoma on a long stalk Single contrast BE shows tubulovil/ous adenoma with a
and a small sessile polyp. large "head" (arrow) and a long stalk (open arrow).
Small sessile polyp (curved arrow) also noted.

• Dependent wall: Radiolucent filling defect

ITERMINOLOGY • Nondependent wall: Ring shadow with
Definitions barium-coated white rim
• A protruding, space-occupying lesion within the • "Bowler hat" sign: Dome of hat points toward
5 colonic lumen lumen of bowel (en face view); brim and dome of
hat represents base and head of polyp
40 o Pedunculated polyps
IIMAGING FINDINGS • "Mexican hat" sign: Characterized by a pair of
concentric rings; outer and inner ring represents
General Features head and stalk of polyp
• Best diagnostic clue: Radiolucent filling defect, o Tubular adenomatous polyps
contour defect or ring shadow • Small size; pedunculated polyps
• Location: Cecum (4%); ascending colon (6%); hepatic • Minor degree of villous changes
flexure (4%); transverse (2%); splenic flexure (8%); o Tubulovillous adenomatous polyps
descending (20%); sigmoid (41%); rectum (23%) • Medium size; sessile polyps
• Morphology • Fine nodular or reticular surface pattern
o Sessile polyps: Broad base with little or no stalk • Filling of barium within interstices of adenoma
o Pedunculated polyps: Arise from narrow stalk o Villous adenomatous polyps
• Other general features • Larger size; sessile polyps
o 2 Types of colon polyps • Barium trapped between frond-like projections ~
• Neoplastic: Adenomatous (tubular, tubulovillous polypoid lesion with granular or reticular pattern
& villous) • "Carpet" lesion: Flat, lobulated; localized or diffuse
• Non-neoplastic: Hyperplastic, hamartomatous and o "Carpet" lesion
inflammatory • Location: Rectum, cecum & ascending colon
• Subtle alteration in surface texture of colon with
Radiographic Findings little or no protrusion into lumen
• Fluoroscopic-guided double contrast barium enema • Irregular contour in contrast to smooth, fine
o Sessile polyps contour of adjacent normal bowel (profile view)

DDx: Filling Defect in Colon

Feces Oivertics. + Polyp Cancer + Lipoma Spasm + Feces

 COLONIC POLYPS
Key Facts
Terminology
• A protruding, space-occupying lesion within the
colonic lumen
Imaging Findings
• Best diagnostic clue: Radiolucent filling defect,
contour defect or ring shadow
• Sessile polyps: Broad base with little or no stalk
• Pedunculated polyps: Arise from narrow stalk
• "Carpet" lesion: Flat, lobulated; localized or diffuse
• Best imaging tool: Air-contrast barium enema
Top Differential Diagnoses
• Retained fecal debris
• Colonic diverticula
• Colon carcinoma
• Intramural mass
• Tiny, coalescent nodules and plaques ~ finely
nodular or reticular pattern with sharply
demarcated border (en face view)
o Hyperplastic polyps
• Location: Rectosigmoid colon
• Smooth round sessile nodules; < 5 mm (common)
• Lobulated or pedunculated; > 1 cm (occasional)
o Hamartomatous polyps
• Multiple, scattered radiolucent filling defects
• Vary in size; no "carpet" lesion
o Inflammatory polyps
• Islands of elevated, inflamed, edematous mucosa
surrounded by ulceration (inflammatory)
• Small & round, long & filiform or bush-like;
simulate villous adenoma (postinflammatory)
CT Findings
• CT "virtual colonoscopy"
o Small or large, sessile or pedunculated lesions
extending from colonic wall
o Polyps ~ 10 mm: Sensitivity 90%
o Adenoma ~ 10 mm: Sensitivity 94%
o Advantages: Shorter procedural time, I risk to
patient and no IV sedation
Ultrasonographic Findings
• Real Time Transrectal Ultrasonography
o Determine depth of invasion by a sessile polyp
Imaging Recommendations
• Best imaging tool: Air-contrast barium enema
• Protocol advice
o Patient rotated 180 or in upright position
0 General Features
• Confirm presence of a pedunculated polyp
• Visualize stalk in profile view
I DIFFERENTIAL DIAGNOSIS
Retained fecal debris
• Mobile & on dependent surface in barium pool
• Inconsistent location; irregular configuration;
impregnated with barium
Pathology
• Spectrum of adenoma: Tubular - tubulovillous -
villous
• Adenoma-carcinoma sequence (7-10 years): Benign
adenoma ~ malignant transformation
Clinical Issues
• Asymptomatic (75%)
• Colonoscopic polypectomy if polyps> 1 cm
• Colonoscopy or fluoroscopic-guided double contrast
barium enema for periodic surveillance
Diagnostic Checklist
• Family history of colonic polyps & colon carcinoma
• Polypectomy if changes noted on follow-up imaging
• If patient has known diverticulosis, single contrast
barium enema is easier for polyp detection
• Adherent stool can be difficult to differentiate; repeat
fluoroscopic-guided barium enema
• Proper cleansing of bowel can reduce confusion
Colonic diverticula
• "Bowler hat" sign: Dome of hat points away from
lumen of bowel
• Nondependent wall: Ring shadow with barium-coated 5
white rim (en face view); simulates polyps
41
o Rotate patient 90 to see outpouchings from wall
0
versus protrusion into lumen (profile view)
• Inverted diverticula can be difficult to differentiate
Colon carcinoma
• Sessile or pedunculated polyps seen in early cancer
• Biopsy is necessary to differentiate
Intramural mass
• Example: Leiomyoma, lipoma
• Leiomyoma
o Filling defect mimics villous adenoma (en face view)
o Abrupt well-defined borders of bowel wall
• Lipoma
o Commonly arises near ileocecal valve
o Soft + deformable with compression
o CT diagnostic with fat density
• Usually single; polyps often multiple
• Biopsy is necessary for diagnosis
I PATHOLOGY
• General path comments
o Neoplastic colonic polyps
• ,From proliferative dysplasia ~ adenoma
• Slow growing (doubling every 10 years)
• Single or multiple (more common)
• Spectrum of adenoma: Tubular - tubulovillous
- villous
o Tubular adenoma
• 80-86% of neoplastic polyps (most common)
• > 80% of glands are branching, tubule type
o Tubulovillous adenoma
 COLONIC POLYPS
• 8-16% of neoplastic polyps
o Villous adenoma
• 3-16% of neoplastic polyps
• > 80% of glands are villiform (shaggy surface)
o Non-neoplastic colonic polyps
• From abnormal mucosal maturation, architecture
or inflammation
• 90% of all epithelial polyps
• Small; occur at distal colon
o Hyperplastic polyps
• Almost never undergo malignant degeneration
o Hamartomatous polyps
• Varied polyp appearances & wide-range of ages,
depends on etiology
o Inflammatory polyps
• Also known as "pseudopolyps"
• 2 Types: Inflammatory and postinflammatory
• Postinflammatory: Mucosal healing ~ overgrowth
• Etiology
o Family history
• Adenomatous polyps (e.g., hereditary
nonpolyposis colorectal cancer syndrome, familial
polyposis, Gardner syndrome & Turcot syndrome)
• Hamartomatous polyps (e.g., Peutz-]eghers
syndrome and juvenile polyposis)
o Acquired
• Adenomatous polyps (e.g., sporadic adenoma)
5 • Hyperplastic polyps
• Hamartomatous polyps (e.g., Cronkhite-Canada
42 syndrome)
• Inflammatory polyps (e.g., ulcerative colitis)
o Risk factors: Diet, alcohol, smoking and obesity
o Pathogenesis
• Adenoma: Precursor to colon carcinoma
• Adenoma-carcinoma sequence (7-10 years):
Benign adenoma ~ malignant transformation
• Epidemiology
o Incidence of colon polyps: 3% in third decade; 5%
in fourth; 7% in fifth; 11% in sixth; 10% in seventh;
18% in eighth; 26% in ninth
o 1 Age ~ incidence of polyps shifts to right colon
o Hyperplastic polyps increases with age
• Associated abnormalities
o Colon carcinoma (adenocarcinoma)
• Polyps < 1 cm: 1% adenocarcinoma
• Polyps 1-2 cm: 10-20% adenocarcinoma
• Polyps> 2 cm: 40-50% adenocarcinoma
• 1 Villous changes or "carpet" lesion ~ 1 risk
• Lobulated contour or a basal indentation ~ 1 risk
• Tubular adenoma: < 1 cm: 1% with cancer; 1-2
cm: 10%; > 2 cm: 35%
• Tubulovillous adenoma: < 1 cm: 4% with cancer;
1-2 cm: 7%; > 2 cm: 46%
• Villous adenoma: < 1 cm: 10% with cancer; 1-2
cm: 10%; > 2 cm: 53%
Gross Pathologic & Surgical Features
• Tubular adenoma: Thin stalk and tufted head
• Villous adenoma: "Cauliflower-like" with broad base
Microscopic Features
• Adenomatous polyps
o Tubular, tubulovillous or villous structure lined by
columnar epithelium
o Tubular: Tubular glands with smooth surface
o Tubulovillous: Mixture of tubular & villous
o Villous: Surface consists of frondlike structures
o ± Cellular atypia, mitosis or loss of normal polarity
• Hyperplastic polyps
o Colonic crypts are elongated and epithelial cells
assume papillary configuration
o No cytologic atypia; epithelium is well-differentiated
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic (75%)
o Lower abdominal pain, rectal bleeding and diarrhea
Demographics
• Age
o Adenomatous polyps: 24-47% > 50 years of age
o Hyperplastic polyps: 50% > 60 years of age
• Gender
o Adenomatous polyps
• M:F = 2:1
Natural History & Prognosis
• Good, after resect benign or carcinoma in situ polyps
• Poor, with invasive colon carcinoma
Treatment
• Colonoscopic polypectomy if polyps> 1 cm
o Completely resect villous adenoma or "carpet" lesion
o In patients with neoplastic polyps caused by genetic
mutations, prophylactic colectomy is required prior
to malignant transformation
• Follow-up (20% recur at 5 years; 50% recur at 15 years)
o Colonoscopy or fluoroscopic-guided double contrast
barium enema for periodic surveillance
I DIAGNOSTIC CHECKLIST
Consider
• Family history of colonic polyps & colon carcinoma
• Polypectomy if changes noted on follow-up imaging
Image Interpretation Pearls
• If patient has known diverticulosis, single contrast
barium enema is easier for polyp detection
I SELECTED REFERENCES
1. YeeJ et al: Colorectal neoplasia: Performance
characteristics of CT colonography for detection in 300
patients. Radiology 219: 685-92, 2001
2. Macari M et al: Comparison of time-efficient CT
colonography with two and three-dimensional colonic
evaluation for detecting colorectal polyps. AjR 174: 1543-9,
2000
3. Levine MS et al: Diagnosis of colorectal neoplasms at
double-contrast barium enema examination. Radiology
216: 11-8, 2000
 COLONIC POLYPS

I IMAGE GALLERY

(Left) Air contrast BEshows

a large sessile polyp (arrow)
in the cecum; villous
adenoma. (Right) Single
contrast BEshows a
cauliflower-like polypoid
cecal mass (arrow); villous
adenoma.

(Left) Air contrast BE shows

a large pedunculated polyp
(arrows) in sigmoid colon;
tubulovillous adenoma.
(Right) Air contrast BE shows 5
numerous diverticula; also a
large pedunculated polyp on 43
a stalk (arrows);
tubulovillous adenoma.

(Left) Air contrast BE shows

small sessile tubular
adenoma (arrow). The dome
of the" bowler hat" points
toward the colonic lumen.
(Right) Air contrast BE shows
a small polyp on a short stalk
(arrow). The outer rim of the
"Mexican hat" is the head of
the polyp; the inner ring is
the stalk.
 COLON CARCINOMA

Graphic shows "apple core" constricting tumor of Single contrast BE shows classic "apple core" lesion of
sigmoid colon with circumferential narrowing of the colon. There is a short segment irregular, circumferential
lumen and a nodular tumor surface. narrowing of the lumen with destroyed mucosa and
nodular "shoulders".

ITERMINOlOGY o Early cancer: Sessile (plaquelike) lesion

• Typical early colon cancer
Definitions • Flat, protruding lesion with a broad base and little
• Malignant transformation of colon mucosa elevation of mucosa (in profile view)
5 • Discrete borders and shallow central ulcers (in
profile view)
44 [IMAGING FINDINGS • Curvilinear or undulating lines (in en face view)
o Early cancer: Pedunculated lesion
General Features • Short and thick polyp stalk
• Best diagnostic clue: Short segment luminal wall • Irregular or lobulated head of polyp
thickening o Advanced cancer: Polypoid lesion (large)
• Location: Cecum (10%); ascending colon (15%); • Dependent wall: Filling defect in barium pool
transverse colon (15%); descending colon (5%); • Nondependent wall: Etched in white
sigmoid colon (25%); rectosigmoid colon (10%); o Advanced cancer: Semi-annular (saddle) lesion
rectum (20%) • Transition to annular carcinoma: Polypoid ~
• Morphology semi-annular ~ annular
o Early cancer: Sessile or pedunculated tumors • Convex barium-etched margins (in profile view)
o Advanced cancer: Annular, semi annular, polypoid o Advanced cancer: Annular (apple-core) lesion
or carpet tumors • Circumferential narrowing of bowel; Shelf-like,
• Other general features overhanging borders (mucosal destruction)
o Radiology is critical for screening, diagnosis, • High-grade obstruction and ischemia:
treatment and follow-up of colon cancer Thumbprinting of dilated proximal colon
o Screening: Fluoroscopic-guided double contrast o Advanced cancer: Carpet lesion
barium enema or CT "virtual colonoscopy" are • Malignant villous tumor may appear as carpet
comparable to colonoscopy for cancer detection lesion with minimal protrusion into lumen
• Radiolucent nodules surrounded by barium-filled
Radiographic Findings grooves; finely nodular or reticular pattern
• Fluoroscopic-guided double contrast barium enema

DDx: luminal Narrowing, Wall Thickening of Colon

Diverticulitis Ischemic Stricture Tuberculosis Endometriosis

 COLON CARCINOMA

Key Facts
Imaging Findings • Infectious colitis
• Best diagnostic clue: Short segment luminal wall Pathology
thickening
• !Fiber + t fat and animal protein diet
• Early cancer: Sessile or pedunculated tumors
• Advanced cancer: Annular, semiannular, polypoid or Clinical Issues
carpet tumors • Melena, hematochezia, iron deficiency
• Asymmetric mural thickening ± irregular surface • Overall 5 year survival is 50%
• Hepatic metastases most common • CT: Follow-up 3-4 months after surgery, then every 6
• Detection: Fluoroscopic-guided double contrast months for 2-3 years, then annually for 5 years
barium enema • PET-CTis best for recurrence and surveillance
• Staging: Helical CT
• Tumor recurrence and surveillance: PET-CT Diagnostic Checklist
• Evaluate entire colon for synchronous lesions
Top Differential Diagnoses • Tumor mass with irregular margins; apple-core lesion;
• Diverticulitis pericolonic extension and distant metastases
• Ischemic colitis

• Fluoroscopic-guided double contrast barium enema

CT Findings o "Thumbprinting" (submucosal edema or bleeding)
• Asymmetric mural thickening ± irregular surface o Stricture: Smooth, tapered margins, but no mass
• Wall thickness: < 3 mm: Normal; 3-6 mm: effect (chronic)
Indeterminate; > 6 mm: Abnormal • CT: Bowel wall thickening; ± pneumatosis,
• Tumor within lumen portomesenteric venous gas
o Smooth outer bowel margins
• Extracolonic tumor extension Infectious colitis 5
o Mass with irregular border • Example: Tuberculosis and Amebiasis
45
o Strands of soft tissue extending from serosal surface • Rare in the U.S.; usually in proximal colon
into perirectal or pericolic fat • Stricture formation may simulate carcinoma (chronic)
o Loss of tissue fat planes between colon and
surrounding muscles
Ulcerative colitis
• Metastases to mesenteric nodes, peritoneum • Significant etiology of colon carcinoma
• Hepatic metastases most common • Bowel wall thickening, luminal narrowing
• Fluoroscopic-guided double contrast barium enema
Nuclear Medicine Findings o Punctate and collar button ulcers
• PET:Fluorine 18-labeled deoxyglucose uptake is 2 fold o Continuous, concentric and symmetric colonic
higher in tumors than normal or nonmalignant involvement
lesions o "Lead pipe" colon and absent haustra (chronic)
• PET-CT
o Combines morphologic information (of CT) with
Extrinsic lesions
metabolic information (of PET) • Endometriosis, ovarian cancer, "drop" metastases
• Smooth, eccentric, obtuse angles with colonic wall
Imaging Recommendations
• Best imaging tool
o Detection: Fluoroscopic-guided double contrast I PATHOLOGY
barium enema
o Staging: Helical CT General Features
o Tumor recurrence and surveillance: PET-CT • General path comments
o Most common cancer of gastrointestinal tract
o Second most common cancer mortality
I DIFFERENTIAL DIAGNOSIS o 5% of colon cancers have synchronous colonic
tumors
Diverticulitis • Genetics
• CT findings o Mutations of genes
o Location: Most common in sigmoid colon • Proto-oncogene (K-ras): 50% of colon cancers
o Bowel wall and fascial thickening; fat stranding; free • Tumor suppressor genes (APC, DCC, SMAD4, p53,
air and fluid TGF-Sl RII): 70-75% of colon cancers
o Pericolic inflammatory changes: Abscess, sinuses, • DNA mismatch repair genes: 15% of colon cancers
fistulas or strictures • Etiology
o Risk factors
Ischemic colitis • !Fiber + t fat and animal protein diet
• Usually seen in watershed areas; focal or diffuse • History of colon adenoma or carcinoma
 COLON CARCINOMA
• Benign polyps> 1 cm • Diagnosis: Colonoscopy with mucosal biopsy
• Inflammatory bowel disease
Demographics
• Family history
o Family history • Age: > 50 years of age; peak at 70 years of age
• Colon cancer in first-degree relatives (1 2-3 fold) • Gender: M:F = 3:2
• Familial Adenomatous Polyposis « 1%), Gardner, Natural History & Prognosis
familial juvenile polyposis and hereditary
• Complications
nonpolyposis colorectal cancer o Hemorrhage, obstruction, perforation and fistula
o Pathogenesis
• Prognosis
• Adenoma-carcinoma sequence (7-10 years): o Overall 5 year survival is 50%
Benign adenoma ~ malignant transformation
• Duke's stage A: 81-85%
• Inflammatory bowel disease: Inflammation ~
• Duke's stage B: 64-78%
dysplasia ~ carcinoma
• Duke's stage C: 27-33%
• De novo colon carcinoma: Normal mucosa ~
• Duke's stage D: 5-14%
small, aggressive, ulcerated tumors
o Hereditary nonpolyposis colorectal cancer (HNPCC) Treatment
• 6% of colon cancer • Complete surgical resection (2. 5 cm on each side of
• Discrete adenoma, not polyposis tumor) with removal of lymphatic drainage vessels ±
.• Autosomal dominant with high penetration adjuvant chemotherapy
• Epidemiology • Pre- & post-operative radiation therapy (selected cases)
0> Common in N. America, Europe & New Zealand • Follow-up
• Incidence in U.S. is 135,000 per year oCT: Follow-up 3-4 months after surgery, then every
• Mortality in U.S. is 57,000 per year 6 months for 2-3 years, then annually for 5 years
o 1 Age ~ incidence of cancers shifts to right colon o CEA titer: If elevated, CT is indicated
• Associated abnormalities o PET-CT is best for recurrence and surveillance
o 5% of colon cancers: Metachronous carcinomas
o 33% of colon cancers: Adenomatous polyps
5 Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
46 • Cecum and proximal colon: Bulky and polypoid, Consider
outgrowing their blood supply ~ necrosis • Evaluate entire colon for synchronous lesions
• Distal colon and rectum: Annular constriction or
"napkin-ring" appearance ~ obstruction & ulceration Image Interpretation Pearls
• Tumor mass with irregular margins; apple-core lesion;
Microscopic Features
pericolonic extension and distant metastases
• Adenocarcinoma (> 95% of colon cancers)
o Mucin-producing glands
o Mucinous: "Signet-ring" cells
o Colloid (15%): Large lakes of mucin contain
I SELECTED REFERENCES
scattered collections of tumor cells 1. Bar-Shalom R et al: Clinical performance of PET/CT in
evaluation of cancer: additional value for diagnostic
• Squamous cell carcinoma « 5%)
imaging and patient management. J Nucl Med.
Staging, Grading or Classification Criteria 44(8):1200-9,2003
2. Cohade C et al: Direct comparison of (18)F-FDG PETand
• Surgical-pathologic (modified Dukes) staging of colon
PET/CT in patients with colorectal carcinoma. J Nucl Med.
cancer with TNM correlation 44(11):1797-803,2003
o Stage A (TlNOMO): Limited to mucosa ± submucosa 3. Pickhardt PJ et al: Computed tomographic virtual
o Stage B (T2 or 3 & NOMO): Limited to serosa or into colonoscopy to screen for colorectal neoplasia in
adjacent tissues asymptomatic adults. N Engl] Med. 349(23):2191-200,
o Stage C (T2 or 3 & N1MO): Lymph node metastases 2003
o Stage D (any T and N, M1): Distant metastases 4. Levine MS et al: Diagnosis of colorectal neoplasms at
double-contrast barium enema examination. Radiology
216: 11-8,2000
5. Gazelle GS et al: Screening for colorectal cancer. Radiology.
I CLINICAL ISSUES 215(2):327-35,2000
6. Horton KM et al: Spiral CT of colon cancer: imaging
Presentation features and role in management. Radiographies.
• Most common signs/symptoms 20(2):419-30, 2000
o Melena, hematochezia, iron deficiency 7. Thoeni RF:Colorectal cancer: cross-sectional imaging for
o Abdominal pain & changes in bowel habit staging of primary tumor and detection of local recurrence.
o Colonic obstruction (50%, most common cause) AJRAm J Roentgenol. 156(5):909-15, 1991
o Weight loss, fever and weakness 8. Balthazar EJ et al: Carcinoma of the colon: detection and
preoperative staging by CT. AJRAm J Roentgenol.
• Lab-Data 150(2):301-6, 1988
o Positive or negative fecal occult blood test 9. Kelvin FM et al: Colorectal carcinoma detected initially
o ± Micro- to normocytic anemia with barium enema examination: site distribution and
o Carcinoembryonic antigen (CEA) > 2.5 I-Ig/L implications. Radiology. 169(3):649-51, 1988
 COLON CARCINOMA
I IMAGE GALLERY
Typical
(Left) Single contrast BE
shows bulky mass at hepatic
flexure with abrupt transition
to normal colon. Little or no
obstruction in spite of large
mass + luminal narrowing.
(Right) Axial CECT shows
mass at hepatic flexure with
circumferential wall
thickening, narrowed lumen.
Infiltrated fat (arrow) +
mesenteric adenopathy
(open arrow) indicate local
spread of disease.

(Left) Single contrast BE

shows short segment luminal
narrowing of ascending
colon with "apple core"
appearance, but no
obstruction. (Right) Axial
5
CECT shows large eccentric
47
mass in ascending colon
with extensive infiltration of
pericolonic fat +
lymphadenopathy (open
arrow).

Variant
(Left) Axial CECT shows wall
thickening + luminal
narrowing of transverse
colon near splenic flexure
with abrupt transition to
normal colon. Mesenteric
adenopathy (open arrow).
(Right) Axial CECT of patient
with colon cancer near
splenic flexure. Long segment
of wall thickening + luminal
narrowing with submucosal
low density. At resection
found to represent ischemic
colitis.
 RECTAL CARCINOMA

Single contrast BE shows mass (arrow) arising from Double contrast BE shows rectal mass (arrows) outlined
anterior rectal wall as a filling defect in the barium pool. by a coating of barium.

• Discrete borders and shallow central ulcers (profile

ITERMINOLOGY view)
Definitions • Curvilinear or undulating lines (en face view)
• Malignant transformation of rectal mucosa o Early cancer: Pedunculated lesion
5 • Short and thick polyp stalk
• Irregular or lobulated head of polyp
48 IIMAGING FINDINGS o Advanced cancer: Polypoid lesion
• Dependent wall: Filling defect in barium pool
General Features • Nondependent wall: Etched in white
• Best diagnostic clue: Polypoid mass with irregular o Advanced cancer: Semi-annular (saddle) lesion
surface • Transition to annular carcinoma
• Morphology • Convex barium-etched margins (profile view)
o Early cancer: Sessile or pedunculated tumors o Advanced cancer: Annular (apple-core) lesion
o Advanced cancer: Annular, semiannular, polypoid • Circumferential narrowing of bowel; shelf-like,
or carpet tumors overhanging borders (mucosal destruction)
o Most common in rectum: Sessile and polypoid • High grade obstruction and ischemia:
• Other general features Thumbprinting of dilated proximal colon
o Radiologic features are similar to colon carcinoma o Advanced cancer: Carpet lesion
o Transrectal ultrasonography for tumor staging • Malignant villous tumor may appear as carpet
o Types of rectal cancer: Adenocarcinoma (80%) and lesion with minimal protrusion into lumen
squamous cell carcinoma (20%) • Radiolucent nodules surrounded by barium-filled
grooves; finely nodular or reticular pattern
Radiographic Findings
• Fluoroscopic-guided barium enema CT Findings
o Early cancer: Sessile (plaque-like) lesion • Mass & focal or circumferential wall thickening
• Most typical early colorectal cancer • Asymmetric mural thickening ± irregular surface
• Flat, protruding lesion with a broad base and little • Wall thickness: < 3 mm: Normal; 3-6 mm:
elevation of mucosa (profile view) Indeterminate; > 6 mm: Abnormal
• Tumor within lumen

DDx: Rectal Mass or Luminal Narrowing

J .~<~
.~
Cervical Cancer Rectal Ulcer Lymphogranuloma Hemorrhoids
 RECTAL CARCINOMA
Key Facts
Imaging Findings
• Best diagnostic clue: Polypoid mass with irregular
surface
• Radiologic features are similar to colon carcinoma
• Types of rectal cancer: Adenocarcinoma (80%) and
squamous cell carcinoma (20%)
• May have lung and bone metastases before liver
metastases
• Metastases to lymph nodes: Spherical, hypoechoic &
distinct margins
• Transrectal ultrasonography: Visualize layers of rectal
wall & depth of tumor penetration
Top Differential Diagnoses
• Local invasion
• Villous adenoma
o Smooth outer bowel margins
• Extracolonic tumor extension
o Mass with irregular border
o Strands of soft tissue extending from serosal surface
into perirectal fat
o Loss of tissue fat planes between rectum and
surrounding muscles
• Metastasis to lymph nodes at external iliac and
para-aortic chain, inguinal, retroperitoneum or porta
hepatis
• May have lung and bone metastases before liver
metastases
MR Findings
• Mass; pericolonic infiltration, lymphadenopathy
shown slightly better than by CT
• Endorectal coil - improves resolution, but may not be
worth the effort
Ultrasonographic Findings
• Real time transrectal ultrasonography
o Hypoechoic mass with disruption of wall segments
o Focal or circumferential wall thickening
o Metastases to lymph nodes: Spherical, hypoechoic &
distinct margins
o Rings of different echogenicities (center ~ outer)
• Innermost ring: Hyperechoic; interface between
balloon and mucosa
• Second ring: Hypoechoic; muscularis mucosae
• Third ring: Hyperechoic; submucosa
• Fourth ring: Hypoechoic; muscularis propria
• Fifth ring: Hyperechoic; perirectal fat or serosa
o Sonographic staging based on TNM classification
• Tl: Confined to mucosa/submucosa; middle
echogenic layer intact
• T2: Confined to rectal wall; outermost echogenic
layer is intact
• T3: Penetrates into perirectal fat; disrupting outer
hyperechoic ring
Nuclear Medicine Findings
• PET: Fluorine I8-labeled deoxyglucose uptake is 2 fold
higher in tumors than normal or nonmalignant
lesions
• Trauma
• Infection
Pathology
• ~ Fiber + 1 fat and animal protein diet
• HIV positive homosexual males
• Human papilloma virus (HPV): Type 16, 18, 45, 46
Clinical Issues
• Hematochezia, rectal pain, change in bowel habits
• Anal pain, anal discharge & tenesmus
• Overall 5 year survival is 50%
Diagnostic Checklist
• Evaluate entire colon for synchronous lesions
• Image detection of perirectal tumor spread is vital;
requires pre-operative radiation ± chemotherapy
• PET-CT
o Combines morphologic information (of CT) with
metabolic information (of PET)
Imaging Recommendations
• Best imaging tool
o Detection: Fluoroscopic-guided barium enema
o Staging: Helical CT and transrectal ultrasonography 5
o Transrectal ultrasonography: Visualize layers of
49
rectal wall & depth of tumor penetration
o Tumor recurrence, surveillance: PET-CT
• Protocol advice: Transrectal ultrasonography: Pass
transducer proximal to tumor into the colon for
complete assessment of mural and nodal pathology
I DIFFERENTIAL DIAGNOSIS
local invasion
• Example: Carcinoma of the cervix, prostate, bladder
• Direct extension to pelvic sidewall and adjacent
structures including rectum
• Circumferential narrowing ± lymphadenopathy
• Depends on size of tumor; can be hard to differentiate
Villous adenoma
• Polypoid lesion with a granular or reticular appearance
• High risk of malignant degeneration
• Similar to "carpet" lesion in advanced rectal cancer
Trauma
• Penetrating injuries: Anal intercourse & insertion of
foreign bodies
• Fibrosis and stricture (chronic) can simulate cancer
• Perianal and rectal mucosa ulceration
Infection
• Mucosal ulceration or granular mucosal pattern
• Mechanism: Anal sex, spread from vaginal discharge
or lymphatic extension from inguinal lymph nodes
• Most common: C. trachomatis ~ lymphatic tissue
infection ~ Lymphogranuloma venereum (LGV)
• Other STDs include N. gonorrhoeae, HSV and syphilis
 RECTAL CARCINOMA
• Progress to fistula, perirectal abscess or stricture o Hematochezia, rectal pain, change in bowel habits
(chronic)i similar in complications of rectal cancer o Perineal or sacral pain (chronic)
o Squamous cell carcinoma:
• Anal pain, anal discharge & tenesmus
I PATHOLOGY • Lab-Data
o ± HIV (PCR) test
General Features o Carcinoembryonic antigen (CEA) > 2.5 I-lg/L
• General path comments • Diagnosis
o Colon cancer: Rectum (20%) & rectosigmoid (15%) o Sigmoidoscopy with mucosal biopsy
o Rectal cancer tends to invade locally (lack serosa)
o Metastases: Upper 2/3 of rectum Demographics
• Portal system - liver • Age: Adenocarcinoma: Age: > 50 years of agei peak at
• Batson vertebral venous plexus - lumbar & 70 years of age
thoracic vertebra • Gender: M:F = 3:2
o Metastases: Lower 1/3 of rectum
Natural History & Prognosis
• Superior hemorrhoidal vein - portal - liver
• Middle hemorrhoidal vein - IVC - lung .• Complications
• Genetics: Adenocarcinoma: Mutation in o Hemorrhage, obstruction, perforation and fistula
proto-oncogene, tumor suppressor genes or DNA • Prognosis
mismatch repair genes o Overall 5 year survival is 50%
• Etiology • Duke's stage A: 81-85%
o Adenocarcinoma • Duke's stage B: 64-78%
• j Fiber + 1 fat and animal protein diet
• Duke's stage C: 27-33%
• History of colorectal adenoma or carcinoma • Duke's stage D: 5-14%
• Benign polyps> 1 cm Treatment
• Family history & Inflammatory bowel disease
• Surgical resection (depends on location) & removal of
o Squamous cell carcinoma
lymphatic drainage vessels ± adjuvant chemotherapy
5 • HIV positive homosexual males
• Human papilloma virus (HPV): Type 16, 18, 45, 46
• Pre- & post-operative radiation ± chemotherapy
therapy (selected cases)
50 • Lubricants, cleansers & mechanical irritation
• Follow-up
o Pathogenesis
oCT: Follow-up 3-4 months after surgery, then every
• Adenocarcinoma: Adenoma-carcinoma sequence
6 months for 2-3 years, then annually for 5 years
• Squamous cell carcinoma: Squamous metaplasia
o CEA titer: If elevated, CT is indicated (PET-CT best)
- dysplasia - carcinoma
• Epidemiology: Adenocarcinoma: More common in N.
America, Europe & New Zealand
I DIAGNOSTIC CHECKLIST
Gross Pathologic & Surgical Features
Consider
• Flat, infiltrative, annular or ulcerative & rolled borders
• Annular constriction or "napkin-ring" appearance - • Evaluate entire colon for synchronous lesions
obstruction, ulceration and intramural spread Image Interpretation Pearls
• Squamous cell carcinoma: Mass from epithelium of • Image detection of perirectal tumor spread is vitali
anorectal junction (dentate line) requires pre-operative radiation ± chemotherapy
Microscopic Features
• Adenocarcinoma: Mucin-producing glands
• Squamous cell (cloacogenic) carcinoma I SELECTED REFERENCES
o Mixture of basaloid cell, transitional cell with 1. Fuchsjager MH et al: Comparison of transrectal sonography
squamous differentiation, adenoid cyst and and double-contrast MR imaging when staging rectal
mucoepithelial cell cancer. A]RAm] Roentgenol. 181(2):421-7,2003
2. Winawer 5] et al: A comparison of colonoscopy and
Staging, Grading or Classification Criteria double-contrast barium enema for surveillance after
• Surgical-pathologic (modified Dukes) staging of colon polypectomy. New Eng] Med 342: 1766-72,2000
3. Maier AG et al: Transrectal sonography of anal sphincter
cancer with TNM correlation
infiltration in lower rectal carcinoma. A]R Am]
o Stage A (TINOMO): Limited to mucosa ± submucosa Roentgenol. 175(3):735-9,2000
o Stage B (T2 or 3 & NOMO): Limited to or invades 4. Levine MS et al: Diagnosis of colorectal neoplasms at
adjacent tissues double-contrast barium enema examination. Radiology
o Stage C (T2 or 3 & NIMO): Lymph node metastases 216: 11-8, 2000
o Stage D (any T and N, Ml): Distant metastases 5. Thompson WM et al: Computed tomography of the
rectum. Radiographies. 7(4):773-807, 1987
6. Cohan RH et al: Computed tomography of epithelial
I CLINICAL ISSUES neoplasms of the anal canal. A]RAm] Roentgenol.
145(3):569-73, 1985
Presentation
• Most common signs/symptoms
 RECTAL CARCINOMA
I IMAGE GALLERY

(Left) Single contrast BE

shows large rectal mass with
markedly irregular surface;
carcinoma arising from
villous adenoma. (Right) Air
contrast BE shows large mass
arising from lateral wall of
the rectum.

Typical
(Left) Axial CECT shows
large mass that fill the rectal
lumen and infiltrates the
perirectal fat. Extensive
lymphadenopathy (arrows).
(Right) Transrectal
5
ultrasonography shows a
51
bulky rectal mass (arrows)
with invasion through
submucosa; T3 stage.

Typical
(Left) Axial CECT shows
calcified periaortic and
retrocrural nodes, no liver
metastases. Mucinous rectal
adenocarcinoma. (Right)
Axial CECT shows extensive
pulmonary metastases from
rectal cancer in a patient
with no liver metastases.
 VILLOUS ADENOMA

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Graphic shows polypoid mass in rectosigmoid colon Single contrast BE shows a polypoid mass in the
having a shaggy, nodular surface, rectosigmoid colon (arrow) with a very nodular surface,
Barium within the rectum is diluted by mucous secreted
by the tumor.

ITERMINOlOGY • Villous adenoma: 10% of neoplastic polyps;

villous change more than 75%
Abbreviations and Synonyms • Tubulovillous adenoma: 15%; villous change
• Villous tumor between 25-50%
5 Definitions
o As adenoma increases in size, degree of villous
change usually increases
52 • Adenomatous polyp that contains predominantly o Villous adenoma has highest risk of malignant
villous elements ("villous" means "shaggy surface") degeneration
o Risk of cancer is related to proportion of villous
change in adenoma
I IMAGING FINDINGS o Greater risk of carcinoma in villous tumors of
stomach & duodenum than colon
General Features • Stomach: Carcinoma in 50% of lesions 2-4 cm &
• Best diagnostic clue: Polypoid lesion with a nodular or 80% in more than 4 cm
frond-like surface on barium enema • Duodenum: Carcinoma in 30-60% of villous
• Location: Rectosigmoid> cecum> ascending colon> tumors more than 4 cm
stomach> duodenum • Colon: Carcinoma in situ in 10% & invasive
• Size carcinoma in up to 45% of cases
o Range from < 1-10 cm in diameter
o Giant villous tumor: 10-15 cm Radiographic Findings'
• Morphology: "Cauliflower-like" sessile growth with a • Fluoroscopic guided double contrast barium enema
broad base or flat "carpet" lesion o Two types of villous adenomas
• Other general features • Polypoid mass
o Villous adenoma is one of the histological types of • "Carpet" lesion
adenomatous polyps (true neoplasms) o Polypoid mass
• Tubular adenoma: 75% of neoplastic polyps; • May look like a cauliflower within colon
villous change less than 25% • Nodular, lace or soap bubble pattern
• Due to trapping of barium between frond-like
projections (interstices)

DDx: Irregular Solitary Filling Defect

Rectal Cancer Colon Cancer Fecal Mass Colon Lipoma

 VILLOUS ADENOMA
Key Facts
Terminology
• Adenomatous polyp that contains predominantly
villous elements ("villous" means "shaggy surface")
Imaging Findings
• Best diagnostic clue: Polypoid lesion with a nodular
or frond-like surface on barium enema
• Location: Rectosigmoid> cecum> ascending colon>
stomach> duodenum
• Range from < 1-10 cm in diameter
• Malignant transformation in a bulky adenoma:
Annular lesion with shelf-like, overhanging borders
• Localized "carpet" lesion: Subtle alteration in surface
texture
• Extensive "carpet" lesion: Involves a large area of
colon, encircling lumen
• Malignant transformation in a bulky adenoma:
Annular lesion with shelf-like, overhanging
borders
o "Carpet" lesion
• Flat, lobulated lesion
• Localized or extensive
o Localized "carpet" lesion: Subtle alteration in surface
texture
o Extensive "carpet" lesion: Involves a large area of
colon, encircling lumen
• En face: Fine nodular, reticular pattern with
sharply demarcated border
• Profile: Irregular contour in contrast to smooth,
fine contour of adjacent normal bowel
o Malignant transformation in "carpet" lesion (1 risk)
• Radiolucent nodules surrounded by barium-filled
grooves (produce fine nodular or reticular pattern)
• Polypoid carcinoma with surrounding mucosal
change represents underlying adenoma
• Seen in rectum, cecum, ascending colon, stomach
& duodenum
CT Findings
• Large villous adenoma
o Low-attenuation irregular polypoid mass
o Convolutional gyral enhancement pattern
o Corrugated, feathery appearance due to trapping of
oral contrast in interstices of villous adenoma
MR Findings
• Large villous adenoma
o Tl WI: Low signal intensity mass with multiple
frond-like projections & central cord-like structure
o T2WI: Frond-like projections will be more
prominent
• Villous adenoma with 1 mucin producing cells
o Short Tl & long T2 times
o Tl WI & T2WI: Adenoma appears hyperintense
Ultrasonographic Findings
• Transrectal sonography
o Determine depth of invasion into colonic wall by
adenoma
Top Differential Diagnoses
• Colon carcinoma
• Fecal mass
• Intramural mass
Pathology
• Family history, idiopathic inflammatory disease
• Malignant potential: 5% in lesions < 1 cm; 10% in
lesions 1-2 cm; 53% in > 2 cm lesions
• Gray-tan lesion
Diagnostic Checklist
• Check for family history of colonic polyps & evaluate
entire colon for synchronous lesions
• Cauliflower-like sessile mass with a broad base or
carpet lesion with reticular or soap-bubble surface
pattern
Imaging Recommendations
• Double-contrast barium enema
o En face, profile & oblique views
• Transrectal US; NE + CECT
I DIFFERENTIAL DIAGNOSIS 5
53
Colon carcinoma
• Barium enema findings
o Early cancer: Sessile (plaquelike) lesion
• Typical early colon cancer
• Flat, protruding lesion with a broad base & little
elevation of mucosa (in profile view)
o Early cancer: Pedunculated lesion
• Short & thick polyp stalk
• Irregular or lobulated head of polyp
o Advanced cancer: Polypoid lesion (large)
• Dependent wall: Filling defect in barium pool
• Nondependent wall: Etched white
o Sessile & pedunculated polypoid cancers may be
indistinguishable from villous adenoma
o Advanced cancer: Semi-annular (saddle) lesion
o Advanced cancer: Annular (apple-core) lesion
• Circumferential narrowing of bowel
• Shelf-like, overhanging borders (mucosal
destruction)
• CT findings
o Asymmetric mural thickening ± irregular surface
o Extracolonic tumor extension
• Mass with irregular borders
• Extension from serosa to pericolic fat
• Loss of fat planes: Colon & adjacent muscles
o Metastases to mesenteric nodes
o Metastases to liver more common
• Diagnosis: Biopsy & histology
Fecal mass
• Large, irregular colonic fecal impaction
o Most common location: Rectum
• Mimic large cauliflower-like sessile polyp
• May cause bowel obstruction + proximal dilatation
 VILLOUS ADENOMA
• Usually seen in elderly, sedentary patients
Demographics
• Diagnosis: Clinical history & colonoscopy
• Age: 60-70 years of age or older
Intramural mass • Gender: Equal in both males & females (M = F)
• Example: Stromal tumors (leiomyoma, sarcoma or
Natural History & Prognosis
GIST)
• Leiomyoma • Complications
o In profile o Malignant transformation or invasion; hemorrhage
• Smooth surface etched in white • Prognosis
• Borders: Right or obtuse angles with adjacent wall o Good: After removal of benign & carcinoma in situ
o En face adenoma
• Seen as a filling defect simulating polypoid type of o Poor: Invasive carcinoma
villous adenoma Treatment
• Intraluminal surface: Abrupt well-defined borders • Colonoscopic, endoscopic or surgical resection
• Leiomyosarcoma
o Bulky stromal tumors most frequently seen in
rectum
o Broad based mass simulating large villous adenoma
I DIAGNOSTIC CHECKLIST
o Large tumors show ulceration or cavitation Consider
o CT shows pericolonic extension (large extraluminal • Check for family history of colonic polyps & evaluate
mass), liver & peritoneal metastases entire colon for synchronous lesions
• Hypervascular on angiography
• Diagnosis: Biopsy Image Interpretation Pearls
• Cauliflower-like sessile mass with a broad base or
carpet lesion with reticular or soap-bubble surface
I PATHOLOGY pattern

General Features
5 • Etiology I SELECTED REFERENCES
o Villous adenoma or tumor
54 1. Smith TR et al: CT appearance of some colonic villous
• Family history, idiopathic inflammatory disease
tumors. A]R Am] Roentgenol. 177(1):91-3,2001
• Malignant potential: 5% in lesions < 1 cm; 10% in 2. Levine MS et al: Diagnosis of colorectal neoplasms at
lesions 1-2 cm; 53% in > 2 cm lesions double-contrast barium enema examination. Radiology
• Epidemiology: Incidence: Least common (10%) of all 216: 11-8, 2000
neoplastic adenomatous polyps 3. Cunnane ME et al: Small flat umbilicated tumors of the
colon: radiographic and pathologic findings. A]RAm]
Gross Pathologic & Surgical Features Roentgenol. 175(3):747-9, 2000
• Usually sessile 4. Chung JJ et al: Large villous adenoma in rectum mimicking
o May be polypoid, broad, flat or carpet-like lesion cerebral hemispheres. A]R Am] Roentgenol. 175(5):1465-6,
o Gray-tan lesion 2000
5. Iida M et al: Endoscopic features of villous tumors of the
• May have a short, broad stalk & focal areas of
colon: correlation with histological findings.
hemorrhage or ulceration Hepatogastroenterology. 37(3):342-4, 1990
Microscopic Features 6. Iida M et al: Villous tumor of the colon: correlation of
histologic, macroscopic, and radiographic features.
• Frond-like papillary projections of adenomatous Radiology. 167(3):673-7, 1988
epithelium 7. Galandiuk S et al: Villous and tubulovillous adenomas of
• ± Well-differentiated areas the colon and rectum. A retrospective review, 1964-1985.
• Carcinoma in situ; invasive cancer Am] Surg. 153(1):41-7, 1987
8. Galandiuk S et al: Villous and tubulovillous adenomas of
the colon and rectum. A retrospective review, 1964-1985.
I CLINICAL ISSUES Am] Surg. 153(1):41-7, 1987
9. Ott D] et al: Single-contrast vs double-contrast barium
Presentation enema in the detection of colonic polyps. A]RAm]
Roentgenol. 146(5):993-6, 1986
• Most common signs/symptoms 10. de Roos A et al: Colon polyps and carcinomas: prospective
o Asymptomatic, diarrhea, pain, rectal bleeding or comparison of the single- and double-contrast examination
melena in the same patients. Radiology. 154(1):11-3, 1985
o Lesion closer to rectum: More likely to have 11. Delamarre] et al: Villous tumors of the colon and rectum:
diarrhea, electrolyte loss double-contrast study of 47 cases. Gastrointest Radiol.
• Lab-data 5(1):69-73, 1980
o Guaiac positive stool
o Iron deficiency anemia
o Decreased protein, K+, Na+
o ± Increased direct bilirubin levels (due to obstruction
of ampulla of Vater (duodenum) by adenoma)
• Diagnosis: Endoscopy, biopsy & histology
 VILLOUS ADENOMA
I IMAGE GALLERY

(Left) Axial CECT shows a

large mass that fills the
rectosigmoid colon with
dilated stool-filled colon,
noted more proximally.
(Right) Axial CECT shows a
large polypoid mass (arrow)
within the rectum.

(Left) Single contrast BE

shows large rectal mass with
frond-like surface. Note
absence of colonic
obstruction. (Right) Air
contrast BE shows rectal
5
mass with nodular surface
55
that fills, but does not
obstruct, the rectal lumen.

(Left) Single contrast BE

shows a cauliflower-like
mass (arrow) in the cecum.
(Right) Air contrast BEshows
cauliflower-like cecal mass;
(arrow) villous adenoma.
 FAMILIAL POLYPOSIS

Graphic shows innumerable small polyps and multifocal Air contrast BE shows innumerable small polyps in
carcinomas (arrows). sigmoid colon.

o Familial polyposis coli: Multiple colonic

!TERMINOlOGY adenomatous polyps
Abbreviations and Synonyms • Entire colonic mucosa is carpeted with polyps
• Familial adenomatous polyposis syndrome (FAPS) o Gardner syndrome: Combination of
5 Definitions
• Familial polyposis coli, osteomas, epidermoid
(sebaceous) cyst
56 • Spectrum of autosomal dominant disease characterized • Soft tissue tumors: Desmoid, mesenteric
by innumerable adenomatous colonic polyps & other fibromatosis, lipoma
associated lesions • Dental abnormalities; periampullary, duodenal &
thyroid carcinomas
o 500-2500 polyps present carpeting colonic mucosa
I IMAGING FINDINGS o Polyps appear around puberty & onset of symptoms
in 3rd or 4th decade
General Features o Most polyps are tubular & tubulovillous,
• Best diagnostic clue: Innumerable colonic filling occasionally villous adenomas
defects or ring shadows ± extraintestinallesions o FAPSadenomas small (80% < 5 mm) & sessile
• Location o Colorectal cancer develops in almost 100% of
o Most common in colon (t predilection-left colon) untreated patients
• Colon> stomach> duodenum> small bowel o 2/3 of afflicted cases are inherited & 1/3 are sporadic
• Size: Varies from pin point to > 1 cm o Abnormal gene has high penetrance (80-100%)
• Morphology: Sessile or pedunculated polypoid lesions o Extracolonic GI tract manifestations of FAPS
• Other general features • Stomach, duodenum, jejunum & ileum
o FAPSis a rare condition, but is most common of o Stomach
polyposis syndromes • Fundic gland polyps & adenomas in > 50% cases
o Two varied expressions of FAPS o Duodenum: Adenomas of 2nd part & periampullary
• Familial polyposis coli: Multiple colonic in > 47% cases
adenomatous polyps • Periampullary cancer: 2nd most frequent site of
• Gardner syndrome cancer outside colon seen in 12% of FAPSpatients

DDx: Multiple Colonic Filing Defects

Retained Feces Ulcerative Colitis Ulcerative Colitis Primary Pneumatosis

 FAMILIAL POLYPOSIS
Key Facts
Terminology
• Familial adenomatous polyposis syndrome (FAPS)
• Spectrum of autosomal dominant disease
characterized by innumerable adenomatous colonic
polyps & other associated lesions
Imaging Findings
• Best diagnostic clue: Innumerable colonic filling
defects or ring shadows ± extraintestinallesions
• Familial polyposis coli, osteomas, epidermoid
(sebaceous) cyst
• Soft tissue tumors: Desmoid, mesenteric fibromatosis,
lipoma
• Dental abnormalities; periampullary, duodenal &
thyroid carcinomas
• FAPSadenomas small (80% < 5 mm) & sessile
o Jejunum & ileum
• Adenomas, lymphoid hyperplasia in > 20% cases
o Associated with 1 incidence: Malignant CNS tumors
Radiographic Findings
• Fluoroscopic guided double contrast barium enema
o Innumerable varied sized radiolucent filling defects
o Carpet entire colon particularly rectosigmoid region
o May be widely scattered radiolucent filling defects
• Fluoroscopic guided double contrast UGI, small bowel
o Multiple small filling defects in stomach,
duodenum, jejunum & ileum
CT Findings
• NECT
o Imaging appearance varies due to relative amounts
of fibroblast proliferation/fibrosis/fat/collagen
content & vascularity of tumor
o Desmoid tumor & mesenteric fibromatosis
• Well or ill-defined; homo-/heterogeneous density
• ± Displacement or compression of bowel loops
• ± Areas of necrosis
• Desmoid location: Mesentery & abdominal wall
• CECT: Both desmoid & mesenteric fibromatosis:
Higher attenuation than muscle
• CT colonography after colonic air insufflation:
Endoluminal images show
o Small or large, sessile or pedunculated polyps
extending from colonic wall
o Polyps 10 mm & above; 90% Sensitivity
MR Findings
• Desmoid tumor & mesenteric fibromatosis
o T1WI: ! Signal intensity relative to muscle
o T2WI: Variable signal intensity (low, medium or
high) relative to muscle
o T1C+: Marked homo-/heterogeneous enhancement
Imaging Recommendations
• Double contrast barium studies (for polyps)
o En face, profile & oblique views
• NE + CECTi MR & T1C+ (for abdominal tumors)
• CT colonography (for polyps)
Top Differential Diagnoses
• Retained feces & food
• Lymphoid hyperplasia
• Metastases & lymphoma
• Pseudo polyps
• Primary colonic pneumatosis
Pathology
• Virtually all untreated patients develop colon cancer
• FAPSis inherited as an autosomal dominant trait
Diagnostic Checklist
• Check for family history: Colonic polyps, abdominal
soft tissue tumors & malignancies at a young age
• 500-2500 polyps carpeting entire colon-rectosigmoid
• Gardner syndrome: Soft tissue tumors, bony
osteomas, dental defects & periampullary cancer
I DIFFERENTIAL DIAGNOSIS
Retained feces & food
• Filling defects in barium pool mimicking polyps
Lymphoid hyperplasia
• Lymphoid follicles
o Aggregates of lymphocytes in muscularis mucosae
5
o Seen in 50% of barium studies (kids); 13% (adults) 57
o Enlarged or hyperplastic: Infectious, neoplastic,
immunologic & inflammatory diseases
• Barium studies
o Innumerable small or tiny radiolucent nodules
o Usually generalized (duodenum, small-bowel, colon)
o Simulate small size adenomatous polyposis
• Distinguished by clinical history & generalized pattern
Metastases & lymphoma
• Metastases (e.g., malignant melanoma, breast, lung)
o Malignant melanoma
• Smooth polypoid submucosal lesions of different
sizes seen as filling defects may mimic polyps
• Polypoid lesion with ulcers & radiating folds form
a typical "spoke-wheel" pattern
o Breast carcinoma: Mural nodules simulating polyps
o Bronchogenic carcinoma
• Single or multiple intramural lesions (flat or
polypoid) indistinguishable from polyps
• Frequently ulcerated, narrowing & obstruction
• Lymphoma
o Distribution: Stomach (51%), small-bowel (33%),
colon (16%), esophagus (<1%)
o Low grade MALT lymphoma
• Seen only in stomach due to H. pylori gastritis
• Confluent varying-sized nodules (filling defects)
• May be indistinguishable from gastric polyps
o Non-Hodgkin lymphoma
• Small or bulky polypoid masses may mimic polyps
• Smooth surface, broad base, sessile lesions ±
central depressions or ulcerations
• Bull's-eye" sign: Polypoid mass with ulceration
• Markedly thickened bowel wall & folds
• Regional or widespread adenopathy seen
 FAMILIAL POLYPOSIS
Pseudopolyps
• Example: Ulcerative colitis (common); granulomatous
colitis
• Two types of pseudopolyps
o Inflammatory pseudopolyps
o Postinflammatory pseudopolyps
• Inflammatory pseudo polyps
o Islands of elevated, inflamed, edematous mucosa
surrounded by ulceration appear as pseudopolyps
• Represent remnants of pre-existing mucosa &
submucosa rather than new growths
o Natural progression of collar button ulcers, which
extend, interconnect & mimics pseudopolyps
• Postinflammatory pseudopolyps (mucosal overgrowth)
o Regenerated mucosa results in polypoid lesions
• May be small & rounded; long & filiform or
bushlike structure simulating a villous adenoma
o Seen during mucosal healing, so they are termed
postinflammatory pseudo polyps
o Also seen after ischemia or after any severe infection
Primary colonic pneumatosis
• Cystic intramural collections of gas in colon
• Asymptomatic
• Not due to ischemia
5 !PATHOLOGY
58 General Features
• General path comments
o Proliferation of mucosa
o Polyps begin in rectosigmoid & spread entire colon
o Polyps are indistinguishable from sporadic
adenomatous polyps
o Virtually all untreated patients develop colon cancer
• Genetics: Abnormal or deletion of APC gene located
on chromosome 5q
• Etiology
o FAPSis inherited as an autosomal dominant trait
o Occasionally due to spontaneous mutations
• Epidemiology: FAPSaffects 1 in 10,000 people in US
Gross Pathologic & Surgical Features
• Innumerable polyps carpeting colonic mucosa
• Desmoid tumor
o Confined to muscle, fascia or deeply infiltrate
o Size: 5-20 em; firm & gritty texture; lack capsule
o Cut surface: Glistening white + trabeculated
Microscopic Features
• Adenomas
o Tubular, tubulovillous & villous; ± atypia or mitosis
• Desmoid tumor
o Spindle shaped cells & dense bands of collagen
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Rectal bleeding & diarrhea (75% cases)
o Asymptomatic, pain, mucus discharge
o Family history of colonic polyps (66% cases)
• Extraintestinal manifestations: Gardner syndrome
o Epidermoid cyst, lipoma, fibroma, desmoid tumors
(3-29%), mesenteric fibromatosis, peritoneal
adhesions, retroperitoneal fibrosis
o Osteomas: Membranous bone-50%; mandible-80%
o Teeth: Odontoma, unerupted supernumerary teeth
o Thyroid cancer: Papillary type more common in
girls & young women
Demographics
• Age: Mean age 16 years; by 35 years 95% have polyps
• Gender: Equal in both males & females
Natural History & Prognosis
• Complications
o Polyps: Malignant transformation
• Colon> periampullary > stomach> jejunum
o Colon carcinoma by age 34-43 years
• Prognosis
o Bad if abdominal desmoids, colonic carcinoma or
ampullary carcinoma develop
Treatment
• Prophylactic total colectomy at about 20 years of age
• Permanent ileostomy, Kock pouch
• Continent endorectal pull-through pouch
I DIAGNOSTIC CHECKLIST
Consider
• Check for family history: Colonic polyps, abdominal
soft tissue tumors & malignancies at a young age
Image Interpretation Pearls
• 500-2500 polyps carpeting entire colon-rectosigmoid
• Gardner syndrome: Soft tissue tumors, bony osteomas,
dental defects & periampullary cancer
I SELECTED REFERENCES
1. Spigelman AD: Extracolonic polyposis in familial
adenomatous polyposis: so near and yet so far. Gut.
53(3):322, 2004
2. Macari M et al: Diagnosis of familial adenomatous
polyposis using two-dimensional and three-dimensional
CT colonography. AJRAm. J. Roentgenol. 173: 249-250,
1999
3. Hizawa K et al: Desmoid tumors in familial adenomatous
polyposis/Gardner's syndrome. J Clin Gastroenterol.
25(1):334-7, 1997
4. Hamed RK et al: The hamartomatous polyposis syndromes:
clinical and radiologic features. AJRAm J Roentgenol.
164(3):565-71, 1995
5. Rustgi AK: Hereditary gastrointestinal polyposis and
nonpolyposis syndromes. N EnglJ Med. 331(25):1694-702,
1994
6. Casillas J et al: Imaging of intra- and extraabdominal
desmoid tumors. RadioGraphies 11: 959-968, 1991
7. Hamed RK et al: Extracolonic manifestations of the
familial adenomatous polyposis syndromes. A]R Am J
Roentgenol. 156(3):481-5, 1991
8. Bartram CI et al: Colonic polyp patterns in familial
polyposis. AJR 142: 305-308, 1984
 FAMILIAL POLYPOSIS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
subtle polypoid thickening of
descending colon, large mass
in cecum. (Right) Axial
CECT, 25 year old woman
with familial polyposis.
Extensive rectal cancer with
local invasion and
lymphadenopathy.

(Left) Air contrast BEshows

innumerable polyps in
rectosigmoid colon. (Right)
Air contrast BE shows
innumerable polyps in 5
rectosigmoid colon.
59

(Left) Upper GI series shows

polyps in duodenum.
Familialpolyposis-Gardner
syndrome. (Right) Axial
CECT shows extensive liver
metastases in 23 year old
man with familial polyposis.
 GARDNER SYNDROME

Axial CECT in a 30 year old man with Gardner Axial CECT shows thickening of the rectosigmoid wall
syndrome shows multiple hepaUc metastases. due to innumerable polyps + invasive rectal carcinoma.

o Familial polyposis: Innumerable varied sized

ITERMINOLOGY radiolucent filling defects
Definitions
CT Findings
• Familial adenomatous polyposis + extracolonic lesions
5 • NECT
o Desmoid tumors & mesenteric fibromatosis: Well or
ill-defined; homo- or heterogeneous masses
60 I IMAGING FINDINGS o Other carcinomas: Isodense or heterogenous
General Features attenuation of the mass
• Best diagnostic clue: Innumerable, colonic, radiolucent • CECT: Desmoid tumors & mesenteric fibromatosis:
filling defects with extraintestinallesions Increased attenuation; greater than muscles
• Other general features • CT colonography
o Combination of familial polyposis coli and o Familial polyposis: Small or large, sessile or
• Osteomas; dental abnormalities pedunculated polyps extending from wall
• Desmoid tumor & mesenteric fibromatosis o May see colon carcinoma, liver mets
• Epidermoid cysts & fibromas of skin Imaging Recommendations
• Adrenal, thyroid & liver carcinomas • Best imaging tool
• Congenital pigmented lesions of retina o Familial polyposis: Fluoroscopic-guided barium
o Not all extracolonic lesions occur in same patient enema or CT colonography
Radiographic Findings o Osteoma: Radiography
• Radiography o Desmoid tumors & mesenteric fibromatosis: CT
o Osteomas
• Cortical thickening of angle of mandible, sinuses,
outer table of skull, flat bones & long bones I DIFFERENTIAL DIAGNOSIS
• Size: Indiscernible to several cm Retained fecal debris
• Single to dozens; localized or diffuse
• Filling defects on dependent surface in barium pool
• Fluoroscopic-guided barium enema & UGI
mimicking polyps

DDx: Multiple Colonic Filing Defects

Retained Feces Lymphoid Hyperplasia Granulom. Colitis

 GARDNER SYNDROME

Key Facts
Imaging Findings Pathology
• Best diagnostic clue: Innumerable, colonic, • A variant of familial polyposis (very rare)
radiolucent filling defects with extraintestinallesions • Etiology: Autosomal dominant inheritance
Top Differential Diagnoses Diagnostic Checklist
• Retained fecal debris • Family history; colectomy to prevent colon
• Lymphoid hyperplasia carcinoma
• Pseudopolyps • Innumerable polyps carpeting entire colon with
• Metastases & lymphoma extraintestinal manifestations

• Osteomas, dental & retinal abnormalities & epidermal

Lymphoid hyperplasia cysts occur prior to puberty & familial polyposis
• Enlarged or hyperplastic lymphoid follicles • Desmoid tumors & mesenteric fibromatosis usually
• Innumerable small or tiny radiolucent nodules occur post-operation
• Usually generalized (duodenum, small bowel or colon) o Histologically benign but aggressive growth; 1
Pseudopolyps morbidity and mortality
• Inflammatory pseudopolyps Treatment
o Examples: Ulcerative or granulomatous colitis • Prophylactic colectomy to prevent colon carcinoma
o Islands of elevated, inflamed, edematous mucosa
surrounded by ulceration
• Postinflammatory pseudopolyps I DIAGNOSTIC CHECKLIST
o Mucosal healing; mimicking villous adenoma
o Small & round; long & filiform; bushlike Consider
Metastases & lymphoma
• Family history; colectomy to prevent colon carcinoma 5
• Metastases (e.g., Malignant melanoma, breast or lung) Image Interpretation Pearls 61
• Lymphoma (e.g., low grade MALT or non-Hodgkin) • Innumerable polyps carpeting entire colon with
extraintestinal manifestations

!PATHOLOGY
I SELECTED REFERENCES
General Features
1. Van Epps KJ et al: Epidermoid inclusion cysts seen on CT
• General path comments of a patient with Gardner's syndrome. AJRAm J
o A variant of familial polyposis (very rare) Roentgenol. 173(3):858-9, 1999
o Dental abnormalities: Unerupted or supernumerary 2. Kawashima A et al: CT of intraabdominal desmoid tumors:
teeth, dentigerous cysts & odontomas is the tumor different in patients with Gardner's disease?
o Epidermoid (sebaceous) cysts & fibromas of skin: AJRAm J Roentgenol. 162(2):339-42, 1994
Common on legs, face, scalp & arms; mm to em 3. Nannery WM et al: Familial polyposis coli & Gardner's
o Congenital pigmented lesions of retina syndrome. N J Med. 87(9):731-3, 1990
• Single, multiple, bilateral; 0.1 to 1.0 disc diameter
• Darkly pigmented; round, oval or kidney-shaped
• Genetics: Mutation in APC gene at Sq22 I IMAGE GALLERY
• Etiology: Autosomal dominant inheritance
• Associated abnormalities: Colonic adenomatous
polyps - colon carcinoma in 100% if not treated

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o rectal bleeding, diarrhea
o Skin, dental or retinal abnormalities
Demographics
• Age: Mean age of diagnosis is 22 years of age (Left) Barium study shows numerous jejunal polyps (adenomas).
• Gender: M:F = 1:1 (Right) Axial CECT in a patient who had colectomy for Gardner
polyposis. Large rapidly-growing mesenteric masses are desmoid
Natural History & Prognosis tumors.
• Congenital pigmented lesions of retina may be earliest
clinically detectable lesion
 SIGMOID VOLVULUS

Graphic shows dilated, twisted, elongated sigmoid Supine radiograph shows dilation of entire colon.
colon with venous engorgement + colonic obstruction. Vertical white line (arrow) represents the apposed walls
of the dilated, inverted sigmoid colon and points toward
the mesenteric volvulus.

0
o Twist> 360 do not resolve spontaneously
/TERMINOLOGY
Radiographic Findings
Abbreviations and Synonyms
• Radiography
• Volvulus of sigmoid colon
5 Definitions
o Sigmoid volvulus
• Vertical dense white line: Apposed inner walls of
62 • Torsion or twisting of sigmoid colon around its sigmoid colon pointing toward the pelvis
mesenteric axis • Closed loop obstruction: Segment of bowel
obstructed at two points
• Gas in proximal small intestine and colon;
I IMAGING FINDINGS absence of gas in rectum
• Absent rectal gas in spite of prone or decubitus
General Features views
• Best diagnostic clue: Dilated sigmoid colon with • "Northern exposure" sign: Dilated, twisted sigmoid
inverted U configuration and absent haustra colon projects above transverse colon
• Location: At midline; directed toward RUQ or LUQ ~ • Apex above TlO vertebra and under left
elevation of hemidiaphragm hemidiaphragm; directed toward right shoulder
• Other general features o Cecal volvulus
o Types of colonic volvulus • Dilated air-filled cecum in an ectopic location
• Sigmoid volvulus: 60-75% • Cecal apex in LUQ
• Cecal volvulus: 22-33% • Kidney or coffee bean-shaped gas-filled cecum
• Transverse colon volvulus: 2-4% • One or two haustral markings usually seen
• Splenic flexure volvulus: < 1% • Markedly distended gas or fluid-filled small bowel;
• Compound volvulus: Very rare little gas in distal colon
o Colonic volvulus is rare in children o Splenic flexure volvulus
• Radiography may be interpreted as normal; used • Dilated, featureless, air-filled bowel loop in LUQ;
to exclude other causes of abdominal pain and separate from stomach
free air o Compound volvulus

DDx: Dilated Colon

Acute Ileus Ogilvie Syndrome Colon Cancer Cecal Volvulus

 SIGMOID VOLVULUS
Key Facts
Terminology
• Torsion or twisting of sigmoid colon around its
mesenteric axis
Imaging Findings
• Best diagnostic clue: Dilated sigmoid colon with
inverted U configuration and absent haustra
• Location: At midline; directed toward RUQ or LUQ -
elevation of hemidiaphragm
• Gas in proximal small intestine and colon; absence of
gas in rectum
• Absent rectal gas in spite of prone or decubitus views
• "Northern exposure" sign: Dilated, twisted sigmoid
colon projects above transverse colon
• "Beaking": Smooth, tapered narrowing or point of
torsion at rectosigmoid junction
• Dilated sigmoid loop in mid-abdomen extending
to RLQ with distended small bowel
• Medially deviated distal left colon
• Fluoroscopic-guided water-soluble contrast enema
o Sigmoid volvulus (can use low pressure barium
enema without balloon inflation)
• "Beaking": Smooth, tapered narrowing or point of
torsion at rectosigmoid junction
• Mucosal folds often show a corkscrew pattern at
point of torsion
• Shouldering: Localized wall thickening at site of
twist (chronic)
o Cecal volvulus
• "Beaking" at mid-ascending colon
o Transverse colon volvulus
• "Beaking" at level of transverse colon
• Two air-fluid levels in dilated transverse colon
(helpful in distinguishing from cecal volvulus)
o Splenic flexure volvulus
• "Beaking" at LUQ
CT Findings
• CECT
o Progressive tapering of afferent and efferent limbs
leading into the twist or "beaking"
o "Whirl" sign: Tightly twisted mesentery and bowel
o Compound volvulus
• Medial deviation of distal left colon with pointed
appearance of its medial border
Imaging Recommendations
• Radiography (supine, upright, prone and decubitus
views)
• Fluoroscopic-guided water-soluble contrast enema;
helical CT
I DIFFERENTIAL DIAGNOSIS
Acute ileus
• Post-op, medication, post-traumatic injury and
ischemia
• Dilated large bowel with no transition point
• "Whirl" sign: Tightly twisted mesentery and bowel
• Radiography (supine, upright, prone and decubitus
views)
Top Differential Diagnoses
• Acute ileus
• Functional megacolon
• Distal colon obstruction
Clinical Issues
• Acute or insidious in onset
• Abdominal pain « 33%), vomiting and distension
Diagnostic Checklist
• Acute abdomen; rule out other causes of obstruction
• Dilated sigmoid colon in inverted U configuration;
absent haustra; "beaking"; "whirl" sign
• Air-fluid levels observed, but no peristalsis
• No colonic obstruction
Functional megacolon
• Gross constipation without organic cause
• Markedly dilated, ahaustral, air or stool-filled colon
• Ogilvie Syndrome - non-obstructive dilation of cecum
5
Distal colon obstruction
63
• Change in stool caliber over several months
• Gas-filled intestinal loops proximal to obstruction; no
gas seen distally
• Abrupt transition at site of obstruction
• Malignancy
o Most common (55%) cause of colonic obstruction
o Insidious in onset
o Weakness, weight loss and anorexia
o "Apple-core" configuration with destruction of
mucosa
o Positive fecal occult blood test is highly suggestive of
colon cancer
• Stricture secondary to diverticulitis
o Second most common (12%) cause of colonic
obstruction
o History of recurrent attacks of diverticulitis
o Other diverticula are present
I PATHOLOGY
General Features
• Etiology
o Major predisposing factors for colonic volvulus
• Redundant segment of bowel that is freely
moveable within the peritoneal cavity
• Close approximation of points of bowel fixation
o Sigmoid volvulus
• Diet: 1 Fiber - 1 bulk of stool and elongates colon
• Chronic constipation and obtundation from
medications - gaseous distension
• Degree of rotation relative to chance of
nonsurgical decompression: 180°:35%; 360°:50%;
540°:10%
 SIGMOID VOLVULUS
o Cecal volvulus
• Congenital defect in attachment
• Postpartum ligamentous laxity and mobile cecum
• Colon distension (pseudo-obstruction; distal
tumor, endoscopy, enema or postoperative ileus)
o Transverse colon volvulus
• Failure of normal fixation of mesentery ~ 1
mobility of right colon and hepatic flexure
o Splenic flexure volvulus
• Postoperative adhesions
• Congenital or surgical removal of normal
attachments to abdominal wall
o Compound volvulus
• Also known as ileosigmoid knot
• Hyperactive ileum winding around narrow pedicle
of a passive sigmoid colon
o Etiology in children
• Malrotation and other mesenteric attachment
abnormalities
• Constipation (mental retardation, Hirschsprung's
disease, cystic fibrosis or aerophagia)
• Epidemiology
o Third most common (10%) cause of colonic
obstruction
o Incidence of colonic volvulus
• U.S. and other western countries: 1-4% of
intestinal obstructions
5 • Africa and Asia: 20-25% of intestinal obstructions
o Incidence of sigmoid volvulus
64 • U.S.: 1-2% of intestinal obstructions
• Increased incidence in elderly men and residents
of nursing homes and mental hospitals
(constipation and obtundation)
• Increased significantly in Scuth America and
Africa (1 fiber in diet)
o Colonic volvulus in children
• Mean is 7 years of age
• Boys to girls: 2-3:1
• Associated abnormalities
o Comorbid disease in sigmoid volvulus
• 30% with psychiatric disease; 13% are
institutionalized at time of diagnosis
Gross Pathologic & Surgical Features
• Twisted narrow segment with marked proximally
dilated bowel loop
Microscopic Features
• Localized thickening of mucosal folds; ischemic and
necrotic changes
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute or insidious in onset
o Abdominal pain « 33%), vomiting and distension
o Transverse colon volvulus
• Severe vomiting (compression of duodenojejunal
junction at root of mesentery)
o Compound volvulus
• Rapid deterioration (greater than other colonic
volvulus)
• Pain out of proportion to physical findings and
absolute constipation
o Diagnosis
• Sigmoid volvulus and cecal volvulus: Diagnosed
by radiography (75%)
• Transverse colon and splenic flexure volvulus:
Diagnosed by fluoroscopic-guided water-soluble
contrast enema
Demographics
• Age
o Sigmoid volvulus: 60-70 years of age
o Cecal volvulus: Younger age than sigmoid
Natural History & Prognosis
• Complications
o Closed loop obstruction ~ strangulation
o Ischemia, necrosis (15-20%) and perforation
o Ileosigmoid knot ~ strangulation and gangrene of
small bowel within hours
• Prognosis
o Uncomplicated: Good; complicated: Poor
o Colonic volvulus
• 8% mortality (from gangrenous bowel)
o Sigmoid volvulus
• 40-50% recurrence after non operative reduction
• 3% recurrence after nonoperative and operative
reduction
o Transverse colon volvulus: Up to 33% mortality
Treatment
• Sigmoid volvulus
o Nonoperative: Proctoscopic or colonoscopic
decompression of obstruction ± stabilization by
inserting rectal tube (successful 70-80% of attempts)
o Nonoperative and operative: Decompression,
mechanical cleansing and elective sigmoid resection
• Complicated cases: Surgical emergency
• Follow-up
o Sigmoid volvulus: Fluoroscopic-guided water-soluble
contrast enema to rule out underlying colon cancer
I DIAGNOSTIC CHECKLIST
Consider
• Acute abdomen; rule out other causes of obstruction
Image Interpretation Pearls
• Dilated sigmoid colon in inverted U configuration;
absent haustra; "beaking"; "whirl" sign
I SELECTED REFERENCES
1. Moore C] et al: CT of cecal volvulus: unraveling the image.
A]R Am] Roentgenol. 177(1):95-8, 2001
2. Lee SH et al: The ileosigmoid knot: CT findings. A]RAm]
Roentgenol. 174(3):685-7,2000
3. Dulger M et al: Management of sigmoid colon volvulus.
Hepatogastroenterology. 47(35):1280-3, 2000
4. ]avors BR et al: The northern exposure sign: A newly
described finding in sigmoid volvulus. A]R 173:571-574,
1999
S. Catalano 0: Computed tomographic appearance of
sigmoid volvulus. Abdominal Imaging 21:314-317, 1996
 SIGMOID VOLVULUS
I IMAGE GALLERY
Typical
(Left) Supine radiograph
shows dilated colon. The
apex of the sigmoid colon
(arrow) is above the
transverse colon, the
"northern exposure" sign of
sigmoid volvulus. (Right)
Single contrast BE shows a
smooth tapered beak
obstructing the lumen of the
sigmoid colon.

Typical
(Left) Axial CECT of sigmoid
volvulus shows a diffuse
dilation of colon. There is a
swirl of sigmoid mesocolic
blood vessels that converge
at the site of volvulus
5
(arrow). (Right) Axial CECT
65
shows swirl of mesocolic
vessels at the base of the
volvulus (arrow).

(Left) Supine radiograph

shows dilated, inverted "U"
shaped sigmoid colon.
(Right) Single contrast BE
shows twist + beak at point
of volvulus with dilated
colon beyond the twist.
 CECAL VOLVULUS

Graphic shows twist (volvulus) of ascending colon, Upright abdominal radiograph shows dilated cecum
obstructing lumen and blood supply. Cecum, on a with air-fluidlevel pointing toward left upper quadrant.
mesentery dilated + displaced toward left upper Remainder of c%n collapsed.
quadrant.

o Markedly distended gas or fluid-filled small bowel;

ITERMINOLOGY little gas in distal colon
Abbreviations and Synonyms • Fluoroscopic-guided water-soluble contrast enema
• Volvulus of cecum, ascending colon o "Bird's beak" sign: Point of torsion at mid-ascending
5 Definitions
colon
CT Findings
66 • Rotational twist of the right colon on its axis;
associated with folding of the right colon • CECT
o Progressive tapering of afferent and efferent limbs
leading into the twist or "beaking"
o "Whirl" sign: Tightly twisted mesentery and bowel
I IMAGING FINDINGS at right mid-abdomen or RUQ
General Features
Imaging Recommendations
• Best diagnostic clue: Dilated, twisted cecum with tip
• Best imaging tool: Fluoroscopic-guided water-soluble
pointing to left upper quadrant
contrast enema
• Location
o Twist is distal to ileocecal valve (term is a misnomer)
o Cecum is located in mid-abdomen or LUQ
o Terminal ileum swings around the dilated bowels
I DIFFERENTIAL DIAGNOSIS
Radiographic Findings Sigmoid volvulus
• Dilated, ahaustral sigmoid loop with inverted U
• Radiography
o Dilated air-filled cecum in an ectopic location configuration
o Single, long air-fluid level • Dilated proximal colon; Gas-less distally
o Cecal apex at LUQ Acute ileus
o Medially placed ileocecal valve produces soft tissue • Dilated colon to rectum with haustra pattern
indentation ~ kidney or coffee bean-shaped
gas-filled cecum Distal colon obstruction
o One or two haustral markings usually seen • Gas and stool-filled colon

DDx: Marked Colonic Distention

Sigmoid Volvulus Acute Ileus Colon Cancer Olgilvie Syndrome

 CECAL VOLVULUS

Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Dilated, twisted cecum with tip • Sigmoid volvulus
pointing to left upper quadrant
• Single, long air-fluid level Clinical Issues
• Medially placed ileocecal valve produces soft tissue • Acute or insidious onset
indentation - kidney or coffee bean-shaped gas-filled • Abdominal pain, distension and vomiting
cecum Diagnostic Checklist
• Markedly distended gas or fluid-filled small bowel;
• Rule out ileus and Ogilvie syndrome
little gas in distal colon

• Diagnosis: 75% by radiography

Functional megacolon
• Gross constipation without organic cause Demographics
• Markedly dilated, ahaustral, air or stool-filled colon • Age: Younger patients than sigmoid volvulus
Ogilvie syndrome Natural History & Prognosis
• Colonic pseudo-obstruction without mechanical cause • Complications
o Ischemia, necrosis (15-20%) and perforation
• Prognosis
I PATHOLOGY o Uncomplicated: Good; Complicated: Poor

General Features Treatment

• General path comments • Colon oscopy to reduce volvulus (higher risk of
o Cecal bascule perforation than sigmoid volvulus)
• Anterior folding (not twisting) of cecum
positioned at mid-abdomen
• Complicated cases: Surgical emergency
• Surgical options: Cecopexy, cecostomy and resection
5
• Possibly due to adhesive band from previous 67
abdominal surgery
o Embryology-Anatomy I DIAGNOSTIC CHECKLIST
• Right colon is incompletely fused to posterior
parietal peritoneum (10-37% adults) Consider
• Etiology • Rule out ileus and Ogilvie syndrome
o Congenital defect in attachment of right colon
Image Interpretation Pearls
o Postpartum ligamentous laxity and a mobile cecum
• Massively dilated cecum at mid-abdomen, distended
o Colon distension (pseudo-obstruction, distal tumor,
loops of small bowel, "bird's beak" sign
endoscopy, enema, or postoperative ileus)
o Chronic constipation and laxative use
• Epidemiology
o One-third of all cases of colonic volvulus
I SELECTED REFERENCES
o 2-3% of colonic obstructions 1. Moore CJ et al: CT of cecal volvulus. AJR. 177:95-98, 2001
o 22-33% of colonic volvulus; second to sigmoid 2. Hemingway AP: Cecal volvulus: A new twist to the barium
• Associated abnormalities enema. British Journal Radiol. 53:806-807, 1980
o One third of patients have concomitant partially
obstructing lesion located more distally in the colon
o Malrotation and long mesentery [IMAGE GALLERY
Gross Pathologic & Surgical Features
• Twisted, markedly dilated segment with moderate
dilation of small intestine
Microscopic Features
• Localized thickening of mucosal folds; ischemic and
necrotic changes

ICLINICAL ISSUES
Presentation
• Most common signs/symptoms (Left) Supine radiograph shows dilated cecum in mid-abdomen,
o Acute or insidious onset pointed toward left upper quadrant. (Right) Cecal bascule. Enema
o Abdominal pain, distension and vomiting fills markedly dilated cecum (ascending colon) which is folded
acutely + displaced. Note ilea-cecal valve (arrow).
 SECTION 6: Spleen

Introduction and Overview

Spleen Anatomy and Imaging Issues 1-6-2

Congenital
Asplenia and Polysplenia 1-6-6
Accessory Spleen 1-6-10

Infection
Splenic Infection and Abscess 1-6-12

Vascular
Splenic Infarction 1-6-16

Trauma
Splenic Trauma 1-6-20

Neoplasm
Splenic Cyst 1-6-22
Splenic Tumors 1-6-26
Splenic Metastases and Lymphoma 1-6-30

Miscellaneous
Splenomegaly and Hypersplenism 1-6-34
 SPLEEN ANATOMY AND IMAGING ISSUES

Graphic shows some of the variations in splenic shape Axial CECT shows prominent medial lobulation of the
on axial sections. spleen, a common variant.

• Splenic red pulp

I IMAGING ANATOMY o Comprises the vascular tissue of the spleen
location • Splenic cords (plates of cells) lie between
• Usually left upper quadrant sinusoidsi red pulp veins drain sinuses
• Intraperitoneal o Most common source of primary
• Supported by gastrosplenic and splenorenalligaments nonhematolymphoid tumors
o Mottled enhancement, typical of early phase
Anatomic Relationships enhanced CT or MR is due to variable flow rates
• Diaphragm through cords and sinuses of red pulp
o Supero-Iaterally • Can simulate or hide splenic pathology on arterial
• Left kidney phase imaging
6 o Posterio-medially • Splenic white pulp
• Pancreas o Comprises the lymphatic tissues, organized similar
2 o Medially to lymph nodes
• Stomach o Gives rise to lymphatic tumors
o Anterio-medially • Most common splenic tumor, lymphoma
• Tail of pancreas inserts into spleno-renalligament • Frequently has notches and indentations on surface
o Pancreatic tail becomes intraperitoneal o On axial CT/MR sections may simulate laceration
o Pancreatitis involving tail can spread directly to the o Key differential feature is absence of perisplenic
spleen, lead to intra splenic pseudocyst hemorrhage
Key Concepts or Questions
I ANATOMY-BASED IMAGING ISSUES I • MR appearance of spleen
o Spleen has relatively long T1 and T2 relaxation
Normal Measurements times
• Size can vary widely among individuals o Appears dark relative to liver on T1 WI
oRange 100 to 250 cm3 , mean ISO cm3 in adults • Similar to renal cortex
• Size varies even in one person • Becomes abnormally dark with iron deposition
o Age, state of nutrition, body habitus, blood volume (transfusion hemochromatosis)
o Average about 12 cm length, 7 cm breadth by 4 cm o Liver metastases often similar in signal to normal
width spleen
o Length x width x breath should not exceed 470 cm3 • Spleen texture
o Can measure volume accurately by CT computation o Soft and pliable, relatively mobile
and summation of splenic area on sequential scans o Easily indented and displaced by masses and even
(limited clinical value) loculated fluid collections
o Splenomegaly often results in convexity of (usually o Changes position in response to resection of
concave) visceral surface adjacent organs
• Structure: Branching trabeculae subdivide the spleen • (E.g., post nephrectomy)
into communicating compartments • Splenic lymphatic tumors
o Branches of arteries, veins, nerves, lymphatics travel o Most common
through trabeculae o Lymphoma, leukemia
 SPLEEN ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
Primary benign splenic tumors Splenic cyst - solitary or multiple
• Hemangioma • Congential cyst
• Lymphangioma • Post-traumatic cyst
• Hamartoma • Parasitic cyst (hydatid)
• Lipoma • Abscess
• (Inflammatory pseudotumor) • Metastasis
• Intrasplenic pseudocyst
Splenic tumors: Malignant - primary
• Lymphoma Multiple complex or solid masses
• Hemangiopericytoma • Lymphoma, leukemia
• Angiosarcoma • Abscesses (immunocompromised)
• Hemangioendothelioma • Candida, mycobacterial, Pneumocystis, etc.
• Angiosarcoma
Splenic tumors: Malignant - metastatic • Sarcoidosis
• Melanoma (50% of cases) • Metastases
• Breast, lung, ovary, etc. • Multiple hemangiomas or lymphangioma

o Imaging appearance: Splenomegaly (often massive • Splenic infection

in leukemia, NHL)
• Splenic metastases
o Usually multiple and part of widespread disease
o Variety of sources, especially melanoma
o Contiguous spread (stomach, pancreas)
o Retrograde spread through splenic vein
• Primary vascular tumors
o Hemangioma
• Peripheral or solid enhancement
o Hamartoma
• Homogeneous hypervascular
o Lymphangioma
• Multicystic, subcapsular
o Littoral cell angioma
• Splenomegaly, multiple nodules
o Peliosis
• Multifocal, heterogeneous masses
o Hemangiopericytoma and hemangioendothelioma
• Solid mass with necrosis
o Angiosarcoma
• Multiple, heterogeneous, hypervascular; also in
liver
• Incidental splenic mass
o Patient with known malignancy
• Very aggressive tumor (e.g., melanoma), or tumor
affecting portal venous system (e.g., pancreatic
cancer); suspect metastasis
o No known primary tumor
• At high risk for lymphoma (e.g., AIDS, transplant
recipient, associated lymphadenopathy); suspect
lymphoma
• Immunosuppressed, chronically ill; suspect
opportunistic infection, peliosis, lymphoma
o Asymptomatic, healthy adult
• Echogenic or peripherally or uniform enhancing
mass; probable hemangioma
• Subcapsular, multicystic mass; probably
lymphangioma
o Symptomatic splenic mass not meeting these crite.ria
• Probably primary vascular tumor
• Probably requires splenectomy for diagnosis and
management
o Histoplasmosis and tuberculosis (TB) commonly
affect spleen
o Otherwise, uncommon, except in
immunocompromised patients
• AIDS, transplant recipients, leukemic, alcoholic
o Multiple small abscesses
• Candida (and other fungal), TB, Pneumocystis
o Single large abscess
• Usually bacterial
o Calcification
• Seen in treated abscesses (TB, fungal,
Pneumocystis)
• Splenic Infarction
o Relatively common cause of acute left upper
6
quadrant pain 3
o Appears as sharply marginated, wedge-shaped,
poorly-enhancing lesions abutting splenic capsule
o Etiologies
• Sickle cell and other hemoglobinopathies
• "Spontaneous" in any cause of splenomegaly
• Embolic (e.g., LV. drug abuse, endocarditis, atrial
fibrillation)
Imaging Pitfalls
• Heterogeneous enhancement of the spleen may
simulate or hide pathology
• Any mass or splenic parenchymal lesion suspected on
the basis of an arterial phase CT/MR image should be
confirmed on venous/parenchymal phase scans
TERMINOLOGY
Embryologic Events
• from dorsal mesogastrium during fifth fetal week
• Normally rotates to left
• Usually fixed into left subphrenic location by
peritoneal reflections linking it to the diaphragm,
abdominal wall, kidney, stomach
• Usually develops as one main "fused" mass of tissue
 SPLEEN ANATOMY AND IMAGING ISSUES

Axial CECT shows a splenic cyst with a calcified wall. Surgical photograph of splenectomy specimen shows a
splenic cyst with a calcified wall.

o Splenic artery ± aneurysm

Practical Implications
• Uncommon
• Failure of fusion o Infarcts
o Accessory spleen found in 10 to 30% of population o Hydatid disease
o Usually small (pea size) and near splenic hilum o Cysts
o Accessory spleen may be aberrant in location, may o Amyloid
increase in size, especially after splenectomy o Healed abscesses
• May enlarge after splenectomy performed for o Hamartomas
hypersplenism or tumor, resulting in relapse o Sickle cell anemia
• Enlarged or ectopic accessory spleen can simulate
tumor Splenomegaly
6 • May present as mass in pancreatic tail
• Diagnosis by radionuclide sulfur colloid or tagged
• Congestive
o E.g., cirrhosis, heart failure
4 RBC scan (more sensitive) • Neoplastic
• Spleen may be on long mesentery o E.g., lymphoma, leukemia, metastases
o "Wandering spleen" may result • Infection
o Spleen may be found in any intraperitoneal location o E.g., hepatitis, HIV, malaria
of the abdomen or pelvis • Hemolytic anemias
o May simulate a mass o E.g., thalassemia, heterozygous sickle cell
o May torse and cause acute splenic infarction • Extramedullary hematopoiesis
• Failure to develop o E.g., polycythemia vera, myelofibrosis
o Asplenia often associated with other congenital • Collagen vascular disease
anomalies including situs inversus and cardiac o E.g., Felty syndrome, rheumatoid arthritis
anomalies • Storage diseases
• High mortality, especially early death from sepsis o E.g., Gaucher, amyloid
• Polysplenia
o Also associated with cardiac and other anomalies,
azygous continuation of IVC I SELECTED REFERENCES
o Also associated with early mortality
1. Abbott RM et al: From the archives of the AFIP:primary
o Can be simulated by splenosis (heterotopic vascular neoplasms of the spleen: radiologic-pathologic
implantation and subsequent growth of splenic correlation. Radiographies. 24(4):1137-63, 2004
tissue following traumatic rupture of spleen) 2. Gore RM et al: Textbook of gastrointestinal radiology:
Spleen: differential diagnosis. 2nd ed. Philadelphia, WB
Saunders. pp 1925-1928, 2000
I CUSTOM DIFFERENTIAL DIAGNOSISI 3. Diihnert W: Radiology review manual. 4th ed,
Philadelphia, Lippincott, Williams and Wilkins, 2000
Multiple splenic lesions: Calcified 4. Reeder MM: Reeder and Felson's gamuts in radiology .3rd
ed. New York, Springer Verlag, 1993
• Common 5.. Warnke R et al: Tumors of the lymph nodes and spleen.
o Granulomas (TB, histoplasmosis) Washington, DC, Armed Forces Institute of Pathology.
o Opportunistic infection 1995
• Pneumocystis, mycobacterial
 SPLEEN ANATOMY AND IMAGING ISSUES
I IMAGE GALLERY

(Left) Axial NECT shows

heterogeneously calcified
spleen in a patient with
sickle cell anemia. (Right)
Photograph of surgical
specimen shows
discoloration of the splenic
parenchyma and capsule
due to chronic infarction and
calcification.

(Left) Axial CECT shows

multiple calcified granulomas
from healed histoplasmosis.
(Right) Axial CECT shows
innumerable focal splenic
lesions and splenomegaly;
sarcoidosis.

6
5

(Left) Axial CECT shows one

of several focal splenic
lesions; metastatic
melanoma. (Right) Axial
CECT shows multiple
wedge-shaped,
capsular-based
nonenhancing lesions;
infarcts due to cardiac assist
pump.
 ASPLENIA AND POLYSPLENIA

Axial CECT in a patient with asplenia shows midline Axial CECT in patient with asplenia shows left-sided IVC
enlarged (congested) liver + right-sided stomach (arrow).
(arrow). Extensive cardiac anomalies.

• Size: PSP: Varied size of splenic masses

ITERMINOlOGY
Abbreviations and Synonyms
• Abbreviations: Asplenia (ASP); polysplenia (PSP)
• Synonyms
o ASP: Asplenia syndrome; Ivemark syndrome;
bilateral right-sidedness
o PSP: Polysplenia syndrome; bilateralleft-sidedness
Definitions
• ASP: Congenital absence of splenic tissue, situs
6 ambiguous & associated anomalies
• PSP: Congenital abnormality characterized by multiple
6 small splenic masses, situs ambiguous & associated
anomalies
IIMAGING FINDINGS
General Features
• Best diagnostic clue
o ASP: Absence of spleen, abdominal aorta & IVC on
same side (usually right) & bilateral distribution of
right-sided viscera
o PSP: Multiple small splenic masses, intrahepatic
interruption of IVC with continuation of azygos
vein, bilateral distribution of left-sided viscera
• Location: PSP, right & left upper abdominal quadrants
• Morphology
o Polysplenia
• PSP: Number of spleens varies from 2-16
• Key concepts
o Asplenia syndrome: Right isomerism
• Situs ambiguous + bilateral right-sidedness
• Cardiovascular malformations (50%): Total
anomalous pulmonary venous return (almost
100%); endocardial cusion defect (85%); single
ventricle (51 %), transposition of great vessels
(58%), pulmonary stenosis or atresia (70%),
dextrocardia(42%), mesocardia, ventricular septal
defect, single atrioventricular valve, bilateral
superior vena cavae; absent coronary sinus
• Pulmonary: Abnormal distribution of lobes,
bilateral trilobed lungs
• ASP-gastrointestinal anomalies: Situs inversus,
imperforate anus, ectopic liver, annular pancreas,
esophageal varices, GB agenesis, Hirschsprung
disease, duplication/hypoplasia of stomach
• Genitourinary (15%): Horseshoe kidney, bilobed
urinary bladder, hydroureter, double collecting
system, cystic kidney
• Miscellaneous: Cleft palate, cleft lip, fused or
horseshoe adrenal, absent left adrenal, scoliosis,
bicornuate uterus, single umbilical artery, lumbar
myelomeningocele

DDx: More or less Than One Spleen

Splenosis Splenosis Splenosis Accessory Spleen

 ASPLENIA AND POLYSPLENIA
Key Facts
Terminology
• Abbreviations: Asplenia (ASP); polysplenia (PSP)
• ASP: Asplenia syndrome; lvemark syndrome; bilateral
right-sidedness
• PSP: Polysplenia syndromei bilateralleft-sidedness
Imaging Findings
• ASP: Absence of spleen, abdominal aorta & lVC on
same side (usually right) & bilateral distribution of
right-sided viscera
• PSP: Multiple small splenic masses, intrahepatic
interruption of IVC with continuation of azygos vein,
bilateral distribution of left-sided viscera
• Location: PSP, right & left upper abdominal
quadrants
• PSP: Number of spleens varies from 2-16
o Polysplenia syndrome: Left isomerism
• Situs ambiguous + bilateralleft-sidedness
• Cardiac: Continuation of azygos vein (65%);
transposition of great vessels (13%), double outlet
right ventricle (13%), pulmonary valvular stenosis
(23%), sub aortic stenosis or atresia
• PSP-gastrointestinal: Esophageal/duodenal atresia,
TEF, semi annular pancreas, gastric duplication,
short bowel, absent GB, biliary atresia,
malrotation
• GU anomalies: Renal agenesis, renal/ovarian cysts
Radiographic Findings
• Radiography
o Asplenia: Show situs ambiguous, situs solitus or situs
inversus
• Spleen: No distinct visible splenic contour
• Liver: Symmetric & midline in its position
• Stomach: Right, left or central in position
• Malrotation of bowel
• Cardia: Mesocardia or dextrocardia
• Right-sided bronchial pattern & a minor fissure
may be seen bilaterally
• Superior mediastinal widening (due to bilateral
superior vena cava)
• Both pulmonary arteries anterior to trachea (on
lateral chest film)
• Bronchography: Bilateral eparterial bronchi on
frontal view (pulmonary arteries inferior to
bronchi)
o Polysplenia
• Frontal view: Para tracheal soft tissue prominence
(dilated azygos or hemiazygos vein) mimicking a
mediastinal mass
• Chest lateral view: Both pulmonary arteries
posterior to trachea & absence of lVC
CT Findings
• Asplenia
o NECT
• Absence of spleen
• Situs abnormalities: Liver, gallbladder, stomach,
bowel, heart, trilobed lungs
• ASP-gastrointestinal anomalies: Situs inversus,
imperforate anus, ectopic liver, annular pancreas,
esophageal varices, GB agenesis, Hirschsprung
disease, duplication/hypoplasia of stomach
• PSP-gastrointestinal: Esophageal/duodenal atresia,
TEF, semi annular pancreas, gastric duplication, short
bowel, absent GB, biliary atresia, malrotation
Top Differential Diagnoses
• Splenectomy
• Splenosis
• Accessory spleens
Diagnostic Checklist
• Centrally located left lobe of liver can simulate as
spleen on US & diagnosis of ASP may be missed
• Differentiate PSP from accessory spleens & splenosis
o CECT
• Vascular anatomy well depicted
• IVC & abdominal aorta lie on same side of spine
(usually right side with aorta lying posteriorly)
• Polysplenia
o NECT
• 2-16 splenic masses in right & left upper
quadrants
• ± Asymmetric liver & midgut malrotation
• ± Abdominal situs solitus or situs inversus
o CECT
• Absence of IVC between renal & hepatic veins
with independent drainage of hepatic veins into
right atrium
• Prominent azygos or hemiazygos vein
6
• Crossing of IVC in front of aorta to enter a 7
common atrium on right ("crossover", not unique
to ASP)
Angiographic Findings
• Conventional
o Asplenia
• Splenic artery absent; entire celiac axis may arise
from superior mesenteric artery
• IVC & abdominal aorta lie on same side (usually
right): Virtually pathognomonic feature of ASP
o Polysplenia
• Multiple spleens + common splenic/celiac artery
• Variations in course of IVC: Absence of
intrahepatic segment of IVCi crossover of IVC in
front of aorta
Nuclear Medicine Findings
• Asplenia
o Tc sulfur colloid or tagged RBC scan
• Absence of spleen
• Hepatic symmetry, prominent left lobe of liver
• Polysplenia: Multiple splenic tissues
o Tc99m labeled heat-damaged RBC scan
• More sensitive in detecting splenic tissue
o Hepatobiliary imaging
• Differentiate hepatic from splenic tissue
• Presence & position of gallbladder
 ASPLENIA AND POLYSPLENIA
Imaging Recommendations • 10-15% may not present clinically until adulthood
• Acute abdominal pain: Due to splenic infarction
• Best imaging tool
o Asplenia: Helical CT & Tc99m RBC scan Demographics
o Polysplenia • Age
• CT; US; MR can demonstrate size, position, o ASP: Newborn or infant
number of spleens & relationship to liver & bowel o PSP: Infant or adult age group
• Gender
o Asplenia: M > F
I DIFFERENTIAL DIAGNOSIS o Polysplenia: M < F
Splenectomy Natural History & Prognosis
• No splenic visualization after surgical splenectomy • Asplenia
o Patients are prone to overwhelming septicemia
Splenosis post-operatively
• Traumatized splenic tissue scattered in abdominal o Prognosis: Usually poor
cavity, where it attaches to adjacent peritoneal surface o Mortality rate: 80% die by end of 1st year of life due
• Multiple small encapsulated sessile implants (few to cardiac failure & post-operative complications
mm-3 cm in size) • Polysplenia
o Prognosis: Usually good
Accessory spleens
o Mortality rate
• Range from one to six in number
• 50-60% mortality in first year of life
• Usually found near splenic hilum along course of
• 25% of patients live up to 5 years of age
splenic vessels or within layers of omentum
• 10% survive to midadolescence
• Imaging findings identical to normal splenic tissue
Treatment
• Asplenia: Prophylactic antibiotics (not needed in PSP)
!PATHOLOGY o Associated cardiac disease: Surgical correction
General Features
• Etiology I DIAGNOSTIC CHECKLIST
o Uncertain
o Asplenia: Delayed embryonic body curvature Image Interpretation Pearls
o Polysplenia: Accelerated embryonic body curvature • Centrally located left lobe of liver can simulate as
o Altered timing in development of embryonic body spleen on US & diagnosis of ASP may be missed
6 curvature leads to visceroatrial situs abnormalities • Differentiate PSP from accessory spleens & splenosis
o Pressure of adjacent structures may interfere with
8 splenic blood supply
• Epidemiology: Asplenia: 1 in 40,000 live births I SELECTED REFERENCES
• Associated abnormalities: Pulmonary, cardiovascular,
1. Combs LSet al: Evaluation of spleen in children with
gastrointestinal, genitourinary & miscellaneous heterotaxia and congenital heart disease. Tenn Med.
anomalies 97(4):161-3,2004
2. Fulcher ASet al: Abdominal manifestations of situs
Gross Pathologic & Surgical Features anomalies in adults. Radiographies. 22(6):1439-56, 2002
• ASP: Congenital absence of spleen 3. Paterson A et al: A pattern-oriented approach to splenic
• PSP: Multiple splenic tissues in upper quadrants imaging in infants and children. Radiographies.
19(6):1465-85, 1999
Microscopic Features 4. Applegate KEet al: Situs revisited: imaging of the
• ASP: Heinz or Howell-Jolly bodies (absent in PSP) heterotaxy syndrome. Radiographies. 19(4):837-52;
o RBC inclusions discussion 853-4, 1999
5. Ruscazio M et al: Interrupted inferior vena cava in asplenia
syndrome and a review of the hereditary patterns of
I CLINICAL ISSUES visceral situs abnormalitie~. AmJ Cardiol. 81(1):111-6,
1998
6. Nakada K et al: Digestive tract disorders associated with
Presentation asplenia/polysplenia syndrome. J Pediatr Surg. 32(1):91-4,
• Most common signs/symptoms 1997
o ASP: Cardiopulmonary disease (83%); cyanosis in 7. Freeman JL et al: CT of congenital and acquired
neonatal period or infancy; bowel obstruction (17%) abnormalities of the spleen. Radiographies. 13(3):597-610,
o PSP 1993
• Cardiac disease; acyanotic left-to-right shunts such 8. Chitayat D et al: Prenatal diagnosis of asplenia/polysplenia
as septal defects syndrome. AmJ Obstet Gynecol. 158(5):1085-7, 1988
• Heart murmur, congestive heart failure, occasional
cyanosis, heart block
• Jaundice (extrahepatic biliary obstruction)
• Bowel malrotation, obstruction, pain (infarction)
 ASPLENIA AND POLYSPLENIA

I IMAGE GALLERY

Typical
(Left) Axial NECT in patient
with polysplenia shows
absent IVe, azygous
continuation. (Right) Axial
CECTshows polysplenia and
congenital absence of all but
the head of the pancreas
(arrow).

Typical
(Left) Axial CECT in patient
with polysplenia shows situs
ambiguous, absent 1Ve,
azygous continuation.
(Right) Axial CECT in a
patient with polysplenia
shows retroaortic right renal
vein leading to azygous
continuation.

6
9

Typical
(Left) Axial CECT in patient
with polysplenia shows liver
abnormalities and azygous
continuation. (Right) Axial
CECT in patient with
polysplenia shows prior
resection of small intestine
due to volvulus and
infarction.
 ACCESSORY SPLEEN

Axial CECT shows small spherical accessory spleen Axial CECT shows hypertrophied accessory spleen,
(arrow) near splenic hilum. following splenectomy.

ITERMINOlOGY o Same enhancement as normal main spleen

o ± Supplying branch of splenic artery
Definitions
MR Findings
• Ectopic splenic tissue of congenital origin
• T1WI: Hypointense; T2WI: Hyperintense
Ultrasonographic Findings
I IMAGING FINDINGS • Real Time
o Splenic artery and vein (90% of cases)
General Features o Parenchymal bridges between main spleen and
• Best diagnostic clue: Small nodule with same texture accessory spleen
and enhancement of normal spleen
6 • Location Angiographic Findings
o In or near splenic hilum or ligaments (most cases) • Conventional: Blood supplied by a branch of splenic
10 o Anywhere in abdomen or retroperitoneum (20% of artery and drained into splenic veins
cases), especially around tail of pancreas
o Embedded within pancreatic tail (rare) Nuclear Medicine Findings
o Usually left upper quadrant, above renal pedicle; • Technetium sulfur colloid
may also be in paratesticular, diaphragmatic, o Functional splenic tissue, differentiate from bleeding
pararenal and gastric sites by consistent size, shape & location
o 1 focus (88%), 2 foci (9%), > 2 foci (3%); multiple Imaging Recommendations
foci usually clustered in 1 location
• Best imaging tool: CT followed by nuclear scintigraphy
• Size: Varies from mm to several em, usually < 2.5 em
• Morphology: Same texture as main spleen
CT Findings I DIFFERENTIAL DIAGNOSIS
• NECT: Single/multiple, round/ovoid, uniform soft
Splenosis
tissue
• Usually result of trauma, portions of disrupted spleen
• CECT
implant anywhere including abdomen, pelvis, chest

DDx: left Upper Quadrant "Mass"

Splenosis Splenosis Polysplenia Polysplenia

 ACCESSORY SPLEEN

Key Facts
Terminology Top Differential Diagnoses
• Ectopic splenic tissue of congenital origin • Metastases
• Visceral mass
Imaging Findings
• Best diagnostic clue: Small nodule with same texture Diagnostic Checklist
and enhancement of normal spleen • Accessory spleen is common, can be mistaken for
• Best imaging tool: CT followed by nuclear tumor
scintigraphy • Hypertrophic accessory spleen may occur after
splenectomy

• Implanted splenic tissue continues to function;

Natural History & Prognosis
enlarged "splenules"
• Complications: Spontaneous rupture, infarction,
Polysplenia torsion
• Congenital disorder with multiple small spleens, • Prognosis: Good
bilateral "left-sidedness" of abdominal viscera,
cardiovascular anomalies
Treatment
• Surgical resection: Complications, recurrence of
Metastases lymphoma or hypersplenism
• E.g., omental, peritoneal metastases
Visceral mass I DIAGNOSTIC CHECKLIST
• E.g., intramural benign gastric tumors, renal, adrenal
or primary retroperitoneal mass, mass in tail of Consider
pancreas • Accessory spleen is common, can be mistaken for
tumor

I PATHOLOGY Image Interpretation Pearls

• Hypertrophic accessory spleen may occur after
General Features splenectomy
• Etiology
o Congenital
o Pathogenesis I SELECTED REFERENCES
6
• Failure of embryonic splenic buds to unite within 1. Harris GN et al: Accessory spleen causing a mass in the tail 11
dorsal mesogastrium of the pancreas: MR imaging findings. AJR Am J
• Extreme lobulation of spleen with pinching off of Roentgenol. 163(5):1120-1, 1994
splenic tissue 2. Freeman JL et al: CT of congenital and acquired
• Epidemiology: Incidence: 10-30% patients at autopsy abnormalities of the spleen. Radiographies. 13(3):597-610,
• Associated abnormalities 1993
o Hypertrophied accessory spleen: Enlarged due to 3. Ambriz P et al: Accessory spleen compromising response to
splenectomy for idiopathic thrombocytopenic purpura.
disease and/or splenectomy
Radiology. 155(3):793-6, 1985
o Splenic neoplasms (e.g., recurrent lymphoma)
o Hematologic diseases (e.g., idiopathic
thrombocytopenic purpura, hereditary
spherocytosis, acquired autoimmune hemolytic
[IMAGE GALLERY
anemia): Recurs after splenectomy
Gross Pathologic & Surgical Features
• Structurally normal splenic tissues r-~l"i\ ,,
~.
t.

. ..:,
' .••.•

.
~

~
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Asymptomatic (most
cases)
•a ~/
• Diagnosis
o Incidentally at surgery, autopsy or imaging
o May be mistaken for tumor (Left) Axial CECT shows one (arrow) of several accessory spleens,
o Hypertrophic accessory spleen may be site of hypertrophied following splenectomy. (Right) Axial CECT shows 2 of
residual lymphoma or hypersplenism several accessory spleens (arrows), hypertrophied following
splenectomy.
 SPLENIC INFECTION AND ABSCESS

Pyogenic splenic abscess on CECT. Note low Pyogenic splenic abscess on axial CECT. Note thin
attenuation abscess bulging splenic parenchyma septations within abscess (arrows),
(arrow).

ITERMINOlOGY CT Findings
Definitions • NECT
o Low attenuation ill-defined lesion within splenic
• Focal collection of liquified pus within splenic
parenchyma
parenchyma
o May rarely contain gas bubbles or air-fluid levels
• CECT: Low attenuation, nonenhancing complex fluid
collection; may extend to subcapsular location, rarely
[IMAGING FINDINGS causes splenic rupture with generalized peritonitis
General Features MR Findings
• Best diagnostic clue: Rounded low attenuation
6 complex fluid collection with mass effect
• Tl WI: Low or intermediate signal lesion
• T2WI: High signal lesion
• Location: Variable; may be located anywhere within • TI C+: Low signal lesion with peripheral enhancement
12 splenic parenchyma
• Size: Variable; typically 3-5 cm for pyogenic abscesses; Ultrasonographic Findings
microabscesses (often fungal) < 1.5 cm • Real Time
• Morphology o Typical pyogenic abscess
o Rounded or with irregular borders • Hypoechoic with internal septations, low-level
o May have multiple locules similar to hepatic "cluster echoes representing pus or debris
sign" of pyogenic abscess • May have little distal acoustic enhancement
o Mass effect on splenic capsule o Atypical pyogenic abscess
o Internal septations common • Reverberation artifacts from gas
• Echogenic
Radiographic Findings o Microabscesses
• Radiography • Target or "bull's eye" appearance similar to hepatic
o Rarely gas bubbles within abscess microabscesses
o Associated with left lower lobe atelectasis and left • Color Doppler
pleural effusion on chest x-ray o Typical pyogenic abscess shows no internal flow

DDx: Splenic lesions Mimicking Infection

,.<f,;';"

..
, ' ,

. rIP"
. . f1' ',,-

~.:,e_/
~'

-'('-
Splenic Infarct Splenic Tumor Splenic Trauma Sarcoidosis
 SPLENIC INFECTION AND ABSCESS

Key Facts
Terminology • Splenic trauma
• Focal collection of liquified pus within splenic • Infiltrating disorders
parenchyma Pathology
Imaging Findings • General path comments: Liquified pus, splenomegaly
• Best diagnostic clue: Rounded low attenuation • Genetics: Hemoglobinopathies (sickle cell) predispose
complex fluid collection with mass effect Clinical Issues
• CECT: Low attenuation, non enhancing complex fluid • Percutaneous drainage for unilocular unruptured
collection; may extend to subcapsular location, rarely abscesses
causes splenic rupture with generalized peritonitis • Splenectomy for multiple pyogenic abscesses and/or
• Protocol advice: 150 ml of l.V. contrast injected at 2.5 abscess rupture
ml!sec; 5 mm slice thickness with 5 mm
reconstruction interval Diagnostic Checklist
• Infarct; necrotic or cystic mets; lymphoma
Top Differential Diagnoses
• Splenic infarct
• Splenic tumor

o Hypoechoic nodular avascular microabscesses

!PATHOlOGY
Nuclear Medicine Findings
• PET: Increased isotope uptake from hypermetabolic
General Features
focus • General path comments: Liquified pus, splenomegaly
• WBC Scan • Genetics: Hemoglobinopathies (sickle cell) predispose
o Increased isotope uptake • Etiology
o Generalized septicemia
Imaging Recommendations o Septic emboli
• Best imaging tool: CECT, US • Endocarditis
• Protocol advice: 150 ml of l.V. contrast injected at 2.5 o Immunosuppression
ml!sec; 5 mm slice thickness with 5 mm • Fungal microabscesses
reconstruction interval o Secondary infection of traumatic splenic hematoma
or infarct
o Hematologic disorders 6
I DIFFERENTIAL DIAGNOSIS • Epidemiology
o Rare: 0.2% of reported autopsies 13
Splenic infarct o 25% are immunocompromised patients
• Wedge-shaped, but may occasionally be rounded in • Associated abnormalities
configuration o Post-operative state
• Low attenuation o Endocarditis
• Peripheral location o Immunocompromised state
• Nonenhancement with contrast o Pancreatitis, colon cancer
Splenic tumor Gross Pathologic & Surgical Features
• Single or multiple lesions • Necrotic areas of liquified pus
• Solid: Lymphoma, melanoma
• Cystic: Ovarian carcinoma, germ cell tumors or Microscopic Features
sarcoma • Liquefactive necrosis
• . Benign: Lymphangioma, hemangioma • Pus with leukocyte debris
• Rounded with variable enhancement • Gram stain for pyogenic abscess
o Fungal stain for mycotic organism
Splenic trauma o TB stains for tuberculous abscesses
• History of blunt injury o Pyogenic: 57% aerobic
• Associated with perisplenic hematoma and • Staphylococcus
hemoperitoneum • Strep E. coli
• May have active arterial extravasation • Salmonella
• Arterial clot adjacent to spleen in small lacerations o Fungal
Infiltrating disorders • Candida most common
• Aspergillus and cryptococcus
• Sarcoid • Tuberculosis (TB) and mycobacterium avium
o Multiple low attenuation lesions
intracellulari (MAl) in AIDS patient
• Gaucher disease
o Multiple low attenuation lesions
 SPLENIC INFECTION AND ABSCESS
abscess. A]R Am] Roentgenol. 179(3):629-32, 2002
Staging, Grading or Classification Criteria 5. Ng KKet al: Splenic abscess: diagnosis and management.
• Pyogenic Hepatogastroenterology. 49(44):567-71, 2002
o Unilocular (65%) 6. Loualidi A et al: Splenic abscess caused by
o Multilocular or multiple (20%) Peptostreptococcus species, diagnosed with the aid of
• Fungal abdominal computerized tomography and treated with
o Microabscesses < 1.5 cm (25%) percutaneous drainage and antibiotics: a case report. Neth]
Med. 59(6):280-5, 2001
• Parasitic
7. Green BT:Splenic abscess: report of six cases and review of
o Echinococcus granulosa the literature. Am Surg. 67(1):80-5, 2001
8. Smyrniotis V et al: Splenic abscess. An old disease with new
interest. Dig Surg. 17(4):354-7,2000
I CLINICAL ISSUES 9. Poggi SH et al: Puerperal splenic abscess. Obstet Gynecol.
96(5 Pt 2):842, 2000
Presentation 10. Mehanna 0 et al: Cat scratch disease presenting as splenic
• Most common signs/symptoms abscess. Aust N Z] Surg. 70(8):622-4, 2000
o Fever 11. Drevelengas A: The spleen in infectious disorders. ]BR-BTR.
83(4):208-10,2000
o Chills
12. Murray AW et al: A case of multiple splenic abscesses
o LUQpain managed non-operatively.] R ColI Surg Edinb.
o Splenomegaly 45(3):189-91, 2000
• Clinical profile 13. Frumiento C et al: Complications of splenic injuries:
o Lab data expansion of the nonoperative theorem. ] Pediatr Surg.
• Leukocytosis 35(5):788-91,2000
• Positive blood cultures 14. Nakao A et al: Portal venous gas associated with splenic
abscess secondary to colon cancer. Anticancer Res.
Demographics 19(6C):5641-4, 1999
15. Bernabeu-Wittel M et al: Etiology, clinical features and
• Age: Adult patients with predisposing factors
outcome of splenic microabscesses in HIV-infected patients
• Gender: M = F with prolonged fever. Eur] Clin Microbiollnfect Dis.
• Ethnicity: No known predilection 18(5):324-9, 1999
16. Duggal RKet al: Splenic abscess as a complication of acute
Natural History & Prognosis pancreatitis.] Assoc Physicians India. 47(3):338-9, 1999
• Variable 17. Alterman P et al: Splenic abscess in geriatric care.] Am
• Excellent prognosis for pyogenic abscesses in Geriatr Soc. 46(11):1481-3, 1998
immunocompetent patient 18. de Bree E et al: Splenic abscess: a diagnostic and
• Guarded prognosis in immunocompromised patients therapeutic challenge. Acta Chir Belg. 98(5):199-202, 1998
with fungal microabscesses 19. AI-Salem AH et al: Splenic abscess and sickle cell disease.
6 Treatment 20.
Am] Hematol. 58(2):100-4,1998
Wang Y et al: CT findings in splenic tuberculosis. ] BeIge
14 • Options, risks, complications Radiol. 81(2):90-1, 1998
21. Kumar N et al: Splenic abscess caused by Clostridium
o Percutaneous drainage for unilocular unruptured
difficile. Eur] Clin Microbiol Infect Dis. 16(12):938-9, 1997
abscesses 22. Vleminckx WG et al: Splenic abscess with Clostridium
• Reported success rate of 67-100% novyi bacteraemia and sepsis. Eur] Gastroenterol Hepatol.
o Splenectomy for multiple pyogenic abscesses and/or 9(3):303-5, 1997
abscess rupture 23. Rypens F et al: Splenic parenchymal complications of
• Mortality post-splenectomy 6% pancreatitis: CT findings and natural history. ] Comput
Assist Tomogr. 21(1):89-93, 1997
24. Yelon]A et al: Splenic abscess associated with
osteomyelitis. Eur] Surg. 162(11):913-4, 1996
I DIAGNOSTIC CHECKLIST 25. Liang]T et al: Splenic abscess: a diagnostic pitfall in the
ED. Am] Emerg Med. 13(3):337-43, 1995
Consider
• Infarct; necrotic or cystic mets; lymphoma
Image Interpretation Pearls
• Single or multiple low attenuation lesions in febrile
patient
• Morphology variable

I SELECTED REFERENCES
1. Tasar M et al: Computed tomography-guided percutaneous
drainage of splenic abscesses. Clin Imaging. 28(1):44-8,
2004
2. Chiang IS et al: Splenic abscesses: review of 29 cases.
Kaohsiung] Med Sci. 19(10):510-5,2003
3. Kaushik R et al: Splenic abscess. Trop Doct. 32(4):246-7,
2002
4. Thanos L et al: Percutaneous CT-guided drainage of splenic
 SPLENIC INFECTION AND ABSCESS
[IMAGE GAllERY
Typical
(Left) Axial CECT of fungal
microabscesses. Note
numerous hypodense
lesions; cultures grew
Candida. (Right) Axial CECT
demonstrates splenic
microabscesses. Note small
< 7 cm lesions diffusely
throughout the spleen.

Typical
(Left) Cas-forming splenic
pyogenic abscess on
transverse sonogram. Note
linear high amplitude echoes
representing gas (arrows).
(Right) Cas-forming
pyogenic splenic abscess on
transverse sonogram. Note
ring down artifacts from gas
bubbles (arrow).

6
15

Typical
(Left) Pyogenic splenic
abscess on transverse
sonogram. Note hypoechoic
abscess (arrow) with little
distal acoustic enhancement.
(Right) Multiple pyogenic
splenic abscesses on
transverse sonogram. Note
multiple hypoechoic round
lesions (arrows).
 SPLENIC INFARCTION

Axial CECT of embolic splenic infarction. Note apical Axial CECT of embolic splenic infarct. Note low
thrombus in left ventricle (arrow). attenuation defect with linear margins (arrow).

ITERMINOlOGY CT Findings
• NECT: Poorly visualized without contrast
Definitions
• CECT
• Global or segmental parenchymal splenic ischemia & o Segmental: Wedge-shaped or rounded low
necrosis caused by vascular occlusion attenuation area on CECT
o Global: Complete non enhancement of spleen with
or without "cortical rim sign" on CECT
IIMAGING FINDINGS
MR Findings
General Features
• TlWI
• Best diagnostic clue: Peripheral wedge-shaped o High signal areas of hemorrhagic infarction if recent
6 non enhancing areas on CECT within splenic o Low signal if chronic
parenchyma in patient with LUQ pain • T2WI: High signal within area of infarct
16 • Location • Tl C+: Wedge-shaped area of low signal
o Variable
o Entire spleen may be infarcted or more commonly Ultrasonographic Findings
segmental areas • Real Time: Hypoechoic or anechoic wedge-shaped or
• Size rounded parenchymal defect
o Variable; global or segmental • Color Doppler: Absent flow in areas of infarction with
o Spleen mayor may not demonstrate splenomegaly color Doppler
• Morphology
o Most commonly wedge-shaped when segmental
Angiographic Findings
o Straight margins indicating vascular lesion • Conventional: Main splenic artery occlusion or
o May be rounded (atypical) segmental emboli

Radiographic Findings Imaging Recommendations

• Radiography: May be associated with left pleural • Best imaging tool: CECT
effusion on chest x-ray • Protocol advice: 150 ml of LV. contrast at 2.5 ml!sec; 5
mm collimation with 5 mm reconstruction interval

DDx: Splenic lesions Mimicking Infarction

):
~_/
Splenic Abscess Splenic Lymphoma Splenic Laceration Splenic Cyst
 SPLENIC INFARCTION

Key Facts
Terminology • Splenic tumor
• Splenic laceration
• Global or segmental parenchymal splenic ischemia &
necrosis caused by vascular occlusion Pathology
Imaging Findings • Genetics: Predisposition among some hematologic
causes such as sickle cell disease, sickle cell trait
• Best diagnostic clue: Peripheral wedge-shaped
non enhancing areas on CECT within splenic Clinical Issues
parenchyma in patient with LUQ pain • LUQ pain and chills
• Segmental: Wedge-shaped or rounded low • Asymptomatic: No treatment
attenuation area on CECT • Symptomatic: Splenectomy for increasing pain or
• Global: Complete nonenhancement of spleen with or splenic rupture
without "cortical rim sign" on CECT
• Protocol advice: 150 ml of LV. contrast at 2.5 ml!sec; Diagnostic Checklist
5 mm collimation with 5 mm reconstruction interval • Consider splenic abscess or tumor
Top Differential Diagnoses
• Splenic abscess

• Secondary cysts most often due to infection, infarction

I DIFFERENTIAL DIAGNOSIS or trauma
Splenic abscess • Primary cysts may be due to parasitic infection
• Complex fluid collection (echinococcal) or epidermoid cysts
• Internal septations and debris • Calcification of cyst wall in 14% of primary cysts, 50%
• Low level echoes on sonography of secondary cysts
• Multiple gas bubbles • Low-level echoes and thin septations on US in both
• Rounded, mass effect primary and secondary cysts
• Multiple small lesions (microabscesses) in fungal
infections in immunocompromised patients
I PATHOLOGY
Splenic tumor
• Primary malignant General Features
o Angiosarcoma
o Hypervascular lesions with prominent areas of
• General path comments: Liquefactive necrosis
• Genetics: Predisposition among some hematologic
6
necrosis causes such as sickle cell disease, sickle cell trait 17
• Primary benign • Etiology
o Hemangioma o Embolic
o May have contrast-enhancement pattern similar to • Atrial fibrillation
hepatic hemangiomas • Aortic atherosclerotic disease
• Secondary • Aortic valve emboli from subacute bacterial
o Lymphoma endocarditis
o Melanoma o Hematologic
o Ovarian carcinoma • Sickle hemoglobinopathies
o Often complex cystic masses • Myelofibrosis
o May have perisplenic cystic implants • Any cause of hypersplenism
• Hypercoagulable states
Splenic laceration • Leukemia and lymphoma
• History of trauma o Splenic vascular disease
• Associated hemoperitoneum • Aneurysm
• May have high attenuation active arterial • Aortic dissection
extravasation on CECT • Splenic venous thrombosis
• High attenuation perisplenic hematoma o Anatomic causes
• Intra-parenchymal low attenuation hematoma on • Splenic torsion
CECT • Torsion secondary to wandering spleen
o Miscellaneous
Splenic cyst
• Pancreatic disease, pseudocysts
• Non-neoplastic cysts divided into two categories • Collagen vascular disease
o True epithelial cysts ("primary") • Gastric tumors invading gastro-splenic ligament
o "Pseudocysts" or "secondary cysts" lacking an • Epidemiology
epithelial lining o Embolic
• Epidermoid cysts are 10-25% of all splenic cysts • Elderly cardiac patients with atrial fibrillation
o Hematologic
 SPLENIC INFARCTION
• Younger patients with sickle hemoglobinopathy 3. Romero JR et al: Wandering spleen: a rare cause of
or myeloproliferative disease abdominal pain. Pediatr Emerg Care. 19(6):412-4, 2003
4. Gorg C et al: Chronic recurring infarction of the spleen:
Gross Pathologic & Surgical Features sonographic patterns and complications. Ultraschall Med.
• Acute infarction 24(4):245-9, 2003
o Hemorrhagic or bland necrosis 5. Sodhi KSet al: Torsion of a wandering spleen: acute
• Chronic infarction abdominal presentation. J Emerg Med. 25(2):133-7, 2003
o Fibrous scar 6. Hatipoglu AR et al: A rare cause of acute abdomen: splenic
infarction. Hepatogastroenterology. 48(41):1333-6, 2001
o Rarely calcifies 7. Toth PP et al: Spontaneous splenic infarction secondary to
Microscopic Features diabetes-induced microvascular disease. Arch Fam Med.
9(2):195-7, 2000
• Coagulative necrosis 8. Barzilai M et al: Noninfectious gas accumulation in an
• Hemorrhage infarcted spleen. Dig Surg. 17(4):402-4,2000
9. Andrews MW: Ultrasound of the spleen. World J Surg.
Staging, Grading or Classification Criteria 24(2):183-7, 2000
• Segmental 10. Nores M et al: The clinical spectrum of splenic infarction.
o Wedge-shaped or round segmental lesion Am Surg. 64(2):182-8, 1998
o Straight margins typical 11. Argiris A: Splenic and renal infarctions complicating atrial
• Global fibrillation. Mt Sinai J Med. 64(4-5):342-9, 1997
o Entire spleen is avascular 12. Rypens F et al: Splenic parenchymal complications of
pancreatitis: CT findings and natural history. J Comput
o May demonstrate "cortical rim" sign Assist Tomogr. 21(1):89-93, 1997
13. Beeson MS: Splenic infarct presenting as acute abdominal
pain in an older patient. J Emerg Med. 14(3):319-22, 1996
I CLINICAL ISSUES 14. Frippiat F et al: Splenic infarction: report of three cases of
atherosclerotic embolization originating in the aorta and
Presentation retrospective study of 64 cases. Acta Clin Belg.
• Most common signs/symptoms 51(6):395-402, 1996
o LUQ pain and chills 15. Collie DA et al: Case report: computed tomography
features of complete splenic infarction, cavitation and
o 69% of patients have fever in embolic infarction
spontaneous decompression complicating pancreatitis. BrJ
• Clinical profile Radiol. 68(810):662-4, 1995
o Anemia in 53% of patients 16. Chin JK et al: Liver/spleen scintigraphy for diagnosis of
o Leukocytosis in 41 % of patients splenic infarction in cirrhotic patients. Postgrad Med J.
o Elevated platelet count in 7% of patients 69(815):715-7, 1993
17. Valentine RJ et al: Splenic infarction after splenorenal
Demographics arterial bypass. J Vasc Surg. 17(3):602-6, 1993
6 • Age: 2-87 yrs, mean age 54 18. Orringer EP et al: Case report: splenic infarction and acute
splenic sequestration in adults with hemoglobin SC
• Gender: Occurs with equal frequency in males and
18 females disease. AmJ Med Sci. 302(6):374-9,1991
19. Ting W et al: Splenic septic emboli in endocarditis.
Natural History & Prognosis Circulation. 82(5 Suppl):IV105-9, 1990
20. Goerg C et al: Splenic infarction: sonographic patterns,
• Highly variable
diagnosis, follow-up, and complications. Radiology. 174(3
o May require no treatment Pt 1):803-7, 1990
o Surgery for increased pain or rupture 21. HaftJI et al: Computed tomography of the abdomen in the
diagnosis of splenic emboli. Arch Intern Med.
Treatment 148(1):193-7, 1988
• Options, risks, complications 22. O'Keefe JH Jr et al: Thromboembolic splenic infarction.
o Asymptomatic: No treatment Mayo Clin Proc. 61(12):967-72, 1986
o Symptomatic: Splenectomy for increasing pain or 23. Jaroch MT et al: The natural history of splenic infarction.
splenic rupture Surgery. 100(4):743-50, 1986
24. Shirkhoda A et al: Computed tomography and
ultrasonography in splenic infarction. J Can Assoc Radiol.
36(1):29-33, 1985
I DIAGNOSTIC CHECKLIST 25. Balthazar EJ et al: CT of splenic and perisplenic
abnormalities in septic patients. AJRAm J Roentgenol.
Consider
144(1):53-6, 1985
• Consider splenic abscess or tumor
Image Interpretation Pearls
• Wedge-shaped peripheral area of nonenhancement

I SELECTED REFERENCES
1. Wilkinson NW et al: Splenic infarction following
laparoscopic Nissen fundoplication: management
strategies. JSLS. 7(4):359-65, 2003
2. Sodhi KSet al: Torsion of a wandering spleen: acute
abdominal presentation. J Emerg Med. 25(2):133-7, 2003
 SPLENIC INFARCTION
I IMAGE GALLERY
Typical
(Left) Global splenic
infarction on axial CECT.
Note complete lack of
enhancement of splenic
parenchyma (arrow). (Right)
Axial CECT of global splenic
infarction demonstrates
peripheral "cortical rim
sign".

Typical
(Left) Axial CECT of acute
splenic infarction
demonstrates peripheral
wedge-shaped infarct
(arrow). (Right) Axial CECT
of chronic splenic infarction
demonstrates calcification
(arrow).

6
19

Typical
(Left) Axial CECT of splenic
infarcts demonstrates
multiple peripheral
wedge-shaped emboli
(arrows). (Right) Axial CECT
demonstrates lack of
enhancement of upper pole
of spleen (arrow).
 SPLENIC TRAUMA

Axial CECT of splenic fracture with active bleeding. Axial CECT of splenic fracture shows jet of active
Note area of high attenuation arterial extravasation hemorrhage in left paracolic gutter (arrow).
(arrows).

o Splenic fracture: Deep laceration extending from

ITERMINOlOGY outer capsule through splenic hilum
Abbreviations and Synonyms o Active arterial extravasation: High attenuation focus
• Splenic laceration, splenic fracture, subcapsular isodense with aorta; surrounded by lower
hematoma of spleen attenuation clot or hematoma

Definitions Ultrasonographic Findings

• Parenchymal injury to spleen with or without capsular • Real Time: Hypo- or isoechoic hematoma or laceration
disruption Angiographic Findings
• Avascular parenchymal laceration; flattened lateral
6 I IMAGING FINDINGS contour 2° subcapsular hematoma; rounded contrast
collections (pseudoaneurysms); amorphous
General Features parenchymal extravasation
20
• Best diagnostic clue: Low attenuation splenic Imaging Recommendations
laceration with high density active bleeding • Best imaging tool: CECT
• Morphology: Lacerations: Linear or jagged edges; • Protocol advice: 150 ml LV. contrast at 2.5 ml/sec with
subcapsular hematoma: Flattened contour of splenic 5 mm collimation
parenchyma; fracture: Laceration extending from
outer cortex to hilum
CT Findings I DIFFERENTIAL DIAGNOSIS
• NECT: High attenuation (> 30 HU) hemoperitoneum Splenic abscess
or perisplenic clot (> 45 HU) • Rounded, irregular, low attenuation lesion; clinical
• CECT signs of infection
o Subcapsular hematoma: Compresses lateral margin
of parenchyma Splenic infarct
o Parenchymal laceration: Jagged linear area of • Wedge-shaped area of low attenuation; associated with
non enhancement due to hematoma splenomegaly; systemic embolization

DDx: Splenic Lesions Mimicking Trauma

Infarct Cyst Lymphoma

 SPLENIC TRAUMA

Key Facts
Terminology Pathology
• Parenchymal injury to spleen with or without • Associated abnormalities: Injuries to left thorax, tail
capsular disruption of pancreas, left liver lobe and/or mesentery
Imaging Findings Diagnostic Checklist
• Best imaging tool: CECT • Congenital cleft if no hemoperitoneum
• Protocol advice: 150 ml IV contrast @ 2.5 ml/sec with • Innocuous injury may lead to life-threatening
5 mm collimation delayed hemorrhage, especially with anticoagulation

Splenic cyst Treatment

• Rounded hypoechoic lesion on USi definable cyst walli • Non-operative management for minor injuriesi
no internal enhancement on CECT angiographic embolization if active arterial
extravasation on CTi splenectomy or splenorrhaphy
lymphoma when surgery required
• Single or multiple hypodense lesionsi splenomegaly

I DIAGNOSTIC CHECKLIST
I PATHOLOGY
Consider
General Features
• Congenital cleft if no hemoperitoneum
• General path comments: Laceration, fractures or
subcapsular hematoma Image Interpretation Pearls
• Etiology: Blunt trauma with blow to LUQ • Innocuous injury may lead to life-threatening delayed
• Epidemiology: Most common abdominal organ injury hemorrhage, especially with anticoagulation
requiring surgery
• Associated abnormalities: Injuries to left thorax, tail of
pancreas, left liver lobe and/or mesentery I SELECTED REFERENCES
Gross Pathologic & Surgical Features 1. Jeffrey RBJr et al: Detection of active intraabdominal
arterial hemorrhage: value of dynamic contrast-enhanced
• Varies according to extent of injury
CT. AJR AmJ Roentgenol. 156(4):725-9, 1991 6
Microscopic Features 2. Jeffrey RBJr: CT diagnosis of blunt hepatic and splenic
injuries: a look to the future. Radiology. 171(1):17-8, 1989 21
• Necrotic injured tissue with surrounding hematoma 3. Federle MP et al: Splenic trauma: evaluation with CT.
Staging, Grading or Classification Criteria Radiology. 162(1 Pt 1):69-71, 1987
• Grading may be misleadingi minor injuries may go on
to devastating delayed bleed
o Grade 1: Subcapsular hematoma or laceration < 1 I IMAGE GALLERY
em
o Grade 2: Subcapsular hematoma or laceration 1-3
em
o Grade 3: Capsular disruptioni hematoma> 3 cmi
parenchymal hematoma> 3 em
o Grade 4A: Active parenchymal or subcapsular
bleeding, pseudoaneurysm or arteriovenous fistulai
shattered spleen
o Grade 4B: Active intraperitoneal bleed

I CLINICAL ISSUES
Presentation (Left) Axial CECT of splenic laceration with active bleeding. Note low
• Most common signs/symptoms: Blunt abdominal attenuation area of parenchymal laceration (open arrow) with
adjacent active bleeding (arrow). (Right) Axial CECT of splenic
traumai LUQ paini hypotension
fracture with active bleeding. Note non-enhancing area of splenic
Natural History & Prognosis fracture (open arrow) and high-density active bleeding (arrow).
• Prone to develop delayed hemorrhagei excellent
prognosis with early diagnosis & intervention (surgery
or embolization)
 SPLENIC CYST
Axial CECT shows a water density cyst, plus calcified
granulomas (old histoplasmosis).
ITERMINOLOGY
Definitions
• Cystic masses of spleen
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Sharply defined spherical lesion
of water density
6 • Location: Usually in lower pole; subcapsular (65%)
• Size: Varies (congenital usually large, acquired small)
22 • Key concepts
o Cystic masses do not commonly occur in spleen
o Classification of splenic cysts based on etiology
• Congenital (primary or true) cyst: Epidermoid
• Acquired (false or pseudo) cyst: Post-traumatic
o Congenital (primary or true) cyst: Epidermoid
• Inner cellular lining (endothelial lining) present
• Account for 10-25% of all splenic cysts
o Acquired (secondary or false or pseudo) cyst:
Post-traumatic (end stage of hematoma)
• Inner cellular lining absent, but has a fibrous wall
• Accounts for 80% of splenic cysts
• Wall calcification seen in 38-50% of cases
• Cystic nature: Due to liquefactive necrosis
DDx: low Density Splenic Mass
Hydatid Cyst Metastatic Melanoma
Axial CECT shows water density splenic cyst.
Radiographic Findings
• Radiography
o Large acquired splenic cysts
• Curvilinear or plaque-like wall calcification
CT Findings
• Congenital (primary or true) cyst: Epidermoid
o Solitary, well-defined, spherical, unilocular, cystic
lesion (water HU)
o Thin wall + sharp interface to normal splenic tissue
o Hemorrhagic, infected, 1 protein: 1 Attenuation
o No rim or intracystic enhancement
o May rarely have calcified wall
• Acquired (false or pseudo) cyst: Post-traumatic
o False or pseudocyst (end stage of splenic hematoma)
• Usually small, solitary, sharply defined, water HU
• ± Wall calcification (may resemble eggshell)
o Hematoma (evolving)
• I HU, clear cut margins, nonspecific cystic lesion
• CECT: No enhancement of contents
MR Findings
• Congenital (primary or true) cyst: Epidermoid
o T1WI: Hypointense
• Variable intensity: Infected or hemorrhagic
o T2WI: Hyperintense
• Acquired (false or pseudo) cyst: Post-traumatic
o T1WI: Hypointense; variable intensity (blood)
Lymphoma Pseudocyst
 SPLENIC CYST
Key Facts
Imaging Findings
• Best diagnostic clue: Sharply defined spherical lesion
of water density
• Location: Usually in lower pole; subcapsular (65%)
• Congenital (primary or true) cyst: Epidermoid
• Acquired (false or pseudo) cyst: Post-traumatic
• Anechoic, smooth borders, non-detectable walls ±
trabeculation (36%) congenital
• Small, anechoic or mixed with internal echoes,
echogenic wall (calcification) acquired
Top Differential Diagnoses
• Inflammatory or infection
• Vascular
• Neoplastic
• Intrasplenic pseudocyst
o T2WI: Hyperintense
o Calcification or hemosiderin deposited in wall
• Hypointense (both Tl & T2WI)
o Hematoma: Varied intensity based on age &
evolution of blood products
• After 3 weeks appears as a cystic mass: T1WI
hypointense; T2WI hyperintense
Ultrasonographic Findings
• Real Time
o Congenital (primary or true) cyst: Epidermoid
• Anechoic, smooth borders, non-detectable walls ±
trabeculation (36%) congenital
• No septations or nodules
• Complicated: Septations, internal echoes (debris),
thickened wall ± calcification
o Acquired (false or pseudo) cyst: Post-traumatic
• Small, anechoic or mixed with internal echoes,
echogenic wall (calcification) acquired
• ± Trabeculation of cyst wall (15%)
Angiographic Findings
• Conventional: Avascular mass; stretched or normal
appearing capsular & intra splenic vessels
Imaging Recommendations
• Helical NE + CECT; US; MR
I DIFFERENTIAL DIAGNOSIS
Inflammatory or infection
• Pyogenic abscess
o Solitary, multiple, well-defined, irregular borders
o 20-40 HU (due to proteinaceous material)
o CECT: ± Rim & no central enhancement
o T1WI: Hypointense; variable intensity (protein or
hemorrhagic content)
o T2WI: Hyperintense
• Fungal abscess
o E.g., Candida, Aspergillus, Cryptococcal
o Usually microabscesses: Multiple, small,
well-defined, low attenuation, no rim-enhancement
o Tl WI hypointense; T2WI hyperintense
Pathology
• Congenital (true) epidermoid: Genetic defect of
mesothelial migration
• Post-traumatic: End stage of splenic hematoma
• Pathogenesis: Liquefactive necrosis, cystic change
• Epidemiology: 7.6 per 10,000 people
• Congenital (true) cyst: Endothelial lining present
• Post-traumatic (false) cyst: Endothelial lining absent
Diagnostic Checklist
• Rule out infective, vascular & neoplastic cystic lesions
• Congenital: Large, well-defined, water density with
thin wall & no rim or intracystic enhancement
• Acquired (post-traumatic): Usually small, sharply
defined, near water HU with thick wall ± calcification
• Differentiation by imaging alone is often impossible
• Granulomatous abscesses
o E.g., Mycobacterium & atypical TB; cat-scratch
o Multiple, small, well-defined, low attenuation, no
rim-enhancement
o T1WI hypointense; T2WI hyperintense
• Parasitic: Echinococcal or hydatid cyst
o Pathology: Inner germinal layer + outer pericyst
o Large unilocular or multilocular; well-defined near
water density cyst
o ± Area of 1 density within cyst (hydatid sand)
o Daughter cysts: j Density than mother cyst
o Curvilinear ring-like calcification (common)
o CECT: Enhancement of cyst wall + septations
o TlWI
• Matrix: Hypointense, rarely hyperintense (j water)
6
• Rim: Hypointense (fibrosis); daughter cyst j signal 23
o T2WI
• Mother & daughter cysts: Hyperintense
• Floating membrane: Low-intermediate signal
o Ultrasound findings
• Well-defined anechoic cyst ± hydatid sand
(internal echoes)
• Multiseptate cyst + daughter cysts
• "Water lily" sign: Cyst + floating membrane +
detached endocyst
o Look for "cysts" in liver & peritoneal cavity
Vascular
• Infarction (arterial or venous)
o Acute phase: Well-defined areas of j attenuation
o Subacute & chronic phases: Near water HU
• Due to liquefactive necrosis
o CECT: No enhancement
o MR findings
• Acute & subacute phases: Vary with age &
evolution of blood products
• Chronic phase: Cystic intensity (due to necrosis)
• T1WI hypointense; T2WI hyperintense
• Peliosis
o Multiple, low HU, rounded lesions of varied size
o CECT: Early peripheral nodular & delayed
centripetal enhancement
o T1WI hypointense; T2WI hyperintense
 SPLENIC CYST
o T1 C+: Same as CECT
Neoplastic
• Benign: E.g., hemangioma & lymphangioma
o Hemangioma
• Have overlapping & variable CT features
preventing accurate diagnosis
• Usually not confused with simple cyst or
metastases
o Lymphangioma
• NECT: Solitary or multiple well-defined, I HU
• CECT: No enhancement
• T1WI hypo intense; T2WI hyperintense
• 1 Signal on Tl WI: Subacute hemorrhage or
proteinaceous fluid
• Malignant: E.g., Lymphoma & metastases
o Lymphoma
• NECT: Miliary multifocal or solitary, I attenuation
• CECT: Mild enhancement
• Necrosis within lesion: Near water density (rare)
• T1WI hypo intense; T2WI hyperintense
• 1 Signal on T2WI: Areas of necrosis
o Metastases
• Relatively common
• E.g., Malignant melanoma, pancreatic & ovarian
cancer may cause "cystic" splenic metastases
• NECT: Ill-/well-defined, unilocular I or water HU
• CECT: Peripheral & septal enhancement
• T1WI hypointense; T2WI hyperintense
• 1 Signal on Tl WI: Subacute hemorrhage, melanin
• 1 Signal on T2WI: Water, necrosis, old bleed
Intrasplenic pseudocyst
• Pancreatitis ~ intrasplenic pseudocyst or abscess
6 • Seen in 1.1-5% of patients with pancreatitis
• Pathogenesis
o Direct extension of pancreatic pseudocyst
24
o Secondary to digestive effects of enzymes on splenic
vessels or parenchyma along splenorenalligament
• Imaging
o Well-defined rounded cystic mass + enlarged spleen
o Density varies: Amount of debris + hemorrhage
o Associated inflammatory changes of pancreas seen
• Peripancreatic fluid collection (especially near tail)
I PATHOLOGY
General Features
• Etiology
o Congenital (true) epidermoid: Genetic defect of
mesothelial migration
o Post-traumatic: End stage of splenic hematoma
• Pathogenesis: Liquefactive necrosis, cystic change
• Epidemiology: 7.6 per 10,000 people
Gross Pathologic & Surgical Features
• Congenital (true) epidermoid cyst
o Usually large, glistening smooth walls
• Post-traumatic (false or pseudocyst)
o Smaller than true cysts, debris, wall calcification
Microscopic Features
• Congenital (true) cyst: Endothelial lining present
• Post-traumatic (false) cyst: Endothelial lining absent
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic; mild pain, palpable mass in LUQ
o Tenderness in LUQ; splenomegaly
Demographics
• Age: 2/3rd below 40 years old
• Gender: M:F = 2:3
Natural History & Prognosis
• Complications: Hemorrhage, rupture, infection
• Prognosis
o Good: Noncomplicated cases; after surgical removal
o Poor: Complicated cases
Treatment
• Small & asymptomatic: No treatment
• Small & symptomatic: Surgery
• Large (> 6 cm): Surgical removal (debatable)
I DIAGNOSTIC CHECKLIST
Consider
• Rule out infective, vascular & neoplastic cystic lesions
Image Interpretation Pearls
• Congenital: Large, well-defined, water density with
thin wall & no rim or intracystic enhancement
• Acquired (post-traumatic): Usually small, sharply
defined, near water HU with thick wall ± calcification
• Differentiation by imaging alone is often impossible
I SELECTED REFERENCES
1. Ito K et al: MR imaging of acquired abnormalities of the
spleen. AJRAm J Roentgenol. 168(3):697-702, 1997
2. Urrutia M et al: Cystic masses of the spleen:
radiologic-pathologic correlation. Radiographies.
16(1):107-29, 1996
3. Shirkhoda A et al: Imaging features of splenic epidermoid
cyst with pathologic correlation. Abdom Imaging.
20(5):449-51, 1995
4. Freeman JI. et al: CT of congenital and acquired
abnormalities of the spleen. Radiographies. 13(3):597-610,
1993
von Sinner WN et al: Hydatid disease of the spleen.
5.
Ultrasonography, CT and MR imaging. Acta Radiol.
33(5):459-61, 1992
6. Maves CK et al: Splenic and hepatic peliosis: MR findings.
AJRAmJ Roentgenol. 158(1):75-6, 1992
7. Dachman AH et al: Nonparasitic splenic cysts: a report of
52 cases with radiologic-pathologic correlation. AJRAm J
Roentgenol. 147(3):537-42, 1986
8. Chintapalli K et al: Differential diagnosis of
low-attenuation splenic lesions on computed tomography.
J Comput Tomogr. 9(4):311-9, 1985
9. Faer MJ et al: Traumatic splenic cyst: radiologic-pathologic
correlation from the Armed Forces Institute of Pathology.
Radiology. 134(2):371-6, 1980
 SPLENIC CYST

I IMAGE GALLERY
Typical
(Left) Axial cECT shows a
splenic cyst with a calcified
capsule. (Right) Axial NECT
shows a rim-calcified splenic
cyst.

(Left) Radiograph shows

"eggshell" calcification of a
splenic cyst. (Right) Axial
N ECT shows "eggshell"
\
- calcification of a splenic cyst.

•~,~ ~ / 6
,,~, WI' 25
~

Typical
(Left) Axial CECT in a
woman with
cardiomyopathy shows
acquired splenic cyst that
developed after prior splenic
infarctions. (Right) Axial
T2WI MR shows
hyperintense splenic cyst
JL (arrow).

~" ..

~r~~~
 SPLENIC TUMORS

Axial CECT shows splenomegaly due to non-Hodgkin Axial CECT shows a multi-cystic mass; lymphangioma.
lymphoma.

ITERMINOlOGY •Rare benign tumor of spleen; no sex predilection

•Autopsy incidence: 0.13%; solitary or multiple
Abbreviations and Synonyms •Incidental at autopsy & exploratory laparotomy
• Splenic mass or lesion •Contain anomalous mixtures of normal elements
of splenic tissue
Definitions o Lymphangioma
• Space occupying benign or malignant tumor of spleen • Rare benign splenic neoplasm (common in neck)
• Solitary/multiple; usually subcapsular in location
• Lymphangiomatosis: Diffuse lymphangiomas
I IMAGING FINDINGS • Most lymphangiomas occur in childhood
o Malignant tumors
6 General Features • Lymphoma, AIDS-related lymphoma
• Best diagnostic clue: Solid or cystic, solitary or • Angiosarcoma, metastases
26 multiple splenic masses • Rare malignant splenic tumors: Malignant fibrous
• Key concepts histiocytoma, leiomyosarcoma & fibrosarcoma
o Classification based on pathology & histology o Lymphoma
• Benign & malignant tumors • Most common malignant tumor of spleen
o Benign tumors • Hodgkin (HD) & non-Hodgkin lymphoma (NHL)
• Hemangioma, hamartoma, lymphangioma • Presence or absence of splenic involvement may
o Hemangioma determine type of therapy
• Most common primary benign neoplasm of spleen • Spleen: Considered as "nodal organ" in Hodgkin &
• Autopsy incidence: 0.03-14% "extranodal organ" in non-Hodgkin lymphoma
• Usually incidental on radiologic studies • Manifest: Focal lesions (> 1 cm) or diffuse (typical)
• Primarily affect adults; peak age 35-55 y • Staging laparotomy or splenectomy: Uncommonly
• Multiple as part of a generalized angiomatosis necessary to determine splenic involvement
(Klippel-Trenaunay-Weber syndrome) • Initial involvement: 23-39% (Hodgkin); 30-40%
• Hemangiomatosis: Diffuse splenic hemangiomas (non-Hodgkin)
o Hamartoma • Primary lymphoma; secondary (more common)

DDx: Splenic Mass

...
,
l
~

--
Q

I
"'V- ,
Cyst (+ Granulomas) Hematoma Artifact (Perfusion)
 SPLENIC TUMORS
Key Facts
Imaging Findings
• Best diagnostic clue: Solid or cystic, solitary or
multiple splenic masses
• "Cystic" splenic metastases: Melanoma;
adenocarcinoma of breast, ovary & endometrium
• Early peripheral & late central enhancement (not as
reliable as for liver hemangioma)
• Small: Isodense or hypodense (hamartoma)
• Solitary mass; multifocal or diffuse infiltration
(lymphoma)
• Solitary or multiple, nodular, irregular margins
(angiosarcoma)
• Multiple, solid (common) or cystic, t attenuation
(metastases)
• Metastases: Iso-/hypo-/hyperechoic, "target"/"halo"
• Primary splenic lymphoma: Typically represents
NHL (B-cell origin)
• Age: Primary (young age); secondary (older age)
o AIDS-related lymphoma
• Intra-abdominal involvement: Seen in 2/3rd cases
• 86% extranodal disease, half involve GI tract
• Histologic subtypes: Small noncleaved (Burkitt &
non-Burkitt type)
o Angiosarcoma
• Very rare malignant tumor of spleen; seen in
patients with previous exposure to Thorotrast
• Most common primary nonlymphoid vascular
malignant tumor of spleen
• Poor prognosis with early, widespread metastases
• Metastases: Liver (70%); lung, pleura, nodes, bone,
brain (30%)
• Usually affect older individuals (mean age at
diagnosis is 50s; M = F)
• Most patients die within 1 year of diagnosis
o Metastases
• Relatively uncommon (autopsy incidence, 7.1%
with malignancy)
• May be multiple (60%), solitary (31.5%), nodular
& diffuse (8.5%)
• Common route: Hematogenous spread (splenic
arterial blood flow)
• Retrograde « common): Via splenic vein (portal
HTN) & lymphatics
• Common primary sites & metastases to spleen:
Breast (21%), lung (18%), ovary (8%), stomach
(7%), melanoma (6%), prostate (6%)
• Frequency of splenic metastases by primary
tumor: Melanoma (34%), breast (12%), lung (9%)
• "Cystic" splenic metastases: Melanoma;
adenocarcinoma of breast, ovary & endometrium
o Peritoneal implants to surface of spleen: Carcinoma
of ovary, GIT, pancreas
o Direct invasion of spleen: Uncommon
• E.g., gastric, colonic, pancreatic tail, left renal
cancer, retroperitoneal sarcoma
CT Findings
• Benign tumors
Top Differential Diagnoses
• Splenic cyst
• Splenic hematoma
• Splenic infection
• Splenic sarcoidosis
Clinical Issues
• Most common signs/symptoms: LUQ pain, palpable
mass, splenomegaly, fever, weight loss
• Complications: Hemorrhage, rupture
• Prognosis: Good (benign tumors); poor (malignant)
Diagnostic Checklist
• Must rely on clinical setting & biopsy, if necessary
• Imaging findings alone are unreliable in
distinguishing solid from pseudocystic splenic masses
o Hemangioma
• Homogeneous, hypodense, solid or cystic masses
• Central punctate or peripheral curvilinear Ca++
• Early peripheral & late central enhancement (not
as reliable as for liver hemangioma)
• Heterogeneous: Cystic + avascular components
o Hamartoma
• Small: Isodense or hypodense (hamartoma)
• Large: Area of t HU (scar or necrosis); calcification
• Enhancement: Variable; uniform on delayed scans
o Lymphangioma
• Thin-walled low density lesions; sharp margins
• Wall enhances; usually subcapsular in location
• Malignant tumors
o Lymphoma
6
• Homogeneously enlarged spleen, no discrete mass 27
• Solitary mass; multi focal or diffuse infiltration
(lymphoma)
• Focal lesions: AIDS-related lymphoma> common
• t HU lesions + minimal enhancement; ± ascites
• Rarely necrosis ± air or cystic mimicking abscess
• Lymphadenopathy: Abdominal or retroperitoneal
o Angiosarcoma
• Solitary or multiple, nodular, irregular margins
(angiosarcoma)
• Heterogeneous density; variable enhancement
• Enlarged spleen; ± hemorrhage & calcification
• ± Hematoma: Intrasplenic/subcapsular/perisplenic
• ± Multiple liver or distant metastases
o Metastases
• Multiple, solid (common) or cystic, I attenuation
(metastases)
• Malignant melanoma: Solid or cystic
• Ovary, breast & endometrium: Hypodense, solid
• Central or peripheral enhancement
• Calcification (rare) except mucinous colon cancer
MR Findings
• Benign tumors
o Hemangioma
• T1WI: Hypointense; T2WI: Hyperintense
• T1 C+: Uniform or heterogeneous enhancement
o Hamartoma
 SPLENIC TUMORS
• Tl WI: Isointensej T2WI: Hypo-to hyperintense
• Tl C+: Variablej uniform on delayed scans Splenic infection
o Lymphangioma • Tuberculosis, fungal (microabscesses) & pyogenic
• TlWI: Hypointense; T2WI: Markedly hyperintense • Solitary or multiple, small or large, low density lesions
• Septa: T2WIj hypointense; enhances on Tl C+ • May show minimal peripheral enhancement
• Malignant tumors Splenic sarcoidosis
o Lymphoma
• Low density splenic nodules ranging from 0.3-2.0 cm
• MR not reliable due to similar Tl, T2 relaxation
• Periaortic & retrocrural adenopathy (small & discrete)
times & proton densities of spleen/lymphoma
• Associated mediastinal, hilar nodes & lung lesions
• SPIO-enhanced MR: Altered signal intensity of
spleen + overall less uptake of SPIO Artifact
o Angiosarcoma • Heterogeneous enhancement during arterial phase of
• Tl & T2WI: Variable signal due to hemorrhage, imaging
necrosis & calcification
• Tl C+: Variable or ring-like enhancement
o Metastases !PATHOLOGY
• Tl WI: Isointense to hypointense
• T2WI: Hyperintense General Features
• Tl C+: Enhancement depends on type of primary • Etiology: Primary splenic tumor (unknown)j
metastases (underlying malignancy)
Ultrasonographic Findings • Epidemiology: Incidence varies based on type of tumor
• Real Time
o Hemangioma Gross Pathologic & Surgical Features
• Echogenic masses with areas of complex echoes • Varies depending on type of tumor
• Complex masses: Solid & cystic areas
o Hamartoma Microscopic Features
• Well-defined, homogeneous echogenic mass • Varies based on histology of tumor
o Lymphangiomas
• Grossly enlarged spleen; multicystic appearance
• Intracystic internal echoes: Proteinaceous material I CLINICAL ISSUES
o Lymphoma
Presentation
• Typically diffuse or focal hypoechoic lesions
• Anechoic/mixed echoicj small or large nodules • Most common signs/symptoms: LUQ pain, palpable
o Angiosarcoma: Solid, mixed echogenic mass mass, splenomegaly, fever, weight loss
6 o Metastases: Iso-/hypo-/hyperechoic, "target"/"halo" Natural History & Prognosis
Angiographic Findings • Complications: Hemorrhage, rupture
28 • Prognosis: Good (benign tumors)j poor (malignant)
• Conventional
o Hemangioma Treatment
• "Cotton wool" appearance: Pooling of contrast • Surgical resection: Primary benign, malignant tumors
• No neovascularity or arteriovenous shunting
• Typically retain contrast beyond venous phase
o Hamartoma & angiosarcoma: Hypervascular I DIAGNOSTIC CHECKLIST
Nuclear Medicine Findings
Consider
• Hemangioma • Must rely on clinical setting & biopsy, if necessary
o Tc-99m labeled RBC scan with SPECT
• Early dynamic scan: Focal defect or less uptake Image Interpretation Pearls
• Delayed scans (over 30-50 min): Persistent filling • Imaging findings alone are unreliable in
distinguishing solid from pseudocystic splenic masses
Imaging Recommendations
• Helical NE + CECTj MR with Tl C+
I SELECTED REFERENCES
I DIFFERENTIAL DIAGNOSIS 1. Abott RM et al: Primary vascular neoplasms of the spleen:
radiologic-pathologic correlation. Radiographies. 24:
Splenic cyst 1137-63,2004
2. Urban BA et al: Helical CT of the spleen. Am.].
• Low density, sharp margins, no enhancement
Roentgenol. 170: 997-1003, 1998
• Rim of dense calcification; anechoic on ultrasound 3. Rabushka 1.5et al: Imaging of the spleen: CT with
Splenic hematoma supplemental MR examination. Radiographies.
14(2):307-32, 1994
• Subcapsular: Low attenuation, crescentic fluid
collection along lateral surface of spleen
• Intrasplenic: Irregular low density fluid collection
 SPLENIC TUMORS
I IMAGE GALLERY
Typical
(Left) Axial CECT in a 20
year old man shows partially
calcified splenic mass;
non-Hodgkin lymphoma
(untreated). (Right) Surgical
photograph of specimen of
spleen from a 20 year old
man with non-Hodgkin
lymphoma.

(Left) Axial CECT shows

innumerable hepatic masses;
angiosarcoma in a young
man. (Right) Axial CECT of a
young man with hepatic
angiosarcoma shows one of
several splenic tumors with
similar appearance.

6
29

Typical
(Left) Axial CECT in a patient
with cavernous
hemangiomas in the spleen,
liver, and body wall. (Right)
Axial CECT shows malignant
fibrous histiocytoma in the
spleen.
 SPLENIC METASTASES AND LYMPHOMA
Axial CECT in June, 2003 shows splenomegaly due to
non-Hodgkin lymphoma (NHL).
ITERMINOlOGY
Definitions
• Splenic metastases from other primary cancer site
• Splenic lymphoma: Malignant tumor of lymphocytes
I IMAGING FINDINGS
General features
• Best diagnostic clue: Solitary or multiple solid lesions
6 (metastases); diffuse infiltrative lesions (lymphoma)
• Size
30 o Splenic metastases: One-third are microscopic
nodules, two-thirds are grossly visible at autopsy
o Splenic lymphoma: Solitary mass or multifocal
lesions 1 cm or greater are detectable on CT
• Key concepts
o Splenic metastases
• Relatively uncommon (autopsy incidence, 7.1%)
• May be multiple (60%), solitary (31.5%), nodular
or diffuse infiltrative lesions (8.5%)
• Common route: Hematogenous (splenic artery)
• Retrograde « common): Via splenic vein (portal
HTN) & lymphatics
• Common primary sites & metastases to spleen:
Breast (21 %), lung (18%), ovary (8%), stomach
(7%), melanoma (6%), prostate (6%)
DDx: Multiple low Density Splenic lesions
Angiosarcoma
Repeat Axial CECT in August, 2003 shows marked
reduction of splenic size following treatment for NHL.
• Frequency of splenic metastases by primary
tumor: Melanoma (34%), breast (12%), lung (9%)
• "Cystic" metastases: Melanoma, adenocarcinoma
of breast, ovary & endometrium
• Serosal implants: Peritoneal carcinomatosis
secondary to ovarian, GIT, pancreatic cancers
• Direct invasion: Uncommon; may occur in large
gastric, colon, pancreatic tail cancer, left renal cell
cancer, neuroblastoma or retroperitoneal sarcoma
o Splenic lymphoma
• Most common malignant tumor of spleen
• Hodgkin (HD) & non-Hodgkin lymphoma (NHL)
• Primary lymphoma; secondary (more common)
• Primary splenic lymphoma: NHL (B-cell origin)
• Initial splenic involvement: Hodgkin (23-34%);
non-Hodgkin (30-40%)
• Spleen: Considered as "nodal organ" in Hodgkin &
"extranodal organ" in non-Hodgkin lymphoma
• Manifest: Focal lesions (> 1 cm) or diffuse (typical)
• Age: Primary (young age); secondary (older age)
• Presence or absence of splenic involvement may
determine type of therapy
• Staging laparotomy or splenectomy: Uncommonly
necessary to determine splenic involvement
o AIDS-related lymphoma
• Histologic subtypes: Small noncleaved (Burkitt &
non-Burkitt type)
• Splenic involvement: NHL > common than HD
Artifact (Perfusion)
 SPLENIC METASTASES AND LYMPHOMA
Key Facts
Imaging Findings
• Best diagnostic clue: Solitary or multiple solid lesions
(metastases); diffuse infiltrative lesions (lymphoma)
• Central or peripheral enhancement (metastases)
• Calcification (rare) except mucinous colon cancer
• Solitary mass; multifocal or diffuse infiltration
(lymphoma)
• Focal lesions: AIDS-related lymphoma> common
• Splenic hilum nodes: NHL (59%); HD (uncommon)
• Lymphadenopathy: Abdominal or retroperitoneal
• Splenic lymphoma (MR nonspecific)
Top Differential Diagnoses
• Splenic primary tumor
• Splenic infection
• Granulomatous
CT Findings
• Splenic metastases
o Decreased attenuation in relation to normal spleen
o Usually appear as well-defined masses
o Multiple, solid (more common) or "cystic", decreased
attenuation (metastases)
o Malignant melanoma: Solid or "cystic"
o Ovary, breast & endometrium: "Cystic"
(homogeneous & hypodense)
o Central or peripheral enhancement (metastases)
o Calcification (rare) except mucinous colon cancer
• Splenic lymphoma
o Homogeneously enlarged spleen, no discrete mass
o Solitary mass; multifocal or'diffuse infiltration
(lymphoma)
o Focal lesions: AIDS-related lymphoma> common
o j HU lesions + minimal enhancement; ± ascites
o Organ specific contrast agent: Ethiodol-Oil-Emulsion
(EOE-13) potentially increase detection rate
o Rarely necrosis ± air or cystic mimicking abscess
o Splenic hilum nodes: NHL (59%); HD (uncommon)
o Lymphadenopathy: Abdominal or retroperitoneal
MR Findings
• Splenic metastases
o T1WI: Isointense to hypointense
o T2WI: Hyperintense
o T1 C+: Enhancement depends on type of primary
• Conspicuity 1 with SPIO-enhanced imaging
• Splenic lymphoma (MR nonspecific)
o MR not reliable due to similar T1 & T2WI relaxation
times, proton densities of normal spleen/lymphoma
Ultrasonographic Findings
• Real Time
o Splenic metastases
• Iso-/hypoechoic, hyperechoic, "target" or "halo"
• Larger lesions more complex than smaller ones
• Echogenic lesions (rare): E.g" plasmacytoma,
hepatoma, melanoma, prostate & ovarian cancer
o Splenic lymphoma
• Typically diffuse or focal hypoechoic lesions
• Anechoic/mixed echoic; small or large nodules
Pathology
• Splenic metastases: Breast (21%), lung (18%), ovary
(8%), stomach (7%), melanoma (6%), prostate (6%)
• Hodgkin (HD) & non-Hodgkin lymphoma (NHL)
• Primary lymphoma; secondary (more common)
• Primary: Usually non-Hodgkin lymphoma (NHL)
• Secondary: Extension from generalized lymphoma
Diagnostic Checklist
• To look for primary cancer, generalized adenopathy
• HD (45-66%); NHL (70%) show diffuse infiltration
making difficult to detect by US, CT & MR
• Splenic size is not a reliable clinical & imaging
indicator of presence or absence of disease
• Imaging impbrtant to suggest & stage malignancy,
evaluate tumor volume/monitor response to therapy
• Large & necrotic: Heterogeneous appearance
• AIDS-related lymphoma: Uniform decreased
echogenicity or focal hypoechoic lesions
Angiographic Findings
• Conventional
o Splenic metastases
• Typically hypovascular or avascular
• Less frequently hypervascular
o Splenic lymphoma
• Nonspecific findings: May be normal; single or
multiple parenchymal defects
Nuclear Medicine Findings
• Tc99m sulfur colloid scintigraphy (liver-spleen scan) 6
o Splenic metastases: Areas of focal defects
o Splenic lymphoma: Nonspecific findings 31
• Areas of decreased tracer uptake within spleen
• Total lack of uptake ("functional asplenia")
• Altered splenic contour (encasement by tumor)
Imaging Recommendations
• Best imaging tool: Helical CT
I DIFFERENTIAL DIAGNOSIS
Splenic primary tumor
• Benign tumor
o Hemangioma
• Homogeneously solid. or multiple cystic masses
• Slightly hypodense on NECT & isodense on CECT
• Central punctate or peripheral curvilinear Ca++
• Malignant tumor
o Angiosarcoma
• Heterogeneous density; variable enhancement
• Solitary or multiple, nodular, irregular margins
• ± Multiple liver or distant metastases
Splenic infarction
• Wedge-shaped peripheral defect
Splenic infection
• Pyogenic (bacterial): Abscess
 SPLENIC METASTASES AND LYMPHOMA
o Low density lesion with thick, irregular dense rim
o ± Gas within fluid collection, left pleural effusion
• Fungal (e.g., Candida, Aspergillus, Cryptococcus)
o Solitary/multiple; small/large; low density lesions
o May show minimal peripheral enhancement
o Candida microabscesses
• Well-defined nonenhancing I attenuation lesions
• Typically 5-10 mm in size (smaller than 2 cm)
• Wheel within wheel pattern: 1 HU central focus
• AIDS: Pneumocystis carinii infection
o Focal low-attenuation splenic lesions
o Large lesions: Calcification, rim-like or punctate type
Granulomatous
• Tuberculosis
o Splenomegaly (SMG)
o Micro & macronodular lesions of low-attenuation
o ± Abdominal adenopathy, high density ascites
• Sarcoidosis
o SMG, low density nodules ranging from 0.3-2.0 cm
o Periaortic & retrocrural adenopathy (small, discrete)
o Associated mediastinal, hilar nodes & lung lesions
Artifact
• Early phase of of LV. bolus contrast injection
o Heterogeneous enhancement, mimic splenic tumor
I PATHOLOGY
General Features
• General path comments
o Splenic metastases
• Usually seen in widespread tumor dissemination
6 • Isolated splenic metastases are rarely seen
• Etiology
o Splenic metastases: Breast (21 %), lung (18%), ovary
32
(8%), stomach (7%), melanoma (6%), prostate (6%)
o Splenic lymphoma
• Hodgkin (HD) & non-Hodgkin lymphoma (NHL)
• Primary lymphoma; secondary (more common)
• Primary: Usually non-Hodgkin lymphoma (NHL)
• Secondary: Extension from generalized lymphoma
• Epidemiology
o Splenic metastases
• 7.1% of patients with malignancy at autopsy
o Splenic lymphoma
• Primary splenic lymphoma: 1% of all cases of NHL
• Hodgkin (23-34%); non-Hodgkin (30-40%)
• Associated abnormalities
o Primary carcinoma in splenic metastases
o Generalized adenopathy in secondary lymphoma
Gross Pathologic & Surgical Features
• Splenic metastases: Solitary or multiple
• Splenic lymphoma
o Solitary, multifocal, diffuse infiltration (> common)
o Homogeneously enlarged spleen, no discrete mass
Microscopic Features
• Splenic metastases: Varies based on primary carcinoma
• Splenic lymphoma: Hodgkin type
o Nodular sclerosing; mixed cellular
o Lymphocyte predominance; lymphocyte depletion
Staging, Grading or Classification Criteria
• Ann Arbor staging: Anatomic extent of HD & NHL
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Splenic metastases
• Asymptomatic; LUQ pain, mass, splenomegaly
• Acute pain: Splenic infarct due to tumor emboli
o Splenic lymphoma
• Fever, weight loss, night sweats, malaise
• LUQ pain, palpable mass, splenomegaly
• Diagnosis: Imaging + clinical; guided splenic biopsy
Demographics
• Age
o Primary splenic lymphoma: Older age group
o Splenic metastases: Any age group
• Gender
o HD: Young age, M:F = 4:1; older age, M:F = 2:1
o NHL: M:F = 1.4:1
Natural History & Prognosis
• Complications
o Splenic metastases: Splenic vein thrombosis, rupture
• Prognosis
o Splenic metastases: Poor prognosis
o Splenic lymphoma: Early stage (good); late (poor)
Treatment
• Chemotherapy, radiation or surgery in isolated lesion
I DIAGNOSTIC CHECKLIST
Consider
• To look for primary cancer, generalized adenopathy
Image Interpretation Pearls
• HD (45-66%); NHL (70%) show diffuse infiltration
making difficult to detect by US, CT & MR
• Splenic size is not a reliable clinical & imaging
indicator of presence or absence of disease
• Imaging important to suggest & stage malignancy,
evaluate tumor volume/monitor response to therapy
I SELECTED REFERENCES
1. Urban BAet al: Helical CT of the spleen. AJRAm J
Roentgenol. 170(4):997-1003, 1998
2. Rabushka 15 et al: Imaging of the spleen: CT with
supplemental MR examination. Radiographies.
14(2):307-32, 1994
3. Freeman J1 et al: CT of congenital and acquired
abnormalities of the spleen. Radiographies. 13(3):597-610,
1993
4. Taylor AJ et al: CT of acquired abnormalities of the spleen.
AJRAm J Roentgenol. 157(6):1213-9, 1991
5. Hahn PF et al: MR imaging of focal splenic tumors. AJRAm
J Roentgenol. 150(4):823-7, 1988
 SPLENIC METASTASES AND LYMPHOMA

I IMAGE GALLERY

(Left) Axial CECT shows

splenic metastases from
melanoma. (Right) Axial
CECT shows splenic, liver
and adrenal metastases from
primary sinus carcinoma.

Typical
(Left) Axial CECT shows
splenic, nodal and adrenal
masses due to NHL. (Right)
Axial CECT shows splenic
metastasis from
choriocarcinoma.

6
33

Typical
(Left) Axial CECT shows
splenic and liver metastases
from melanoma. (Right)
Axial CECTshows splenic
metastases from melanoma.
 SPLENOMEGALY AND HYPERSPLENISM

Axial CECT shows heterogeneous enlargement of liver Axial CECT shows splenomegaly due to chronic
and spleen due to amyloidosis. lymphocytic leukemia.

ITERMINOLOGY • Morphology
o Enlarged spleen tends to become directed anteriorly
Abbreviations and Synonyms o Splenic tip extends below tip of right lobe of liver
• Splenomegaly (SMG); hypersplenism (HS) o Mild, moderate or marked splenomegaly

Definitions Radiographic Findings

• Splenomegaly: Enlarged spleen; volume> 500 cm3
• Hypersplenism: Syndrome consisting of splenomegaly
& pancytopenia in which bone marrow is either
normal or hyperreactive
6 o Calcification within or adjacent to spleen
I IMAGING FINDINGS
34
General Features
• Best diagnostic clue: Increased volume of spleen with
convex medial border
• Location: Spleen occupies LUQ with tip extending
inferiorly below 12th rib
• Size
o Normal spleen in adult measures up to 13 cm;
enlarged if it is 14 cm or longer
o Splenic index: Normally 120-480 cm3 (product of
length, breadth & width of spleen)
o Splenic weight: Splenic index x 0.55
• Normal weight: 100-250g
o SMG: Anteroposterior (AP) diameter> two-thirds
distance of AP diameter of abdominal cavity
• Radiography
o Splenic tip below 12th rib
o Marked SMG may displace stomach medially
o Displacement of splenic flexure of colon (splenic
flexure usually anterior to spleen)
CT Findings
• SMG: Medial margin of spleen is convex on CT
• Congestive SMG
o Portal hypertension: SMG with varices, nodular
shrunken liver, ascites
o Splenic vein occlusion or thrombosis (often
secondary to pancreatitis or pancreatic tumors)
o Sickle-cell disease: Splenic sequestration
• Peripheral j HU areas + areas 1 attenuation
• Represent areas of infarct & hemorrhage
• Storage disorders
o Gaucher disease
• Spleen may have abnormal low attenuation
• Marked SMG, often extending into pelvis
o Amyloidosis .
• NECT & CECT: Generalized or focal j densIty

DDx: Splenic or left Upper Quadrant Mass

Gastric Tumor Candida Abscesses Melanoma Metastases Splenic MAl, AIDS

 SPLENOMEGALY AND HYPERSPLENISM
Key Facts
Terminology
• Splenomegaly (SMG); hypersplenism (HS)
• Splenomegaly: Enlarged spleen; volume> 500 cm3
• Hypersplenism: Syndrome consisting of
splenomegaly & pancytopenia in which bone marrow
is either normal or hyperreactive
Imaging Findings
• Best diagnostic clue: Increased volume of spleen with
convex medial border
• Normal spleen in adult measures up to 13 cm;
enlarged if it is 14 cm or longer
• Splenic tip below 12th rib
• Marked SMG may displace stomach medially
• Displacement of splenic flexure of colon (splenic
flexure usually anterior to spleen)
o Primary hemochromatosis
• Density of spleen is normal (unlike that of liver)
o Secondary hemochromatosis
• Increased attenuation values in liver & spleen
• Space occupying lesions: Cysts, abscess, tumor
• Splenic infarction (veno-occlusion caused by sickling)
o SMG with focal infarcts; peripheral areas of low
attenuation & hemorrhage associated with SMG
• Hemosiderosis
o 1 Attenuation of spleen (hemosiderin deposition)
o Consequence of multiple blood transfusions
(thalassemia, hemophilia)
• Splenic trauma
o Splenic laceration or subcapsular hematoma
o Surrounding perisplenic hematoma (> 30 HU)
• Extramedullary hematopoiesis
o Spleen may be diffusely enlarged
o CECT: Focal masses of hematopoietic tissue that are
isoattenuating relative to normal splenic tissue
MR Findings
• Congestive SMG
o Portal hypertension
• Multiple tiny (3-8 mm) foci of decreased signal
• Hemosiderin deposits; organized hemorrhage
(Gamna-Gandy bodies or siderotic nodules)
o Sickle cell disease (splenic sequestration)
• Areas of abnormal signal intensity
• Hyperintense with dark rim on Tl WI (subacute
hemorrhage)
o Hemochromatosis
• Primary: Normal signal & size of spleen
• Secondary: Marked signal loss; enlarged spleen
o Gaucher disease: Increased signal intensity on Tl WI
o Infarction
• Peripheral, wedge-shaped areas of abnormal signal
• Low signal resulting from iron deposition
o Hemosiderosis
• ! Signal intensity of spleen on both Tl & T2WI
o Extramedullary hematopoiesis
• Focal hypointense nodules
Top Differential Diagnoses
• Other LUQ masses
• Lymphoma & metastases
• Primary splenic tumor
Diagnostic Checklist
• SMG, most common cause of left upper quadrant
mass
• SMG, usually a systemic cause rather than primary
• US can confirm presence of enlarged spleen or space
occupying lesions
• CT & MR can further clarify abnormalities in size,
shape & define parenchymal pathology
• Radioisotope scanning can diagnose HS & provide
functional status of spleen
Ultrasonographic Findings
• Real Time
o SMG with normal echogenicity
• Infection, congestion (portal HT), early sickle cell
• H. spherocytosis, hemolysis, Felty syndrome
• Wilson disease, polycythemia, myelofibrosis,
leukemia
o SMG with hyperechoic pattern
• Leukemia, post chemo & radiation therapy
• Malaria, TB, sarcoidosis, polycythemia
• Hereditary spherocytosis, portal vein thrombosis,
hematoma, metastases
o SMG with hypo echoic pattern
• Lymphoma, multiple myeloma, chronic
6
lymphocytic leukemia
35
• Congestion from portal HT, noncaseating
granulomatous infection
o Sickle cell disease: Immediately after sequestration,
peripheral hypoechoic areas seen
o Gaucher disease: Multiple, well-defined, discrete
hypoechoic lesions; fibrosis or infarction
Nuclear Medicine Findings
• Chromium 51-labeled RBCs or platelets
o HS is diagnosed if injected RBCs exhibit shortened
half-life (average half-life of 25-35 days)
• Tc99m sulfur colloid scan: Detect splenic function
Imaging Recommendations
• Best imaging tool: Helical CT
I DIFFERENTIAL DIAGNOSIS
Other lUQ masses
• E.g., gastric, renal, adrenal tumor
lymphoma & metastases
• Lymphoma: Various patterns of splenic involvement
o Homogeneous enlargement without discrete mass
o Solitary or multifocallesions: Discrete! HU lesions
o Diffuse infiltration with SMG
• Metastases
 SPLENOMEGALY AND HYPERSPLENISM
o I Attenuation; "cystic" or solid masses; SMG o Extramedullary hematopoiesis: Osteopetrosis,
o "Cystic" metastases: "Homogeneous & hypodense" myelofibrosis
• E.g., melanoma, ovary, breast & endometrium o Collagen disease: SLE, RA, Felty syndrome
o Splenic trauma; sarcoidosis; hemodialysis
Primary splenic tumor • Associated abnormalities: HS seen in association with
• Benign tumor hemoglobinopathies & autoimmune diseases
o Hemangioma
• Homogeneously solid or multiple cystic masses Microscopic Features
• Central punctate or peripheral curvilinear Ca++ • Varies depending on underlying etiology
• Slight hypodense on NECT & isodense on CECT
o Hamartoma
• Iso-hypodense on NECT; variable enhancement I CLINICAL ISSUES
• Central star-like scar or necrosis, focal calcification
o Lymphangioma Presentation
• Thin-walled low density lesions; sharp margins • Most common signs/symptoms
• CECT: Enhancement of walls but not contents o Asymptomatic, splenomegaly, abdominal pain
• Usually subcapsular in location; ± calcification o Signs & symptoms related to underlying cause
• Malignant tumor • Lab data: Abnormal CBC, LFT, antibody titers, cultures
o Angiosarcoma or bone marrow exam
• Solitary or multiple, nodular, irregular margins
Natural History & Prognosis
• Heterogeneous density; variable enhancement
• Enlarged spleen; ± hemorrhage & calcification • Complications
o Splenic rupture, shock & death
• ± Multiple liver or distant metastases
• HS: Usually develops as a result of SMG
• ± Hematoma: Intrasplenic/subcapsular/perisplenic
o Hyperfunctioning spleen removes normal RBC,
Splenic infection WBC & platelets from circulation
• Pyogenic (bacterial) • Prognosis
o Abscess o Splenic rupture, sequestration in SC disease: Poor
• Low density lesion with thick, irregular dense rim
Treatment
• ± Gas within fluid collection, left pleural effusion
• Treatment varies based on underlying condition
• Fungal (e.g., Candida, Aspergillus, Cryptococcus)
• Splenectomy in symptomatic & complicated cases
o Solitary/multiple; small/large; low density lesions
o May show minimal peripheral enhancement
o Candida: Microabscesses (nonenhancing lesions)
I DIAGNOSTIC CHECKLIST
6 • AIDS: Pneumocystis, mycobacterial (e.g., MAl)
o Focal low attenuation splenic lesions Consider
o Large lesions: Calcification, rim-like or punctate type
36 • SMG, most common cause of left upper quadrant mass
Granulomatous • SMG, usually a systemic cause rather than primary
• TB: Micro & macronodular lesions of low attenuation Image Interpretation Pearls
• Sarcoidosis • us can confirm presence of enlarged spleen or space
o SMG with low density intra splenic lesions
occupying lesions
o Abdominal & pelvic lymphadenopathy • CT & MR can further clarify abnormalities in size,
• Mycobacterium avium intracellulare (in AIDS patients) shape & define parenchymal pathology
o Low density splenic lesions; marked SMG • Radioisotope scanning can diagnose HS & provide
functional status of spleen
I PATHOLOGY
General Features I SELECTED REFERENCES
• Etiology 1. Peck-Radosavljevic M: Hypersplenism. Em J Gastroenterol
o Congestive SMG Hepatol. 13(4):317-23,2001
• CHF, portal HT, cirrhosis, cystic fibrosis, splenic 2. McCormick PA et al: Splenomegaly, hypersplenism and
coagulation abnormalities in liver disease. Clin
vein thrombosis, sickle cell (SC) sequestration
Gastroenterol. 14(6):1009-31,2000
o Neoplasm: Leukemia, lymphoma, metastases, 3. Paterson A et al: A pattern-oriented approach to splenic
primary neoplasm, Kaposi sarcoma imaging in infants and children. Radiographies.
o Storage disease: Gaucher, Niemann-Pick, gargoylism, 19(6):1465-85, 1999
amyloidosis, DM, hemochromatosis, histiocytosis 4. Bowdler AJ: Splenomegaly and hypersplenism. Clin
o Infection: Hepatitis, malaria, mononucleosis, TB, Haematol. 12(2):467-88, 1983
typhoid, kala-azar, schistosomiasis, brucellosis 5. Mittelstaedt CA et al: Ultrasonic-pathologic classification of
o Hemolytic anemia: Hemoglobinopathy, hereditary splenic abnormalities: gray-scale patterns. Radiology.
134(3):697-705, 1980
spherocytosis, primary neutropenia,
thrombocytopenic purpura
 SPLENOMEGALY AND HYPERSPLENISM
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
acquired splenic cyst
following infarct (cardiac
assist device). (Right) Axial
NECT shows splenomegaly
due to myeloproliferative
disorder.

Typical
(Left) Axial CECT in June,
2003 shows splenomegaly
due to non-Hodgkin
lymphoma. (Right) Axial
CECT in August, 2003 shows
marked reduction in splenic
size following treatment for
lymphoma.

6
37

Typical
(Left) Axial CECT shows
spontaneous rupture of
spleen due to leukemia.
(Right) Axial CECT shows
splenomegaly due to
cirrhosis and portal
hypertension.
 PART II 3
Hepatobillary and Pancreas

iver [I]
I iary System [I)
Pancreas [}]
 SECTION 1: Liver

Introduction and Overview Metabolic - Inherited

Liver Anatomy and Imaging Issues 11-1-2 Hemochromatosis 11-1-84
Wilson Disease 11-1-88
Congenital
Congenital Absence of Hepatic Segments 11-1-6
Trauma
Congenital Hepatic Fibrosis 11-1-8 Hepatic Trauma 11-1-90
AD Polycystic Disease, Liver 11-1-12 Biliary Trauma 11-1-94

Infection Neoplasm, Benign

Hepatitis 11-1-16 Hepatic Cyst 11-1-98
Hepatic Candidiasis 11-1-20 Hepatic Cavernous Hemangioma 11-1-102
Hepatic Pyogenic Abscess 11-1-24 Focal Nodular Hyperplasia 11-1-106
Hepatic Amebic Abscess 11-1-28 Hepatic Adenoma 11-1-110
Hepatic Hydatid Cyst 11-1-32 Biliary Hamartoma 11-1-114
Hepatic Angiomyolipoma 11-1-118
Inflammation
Steatosis (Fatty Liver) 11-1-36
Neoplasm, Malignant
Cirrhosis 11-1-40 Hepatocellular Carcinoma 11-1-120
Focal Confluent Fibrosis 11-1-44 Fibrolamellar HCC 11-1-124
Primary Biliary Cirrhosis 11-1-48 Cholangiocarcinoma (Peripheral) 11-1-128
Nodular Regenerative Hyperplasia 11-1-52 Epithelioid Hemangioendothelioma 11-1-132
Hepatic Sarcoidosis 11-1-56 Biliary Cystadneocarcinoma 11-1-136
Angiosarcoma, Liver 11-1-140
Vascular Disorder Hepatic Metastases and Lymphoma 11-1-144

Hepatic AVMalformation (O-W-R) 11-1-60

Budd-Chiari Syndrome 11-1-64
Treatment Related
Passive Hepatic Congestion 11-1-68 Radiation Hepatitis 11-1-148
HELLP Syndrome 11-1-72
Hepatic Infarction 11-1-76
Peliosis Hepatis 11-1-80
 LIVER ANATOMY AND IMAGING ISSUES
1
2
A
8
4A
• 2

~
I..., B
4A
1 2

B
) 8
1

•
_c_ 7

Tc
y-
,
D
48 . 3
t 5
6

0 5 48 3

Graphic shows 4 sections through the liver depicting the Axial CECT sections correspond to levels in graphic.
8 segments of the liver which are separated by vertical Liver segments are numbered. Falciform ligament plane
planes through the hepatk veins & horizontal plane separates medial (seg. 4) from lateral (segs. 2 &3) left
through the portal vein. lobe. Seg. 3 not shown.

o Abnormally increased density usually due to iron

I TERMINOLOGY deposition (primary or secondary hemochromatosis)
Definitions or amiodarone Rx (antiarrhythmic drug)
• Phases of dynamic CT/MR imaging following a rapid • Is there evidence of cirrhosis?
bolus infusion of contrast material o Look for widened fissures, relative increase of
o Early arterial (- 18-25 see) caudate to right lobe width ratio (> 0.6), nodular
• Indications: Best for hepatic arterial angiography surface contour, signs of portal hypertension
o Late arterial (portal venous inflow) (- 35-40 see) (splenomegaly, ascites, varices)
• Indications: Best for detecting + characterizing • What is the status of the major hepatic vessels?
hypervascular tumors o Hepatic veins dilated?
o Portal venous (parenchymal) (- 70 see) • Consider passive congestion (cardiac dysfunction),
• Indications: Should be obtained in almost all arteriovenous shunts (e.g., Osler-Weber-Rendu)
abdominal CT/MR scans o Hepatic vein(s) occluded?
o Equilibrium (delayed) (2-10 minutes) • Consider Budd-Chiari, hypercoagulable state,
• Indications: As an added sequence to characterize tumor encasement
a known or suspected hepatic mass o Portal vein dilated?
• Especially for hemangioma or • Consider "early" portal hypertension, tumor
cholangiocarcinoma invasion (especially hepatocellular carcinoma)
o Portal vein occluded?
• Consider "late" portal hypertension with shunts,
IMAGING ANATOMY hypercoagulable state, septic thrombophlebitis
(e.g., diverticulitis), tumor encasement
Location o Hepatic artery dilated?
• Liver is divided into 8 segments (Bismuth system) • Consider cirrhosis (portal hyperention),
o Vertical planes through the course of hepatic veins hypervascular liver tumor, congenital hepatic
o Horizontal plane through the right and left portal fibrosis, portal vein occlusion, arteriovenous
vein shunts
• What is the morphology, vascularity, and density of a
tumor relative to liver, preferably defined on
I ANATOMY-BASED IMAGING ISSUES I nonenhanced and multiphasic enhanced scans (CT or
MR)?
Key Concepts or Questions o Most tumors are spherical, hypovascular, and
• What is the overall density (attenuation) of the liver? hypodense to normal liver on all phases of imaging
o Should be about 8-10 HU greater than that of spleen o Simple cysts are near water density, do not enhance,
or muscle have no mural nodularity
o Abnormally decreased density usually due to fatty o Abscesses are above water density, do not enhance
infiltration (steatosis), but may occur with diffuse (contents), have mural irregularities +/- septations
tumor (e.g., lymphoma), infection (e.g., acute severe o All tumors (viable) enhance and almost always show
viral hepatitis, opportunistic infections) or acute some solid component
drug toxicity
 LIVER ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
1
Benign Liver Masses Malignant Liver Tumors 3

Hepatocellular origin Hepatocellular origin

• Hepatic adenoma • Hepatocellular carcinoma (HCC)
• Focal nodular hyperplasia • Fibrolamellar HCC
• Hyperplastic + dysplastic nodules • Hepatoblastoma
• Clear cell + giant cell carcinoma
Cholangiocellular origin • Carcinosarcoma
• Simple hepatic cyst
• Congenital hepatic fibrosis/polycystic Cholangiocellular origin
• Cholangiocarcinoma
Mesenchymal origin • Biliary cystadenocarcinoma
• Cavernous hemangioma
• Lipoma, angiomyolipoma Mesenchymal origin
• Mesenchymal hamartoma • Epithelioid hemangioendothelioma
• Lymphangioma • Angio-,leiomyo-, fibro-, osteosarcoma
• Leiomyoma, fibroma; heterotopic tissue • Primary lymphoma + MFH

o Hypervascular tumors have diffuse or partial
hyperdensity (or intensity, MR) compared with liver
on arterial phase images
o Tumors with fibrous stroma (e.g.,
cholangiocarcinoma) or large vascular spaces (e.g.,
cavernous hemangioma) are hyperdense to normal
liver on delayed imaging
• Are non enhanced CT (or MR) images needed in
addition to enhanced images?
o Not for "acute abdomen" indications (e.g., trauma,
abscess, etc.)
o Not for most follow-up scans in most oncology
patients
o Yes, for patients with liver dysfunction or new
diagnosis of cancer
o Parenchymal phase (portal venous): Portal and
hepatic veins densely opacified and hyperdense to
liver parenchyma which is maximally enhanced
o Equilibrium (or delayed phase) should never be
obtained alone, only in addition to other phases:
Liver, vessels, nodes, most masses, etc. all isodense
I EMBRYOLOGY
Embryologic Events
• Anomalies may occur during embryologic
development of the ductal plate that surrounds the
portal vein
• 0 May result in variety of "fibropolycystic" defects

o Nonenhanced (+ enhanced) images help to detect (e.g., polycystic disease, Caroli, congenital hepatic
and distinguish fatty infiltration and benign hepatic fibrosis)
lesions with greater confidence (e.g., small lesion
less than blood density on non enhanced scan that
shows no change in size or apparent enhancement is I CUSTOM DIFFERENTIAL DIAGNOSISI
a cyst, not a "lesion too small to characterize")
• Which patients require evaluation by multiphasic CT Focal hyperdense (noncalcified) lesion
(or MR)? • Noncontrast CT
o Patients with known or suspected cirrhosis, primary • "Any" mass in a fatty liver
hepatic mass, known hypervascular primary tumor o Even focal sparing
(e.g., endocrine cancer) • Mucinous metastases
• What are the technical requirements and timing • Acute hemorrhage
necessary to obtain multiphase CT (or MR) scans of o Trauma
the liver? o Bleeding tumor
o Adequate volume + rate of IV contrast medium o Anticoagulated state
o For CT, ~ 125 ml or 40 gm of iodine, at ~ 3 mllsec • Budd-Chiari + primary sclerosing cholangitis
o Arterial phase (= portal venous inflow phase) at o "Spared" hypertrophied segments
about 3S sec delay (assuming normal cardiac output) Focal hypervascular liver lesion
o Portal venous phase (= hepatic parenchymal phase)
• Benign
at about 70 sec delay (longer for elderly patients,
o Focal nodular hyperplasia
shorter for injection rates of ~ 4 ml/sec)
o Arterioportal shunt or THAD
• How can you determine the effective phase and
o Hepatic adenoma
adequacy of an hepatic CT scan?
o Dysplastic or regenerative nodule*
o Arterial phase: Arteries densely opacified, portal
• Malignant
veins moderately enhanced, liver parenchyma +
o Hepatocellular carcinoma (HCC)
hepatic veins not, or minimally enhanced
o Fibrolamellar HCC
o Cholangiocarcinoma*
 LIVER ANATOMY AND IMAGING ISSUES
1
4

Graphic shows variation of hepatic arterial (HA) CT Angiogram shows accessory RHA (arrow) arising
anatomy. 1 = conventional; 2 = accessory LHA; 3 = from SMA. Other hepatic arteries supplied by the celiac
accessory RHA; 4 = replaced RHA from SMA; 5 = trunk. Gastroduodenal artery (open arrow).
totally replaced hepatic artery from SMA

o Metastases • Cholangiocarcinoma
• Endocrine (islet cell, thyroid, carcinoid) • Focal confluent fibrosis
• Renal cell carcinoma • Epithelioid hemangioendothelioma
• Sarcoma • Primary sclerosing cholangitis
• Breast* + Melanoma* • Hemangioma (rarely)
• * Uncommonly
• Hyperperfusion abnormalities (transient hepatic Liver mass with scar
attenuation difference = THAD) . • FNH (focal nodular hyperplasia)
o Small, subcapsular • HCC - fibrolamellar
• Idiopathic, probably small portal vein (PV) • Cavernous hemangioma (large)
thrombus • Cholangiocarcinoma
• Usually insignificant • Adenoma (rare)
o Subsegmental • HCC - conventional*
• Arterioportal (AP) fistula following biopsy • (Metastasis)*
• Cirrhosis with spontaneous AP shunt • * Usually central necrosis, not scar
• Adjacent hypervascular mass or abscess Periportal lucency or edema
• PV obstruction or thrombus (often malignant)
• (Biliary dilation)
o Segmental/lobar
• Overhydration
• PV obstruction or thrombus (often malignant)
• Congestive heart failure
• Mass effect on PV
• Acute hepatitis
• HCC with AP shunt
• Obstructed lymphatics
Fat containing liver masses o E.g., porta hepatis tumor
• Hepatocellular carcinoma • Liver transplantation
• Hepatic adenoma • Liver tumor
• Metastasis (liposarcoma, teratoma)
• Angiomyolipoma, lipoma
• (Focal fatty infiltration) I SELECTED REFERENCES
1. Diihnert W: Radiology Review Manual (4th ed). Baltimore:
Complex cystic mass Williams & Wilkins, 1999
• Abscess 2. Venbrux AC, Friedman AC: Diffuse hepatocellular diseases,
o Pyogenic, amebic, hydatid portal hypertension, and vascular diseases. In Friedman
• Cystic or necrotic metastases AC, Dachman AH (eds): radiology of the Liver, Biliary
• Necrotic or ablated HCC Tract, and Pancreas. St. Louis: CV Mosby, 49-168, 1994
• Biliary cystadenoma/carcinoma 3. Friedman AC, Frazier S, Hendrix TM, et al: Focal disease. In
Friedman AC, Dachman AH (eds): radiology of the LIver,
• Hemorrhagic cyst
Biliary Tract, and Pancreas. St Louis: CV Mosby, 169-328,
• Biloma or old hematoma 1994
• Intrahepatic pseudocyst 4. Reeder, MM: Reeder and Felson's Gamuts in Radiology (3rd
ed). New York: Springer-Verlag, 1993
Focal lesion with capsular retraction
• Metastasis (usually post-treatment)
 LIVER ANATOMY AND IMAGING ISSUES
IMAGE GALLERY 1
5
Typical
(Left) Axial CECT in
parenchymal phase, shows
"hyperdense" enlarged
caudate lobe which is
normal hypertrophied liver in
this patient with Budd-Chiari
syndrome. (Right) Axial
CECT arterial phase (A) +
parenchymal phase (B)
show multiple THAD (open
arrows) due to portal venous
branch occlusions from
metastases (arrows).

(Left) Axial CECT in arterial

phase, shows prototype of
hypervascular liver mass, a
focal nodular hyperplasia
(FNH) (arrow). (Right) Axial
CECT in parenchymal phase,
shows prototype tumor with
scar, a fibrolamellar HCC
(arrows).

Typical
(Left) Axial CECT in
parenchymal phase, shows
prototype complex cystic
mass, biliary cystadenoma.
(Right) Axial CECT in
parenchymal phase shows
prototype mass with capsular
retraction,
cholangiocarcinoma.
 CONGENITAL ABSENCE OF HEPATIC SEGMENTS
1
6

Axial NECT shows absence of anterior and medial Axial CECT shows congenital absence of the medial
segments of the liver. segment with the lateralsegment of the liver contiguous
to the spleen.

o Severe distortion of hepatic morphology

!TERMINOLOGY • Ectopy of gall bladder
Abbreviations and Synonyms CT Findings
• Agenesis or hypoplasia of hepatic lobes/segments
• Altered normal topography of upper abdomen
Definitions • Right lobar agenesis: Absence of liver tissue, right of
• Lobar agenesis is a rare developmental anomaly with main interlobar plane
absence of liver tissue to the right or left of gallbladder o Absence of right hepatic vein, portal vein & its
fossa without prior surgery or disease branches, & right intrahepatic ducts
o Suprahepatic/ subdiaphragmatic/infrahepa tic
location of gallbladder
IIMAGING FINDINGS o Colonic interposition/ high position of right
kidney/U- or hammock-shaped stomach
General Features o Direct contact of inferior vena cava to posterior
• Best diagnostic clue surface of medial segment of left lobe
o Absence of right or left hepatic vein, portal vein & • Agenesis of left lobe: Absence of liver tissue to the left
its branches, & intrahepatic ducts of main interlobar plane
• If none of these structures are visible, agenesis is o Failure to visualize falciform ligament or
substantiated ligamentum teres
• Location o Stomach & splenic flexure of colon migrate
o Right lobe: Segments V & VIII (anterior segments) superiorly & medially, low-lying hepatic. flexure
• Segments VI & VII (posterior segments) o High position of duodenal bulb/U-shaped stomach
o Left lobe: Segments II & III (lateral segment)
Angiographic Findings
• Segment IVa & IVb: Medial segment
• Conventional: Absence of right/left hepatic artery &
o Segment I is caudate lobe
portal vein & its branches
• Size: Compensatory hypertrophy of remaining
segments Other Modality Findings
• Key concepts • Cholangiography: Absence of right/left hepatic duct

DDx: Absence of Hepatic Segment(s)

Cirrhotic Atrophy Right Hepatectomy Chemoem bo/i za tion Chemoembo/ization

 CONGENITAL ABSENCE OF HEPATIC SEGMENTS

Key Facts
Terminology
• Lobar agenesis is a rare developmental anomaly with
absence of liver tissue to the right or left of
gallbladder fossa without prior surgery or disease
Imaging Findings
• Absence of right or left hepatic vein, portal vein & its
branches, & intrahepatic ducts
1
Clinical Issues 7
• Symptoms of associated: Biliary tract disease, portal
hypertension & volvulus of stomach
Diagnostic Checklist
• Conditions including cirrhosis, atrophy secondary to
biliary obstruction, hepatic surgery & trauma can
mimic agenesis, & should first be ruled out

Imaging Recommendations Gross Pathologic & Surgical Features

• Best imaging tool: CT is most commonly used • Unusual enlargement of remaining segments, resultant
• Protocol advice: NECT + CECT displacement of gallbladder & change in axis of fissure
of ligamentum venosum

I DIFFERENTIAL DIAGNOSIS
I CLINICAL ISSUES
Acquired atrophy after infarction, fibrosis
• At least one of these structures (hepatic vein, portal Presentation
vein & dilated intrahepatic ducts) is recognizable • Discovered incidentally on imaging studies
• Atrophy of anterior + medial segments commonly • Symptoms of associated: Biliary tract disease, portal
follows development of focal confluent fibrosis in hypertension & volvulus of stomach
cirrhosis
Natural History & Prognosis
Post surgical resection • Calculus formation & biliary malignancy are very rare
• For hepatic resection, incisions can be made along
longitudinal or transverse scissurae or both combined
Post chemoembolization
I DIAGNOSTIC CHECKLIST
• Hyperattenuation of atrophic liver parenchyma Consider
• Conditions including cirrhosis, atrophy secondary to
biliary obstruction, hepatic surgery & trauma can
!PATHOLOGY mimic agenesis, & should first be ruled out
General Features
• Etiology I SELECTED REFERENCES
o Agenesis of right lobe is thought to result from:
Either failure of right portal vein to develop or an 1. Gathwala G et al: Agenesis of the right lobe of liver. Indian
J Pediatr. 70(2):183-4,2003
error in mutual induction between septum 2. Sato N et al: Agenesis of the right lobe of the liver: report of
transversum (primitive diaphragm) & endodermal a case. Surg Today. 28(6):643-6, 1998
diverticulum (primitive liver) 3. Chou CK et al: CT of agenesis and atrophy of the right
o Left lobe agenesis results from extension of hepatic lobe. Abdom Imaging. 23(6):603-7, 1998
obliterative process that closes ductus venosus to It.
branch of portal vein
• Epidemiology I IMAGE GALLERY
o Incidence of lobar agenesis: 0.005% of 19,000
autopsy caseSj about 42 cases of agenesis of right
lobe are reported in literature
-- -.
.J>r
,.

,
o Left lobe agenesis slightly more common than !Ji'
right-sided anomalies
*
~,.
• Associated abnormalities
o With agenesis of right lobe: Partial or complete
absence of right hemidiaphragm
o Intestinal malrotation/choledochal cysts/agenesis of
gall bladder/intrahepatic venovenous shunt
o With agenesis of left lobe: Partial or complete
absence of left hemidiaphragm & gastric volvulus
~
, ~
~ '
••
(Left) Axial CECT shows congenital hypoplasia of left hepatic lobe.
(Right) Axial CECT shows congenital absence of anterior and medial
segments of the liver.
 CONGENITAL HEPATIC FIBROSIS
1
8

Cross section of explanted liver shows parenchymal Axial CECT shows dysmorphic liver with varices and
distortion with extensive fibrosis, especially in anterior splenomegaly large left lobe, atrophic right.
and medial segments.

• Severe renal disease & mild CHF/liver cysts

!TERMINOlOGY • Severe liver (CHF/liver cysts) & mild renal disease
Abbreviations and Synonyms o All patients with ARPKDhave findings of CHF at
• Congenital hepatic fibrosis (CHF), also referred as liver biopsy
fibropolycystic liver disease • Not all patients with CHF have ARPKD
o Variants of congenital hepatic fibrosis (CHF)
Definitions • Biliary hamartomas; polycystic liver disease &
• Part of a spectrum of congenital abnormalities Caroli disease
resulting in variable degrees of fibrosis & cystic o CHF: Exists by itself (very rare)
anomalies of liver & kidneys • Coexists more commonly other with polycystic
liver diseases

IIMAGING FINDINGS Radiographic Findings

• ERCP
General Features o ± Dilatation of intrahepatic bile ducts
• Best diagnostic clue: Combination of cystic dilatation CT Findings
of bile ducts + renal collecting duct ectasia
• Mild CHF
• Location: Both lobes of liver
o Liver may appear normal
• Size: Hepatic cystic size varies based on severity of CHF
• Moderate to severe CHF
• Other General Features
o Bile ducts: Normal to irregularly dilated
o Always present in autosomal recessive polycystic
o Enlarged dysmorphic liver; left lobe hypertrophy,
kidney disease (ARPKD)
right lobe atrophy
• Sometimes with autosomal dominant PKD
o Splenomegaly
o Two constant features of ARPKDinvolve
o Varices; increased size and number of hepatic
• Kidney: Tubular ectasia (cysts) & fibrosis
arteries
• Liver: CHF (dilated bile ducts; portal tracts
o May develop hypervascular benign large
enlarged/fibrotic) & multiple cysts
regenerative nodules
o Relative severity of organ involvement varies
• Similar to those seen in Budd-Chiari syndrome

DDx: Dysmorphic Liver with Abnormal Bile Ducts

ADPLD Sclerosing Cholangitis Caroli Disease Biliary Hamartomas

 CONGENITAL HEPATIC FIBROSIS

Key Facts
Terminology
• Part of a spectrum of congenital abnormalities
resulting in variable degrees of fibrosis & cystic
anomalies of liver & kidneys
Imaging Findings
• Best diagnostic clue: Combination of cystic dilatation
of bile ducts + renal collecting duct ectasia
• Always present in autosomal recessive polycystic
kidney disease (ARPKD)
• Severe renal disease & mild CHF/liver cysts
• Severe liver (CHF/liver cysts) & mild renal disease
Top Differential Diagnoses
• Isolated polycystic liver disease
• Primary sclerosing cholangitis
• Caroli disease
1
• Biliary hamartomas 9
Pathology
• Periportal fibrosis ~ portal HTN ~
hepatosplenomegaly ~ esophageal varices
• Sporadic or autosomal recessive inheritance pattern
• Embryological: Ductal plate malformation
• ARPKD: 100%; sometimes autosomal dominant PKD
Clinical Issues
• CHF is variable in its severity/age at
presentation/clinical manifestations
Diagnostic Checklist
• No further evaluation needed in a child with: Hepatic
ductal dilatation + enlarged portal tracts +
hepatic/renal cysts

• Associated polycystic disease of liver & kidney

o Multiple hypodense (water density) hepatic/renal
I DIFFERENTIAL DIAGNOSIS
cysts of varied size Isolated polycystic liver disease
o CECT: No enhancement (simple/complicated cysts) • May have autosomal dominant polycystic liver disease
MR Findings (ADPLD)without congenital hepatic fibrosis
• MR Cholangiography (MRC) Primary sclerosing cholangitis
o Dilated intrahepatic bile ducts & biliary cysts • Irregular strictures/dilation of intra-/extra-hepatic bile
• Associated polycystic disease liver/kidney ducts
o T1WI: Hypointense • Often leads to cirrhotic, dysmorphic liver
o T2WI: Hyperintense • Often associated with inflammatory bowel disease
o T1 C+: No enhancement
o Heavily T2WI: 1 Signal intensity due to pure fluid Caroli disease
content • Simple type: Cystic dilatation of bile ducts without
periportal fibrosis
Ultrasonographic Findings
• Periportal type
• Real Time o Ductal dilatation + cysts + periportal fibrosis
o Moderate to severe CHF o Indistinguishable from congential hepatic fibrosis
• Bile ducts: Moderate to severe dilatation • Best imaging clue on CECT
• Liver & spleen: Enlarged o Enhancing tiny dot (portal radicle) within dilated
• Splenic & portal veins: Dilated cystic intrahepatic ducts
• Portal tracts: Distinct 1 echogenicity
o Associated polycystic disease liver & kidney Biliary hamartomas
• Uncomplicated: Anechoic lesions/smooth • Rare benign/congenital malformation of bile ducts
borders/non -detectable walls • Location: Subcapsular or intra parenchymal
• Complicated: Septations/internal echoes/wall • Innumerable subcentimeter nodules in both lobes of
thickening liver
• Color Doppler • Diagnosis: Biopsy & histologic exam
o Depicts collaterals/direction & velocity of blood flow
in splenic/portal veins
• Direction: Hepatofugal or hepatopetal !PATHOlOGY
Imaging Recommendations General Features
• Best imaging tool: High-resolution CT or MRC • General path comments
• Protocol advice o Pathophysiology
o Helical CT: 5 mm collimated scans reconstructed • Periportal fibrosis ~ portal HTN ~
every 2.5 mm hepatosplenomegaly ~ esophageal varices
o MR: Heavily T2WI/MRC o Periportal fibrosis: Fetal type (more common); adult
type (rare)
• Genetics
o Sporadic or autosomal recessive inheritance pattern
o Probably linked to gene on chromosome 6p
• Etiology
 CONGENITAL HEPATIC FIBROSIS
1 o Embryological: Ductal plate malformation o Moderate to severe CHF
• Abnormal bile duct formation & resorption • Variceal sclerotherapy
10 • Epidemiology: Incidence varies due to various degrees • Porto systemic shunting
of expression • Liver transplantation
• Associated abnormalities o Hypersplenism: Splenectomy
o ARPKD:100%; sometimes autosomal dominant PKD
o Medullary sponge kidney: 80%
o Caroli disease; vaginal atresia; tuberous sclerosis I DIAGNOSTIC CHECKLIST
o Juvenile nephronophthisis
o Meckel-Gruber syndrome Consider
• High-resolution helical CT or MRC
Gross Pathologic & Surgical Features o To detect early stage of CHF
• Liver: Normal/enlarged/lobulated; dilated bile o To differentiate communicating/noncommunicating
ducts/cysts biliary abnormalities
• Hepatic fibrosis, polycystic disease (liver and kidney),
Microscopic Features
Caroli and biliary hamartomas can occur in isolation
• Periportal fibrosis; malformed, dilated, nonobstructive or in any combination
bile ducts
Image Interpretation Pearls
• No further evaluation needed in a child with: Hepatic
I CLINICAL ISSUES ductal dilatation + enlarged portal tracts +
hepatic/renal cysts
Presentation
• Most common signs/symptoms
o Mild CHF: Asymptomatic I SELECTED REFERENCES
o Moderate to severe CHF
1. Brancatelli G et al: Fibropolycystic liver disease: CT and MR
• Childhood type (more common); adult type (rare)
imaging findings. Scientific exhibit. Radiol. Soc. North
• Portal HTN/hepatosplenomegaly/bleeding varices America, Chicago, Illinois, 2003
• Clinical profile 2. Khan K et al: Morbidity from congenital hepatic fibrosis
o CHF is variable in its severity/age at after renal transplantation for autosomal recessive
presen tation/ clinical manifestations polycystic kidney disease. Am] Transplant. 2(4):360-5,
o Usually by adolescence have portal hypertension 2002
• Lab 3. Lonergan G] et al: Autosomal recessive polycystic kidney
o Normal liver function tests disease: radiologic-pathologic correlation. Radiographics.
20(3):837-55,2000
o ± Leukopenia/thrombocytopenia/anemia
4. Ernst 0 et al: Congenital hepatic fibrosis: findings at MR
• Due to hypersplenism cholangiopancreatography. A]R Am] Roentgenol.
• Diagnosis 170(2):409-12, 1998
o Hepatic/renal cysts (ARPKD) 5. Desmet V]: Ludwig symposium on biliary disorders--part I.
o ± Hepatosplenomegaly Pathogenesis of ductal plate abnormalities. Mayo Clin
o Normal liver function tests; liver biopsy Proc. 73(1):80-9, 1998
6. Kaplan BSet al: Variable expression of autosomal recessive
Demographics polycystic kidney disease and congenital hepatic fibrosis
• Age: Usually 5-13 years & rarely adult age group within a family. Am] Med Genet. 29(3):639-47,1988
• Gender: M = F 7. Premkumar A et al: The emergence of hepatic fibrosis and
portal hypertension in infants and children with
Natural History & Prognosis autosomal recessive polycystic kidney disease. Initial and
• Rare developmental malformation of ductal plate follow-up sonographic and radiographic findings. Pediatr
Radiol. 18(2):123-9, 1988
• CHF predominant features: ± intra-hepatic bile duct
8. Proesmans W et al: Association of bilateral renal dysplasia
(IHBD) + periportal fibrosis and congenital hepatic fibrosis. Int] Pediatr Nephrol.
o ± Ductal dilatation + periportal fibrosis 7(2):113-6, 1986
• Variable in its age of onset, severity & clinical
presentation
• Complications
o Biliary: Cholangitis/obstruction/sepsis
o Portal: Hypertension & bleeding varices
• Prognosis
o Early intervention
• Slow progress or arrest of disease
• Increased life expectancy & quality of life
o Those who survive childhood
• Relatively good prognosis & live into midlife
Treatment
• Options
o Mild CHF (asymptomatic): No treatment
 CONGENITAL HEPATIC FIBROSIS

I IMAGE GALLERY 1
11
Typical
(Left) Axial CECT shows
dysmorphic liver with
enlarged multiple hepatic
arteries and fibrotic
multicystic kidneys. (Right)
Axial CECT shows large
dysmorphic liver with
enlarged multiple hepatic
arteries. Kidneys were
scarred and had multiple
cysts.

Typical
(Left) Cholangiogram shows
fusiform and cystic dilatation
in intrahepatic bile ducts
(Caroli disease) along with
distortion and deviation due
to hepatic fibrosis. (Right)
Axial T2WI MR shows
innumerable biliary
hamartomas (arrows) and
large regenerative nodules
(open arrows). The liver is
large and dysmorphic.

Typical
(Left) Axial NECT shows
large dysmorphic liver in
patient with congenital
hepatic fibrosis with
intrahepatic "cysts" (arrows)
due to associated Caroli
disease. (Right) Axial T2WI
MR shows large left hepatic
lobe, tiny right lobe. Note
position of gallbladder
(arrow) and multiple renal
cysts (open arrow).
 AD POLYCYSTIC DISEASE, LIVER
1
12

Cross pathology photograph of hepatectomy specimen Coronal T2WI MR shows innumerable cysts of varying
shows numerous cysts replacing liver parenchyma. size, of high signal intensity, nearly completely replacing
Cysts range in size from microscopic to 5.0 cm in hepatic parenchyma. Multiple cysts within left kidney
greatest dimension, contain clear fluid. are also visualized.

ITERMINOLOGY Radiographic Findings

Abbreviations and Synonyms • ERCP
o No communication with biliary tree & cysts do not
• Autosomal dominant polycystic liver disease (ADPLD) opacify
or adult PLD
CT Findings
Definitions
• NECT
• Polycystic liver disease is a rare inherited disorder o Multiple to innumerable, homogeneous &
• Part of spectrum of fibropolycystic liver disease; hypoattenuating cystic lesions
constitutes group of related lesions of liver & biliary • Cyst contents often greater than water density due
tract caused by abnormal development of to hemorrhage (less commonly, infection)
embryological ductal plate • Calcification in cyst wall often seen: Due to old
hemorrhage
• CECT
IIMAGING FINDINGS o No wall or content enhancement
General Features o Cysts complicated by infection or hemorrhage may
have septations &/or internal debris, as well as
• Best diagnostic clue: Multiple cysts of varying size
enhancement of wall
• Location
o Cysts may contain fluid levels
o Extent of hepatic involvement ranges from limited
sporadic area of cystic disease to diffuse involvement MR Findings
of all lobes of liver • T1WI: Cysts have very low signal intensity
• ± Cysts in kidneys & in other organs • T2WI
• Size: Range from < 1 mm to > 12 em o Owing to their pure fluid content, homogeneous
• Key concepts high signal intensity is demonstrated on T2WI &
o Numerous large & small cysts coexist with fibrosis heavily T2WI
• Round-or oval shape, smooth thin wall, absence • Intracystic hemorrhage
of internal structures

DDx: Multiple Hepatic Cysts

~

Hepatic Cysts Biliary Hamartomas Caroli Disease Cystic Metastases

 AD POLYCYSTIC DISEASE, LIVER

Key Facts
Terminology
• Polycystic liver disease is a rare inherited disorder
Imaging Findings
• Size: Range from < 1 mm to > 12 cm
• Morphology:
• Multiple to innumerable, homogeneous &
hypoattenuating cystic lesions
• Cyst contents often greater than water density due to
hemorrhage (less commonly, infection)
• Calcification in cyst wall often seen: Due to old
hemorrhage
• MR Cholangiography (MRC)
• No communication with each other or biliary tree
1
Pathology 13
• Hepatobiliary & renal anomalies frequently coexist in
various combinations suggesting an expression of a
common underlying genetic abnormality
• Isolated PLD is distinct genetic disease, unlinked to
polycystic kidney disease (PKD) 1 & PKD 2
• Due to ductal plate malformation of small
intrahepatic bile ducts
• Ducts lose communication with biliary tree
Clinical Issues
• Often causes massive hepatomegaly
• Complications: Spontaneous intracystic hemorrhage,
rupture, infection

• T1 C+: Nonenhancing after administration of o When more than 10 cysts are seen, one of
gadolinium contrast material fibropolycystic disease should be considered
• MR Cholangiography (MRC)
o No communication with each other or biliary tree Biliary hamartomas
• Solitary or multiple (more common), well-defined
Ultrasonographic Findings nodules of varied density/subcapsular or
• Real Time intraparenchymal, scattered in both lobes of liver
o Anechoic masses, with smooth borders, thin-walled, o Typically measuring less than 1.S cm in diameter
no septations or mural nodularity • Varied enhancement based on cystic/solid
o Acoustic enhancement beyond each cyst may components of lesions
produce impression of an abnormal liver pattern in o Predominantly cystic (water density) lesions: No
addition to cysts enhancement
o Predominantly solid (fibrous stroma) lesions:
Nuclear Medicine Findings Enhance & become isodense with liver parenchyma
• Tc-99m DISIDA scintigraphy: Permits differential • MRC: Markedly hyperintense nodules, no
diagnosis between Caroli disease & PLD communication with biliary tree
o In Caroli disease: Areas of focally 1 radiotracer
accumulation that persist more than 120 minutes Caroli disease
o In PLD: Areas of focally I radio tracer accumulation • Congenital communicating cavernous ectasia of
with normal liver washout & biliary excretion biliary tract, autosomal recessive
• Multiple small rounded hypodense/hypointense
Imaging Recommendations saccular dilatation of intrahepatic bile ducts, multiple
• Best imaging tool: Although diagnosis is easily made intrahepatic calculi
with both CT & MR imaging, MR is more sensitive for • "Central dot" on CECT: Enhancing tiny dots (portal
detection of complicated cysts radicles) within dilated cystic intrahepatic ducts
• Protocol advice: Heavily weighted T2WI, MRC, T1 • MRC: Communicating bile duct abnormality
gadolinium-enhanced sequences
Cystic metastases
• Hypervascular metastases from neuroendocrine
I DIFFERENTIAL DIAGNOSIS tumors, sarcoma, melanoma, subtypes of lung & breast
carcinoma with necrosis & cystic degeneration
Hepatic (bile duct) cysts o CECT & MR show multiple lesions with strong
• Unilocular cyst lined by cuboidal, bile duct contrast-enhancement of peripheral viable &
epithelium, containing serous fluid irregularly defined tissue
o Wall is 1 mm or less in thickness (nearly • Cystic metastases with mucinous adenocarcinoma
imperceptible) (pancreatic or ovarian)
• Adjacent liver is normal o Cystic serosal implants on visceral peritoneal surface
• Multiple, round or ovoid, well-defined, of liver & parietal peritoneum of diaphragm
nonenhancing, water density lesions
• Homogeneous very low signal intensity on T1WI &
homogeneous very high signal intensity on T2WI
o Owing to their fluid content, an 1 in signal intensity
seen on heavily T2WI
• Usually solitary, but can number fewer than 10
 AD POLYCYSTIC DISEASE, LIVER
1 I PATHOLOGY
14
General Features
• General path comments: Hepatic cysts are
pathologically identical to simple or bile duct cysts
• Genetics
o Autosomal dominant
o Hepatobiliary & renal anomalies frequently coexist
in various combinations suggesting an expression of
a common underlying genetic abnormality
• Isolated PLD is distinct genetic disease, unlinked
to polycystic kidney disease (PKD) 1 & PKD 2
• Etiology
o Due to ductal plate malformation of small
intrahepatic bile ducts
• Ducts lose communication with biliary tree
• Epidemiology
o Incidence is difficult to determine because of various
degrees of expression; variable degrees of fibrosis &
cystic anomalies
o In patients with autosomal dominant PKD, there is
hepatic involvement in approximately 30-40%
• Approximately 70% of patients with PLD also
have PKD
• Associated abnormalities
o Biliary hamartomas
o Congenital hepatic fibrosis is part of spectrum of
hepatic cystic diseases
o Often coexists with PKD
Gross Pathologic & Surgical Features
• Presence of multiple cysts in liver may distort normal
liver architecture considerably
o Liver surrounding cysts frequently contains biliary
hamartomas & t fibrous tissue
Microscopic Features
• Cuboidal & flat monolayer epithelium with no
dysplasia in wall of cysts
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic; dull abdominal pain; abdominal
distention; dyspnea; cachexia
o Other signs/symptoms
• Often causes massive hepatomegaly
• Clinical profile
o Extrinsic compression of intrahepatic bile ducts
o Hepatic venous outflow obstruction: Mechanical
compression by cysts & associated formation of
thrombi in small hepatic vein tributaries
o Transudative ascites, portal hypertension due to
distortion of portal venules by cysts & fibrosis
o Lab data: ADPLD rarely affects liver function
Demographics
• Age: Adult manifestation
• Gender: Females have a significantly higher mean cyst
score than male patients
Natural History & Prognosis
• Liver gradually enlarges as it is replaced by cysts
• In advanced disease: Liver failure, or Budd-Chiari
syndrome
• Complications: Spontaneous intracystic hemorrhage,
rupture, infection
• Prognosis: Surgical intervention has significant
morbidity & inconsistent long term palliation
o Orthotopic liver transplantation has excellent long
term results, but substantial morbidity & mortality
Treatment
• Options, risks, complications
o Simple unroofing; cyst fenestration alone;
fenestration combined with resection
o Total hepatectomy & orthotopic liver
transplantation for patients with severe ADPLD
o Ultrasound-guided percutaneous aspiration;
multiple cyst punctures & alcohol sclerotherapy in
patients with high surgical risk
o Combined liver & kidney transplantation because of
renal cystic involvement with renal insufficiency
• One year survival rate: 89% with excellent
symptomatic relief & improved quality of life
I DIAGNOSTIC CHECKLIST
Consider
• Isolated ADPLD is underdiagnosed & genetically
distinct from PLD associated with ADPKD but with
similar pathogenesis, manifestations & management
• Clinical implications of & therapeutic strategies for
cystic focal liver lesions vary according to their causes
o Understanding of classic CT & MR appearances of
cystic focal liver lesions will allow more definitive
diagnosis & shorten diagnostic work-up
Image Interpretation Pearls
• MR findings that are important to recognize to
differentiate cystic lesions of liver are: Size of lesion;
presence & thickness of wall; presence of septa,
calcifications, or internal nodules; enhancement
pattern; MRC appearance; & signal intensity spectrum
I SELECTED REFERENCES
1. Qian Q et al: Clinical profile of autosomal dominant
polycystic liver disease. Hepatology. 37(1):164-71, 2003
2. Mortele KJet al: Cystic focal liver lesions in the adult:
differential CT and MR imaging features. Radiographies.
21(4):895-910,2001
3. Steinberg ML et al: MRI and CT features of polycystic liver
disease. N J Med. 90(5):398-400, 1993
4. Wan SKet al: Sonographic and computed tomographic
features of polycystic disease of the liver. Gastrointest
Radiol. 15(4):310-2, 1990
5. Wilcox DM et al: MR imaging of a hemorrhagic hepatic
cyst in a patient with polycystic liver disease. J Com put
Assist Tomogr. 9(1):183-5, 1985
6. Segal AJ et al: Computed tomography of adult polycystic
disease. J Comput Assist Tomogr. 6(4):777-80, 1982
 AD POLYCYSTIC DISEASE, LIVER
I IMAGE GALLERY 1
15
Typical
(Left) Axial NECT shows
innumerable, homogeneous
& hypoattenuating cystic
lesions with smooth
thin-walls & absence of
internal structures. Note
peripheral calcification in
cyst wall (arrows). (Right)
Axial CECT shows
innumerable cysts on portal
venous phase. These are
uncomplicated cysts with no
wall or content enhancement
& absence of internal
debris/septations. Pancreatic
cysts are also seen.

Typical
(Left) Ultrasound of RUQ
demonstrating anechoic,
multiple hepatic cysts, with
smooth borders, thin-walled,
no septations or mural
nodularity, in patient with
ADPLD. (Right) Axial CECT
through liver & kidneys.
Note lack of involvement of
kidneys in a patient with
isolated ADPLD.

Typical
(Left) Axial T2WI MR shows
hemorrhaghic cyst (arrow) in
patient with ADPLD being
less hyperintense than
noncomplicated cyst (open
arrow). (Right) Axial CECT
shows that liver is markedly
enlarged as it is replaced
with innumerable cysts. Note
also involvement of both
kidneys; PLD coexisting with
PKD.
 HEPATITIS
1
16

Cross section of explanted specimen shows shrunken, Axial CECT in a patient with cirrhosis due to chronic
nodular liver with bridging fibrosis due to chronic active viral hepatitis shows nodular contour of liver with right
hepatitis B. lobe atrophy and lateral segment hypertrophy.

• Responsible for 60% of cases of fulminant hepatic

ITERMINOLOGY failure in U.S.
Definitions o Alcoholic hepatitis: Acute & chronic
• Nonspecific inflammatory response of liver to various o Nonalcoholic steatohepatitis (NASH)
agents • Significant cause of acute & progressive liver
disease
• May be an underlying cause of cryptogenic
IIMAGING FINDINGS cirrhosis
o Imaging of viral/alcoholic hepatitis done to exclude
General Features • Obstructive biliary disease/neoplasm
• Best diagnostic clue • To evaluate parenchymal damage noninvasively
o Acute viral hepatitis on US
CT Findings
• "Starry-sky" appearance: 1 Echogenicity of portal
venous walls • NECT
o Acute viral hepatitis
• Hepatomegaly & periportal lucency (edema)
• Location: Diffusely; involving both lobes • Hepatomegaly, gallbladder wall thickening
• Periportal hypodensity (fluid/lymphedema)
• Size
o Chronic active hepatitis
o Acute: Enlarged liver
o Chronic: Decrease in size of liver • Lymphadenopathy in porta hepatis/gastrohepatic
ligament & retroperitoneum (in 65% of cases)
• Other general features
• Hyperdense regenerating nodules
o Leading cause of hepatitis is viral infect~on ..
o Acute alcoholic hepatitis
o In medical practice, hepatitis refers to vual mfectlOn
• Hepatomegaly
o Viral hepatitis .
• Diffuse hypodense liver (due to fatty infiltration)
• Infection of liver by small group of hepatotroplC
viruses • Fatty infiltration may be focal/lobar/segmental
o Chronic alcoholic hepatitis
• Stages: Acute, chronic active hepatitis (CAH) &
chronic persistent hepatitis • Mixture of steatosis & early cirrhotic changes
• Steatosis: Liver-spleen attenuation difference will
be less than 10 HU

DDx: Diffuse Hepatomegaly

'"
- . -
, .~ j

Steatosis Passive Congestion Diffuse Lymphoma Amyloidosis

 HEPATITIS
Key Facts
Imaging Findings
• "Starry-sky" appearance: 1 Echogenicity of portal
venous walls
• Hepatomegaly, gallbladder wall thickening
• Periportal hypodensity (fluid/lymphedema)
• Hyperdense regenerating nodules
• Increase in T1 & T2 relaxation times of liver
• Alcoholic steatohepatitis (diffuse fatty infiltration)
Top Differential Diagnoses
• Steatosis (fatty liver)
• Passive hepatic congestion
• Diffuse lymphoma
• Myeloproliferative & infiltrative disorders
Pathology
• Cellular dysfunction, necrosis, fibrosis, cirrhosis
• Normal liver has slightly 1 attenuation than
spleen
o Nonalcoholic steatohepatitis (NASH)
• Indistinguishable from alcoholic hepatitis
• CECT
o Acute & chronic viral hepatitis
• ± Heterogeneous parenchymal enhancement
o Chronic hepatitis: Regenerating nodules may be
isodense with liver
MR Findings
• Viral hepatitis
o Increase in T1 & T2 relaxation times of liver
o T2WI: High signal intensity bands paralleling portal
vessels (periportal edema)
• Alcoholic steatohepatitis (diffuse fatty infiltration)
o T1WI in-phase GRE image: Increased signal intensity
of liver than spleen or muscle
o T1WI out-of-phase GRE image: Decreased signal
intensity of liver (due to lipid in liver)
Ultrasonographic Findings
• Real Time
o Acute viral hepatitis
• 1 In liver & spleen size; I echogenicity of liver
• "Starry-sky" appearance: Increased echogenicity of
portal venous walls
• Periportal hypo-/anechoic area (hydropic swelling
of hepatocytes)
• Thickening of GB wall; hypertonic GB
o Chronic viral hepatitis
• Increased echogenicity of liver & coarsening of
parenchymal texture
• "Silhouetting" of portal vein walls (loss of
definition of portal veins)
• Adenopathy in hepatoduodenalligament
o Acute alcoholic hepatitis
• 1 Echogenicity (fatty infiltration), 1 size of liver
o Alcoholic steatohepatitis
• Liver parenchyma: Increased echogenicity &
sound attenuation
• Indistinguishable from liver fibrosis
o Late stage of alcoholic hepatitis
1
• HBV: Sensitized cytotoxic ~ T cells hepatocyte 17
necrosis ~ tissue damage
• Alcoholic hepatitis: Inflammatory reaction leads to
acute liver cell necrosis
Clinical Issues
• Malaise/ anorexia/fever/pain/hepatomegaly /jaundice
• Lab data: 1 Serologic markers; 1 liver function tests
• Age: Any age group (particularly teen-/middle-age)
• Self-limited; more progressive & chronic in nature
• Cirrhosis: 10% of HBV & 20-50% of HCV
• HCC: Particularly among carriers of HBsAg
Diagnostic Checklist
• Ruling out other causes of "diffuse hepatomegaly"
• Two most consistent findings in acute hepatitis:
Hepatomegaly & periportal edema
• Liver is atrophic & micronodular pattern
Imaging Recommendations
• Best imaging tool
o Helical NECT
o MR (in & out-of-phase GRE images)
• Alcoholic steatohepatitis
• Protocol advice: NECT and CECT, or MR with in and
out of phase GRE
I DIFFERENTIAL DIAGNOSIS
Steatosis (fatty liver)
• Diffuse decreased attenuation of enlarged liver
• T1WI out-of-phase GREI: Decreased signal of liver
• Normal vessels course through "lesion" (fatty
infiltration)
Passive hepatic congestion
• Diffuse hepatomegaly
• Early enhancement of dilated IVC & hepatic veins
• Doppler: Loss of triphasic pattern in IVC/hepatic veins
Diffuse lymphoma
• Hepatomegaly due to diffuse infiltration
• Large, lobulated low density discrete masses
• More common in immune-suppressed patients
• Examples: AIDS & organ transplant recipients
Myeloproliferative & infiltrative disorders
• Sickle cell: Diffuse hepatomegaly (due to congestion)
• Amyloidosis: Hepatomegaly & low attenuation areas
I PATHOLOGY
General Features
• General path comments
o Different stages of hepatitis
• Cellular dysfunction, necrosis, fibrosis, cirrhosis
o HBV: Sensitized cytotoxic ~ T cells hepatocyte
necrosis ~ tissue damage
 HEPATITIS
1 o Alcoholic hepatitis: Inflammatory reaction leads to • Malaise/ anorexia/ fever/pain/hepatomegaly / jaundice
acute liver cell necrosis o Acute HBV: May present with serum sickness-like
18 • Etiology syndrome
o Viral hepatitis: Caused by one of 5 viral agents • Clinical profile: Teenage or middle-aged patient with
• Hepatitis A (HAV), B (HBV), C (HCV) viruses history of fever, RUQ pain, hepatomegaly & jaundice
• Hepatitis D (HDV), E (HEV) viruses • Lab data: t Serologic markers; t liver function tests
o Other causes of hepatitis • Diagnosis: Based on
• Alcohol abuse o Serologic markers; virological; clinical findings
• Bacterial or fungal o Liver function tests & liver biopsy
• Autoimmune reactions; metabolic disturbances
• Drug induced injury; exposure to environmental Demographics
agents; radiation therapy • Age: Any age group (particularly teen-/middle-age)
• Epidemiology • Gender: M = F
o HBV (serum) Natural History & Prognosis
• In U.S. incidence is 13.2 cases/l00,000 population
• Hepatitis can be
• In U.S. & Europe, carrier rate is < 1%
o Self-limited; more progressive & chronic in nature
• In Africa & Asia, carrier rate is 10%
• Complications
• Endemic areas: HCC accounts 40% of all cancers
o Relapsing & fulminant hepatitis
Gross Pathologic & Surgical Features o Of chronic viral (HBY, HCV) & alcoholic hepatitis
• Acute viral hepatitis: Enlarged liver + tense capsule • Cirrhosis: 10% of HBV & 20-50% of HCV
• Chronic fulminant hepatitis: Atrophic liver • HCC: Particularly among carriers of HBsAg
• Alcoholic steatohepatitis: Enlarged, yellow, greasy liver • Prognosis
o Acute viral & alcoholic: Good
Microscopic Features o Chronic persistent hepatitis: Good
• Acute viral: Coagulative necrosis with t eosinophilia o Chronic active hepatitis (CAH): Not predictable
• Chronic viral: Lymphocytes/macrophages/plasma o Fulminant hepatitis: Poor
cells/piecemeal necrosis
• Alcoholic hepatitis: Neutrophils/necrosis/Mallory Treatment
bodies (alcoholic hyaline) • Acute viral hepatitis: No specific treatment;
prophylaxis-IG, HBIG, vaccine
Staging, Grading or Classification Criteria • Chronic viral hepatitis: Interferon for HBV & HCV
• Hepatitis A (HAV) • Alcoholic hepatitis: Alcohol cessation & good diet
o Virus: ssRNA
o Transmission: Fecal-oral
o Incubation period: 2-6 weeks I DIAGNOSTIC CHECKLIST
o No carrier & chronic phase
• Hepatitis B (HBV) Consider
o Virus: DNA • Ruling out other causes of "diffuse hepatomegaly"
o Transmission: Parenteral + sexual • Liver biopsy for diagnosis and staging
o Incubation period: 1-6 months
o Carrier & chronic phase present
Image Interpretation Pearls
• Hepatitis C (HCV) • Two most consistent findings in acute hepatitis:
o Virus: RNA Hepatomegaly & periportal edema
o Transmission: Blood transfusion
o Incubation period: 2-26 weeks
o Carrier & chronic phase present I SELECTED REFERENCES
• Hepatitis D (HDV) 1. Mortele KJ et al: Imaging of diffuse liver disease. Seminars
o Virus: RNA In Liver Disease 21, number 2: 195-212,2001
o Transmission: Parenteral + sexual 2. Okada Y et al: Lymph nodes in the hepatoduodenal
o Incubation period: I-several months ligament: US appearance with CT and MR correlation. Clin
Radiol. 51 (3): 160-6, 1996
o Carrier with HBV; chronic phase present 3. Murakami T et al: Liver necrosis and regeneration after
• Hepatitis E (HEV) fulminant hepatitis: pathologic correlation with CT and
o Virus: ssRNA MR findings. Radiology. 198(1):239-42, 1996
o Transmission: Water-borne 4. Kurtz AB et al: Ultrasound findings in hepatitis. Radiology.
o Incubation period: 6 weeks 136: 717-23, 1980
o No carrier & chronic phase

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute & chronic hepatitis
 HEPATITIS
I IMAGE GALLERY 1
19
Typical
(Left) Sagittal sonogram in a
patient with acute hepatitis
shows enlarged hypoechoic
liver with increased
echogenicity of portal
venous walls ("starry sky").
(Right) Axial CECT shows
marked gallbladder wall
edema (arrow) and ascites in
Morison pouch.

Typical
(Left) Axial CECT shows
heterogeneous enhancement
of the liver and ascites in a
patient with acute hepatitis.
(Right) Axial CECT shows
hepatosplenomegaly,
periportal lucency
(lymphedema) and
lymphadenopathy.

Typical
(Left) Sagittal sonogram in
patient with acute viral
hepatitis shows marked
thickening of gallbladder
wall (arrow). (Right) Axial
CECT in a patient with
chronic active viral hepatitis
shows marked gallbladder
wall edema and portacaval
enlarged lymph nodes
(curved arrow).
 HEPATIC CANDIDIASIS
1
20

Axial CECT in an immunocompromised patient shows Axial CECT shows numerous "microabscesses" with
numerous hypodense "microabscesses" proven to peripheral enhancement. Also ascites.
represent hepatic candidiasis.

ITERMINOlOGY CT Findings
• NECT
Definitions o Multiple small hypodense lesions
• A systemic fungal infection (Candida albicans) that o ± Periportal areas of increased attenuation (fibrosis)
often affects abdominal viscera o ± Scattered areas of calcific density (healing phase)
• CECT
o Nonenhancing hypodense areas
jlMAGING FINDINGS o ± Peripheral enhancement
General Features o Central or eccentric "dot" felt to represent hyphae
• Best diagnostic clue: Multiple well-defined, rounded MR Findings
microabscesses in liver • T1WI: Hypointense
• Location: Both lobes of liver • T2WI: Hyperintense
• Size: Less than 1 cm (micro abscesses) • STIR: Short T1 inversion recovery (STIR): Hyperintense
• Other general features • T1 C+: Nonenhancing hypointense lesions
o Most common fungal infection in • Contrast-enhanced FLASH (fast low-angle shot) images
immunocompromised patients o Detect more lesions
o More common in patients with
• Acquired immunodeficiency syndrome (AIDS) Ultrasonographic Findings
• Intensive chemotherapy • Real Time
• Acute leukemia (50-70%) recovering from o Four major patterns of hepatic Candidiasis are seen
profound neutropenia • "Wheel within a wheel": Peripheral zone
• Lymphoma (50%) at the time of autopsy surrounds inner echogenic wheel, in turn
• Chronic granulomatous disease of childhood surrounds a central hypoechoic nidus (early stage)
• Renal transplant • "Bull's eye": 1-4 mm lesion with a hyperechoic
o Chronic disseminated candidiasis center surrounded by a hypoechoic rim (seen
• Involvement of several organs when neutrophil count returns to normal)

DDx: Innumerable Hypodense liver lesions

I. , .
I : •..
. •.. '
... ~,~
..
'It."
. ~. t '~'.. ;~':.
~
'" ·'·-·IID.·~"
.,~
"
~
.

4 ;

, - . & \ ••
, - •••..".!.' ,..

CJQ~ Caroli Disease

Metastases Lymphoma/Leukemia Biliary Hamartomas
 HEPATIC CANDIDIASIS

Key Facts
1
Terminology Pathology 21
• A systemic fungal infection (Candida albicans) that • Candida albicans
often affects abdominal viscera • Originates from intestinal seeding of portal & venous
circulation
Imaging Findings
• Best diagnostic clue: Multiple well-defined, rounded Clinical Issues
microabscesses in liver • Asymptomatic or abdominal pain
• Location: Both lobes of liver • Erythematous papules on skin
• Size: Less than 1 cm (microabscesses) • Clinical profile: Immunocompromised patients
• Most common fungal infection in recovering from neutropenia (examples: Acute
immunocompromised patients leukemia, lymphoma, AIDS, chemotherapy & organ
transplant recipient)
Top Differential Diagnoses • Antifungal therapy (amphotericin B & fluconazole)
• Metastases
• Lymphomatous/leukemic foci in liver Diagnostic Checklist
• Biliary hamartomas • Rule out other "innumerable hypodense liver lesions"
• Caroli disease • Biopsy & send specimen for histology/microbiology

• "Uniformly hypoechoic": Most common o Simple: Cystic dilatation of bile ducts without
appearance (due to fibrosis & debris) periportal fibrosis
• "Echogenic": Caused by scar formation o Periportal: Ductal dilatation, cysts & periportal
o After antifungal therapy: Lesions fibrosis
• Increase in echogenicity
• Decrease in size or often disappear altogether
Nuclear Medicine Findings
!PATHOLOGY
• Candida microabscesses General Features
o Technetium sulfur colloid • Etiology
• Cold lesions (due to decreased uptake) o Candida albicans
o Gallium scan • Most common cause of Candidiasis
• Cold lesions (due to diminished uptake) o Candida tropicalis
Imaging Recommendations • Accounts for 1/3 of deep candidiasis cases
• Usually in tropical countries
• Best imaging tool: Helical CT or MR
o Originates from intestinal seeding of portal &
• Protocol advice venous circulation
oCT: Thin sections (::s; 5 mm)
• Epidemiology: More commonly seen in areas with
o MR: FLASHsequences
endemic AIDS
o Both pre-contrast & post-<;ontrast studies
• Associated abnormalities
o Acquired immunodeficiency syndrome
o Underlying malignancy
I DIFFERENTIAL DIAGNOSIS o Leukemia
Metastases o Lymphoma
o Neutropenia due to other causes
• Less numerous, larger & usually do not affect spleen
• Epithelial metastases: Rim-enhancement • Chemotherapy
• Can be cystic or calcified • Post radiation therapy
• Organ transplant
lymphomatous/leukemic foci in liver
Gross Pathologic & Surgical Features
• Less well-defined; less numerous; larger
• Usually foci can also be seen in spleen • Multiple microabscesses of liver

Biliary hamartomas Microscopic Features

• Rare benign congenital malformation of bile ducts • Simple media: Oval, budding cells
• Location: Intraparenchymal or subcapsular • Special culture
o Hyphae
• Innumerable subcentimeter nodules in both lobes
o Elongated branching called pseudohyphae
• Diagnosis: Biopsy & histologic exam
• In serum
Caroli disease o Germ tubes
• Best imaging clue on CECT o Thick-walled spores called chlamydospores
o "Central dot" sign: Enhancing tiny dot (portal
radicle) within dilated cystic intrahepatic ducts
• Two types: Simple & periportal
 HEPATIC CANDIDIASIS
1 I CLINICAL ISSUES Comparison of short-inversion-time inversion-recovery,
T1-weighted spin-echo, and dynamic
22 gadolinium-enhanced MR imaging. Radiology. 191:539-43,
Presentation 1994
• Most common signs/symptoms 5. Meunier F: Candidiasis. Eur J Clin Microbiol Infect Dis.
o Asymptomatic or abdominal pain 8(5):438-47, 1989
o Fever 6. Pastakia B et al: Hepatosplenic candidiasis: Wheels within
o Erythematous papules on skin wheels. Radiology. 166:417-21, 1988
• Acute candidemia (neutropenic patients) 7. Cunha BA:Systemic infections affecting the liver. Some
cause jaundice, some do not. Postgrad Med. 84(5):148-58,
o Rarely hepatomegaly
161-3, 166-8, 1988
• Clinical profile: Immunocompromised patients 8. Maxwell AJ et al: Fungal liver abscesses in acute
recovering from neutropenia (examples: Acute leukaemia--a report of two cases. Clin Radiol.
leukemia, lymphoma, AIDS, chemotherapy & organ 39(2):197-201, 1988
transplant recipient) 9. Thaler M et al: Hepatic candidiasis in cancer patients: the
• Fever in neutropenic patients whose WBC count is evolving picture of the syndrome. Ann Intern Med.
returning to normal 108(1):88-100, 1988
10. Tashjian LS et al: Focal hepatic candidiasis: a distinct
• Lab data: 1 Alkaline phosphatase
clinical variant of candidiasis in immunocompromised
• Diagnosis: By histologic section of biopsy specimens patients. Rev Infect Dis. 6(5):689-703, 1984
o Pseudohyphae in central necrotic portion of lesion
o Difficult on clinical grounds because blood cultures
are positive in only 50% of affected patients
Demographics
• Age: Any age group
• Gender: M = F
Natural History & Prognosis
• Systemic fungal infection
• Origin: Intestinal seeding
• Liver lesions via portal & venous circulation
• Affected individuals; particularly
immunocompromised patients
• Complications (rare)
o Rupture of microabscesses
o Cholangitis due to candidiasis of biliary tract
• Prognosis
o Usually good with prompt diagnosis & treatment
Treatment
• Liver microabscesses
o Antifungal therapy (amphotericin B & fluconazole)
o Very rarely surgical or percutaneous drainage

I DIAGNOSTIC CHECKLIST
Consider
• Rule out other "innumerable hypodense liver lesions"
• Biopsy & send specimen for histology/microbiology
Image Interpretation Pearls
• Both pre- & post-contrast studies
o CT & MR (FLASH) sequences show
• Multiple small, rounded lesions

I SELECTED REFERENCES
1. Wig JD et al: Cholangitis due to candidiasis of the
extra-hepatic biliary tract. HPB Surg. 11(1):51-4, 1998
2. Semelka RC et al: Hepatosplenic fungal disease: Diagnostic
accuracy and spectrum of appearances on MR imaging. AJR
169:1311-6, 1997
3. Giamarellou H et al: Epidemiology, diagnosis, and therapy
of fungal infections in surgery. Infect Control Hosp
Epidemiol. 17(8):558-64, 1996
4. Lamminen AE et al: Infectious liver foci in leukemia:
 HEPATIC CANDIDIASIS
I IMAGE GALLERY 1
23
Typical
(Left) Axial CECT in patient
with AIDS shows
innumerable hypodense
"microabscesses" from
hepatic Candidiasis. (Right)
Axial CECT shows
innumerable hypodense
"microabscesses" scattered
throughout the liver.

Typical
(Left) Axial CECT shows
small hypodense lesions
within the hepatic dome,
some which demonstrate
peripheral enhancement.
(Right) Axial CECT
demonstrating some of the
small hypodense
"microabscesses" having an
eccentric "dot" representing
hyphae.

Typical
(Left) Axial CECT in patient
with AIDS shows diffuse
microabscesses in liver and
spleen. (Right) Axial US in
immunocompromised
patient shows multiple, small
hypoechoic masses (arrow)
from hepatic Candida lesions
some with bull's eye
appearance.
 HEPATIC PYOGENIC ABSCESS
1
24

Graphic shows peripheral multiloculated collections of Axial CECT shows multiple coalescing cystic lesions with
pus with surrounding inflamed liver. enhancing septa. Pyogenic abscess resulted from
diverticulitis

• Pyogenic accounts: 88% (bacterial)

ITERMINOLOGY • Amebic: 10% (Entamoeba histolytica)
Abbreviations and Synonyms • Fungal: 2% (Candida albicans)
o Most common causes of pyogenic abscess
• Liver pyogenic abscess
• Diverticulitis
Definitions • Ascending cholangitis
• Localized collection of pus in liver due to bacterial • Infection of infarcted tissue (e.g., post liver
infectious process with destruction of hepatic transplantation, necrotic tumor)
parenchyma & stroma o Pyogenic abscesses may be single or multiple
• Biliary tract origin: Multiple small abscesses
• Portal origin: Usually solitary larger abscess
I IMAGING FINDINGS • Direct extension & trauma: Solitary large abscess
o Developing countries: Liver abscesses
General Features • Mostly due to parasitic infections
• Best diagnostic clue: "Cluster" sign - cluster of small • Amebic, echinococcal or other
pyogenic abscesses coalesce into a single large cavity protozoal/helminthic
• Location
o Varies based on origin Radiographic Findings
• Portal origin: Right lobe (65%); left lobe (12%); • Radiography
both lobes (23%) o Chest x-ray
• Biliary tract origin: 90% involve both lobes • Elevation of right hemidiaphragm
• Size: Varies from few millimeters to 10 centimeters • Right lower lobe atelectasis
• Other General Features • Infiltrative lesions, right pleural effusion
o Western countries: Liver abscess o Plain x-ray abdomen
• Usually pyogenic (bacterial in origin) • Hepatomegaly, intrahepatic gas, air-fluid level
• Typically due to complication of infection o Contrast studies of gut & urinary tract: May show
elsewhere cause of abscess
o Among all liver abscesses • Diverticulitis, perforated ulcer & renal abscess
• ERCP

DDx: Cystic Liver lesion with/without Gas

p:"<.~ ~:
Il
0
~

Cystic Metastases
'\
••••••
Liver Infarction
fi. II

(OLT) Hydatid Cyst

 HEPATIC PYOGENIC ABSCESS

Key Facts
1
Terminology • Infarction in liver transplant (OLT) 25
• Localized collection of pus in liver due to bacterial • Hepatic hydatid cyst
infectious process with destruction of hepatic • Biliary cystadenocarcinoma
parenchyma & stroma Pathology
Imaging Findings • Pyogenic: Accounts 88% of all liver abscesses
• Best diagnostic clue: "Cluster" sign - cluster of small • E. coli (adults) & S. aureus (children)
pyogenic abscesses coalesce into a single large cavity • Diverticulitis, appendicitis
• Portal origin: Right lobe (65%); left lobe (12%); both Clinical Issues
lobes (23%) • Fever, RUQ & usually left lower quadrant pain
• Biliary tract origin: 90% involve both lobes
• Pyogenic abscesses may be single or multiple Diagnostic Checklist
• Right lower lobe atelectasis • Rule out: Amebic/fungal liver abscesses; cystic tumors
• Check for history of transplantation or
Top Differential Diagnoses
ablation/chemotherapy for liver tumor
• Metastases (especially after treatment)
• Hepatic amebic abscess

o Accurately define level & cause of biliary obstruction o Variable in shape & echogenicity
o Usually spherical or ovoid in shape
CT Findings o Wall: Irregular hypoechoic/mildly echogenic
• NECT o Echogenicity of abscesses
o Simple pyogenic abscess • Anechoic (50%), hyperechoic (25%), hypoechoic
• Well-defined, round, hypodense mass (0-45 HU) (25%)
o "Cluster" sign o ± Septa or fluid level within abscess
• Small abscesses aggregate to coalesce into a single o ± Debris & posterior enhancement
big cavity, usually septated o Early lesions tend to be echogenic & poorly
o Complex pyogenic abscess: "Target" lesion demarcated
• Hypodense rim o May evolve into well-demarcated, nearly anechoic
• Isodense periphery lesions
• Decreased HU in center o Gas in an abscess seen as brightly echogenic foci
o Specific sign: Abscess with central gas with posterior artefacts
• Seen as air bubbles or an air-fluid level
• Present in less than 20% of cases Nuclear Medicine Findings
o Large air-fluid or fluid-debris level • Hepato biliary & sulfur colloid scans
• Often associated with gut communication or o Rounded, cold areas
necrotic tissue o Occasionally, communication between abscess
• CECT cavity & biliary system can be seen
o Sharply-defined, round, hypodense mass • Gallium scan (Gallium citrate Ga 67)
o Rim- or capsule- and septal-enhancement o Hot lesions
o Right lower lobe atelectasis & pleural effusion o Mixed lesion: Cold center & hot rim
o Non-liquified infection may simulate hypervascular • WBC Scan
tumor o Hot lesions (due to WBC accumulation)
o Highly specific for pyogenic abscesses compared to
MR Findings any nuclear or cross-sectional imaging
• T1WI: Hypointense
• T2WI Imaging Recommendations
o Hyperintense mass • Best imaging tool: CECT
o High signal intensity perilesional edema • Image guided aspiration
• T1 C+
o Hypointense mass
o Rim or capsule enhancement I DIFFERENTIAL DIAGNOSIS
o Small abscesses less than 1 cm
• May show homogeneous enhancement Metastases (especially after treatment)
• Mimicking hemangiomas • Usually do not appear as a cluster or septated cystic
• MRCP mass
o Highly specific in detecting • Usually no elevation of diaphragm or atelectasis
• Obstructive biliary pathology • No fever or i WBC with metastases
• Leading cause of cholangitis ~ pyogenic abscess • Treated necrotic metastases may be indistinguishable
from abscess
Ultrasonographic Findings
• Real Time
 HEPATIC PYOGENIC ABSCESS
1 Hepatic amebic abscess
26 • Compared to pyogenic: Amebic abscesses are
o Usually peripheral, round or oval shape
o Sharply-defined hypoechoic or low attenuation
• Most often solitary (85%)
• Affects right lobe more often (72%) than left lobe
(13%)
• Abuts liver capsule
o US shows homogeneous echoes + distal
enhancement
• More common in recent immigrants, institutionalized,
homosexuals
• Dark, reddish-brown, consistency of anchovy paste
Infarction in liver transplant (OlT)
• Hepatic artery thrombosis (HAT) ~ hepatic and biliary
necrosis
• Indistinguishable from pyogenic abscess
Hepatic hydatid cyst
• Large cystic liver mass + peripheral daughter cysts
• ± Curvilinear or ring-like peri cyst calcification
• ± Dilated intrahepatic bile ducts: Due to mass effect
and/or rupture into bile ducts
Biliary cystadenocarcinoma
• Rare, multiseptated, water density cystic mass
• No surrounding "inflammatory changes"
[PATHOLOGY
General Features
• General path comments
o Pyogenic abscess can develop via five major routes
o Biliary: Ascending cholangitis from
• Choledocholithiasis
• Benign or malignant biliary obstruction
o Portal vein: Pylephlebitis from
• Appendicitis, diverticulitis
• Proctitis, inflammatory bowel disease
• Right colon infection spreads via: Superior
mesenteric vein ~ portal vein ~ liver
• Left colon infection via: Inferior mesenteric vein
~ splenic vein ~ portal vein ~ liver
o Hepatic artery: Septicemia from bacterial
endocarditis, pneumonitis, osteomyelitis
o Direct extension
• Perforated gastric or duodenal ulcer
• Subphrenic abscess, pyelonephritis
o Traumatic: Blunt or penetrating injuries
• Etiology
o Pyogenic: Accounts 88% of all liver abscesses
o Most common bacterial organisms
• E. coli (adults) & S. aureus (children)
• Epidemiology: Incidence rate is increasing in Western
countries due to ascending cholangitis & diverticulitis
• Associated abnormalities
o Diverticulitis, appendicitis
o Benign or malignant biliary obstruction
o Perforated gastric or duodenal ulcer
o Bacterial endocarditis, pneumonitis, osteomyelitis
Gross Pathologic & Surgical Features
• Pyogenic abscess: Multiple or solitary lesions
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Fever, RUQ pain, rigors, malaise
o Nausea, vomiting, weight loss, tender hepatomegaly
o If subphrenic then atelectasis and pleural effusion
possible
• Clinical profile
o Middle-aged/elderly patient with history of
• Fever, RUQ & usually left lower quadrant pain
• Tender hepatomegaly & increased WBC count
• Lab data
o Increased leukocytes & serum alk phosphatase
• Diagnosis: Fine needle aspiration cytology (FNAC)
Natural History & Prognosis
• Complications
o Spread of infection to subphrenic space
• Causes atelectasis & pleural effusion
• Prognosis
o Good after medical therapy & aspiration
o Catheter drainage failure rate 8.4%
o Recurrent abscess rate 8%
Treatment
• Antibiotics
• Percutaneous aspiration + parenteral antibiotics
• Percutaneous catheter drainage
• Surgical drainage
I DIAGNOSTIC CHECKLIST
Consider
• Rule out: Amebic/fungal liver abscesses; cystic tumors
o Amebic: Entamoeba histolytica
o Fungal: Candida albicans
o Hepatic hydatid or simple cyst, biliary cystadenoma
• Check for history of transplantation or
ablation/chemotherapy for liver tumor
Image Interpretation Pearls
• "Cluster" sign: Small abscesses coalesce into big cavity
• Specific sign: Presence of central gas or fluid level
• Elevation of right hemidiaphragm
• Right lower lobe atelectasis & pleural effusion
• Non-liquified abscess may simulate solid tumor
[SELECTED REFERENCES
1. Giorgio A et al: Pyogenic liver abscesses: 13 years of
experience in percutaneous needle aspiration with US
guidance. Radiology. 195: 122-4, 1995
2. Mendez RZ et al: Hepatic abscesses: MR imaging findings.
Radiology. 190: 431-6, 1994
3. Jeffrey RB et al: CT small pyogenic hepatic abscesses: The
cluster sign. AJR. 151(3): 487-9, 1988
 HEPATIC PYOGENIC ABSCESS
I IMAGE GALLERY 1
27

(Left) Axial CECT shows

cluster of small abscesses
coalescing into large
septated mass. Note pleural
effusion. (Source,
cholangitis). (Right) Axial
CECT shows cluster of
peripheral hypodense
abscesses, some containing
gas (source, diverticulitis).

Typical
(Left) Axial CECT shows
cluster of abscesses in both
lobes of the liver in a patient
with prior history of
diverticulitis. (Right) Axial
CECT shows almost
complete resolution of
multiple bilobar pyogenic
abscesses following
antibacterial treatment.

Variant
(Left) Axial CECT shows
shows early, non-liquified
pyogenic abscess (arrow) in
a patient with diverticulitis.
Mass resembles a
hypervascular tumor. (Right)
Axial CECT shows multiple
abscesses with gas-fluid
levels following Whipple
procedure for pancreatic
carcinoma. Catheter
drainage (curved arrow) was
therapeutically effective.
 HEPATIC AMEBIC ABSCESS
1
28

Graphic shows unilocular encapsulated mass with Axial CECT shows homogeneous hypodense
"anchovy paste" contents. nonenhancing mass with thick capsule or wall.

• Human carriers who pass amebic cysts into stool

ITERMINOLOGY o May become secondarily infected with pyogenic
Definitions bacteria
• Localized collection of pus in liver due to entamoeba Radiographic Findings
histolytica with destruction of hepatic parenchyma & • Radiography
stroma o Elevation of right hemidiaphragm
o Right lower lobe atelectasis or infiltrate
o Right pleural effusion
IIMAGING FINDINGS o Ruptured amebic abscess into chest may show
• Lung abscess, cavity, hydropneumothorax
General Features
• Pericardial effusion
• Best diagnostic clue: Peripherally located, o Barium enema often shows changes of amebic colitis
sharply-defined, round, hypodense mass with
enhancing capsule CT Findings
• Location • NECT: Peripheral, round or oval hypodense mass
o Right lobe: 72% (10-20 HU)
o Left lobe: 13% • CECT
o Usually peripheral o Lesions may appear unilocular or multilocular
• Size: Varies from few millimeters to several centimeters o May demonstrate nodularity of margins
• Other General Features o Show rim- or capsule-enhancement
o Most common extraintestinal manifestation of o Extrahepatic abnormalities
amebic infestation • Right lower lobe atelectasis
o Most common in developing countries • Right pleural effusion
o Western nations: High risk groups are • Usually colonic & rarely gastric changes
• Recent immigrants, institutionalized &
homosexuals MR Findings.
o Most often solitary (85%) • Tl WI: Hypointense abscess
o Primary source of infection • T2WI

DDx: Complex Cystic Mass

Treated Metastases Pyogenic Abscess Post Transplant HAT Hydatid Cyst

 HEPATIC' AMEBIC ABSCESS

Key Facts
1
Terminology • Hepatic hydatid cyst 29
• Localized collection of pus in liver due to entamoeba • Biliary cystadenocarcinoma
histolytica with destruction of hepatic parenchyma & Pathology
stroma • Entamoeba histolytica
Imaging Findings Clinical Issues
• Best diagnostic clue: Peripherally located, • Clinical profile: Patient with history of diarrhea
sharply-defined, round, hypodense mass with (mucus), RUQ pain & tender hepatomegaly
enhancing capsule • Indirect hemagglutination positive in 90% cases
• Most often solitary (85%)
• Right lower lobe atelectasis or infiltrate Diagnostic Checklist
• Right pleural effusion • Rule out other liver pathologies: Pyogenic or fungal
abscess & cystic lesions, which may simulate amebic
Top Differential Diagnoses
abscess on imaging
• Treated (cystic or necrotic) metastases • Check for history of transplantation & ablation or
• Hepatic pyogenic abscess chemotherapy for liver tumor or metastasis
• Infarcted liver after transplantation

o Hyperintense abscess • Specific sign: Abscess with central gas

o Perilesional edema: High signal intensity o Air bubbles or an air-fluid level
• T1 C+
o Abscess contents: No enhancement Infarcted liver after transplantation
o Rim or capsule: Shows enhancement • Hepatic artery thrombosis (HAT) causes biliary &
hepatic necrosis
Ultrasonographic Findings • Can look exactly like an abscess with or without gas
• Real Time
o Usually round or oval, sharply-defined hypoechoic Hepatic hydatid cyst
mass • Large well-defined cystic liver mass
o Abuts liver capsule with homogeneous echoes & • Numerous peripheral daughter cysts
distal enhancement • May show curvilinear or ring-like pericyst calcification
o Compared to pyogenic • Intrahepatic duct dilatation may be seen
• Amebic is more likely to have a round or oval Biliary cystadenocarcinoma
shape (82:60%)
• Rare, multi septated, water density cystic mass
• Hypoechoic with fine internal echoes (58:36%)
• No surrounding inflammatory changes
Nuclear Medicine Findings
• Hepatobiliary scan (HIDA)
o Cold lesion with a hot periphery I PATHOLOGY
• Technetium sulfur colloid
General Features
o Cold defects
• General path comments
• WBC Scan
o Cold center & hot rim o Cystic form of E. histolytica gains access to body via
contaminated water
Imaging Recommendations o Mature cysts resistant to gastric acid, pass
• Best imaging tool: CECT unchanged into intestine
• Protocol advice: Scan to include lung bases through o Cyst wall is digested by trypsin & invasive
pelvis trophozoites are released
o Trophozoites enter mesenteric venules & lymphatics
o Usually spread from colon to liver
I DIFFERENTIAL DIAGNOSIS • Via portal vein (most common) & lymphatics
o Rarely direct spread
Treated (cystic or necrotic) metastases • Colonic wall to peritoneum
• May be indistinguishable from amebic abscess • Peritoneum to liver capsule & finally liver
• Usually no elevation of diaphragm or atelectasis • Etiology
• No fever or increased WBC o Entamoeba histolytica
o May become secondarily infected with pyogenic
Hepatic pyogenic abscess bacteria
• Simple pyogenic abscess • Epidemiology: Approximately 10% of world's
o Well-defined round, hypodense mass (0-45 HU) population is infected with E. histolytica
o "Cluster" sign • Associated abnormalities: Amebic colitis
• Aggregation of small abscesses, sometimes
coalesce into a single septated cavity
 HEPATIC AMEBIC ABSCESS
1 Gross Pathologic & Surgical Features
30 • Usually solitary abscess
• Predominantly in right lobe
• Fluid-dark, reddish-brown
• Consistency of "anchovy paste"
Microscopic Features
• Blood, destroyed hepatocytes
• Necrotic tissue & rarely trophozoites
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o RUQ pain, tender hepatomegaly
o Diarrhea with mucus
• Clinical profile: Patient with history of diarrhea
(mucus), RUQ pain & tender hepatomegaly
• Lab data
o Stool exam: Usually nonspecific or negative
o Indirect hemagglutination positive in 90% cases
Demographics
• Age
o More common in 3rd-Sth decade
o Can occur in any age group
• Gender: M:F = 4:1
Natural History & Prognosis
• Complications
o Pleuropulmonary amebiasis (20-3S%)
• Pulmonary consolidation or abscess
• Effusion, empyema or hepatobronchial fistula
o Peritoneal amebiasis (2-7.S%)
o Pericardial or renal amebiasis
• Prognosis
o Usually good after amebicidal therapy
o Poor in individuals who develop complications
o Mortality rate in US: < 3%
• < 1% when confined to liver
• 6% with extension into chest
• 30% with extension into pericardium
Treatment
• 90% respond to antimicrobial therapy
o Metronidazole or chloroquine
• 10% require aspiration & drainage
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other liver pathologies: Pyogenic or fungal
abscess & cystic lesions, which may simulate amebic
abscess on imaging
• Check for history of transplantation & ablation or
chemotherapy for liver tumor or metastasis
Image Interpretation Pearls
• On CT: Peripheral, round or oval hypodense mass
with rim or capsule enhancement
• On US: Abuts liver capsule with homogeneous echoes
& distal enhancement
I SELECTED REFERENCES
1. Ralls PW: Inflammatory disease of the liver. Clin Liver Dis.
6(1):203-25, 2002
2. Bald NC et al: MR imaging of infective liver lesions. Magn
Reson Imaging Clin N Am. 10(1):121-35, vii, 2002
3. Sharma MP et al: Management of amebic and pyogenic
liver abscess. Indian] Gastroenterol. 20 Suppl1:C33-6,
2001
4. Hughes MA et al: Amebic liver abscess. Infect Dis Clin
North Am. 14(3):565-82, viii, 2000
5. Natarajan A et al: Ruptured liver abscess with fulminant
amoebic colitis: case report with review. Trop
Gastroenterol. 21(4):201-3, 2000
6. Das P et al: Molecular mechanisms of pathogenesis in
amebiasis. Indian] Gastroenterol. 18(4):161-6, 1999
7. Rajak CL et al: Percutaneous treatment of liver abscesses:
needle aspiration versus catheter drainage. A]R Am]
Roentgenol. 170(4):1035-9,1998
8. Ralls PW: Focal inflammatory disease of the liver. Radiol
Clin North Am. 36(2):377-89, 1998
9. Kimura K et al: Amebiasis: modem diagnostic imaging with
pathological and clinical correlation. Semin Roentgenol.
32(4):250-75, 1997
10. Fujihara T et al: Amebic liver abscess.] Gastroenterol.
31(5):659-63,1996
11. Takhtani D et al: Intrapericardial rupture of amebic liver
abscess managed with percutaneous drainage of liver
abscess alone. Am] Gastroenterol. 91(7):1460-2,1996
12. Giorgio A et al: Pyogenic liver abscesses: 13 years of
experience in percutaneous needle aspiration with US
guidance. Radiology. 195: 122-124, 1995
13. Mendez RZ et al: Hepatic abscesses: MR imaging
findings. Radiology. 190: 431-436, 1994
14. Van Allan R] et al: Uncomplicated amebic liver abscess:
prospective evaluation of percutaneous therapeutic
aspiration. Radiology. 183(3):827-30, 1992
15. Gibney E]: Amoebic liver abscess. Br] Surg. 77(8):843-4,
1990
16. Ken]G et al: Perforated amebic liver abscesses: successful
percutaneous treatment Radiology. 170: 195-197, 1989
17. Sarda AK et al: Intraperitoneal rupture of amoebic liver
abscess. Br] Surg. 76(2):202-3, 1989
18. Singh]P et al: A comparative evaluation of percutaneous
catheter drainage for resistant amebic liver abscesses. Am]
Surg. 158(1):58-62, 1989
19. Ken]G et al: Perforated amebic liver abscesses: successful
percutaneous treatment. Radiology. 170(1 Pt 1):195-7,
1989
20. Rustgi AK et al: Pyogenic and amebic liver abscess. Med
Clin North Am. 73(4):847-58, 1989
21. Frey CF et al: Liver abscesses. Surg Clin North Am.
69(2):259-71, 1989
22. Jeffrey RB et al: CT small pyogenic hepatic abscesses: The
cluster sign. A]R. 151(3): 487-9,·1988
23. Greenstein A] et al: Pyogenic and amebic abscesses of the
liver. Semin Liver Dis. 8(3):210-7, 1988
24. G Elizondo et al: Amebic liver abscess: diagnosis and
treatment evaluation with MR imaging Radiology. 165:
795-800, 1987
25. Ralls PW et al: Amebic liver abscess: MR imaging
Radiology. 165: 801-804, 1987
 HEPATIC AMEBIC ABSCESS

I IMAGE GALLERY
1
31

(Left) Axial CECT shows

typical peripheral hypodense
mass abutting hepatic
capsule. Elevation of
hemidiaphragm, atelectasis
and pleural effusion. (Right)
Sagittal sonogram shows
hypoechoic mass with fine
internal echoes and posterior
acoustic enhancement.

Variant
(Left) Axial CECT in an Asian
immigrant shows unusually
large amebic abscess of the
liver. Note shaggy wall and
no prominent septations.
(Right) Axial NEeT shows
unusually small isolated
amebic abscess.

Other
(Left) Sagittal US shows
complex mass with thick
capsule and coarse internal
echoes in a patient with
amebiasis. (Right) Sagittal US
guided fine needle aspiration
yielded reddish-brown thick
fluid. Microbiological results
were positive for E.
histolytica.
 HEPATIC HYDATID CYST
1
32

Graphic shows eccentric cystic mass with numerous Axial CECT shows oblong hypodense cystic mass with
peripheral daughter cysts. peripheral "daughter" cysts.

• Liver & lungs

ITERMINOLOGY
Radiographic Findings
Abbreviations and Synonyms
• Radiography
• Echinococcal or hydatid disease; echinococcosis o E. granulosus
Definitions • Curvilinear or ring-like pericyst calcification
• Infection of humans caused by larval stage of • Seen in 20-30% of abdominal plain films
Echinococcus granulosus or multilocularis o E. multilocularis (alveolaris)
• Microcalcifications in 50% of cases
• ERCP
I IMAGING FINDINGS o Hydatid cyst may communicate with biliary tree
• Right hepatic duct (55%); left hepatic duct (29%)
General Features • Common hepatic duct (9%)
• Best diagnostic clue: Large well-defined cystic liver • Gallbladder (6%) & common bile duct (1%)
mass with numerous peripheral daughter cysts
CT Findings
• Location: Right lobe more than left lobe of liver
• Size • NECT
oVaries o E. granulosus
• Average size: 5 cm • Large unilocular/multilocular well-defined
hypo dense cysts
• Maximum size: Up to 50 cm
• May contain up to 15 liters of fluid • Contains multiple peripheral daughter cysts of less
density than mother cyst
• Key concepts
o E. granulosus: Most common form of hydatid • Curvilinear ring-like calcification
disease • Calcified wall: Usually indicates no active
infection if completely circumferential
• Up to 60% of cysts are multiple
o E. multilocularis (alveolaris): Less common but • Dilated intrahepatic bile duct (IHBD): Due to
aggressive form compression/rupture of a cyst into bile ducts
o Most common sites for hydatid cyst o Dilated ducts within vicinity of a cyst
o E. multilocularis (alveolaris)

DDx: Complex or Septated Cystic Hepatic Mass

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1/ '~ .•

.I"·" :.
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, !il@.~
Cystadenocarcinoma Pyogenic Abscess Cystic Metastases Hemorrhagic Cyst
 HEPATIC HYDATID CYST
Key Facts
Terminology
• Echinococcal or hydatid disease; echinococcosis
• Infection of humans caused by larval stage of
Echinococcus granulosus or multilocularis
Imaging Findings
• Best diagnostic clue: Large well defined cystic liver
mass with numerous peripheral daughter cysts
• Location: Right lobe more than left lobe of liver
• Average size: 5 cm
• Curvilinear or ringlike pericyst calcification
Top Differential Diagnoses
• Biliary cystadenocarcinoma
• Complex pyogenic abscess
• "Cystic" metastases
• Hemorrhagic or infected cyst
• Extensive, infiltrative cystic and solid masses of
low density (14-40 HU)
• Margins are irregular/ill-defined
• Amorphous type of calcification
• Can simulate a primary or secondary tumor
• CECT
o E. granulosus
• Enhancement of cyst wall and septations
o E. multilocularis
• Minimal enhancement of noncalcified portion
MR Findings
• TlWI
o Rim (pericyst): Hypointense (fibrous component)
o Mother cyst (hydatid matrix)
• Usually intermediate signal intensity
• Rarely hyperintense: Due to reduction in water
content
o Daughter cysts: Less signal intensity than mother
cyst (matrix)
o Floating membrane: Low signal intensity
o Calcifications: Difficult to identify on MR images
• Display low signal on both Tl & T2WI
• T2WI
o Rim (pericyst): Hypointense (fibrous component)
o First echo T2WI: Increased signal intensity
• Mother cysts more than daughter cysts
o Strong T2WI: Hyperintense
• Mother & daughter cysts have same intensity
o Floating membrane
• Low-intermediate signal intensity
• Tl C+
o E. granulosus
• Enhancement of cyst wall and septations
o E. multilocularis
• Minimal enhancement of noncalcified portion
• ± Transdiaphragmatic spread to: Pleura, lung,
pericardium & heart
• MRCP
o ± Demonstrate communication with biliary tree
Ultrasonographic Findings
• Real Time
1
Pathology 33
• Larvae ~ portal vein ~ liver (75%)
• Develop into hydatid stage (4-5 days) within liver
• Hydatid cysts grow to 1 cm during first 6 months
• 2-3 cm annually
Clinical Issues
• Cysts: Initially asymptomatic
• Symptomatic when size l/infected/ruptured
• Pain, fever, jaundice, hepatomegaly
• Serologic tests positive in more than 80% of cases
Diagnostic Checklist
• Daughter cysts can float freely within mother cyst
• Altering patient's position may change position of
daughter cysts
• Confirms diagnosis of echinococcal disease
o Hepatic hydatid cyst manifests in different ways
• Based on stage of evolution & maturity
o E. granulosus
• A well-defined anechoic cyst
• An anechoic cyst except for hydatid "sand"
• A multi septate cyst with daughter cysts &
echogenic material between cysts (characteristic)
• "Water lily" sign: A cyst with a floating,
undulating membrane with a detached endocyst
• A densely calcified mass
o E. multilocularis
• Single/multiple echogenic,lesions
• Usually right lobe of liver
• Irregular necrotic regions & micro calcifications
• ± Intrahepatic bile duct dilatation
o US also used to monitor efficacy of
• Medical antihydatid therapy
o Positive response findings include
• Reduction in cyst size
• Membrane detachment
• Progressive increase in cyst echogenicity
• Mural calcification
Imaging Recommendations
• Best imaging tool: Helical NECT + CECT
• Protocol advice
o Multiplanar imaging show
• Extrahepatic extension of E. multilocularis
I DIFFERENTIAL DIAGNOSIS
Biliary cystadenocarcinoma
• Rare, multiseptated water density cystic mass
• No surrounding inflammatory changes
Complex pyogenic abscess
• "Cluster of grapes": Confluent complex cystic lesions
"Cystic" metastases
• E.g., cystadenocarcinoma of pancreas or ovary
• May present with debris, mural nodularity,
rim-enhancement
 HEPATIC HYDATID CYST
1 Hemorrhagic or infected cyst
34 • Complex cystic heterogeneous mass
• Septations, fluid-levels & mural nodularity
• Calcification mayor may not be seen
[PATHOLOGY
General Features
• General path comments
o Definitive host: Dog or fox
o Intermediate host: Human, sheep or wild rodents
o Germinal layer (endocyst) ~ scolices ~ larval stage
o Hydatid sand: Free floating brood capsules & scolices
form a white sediment
o Larvae ~ portal vein ~ liver (75%)
• Lungs (15%); other tissues (10%)
o E. granulosus
• Develop into hydatid stage (4-5 days) within liver
• Hydatid cysts grow to 1 cm during first 6 months
• 2-3 cm annually
o E. multilocularis
• Larvae proliferate & penetrate surrounding tissue
• Cause a diffuse & infiltrative process
• Simulates a malignancy
• Induce a granulomatous reaction
• Necrosis ~ cavitation ~ calcification
• Etiology
o Caused by two types of parasites
• E. granulosus & E. multilocularis
o Hydatid disease
• Caused by larval stage of Echinococcus tapeworm
• Epidemiology
o E. granulosus: Mediterranean region, Africa, South
America, Australia & New Zealand
o E. multilocularis: France, Germany, Austria, USSR,
Japan, Alaska & Canada
Gross Pathologic & Surgical Features
• E. granulosus
o Large unilocular/multilocular cystic mass
• E. multilocularis or alveolaris
o Multilocular or irregular solid mass
Microscopic Features
• E. granulosus: Pericyst; ectocyst; endocyst
• E. alveolaris: Lamellated wall/liver necrosis + giant
cells + lymphocytes
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Cysts: Initially asymptomatic
• Symptomatic when size t /infected/ruptured
o Pain, fever, jaundice, hepatomegaly
o Allergic reaction; portal hypertension
• Clinical profile
o Middle-aged patient with
• RUQ pain, palpable mass, jaundice
• Eosinophilia, urticaria + anaphylaxis
• Lab data
o Eosinophilia; t serologic titers
o ± t Alkaline phosphatase
o ± t Gamma-glutamyl transpeptidase (GGTP)
• Diagnosis
o Serologic tests positive in more than 80% of cases
o Percutaneous aspiration of cyst fluid
• Danger of peritoneal spill & anaphylactic reaction
Demographics
• Age
o Hydatid disease usually acquired in childhood
o Not diagnosed until 30-40 years of age
• Gender: M = F
Natural History & Prognosis
• Complications
o Compression/infection or rupture into biliary tree
o Rupture into peritoneal or pleural cavity
o Spread of lesions to lungs, heart, brain & bone
• Prognosis
o E. granulosus: Good
o E. alveolaris: Fatal-left untreated within 10-15 years
Treatment
• E. granulosus
o Medical: Albendazole/mebendazole
o Direct injection of scolicidal agents
o Percutaneous aspiration & drainage of cyst
o Surgical: Segmental or lobar hepatectomy
• E. multilocularis
o Partial hepatectomy/hepatectomy + liver transplant
• Surgical: For exophytic groth of hydatid cyst
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other complex or septated cystic liver masses
o Biliary cystadenoma, pyogenic liver abscess, cystic
metastases & hemorrhagic or infected cyst
o E. multilocularis imaging and clinical behavior
simulates solid malignant neoplasm
Image Interpretation Pearls
• Daughter cysts can float freely within mother cyst
o Altering patient's position may change position of
daughter cysts
o Confirms diagnosis of echinococcal disease
I SELECTED REFERENCES
1. Polat P et al: Hydatid disease from head to toe.
2.
Radiographies. 23(2):475-94; quiz 536-7, 2003
Mortele KJ et al: Cystic focal liver lesions in the adult:
differential CT and MR imaging features. Radiographies.
21(4):895-910, 2001
3. Pedrosa I et al: Hydatid disease: Radiologic and pathologic
features and complications. RadioGraphies. 20: 795-817,
2000
4. Taourel P et al: Hydatid cyst of the liver: Comparison of CT
and MRI. Journal of Computer Assisted Tomography.
17(1): 80-5, 1993
 HEPATIC HYDATID CYST
I IMAGE GALLERY
1
35
Typical
(Left) Axial CECT shows
large cystic mass with
partially calcified wall. Note
hypodense septa and floating
debris (scolices). (Right)
Sagittal sonogram shows
complex echogenic mass
with enhanced transmission.

Typical
(Left) Axial T2WI MR shows
complex cystic mass with
peripheral daughter cysts.
(Right) Axial CECT shows
disseminated hydatid disease
with cystic masses in the
spleen, liver and throughout
the peritoneal cavity. Note
the calcified wall especially
in the splenic cystic masses.

(Left) Intra-operative
cholangiogram shows dilated
biliary tree with filling defects
(arrow) due to rupture of
hydatic cyst into intrahepatic
bile ducts. (Right) Axial
CECT in a Mediterranean
immigrant with E.
multilocularis, shows
extensive cystic and solid
infiltrative mass with
ill-defined margins and foci
of calcification (arrows).
 STEATOSIS (FATTY LIVER)
1
36

Cut section of explanted liver shows yellowish, greasy, Axial NECT shows diffuse low attenuation of liver. Note
pale appearance due to steatosis. relative hyperdensity of vessels and spleen.

ITERMINOLOGY • Amount of fat deposited in liver

Abbreviations and Synonyms
• Hepatic steatosis or hepatic fatty metamorphosis
Definitions
• Steatosis is a metabolic complication of a variety of
toxic, ischemic & infectious insults to liver
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Decreased signal intensity of liver
on T1W out-of-phase gradient echo images
• Location: Both lobes of liver
• Size: Diffuse fatty infiltration: Enlarged liver
• Key concepts
o Diffuse (more common) or focal fatty infiltration
o Often lobar, segmental or wedge shaped
o Rarely, unifocal or multifocal sphericallesionsi
simulating metastases or primary tumor
o Fatty replacement occurs where glycogen is depleted
from liver
o Key on all imaging modalities
• Presence of normal vessels coursing through
"lesion" (fatty infiltration)
o Imaging features of fatty liver: Variable based on
• Fat distribution within liver
• Presence of associated hepatic disease
CT Findings
• NECT
o Diffuse or focal
• Decreased attenuation of liver compared to spleen
• Normal: Liver 8-10 HU more than spleen on NECT
o Focal nodular fatty infiltration: Low attenuation
• Common location: Adjacent to falciform ligament
• Cause: Due to nutritional ischemia
• Because it is a vascular watershed area
o Lobar, segmental or wedge-shaped fatty infiltration
• Decreased attenuation
• May have a straight-line margin
• Extending to liver capsule without mass effect
• CECT
o Detect fatty infiltration due to variations in
• Degrees of liver & splenic relative enhancement
o Normal vessels course through "lesion" (fatty
infiltration)
o CECT has lower sensitivity in detecting fatty liver
MR Findings
• T1WI out-of-phase gradient echo image
o Decrease or loss of signal intensity of fatty liver
• T1WI in-phase gradient echo image
o Increased signal intensity of fatty liver than spleen

DDx: Diffuse or Geographic Hypodense liver

Steatohepatitis Diffuse Lymphoma Cholangiocarcinoma Acute Hepatitis

 STEATOSIS (FATTY LIVER)
Key Facts
Terminology
• Steatosis is a metabolic complication of a variety of
toxic, ischemic & infectious insults to liver
Imaging Findings
• Best diagnostic clue: Decreased signal intensity of
liver on T1W out-of-phase gradient echo images
• Diffuse increased hepatic echogenicity
• Focal fatty sparing: Pseudotumor variations
Top Differential Diagnoses
• Alcoholic steatohepatitis
• Diffuse lymphoma or other tumor
Pathology
• Most frequently seen on liver biopsies of alcoholics
• Seen in up to 50% of patients with diabetes mellitus
• T1 C+ out-of-phase GRE image
o Paradoxical decreased signal intensity of liver
• STIR (short T1 inversion recovery)
o Shows fatty areas as low signal intensity
• MR spectroscopy (MRS)
o Fatty liver demonstrates an increase in intensity of
lipid resonance peak
o Used for quantitative assessment of fatty infiltration
of liver
Ultrasonographic Findings
• Real Time
o Diffuse fatty infiltration
• Diffuse increased hepatic echogenicity
• Increased attenuation of ultrasound beam (feature
of fat, not fibrosis)
o Hepatic steatosis & fibrosis frequently coexist
• Produce similar sonographic findings
o Poor visualization of portal & hepatic veins
o Focal fatty infiltration
• Hyperechoic nodule
• Multiple confluent hyperechogenic lesions
o Focal fatty sparing: Pseudotumor variations
• Target lesion: Hypoechoic area with a central
hyperechoic core
• Ovoid or spherical hypoechoic area in an
otherwise echogenic liver
• Usually seen in segment IV of fatty liver
• Often borders of gallbladder fossa
Nuclear Medicine Findings
• Technetium Tc 99m sulfur colloid
o Differentiates true space occupying lesion from focal
fat
o Fat does not displace reticuloendothelial cells
o Diffuse fatty infiltration
• Inhomogeneous radionuclide uptake
• Xenon 133
o Highly fat soluble
o Accumulation of isotope in fatty areas of liver
• Specific sign of hepatic steatosis
1
• Quite prevalent in general population with obesity 37
Clinical Issues
• Asymptomatic, but often with abnormal LFTs
• 2/3 alcoholics: RUQ pain, tenderness, hepatomegaly
• Removal of alcohol or offending toxins
• Correction of metabolic disorders
Diagnostic Checklist
• Rule out other liver pathologies which may mimic
focal or diffuse steatosis (fatty liver)
• Key on all imaging modalities is presence of normal
vessels coursing through "lesion" (fatty infiltration)
Imaging Recommendations
• Best imaging tool: NECT or T1Win & out-of-phase
gradient echo images
I DIFFERENTIAL DIAGNOSIS
Alcoholic steatohepatitis
• Acute phase
o Hepatomagaly
o Diffuse hypodense liver (due to fatty infiltration)
o Fatty infiltration: May be focal, lobar, segmental
• Chronic phase
o Mixture of steatosis & early cirrhotic changes
o Liver-spleen attenuation difference more than 10
HU
• Nonalcoholic steatohepatitis (NASH)
o Indistinguishable from alcoholic hepatitis
Diffuse lymphoma or other tumor
• Diffuse lymphoma infiltration: Indistinguishable from
normal liver or steatosis
• Confluent tumor distorts vessels and bile ducts
• Usually secondary deposits are multiple, well-defined,
low density masses
Acute hepatitis
• Diffuse hypodensity of the liver
• Associated gallbladder wall and periportal edema
• Clinical presentation suggests diagosis
!PATHOlOGY
General Features
• General path comments
o Fat is deposited in liver due to
o Ethanol; increased hepatic synthesis of fatty acids
o Carbon tetrachloride & high dose tetracycline;
decreased hepatic oxidation or utilization of fatty
acids
o Starvation, steroids & alcohol
• Impaired release of hepatic lipoproteins
 STEATOSIS (FATTY LIVER)
1 • Excessive mobilization of fatty acids from adipose
tissue
38 o Segmental areas of fatty infiltration occurs where
glycogen is depleted from liver
• Due to traumatic & ischemic insults
• Decreased nutrients & insulin ~ decreased
glycogen
• Causes: Secondary to a mass, Budd-Chiari
syndrome or tumor thrombus
• Etiology
o Metabolic derangement
• Poorly controlled diabetes mellitus (50%)
• Obesity & hyperlipidemia
• Severe hepatitis & protein malnutrition
• Parenteral hyperalimentation
• Malabsorption (jejunoileal bypass)
• Pregnancy, trauma
• Inflammatory bowel disease
• Cystic fibrosis, Reye syndrome
o Hepatotoxins
• Alcohol (> 50%)
• Carbon tetrachlorides, phosphorus
o Drugs
• Tetracycline, amiodarone, corticosteroids
• Salicylates, tamoxifen, calcium channel blockers
• Epidemiology
o Most frequently seen on liver biopsies of alcoholics
o Seen in up to 50% of patients with diabetes mellitus
o Quite prevalent in general population with obesity
o Seen in 25% of nonalcoholics
• Healthy adult males meeting accidental deaths
• Associated abnormalities
o Nonalcoholic steatohepatitis (NASH)
• Seen in patients with hyperlipidemia & diabetes
• May lead to "cryptogenic" cirrhosis
Gross Pathologic & Surgical Features
• Liver may weigh 4-6 kg
• Soft, yellow, greasy cut surface
Microscopic Features
• Macrovesicular fatty liver (most common type)
o Hepatocytes with large cytoplasmic fat vacuoles
displacing nucleus peripherally
• Examples: Alcohol & diabetes mellitus
• Microvesicular
o Fat is present in many small vacuoles
o Example: Reye syndrome
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, but often with abnormal LFTs
o Enlarged liver in obese or diabetic patient
o Alcoholic patients
• 1/3 Asymptomatic
• 2/3 alcoholics: RUQ pain, tenderness,
hepatomegaly
• Clinical profile
o Asymptomatic obese or diabetic patient with
enlarged liver
• Lab data
o Asymptomatic fatty liver
• Normal to mildly elevated liver function tests
o Alcoholic patients
• Abnormal liver function tests
o Steatohepatitis
• May have markedly abnormal liver functions
• Diagnosis
o Biopsy & histology
Natural History & Prognosis
• Complications
o Acute fatty liver
• Alcoholic binge, pregnancy, CCL4 exposure
• Present with jaundice, acute hepatic failure &
encephalopathy
• Prognosis
o Alcoholics: Gradual disappearance of fat from liver
after 4-8 weeks of adequate diet & abstinence from
alcohol
o Resolves in 2 weeks after discontinuation of
parenteral hyperalimentation
o Steatohepatitis
• May progress to acute or chronic liver failure
Treatment
• Removal of alcohol or offending toxins
• Correction of metabolic disorders
• Lipotropic agents like choline when indicated
o Patient must avoid alcohol & control diabetes
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other liver pathologies which may mimic
focal or diffuse steatosis (fatty liver)
Image Interpretation Pearls
• Decreased attenuation of liver compared to spleen
• Key on all imaging modalities is presence of normal
vessels coursing through "lesion" (fatty infiltration)
I SELECTED REFERENCES
1. RubaltelliL et al: Targetappearance of pseudotumors in
segment IVof the liver on sonography. AJR.178: 75-7,
2002
2. KemperJ et al: CTand MRIfindings of multifocal hepatic
steatosismimicking malignancy. Abdom Imaging.
27(6):708-10,2002
3. Outwater EKet al: Detection of lipid in abdominal tissues
with opposed-phase gradient-echo images at 1.5 T:
Techniquesand diagnostic importance. RadioGraphies.18:
1465-80, 1998
4. Thu HD et al: Valueof MRimaging in evaluating focal
fatty infiltration of the liver: preliminary study.
Radiographies.11(6):1003-12, 1991
 STEATOSIS (FATTY LIVER)

I IMAGE GAllERY 1
39
Typical
(Left) Axial CECT with focal
steatosis shows large
hypodense "masses" within
liver. Note vessels traversing
"masses". (Right) Axial T2WI
MR in patient with focal
steatosis shows no apparent
mass with normal branching
of intrahepatic vessels.

Typical
(Left) Axial TlWI CRE MR
in-phase shows no clear
"mass" appearance in areas
of steatosis. (Right) Axial
TlWI CRE MR out-of-phase
image shows striking signal
loss from areas of hepatic
fatty infiltration.

Typical
(Left) Axial CECT shows
multiple focal low density
lesions mimicking
metastases. Normal
appearing blood vessels
withing "lesions" are clue to
multifocal fatty infiltration.
(Right) Axial CECT shows
diffuse fatty infiltration of the
liver (decreased
attenuation). "Hyperdense
mass" along dorsal surface of
left lobe (arrow) is normal
liver (focal sparing).
 CIRRHOSIS
1
40

Graphic illustrates nodular surface of liver, fibrosis, Axial CECr shows nodular surface of scarred liver with
relaUve enlargement of caudate lobe and lateral fibrotic, small right lobe and enlarged caudate lobe and
segment. lateral segment. Splenomegaly, varices and ascites also
noted.

o Alcohol abuse is most common cause in West

ITERMINOLOGY o One of 10 leading causes of death in Western world
Definitions o 6th leading cause of death in U.S.
• Chronic liver disease characterized by diffuse eT Findings
parenchymal necrosis with extensive fibrosis &
• Nodular liver contour
regenerative nodule formation
• Atrophy of right lobe & medial segment of left lobe
• Enlarged caudate lobe & lateral segment of left lobe
• Widened fissures between segments/lobes
I IMAGING FINDINGS • Regenerative nodules; fibrotic & fatty changes
General Features • Varices, ascites, splenomegaly & peribiliary cysts
• Siderotic regenerative nodules
• Best diagnostic clue: Nodular contour, widened
o NECT: Increased attenuation due to iron content
fissures & hyperdense nodules on NECT that disappear
o CECT: Nodules disappear after contrast
on CECT (cirrhosis with siderotic nodules)
• Nodules & parenchyma enhance to same level
• Location: Diffuse liver involving both lobes
• Dysplastic regenerative nodules
• Size: Liver usually reduced in size
o NECT
• Key concepts
• Large nodules: Hyperdense (1 iron + 1 glycogen)
o Common end response of liver to a variety of insults
• Small nodules: Isodense with liver (undetected)
and injuries
o CECT
o Classification of cirrhosis based on morphology,
• Usually enhance as normal liver
histopathology & etiology
• Sometimes hypervascular
o Classification
• Fibrotic & fatty changes
• Micronodular (Laennec) cirrhosis: Alcoholism
o NECT
(60-70% cases in U.S.)
• Fibrosis: Diffuse lacework, thick bands & mottled
• Macronodular (postnecrotic) cirrhosis: Viral
areas of decreased density
hepatitis (10% in U.S.; majority of cases
worldwide) • Fatty changes: Mottled areas of low attenuation
o CECT
• Mixed cirrhosis

DDx: Nodular Dysmorphic liver

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.•,.' ':"'~0/., ~.~ ..•
,
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~ •• -,- :. ~~? ~
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,. '" ." • ' _.J'

. ./
Budd-Chiari Syndrome Treated Metastases Sarcoidosis
 CIRRHOSIS

Key Facts
Imaging Findings
• Best diagnostic clue: Nodular contour, widened
fissures & hyperdense nodules on NECT that
disappear on CECT (cirrhosis with siderotic nodules)
• Atrophy of right lobe & medial segment of left lobe
• Enlarged caudate lobe & lateral segment of left lobe
• Regenerative nodules; fibrotic & fatty changes
• Varices, ascites, splenomegaly & peribiliary cysts
• Gamna-Gandy bodies (siderotic nodules in spleen)
Top Differential Diagnoses
• Budd-Chiari syndrome
• Treated metastatic disease
• Hepatic sarcoidosis
1
• Macronodular (postnecrotic) cirrhosis: Viral hepatitis 41
• Mixed cirrhosis
• Steatosis ~ hepatitis ~ cirrhosis
• Alcohol (60-70%), chronic viral hepatitis B/C (10%)
• 3rd leading cause of death for men 34-54 years
• U.S: Hepatitis C (cirrhosis) causes 30-50% of HCC
• Japan: Hepatitis C (cirrhosis) 70% of HCC cases
Clinical Issues
• Splenomegaly, varices, caput medusae
• Fatigue, jaundice, ascites, encephalopathy
• Gynecomastia & testicular atrophy in males
• Virilization in females
• Advanced stage: Liver transplantation

Pathology Diagnostic Checklist

• Micronodular (Laennec) cirrhosis: Alcohol • Rule out other causes of "nodular dysmorphic liver"

• Confluent fibrosis: May show delayed persistent • Transhepatic, gastroesophageal, paraesophageal

enhancement • Paraumbilical, intrahepatic, splenorenal
• Fatty changes: Areas of low attenuation
• Cirrhosis-induced hepatocellular carcinoma (HCC) Ultrasonographic Findings
o NECT: Hypodense or heterogeneous; ± fat • Real Time
o CECT o Increased liver echogenicity/loss of normal triphasic
• Intense or heterogeneous enhancement on arterial hepatic vein Doppler tracing/increased pulsatility of
phase; usually iso- to hypodense on venous and portal vein Doppler tracing and same as CT findings
delayed phase scans o Nodular liver contour
• ± Capsule enhancement o Increased liver echogenicity
o Enlarged caudate lobe & lateral segment of left lobe
MR Findings o Atrophy of right lobe & medial segment of left lobe
• Siderotic regenerative nodules: Paramagnetic effect of o Regenerating nodules
iron within nodules o Features of portal hypertension (PHT)
o T1WI: Hypointense • Portal vein (> 13 mm), splenic vein (> 11 mm)
o T2WI: Increased conspicuity of low signal intensity • Superior mesenteric vein (> 12 mm)
o T2 Gradient-echo & fast low-angle shot (FLASH) • Coronary veins (> 7 mm)
images • Increased pulsatility of portal vein Doppler tracing
• Markedly hypo intense • Dilated hepatic & splenic arteries with increased
o Gamna-Gandy bodies (siderotic nodules in spleen) flow
• Seen in cirrhotic patients with portal hypertension • Portal cavernoma, ascites, splenomegaly & varices
• Caused by hemorrhage into splenic follicles • Siderotic nodules
• Composed of fibrous tissue encrusted with • Color Doppler
hemosiderin & calcium o Used to determine portal vein patency & direction
• Tl & T2WI: Hypointense of flow
• T2 GRE & FLASHimages: Markedly hypointense o When portal venous flow pattern is hepatofugal
• Dysplastic regenerative nodules • Patient is not a candidate for splenorenal shunt
o Tl WI: Hyperintense compared to liver parenchyma • Must undergo a total shunt (portacaval or
o T2WI: Hypointense relative to liver parenchyma mesocaval)
• HCC nodule o To guide shunt procedures & to assess blood flow
o Tl WI: Isointense or hypointense • Transjugular intrahepatic portosystemic shunt
o T2WI: Hyperintense
o TIC+: Increased enhancement Imaging Recommendations
• Fibrotic & fatty changes • Best imaging tool: Helical NECT & CECT
o TlWI
• Fibrosis: Hypointense
• Fat: Hyperintense I DIFFERENTIAL DIAGNOSIS
o T2WI
Budd-Chiari syndrome
• Fibrosis: Hyperintense
• Fat: Hypointense • Liver damaged, but no bridging fibrosis
• MR angiography • Occluded or narrowed IVC ± hepatic veins
o Varices: Tortuous structures of high signal intensity • Chronic phase: "Large regenerative nodules"
o Major collateral channels in portal hypertension • Central hypertrophy, peripheral atrophy
 CIRRHOSIS
1 • Chronic phase: "Large regenerative nodules"
• Central hypertrophy, peripheral atrophy
42
Treated metastatic disease
• Example: Breast cancer metastases to liver
o May shrink and fibrose with treatment
o Simulating nodular contour of cirrhotic liver
Hepatic sarcoidosis
• Systemic noncaseating granulomatous disorder
• Hypoattenuating nodules (size: Up to 2 em)
• Hypointense nodules on Tl & T2WI MR
I PATHOLOGY
General Features
• General path comments
o Micronodular (Laennec) cirrhosis: Alcohol
o Macronodular (postnecrotic) cirrhosis: Viral
hepatitis
o Mixed cirrhosis
o Catalase oxidation of ethanol ~ damage cellular
membranes & proteins
o Cellular antigens ~ inflammatory cells ~ immune
mediated cell damage
o Steatosis ~ hepatitis ~ cirrhosis
o Regenerative (especially siderotic) nodules ~
dysplastic nodules ~ HCC
• Dysplastic nodules considered premalignant
• Etiology
o Alcohol (60-70%), chronic viral hepatitis B/C (10%)
o Primary biliary cirrhosis (5%)
o Hemochromatosis (5%)
o Primary sclerosing cholangitis, drugs, cardiac causes
o Malnutrition, hereditary (Wilson), cryptogenic
o In children: Biliary atresia, hepatitis, <x-I antitrypsin
deficiency
• Epidemiology
o 3rd leading cause of death for men 34-54 years
o Risk ofHCC
• U.S.: Hepatitis C (cirrhosis) causes 30-50% of HCC
• Japan: Hepatitis C (cirrhosis) 70% of HCC cases -
• 2.5 times higher in cirrhotic hepatitis B positive
• Alcohol & primary biliary cirrhosis: 2-5 fold t risk
o Mortality due to complication
• Ascites (50%)
• Variceal bleeding (25%)
• Renal failure (10%)
• Bacterial peritonitis (5%)
• Complications of ascites therapy (10%)
Gross Pathologic & Surgical Features
• Alcoholic cirrhosis
o Early stage: Large, yellow, fatty, micronodular liver
o Late stage: Shrunken, brown-yellow, hard organ
with macronodules
• Postnecrotic cirrhosis
o Macronodular (> 3 mm-l em); fibrous scars
Microscopic Features
• Portal-central, portal-portal fibrous bands
• Micro & macronodules; mononuclear cells
• Abnormal arteriovenous interconnections
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Alcoholic cirrhosis
• May be clinically silent
• 10-40% cases found at autopsy
o Nodular liver, anorexia, malnutrition, weight loss
o Signs of portal hypertension
• Splenomegaly, varices, caput medusae
o Fatigue, jaundice, ascites, encephalopathy
o Gynecomastia & testicular atrophy in males
o Virilization in females
• Clinical profile: Patient with history of alcoholism,
nodular liver, jaundice, ascites & splenomegaly
• Lab data: Increase in liver function tests; anemia
o Alcoholic cirrhosis: Severe increase in AST (SGOT)
o Viral: Severe increase in ALT (SGPT)
Demographics
• Age: Middle & elderly age group
• Gender: Males more than females
Natural History & Prognosis
• Complications
o Ascites, variceal hemorrhage, renal failure, coma
o HCC: Due to hepatitis B, C & alcoholism
• Prognosis
o Alcoholic cirrhosis: 5 year survival in less than 50%
o Advanced disease: Poor prognosis
o Liver transplantation: Increases survival period
Treatment
• Alcoholic cirrhosis
o Abstinence; decrease protein diet; multivitamins
o Prednisone; diuretics (for ascites)
• Management limited to
o Treatment of complications & underlying cause
• Advanced stage: Liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of "nodular dysmorphic liver"
Image Interpretation Pearls
• Nodular liver contour; lobar atrophy & hypertrophy
• Regenerative nodules, ascites, splenomegaly, varices
I SELECTED REFERENCES
1. Krinsky GA et al: Hepatocellular carcinoma and dysplastic
nodules in patients with cirrhosis: Prospective diagnosis
with MR imaging & explantation correlation. Radiology.
219:445-54, 2001
2. Lim JH et al: Detection of hepatocellular carcinomas and
dysplastic nodules in cirrhotic livers. AJR.175:693-8, 2000
3. Dodd GO 3rd et al: End-stage primary sclerosing
cholangitis: CT findings of hepatic morphology in 36
patients. Radiology. 211(2):357-62, 1999
4. Dodd GD et al: Spectrum of imaging findings of the liver
in end-stage cirrhosis: Part I, gross morphology and diffuse
abnormalities. AJR:173:1031-1036, 1999
 CIRRHOSIS
I IMAGE GALLERY 1
43
Typical
(Left) Axial CECT shows
shrunken dysmorphic liver;
ascites, large varices. (Right)
Axial CECTshows large
periumbilical varices ("caput
medusae"), ascites,
mesenteric edema; all
manifestations of portal
venous hypertension.

(Left) Axial NECT shows

dysmorphic liver and ascites,
along with dozens of
hyperdense nodules, 0.5-2
cm diameter, representing
siderotic regenerative
nodules. (Right) Axial T2WI
MR shows innumerable
subcentimeter hypointense
lesions throughout a cirrhotic
liver;representing siderotic
nodules.

Typical
(Left) Axial CECT in arterial
phase shows dysmorphic
liver with widened fissures.
Heterogeneous
hypervascular lesion (arrow)
is hepatocellular carcinoma
(HCC). (Right) CECT shows
dysmorphic liver with right .
lobe and medial segment
atrophy, hypertrophy of
caudate, and irregular
dilatation of intrahepatic bile
ducts. Cirrhosis due to
primary sclerosing
cholangitis.
 FOCAL CONFLUENT FIBROSIS
1
44
Cut section of liver shows nodular, cirrhotic morphology
and area of confluent fibrosis, the pale yellow tissue
(arrow) with overlying capsular retraction.
ITERMINOLOGY
Abbreviations and Synonyms
• Confluent hepatic fibrosis (CHF)
Definitions
• Mass-like fibrosis in advanced cirrhosis
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Pre-contrast CT showing
hypo attenuating lesion with volume loss that becomes
isoattenuating or minimally hypoattenuating at
post-contrast CT, especially if wedge-shaped, located
in medial segment of left lobe &/or anterior segment
of right lobe, in patients with advanced cirrhosis
• Location
o Wedge-shaped lesions radiate from porta hepatis &
extend to hepatic capsule
o 90% of wedge-shaped fibrosis involve medial
segment of left lobe &/or anterior segment of right
lobe, with sparing of caudate lobe
o Peripheral lesions are remote from porta hepatis
o Lobar or segmental involvement, most commonly
in lateral segment of left lobe
• Size: May range from 2 x 1.5 cm to 15 x 6 cm
DDx: Focal Hepatic lesions with Capsular Retraction
Cholangiocarcinoma Treated Metastases
Axial NECT shows cirrhotic morphology, ascites, and
hypodense lesion (confluent fibrosis) with overlying
capsular retraction.
• Key concepts
o Wedge-shaped; peripheral are band-shaped
o Total lobar or segmental fibrosis
CT Findings
• NECT
o Wedge-shaped area of lesser attenuation than
adjacent liver parenchyma
• Retraction of overlying liver capsule (90%)
o Peripheral band-like hypo attenuating lesion
o Total lobar or segmental involvement
• Seen as areas of low attenuation involving entire
segment or lobe, with marked shrinkage
o In advanced cirrhosis, there may be no apparent
medial segment of left lobe or anterior segment of
right lobe, producing bizarre contour of liver at CT
• CECT
o Lesions are isoattenuating to adjacent liver
parenchyma post-contrast (80%)
• May appear minimally or substantially
hypoattenuating post-contrast
• Or may be of higher attenuation compared to
surrounding parenchyma (delayed scans)
o Mechanism of variability in contrast enhancement
of confluent fibrosis relates to relative vascularity &
extent of fibrosis
• May show delayed persistent enhancement like
other fibrotic liver lesions
Sclerosing Cholangitis EHE of Liver
 FOCAL CONFLUENT FIBROSIS
Key Facts
Terminology
• Confluent hepatic fibrosis (CHF)
Imaging Findings
• Best diagnostic clue: Pre-contrast CT showing
hypo attenuating lesion with volume loss that
becomes isoattenuating or minimally
hypoattenuating at post-contrast CT, especially if
wedge-shaped, located in medial segment of left lobe
&/or anterior segment of right lobe, in patients with
advanced cirrhosis
• Wedge-shaped lesions radiate from porta hepatis &
extend to hepatic capsule
• Peripheral lesions are remote from porta hepatis
• Lobar or segmental involvement, most commonly in
lateral segment of left lobe
• Greater enhancement than adjacent liver
parenchyma in arterial phase (13%), may relate to
1 hepatic arterial flow
o Crowding of blood vessels, or bile ducts, within
collapsed area of hepatic parenchyma
MR Findings
• TIWI
o Lesions appear as regions of hypointense signal
relative to adjacent liver parenchyma
• May be isointense (less common)
• T2WI
o Lesions are hyperintense
• Due to prominent edema & approximation of
remnant portal triads within fibrotic areas
• STIR:Hyperintense lesions
• Tl C+
o Lesions are slightly hypointense to liver on
immediate post-gadolinium sequences (80%)
• During later dynamic phase, portions of fibrotic
lesions may become isointense with liver
• Delayed progressive increased enhancement on
portal venous & equilibrium phase images
o May be slightly hyperintense, due to pooling of
contrast material within fibrotic stroma
• Ferumoxide-enhanced MR: Wedge-shaped area of high
signal intensity (corresponds to distribution of fibrosis)
with internal focal areas of low signal intensity
(correspond to residual functioning liver parenchyma)
Imaging Recommendations
• Best imaging tool
o CT or MR
• MR imaging does show morphological changes &
characteristic locations that suggest diagnosis, but
no more so than CT
o Lesion conspicuity better at pre-contrast than
post-contrast CT
• Protocol advice: NECT & CECT or MR & CEMR
.
1
• MR imaging does show morphologICal changes & 45
characteristic locations that suggest diagnosis, but no
more so than CT
Pathology
• Can be seen on imaging in approximately 14% of
patients with advanced cirrhosis who are candidates
for liver transplantation
• Associated volume loss seen as retraction of overlying
hepatic capsule or total shrinkage of segment or lobe
Diagnostic Checklist
• Consider cholangiocarcinoma or treated malignancy
in differential diagnosis
I DIFFERENTIAL DIAGNOSIS
Cholangiocarcinoma (CC)
• May cause segmental volume loss, capsular retraction,
delayed enhancement
o Capsular retraction; because these tumors have
prominent fibrous stroma & because they often
cause intrahepatic bile duct obstruction
• Look for biliary obstruction
o In confluent fibrosis, unlike in cholangiocarcinoma,
bile ducts within affected segments are not dilated
• Peripheral form of CC, most often associated with
capsular retraction, is usually spherical, hypodense
mass on NECT
o CECT: 1 & prolonged enhancement (fibrosis)
• Clinical clues: History of primary sclerosing
cholangitis or other chronic bile duct inflammation
• May require biopsy for diagnosis
Treated malignancies
• Treatment (chemotherapy, ablation, etc.) may result in
volume loss + fibrosis of tumor & surrounding liver
• May be indistinguishable from confluent fibrosis
• Check for prior imaging or clinical evidence of tumor
Intrahepatic biliary obstruction
• May present as focal hepatic lesion with capsular
retraction
• Malignant or benign biliary obstruction leads to
hepatic atrophy of segments drained by obstructed
bile ducts
o Chronic primary sclerosing cholangitis results in
disproportionate atrophy of periphery of liver with
confluent fibrosis & capsular retraction
o Compensatory hypertrophy of deep right lobe &
caudate lobe may result in "pseudotumor"
appearance
oCT: Irregular strictures & focal dilatations of
intrahepatic bile ducts
Cavernous hemangiomas
• Hepatic hemangioma, especially in cirrhotic livers may
have retraction of liver capsule (24%)
 FOCAL CONFLUENT FIBROSIS
1 • Cirrhotic liver hemangiomas often undergo
progressive fibrosis & diminution in size, often
46 resulting in hyalinized scar that no longer maintains
typical radiologic & pathologic features of
hemangioma
• Hemangiomas in cirrhotic livers: May be
subcapsular/demonstrate exophytic growth/peripheral
progressive nodular enhancement/& near
isoattenuation with blood vessels
Epithelioid hemangioendothelioma (EHE)
• Predominantly involves peripheral portion of liver
• Capsular retraction is frequently seen
• Usually occurs in young patients without cirrhosis
• Almost always multiple & more nodular
I PATHOLOGY
General Features
• Etiology: Cause of liver cirrhosis: Viral
infection/alcohol abuse/biliary disease (primary biliary
cirrhosis, sclerosing cholangitis, biliary atresia),
autoimmune hepatitis, ()(l-antitrypsin deficiency,
hemochromatosis, cryptogenic or uncertain
• Epidemiology
o Can be seen on imaging in approximately 14% of
patients with advanced cirrhosis who are candidates
for liver transplantation
o It occurs most commonly in cirrhosis secondary to
primary sclerosing cholangitis (56%)
• Associated abnormalities
o Wedge-shaped fibrosis seen frequently with
alcoholic cirrhosis (19%), only 6% in cirrhosis due
to viral infection; reason is unclear
o Lobar or segmental atrophy of liver parenchyma
associated with primary sclerosing cholangitis
Gross Pathologic & Surgical Features
• CHF appears as a regional mass; area of yellowish color
with little intervening liver parenchyma
• Associated volume loss seen as retraction of overlying
hepatic capsule or total shrinkage of segment or lobe
Microscopic Features
• Fibrosis with prominent edema & approximation of
remnant portal triads with little intervening
regenerating nodules
• Bile-duct proliferation & lymphocyte infiltration
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Signs/symptoms relate to cause + extent of cirrhosis
o CHF seen incidentally in patients with advanced
cirrhosis who undergo pretransplant imaging
• Clinical profile
o Lab: 1 LFT;alcoholic cirrhosis- 11 AST;viral- 11 ALT
o Diagnosis: Liver biopsy to differentiate CHF from
hepatic malignancy, as some overlap of findings are
seen at imaging
Demographics
• Age
o Adults, mean age: 51 years
o Case report of CHF in children, in hepatic damage
associated with anti-tuberculous drugs
• Gender: M > F (related to cirrhosis)
Natural History & Prognosis
• Severe fibrosis with capsular retraction can result in
thinning of the involved segments to such a degree
that they are no longer present
• Medial segment of left lobe & anterior segment of
right lobe are most easily damaged, this might be
related to impaired portal microcirculation
• Massive fibrosis takes long time to develop
• Complications: Cirrhotic patients are at high risk of
developing hepatocellular carcinoma
• Advanced cirrhosis: Poor prognosis - 1 survival period
with liver transplantation
Treatment
• Management limited to treatment of complications &
underlying cause of cirrhosis
• Advanced disease: Liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Fibrosis is present in all cirrhotic livers
o Confluent is just one pattern that is evident on
imaging + gross pathology
o Notable because it may simulate tumor, especially
cholangiocarcinoma
• MR is not complementary to CT; shows same features
Image Interpretation Pearls
• Characteristic location (medial segment of left lobe,
anterior segment of right lobe or both) & shape
(wedge-shape with capsular retraction & volume loss) -
enables correct diagnosis & may prevent unnecessary
biopsy
• Consider cholangiocarcinoma or treated malignancy
in differential diagnosis
I SELECTED REFERENCES
1. Blachar A et al: Hepatic capsular retraction: spectrum of
benign and malignant etiologies. Abdom Imaging.
27(6):690-9,2002
2. Matsuo M et al: Confluent hepatic fibrosis in cirrhosis:
ferumoxides-enhanced MR imaging findings. Abdom
Imaging. 26(2):146-8, 2001
3. Ooi CG et al: Confluent hepatic fibrosis in monozygotic
twins. Pediatr Radiol. 29(1):53-5,1999
4. Ahn 10 et al: Early hyperenhancement of confluent
hepatic fibrosis on dynamic MR imaging. AJRAm J
Roentgenol. 171(3):901-2, 1998
5. Ohtomo K et al: Confluent hepatic fibrosis in advanced
cirrhosis: appearance at CT. Radiology. 188(1):31-5, 1993
6. Ohtomo K et al: Confluent hepatic fibrosis in advanced
cirrhosis: evaluation with MR imaging. Radiology.
189(3):871-4, 1993
 FOCAL CONFLUENT FIBROSIS

I IMAGE GAllERY 1
47
Other
(Left) Axial NECT shows
hypodense lesion in anterior
and medial segments with
capsular retraction. (Right)
Axial T2WI MR shows
wedge-shaped hyperintense
lesion in anterior and medial
segments with capsular
retraction, representative of
focal confluent fibrosis.

Other
(Left) Axial CECT shows
cirrhotic morphology and
subtle low attenuation
throughout the anterior right
lobe from early focal
confluent fibrosis. (Right)
Axial CECT 3 months
following prior image shows
marked volume loss of
anterior segment and
capsular retraction.

Typical
(Left) Axial NECT shows
wedge-shaped focal
confluent fibrosis with
capsular retraction in a
patient with cirrhosis. (Right)
Axial CECT in portal venous
phase shows heterogeneous
enhancement of fibrotic
lesion.
 PRIMARY BILIARY CIRRHOSIS
1
48

Transverse cut section of explanted liver shows Axial CECT shows nodular heterogeneous cirrhotic liver
regenerating nodules and lace-like fibrosis. with prominent porta hepatis lymphadenopathy
(arrow). Note enlarged spleen secondary to portal
hypertension.

o In less advanced disease, liver is enlarged & smooth,

ITERMINOlOGY as disease progresses, becomes more nodular &
Abbreviations and Synonyms eventually grossly cirrhotic
• Primary biliary cirrhosis (PBe) o Smooth liver contour in 71% of less advanced &
43% of advanced PBC
Definitions o Nodular contour in 29% of less advanced & 57% of
• PBC is a chronic progressive cholestatic liver disease advanced PBC cases
characterized by non-suppurative destruction of
interlobular bile ducts leading to advanced fibrosis, CT Findings
cirrhosis, & liver failure • NECT
o Heterogeneously attenuating liver parenchyma in
both advanced & less advanced cases of PBC
I IMAGING FINDINGS • In less advanced PBC, liver parenchyma may be
homogeneously attenuating
General Features • 1 Caudate lobe to right lobe ratio; 43-48% with
• Best diagnostic clue: Liver biopsy; to identify cause of advanced & less advanced PBC
cirrhosis & histopathology stage • Focal or diffuse atrophy; 65% with advanced PBC,
• Location: Diffuse involvement 52% with less advanced PBC
• Size • Global or segmental hypertrophy; 51% with
o Normal or 1 hepatic volume at time of diagnosis advanced, 67% in less advanced PBC
o Trend toward decreasing hepatic volume with more • Segmental hypertrophy typically in lateral &
advanced disease; some patients with advanced caudate segments, segmental atrophy of right lobe
disease may have grossly enlarged liver & medial segment.
o Mean nodule diameter in liver: Having nodular o Fibrosis: Lace-like pattern of thin or thick bands of
contour is 0.5 cm, with smooth contour it is 0.3 cm low attenuation that surround regenerating nodules
o Lymph nodes: Moderate enlargement: 1.5-2 cm (seen in one-third of patients, regardless of stage, &
• Key concepts seems to be characteristic of PBe)

DDx: Heterogeneous Hepatomegaly with Lymphadenopathy

Early ETOH Cirrhosis Diffuse Metastases Amyloidosis Sickle Cell Disease

 PRIMARY BILIARY CIRRHOSIS
I IMAGE GALLERY 1
51
Typical
(Left) Axial NECT shows
hepatomegaly and thin
interconnecting bands of low
attenuation from lace-like
from fibrosis. (Right) Axial
CECT in portal venous phase
shows heterogeneity of the
liver, with enhancing bands
of fibrosis. There is no
discrete mass.

Typical
(Left) Axial TlWI MR shows
lace-like hypointense fibrosis
of the liver. (Right) Axial
T2WI MR shows
innumerable subcentimeter
hypointense regenerating
nodules surrounded by thin
bands of hyperintense
fibrosis.

Typical
(Left) Axial CECT shows
cirrhotic morphology of the
liver with wide fissures;
prominent porta hepatis,
lymphadenopathy and

•.
splenomegaly. Despite
cirrhosis, there is a smooth

~.
: -..
~
_ ...•~
liver contour. (Right) ERC?
shows pruned, intrahepatic
bile ducts with decreased
arborization; "tree in winter"

" ..~~A:
~ ")
, '

appearance.

~.

'C» ~
 NODULAR REGENERATIVE HYPERPLASIA
1
52

Cut section of dysmorphic liver from a patient with Axial CECT of paUent with Budd-Chiari syndrome,
Budd-Chiari syndrome shows caudate hypertrophy, shows dysmorphic liver with numerous hypervascular
lateral segment atrophy, large and numerous orange lesions (arrows)represenUngregenerativenodules.
regenerativenodules (arrows).

o Multiple nodules of hyperplastic hepatocytes with

ITERMINOlOGY atrophy or compression of intervening parenchyma
Abbreviations and Synonyms CT Findings
• Nodular regenerative hyperplasia (NRH); large
• NECT
(multiacinar) regenerative nodules o Due to small size of nodules & preserved framework,
Definitions imaging examinations may show normal findings
• NRH of liver is a rare disorder characterized by diffuse o Nodules are usually isoattenuating to normal liver
micronodular transformation of hepatic parenchyma o Diffuse low attenuation in Budd-Chiari syndrome or
without fibrous septa between nodules steatosis or aggregation of blood-filled spaces may
• Larger lesions are called multi acinar (large) result in hyperattenuation of nodules
regenerative nodules • CECT
o Helical CT may show solitary or multiple uniformly
hypervascular, & hyperattenuating lesions on both
I IMAGING FINDINGS arterial & portovenous phases
o Large regenerative nodules have an 1 arterial supply
General Features corresponding to their bright enhancement
• Best diagnostic clue: Liver biopsy; multiple mono- or • ± Perinodular hypoattenuating rim
multi acinar regenerative nodules of hyperplastic o Large regenerative nodules can be recognized as
hepatocytes discrete focal hypervascular lesions on CECT
• Location: Diffuse involvement; microscopic nodules • Pseudotumoral presentation: Peripheral rim of
predominantly distributed in periportal region enhancement due to peliosis surrounding nodules
• Size • Portal hypertension: Varices, ascites, splenomegaly due
o Monoacinar lesions in NRH are only about 1 mm in to underlying liver disease (e.g., Budd-Chiari)
diameter, with clusters of lesions up to 10 mm MR Findings
o Large regenerative nodules: 0.5-4 cm
• TlWI: Larger nodules: Hyperintense on TlWI (75%)
• Key concepts • T2WI
o Isointense or hypointense nodules on T2WI

DDx: Multiple Hypervascular lesions in Dysmorphic liver

Multifocal HCC Multifocal HCC AP Shunts in Cirrhosis AP Shunts in Cirrhosis

 NODULAR REGENERATIVE HYPERPLASIA
Key Facts
Terminology
• NRH of liver is a rare disorder characterized by diffuse
micronodular transformation of hepatic parenchyma
without fibrous septa between nodules
Imaging Findings
• Large regenerative nodules: 0.5-4 em
• Helical CT may show solitary or multiple uniformly
hypervascular, & hyperattenuating lesions on both
arterial & portovenous phases
• Bright homogeneous enhancement
• ± Ring (halo) enhancement
Pathology
• Local hyperplastic response of hepatocytes, probably
due to chronic ischemia
o May appear hyperintense (due to infarction)
o "Halo sign": Nodule surrounded by peliosis
• Multiphasic enhanced MR
o Bright homogeneous enhancement
o ± Ring (halo) enhancement
• MR with gadobenate dimeglumine: Uptake & delayed
clearance (prolonged enhancement) due to metaplastic
bile ductules in some large regenerative nodules
Ultrasonographic Findings
• Real Time: Nodules may appear as hypo echoic
(38%)/isoechoic (l0%)/hyperechoic (53%) lesions
• Color Doppler: Occlusion or narrowing of IVC or
hepatic veins (Budd-Chiari); obliteration or congenital
absence or portal vein
Angiographic Findings
• Conventional: Nodules may fill from periphery on
angiography, are vascular, & sometimes contain small
hypovascular areas due to hemorrhage or scar
Nuclear Medicine Findings
• Technetium sulfur colloid
o Nodules take up technetium sulfur colloid
Imaging Recommendations
• Best imaging tool: NECT + CECT; or MR
• Protocol advice: NE & multiphasic CECT or MR
I DIFFERENTIAL DIAGNOSIS
Multifocal hepatocellular carcinoma (HCC)
• Hypoattenuating to liver on NECT & delayed CT
• Hyperattenuating on arterial phase, & may be hypo-,
iso-, or hyperattenuating on portovenous phase
• Hypointense on Tl WI! hyperintense on T2WI
• Other characteristics of HCC: Heterogeneity,
multiplicity, encapsulation, venous invasion
Multiple arterioportal (AP) shunts in cirrhosis
• Transsinusoidal arterioportal shunting in advanced
cirrhosis
1
• Chronic Budd-Chiari syndrome (most common 53
setting)
• Monoacinar lesions present in liver that is not
fibrotic or cirrhotic
Clinical Issues
• Clinical profile: Diagnosis: Liver biopsy of large
regenerative nodules shows hyperplastic liver tissue, ±
inflammation, scar (similar to FNH)
• Multiple hypervascular lesions in dysmorphic
(cirrhotic) liver
o Hypervascular & hyperattenuating on both arterial
& portovenous phases (isodense to blood vessels)
[PATHOLOGY
General Features
• General path comments
o Large regenerative nodules are caused by vascular
derangement of liver due to decreased portovenous
or hepatovenous flow
• Resulting multifocal hepatic arterial dilatation
leads to focal hyperplasia & proliferation of
hepatocytes
o Existence of familial cases of NRH of liver
• Occurring without underlying or associated
systemic disease & is characterized by poor clinical
course & often associated with renal failure
• Etiology
o Unknown etiology of NRH; various theories
• Local hyperplastic response of hepatocytes,
probably due to chronic ischemia
• Prolonged exposure to hepatopoietins
• Antiphospholipid antibodies may playa role
• Coexpression of interleukins, IL-6 & soluble IL-6R
• Drugs: Azathioprine (AZA), steroids, & Thorotrast
• Epidemiology
o Rare entity; incidence: 0.6-2.6% on autopsy series
o NRH is seldom reported, most reports have been
single cases, prevalence is not exactly known
o Probably underdiagnosed owing to a lack of
recognition of entity & limited sampling by biopsy
• Associated abnormalities
o Chronic Budd-Chiari syndrome (most common
setting)
o Restrictive cardiomyopathy, congenital absence of
portal vein, idiopathic superior mesenteric
arteriovenous fistula
o Systemic lupus erythematosus, systemic sclerosis,
rheumatoid arthritis, polyarteritis nodosa
o Hematologic diseases (myeloma or lymphoma)
 NODULAR REGENERATIVE HYPERPLASIA
1 o Hepatic metastases from breast carcinoma
o Common variable immunodeficiency
54 o Secondary to organ transplantation
Gross Pathologic & Surgical Features
• Round, orange-brown, well-demarcated, soft to firm
nodules, scattered in dysmorphic liver
• Obliteration of portal veins, portal hypertension,
chronic hepatovenous outflow obstruction
Microscopic Features
• Multi-acinar nodules, consist of different-sized
hepatocytes one or two plates wide & narrow sinusoids
organized to form large regenerative nodules
• Interspersed between nodules are areas of centrilobular
atrophy with curvilinear areas of sinusoidal dilation,
marked congestion & paucity of fibrosis
• Monoacinar lesions present in liver that is not fibrotic
or cirrhotic
• Larger (multi-acinar) nodules may have hepatic
fibrosis between them
• May have central scar; indistinguishable from FNH
• ± Proliferation of bile ductules or metaplastic
transformation of hepatocytes into bile ductules
• ± Mineral deposits & copper accumulation in nodules
ICLINICAllSSUES
Presentation
• Most common signs/symptoms: NRH generally
asymptomatic; signs, symptoms & biochemical
abnormalities relate to underlying liver disease
• Clinical profile: Diagnosis: Liver biopsy of large
regenerative nodules shows hyperplastic liver tissue, ±
inflammation, scar (similar to FNH)
Demographics
• Age: Adults; rare entity, especially in children
• Gender: No gender predilection known
Natural History & Prognosis
• Large regenerative nodules are prone to congestion &
infarction if venous drainage is impaired, as in
Budd-Chiari syndrome or cardiomyopathy
• NRH may bleed, may be associated with portal
hypertension in one-half of cases, & often associated
with systemic diseases (myelo- or lymphoproliferative)
• Diagnosis of NRH is often missed; present with
secondary complications of underlying liver disease
• Variceal bleeding is main source of mortality
• Liver failure is uncommon due to satisfactory
preservation of liver function
• NRH associated with azathioprine (AZA) represents a
risk factor for HCC; AZA should be stopped in patients
with NRH & patients should be screened for HCC
• Prognosis is related to consequences of portal
hypertension & severity of associated diseases
o Prognosis in absence of portal hypertension is good
Treatment
• Management directed to portal hypertension &
variceal bleeding, with beta-blockers, sclerotherapy,
mesenteric-caval shunt & transjugular intrahepatic
porto systemic shunt (TIPS)
• Orthotopic liver transplantation for progressive
hepatic failure & clinical end-stage liver disease
I DIAGNOSTIC CHECKLIST
Consider
• NRH (with large multi acinar nodules) is easily
confused with other masses such as HCC in cirrhosis
o Important to recognize underlying liver disorder
(e.g., Budd-Chiari) + characteristic appearance of
NRH to avoid mistakes
I SELECTED REFERENCES
1. Maetani Y et al: Benign hepatic nodules in Budd-Chiari
syndrome: radiologic-pathologic correlation with emphasis
on the central scar. A]R Am] Roentgenol. 178(4):869-75,
2002
2. Brancatelli G et al: Benign regenerative nodules in
Budd-Chiari syndrome and other vascular disorders of the
liver: radiologic-pathologic and clinical correlation.
Radiographies. 22(4):847-62, 2002
3. Brancatelli G et al: Large regenerative nodules in
Budd-Chiari syndrome and other vascular disorders of the
liver: CT and MR imaging findings with clinicopathologic
correlation. A]R Am] Roentgenol. 178(4):877-83, 2002
4. Horita T et al: Significance of magnetic resonance imaging
in the diagnosis of nodular regenerative hyperplasia of the
liver complicated with systemic lupus erythematosus: a
case report and review of the literature. Lupus. 11(3):193-6,
2002
5. Trenschel GM et al: Nodular regenerative hyperplasia of
the liver: case report of a 13-year-old girl and review of the
literature. Pediatr Radiol. 30(1):64-8, 2000
6. Zhou H et al: Multiple macro regenerative nodules in liver
cirrhosis due to Budd-Chiari syndrome. Case reports and
review of the literature. Hepatogastroenterology.
47(32):522-7,2000
7. Soler R et al: Benign regenerative nodules with copper
accumulation in a case of chronic Budd-Chiari syndrome:
CT and MR findings. Abdom Imaging. 25(5):486-9, 2000
8. Rha SE et al: Nodular regenerative hyperplasia of the liver
in Budd-Chiari syndrome: CT and MR features. Abdom
Imaging. 25(3):255-8, 2000
9. Grazioli L et al: Congenital absence of portal vein with
nodular regenerative hyperplasia of the liver. Eur Radiol.
10(5):820-5, 2000
10. Vilgrain V et al: Hepatic nodules in Budd-Chiari syndrome:
imaging features. Radiology. 210(2):443-50, 1999
11. Clouet M et al: Imaging features of nodular regenerative
hyperplasia of the liver mimicking hepatic metastases.
Abdom Imaging. 24(3):258-61, 1999
12. Morla RM et al: Nodular regenerative hyperplasia of the
liver and antiphospholipid antibodies: report of two cases
and review of the literature. Lupus. 8(2):160-3, 1999
13. Casillas C et al: Pseudo tumoral presentation of nodular
regenerative hyperplasia of the liver: imaging in five
patients including MR imaging. Eur Radiol. 7(5):654-8,
1997
14. Wanless IR: Micronodular transformation (nodular
regenerative hyperplasia) of the liver: a report of 64 cases
among 2,500 autopsies and a new classification of benign
hepatocellular nodules. Hepatology. 11(5):787-97, 1990
 NODULAR REGENERATIVE HYPERPLASIA
I IMAGE GALLERY 1
55

(Left) Axial CECTshows

,.., ~~~
.,
dysmorphic liver with
collateral blood vessels on
the surface of the liver.
Hypervascular lesion with
':1." < 't. ' .. -':. ~ ", ' ..

hypodense ring (arrow)

, :~ '-,>::~'':'" ..--.' represents a focus of nodular
,?;;1.c.· ~', 't(J...
". \.
... ..
'" ':' , ~ regenerative hyperplasia
(Right) Axial CECT shows

I
;

,.
/.
.'- " "
""
'
"'
.•-
. -'''' I'
dysmorphic liver; intra and
extrahepatic collaterals
bypassing occluded portal
vein.

';'J1~ ~

(Left) Axial T7 C+ MR shows

numerous 2 em hyperintense
lesions in a patient with
Budd-Chiari syndrome.
(Right) Axial T2WI MR
demonstrates inconspicuous
hypointense foci of nodular
regenerative hyperplasia
(arrow) in the right hepatic
lobe.

Typical
(Left) Axial CECT shows in
patient with Budd-Chiari
syndrome (note IVC stent).
Innumerable hyper vascular
foci in liver are large
regenerative hyperplastic
nodules. (Right) Axial CECT
shows synthetic mesocaval
shunt (arrow) and
subcutaneous collateral
veins. Hepatic imaging
demonstrated dysmorphic
morphology with multiple
large regenerative
hyperplastic nodules.
 HEPATIC SARCOIDOSIS
1
56

Axial CECT shows multiple small hypodense lesions in Axial CECT shows bilateral hilar and mediastinal
liver and spleen. Note porta hepatis lymphadenopathy lymphadenopathy.

ITERMINOLOGY o It has been suggested that radiologically visib

Abbreviations and Synonyms
• Boeck sarcoid
Definitions
• Sarcoidosis is a relatively common, chronic,
multisystem disease of unknown origin characterized
by presence of non caseating epithelioid granulomas
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Liver biopsy showing diffuse
small noncaseating granulomas
• Location
o Sarcoidosis can affect almost every organ
o Most common site of involvement is lung
o Lymph nodes, spleen, liver, eyes, skin, salivary
glands, bones, nervous system, heart
• Size
o Granulomas are generally 50 to 300 ]Jm in size, &
even though they may aggregate into larger clusters,
they usually remain smaller than 2 mm in diameter
• Large nodules may be up to 2 cm
• Key concepts
o Granulomas are usually inconspicuous & featureless
nodules do not simply represent increasing numbers
of microscopic granulomas
• May instead represent a more vigorous
immunologic response to unknown causative
agent of sarcoidosis
• Larger coalescence of granulomas are coupled with
exuberant deposition of reticulin, evolving to
fibrosis around granulomatous aggregates
CT Findings
• NECT
o Most common finding is nonspecific
hepatosplenomegaly
o Diffuse parenchymal heterogeneity or multinodular
pattern in liver, spleen, or both
• Multiple low-attenuation nodules
o Upper abdominal lymphadenopathy is often present
• CECT
o Low density nodules before contrast agent injection,
usually become rapidly isodense with rest of liver
parenchyma on enhanced scans
o Advanced disease may cause or simulate cirrhosis
MR Findings
• Tl WI: When visualized, nodules appear hypointense
to adjacent liver parenchyma
• T2WI: Hypointense

DDx: Heterogeneous Hepatomegaly with lymphadenopathy

...

I
,
, ., ,-
~
.~
""

Biliary Cirrhosis Lymphoma Opportunistic Infection Amyloidosis

 HEPATIC SARCOIDOSIS
Key Facts
Terminology
• Sarcoidosis is a relatively common, chronic,
multisystem disease of unknown origin characterized
by presence of noncaseating epithelioid granulomas
Imaging Findings
• Sarcoidosis can affect almost every organ
• Most common finding is nonspecific
hepatosplenomegaly
• Diffuse parenchymal heterogeneity or multinodular
pattern in liver, spleen, or both
• Upper abdominal lymphadenopathy is often present
• Low density nodules before contra'st agent injection,
usually become rapidly isodense with rest of liver
parenchyma on enhanced scans
• Advanced disease may cause or simulate cirrhosis
Ultrasonographic Findings
• Real Time
o Nonspecific, diffuse parenchymal heterogeneity
o Granulomas: Hypoechoic nodules
Nuclear Medicine Findings
• Gallium scan
o Gallium-67 localizes in areas of granulomatous
infiltrates; however, it is nonspecific
Other Modality Findings
• Cholangiography may be useful in evaluation of
patients with cholestatic sarcoid liver disease
o Diffuse intrahepatic biliary strictures
Imaging Recommendations
• Best imaging tool
o CT & MR are more sensitive than US
• Able to show multinodular pattern
• Protocol advice: NECT & CECT followed by biopsy
I DIFFERENTIAL DIAGNOSIS
Primary biliary cirrhosis (PBC)
• Disease of hepatic parenchyma with epithelioid cell
granulomas affecting intra-hepatic biliary tree
• Idiopathic, progressive, nonsuppurative, destructive
cholangitis of interlobar bile ducts/nodular
regeneration/shrinkage of hepatic parenchyma
• PBC may also have heterogeneous hepatomegaly &
upper abdominal lymphadenopathy
• "Lace-like"diffuse fibrosis & subtle high density
nodules more characteristic of PBC
• Mitochondrial antibody test can differentiate among
these; test is negative in sarcoid & usually positive in
primary biliary cirrhosis
Lymphoma
• Secondary lymphoma is either multinodular or
diffusely infiltrative
• Homogeneous hepatomegaly &/or hypo echoic focal
nodules
1
Clinical Issues 57
• 3 well-recognized clinical syndromes: Chronic
intrahepatic cholestasis, portal hypertension &
Budd-Chiari syndrome are quite sporadic
• Complications: Hepatic failure is due to intrahepatic
cholestasis & portal hypertension
Diagnostic Checklist
• Presence of hepatic nodules at imaging is not
correlated with advanced pulmonary disease
• Sarcoidosis can appear in an atypical fashion, & it
should be kept in mind in differential diagnosis of
focal & diffuse liver disease
• Do not assume that heterogeneous hepatomegaly &
abdominal lymphadenopathy are always malignant
• Lower frequency of retrocrural adenopathy in
sarcoidosis than in non-Hodgkin lymphoma (NHL)
• Mean nodal size is greater in NHL & nodes tend to be
more confluent in NHL, discrete in sarcoidosis
Opportunistic infection
• In immunocompromised hosts
• Infection by fungi, mycobacteria, viruses, etc.
• Heterogeneous hepatosplenomegaly ± abdominal
lymphadenopathy may be indistinguishable by
imaging alone
Gaucher disease
• Glucocerebroside accumulates in reticuloendothelium
• Well-defined hypoechoic areas on US/low-density
lesions lesions on CT
• Early onset of significant hepatosplenomegaly
Amyloidosis
• Heterogeneous hepatomegaly, ± lymphadenopathy
• Generalized or focal! liver density, both on pre &
post-contrast-enhanced scans
I PATHOLOGY
General Features
• General path comments
o Sarcoidosis is a main cause of hepatic granulomas
o Although involvement of abdominal organs is
frequent in course of systemic sarcoidosis, its clinical
manifestations are usually documented after
diagnosis has been made on basis of thoracic
manifestations
• Etiology: Mechanisms that initiate formation of
sarcoid granulomas are unknown
• Epidemiology
o Prevalence: 1-6:100,000
o 24-79% of patients have liver involvement
• Associated abnormalities
o Association between sarcoidosis & primary
sclerosing cholangitis has been suggested
o Coexistence of sarcoidosis & a wide range of
autoimmune disorders
 HEPATIC SARCOIDOSIS
1 Gross Pathologic & Surgical Features
58 • Hepatomegaly (18-29%)
• Scattered nodular lesions (5%)
Microscopic Features
• Noncaseating epithelioid granulomas with
multinucleated giant cells of Langhans type are
scattered throughout liver
• Characteristic inclusions in giant cells (for example,
Schaumann bodies & asteroid bodies) are not seen in
all cases & are not pathognomonic
• Confluent granulomas & fibrosis can be present in
cases with severe hepatic involvement
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Overt clinical manifestations are uncommon
o Asymptomatic
o Hepatosplenomegaly in about 20% of cases
o Abdominal &/or pelvic lymphadenopathy
o Rarely it can lead to chronic inflammation, chronic
hepatitis, & cirrhosis, or nodular hyperplasia
• Cirrhosis & focal fibrosis may be caused by
ischemia secondary to primary granulomatous
phlebitis of portal & hepatic veins
• Clinical profile
o Diverse clinical presentations
o Lab: Mild elevation of liver enzymes (4%)
• Hypercalcemia, hypercalciuria,
hypergammaglobenemia, anemia, leukopenia
• Angiotensin-converting enzyme; elevated in 60%
of patients with sarcoidosis - nonspecific &
generally not useful in following course of disease
o Diagnosis: Liver biopsy; showing diffuse small
non caseating granulomas, usually < 2 mm in size
o Fine-needle biopsy of palpable or radiologically
visible lesions has been proposed recently as reliable,
cost-effective method for diagnosis of sarcoidosis
Demographics
• Age: 20-40 years
• Gender: M:F = 1:3
• Ethnicity: African-Americans:Caucasians = 14:1
Natural History & Prognosis
• Variable natural history; small granulomas may heal
without a trace, but confluent granulomas can result
in extensive, irregular scarring
• 3 well-recognized clinical syndromes: Chronic
intrahepatic cholestasis, portal hypertension &
Budd-Chiari syndrome are quite sporadic
o Occlusion of intrahepatic portal vein branches by
granulomatous inflammation probably accounts for
development of portal hypertension in some cases
o Granulomatous cholangitis leading to ductopenia
seems to be underlying pathogenetic mechanism of
chronic cholestatic syndrome of sarcoidosis
• Life-threatening situations are extremely rare
o May be due to failure of vital organs--Iungs, heart,
kidney, liver; & usually due to irreversible fibrosis
• Complications: Hepatic failure is due to intrahepatic
cholestasis & portal hypertension
• Rare complications: Budd-Chiari syndrome &
obstructive jaundice, attributable to hepatic hilar
lymphadenopathy or strictures of bile ducts
• Sarcoidosis-lymphoma syndrome: Sarcoidosis
complicated by non-Hodgkin lymphoma (infrequent
but well-described event)
• Prognosis: 10% mortality (cor pulmonale/CNS/lung
fibrosis/liver cirrhosis)
Treatment
• Spontaneous remission
• Corticosteroids, anti-inflammatory agents & cytotoxic
drugs: Prednisone, chloroquine, methotrexate
• Follow-up US &/or CT show good correlation with
improvement in liver enzyme levels after steroid
therapy & normalization of liver pattern
I DIAGNOSTIC CHECKLIST
Consider
• Most granulomas at pathology are small; imaging
studies depict nodular changes in only approximately
one-third of affected patients
• Marked abdominal CT findings are uncommon in
sarcoidosis & correlate with disease activity but not
chest radiographic stage
o Presence of hepatic nodules at imaging is not
correlated with advanced pulmonary disease
• Important to differentiate sarcoidosis from other
causes of hepatic granulomas, such as infectious
diseases, in which treatment with corticosteroids could
be fatal
• Sarcoidosis can appear in an atypical fashion, & it
should be kept in mind in differential diagnosis of
focal & diffuse liver disease
• US can play useful role in reaching diagnosis &
monitoring response to treatment, despite its
negligible usefulness for most patients with sarcoidosis
who have typical pulmonary & nodal manifestations
Image Interpretation Pearls
• Do not assume that heterogeneous hepatomegaly &
abdominal lymphadenopathy are always malignant
• Biopsy is key to diagnosis
I SELECTED REFERENCES
1. Amarapurkar DN et al: Hepatic sarcoidosis. Indian J
Gastroenterol. 22(3):98-100, 2003
2. Sartori S et al: Sonographically guided biopsy and
sonographic monitoring in the diagnosis and follow-up of
2 cases of sarcoidosis with hepatic nodules and
inconclusive thoracic findings. J Ultrasound Med.
21(9):1035-9,2002
3. Scott GC et al: CT patterns of nodular hepatic and splenic
sarcoidosis: a review of the literature. J Com put Assist
Tomogr. 21(3):369-72, 1997
4. Warshauer DM et al: Abdominal CT findings in sarcoidosis:
radiologic and clinical correlation. Radiology. 192(1):93-8,
1994
5. Britt AR et al: Sarcoidosis: abdominal manifestations at CT.
Radiology. 178(1):91-4, 1991
 HEPATIC SARCOIDOSIS

I IMAGE GALLERY 1
59
Typical
(Left) Axial CECT shows
innumerable hypodense
nodules in liver and spleen.
(Right) Axial CECT shows
multiple hypodense nodules
and some fibrosis, suggested
by irregular contour of the
liver.

(Left) Axial T2WI MR shows

heterogeneous liver with
nodular surface and
parenchyma secondary to
underlying sarcoidosis.
Granulomas appear
hypointense to surrounding
liver parenchyma (arrow).
(Right) Axial CECT shows
cirrhotic morphology (wide
fissures, enlarged caudate
and lateral segment), ascites
and multinodular liver;all
due to sarcoidosis.

Typical
(Left) Axial CECT shows
multinodular liver and spleen
with splenic lesions
substantially larger and more
evident. (Right) Sagittal
sonogram shows
heterogeneous liver with
innumerable subcentimeter
hypoechoic nodules (arrow),
ascites.
 HEPATIC AV MALFORMATION (O-W-R)
1
60

Graphic shows dilated hepatic veins and arteries with Axial CECT in late arterial phase shows early filling of
direct intraparenchymal communication through dilated hepatic veins and innumerable irregular vascular
tortuous vascular channels. channels connecting arteries and veins, more apparent
in lateral segment.

o Focal sinusoidal ectasia, arteriovenous (AV)shunts

ITERMINOLOGY
Abbreviations and Synonyms
• Arterio-venous malformation (AVM)
• Hereditary hemorrhagic telangiectasia (HHT);
Osler-Weber-Rendu disease (O-W-R)
Definitions
• Hereditary multi organ disorder that results in
fibrovascular dysplasia with development of
telangiectasias & AVMs
• Direct connection between arteries & veins with
absence of capillaries (telangiectasias are small AVMs)
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Dilated hepatic/portal veins and
arteries with direct intra parenchymal communication
through tortuous vascular channels
• Location: Skin, lungs, liver, mucus membranes,
gastrointestinal tract (GIT) & brain
• Size: Few mm to several cm; composed of punctate
spots with diameter of 1-4 mm diameter
• Key concepts
through abnormal direct communications between
arterioles & ectatic sinusoids, & portovenous shunts
due to frequent & large communications between
portal veins & ectatic sinusoids
CT Findings
• CECT
o Prominent extra-hepatic or both extra- &
intra-hepatic hepatic artery; dilated hepatic and/or
portal veins
o Early filling of portal venous or hepatic venous
trunks on helical CT indicates AV shunt (52%)
o Intrahepatic AVfistulas may also be identified
o ± Focal bile duct dilatation (external compression)
o Arterial phase: Tortuous, irregular & poorly defined
hepatic arterial branches
o Venous phase: Early opacification of ectatic veins
o Parenchymal phase: Hepatogram is heterogeneous
o Transient hepatic parenchymal enhancement (65%);
indirect sign of presence of arterioportal shunt
o Telangiectases (63%): Small vascular spots; more
readily recognizable on reconstructed multi planar
reformatted & MIP images
o Large confluent vascular masses (25%); appear as
larger vascular pools with early & persistent
enhancement during arterial phases
• These are large areas of multiple telangiectases
that coalesce or large shunts

DDx: Hepatic Arteriovenous (AV) Shunts

'- .•....
,.• I
.....
~_l: .'~

...
, ~.. '
:
~
.~
~
AV Shunt in Cirrhosis Post Bx AV Fistula HCC with AV Shunting Budd-Chiari Syndrome
 HEPATIC AV MALFORMATION
Key Facts
Terminology
• Hereditary multiorgan disorder that results in
fibrovascular dysplasia with development of
telangiectasias & AVMs
Imaging Findings
• Best diagnostic clue: Dilated hepatic/portal veins and
arteries with direct intraparenchymal communication
through tortuous vascular channels
• Numerous irregular areas of dense contrast
accumulation throughout liver parenchyma
Top Differential Diagnoses
• Sinusoidal & arterial changes in cirrhosis
• Traumatic intrahepatic arteriovenous fistulas
• Arteriovenous shunting with tumors
• Budd-Chiari syndrome
• CTA: Multiple ectatic vessels & AVMs
MR Findings
• TlWI
o Network of vessels with flow voids on spin echo
o Telangiectasias: Hypo- to isointense
• STIR
o Best for extent of malformations
• MRA alone may underestimate extent
• T1 C+
o Telangiectasias: Small homogeneously enhancing
o Early enhancement of peripheral portal veins &
wedge-shaped transient parenchymal enhancement
during hepatic arterial phase
o Dynamic gradient echo after GD-DTPA for analysis
of filling kinetics
·MRA
o Number & size of feeding arteries & draining
veins/depict map of anomalous vessels
o Flow tagging makes it possible to define flow
direction & to estimate flow velocity
o Time between early arterial phase & enhancement of
malformation used to distinguish high- & low-flow
lesions
• High-flow AVMs show early, intense
enhancement
• Venous malformations: Either not visible or show
late enhancement of veins
Ultrasonographic Findings
• Real Time: Dilated hepatic arteries, multiple
arteriovenous malformations & abnormal hepatic
echogenicity
• Pulsed Doppler
o High hepatic artery velocities = 153 +/- 65.2 cm/sec
o Hepatic artery to portal vein shunts cause pulsatility
of portal flow with phasic or continuous reversal
o Hepatic artery to hepatic vein shunts show
significant changes in Doppler waveform of hepatic
vein; only in severe stages of disease
• Color Doppler: Tangled masses of enlarged tortuous
arteries or multiple aneurysms of hepatic arteriole
branches within liver
(O-W-R)
1
Pathology 61
• Pulmonary AVMs are more likely to cause symptoms
& complications in patients with O-W-R
• Hepatic angiodysplastic vascular changes include
telangiectasias, cavernous hemangiomas, aneurysm of
intraparenchymal branches of hepatic artery &
intraparenchymal hepato-portal & arterio-venous
fistulas
Clinical Issues
• Clinical profile: Diagnostic criteria: Family history,
epistaxis, mucocutaneous telangiectasias, AVMs
• Complications: High-output congestive heart failure,
portal hypertension, hepatic porto systemic
encephalopathy, biliary ischemia & liver failure
Angiographic Findings
• Conventional
o Tortuous dilated hepatic arterial branches
o Numerous irregular areas of dense contrast
accumulation throughout liver parenchyma
o Diffuse angiectases & diffuse mottled capillary blush
o Early filling of hepatic or portal vein in shunts
o Appearance depends on stage of development: All
findings are present if shunting is severe
• Isolated parenchymal modifications are found
only in case of mild intrahepatic shunt
Imaging Recommendations
• Best imaging tool
o Color Doppler as non-invasive screening modality
o Angiography useful to delineate extent before
surgical or angiographic interventions
o Multi-detector row helical CT & multi planar &
angiographic reconstructions depict complex
hepatic vascular alterations typical of HHT
• Protocol advice: Combine dynamic contrast-enhanced
3D gradient-echo MRI with STIR sequences
I DIFFERENTIAL DIAGNOSIS
Sinusoidal & arterial changes in cirrhosis
• Dilation & 1 number of hepatic arteries; 2 or 3
branches run parallel: "Duplication" &" trifurcation"
• "Corkscrew" appearance produced by combination of 1
in arterial flow & j in liver size
• Transsinusoidal arterioportal shunting in advanced
cirrhosis; intra segmental hepatofugal portal flow
Traumatic intrahepatic arteriovenous fistulas
• Causes: Biopsy (Bx), transhepatic biliary drainage,
blunt or penetrating injury, rupture of hepatic
aneurysm into portal vein
• Dilatation of feeding artery, early opacification of
draining vein & poor visualization of artery distal to
fistula due to steal of blood by fistula
 HEPATIC AV MALFORMATION
1 Arteriovenous shunting with tumors
62 • With hepatomas & metastatic tumors, shunting
suggests venous invasion by tumors
Budd-Chiari syndrome
• Obstruction of hepatic venous outflow; collateral
channels develop between hepatic, portal & systemic
venous systems
• CT: Heterogeneous parenchymal density with
periportal & peripheral enhancement, caudate lobe
enlargement
• Arteriography: Stretching & attenuation of
intrahepatic arteries & inhomogeneous parenchyma
I PATHOLOGY
General Features
• General path comments
o Pathogenesis is not known, involves several factors:
Special formation of venules, capillaries & arterioles,
abnormal perivascular connective tissue &
endothelial cells
o Small telangiectasis = focal dilatation of post
capillary venules with prominent stress fibers in
pericytes along luminal borders
o Fully developed telangiectasis = markedly dilated &
convoluted venules with excessive layers of smooth
muscle without elastic fibers directly connecting to
dilated arterioles
• Genetics
o Autosomal dominant trait & exhibits high
penetrance & great genetic heterogeneity
o HHT phenotypes: HHT1, mutations at chromosome
9 alter protein endoglin & in HHT2, mutations at
chromosome 12 alter protein activine or ALK-1
• Etiology: Gene encoding a protein that binds
transforming growth factor
• Epidemiology: 10-20:100,000
• Associated abnormalities
o 60% of pulmonary AVMs occur in patients with
O-W-R; 15% of patients with O-W-R will have
pulmonary AVMs
o Pulmonary AVMs are more likely to cause symptoms
& complications in patients with O-W-R
Gross Pathologic & Surgical Features
• Hepatic angiodysplastic vascular changes include
telangiectasias, cavernous hemangiomas, aneurysm of
intraparenchymal branches of hepatic artery &
intraparenchymal hepato-portal & arterio-venous
fistulas
Microscopic Features
• Clusters of dilated small blood vessels, lined by a
single layer of endothelium
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic; anemia due to recurrent bleeds
(O-W-R)
o Multiple mucocutaneous telangiectasias with
multi organ involvement
• Nasal mucosa: Recurrent epistaxis
• Skin: Lips, tongue, palate, face, conjunctiva & nail
bed
• CNS (cerebral or spinal AVM): Seizures,
paraparesis, subarachnoid hemorrhage
• Gastrointestinal: GI bleed & angiodysplasias
• Pulmonary: Cyanosis, polycythemia, dyspnea on
effort, clubbing, bruit
o Liver: Often asymptomatic; rarely causes
parenchymal fibrosis, biliary ischemia, liver failure
• Hepatic parenchyma: Fibrosis, atypical cirrhosis,
chronic active hepatitis (rare)
• Clinical profile: Diagnostic criteria: Family history,
epistaxis, mucocutaneous telangiectasias, AVMs
Demographics
• Age: Onset: Adult life; hepatic involvement diagnosed
10-20 years after first appearance of telangiectasias
• Gender: M = F
Natural History & Prognosis
• Complications: High-output congestive heart failure,
portal hypertension, hepatic porto systemic
encephalopathy, biliary ischemia & liver failure
o With extrahepatic involvement: Hemoptysis,
hemothorax, cerebrovascular accident, cerebral
abscess
• Prognosis: Usually good
Treatment
• Supportive: Iron/blood transfusion
• Hepatic arterial coil embolization, surgical ligation of
hepatic artery, liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Due to high prevalence of pulmonary & cerebral
AVMs, all patients with HHT should be screened for
their presence
• Existence of arteriosystemic or arterioportal
intrahepatic shunts that are not correlated with other
pathologic conditions (Le., neoplasia, cirrhosis,
trauma) should raise suspicion of HHT
• Relatives of patients with HHT should be investigated
for presence of disease
I SELECTED REFERENCES
1. Hashimoto M et al: Angiography of hepatic vascular
malformations associated with hereditary hemorrhagic
telangiectasia. Cardiovasc Intervent Radiol. 26(2):177-80,
2003
2. Larson AM: Liver disease in hereditary hemorrhagic
telangiectasia. J Clin Gastroenterol. 36(2):149-58, 2003
3. Hatzidakis AA et al: Hepatic involvement in hereditary
hemorrhagic telangiectasia (Rendu-Osler-Weber disease).
Em Radiol. 12 SuppI3:S51-5, 2002
4. Matsumoto S et al: Intrahepatic porto-hepatic venous
shunts in Rendu-Osler-Weber disease: imaging
demonstration. Em Radiol. 2003
 HEPATIC AV MALFORMATION (O-W-R)
I IMAGE GALLERY 1
63
Typical
(Left) Thick section axial
CECT during arterial phase
shows dilated hepatic artery,
early opacification of large
left portal vein and large
arterio-portal fistula (arrow)
in lateral segment. (Right)
Color Doppler sonography
shows tangled vascular mass
along lateral segment of the
liver, representative of
intraparenchymal
arterio-portal shunt.

Typical
(Left) Thick section axial
CECT shows dilated hepatic
artery and portal vein as well
as early enhancement of
diffusely dilated intrahepatic
veins. (Right) Thick section
axial CECT shows massive
dilatation and early filling of
hepatic and portal veins due
to vascular malformations.

(Left) Angiography shows

dilated, tortuous hepatic
arteries and early filling of
innumerable telangiectasias.
(Right) CECT (arterialphase)
shows heterogeneous
enhancement, early filling of
dilated hepatic veins (open
arrows), small telangiectasias
(curved arrow), and larger
confluent vascular masses
(arrow).
 BUDD-CHIARI SYNDROME
1
64

Axial CECT shows dysmorphic liver, ascites and Axial CECT shows subcutaneous collaterals and ascites.
subcutaneous venous collaterals. Central liver enhances Liver enhances heterogeneously Note thrombosed IVC
normally and is hypertrophied while peripheral liver is (arrow) and intrahepatic collateral (curved arrow), a
hypodense and scarred. veno-venous shunt.

ITERMINOLOGY • Other general features

o Budd-Chiari is a rare syndrome
Abbreviations and Synonyms o Classified based on cause & pathophysiology
• Hepatic venous outflow obstruction o Primary type: Congenital, injury, infection
• Secondary type: Usually due to thrombosis
Definitions
• Global or segmental hepatic venous outflow CT Findings
obstruction (at level of large hepatic veins or • NECT
suprahepatic segment of IVC) o Acute phase
• Diffusely hypodense enlarged liver
• Narrowed IVC + hepatic veins & ascites
I IMAGING FINDINGS • Hyperdense IVC & hepatic veins (due to 1
attenuation of thrombus)
General Features o Chronic phase
• Best diagnostic clue: "Bicolored" hepatic veins (due to • Diffusely hypodense liver
intrahepatic collateral pathways) pathognomonic of • Non-visualization of IVC & hepatic veins
chronic Budd-Chiari on color Doppler sonography • Hypertrophy of caudate lobe
• Location: Hepatic veins, IVC or centrilobar veins • Atrophy of peripheral segments
• Size • Ratio of caudate width to right lobe: 2': 0.55:1
o Acute phase: Markedly enlarged liver • CECT
o Chronic phase o Acute phase
• Right & left lobes of liver: Atrophy • Classic "flip-flop" pattern is seen
• Caudate lobe: Hypertrophy • Early enhancement of caudate lobe & central
• Key concepts portion around IVC, with decreased liver
o Hepatic venous outflow obstruction enhancement peripherally
o Characteristic finding of Budd-Chiari syndrome • Later decreased enhancement centrally with
• Large regenerative nodules (nodular regenerative increased enhancement peripherally
hyperplasia) in dysmorphic liver • Narrowed hypodense hepatic veins & IVC with
hyperdense walls

DDx: Dysmorphic liver with lobular Contour

Cardiac Cirrhosis Cirrhosis Sclerosing Cholangitis Sclerosing Cholangitis

 BUDD-CHIARI SYNDROME
Key Facts
Terminology
• Hepatic venous outflow obstruction
• Global or segmental hepatic venous outflow
obstruction (at level of large hepatic veins or
suprahepatic segment of IVC)
Imaging Findings
• Best diagnostic clue: "Bicolored" hepatic veins (due to
intrahepatic collateral pathways) pathognomonic of
chronic Budd-Chiari on color Doppler sonography
• Non-visualization of IVC & hepatic veins
• Hypertrophied caudate vein
• "Spider web" pattern of hepatic venous collaterals
Top Differential Diagnoses
• Hepatic cirrhosis
• Primary sclerosing cholangitis (PSC)
o Chronic phase
• Total obliteration of IVC & hepatic veins
• "Large regenerative nodules": Nodular regenerative
hyperplasia
• Enhancing 1-4 cm hyperdense nodules ±
hypodense ring
• CTA: Hepatic venous outflow obstruction
MR Findings
• TlWI
o Increased intensity of liver centrally with peripheral
heterogeneity
o Narrowed or absent hepatic veins & IVC
o Hyperintense nodules & enlarged caudate lobe
• T2WI
o Fail to visualize hepatic veins & IVC
o Isointense or hypointense regenerative nodules
• T2* GRE: Fails to show flow in hepatic veins or IVC
• Tl C+
o Tumor thrombus (rare cause) may show
contrast -enhancement
o Acute phase
• Involved parenchyma enhances less than
surrounding liver
o Congested liver with t water content
• Peripheral liver enhances less than central liver
due to increased parenchymal pressure &
decreased blood supply
o Chronic phase
• Enhancement is more variable & may be increased
• Nodules: Intense homogeneous enhancement
• MRA: Depicts thrombus & level of venous obstruction
Ultrasonographic Findings
• Real Time
o Hepatic veins
• Narrowed, not visualized or filled with thrombus
• Hypertrophied caudate vein
• Color Doppler
o Hepatic veins & IVC
• Absent or flat flow in hepatic veins
• Reversed flow in hepatic veins or IVC
1
Pathology 65
• Primary: Venous outflow membranous obstruction
• Secondary: Thrombotic; rarely nonthrombotic
• Type I: Occlusion of IVC ± hepatic veins
• Type II: Occlusion of major hepatic veins ± IVC
• Type III: Occlusion of small centrilobar veins
Diagnostic Checklist
• Rule out cirrhosis & primary sclerosing cholangitis
• Absent, reversed or flat flow in hepatic veins &
reversed flow in IVC on color-Doppler sonography
• Check for hypercoagulable conditions, prior
chemotherapy or marrow transplant
• "Bicolored" hepatic veins: Due to intrahepatic
collateral pathways
• Sensitivity: 87.5%
o Portal vein
• Slow hepatofugal flow: < 11 cm/sec
• Congestion index: > 0.1
o Hepatic artery: Resistive index;::: 0.75
Angiographic Findings
• Inferior venacavography or hepatic venacavography
o "Spider web" pattern of hepatic venous collaterals
o Thrombus in hepatic veins or IVC
o Long segmental compression of IVC
• Acute phase: Due to diffuse hepatomegaly
• Chronic phase: Hypertrophy of caudate lobe
o Hepatic arteries
• Acute phase: Narrowing, stretching, bowing
• Chronic phase: Dilated & arterioportal shunts
Imaging Recommendations
• Best imaging tool: Color Doppler sonography or
angiography
I DIFFERENTIAL DIAGNOSIS
Hepatic cirrhosis
• Hypertrophy: Caudate & lateral segment of left lobe
• Atrophy: Right lobe & medial segment of left lobe
• Varices, ascites, splenomegaly
• Patent hepatic veins & IVC
• Regenerative nodules
o Usually small in size compared to Budd-Chiari
o Cirrhotic nodules often have increased iron
• Usually hypovascular; decreased signal on T2WI
Primary sclerosing cholangitis (PSC)
• Chronic cholestatic disease of unknown cause
• 70% of cases: Associated with ulcerative colitis
• Bile ducts on cholangiography: Segmental strictures,
beading, pruning, nodular thickening, skip dilatations
• Atrophy/hypertrophy of lobes; ± cirrhotic changes
 BUDD-CHIARI
1 I PATHOLOGY
66 General Features
• General path comments
o Embryology-anatomy
• Primary type: Total or incomplete membranous
obstruction of hepatic venous outflow
• Deviations of complex embryologic process of IVC
• Etiology
o Primary: Venous outflow membranous obstruction
• Controversial etiology
• Congenital, injury or infection
o Secondary: Thrombotic; rarely non thrombotic
• Obstruction of central & sublobular veins:
Chemotherapy & radiation
• Obstruction of major hepatic veins:
Hypercoagulable states (e.g., oral contraceptives,
polycythemia, protein C deficiency)
• Obstruction of small centrilobular veins
(veno-occlusive disease): Bone marrow
transplantation & antineoplastic drugs
• Nonthrombotic: Hepatic & extrahepatic masses
• Epidemiology
o Primary (congenital-membranous type)
• Common in Japan, India, Israel & South Africa
o Secondary (thrombotic)
• Most common in Western countries
• Usually due to hypercoagulable state
o Secondary (nonthrombotic)
• 2nd most common in Western countries
Gross Pathologic & Surgical Features
• Acute phase
o Liver enlarged, congested
o Occlusion of hepatic veins & IVC
• Chronic phase
o Liver: Nodular, shrunken, may be cirrhotic
o Hypertrophy of caudate lobe & atrophy other lobes
Microscopic Features
• Centrilobular congestion, dilated sinusoids
• Fibrosis, necrosis & cell atrophy
Staging, Grading or Classification Criteria
• Classified into three types based on location
o Type I: Occlusion of IVC ± hepatic veins
o Type II: Occlusion of major hepatic veins ± IVC
o Type III: Occlusion of small centrilobar veins
• Defined as "veno-occlusive disease"
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute phase
• Rapid onset RUQ pain, tender liver, hypotension
o Chronic phase
• RUQ pain, hepatomegaly, splenomegaly
• Jaundice, ascites, varices
• Lab data
o Acute
• Liver function tests: Mild to markedly increased
• Clotting factors: Decreased
SYNDROME
o Chronic:
• Transaminases: Normal or moderately increased
• Albumin & clotting factors: Decreased
Demographics
• Age: Any age group
• Gender: Females more than males
Natural History & Prognosis
• Complications
o Acute: Liver failure, emboli from IVC thrombus
o Chronic: Variceal bleeding (cirrhosis), portal HTN
o Membranous obstruction of IVC
• Complicated by hepatocellular carcinoma in
20-40% cases in Japan & South Africa
• Prognosis
o Based on rate/degree of hepatic outflow obstruction
• Mild & moderate obstruction: Good
• Severe obstruction: Poor
o Acute early phase (good); acute late phase (poor)
o Chronic phase: Poor (with or without treatment)
o Veno-occlusive disease: Varies from fulminant
failure & death to mild with complete recovery
Treatment
• Medical management
o Steroids, nutritional therapy, anticoagulants
• Membranous occlusion of IVC & hepatic veins
o Balloon angioplasty, lasers, stent insertion
• TIPS (trans jugular intrahepatic porto systemic shunt)
• Surgical alternatives
o Membranotomy, membranectomy
o Cavoplasty, liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Rule out cirrhosis & primary sclerosing cholangitis
Image Interpretation Pearls
• Absent, reversed or flat flow in hepatic veins &
reversed flow in IVC on color-Doppler sonography
• Characteristic large benign regenerative nodules
• Check for hypercoagulable conditions, prior
chemotherapy or marrow transplant
I SELECTED REFERENCES
1. Brancatelli G et al: Benign regenerative nodules in
Budd-Chiari syndrome and other vascular disorders of the
liver: Radiologic-pathologic and clinical correlation.
RadioGraphies. 22: 847-62, 2002
2. Vilgrain V et al: Hepatic nodules in Budd-Chiari syndrome:
Imaging features. Radiology. 210: 443-50, 1999
3. Kane R et al: Diagnosis of Budd-Chiari syndrome:
comparison between sonography and MR angiography.
Radiology. 195(1):117-21, 1995
4. Millener P et al: Color Doppler imaging findings in
patients with Budd-Chiari syndrome: correlation with
venographic findings. AJRAm J Roentgenol. 161(2):307-12,
1993
5. Ralls PW et al: Budd-Chiari syndrome: detection with color
Doppler sonography. AJRAm J Roentgenol. 159(1):113-6,
1992
 BUDD-CHIARI SYNDROME

I IMAGE GAllERY 1
67

(Left) Axial CECT shows

caudate hypertrophy,
peripheral scarring and
heterogeneous
enhancement. (Right)
Hepatic venography shows
no patency of hepatic veins;
filling of collateral veins and
"spider web" pattern of
intrahepatic collateral
vessels.

Typical
(Left) Axial CECT shows
caudate hypertrophy, large
caudate collateral vein
(arrow), and peripheral
atrophy and heterogeneity.
(Right) Color Doppler US
shows large "bicolored"
intrahepatic collateral vein.

Typical
(Left) Axial CECT in arterial
phase shows dysmorphic
liver;subcutaneous
collaterals and ascites. Also
note hypervascular nodules
(arrows), the largest of
which resembles FNH, with
central scar (open arrow).
(Right) Axial CECT in portal
venous phase shows less
apparent hypervascular
nodules (arrows) nearly
isodense to liver.
 PASSIVE HEPATIC CONGESTION
1
68

Graphic shows massive diffuse dilatation of hepatic Axial CECT in arterial phase shows early filling, by reflux
veins and mildly heterogeneous liver parenchyma. through heart, of dilated hepatic veins and IVC.

•Congestive heart failure (CHF)

ITERMINOLOGY •Constrictive pericarditis
Abbreviations and Synonyms •Tricuspid insufficiency
• Congested liver in cardiac disease •Right heart failure (e.g., pulmonary artery
obstruction caused by lung cancer)
Definitions o Characteristic sign on physical exam
• Definition: Stasis of blood within liver parenchyma as • Hepatojugular reflux
a result of impaired hepatic venous drainage
CT Findings
• Early enhancement of dilated IVC & hepatic veins
I IMAGING FINDINGS o Due to contrast reflux from right atrium into IVC
• Heterogeneous, mottled, reticulated mosaic
General Features parenchymal pattern
• Best diagnostic clue: Dilated hepatic veins with • Linear & curvilinear areas of poor enhancement
to-and-fro blood flow on color Doppler o Due to delayed enhancement of small &
• Location: Liver, hepatic veins & IVC medium-sized hepatic veins
• Size • Peripheral large patchy areas of poor/delayed
o Acute phase enhancement
• Increase in liver size • Periportal low-attenuation (perivascular lymphedema)
o Chronic phase • Decreased attenuation around intrahepatic IVC
• Decrease in liver size ("cardiac cirrhosis") • Hepatomegaly & ascites
• Key concepts • Chest findings
o Manifestations of liver in cardiac disease o Cardiomegaly
• Acute or early manifestation: Enlarged, o ± Pericardial or pleural effusions
heterogeneous liver
MR Findings
• Chronic or late manifestation: Small cirrhotic liver
(may resemble cirrhosis of other causes) • T2WI: Periportal high signal intensity (periportal
edema)
o Passive hepatic congestion usually secondary to

DDx: Hepatomegaly with Heterogeneous Enhancement

Budd-Chiari Syndrome Budd-Chiari Syndrome Cirrhosis with Steatosis Acute Hepatitis

 PASSIVE HEPATIC CONGESTION
Key Facts
Terminology
• Congested liver in cardiac disease
• Definition: Stasis of blood within liver parenchyma as
a result of impaired hepatic venous drainage
Imaging Findings
• Best diagnostic clue: Dilated hepatic veins with
to-and-fro blood flow on color Doppler
• Early enhancement of dilated IVC & hepatic veins
• Cardiomegaly
• ± Pericardial or pleural effusions
• Dilated IVC/hepatic veins; hepatomegaly; ± ascites
Top Differential Diagnoses
• Budd-Chiari syndrome
• Hepatic cirrhosis with steatosis
• Acute viral hepatitis
• T2* GRE: Slow or even absent ante grade flow within
IVC
• T1 C+
o Liver enhancement pattern
• Reticulated mosaic pattern of low signal intensity
linear markings
• Within 1-2 minutes liver becomes more
homogeneous
o Hepatic veins & suprahepatic IVC
• Early enhancement due to reflux from atrium
o Portal vein
• Diminished, delayed or absent enhancement
o Fast low-angle shot (FLASH)contrast-enhanced MR
images
• Early reflux of contrast into dilated hepatic veins
&IVC
• MRA: Slow or absent ante grade flow within IVC
Ultrasonographic Findings
• Real Time
o Dilated IVC/hepatic veins; hepatomegaly; ± ascites
o Diameter of hepatic vein
• Normal: 5.6 to 6.2 mm
• Mean diameter: 8.8 mm (in passive congestion)
• Increases up to 13 mm with pericardial effusion
• Color Doppler
o Spectral velocity pattern (lVC & hepatic veins)
• Loss of normal triphasic flow pattern
• Spectral signal may have an "M" shape
• Cardiac cirrhosis: Flattening of Doppler wave form
in hepatic veins
o Spectral velocity pattern (portal vein)
• Increased pulsatility of portal venous Doppler
signal
• Normal continuous flow pattern
• Mild respiratory variation
o To-and-fro motion in hepatic veins & IVC
o Tricuspid regurgitation: Normal triphasic hepatic
vein shows
• Decrease In size of ante grade systolic wave
• Systolic/diastolic flow velocity ratio less than 0.6
(normal more than 4.0)
1
Pathology 69
• CHF, right heart failure, constrictive pericarditis
• Increased right atrial central venous pressure
• Pressure transmitted to IVC & hepatic veins
• Enlarged reddish-purple color liver
• "Nutmeg liver"
Clinical Issues
• Liver enlarged, tender
• Positive hepatojugular reflux
• Clinical profile: A cardiac disease patient with
hepatomegaly & positive hepatojugular reflux
Diagnostic Checklist
• Differentiate acute Budd-Chiarisyndrome, acute viral
hepatitis from acute passive hepatic congestion, &
viral or alcoholic cirrhosis from cardiac cirrhosis
Imaging Recommendations
• Best imaging tool: Color Doppler sonography
• Protocol advice: Bi-phasic CT or MR to evaluate extent
of liver damage
I DIFFERENTIAL DIAGNOSIS
Budd-Chiari syndrome
• Global or segmental hepatic venous outflow
obstruction at level of hepatic veins or IVC
• Acute phase
o Diffuse hypodense enlarged liver
o Narrowed IVC or hepatic veins & ascites
o Classic "flip-flop" pattern is seen
• Early enhancement of caudate lobe & central
portion around IVC
• Decreased enhancement peripherally
• Later decreased enhancement centrally &
increased enhancement peripherally
• Chronic phase
o Nodular regenerative hyperplasia ("large regenerative
nodules")
o Enhancing 1 to 4 cm hyperdense nodules
• Color Doppler
o Absent or flat or reversed flow in hepatic veins
o Bicolored" hepatic veins
• Due to intrahepatic collateral pathways
• Pathognomonic of chronic phase of Budd-Chiari
Hepatic cirrhosis with steatosis
• Nodular liver contour
• Hepatic veins: Normal caliber & flow pattern
• Portal vein: May be large; possible hepatofugal flow
• Atrophy of right lobe & medial segment of left lobe
• Enlarged caudate lobe & lateral segment of left lobe
• Regenerative nodules are hypovascular
• Diagnosis: Biopsy & histology
Acute viral hepatitis
• Hepatomegaly
• Periportal hypodensity (fluid/lymphedema)
• Hepatic & portal veins: Normal caliber & flow pattern
 PASSIVE HEPATIC CONGESTION
1 • Gallbladder wall thickening • Usually not made clinically because signs &
• "Starry-sky" appearance on sonography symptoms of cardiac failure overshadow those of
70 • Acute HBV liver disease
o May present with serum sickness-like syndrome • Clinical profile: A cardiac disease patient with
• Urticaria/ arthri tis/vasculi tis/ glomerulonephritis hepatomegaly & positive hepatojugular reflux
• Lab data
o Markedly elevated liver function tests Demographics
o Elevated serologic markers • Age: Any age group
• Gender: M = F
Natural History & Prognosis
I PATHOLOGY • Complications
General Features o Hepatic failure
o Cardiac cirrhosis
• General path comments
o CHF, right heart failure, constrictive pericarditis • Prognosis
o Increased right atrial central venous pressure o Acute phase: Good
o Chronic phase: Poor
o Pressure transmitted to IVC & hepatic veins
o Engorgement & dilatation of hepatic sinusoids with Treatment
blood • Acute or early phase
o Enlarged liver o Full recovery once patient's cardiac disease is
• Etiology corrected
o Congestive heart failure (CHF) • Chronic or late phase
o Constrictive pericarditis o Cardiac cirrhosis may be irreversible, even with
o Pericardial effusion correction of cardiac function
o Right-sided valvular diseases
• Tricuspid & pulmonary
o Cardiomyopathy I DIAGNOSTIC CHECKLIST
Gross Pathologic & Surgical Features Consider
• Enlarged reddish-purple color liver
• Differentiate acute Budd-Chiari syndrome, acute viral
• "Nutmeg liver" hepatitis from acute passive hepatic congestion, &
o Congestion of central veins
viral or alcoholic cirrhosis from cardiac cirrhosis
o Congestion of centrilobular hepatic sinusoids
Image Interpretation Pearls
Microscopic Features
• Cardiomegaly
• Acute or early phase
• Inferior venacava & hepatic veins
o Centrilobular congestion
o Dilated & early enhancement (due to reflux)
o With or without sinusoidal dilatation
o To-and-fro motion on color Doppler
• Chronic or late phase o Loss of normal triphasic velocity flow pattern
o Atrophy
o Centrilobular necrosis
o Fibrosis
o Finally sclerosis
I SELECTED REFERENCES
1. Gore RM et al: Passive hepatic congestion: cross-sectional
imaging features. A]R Am] Roentgenol. 162(1):71-5, 1994
2. Abu-Yousef MM et al: Pulsatile portal vein flow: a sign of
ICLINICAL ISSUES tricuspid regurgitation on duplex Doppler sonography. A]R
Am] Roentgenol. 155: 785-788, 1990
Presentation 3. Holley HC et al: Inhomogeneous enhancement of liver
• Most common signs/symptoms parenchyma secondary to passive congestion:
o Liver enlarged, tender contrast-enhanced CT. Radiology. 170(3 Pt 1):795-800,
o Right upper quadrant pain due to stretching of 1989
Glisson capsule 4. Moulton]S et al: Passive hepatic congestion in heart
o Positive hepatojugular reflux failure: CT abnormalities. A]R Am] Roentgenol. 151:
o Pulsatile liver in acute phase 939-942, 1988
5. Tani I et al: MR imaging of diffuse liver disease. A]R Am]
o Splenomegaly in late phase
Roentgenol. 2000
o Rarely cardiomyopathy patient may present with
hepatic failure before cardiac disease is diagnosed
o Lab data
• Acute: Mild abnormal liver function tests (LFT)
• Chronic: Grossly abnormal LFT
o Diagnosis
• Based on radiological, pathological & clinical
findings
 PASSIVE HEPATIC CONGESTION

I IMAGE GALLERY 1
71
Typical
(Left) Increased pulsatility of
portal vein (MPV) Doppler
signal is demonstrated in this
patient with passive hepatic
congestion secondary to
tricuspid insufficiency.
(Right) Axial grayscale US
shows dilated hepatic veins
and IVC in a patient with
passive hepatic congestion.

Typical
(Left) Axial CECT shows
chronic constrictive
pericarditis with soft tissue
and calcified thickening of
pericardium (arrows),
deviation of interventricular
septum. (Right) Axial CECT
in portal venous phase
shows mottled enhancement
of liver; halo of lymphedema
around we. This patient
presented with passive
hepatic congestion
secondary to constrictive
pericarditis.

Typical
(Left) Axial CECT shows
typical changes from cardiac
cirrhosis. Liver is small and
dysmorphic with
heterogeneous
enhancement. (Right) Axial
CECT shows dysmorphic
liver with atrophic right lobe,
hypertrophied lateral
segment and heterogeneous
enhancement. Ascites.
 HELLP SYNDROME
1
72

Axial CECT shows massive hemoperitoneum, liver Axial CECT shows hemoperitoneum. Normal gravid
parenchymal hemorrhage, and active extravasation uterus,placenta (arrow),and full term fetus.
(arrows) in a female patient during third trimester
pregnancy.

o Based on classification of American college of

ITERMINOLOGY Obstetricians/Gynecologists
Abbreviations and Synonyms • Bilirubin: More than 1.2 mg/dL
• Hemolysis, elevated liver enzymes, low platelets • Lactate dehydrogenase: More than 600U /L
(HELLP) • Aspartate aminotransferase: More than 70U /L
• Platelet count: Less than lOO,OOO/mm3
Definitions o Radiologically
• HELLP syndrome: A severe variant of preeclampsia • US features may be seen before increase in
biological markers (41 % cases)
• Helps in differentiating from other medical &
I IMAGING FINDINGS surgical conditions

General Features CT Findings

• Best diagnostic clue: Intrahepatic or subcapsular fluid • Liver hematomas
collection (hematoma) on US or CT o Well-defined hyperdense or hypodense
• Location o Nonenhancing
oLiver o Acute: Hyperattenuating (first 24-72 hours)
• Subcapsular or intraparenchymal o Chronic: Decreased attenuation (after 72 hours)
• Key concepts o Location: Subcapsular or intraparenchymal
o HELLP syndrome is a variant of toxemia in • Liver infarction
primigravidas o Small or large areas of low attenuation
• Usually preeclampsia & occasionally eclampsia o Usually peripheral & wedge-shaped
• Usually before birth in 3rd trimester (antepartum) • Occasionally active contrast extravasation sites or
• Occasionally soon after birth (postpartum) ascites
• Rarely seen in multiparous patients MR Findings
o Preeclampsia: Leading cause of maternal death in
USA & Europe • TlWI &T2WI
o Varied signal intensity depending on
• Degree & age of hemorrhage or infarct

DDx: Diffuse or Focal liver lesion with Hemorrhage

Hemorrhagic Adenoma Coagulopathy Hepatic Trauma Hepatic Trauma

 HELLP SYNDROME
Key Facts
Terminology
• Hemolysis, elevated liver enzymes, low platelets
(HELLP)
• HELLP syndrome: A severe variant of preeclampsia
Imaging Findings
• Best diagnostic clue: Intrahepatic or subcapsular fluid
collection (hematoma) on US or CT
• Acute: Hyperattenuating (first 24-72 hours)
• Chronic: Decreased attenuation (after 72 hours)
Top Differential Diagnoses
• Bleeding hepatic tumor (adenoma or HCC)
• Spontaneous bleed (coagulopathy)
• Hepatic trauma
• Degree of necrosis & steatosis
• Greater degree of edema & cellular necrosis
o T1WI: Low signal intensity
o T2WI: High signal intensity
Ultrasonographic Findings
• Real Time
o Liver hemorrhage or infarct
• Irregular or wedge shaped
• Increased echogenicity
• Location: Usually peripheral
o Periportal halo sign
• Hyperechoic thickening of periportal area
o Subcapsular hematoma
• Complex echogenicity of fluid collection
o Enlarged liver (predominantly right lobe)
o Occasionally ascites
Imaging Recommendations
• Best imaging tool: Ultrasonography
• Try to avoid CT because of radiation to fetus
I DIFFERENTIAL DIAGNOSIS
Bleeding hepatic tumor (adenoma or HCC)
• May bleed & present as fluid collection on US or CT
o Intraparenchymal or subcapsular
o Indistinguishable from HELLP syndrome
• Look for enhancing heterogeneous spherical hepatic
mass
Spontaneous bleed (coagulopathy)
• History of bleeding disorder
• Lab data: Abnormal bleeding time, clotting time,
prothrombin time & partial thromboplastin time
• On imaging
o Subcapsular or intrahepatic blood collection
o Occasionally active extravasation site may be seen
o Indistinguishable from HELLP syndrome without
history
Hepatic trauma
• History of injury to liver
1
Clinical Issues 73
• Acute epigastric & RUQ pain: Present in 90% of cases
• Clinical profile: Black female, primigravida with
features of preeclampsia & lab data showing findings
of hemolysis, elevated liver enzymes & low platelets
• Age: 2nd & 3rd decades
Diagnostic Checklist
• Rule out bleeding liver tumors like adenoma, HCC &
other liver pathologies like acute viral hepatitis &
acute fatty liver of pregnancy
• Clinically can mimic: Cholecystitis, biliary colic &
hepatitis
• Very rarely can occur without classic preeclampsia
triad: Hypertension, proteinuria & edema
• On imaging
o Intraparenchymal or subcapsular hematomas
o Lacerations, wedge-shaped areas of infarction
o Areas of active hemorrhage (isodense with vessels)
o Hemoperitoneum & pseudo aneurysm
Acute fatty liver of pregnancy
• Usually diffuse increase echogenicity of liver on US
• No intraparenchymal or subcapsular fluid collection
o Indicates no hemorrhage
I PATHOLOGY
General Features
• General path comments
o Pathophysiology of HELLP syndrome: Begins in
placental bed
• Arteriolar vasospasm ~ endothelial damage ~
fibrin deposition
• Platelet deposition on fibrin aggregates ~ decrease
number of circulating platelets
o RBC destruction by fibrin aggregates (hemolytic
anemia)
• Abnormal cells in peripheral smear (burr cells &
schistocytes)
• Elevated indirect bilirubin levels & anemia
o Hepatocyte destruction: Due to hepatic microemboli
(1 LFT levels)
• Distention of liver: Due to impeded blood flow
(RUQpain)
• Severe cases: Liver rupture & subcapsular
hematoma
o Pathophysiology of preeclampsia
• Primary site: Increased size of glomerular
endothelial cells
• Abnormal vasoconstriction + hyperactive vascular
smooth muscle
• Hypertension ~ proteinuria ~ edema
• Etiology
o Variant of severe preeclampsia & occasionally
eclampsia
o Preeclampsia & eclampsia: May be due to
 HELLP SYNDROME
1 • Coagulation abnormalities • Hypertension, diabetes, renal disease
• Hormonal factors • Complications
74 • Uteroplacental ischemia o Rupture of subcapsular hematoma
• Immune mechanisms o Hepatic necrosis
• Epidemiology o Disseminated intravascular coagulation (DIC)
o Prevalence o Abruptio placenta & renal failure
• 4-12% of patients with severe preeclampsia o Pulmonary edema & hypoglycemia
• 1 per 150 live births o Maternal mortality rate: 3.5% (due to liver rupture)
• Toxemia occurs in 6% of pregnancies • In delayed diagnosis & treatment
o Maternal mortality rate (MMR) in severe
preeclampsia due to HELLP syndrome is 3.5% Treatment
• Majority of cases: Supportive treatment
Gross Pathologic & Surgical Features • Standard treatment: Expeditious delivery of fetus
• Enlarged liver • Hepatic rupture & intra-abdominal bleeding
• Parenchymal hemorrhage or infarct o Surgery & selective embolization
• Subcapsular hematoma
Microscopic Features I DIAGNOSTIC CHECKLIST
• Periportal necrosis
• Microthrombi Consider
• Fibrin deposits in sinusoids & portal veins • Rule out bleeding liver tumors like adenoma, HCC &
other liver pathologies like acute viral hepatitis &
acute fatty liver of pregnancy
I CLINICAL ISSUES • Preeclampsia & HELLP syndrome
o Must be routinely checked for in all pregnant
Presentation women with acute abdominal (epigastric/RUQ) pain
• Most common signs/symptoms • HELLP syndrome
o Acute epigastric & RUQ pain: Present in 90% of o Clinically can mimic: Cholecystitis, biliary colic &
cases hepatitis
o Other signs/symptoms o Very rarely can occur without classic preeclampsia
• Malaise, nausea, vomiting, weight gain triad: Hypertension, proteinuria & edema
• Edema, headache, visual impairment, jaundice
o Preeclampsia: Classic triad Image Interpretation Pearls
• Hypertension, proteinuria & edema • Subcapsular hematoma
o Eclampsia o Complex echogenicity of fluid collection
• Classic triad of preeclampsia o Indistinguishable from fluid collections of bleeding
• Associated with convulsions & coma tumors like adenoma & HCC
o Clinical differential diagnosis • Look for heterogeneous enhancing spherical liver
• Viral hepatitis, gallstones, peptic ulcer tumors
• Pancreatitis, acute fatty liver • Liver hemorrhage or infarct
• Hemolytic uremic syndrome o Usually peripheral, irregular or wedge shaped
• Idiopathic thrombocytopenic purpura (ITP) o Increased echogenicity
• Clinical profile: African-American female, primigravida
with features of preeclampsia & lab data showing
findings of hemolysis, elevated liver enzymes & low I SELECTED REFERENCES
platelets 1. Di Salvo DN: Sonographic imaging of maternal
• Lab data complications of pregnancy. ] Ultrasound Med.
o Hemoglobin: Less than 11 g/dL 22(1):69-89,2003
o Bilirubin: More than 1.2 mg/dL 2. Suarez B et al: Abdominal pain and preeclampsia:
o Lactate dehydrogenase: More than 600 U/L sonographic findings in the maternal liver. ] Ultrasound
o Aspartate aminotransferase: More than 70 U/L Med. 21(10):1077-83; quiz 1085-6, 2002
o Platelet count: Less than 100,OOO/mm3 3. Casillas V] et al: Imaging of non traumatic hemorrhagic
hepatic lesions. Radiographies. 20(2):367-78, 2000
Demographics 4. Barton]R et al: Hepatic imaging in HELLPsyndrome
(hemolysis, elevated liver enzymes, and low platelet
• Age: 2nd & 3rd decades
count). Am] Obstet Gynecol. 174(6):1820-5; discussion
• Gender: Females 1825-7, 1996
• Ethnicity: More frequent in African-Americans 5. Peitz U et al: Sonographic findings of liver and gallbladder
in early hemolysis, elevated liver enzymes, and low platelet
Natural History & Prognosis count syndrome.] Clin Ultrasound. 21(8):557-60,1993
• Usually seen in primigravidas with preeclampsia 6. Kronthal A] et al: Hepatic infarction in preeclampsia.
• Occasionally seen in eclampsia patients Radiology. 177(3):726-8, 1990
• Maternal risk factors
o Nulliparity, young age (2nd & 3rd decades)
o African-American females; familial
o Underlying diseases
 HELLP SYNDROME
I IMAGE GALLERY 1
75
Typical
(Left) Oblique sonogram
shows peripheral lentiform
subcapsular hematoma with
low level echogenicity.
(Right) Sagittal sonogram in
patient with HELLP
syndrome shows peripheral
subcapsular hematoma.

Typical
(Left) Axial CECT shows
lentiform subcapsular
hematoma deforming lateral
contour of the liver and
hemoperitoneum. (Right)
Axial CECT shows large
hemoperitoneum and left
rectus sheath hematoma in
patient with HELLP
syndrome.

Typical
(Left) Axial CECT shows
heterogeneous liver
parenchyma consistent with
bleeding and/or infarction,
and hemoperitoneum.
(Right) Axial CECT shows
large areas of nonenhancing
liver, consistent with liver
infarction or "old"
hemorrhage.
 HEPATIC INFARCTION
1
76

Axial CECT following blunt trauma shows no Thick axial reconstructed CECT following liver
enhancement of anterior right lobe. Hepatic artery to transplantation.The hepatic artery is thrombosed at the
this segment is transected with acute extravasation anastomosis (arrow)with a largeliverinfarction.
(arrow).

o Wedge-shaped, rounded or oval, or irregularly

ITERMINOlOGY shaped low attenuation areas paralleling bile ducts
Abbreviations and Synonyms • Acute: Poorly demarcated low density lesions
• Liver infarction • Subacute: Confluent with more distinct margins
o ± Gas formation within sterile or infected infarcts
Definitions o Bile lakes seen as late sequela: Cystic changes
• Development of area of coagulation necrosis due to • CECT
local ischemia resulting from obstruction of o Lesions may have geographic segmental distribution
circulation to the areai most commonly by a thrombus with straight margins
or embolus o Lesions on NECT are more conspicuous after
enhancement (perfusion defects)
o Heterogeneous patchy enhancement with zones of
IIMAGING FINDINGS enhancement equal to liver parenchyma
o Components of lesions remaining hypodense on
General Features arterial, portal venous & delayed phase
• Best diagnostic clue: Peripheral wedge shaped, • Represent regions of necrotic tissue, hemorrhage
rounded or ovoid low attenuation areas with absent or or fibrous tissue with no or minimum
heterogeneous enhancement revascularization on histology
• Location o Lesions isoenhancing with surrounding liver
o Usually peripheral and wedge-shaped parenchyma on portal venous phase
o Can be more central and rounded • Histologically consistent with retained viable
• Size: Variable: Few mm to centimeters tissue or fibrotic tissue with revascularization
• Key concepts
o Usually single and focal MR Findings
o Can be multiple or diffuse • TIWI
o Small, relatively well-defined, hypointense
CT Findings o Edema of infarction: Lower signal intensity on Tl
• NECT • T2WI
o Heterogeneous appearance of liver parenchyma

DDx: Segmental/lobar Hypodensity or Decreased Enhancement

Focal Steatosis Focal Steatosis Hepatic Abscess Hepatic Abscess

 HEPATIC INFARCTION
Key Facts
Imaging Findings
• Best diagnostic clue: Peripheral wedge shaped,
rounded or ovoid low attenuation areas with absent
or heterogeneous enhancement
• Lesions may have geographic segmental distribution
with straight margins
• Lesions on NECT are more conspicuous after
enhancement (perfusion defects)
• CT or MR angiography can be diagnostic
• Catheter angiography may be necessary for diagnosis
and treatment
Top Differential Diagnoses
• Focal steatosis
• Hepatic abscess
o Edema of infarction: Higher signal intensity on T2
• Tl C+
o Heterogeneous parenchymal enhancement & areas
of perfusion defect
o Necrotic areas: Predominantly hypointense
compared with enhancing parenchyma in arterial,
portal venous & delayed phases
Ultrasonographic Findings
• Real Time
o In native liver
• Early: Hypoechoic lesion with indistinct margins
(when sufficient edema & round cell infiltration)
• Small bile duct cysts; large bile duct lakes (as
necrotic tissue is resorbed)
o In liver transplant recipients
• Geographic areas hypoechoic with preservation of
portal tracts (early sign of ischemia)
• Development of transient small hyperechoic
lesions (progression to true infarction)
• Color Doppler
o Hepatic artery thrombosis: Absence of normal
hepatic artery signal
• Hepatic artery thrombosis much more common
than portal vein thrombosis
o Transplant vasculature or portal vein thrombosis
o Porto-systemic shunting, collateral supply
Angiographic Findings
• Conventional: To confirm occlusion of hepatic artery
suggested by US, CT, or MR
Nuclear Medicine Findings
• Hepato biliary scan
o Peripheral wedge shaped sharply defined lesion
o Communication with bile lakes for infarcts
following transplantation
• Technetium sulfur colloid
o Photopenic area
• Cholescintigraphy: Communication with bile lakes for
infarcts following transplantation
Imaging Recommendations
• Best imaging tool
1
Pathology 77
• Rarity of hepatic infarction due to dual blood supply
from hepatic artery & portal vein & extensive
collateral pathways
Clinical Issues
• Infarction is serious complication of liver
transplantation with significant morbidity &
mortality & often requiring retransplantation
Diagnostic Checklist
• New focal liver lesion with branching pattern in
transplant patient with deteriorating function
suggests infarction (usually hepatic artery
thrombosis)
o Real time B mode & Doppler: Often first modality to
evaluate allograft dysfunction/post-operative
complications
o Triphasic helical CT with CT angiography
• CT or MR angiography can be diagnostic
• Protocol advice
o CECT + CTA or dynamic contrast-enhanced
gradient-echo & contrast-enhanced TI weighted
spin-echo images in axial plane with MRA
o Catheter angiography may be necessary for
diagnosis and treatment
I DIFFERENTIAL DIAGNOSIS
Focal steatosis
• May be geographic, wedge-shaped
• Preserved patent vessels; preservation of enhancing
vessels within "lesion"
• Characteristic suppression of signal on
opposed-phased GRE MR
Hepatic abscess
• Usually spherical, often septated
• Central non enhancing contents, enhancing rim
!PATHOLOGY
General Features
• General path comments
o Rarity of hepatic infarction due to dual blood supply
from hepatic artery & portal vein & extensive
collateral pathways
o In most cases superimposition of portal thrombosis
on hepatic arterial occlusion results in chronic
insufficiency & infarction
o Infarcted regenerative nodules in cirrhosis develop
from hypoperfusion of liver followed by ischemic
necrosis of nodules that are vulnerable to hypoxia
o Hepatic artery thrombosis in liver allograft recipients
more likely to lead to infarction as collateral supply
is severed during transplant
 HEPATIC INFARCTION
1 • Etiology
o Iatrogenic
78 • Cholecystectomy, hepatobiliary surgery,
intrahepatic chemoembolization, transjugular
intrahepatic porto systemic shunt (TIPS) procedure
o Liver transplantation
• Hepatic artery stenosis or thrombosis
o Blunt trauma
• Hepatic artery & portal vein laceration
o Hypercoagulable states
• Sickle cell/antiphospholipid antibody syndrome
o Vasculitis
• Polyarteritis, lupus, etc.
o Infection
• Rare "emphysematous hepatitis"
• Following sepsis & shock
• Epidemiology
o Hepatic infarction is uncommon
o Hepatic artery thrombosis following transplant
reported in 3% adults, 12% children or in 7-8% of
mixed population
Gross Pathologic & Surgical Features
• Liver at autopsy: Atrophic, hard & irregularly surfaced
• Focal, multiple necrotic areas, peripheral collapse of
parenchymal tissue with fibrosis
Microscopic Features
• Central congestion & centrilobular necrosis
surrounded by hemorrhagic rims
• Infarcted nodules have central core of amorphous
eosinophilic material representing remnants of
necrotic hepatocytes
o Cells with foamy cytoplasm representing
macrophages surround necrotic core
o Ultimate replacement by fibrovascular tissue
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Diagnosed at laparotomy, autopsy or imaging
o Asymptomatic, nonspecific: Right upper quadrant or
back pain, fever
o Massive infarction: Coma, ascites, jaundice, renal
failure
• Clinical profile
o Lab: Leukocytosis, abnormal liver function tests
o In pregnancy: Associated with hemolytic anemia
with elevated liver enzymes & low platelets (HELLP),
pre-eclampsia, eclampsia
Demographics
• Age: Any age group
• Gender: M = F
Natural History & Prognosis
• Parenchymal atrophy & scarring, progressive
liquefaction, or both; affects center of hepatic lobule
(venous) most prominently with relative sparing of
portal (arterial) end
• Infarction is serious complication of liver
transplantation with significant morbidity & mortality
& often requiring retransplantation
• Complications:
o Native liver: Liver failure, fibrosis
o Transplanted liver: Biliary strictures, bilomas, abscess
Treatment
• Options: Revascularization, retransplantation,
spontaneous resolution
I DIAGNOSTIC CHECKLIST
Consider
• Pre-TIPS evaluation of arterial supply to liver by
Doppler/ angiography; sufficient arterial perfusion
crucial to avoid infarction
• Post TIPS: If pain develops in right upper quadrant,
fever, shock & disseminated intravascular coagulation
• Recognize infarction as separate entity among
spectrum of pregnancy-related liver disorders to avoid
delay in diagnosis & treatment
• Ultrasound & CT suggest diagnosis, angiography often
necessary for confirmation
• Ischemia alone may produce "typical sonographic
features of infarction"; if recognized early enough, may
be reversible
Image Interpretation Pearls
• Preservation of portal tracts: Feature worthy of
emphasis as it helps differentiate infarction from other
causes of focal hypoechoic areas in post transplant
liver e.g., abscess, biloma or hematoma following
biopsy
• New focal liver lesion with branching pattern in
transplant patient with deteriorating function suggests
infarction (usually hepatic artery thrombosis)
I SELECTED REFERENCES
1. Blachar A et al: Acute fulminant hepatic infection causing
fatal "emphysematous hepatitis": case report. Abdom
Imaging. 27(2):188-90, 2002
2. Mayan H et al: Fatal liver infarction after transjugular
intrahepatic porto systemic shunt procedure. Liver.
21(5):361-4,2001
3. Quiroga S et al: Complications of orthotopic liver
transplantation: spectrum of findings with helical CT.
Radiographies. 21(5):1085-102, 2001
4. Kim T et al: Infarcted regenerative nodules in cirrhosis: CT
and MR imaging findings with pathologic correlation. A]R
Am] Roentgenol. 175(4):1121-5,2000
5. Smith GS et al: Hepatic infarction secondary to arterial
insufficiency in native livers: CT findings in 10 patients.
Radiology. 208(1):223-9, 1998
6. Holbert BI. et al: Hepatic infarction caused by arterial
insufficiency: spectrum and evolution of CT findings. A]R
Am] Roentgenol. 166(4):815-20, 1996
 HEPATIC INFARCTION
I IMAGE GALLERY 1
79
Typical
(Left) Axial CECT shows
minimal enhancement of
infarcted posterior right lobe.
Hepatic artery
pseudoaneurysm (arrow)
with embolic occlusion of
right artery. (Right) Axial
CECT in a patient with
iatrogenic infarction
following laparoscopic
cholecystectomy (occluded
right hepatic artery).

Typical
(Left) Axial CECT in liver
transplant recipient shows
rounded and branching
hypodense liver lesions due
to hepatic infarction and
biliary necrosis. Pigtail
catheter placed to drain
biloma. (Right) Celiac
arteriogram shows lack of
arterial blood supply to the
liver with occlusion of
hepatic artery (arrow) in
patient who had undergone
recent liver transplant.

Variant
(Left) Axial NECT shows gas
replacing right lobe of liver
due to spontaneous
infarction and infection in a
diabetic patient with sepsis.
(Right) Axial NECT using
lung window settings shows
nearly complete replacement
of liver parenchyma with gas
("emphysematous
hepatitis") and no apparent
purulent collections.
 PELIOSIS HEPATIS
1
80

CECT in a patient with AIDS and biopsy proven peliosis Delayed arterial phase of celiac arteriogram shows
due to Bartonella infection, Innumerable hypodense persistent "puddling" of contrast in innumerable
liver lesions with peripheral enhancement. Hypodense vascular hepatic lesions, Lesions resolved completely
porta hepatis nodes (arrow), with antibiotics

ITERMINOLOGY CT Findings
Abbreviations and Synonyms • NECT
o Multiple hepatic areas of low attenuation
• Hepatic peliosis
o CT findings differ with size of lesions, presence or
Definitions absence of thrombus within cavity & presence of
• Rare benign disorder causing sinusoidal dilatation & hemorrhage
presence of multiple blood filled lacunar spaces within • If peliotic cavities < 1 cm diameter, CT findings
liver may appear normal
• CECT
o Larger cavities communicating with sinusoids have
I IMAGING FINDINGS same attenuation as blood vessels
o Thrombosed cavities will have same appearance as
General Features non enhancing nodules
• Best diagnostic clue o Arterial phase: Early globular vessel-like
o Strong contrast-enhancement on delayed imaging enhancement
with "branching" appearance caused by vascular • Multiple small accumulations of contrast,
component hyperdense in center or periphery of lesion
o Spherical lesion with centrifugal or centripetal o Portal phase: Centrifugal or centripetal
enhancement enhancement without mass effect on hepatic vessels
• Location o Delayed phase: Late diffuse homogenous
o No preferential location within hepatic lobule hyperattenuation characteristic of phlebectatic type
o Spleen, bone marrow, lymph node, lungs, pleura, MR Findings
kidneys, adrenals, stomach, ileum
• Size: Varies from 1 mm to several centimeters • TIWI
o Hypointense
• Key concepts
o 1 Signal due to presence of subacute blood
o Irregularly shaped blood-filled hepatic cavities
suggestive of hemorrhagic necrosis
• T2WI

DDx: Heterogeneous Hypervascular Mass(es)

.~'.'t.' .•....
,~,'
/'

.~

_ .Ck.~,JI
- 'j!!
, .. '1'_::
I

i' ,"
,. -
"
,

Hepatic Adenoma Hepatic Hemangioma Atypical

, FNH Metastases
 PELIOSIS HEPATIS

Key Facts
1
Terminology Pathology 81
• Rare benign disorder causing sinusoidal dilatation & • Associated with chronic wasting diseases
presence of multiple blood filled lacunar spaces • Associated with steroid medications, sprue, diabetes,
within liver vasculitis, hematological disorders
• Bacillary peliosis hepatis caused by Bartonella species
Imaging Findings in HIV-positive patients
• Spherical lesion with centrifugal or centripetal
enhancement Clinical Issues
• Size: Varies from 1 mm to several centimeters • Complications: Liver failure/cholestasis/portal
• Best imaging tools: Multiphase helical CT and/or MRI hypertension/liver rupture leading to shock
Top Differential Diagnoses Diagnostic Checklist
• Hepatic adenoma • Multiphase enhanced CT or MR showing
• Hepatic cavernous hemangioma heterogeneous liver lesion with centrifugal or
• Focal nodular hyperplasia (FNH) centripetal enhancement
• Hypervascular metastases

o Hyperintense • Differentiate from peliosis by contrast-enhancement

o Multiple foci of t signal due to presence of subacute pattern on triphasic CT & dynamic MRI
blood • Presence of fatty contents helpful in narrowing
• Tl C+ diagnosis
o Lesions usually show contrast-enhancement • Uncommonly, totally hyperdense in arterial phase,
o Cystic cavity with enhancing rim representing becoming isodense to liver in portal phase
hematoma • Biopsy often necessary
o Strong contrast-enhancement with "branching"
appearance caused by vascular component on Hepatic cavernous hemangioma
fat-suppressed T1 in delayed imaging • Typical enhanced pattern (peripheral enhancement
with centripetal progression)
Ultrasonographic Findings • Enhancement similar to peliosis; but discontinuous
• Real Time nodular or globular for hemangioma, continuous ring
o Heterogeneous hepatic echopattern with for peliosis
hyper/hypoechoic regions • Larger lesions produce mass effect on hepatic vessels
• Homogenous hypoechoic lesions (in patients with
steatosis) Focal nodular hyperplasia (FNH)
• But also heterogeneous hypoechoic (complicated • Homogenous hypervascular mass on arterial phase,
with hemorrhage) isodense to liver on portal & delayed phases
• Or hyperechoic (in patients with normal liver) o Central scar with! attenuation on arterial & portal
patterns phases & enhancement on delayed images

Angiographic Findings Hypervascular metastases

• Conventional
o Multiple nodular vascular lesions; accumulations of
contrast material on late arterial phase
• ± Simultaneous opacification of hepatic veins
• More prominent during parenchymal phase &
persist on venous phase
• Angiographic evaluation may be diagnostic in
difficult cases
Imaging Recommendations
• Protocol advice
o Multiphase enhanced helical CT imaging
o Dynamic Tl C+ MR with fat-suppression
• Best imaging tools: Multiphase helical CT and/or MRI
I DIFFERENTIAL DIAGNOSIS
Hepatic adenoma
• Might also be associated with long term use of
estrogen
• Usually totally hypodense or isodense in delayed
phase because of rapid washout of contrast material
Hepatic abscess
• Important to differentiate from peliosis to avoid
percutaneous drainage of peliotic lesions which can be
dangerous & fatal
• Pyogenic abscess
o Multiseptated mass; "cluster of grapes" appearance
o Nonenhancing contents
o Typical clinical presentation; sepsis
I PATHOLOGY
General Features
• General path comments
o Pathogenesis remains uncertain
o Pathogenesis theories
• Outflow obstruction at sinusoidal level
• Hepatocellular necrosis leading to cyst formation
 PELIOSIS HEPATIS
1 • Dilatation of portion of central vein of hepatic
lobule
82 • Direct lesions of sinusoidal barrier
• Etiology
o Anabolic steroids, corticosteroids, tamoxifen, oral
contraceptive, diethylstilbestrol, Azathioprine
o After renal/cardiac transplant
o Polyvinyl chloride/arsenic/thorium oxide exposure
o Congenital-angiomatous malformation
• Epidemiology
o Peliosis is a rare entity
o Increasing incidence of cases of bacillary peliosis &
angiomatosis in immunocompromised patients
• Associated abnormalities
o Associated with chronic wasting diseases
• TB, leprosy, malignancy (HCC), AIDS
o Associated with steroid medications, sprue, diabetes,
vasculitis, hematological disorders
o Bacillary peliosis hepatis caused by Bartonella
species in HIV-positive patients
Gross Pathologic & Surgical Features
• Irregularly shaped blood-filled hepatic cavities
Microscopic Features
• Cystic dilated sinusoids filled with red blood cells &
bound by cords of liver cells
• Phlebectatic type: Endothelial lined blood filled spaces
& aneurysmal dilatation of central vein & sinusoids
• Parenchymal type: Not lined by endothelium &
usually associated with hemorrhagic parenchymal
necrosis
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Other signs/symptoms
• ± Hepatomegaly/ascites/portal hypertension
• Lymphadenopathy with Bartonella henselae &
neurological symptoms with Bartonella quintana
bacillary peliosis
• Clinical profile: Found incidentally at autopsy
Demographics
• Age: Fetal life (rare) to adult life
• Gender: M = F
Natural History & Prognosis
• Regression after drug withdrawal, cessation of steroid
therapy, resolution of associated infectious disease
• Pseudotumoral & hemorrhagic evolution
• Complications: Liver failure/cholestasis/portal
hypertension/liver rupture leading to shock
• If untreated may be rapidly fatal
Treatment
• Options, risks, complications
o Withdrawal of inciting agents
o Surgical resection of involved liver section
o Resolves spontaneously (uncommon)
o Clinical improvement with antibiotics
(erythromycin) in HIV related peliosis hepatis
caused by Bartonella henselae
I DIAGNOSTIC CHECKLIST
Consider
• Clinical setting (e.g., AIDS, chronic illness,
medications)
Image Interpretation Pearls
• Multiphase enhanced CT or MR showing
heterogeneous liver lesion with centrifugal or
centripetal enhancement
I SELECTED REFERENCES
1. Resto-Ruiz S et al: The role of the host immune response in
pathogenesis of Bartonella henselae. DNA Cell BioI.
22(6):431-40, 2003
2. Chomel BBet al: Clinical impact of persistent Bartonella
bacteremia in humans and animals. Ann N Y Acad Sci.
990:267-78,2003
3. Gouya H et al: Peliosis hepatis: triphasic helical CT and
dynamic MRI findings. Abdom Imaging. 26(5):507-9, 2001
4. Wang SYet al: Hepatic rupture caused by peliosis hepatis. J
Pediatr Surg. 36(9):1456-9, 2001
5. Ferrozzi F et al: Peliosis hepatis with pseudo tumoral and
hemorrhagic evolution: CT and MR findings. Abdom
Imaging. 26(2):197-9, 2001
6. Dehio C: Bartonella interactions with endothelial cells and
erythrocytes. Trends Microbiol. 9(6):279-85, 2001
7. Vignaux 0 et al: Hemorrhagic necrosis due to peliosis
hepatis: imaging findings and pathological correlation. Eur
Radiol. 9(3):454-6, 1999
8. Walter E et al: Images in clinical medicine. Peliosis hepatis.
N Engl] Med. 337(22):1603, 1997
9. Muradali D et al: Peliosis hepatis with intrahepatic
calcifications. J Ultrasound Med. 15(3):257-60, 1996
10. Saatci I et al: MR findings in peliosis hepatis. Pediatr
Radiol. 25(1):31-3, 1995
11. Jamadar DA et al: Case report: radiological appearances in
peliosis hepatis. Br J Radiol. 67(793):102-4, 1994
12. Toyoda S et al: Magnetic resonance imaging of peliosis
hepatis: a case report. Eur J Radiol. 16(3):207-8, 1993
13. Maves CK et al: Splenic and hepatic peliosis: MR findings.
AJRAm J Roentgenol. 158(1):75-6, 1992
14. Radin DR: Spontaneous resolution of peliosis of the liver
and spleen in a patient with HIV infection. AJRAm J
Roentgenol. 158(6):1409, 1992
15. Tsukamoto Y et al: CT and angiography of peliosis hepatis.
AJRAmJ Roentgenol. 142(3):539-40, 1984
16. Lyon J et al: Peliosis hepatis: diagnosis by magnification
wedged hepatic venography. Radiology. 150(3):647-9, 1984
17. SmathersRL et al: Computed tomography of fatal hepatic
rupture due to peliosis hepatis. J Comput Assist Tomogr.
8(4):768-9, 1984
 PELIOSIS HEPATIS

I IMAGE GALLERY 1
83
Typical
(Left) Axial CECTshows
multiple hypo dense hepatic
lesions (arrows) with
peripheral enhancement;
peliosis due to Bartonella
infection which resolved
after antibiotic treatment.
Spleen is also involved.
(Right) Axial CECT shows
extensive brightly enhancing
lymphadenopathy in this
patient with peliosis hepatis
due to Bartonella infection.

Typical
(Left) Axial CECT during
arterial phase shows
hypodense lesion with bright
continuous peripheral
enhancement. (Centripetal
progression of enhancement
on venous phase). Biopsy
proven peliosis hepatis.
(Right) Axial color Doppler
sonogram show hyperechoic
liver mass without prominent
vascularity.

Variant
(Left) Axial CECT in venous
phase shows multiple
hypodense lesions (arrows)
with enhanced periphery. 42
year old woman with 25
year use of oral
contraceptives. (Right) Axial
T2WI MR shows
hyperintense lesion in left
lobe (arrow); biopsy proven
peliosis. Other liver lesions
had similar appearance.
These partially resolved after
discontinuation of
contraceptives.
 HEMOCHROMATOSIS
1
84

Axial NEeT shows liver parenchyma of much higher Axial T2WI MR shows marked hypointensity throughout
attenuation than spleen (or muscle); primary liver; primary hemochromatosis.
hemochromatosis.

• Secondary: Due to increased iron intake,

ITERMINOLOGY ineffective erythropoiesis, multiple blood
Definitions transfusions, alcoholic cirrhosis & after portacaval
• Iron overload disorder in which there is structural & shunts
functional impairment of involved organs (total body • Total body iron may be 50-60 grams
iron may be 50-60 grams) o Hemosiderosis
• Increased iron deposition without organ damage
• Usually seen with body iron stores of 10-20 g
I IMAGING FINDINGS o Normal body iron storage: 2 to 6 g of iron
• 80% Functional iron: Hemoglobin, myoglobin &
General Features iron containing enzymes
• Best diagnostic clue: Hyperdense liver on NECT & • 20% In storage form: Hemosiderin or ferritin
markedly hypointense on T2WI • Liver contains up to one third of total body store
• Location of iron
o Primary hemochromatosis CT Findings
• Parenchymal cells of liver, pancreas & heart
o Secondary hemochromatosis • NECT
o Homogeneously increased liver density
• Initially reticuloendothelial system (RES)
• Up to 75-135 HU (normal 45-65 HU)
• After saturation of RES, parenchymal cells of liver,
o Prominent low attenuated hepatic & portal veins
pancreas, myocardium, kidneys & endocrine
o Dual energy CT (at 80 & 120 kVp) technique used to
glands
• Establish diagnosis if attenuation is borderline
• Size • To quantitate amount of iron deposition in liver
o Pre cirrhotic stage: Increase in liver size
• To follow efficacy of therapy
o Cirrhotic stage: Decrease in liver size
o Late stage
• Key concepts
• Liver shows cirrhotic features
o Hemochromatosis: Classified into two types
• Primary (idiopathic): Inherited autosomal • CECT
o Decreases inherent contrast differences
recessive disorder
• Between liver, blood vessels & tumor

DDx: Diffusely Increased liver Density

Hemosiderosis H~mosiderosis Glycogen Storage Amiodarone Therapy

 HEMOCHROMATOSIS

Key Facts
1
Terminology • Secondary hemochromatosis 85
• Iron overload disorder in which there is structural & Clinical Issues
functional impairment of involved organs (total body • Clinical profile: Patient with family history,
iron may be 50-60 grams) hepatomegaly, diabetes mellitus, hyperpigmentation
Imaging Findings & elevated blood iron/ferritin levels
• Best diagnostic clue: Hyperdense liver on NECT & Diagnostic Checklist
markedly hypointense on T2WI • Rule out other conditions like hemosiderosis,
Top Differential Diagnoses glycogen storage disease, amiodarone & gold therapy
• Hemosiderosis which can cause diffusely hyperdense liver on NECT
• Glycogen storage disease simulating hemochromatosis
• Amiodarone therapy • On T2WI: Marked signal loss of liver in primary type
& marked signal loss of both liver/spleen in
Pathology secondary type of hemochromatosis
• Primary: Gene is human leukocyte antigen (HLA-A3 • MR advantage: Other disorders do not simulate
& B14) linked located on short arm of chromosome 6 appearance of liver iron overload at MR like CT

MR Findings
• TlWI
o Primary hemochromatosis
• Decreased signal intensity in liver
• T2WI
o Primary: Marked signal loss in liver
o Secondary: Marked signal loss in both liver & spleen
• T2* GRE
o Signal intensity ratios of liver/muscle or liver/fat
• Establishes direct correlation with liver iron'
content better than T2 relaxation measurements
• Accurate in quantifying liver iron content
Ultrasonographic Findings
• Real Time: Has no role in the diagnosis of hepatic iron
overload
Imaging Recommendations
• Best imaging tool: MR T2* GRE for diagnosing hepatic
hemochromatosis
• Protocol advice
o For estimation of hepatic iron concentration
• T2 GRE image (18/5, 10° flip angle)
• Heavily T2W fast spin-echo sequence
I DIFFERENTIAL DIAGNOSIS
Hemosiderosis
• Decrease signal intensity in both liver & spleen
• Early stage: Indistinguishable from hemochromatosis
Glycogen storage disease
• Increase or decrease attenuation of liver on NECT
• Associated with multiple hepatic adenomas (60%)
Amiodarone therapy
• Iodine containing anti-arrhythmic medication
• Diffuse homogeneous dense liver on NECT
I PATHOLOGY
General Features
• Genetics
o Primary: Gene is human leukocyte antigen (HLA-A3
& B14) linked located on short arm of chromosome
6
o Mutations in HFE gene responsible for common
form of HLA-linked hereditary hemochromatosis
• Etiology
o Primary hemochromatosis
• Autosomal recessive disorder
• Relatively common & underdiagnosed cause of
liver disease
• Abnormal increase iron absorption by mucosa of
duodenum & jejunum
• Excess iron stored as cytoplasmic ferritin &
lysosomal hemosiderin
• Organs affected: Parenchymal cells (liver,
pancreas, heart); joints, endocrine glands & skin
• Does not affect Kupffer cells & reticuloendothelial
cells of bone marrow, spleen
o Secondary hemochromatosis
• Patients with increased iron intake: Increased
consumption of medicinal iron, iron laden wine,
Kaffir beer & multiple blood transfusions
• Anemic patients with infective erythropoiesis &
multiple blood transfusions (e.g., thalassemia
major, sideroblastic anemia)
• Patients with alcoholic cirrhosis & after portacaval
shunts
• Initially iron deposition in RES, sparing
parenchymal cells
• After saturation of RESiron accumulates in
parenchymal cells of liver, pancreas, myocardium
• Epidemiology
o Primary or idiopathic
• Increase prevalence in non-Jewish Caucasians of
northern European origin (1:220)
• Homozygote frequency: 0.25-0.50%
• Heterozygote carriers: More than 10%
 HEMOCHROMATOSIS
1 Gross Pathologic & Surgical Features
• Gender
o M:F = 10:1
86 • Early stage o Women are usually protected from this disorder
o Liver is slightly larger & dense • Due to iron loss during normal menstruation,
o Chocolate brown (ferritin) pregnancy & lactation
o Golden yellow granules (hemosiderin)
• Late stage Natural History & Prognosis
o Decrease in liver size • Normal life expectancy with early diagnosis &
o Cirrhotic micronodules & fibrous septa treatment
• Pancreas • Life expectancy of untreated patients: 4.4 years
o Skin pigmentation ("bronze diabetes"), atrophy &
fibrosis Treatment
• Deferoxamine (iron chelation therapy)
Microscopic Features • Phlebotomies in precirrhotic,stage
• Prussian blue staining
o Hemosiderin deposits in hepatocytes, Kupffer cells &
lysosomes I DIAGNOSTIC CHECKLIST
• In late stages
o Hepatocellular necrosis, scarring, fibrosis & cirrhosis Consider
• Rule out other conditions like hemosiderosis, glycogen
storage disease, amiodarone & gold therapy which can
I CLINICAL ISSUES cause diffusely hyperdense liver on NECT simulating
hemochromatosis
Presentation
• Most common signs/symptoms
Image Interpretation Pearls
o Asymptomatic during 1st decade of disease • On T2WI: Marked signal loss of liver in primary type
o Hepatomegaly in 95% of cases & marked signal loss of both liver/spleen in secondary
o Splenomegaly in 50% of case type of hemochromatosis
o Classic triad of hemochromatosis • MR advantage: Other disorders do not simulate
• Micronodular cirrhosis appearance of liver iron overload at MR like CT
• Diabetes mellitus
• Hyperpigmentation of skin
o Other signs/symptoms I SELECTED REFERENCES
• Congestive heart failure, arrhythmias 1. Kim MJ et al: Hepatic iron deposition on magnetic
• Arthralgias resonance imaging: correlation with inflammatory activity.
• Loss of libido, impotence J Comput Assist Tomogr. 26(6):988-93, 2002
• Amenorrhea, testicular atrophy 2. Pomerantz S et al: MR imaging of iron depositional disease.
Magn Reson Imaging Clin N Am. 10(1):105-20, vi, 2002
• Clinical profile: Patient with family history,
3. Bonkovsky HL et al: Hepatic iron concentration:
hepatomegaly, diabetes mellitus, hyperpigmentation Noninvasive estimation by means of MR imaging
& elevated blood iron/ferritin levels techniques. Radiology. 212: 227-34, 1999
• Lab data 4. Ito K et al: Hepatocellular carcinoma: Association with
o Serum iron: Above 250 mg/DL (normal SO-ISO increased iron deposition in cirrhotic liver at MR imaging.
mg/DL) Radiology. 212: 235-40, 1999
o Serum ferritin: Above 500 ng/DL (normal below 150 5. Press RD et al: Hepatic iron overload: direct HFE (HLA-H)
ng/DL) mutation analysis vs quantitative iron assays for the
diagnosis of hereditary hemochromatosis. Am J Clin
o Transferrin saturation: Approaches 100% (normal
Pathol. 109(5):577-84, 1998
25-30%) 6. Ernst 0 et al: Hepatic iron overload: diagnosis and
• Earliest & most sensitive indicator of increased quantification with MR imaging. AJRAm J Roentgenol.
iron stores 168(5):1205-8, 1997
o Liver iron index: More than 2 7. Siegelman ES et al: Abdominal iron deposition:
o Increased blood glucose metabolism, MR findings, and clinical importance.
o Urine analysis: Glycosuria Radiology. 199(1):13-22, 1996
• Complications 8. Gandon Y et al: Hemochromatosis: diagnosis and
quantification of liver iron with gradient-echo MR
o Periportal fibrosis leads to cirrhosis in late stage
imaging. Radiology. 193(2):533-8, 1994
• If iron concentration: Above 22,000 Ilg/g of tissue 9. Siegelman ES et al: Idiopathic hemochromatosis: MR
o Hepatocellular carcinoma (14-30%) imaging findings in cirrhotic and pre cirrhotic patients.
o IDDM (30-60%) Radiology. 188(3):637-41, 1993
o Hepatic coma (15%); hematemesis (14%) 10. Siegelman ES et al: Parenchymal versus reticuloendothelial
o Cardiac failure (30%) iron overload in the liver: distinction with MR imaging.
Radiology. 179(2):361-6, 1991
Demographics 11. Guyader D et al: Evaluation of computed tomography in
• Age the assessment of hepatic iron overload. Gastroenterology .
o Primary: Usually present in 4th or 5th decade 97: 747-53, 1989
o Secondary: Usually present at earlier age
 HEMOCHROMATOSIS

I IMAGE GALLERY 1
87
Typical
(Left) Axial NECT shows
hyperdense liver and very
dense lymph nodes (arrow).
(Right) Axial T2* GRE MR
shows decreased signal
intensity of liver and spleen
when compared with that of
paraspinal muscle;
secondary hemochromatosis.

Typical
(Left) Axial NECT shows
marked diffuse increased
density in liver; the spleen is
surgically absent; secondary
hemochromatosis from
multiple transfusions. (Right)
Axial T2WI MR shows
shrunken cirrhotic, markedly
hypointense liver; ascites,
varices; primary
hemochromatosis.

Typical
(Left) Axial NECT in patient
with primary
hemochromatosis and
cirrhosis shows dense liver
with mass representing
hepatocellular carcinoma
(HCC). (Right) Axial NECT
in patient with primary
hemochromatosis.
Hyperdense liver with focal
HCC (arrow). Note the
attenuation difference of the
liver when compared to that
of the enlarged spleen.
 WILSON DISEASE
1
88

Axial NECT shows cirrhotic morphology and multiple Axial CECT shows cirrhotic morphology and ascites.
discrete hyperdense regenerating nodules (arrow) Regenerating nodules are isodense with liver and
which were more apparent than on CECT. undedectable. Patient with Wilson disease being
evaluated for livertransplantation.

• Although Cu has high atomic number & can cause

ITERMINOLOGY elevation of liver density on CT, this is an unusual
Abbreviations and Synonyms finding perhaps because coexisting fatty infiltration
• Wilson disease (WD) diminishes hepatic parenchymal attenuation
• Hepatolenticular degeneration • Multiple, small, dysplastic nodules enhancing at
arterial phase & thickened perihepatic fat layer;
Definitions unusual finding
• Autosomal recessive disorder in which copper (Cu) • Multiple hyperdense regenerating nodules (on NECT)
accumulates pathologically primarily within liver &
subsequently in neurologic system & other tissues
MR Findings
• Copper deposition has no ferromagnetic effect on MR
imaging
I IMAGING FINDINGS • Iron in regenerative nodules cause hypointensity on
T1 and T2WI
General Features
Ultrasonographic Findings
• Best diagnostic clue: Liver biopsy for copper analysis
• Most commonly diffusely 1 hepatic echogenicity
• Location: Early on, diffuse distribution of Cu in liver
(cirrhosis)
cytoplasm, later on within lysosomes & then
throughout liver nodules Imaging Recommendations
• Size: Diffuse involvement • Best imaging tool: CT & MR; however no major role in
• Key concepts diagnosing WD
o Fatty infiltration, acute or chronic active hepatitis, • Imaging alone cannot distinguish WD from other
cirrhosis or massive liver necrosis forms of hepatitis or cirrhosis
CT Findings
• Spectrum of hepatic injury is nonspecific; changes of
fatty infiltration or cirrhosis frequently
indistinguishable from those of other etiologies

DDx: Diffusely Decreased liver Density

Steatosis Acute Hepatitis Acute Hepatitis Diffuse Lymphoma

 WILSON DISEASE

Key Facts
1
Terminology Pathology 89
• Autosomal recessive disorder in which copper (Cu) • Hepatic sinusoidal and periportal deposition of Cu
accumulates pathologically primarily within liver & • Cu deposition incites inflammatory reaction leading
subsequently in neurologic system & other tissues to cirrhosis
Imaging Findings Clinical Issues
• Spectrum of hepatic injury is nonspecific; changes of • Most common signs/symptoms:
fatty infiltration or cirrhosis frequently • Lab data: Serum ceruloplasmin < 20 mg/dL
indistinguishable from those of other etiologies • Diagnosis: Liver biopsy & Cu quantitation

I DIFFERENTIAL DIAGNOSIS Natural History & Prognosis

• Deposition to toxic levels occurs in basal ganglia, renal
Steatosis
tubules, cornea, bones, joints
• NECT: Density of hepatic parenchyma less than spleen • Patients with cirrhosis associated with WD are also
• Presence of normal vessels coursing through predisposed to hepatocellular carcinoma
Hepatitis • Acute & early presentations like fulminant hepatic
• Marked hepatomegaly, ascites, or both in conjunction failures have poor outcome
with decreased attenuation of liver parenchyma on CT • Timely & appropriate utilization of current modes of
treatment offer patients excellent long term survival
lymphoma
Treatment
• Diffuse or focal hepatic hypodensity
• Initial & maintenance therapy with Cu-chelator
• Liver transplantation
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
• Genetics: Autosomal recessive disorder
• Etiology: !Biliary excretion of Cu, t intestinal
Consider
absorption of Cu, abnormal urinary excretion of Cu • Myriad manifestations of WD make its diagnosis
• Epidemiology: Prevalence: 1:30,000 individuals dependent on a high index of suspicion

Gross Pathologic & Surgical Features

• Steatosis, followed by fibrosis & ultimately cirrhosis I SELECTED REFERENCES
Microscopic Features 1. Akhan 0 et al: Unusual imaging findings in Wilson's
disease. Eur Radiol. 12 Suppl 3:566-9, 2002
• Hepatic sinusoidal and periportal deposition of Cu 2. Ko 5 et al: Unusual liver MR findings of Wilson's disease in
• Cu deposition incites inflammatory reaction leading an asymptomatic 2-year-old girl. Abdom Imaging.
to cirrhosis 23(1):56-9, 1998
3. Garg RK et al: Wilson's disease: unusual clinical and
radiological features. J Assoc Physicians India. 42(3):253-4,
I CLINICAL ISSUES 1994

Prese nta ti 0 n
• Clinical profile I IMAGE GALLERY
o Chronic hepatitis, cirrhosis, acute liver failure
o Acute fulminant hepatitis: Presents acutely with
signs of jaundice, ascites that progresses to
encephalopathy, & liver failure
o Lab data: Serum ceruloplasmin < 20 mg/dL
o Diagnosis: Liver biopsy & Cu quantitation
• Hepatic Cu content> 250 ug/g dry weight
• Presence of Kayser-Fleisher rings & low level of
ceruloplasmin is sufficient to diagnose WD
Demographics
• Gender: Acute fulminant presentation of WD is most
often seen in females (M:F = 1:2)
(Left) Coronal T2WI MR shows diffusely hypointense and shrunken
cirrhotic liver. Ascites. (Right) Axial NECT shows multiple hyperdense
regenerating nodules within a cirrhotic liver. Patient with Wilson
disease and acute fulminant hepatitis.
 HEPATIC TRAUMA
1
90

Axial CECT shows parenchymal laceration/hematoma, Axial CECT shows multiple linear and stellate planes of
with active bleeding (arrow). laceration but no active bleeding. There is minimal
hemoperitoneum.

ITERMINOLOGY CT Findings
• Lacerations
Abbreviations and Synonyms
o Simple or stellate (parallel to portal/hepatic vein
• Liver or hepatic injury branches)
• Simple: Hypodense solitary linear laceration
• Stellate: Hypodense branching linear lacerations
I IMAGING FINDINGS • Parenchymal & subcapsular hematomas (lenticular
General Features configuration)
o Unclotted blood (35-45 HU) soon after injury
• Best diagnostic clue: CT evidence of irregular
• NECT: May be hyperdense relative to normal liver
parenchymal lesions with intra & perihepatic
• CECT: Hypodense compared to enhancing normal
hemorrhage
liver tissue
• Location o Clotted blood (60-90 HU)
o Right lobe (75%); left lobe (25%)
• More dense than unclotted blood & normal liver
• Intraparenchymal or subcapsular
• May be more dense than un enhanced liver
• Key concepts
• Active hemorrhage or pseudoaneurysm
o Liver 2nd most frequently injured solid
o CECT: Active hemorrhage
intra-abdominal organ after spleen
• Isodense to enhanced vessels
• Due to its anterior & partii,llly subcostal location
• Seen as contrast extravasation (85-350 HU)
o Most common causes of hepatic trauma
• Extravasated contrast material & surrounding
• Blunt (more common), penetrating & iatrogenic
decreased attenuation clot
injuries
• Hemoperitoneum: Perihepatic and peritoneal recess
o Iatrogenic injury due to liver biopsy
collections of blood
• Most common cause of subcapsular hematoma in
• Periportal tracking: Linear, focal or diffuse periportal
US
zones of decreased HU
o Abdominal trauma
o Due to dissecting blood, bile or dilated periportal
• Leading cause of death in United States « 40 yrs)
lymphatics
o DDx: Overhydration (check for distended IVe)

DDx: Focal liver lesion with Hemorrhage

HELLP Syndrome Hepatic Adenoma Metastases

 HEPATIC TRAUMA

Key Facts
1
Imaging Findings Clinical Issues 91
• Best diagnostic clue: CT evidence of irregular • Clinical profile: Patient with history of motor vehicle
parenchymal lesions with intra & perihepatic accident, RUQ tenderness, guarding & hypotension
hemorrhage • Mortality: 10-20%
• Right lobe (75%)i left lobe (25%)
• Intraparenchymal or subcapsular Diagnostic Checklist
• Morphology: • Differentiate from HELLP syndromei spontaneous
hemorrhage (coagulopathy) & bleeding hepatic
Top Differential Diagnoses tumors like HCC or adenoma
• HELLP syndrome • CT evidence of active extravasation (intra- or
• Spontaneous hemorrhage (coagulopathy) extra-hepatic collection, isodense with vessels)
• Bleeding hepatic tumor (e.g.,: HCC or adenoma) usually indicates need for embolization or surgery
regardless of "grade" of injury
Pathology • Laceration of left hepatic lobe often associated with
• Blunt trauma (more common) bowel and pancreatic injury

• Elevated venous pressure & transudation o Angiography: To localize active hemorrhage &
• Areas of infarction embolization
o Small or large areas of low attenuation • Protocol advice: Helical CECT: Include lung bases and
o Usually wedge-shapedi segmental or lobar pelvis
o Intrahepatic/subcapsular gas (due to hepatic
necrosis)
• CT diagnosis of liver trauma I DIFFERENTIAL DIAGNOSIS
o Accuracy: 96%
o Sensitivity: 100% HEllP syndrome
o Specificity: 94% • Severe variant of preeclampsia
• HELLP: Hemolysis, elevated liver enzymes & low
MR Findings platelets
• TlWI & T2WI • On imaging
o Varied signal intensity depending on o Intrahepatic or subcapsular fluid collection
• Degree & age of hemorrhage or infarct (hematoma)
o Wedge-shaped areas of infarction
Ultrasonographic Findings
o Occasionally active extravasation
• Real Time
o Subcapsular hematoma: Lentiform or curvilinear Spontaneous hemorrhage (coagulopathy)
fluid collection • History of bleeding disorder
• Initially: Anechoic • Lab data: Abnormal hematologic coagulation values
• After 24 hrs: Echogenic • On imaging
• After 4-5 days: Hypoechoic o Subcapsular or intrahepatic blood collection
o After 1-4 weeks: Internal echoes & septations o Indistinguishable from hepatic trauma without
develop within hematoma history
o Intraparenchymal hematoma
• Rounded echogenic or hypoechoic foci Bleeding hepatic tumor (e.g.,: HCC or
o Bilomas adenoma)
• Rounded/ellipsoid, anechoic, loculated structures • Spherical enhancing parenchymal masses
• Well-defined sharp margins, close to bile ducts • Hepatocellular carcinoma
o Parenchymal tears o Vascular, nodal & visceral invasion (common)
• Irregular defects
• Abnormal echotexture relative to normal liver
Angiographic Findings I PATHOLOGY
• Conventional General Features
o Demonstrate
• Etiology
• Active extravasation, pseudo aneurysm o Blunt trauma (more common)
• A-V,arteriobiliary or portobiliary fistulas • Motor vehicle accidents (more common)
Imaging Recommendations • Falls and assaults
• Best imaging tool o Penetrating injuries
o Helical CECT: In hemodynamically stable cases • Gunshot and stab injuries
o Iatrogenic
 HEPATIC TRAUMA
1 • Liver biopsy, chest tubes, transhepatic
Natural History & Prognosis
cholangiography
92 • Epidemiology • Complications
o 5"10% blunt abdominal trauma have liver injury o Hemobilia, bilomas, A-V fistula, pseudoaneurysm
o Mortality from hepatic trauma: 10-20% • Prognosis
• Associated abnormalities o Grade I, II & III: Good
o Splenic injury (45%); bowel injury (5%); rib fractures o Grade IV, V & VI: Poor
o Left hepatic lobe laceration often associated with o May not necessarily correlate with AAST grading
bowel or pancreatic injury o Mortality: 10-20%
• 50% due to liver injury itself
Gross Pathologic & Surgical Features • Rest from associated injuries
• Laceration or contusion
• Subcapsular or intraparenchymal hematoma Treatment
• Grade I, II, III
Staging, Grading or Classification Criteria o Conservative management for almost all injuries
• Clinical classification based on American Association diagnosed on CT
for Surgery of Trauma (AAST) • Implies some degree of clinical stability
o Grade I • Grade IV, V, VI
• Subcapsular hematoma: Less than 10% surface o Surgical intervention for shock & peritonitis
area • Control hemorrhage, drainage & repair
• Laceration: Capsular tear, less than 1 cm o Embolization for active extravasation
parenchymal depth
o Grade II
• Subcapsular hematoma: 10-50% surface area [DIAGNOSTIC CHECKLIST
• Intraparenchymal hematoma: Less than 10 cm
diameter Consider
• Laceration: 1-3 cm parenchymal depth, less than • Differentiate from HELLP syndrome; spontaneous
10 cm in length hemorrhage (coagulopathy) & bleeding hepatic
o Grade III tumors like HCC or adenoma
• Subcapsular hematoma: More than 50% surface
Image Interpretation Pearls
area; expanding/ruptured subcapsular or
parenchymal hematoma • CT evidence of active extravasation (intra- or
extra-hepatic collection, isodense with vessels) usually
• Intraparenchymal hematoma: More than 10 cm or
indicates need for embolization or surgery regardless
expanding
of "grade" of injury
• Laceration: Parenchymal fracture more than 3 cm
• Laceration of left hepatic lobe often associated with
deep
bowel and pancreatic injury
o Grade IV
• Laceration: Parenchymal disruption involving
25-75% of hepatic lobe or 1-3 Couinaud segments
within a single lobe
I SELECTED REFERENCES
o Grade V 1. Yao DC et al: Using contrast-enhanced helical CT to
• Laceration: Parenchymal disruption involving> visualize arterial extravasation after blunt abdominal
75% of hepatic lobe or > 3 Couinaud segments trauma: incidence and organ distribution. AJRAm J
Roentgenol. 178(1):17-20,2002
within a single lobe
2. Patten RM et al: CT detection of hepatic and splenic
• Vascular: ]uxtahepatic venous injuries injuries: usefulness of liver window settings. AJRAm J
(retrohepatic venacava, major hepatic veins) Roentgenol. 175(4):1107-10,2000
o Grade VI 3. Poletti PA et al: CT criteria for management of blunt liver
• Vascular: Hepatic avulsion trauma: correlation with angiographic and surgical
findings. Radiology. 216(2):418-27, 2000
4. Becker CD et al: Blunt hepatic trauma in adults: correlation
I CLINICAL ISSUES of CT injury grading with outcome. Radiology.
201(1):215-20, 1996
Presentation 5. Mirvis SE et al: Blunt hepatic trauma in adults: CT-based
classification and correlation with prognosis and
• Most common signs/symptoms treatment. Radiology. 171(1):27-32, 1989
o RUQ pain, tenderness, guarding, rebound tenderness
o Hypotension, tachycardia, jaundice
o Hematemesis or melena (due to hemobilia)
• Clinical profile: Patient with history of motor vehicle
accident, RUQ tenderness, guarding & hypotension
• Lab data
o Decreased hematocrit (not acutely)
o Increased direct/indirect bilirubin
o Increased alkaline phosphatase levels
 HEPATIC TRAUMA
I IMAGE GALLERY
1
93
Typical
(Left) Axial CECT shows
laceration of lateral segment
(arrow) and
hemoperitoneum. (Right)
Axial CECT shows jejunal
injury with clotted blood
("sentinel clot") (arrow) in
mesentery between bowel
loops. Imaging through the
liver demonstrated lateral
segment laceration.

Typical
(Left) Axial CECT shows
deep linear laceration of
right hepatic lobe in a
patient who was managed
conservatively. (Right) Axial
CECT obtained two weeks
after blunt trauma
demonstrates considerable
healing of deep hepatic
laceration.

Typical
(Left) Axial CECT shows
linear laceration through
base of caudate lobe
extending to the bare area of
the liver and resulting in
retroperitoneal hematoma
(arrow). (Right) Axial CECT
shows large subcapsular
hematoma. Patient had
previous recent motor
vehicle accident without
medical evaluation, and self
medicated with aspirin and
ibuprofen.
 BILIARY TRAUMA
1
94

Axial CECT shows deep liver laceration, small Cholangiogram performed in patient who had
hemoperitoneum. The severity of the injury transected developed peritoneal symptoms shows extravasationof
intrahepaticbile ducts. bile (arrow) from transected intrahepatic duct.
Successfully treated with biliarystenting.

• Biliary-vascular fistulas: To portal vein, hepatic

ITERMINOLOGY
Abbreviations and Synonyms
• Bile duct injury
Definitions
• Hemobilia: Bleeding into biliary tract
• Bilhemia: Condition in which bile enters veins of
liver, & is rare
I IMAGING FINDINGS
General Features
• Best diagnostic clue
o Clinical history can suggest diagnosis
o Percutaneous transhepatic (PTC) or endoscopic
retrograde cholangiography (ERCP): "Gold standard
for diagnosis of bile duct injuries
• Location
o In major bile duct injuries, common bile
duct/common hepatic duct are most frequently
injured (61.1 %)
o Post-operative strictures: Common hepatic duct
(45-64%); hepatic hilus (20-33%)
o Biliary fistulas: Internal (communication with
duodenum, colon, bronchi, etc.) or external (skin)
artery, hepatic veins
• Size: Focal or diffuse involvement
• Morphology: Bile leakage, strictures, biliary tree
obstruction, various types of biliary fistulas, hemobilia
Radiographic Findings
• Radiography: Biliary-enteric fistula: Pneumobilia
• Fluoroscopy
o Biliary-enteric fistula: Barium filling of biliary tree
o Nonionic or oil-based contrast material is indicated
when biliary-bronchial fistula is suspected
• ERCP
o To evaluate: Level, length, contour of strictures
• Posttraumatic strictures are typically focal, smooth
areas of narrowing with proximal dilation
o ERCP can facilitate definitive diagnosis & treatment
III
of bile leaks & simple strictures
o Visualization & cannulation of fistula orifice &
permits good-quality cholangiographic evaluation
o May see active bleeding coming from major papilla
CT Findings
• Helical CT cholangiography or helical CT after
administration of biliary Lv. contrast material to verify
& localize bile duct leakage; may help avoid ERCP
• Biliary-enteric fistula: Oral contrast media in both
bowel & biliary tree

DDx: Biliary "Leak"

Biliary Leak (OLT) HAT/Biliary Necrosis HAT/Biliary Necrosis

 BILIARY TRAUMA
Key Facts
Terminology
• Bile duct injury
• Hemobilia: Bleeding into biliary tract
Imaging Findings
• Clinical history can suggest diagnosis
• Percutaneous transhepatic (PTC) or endoscopic
retrograde cholangiography (ERCP): "Gold standard'"
for diagnosis of bile duct injuries
• Morphology: Bile leakage, strictures, biliary tree
obstruction, various types of biliary fistulas,
hemobilia
• Posttraumatic strictures are typically focal, smooth
areas of narrowing with proximal dilation
• ERCP can facilitate definitive diagnosis & treatment
of bile leaks & simple strictures
• Extent & localization of parenchymal destruction in
bilio-vascular fistula
• Presence of biliary dilatation, configuration of injured
bile duct, & ancillary abdominal findings
• Hemobilia: Blood may appear as high-attenuation
material (> SO HU) in ducts/gallbladder
o Liver laceration, hematoma, other potential sources
of blood may also be detected
• CT-guided drainagei non operative management of
parenchymal & (retro)-peritoneal collections
MR Findings
• MRCP: Noninvasive, non-ionizing tool for diagnosis of
wide variety of bile duct injuries
• Can use hepatobiliary contrast agent (Mangafodipir)
to directly visualize leak or stricture
Ultrasonographic Findings
• Proximal biliary dilation with gradual tapering of duct
diameter, without a surrounding mass present
• Blood: Echogenic material in bile-ducts
• Bilioma: Echo-free well-marginated fluid collection
• Ultrasound-guided drainage of collections
Angiographic Findings
• Hemobilia: Diagnosis is most commonly confirmed by
selective hepatic arteriography, demonstrating
extravasation of contrast material into biliary tree
o Excludes hepatic artery aneurysm/pseudoaneurysm
(10% of cases of biliary bleeding)
o Facilitates embolic occlusion therapy
• If vessel injury, or hemobilia are suspected on CT scan,
angiography should be carried out, even in active
bleedingi can be therapeutic, thereby avoiding surgery
Nuclear Medicine Findings
• Technetium 99m dimethyliminodiacetic acid (HIDA)
scan to detect occult ductal injuries, confirm bile leak
• Hepatobiliary scintigraphy is safe means of
investigating a possible biliary-bronchial fistula
1
• Hemobilia: Diagnosis is most commonly confirmed 95
by selective hepatic arteriography, demonstrating
extravasation of contrast material into biliary tree
• Best imaging tool: PTC is generally more valuable
than ERCPi in that it defines anatomy of proximal
biliary tree that is to be used in surgical
reconstruction
Pathology
• Trauma: Blunt or penetrating
• Iatrogenic: Laparoscopic or conventional open
cholecystectomy
Clinical Issues
• Biliary cirrhosis from long-standing obstruction
Other Modality Findings
• Cholangiography: May demonstrate clotted blood as
cast-like filling defect in bile ducts & may reveal other
potential causes of hemorrhage
Imaging Recommendations
• Best imaging tool: PTC is generally more valuable than
ERCPi in that it defines anatomy of proximal biliary
tree that is to be used in surgical reconstruction
• Protocol advice: ERCP/PTC/MRCPi angiography
! DIFFERENTIAL DIAGNOSIS
Iatrogenic injury
• After liver transplantation (OLT)i bile leak at T-tube
site, in non anastomotic location in donor biliary tree
or at the anastomosis
Hepatic artery thrombosis (HAT)
• Ischemia of bile ducts, nonanastomotic leak in hilar
region or intrahepatic ducts
• Ballooning of necrotic ducts into irregular cystic
spaces from distended, debris-filled, necrotic bile ducts
!PATHOlOGY
General Features
• General path comments
o Iatrogenic injury occurs as a result of technical errors
or misidentification of biliary anatomy
• Inexperience, inflammation, aberrant anatomy
• Etiology
o Trauma: Blunt or penetrating
o Iatrogenic: Laparoscopic or conventional open
cholecystectomy
• Other: Percutaneous liver puncturei percutaneous
biliary drainagei during biliary tract explorationi
hepatic artery embolizationi infusion of
chemotherapeutic agentsi gastrectomYi hepatic
resectioni cautery-inducedi etc.
• Epidemiology
 BILIARY TRAUMA
1 o 95% of strictures are secondary to surgical injury & >
80% from trauma during cholecystectomy
96 o t Incidence over past decade with introduction of
laparoscopic cholecystectomy; affecting::::; 2,000
patients annually in United States
• Higher incidence during laparoscopic than open
cholecystectomy; at least double the rate
o 50% of cases of hemobilia are due to blunt trauma
Gross Pathologic & Surgical Features
• Post-Iaparoscopic cholecystectomy: Spectrum of injury
ranges from cystic duct stump leakage to partial
obstruction to complete occlusion of ducts & common
hepatic or common bile duct ischemic strictures
Microscopic Features
• Disruption of duct epithelium, communication
between ducts & other organs, narrowing of lumen
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Patients with biliary stricture after blunt abdominal
trauma may exhibit a delayed onset of symptoms
o Post-operative bile duct injuries: May present early
with obstructive jaundice or evidence of a bile leak
• In patients presenting months to years after
surgery, cholangitis is most common symptom
• In only 10-25% of patients with
postcholecystectomy injury is the problem
recognized within first week, but nearly 70% are
recognized within first 6 months
o Triad of GI blood loss, biliary colic, jaundice
suggests presence of hemobilia, although both pain
& jaundice may be absent
• Clinical profile
o Lab data: An excessively high serum level of direct
bilirubin & only moderately elevated liver enzymes
indicate bilhemia in trauma patients
o Thoracentesis: Presence of bile in pleural cavity is
considered proof of pleural-biliary fistula
Demographics
• Age: Iatrogenic trauma more common in adults
• Gender
o Males: More blunt trauma
o Females: More iatrogenic injuries (more frequent
cholecystectomies)
Natural History & Prognosis
• Major, profuse hemobilia is rare but may be
life-threatening; minor hemobilia is more frequent &
often clinically silent
o Bleeding may often be delayed by 3-4 weeks & even
by as much as 12 weeks after liver injury
• Causes of delayed complications are multiple &
include: Abnormal or insufficient injury healing
process; retention of necrotic tissue; secondary
infection of initially sterile collections;
underestimation of injury severity
• Biliary cirrhosis from long-standing obstruction
• Significant morbidity & mortality associated with
non-surgical trauma to extrahepatic biliary tract
Treatment
• Principles: Definition of anatomy, relief of any
impedance to biliary flow, & drainage of collections
• In minor leaks, endoscopic diversion by
sphincterotomy or stenting
• More severe injury, ERCP (or PTC) to assess injury and
plan operative repair
o Surgical treatment for strictures or major leak:
Roux-en-Y hepaticojejunostomy
• Hemobilia: Conservatively (if minor bleeding);
trans arterial embolization; surgery
• Lesions detected during cholecystectomy should be
repaired immediately; biliary-enteric anastomosis
(41.8%) or t-tube or stent (27.5%)
• Bile duct fistula: Spontaneous closure, suture of fistula
& T-tube drainage, decompression
I DIAGNOSTIC CHECKLIST
Consider
• Patients with major bile duct injuries should be
evaluated for concomitant hepatic arterial injury;
management & outcome may be influenced by
absence of arterial blood flow to injured ducts & liver
• High index of suspicion is mandatory in patients
complaining of discomfort several days after surgery
I SELECTED REFERENCES
1. Federle MP et al: Complications of liver transplantation:
imaging and intervention. Radiol Clin North Am.
41(6):1289-305, 2003
2. Familiari L et al: An endoscopic approach to the
management of surgical bile duct injuries: nine years'
experience. Dig Liver Dis. 35(7):493"7, 2003
3. Wong YC et al: Magnetic resonance imaging of
extrahepatic bile duct disruption. Eur Radiol.
12(10):2488-90, 2002
4. Goffette PP et al: Traumatic injuries: imaging and
intervention in post-traumatic complications (delayed
intervention). Eur Radiol. 12(5):994-1021, 2002
5. Green MH et al: Haemobilia. Br J Surg. 88(6):773-86, 2001
6. Yoon KH et al: Biliary stricture caused by blunt abdominal
trauma: clinical and radiologic features in five patients.
Radiology. 207(3):737-41, 1998
7. Slanetz PJ et al: Imaging and interventional radiology in
laparoscopic injuries to the gallbladder and biliary system.
Radiology. 201(3):595-603, 1996
 BILIARY TRAUMA
I IMAGE GALLERY 1
97
Typical
(Left) Axial CECT following
blunt trauma shows
hemoperitoneum including
hematoma (arrow) between
duodenum and pancreatic
head. Further evaluation
demonstrated transection of
the distal common bile duct.
(Right) Percutaneous
transhepatic cholangiogram
shows active extravasation
from transected common
bile duct (arrow); no flow of
contrast into duodenum.

(Left) Percutaneous
transhepatic cholangiogram
shows complete obstruction
and contrast extravasation
from common hepatic duct
following laparoscopic
cholecystectomy. (Right)
Hepatic arteriogram shows
complete occlusion beyond
the origin of the right hepatic
artery (arrow) in a patient
who also had iatrogenic
biliary injury following
laparoscopic
cholecystectomy.

Typical
(Left) Axial CECT following
surgery for blunt abdominal
trauma. Stellate fracture of
lateral segment (arrow) is
seen. Intrahepatic biliary
injury was not appreciated
during initial laparotomy.
(Right) Tc HIDA scan shows
foci of increased activity
(arrows) due to bile
extravasation from lateral
segment in a patient who
had prior blunt abdominal
trauma.
 HEPATIC CYST
1
98

Axial CECT shows multiple hypodense lesions in liver. Sagittal ultrasound shows anechoic lesion (arrow) with
The largest lesion is sharply defined and had a ROI of 2 no visible wall. Through transmission of sound (open
H(J, while the smaller lesions are too small to arrow).
characterize with confidence.

o Congenital or developmental: Simple hepatic or bile

ITERMINOlOGY duct cyst
Abbreviations and Synonyms • Often solitary
• Simple hepatic or bile duct cyst • Occasionally multiple: Less than 10
• No communication with bile ducts
Definitions • More prevalent in women
• Simple hepatic cyst is a benign congenital • Usually asymptomatic
developmental lesion derived from biliary o When more than 10 in number, one of
endothelium fibropolycystic diseases must be considered
• Example: Autosomal dominant polycystic liver
disease (ADPLD)or biliary hamartomas
I IMAGING FINDINGS o Acquired cyst-like hepatic lesions
• Trauma (seroma or biloma)
General Features • Infection: Pyogenic or parasitic
• Best diagnostic clue: Anechoic lesion with through • Neoplasm: Primary or metastatic
transmission & no mural nodularity on US
• Location CT Findings
o Simple cyst • NECT
• Typically occurs beneath the surface of liver o Simple liver or bile duct cyst
• Some may occur deeper • Sharply defined margins
• Size: Varies from few mm to 10 cm • Smooth, thin walls
• Key concepts • Water density (-10 to +10 HU)
o Current theory • Usually no septations (rarely up to 2 thin septa)
• True hepatic cysts arise from hamartomatous • No fluid-debris levels
tissue • No mural nodularity or wall calcification
o 2nd most common benign hepatic lesion after o Hemorrhage into cyst may be indistinguishable
cavernous hemangioma from tumor
o Classified based on etiology & pathogenesis • Mural nodularity
• With or without calcification & fluid level

DDx: Cystic Hepatic lesion

(.;! ....
-;- ..
1~£.:1
Cystic Metastases Cystic Metastases
- .y
Cystadenocarcinoma Hepatic Abscess
 HEPATIC CYST

Key Facts
1
Terminology Pathology 99
• Simple hepatic cyst is a benign congenital • Defective development of intrahepatic biliary duct
developmental lesion derived from biliary • No communication with bile ducts
endothelium
Clinical Issues
Imaging Findings • Usually asymptomatic
• Best diagnostic clue: Anechoic lesion with through • Pain & fever (intracystic hemorrhage or infection)
transmission & no mural nodularity on US
• Morphology:
Diagnostic Checklist
• Rule out cyst-like hepatic lesions (infection, tumor)
Top Differential Diagnoses • CT: Nonenhancing, well-defined, round,
• Cystic or 'necrotic metastases homogeneous, water-density lesion
• Biliary cystadenocarcinoma • Small lesion less than blood density on NECT is
• Typical hepatic hemangioma probably a cyst
• Pyogenic abscess
• Hepatic hydatid cyst

o Autosomal dominant polycystic liver disease • Anechoic mass

• Multiple cysts (more than 10) • Smooth borders
• Vary in size: 1-10 cm • Thin or non-detectable wall
• Water or hemorrhagic density • No or few septations
• Calcification of some cyst walls is seen • No mural nodules
• No septations or mural nodularity • No wall calcification
• Occasionally fluid levels seen o Hemorrhagic or infected hepatic cyst
• Liver often distorted by innumerable cysts • Septations
• CECT • Internal debris
o Simple cyst • Thickened wall
• Uncomplicated: No enhancement • With or without calcification
• Complicated (infected): No enhancement
o Autosomal dominant polycystic liver disease Imaging Recommendations
• Uncomplicated: No enhancement • Best imaging tool: Ultrasonography or NE + CECT
• Complicated (infected): No enhancement
MR Findings I DIFFERENTIAL DIAGNOSIS
• Simple hepatic cyst & ADPLD
o T1WI: Hypointense Cystic or necrotic metastases
o T2WI: Hyperintense • Demonstrate (e.g., ovarian cystadenocarcinoma &
o Heavily T2W images metastatic sarcoma)
• Markedly increased signal intensity due to pure o Debris
fluid content o Mural nodularity
• Sometimes indistinguishable from a typical o Thick septa
hemangioma o Wall enhancement
o MRCP: No communication with bile duct
.• Complicated (hemorrhagic) cyst Biliary cystadenocarcinoma
o TlWI & T2WI • Usually large in size
• Varied signal intensity (due to mixed blood • Homogeneous, hypodense, water density mass
products) o Rarely non septated
• With or without a fluid level o Indistinguishable from large simple cyst
• TIC+ • Almost always has septations
o Uncomplicated cysts • May show fine mural or septal calcifications
• No enhancement • On contrast study
o Complicated cysts o Unilocular or multilocular
• No enhancement o Enhancement of capsule, septa & mural nodules
• MRCP Typical hepatic hemangioma
o Simple hepatic cyst & ADPLD
• Well-defined margins
• Shows no communication with bile ducts
• NECT: Isodense to blood
Ultrasonographic Findings • CECT: Early peripheral nodular & delayed centripetal
• Real Time enhancement isodense to enhanced vessels
o Uncomplicated simple (bile duct) cyst • T1W: Hypointense
• T2WI: Markedly hyperintense
 HEPATIC CYST
1 • Sometimes indistinguishable from simple hepatic cyst • Budd-Chiari syndrome
on MR images • Clinical profile: Asymptomatic patient with incidental
100 detection of simple hepatic cyst on imaging or at time
Pyogenic abscess
of autopsy
• Complex cystic mass
• Lab data
• Heterogeneous density
o Patients with large hepatic cyst & mass effect: 1
• Thick or thin multiple septations
Direct bilirubin levels
• May show mural nodularity
o Patients with advanced disease of ADPLD: 1 LFTs
• With or without hemorrhage
• Diagnosis
• May show fluid-debris levels
o Fine needle aspiration & cytology (rarely necessary)
• Wall enhancement may be seen
• Absent or heterogeneous enhancement of lesion Demographics
Hepatic hydatid cyst • Age
o Seen in any age group
• Large well-defined cystic liver mass with numerous
o Usually discovered incidentally in 5th-7th decades
peripheral daughter cysts
• Gender: M:F = 1:5
• With or without calcification & dilated bile ducts
Natural History & Prognosis
• Complications
I PATHOLOGY o Infection
o Hemorrhage
General Features o Large cyst: Compression of IHBD & jaundice
• Etiology • Prognosis
o Congenital simple hepatic cyst o Small & large hepatic cysts: Good prognosis
• Defective development of intrahepatic biliary duct o Advanced disease of ADPLD: Good prognosis
o Acquired hepatic cyst: Secondary to
• Trauma, inflammation Treatment
• Neoplasia, parasitic infestation • Asymptomatic simple hepatic cyst & ADPLD
• Epidemiology o No treatment
o Reported to occur in 2.5% of population • Large, symptomatic, infected hepatic cyst
o Incidence: 1-14% in autopsy series o Percutaneous aspiration & sclerotherapy with
• Associated abnormalities alcohol
o Autosomal dominant polycystic liver disease o Surgical resection or marsupialization
• 50% have polycystic kidney disease • Advanced disease of ADPLD
• M:F = 1:2 o Partial liver resection
o Polycystic kidney disease: 40% have hepatic cysts o Liver transplantation
o Tuberous sclerosis
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
• Simple hepatic cyst
o Cyst wall: ::s;1 mm thick Consider
o Seen beneath the surface of liver • Rule out cyst-like hepatic lesions (infection, tumor)
o Some are deeper
Image Interpretation Pearls
Microscopic Features • us: Anechoic, thin wall, through transmission
• True simple hepatic cyst • CT: Nonenhancing, well-defined, round,
o Single unilocular cyst with serous fluid homogeneous, water-density lesion
o Lined by o Small lesion less than blood density on NECT is
• Cuboidal bile duct epithelium probably a cyst
• A thin underlying rim of fibrous stroma
o No communication with bile ducts
I SELECTED REFERENCES
1. Mortele KJet al: Cystic focal liver lesions in the adult:
I CLINICAL ISSUES Differential CT and MR imaging features. RadioGraphies.
21: 895-910, 2001
Presentation 2. Martin DR et al: Imaging of benign and malignant focal
• Most common signs/symptoms liver lesions. Magn Reson Imaging Clin N Am.
o Uncomplicated simple cysts & ADPLD 9(4):785-802, vi-vii, 2001
• Usually asymptomatic 3. Casillas VJ et al: Imaging of nontraumatic hemorrhagic
o Complicated cyst hepatic lesions. RadioGraphies. 20: 367-78, 2000
• Pain & fever (intracystic hemorrhage or infection) 4. Horton KMet al: CT and MR imaging of benign hepatic
and biliary tumors. Radiographies. 19(2):431-51, 1999
o Large cysts present with symptoms of mass effect 5. Murphy BJet al: The CT appearance of cystic masses of the
• Abdominal pain, jaundice, palpable mass liver. RadioGraphies. 9: 307-22, 1989
o Advanced disease of ADPLD patients present with
• Hepatomegaly, liver failure (rarely)
 HEPATIC CYST
I IMAGE GALLERY 1
101
Typical
(Left) Axial CECTshows
multiple hepatic cysts of
varying size. Water density,
no enhancement. (Right)
Sagittal sonogram shows
anechoic lesions with thin
walls, through transmission,
no mural nodularity.

Typical
(Left) Axial CECT shows
multiple simple cysts. Smaller
ones appear of higher than
water density due to partial
volume averaging. (Right)
Axial CECT of patient with
autosomal dominant
polycystic liver disease.
Innumerable hepatic cysts;
no renal cysts.

Variant
(Left) Axial T2WI MR shows
a large and very
hyperintense hepatic cyst
with no mural nodularity or
septations. (Right) Axial
CECT following blunt
trauma. Peripheral water
density contents have
ruptured through the capsule
of the liver. Higher density
hemorrhage is present in
dependent aspect of cyst.
 HEPATIC CAVERNOUS HEMANGIOMA
1
102

Graphic shows nonencapsulated collections of blood Axial CECT in venous-parenchymal phase shows
within enlarged sinusoidal spaces. Otherwise normal spherical mass with nodular discontinuous peripheral
liver. enhancement that is nearly isodense to blood vessels.

o May be multiple in up to 50% of cases

ITERMINOLOGY
Abbreviations and Synonyms
• Cavernous hemangioma of liver; capillary
hemangioma (small lesion)
Definitions
• Benign tumor composed of multiple vascular channels
lined by a single layer of endothelial cells supported by
a thin fibrous stroma
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Peripheral nodular enhancement
on arterial phase (AP) scan with slow progressive
centripetal enhancement isodense to vessels
• Location: Common in subcapsular area in posterior
right lobe of liver
• Size
o Vary from few millimeters to more than 20 cm
o Giant hemangiomas: Larger than 10 cm (arbitrary)
• Morphology
o Most common benign tumor of liver
o Second most common liver tumor after metastases
o More commonly seen in postmenopausal women
o Usually solitary & grow slowly
o Calcification is rare (less than 10%)
• Usually in scar of giant hemangioma
CT Findings
• NECT
o Small (1-2 cm) & typical hemangioma (2-10 cm)
• Well-circumscribed, spherical to ovoid mass
isodense to blood
o Giant hemangioma (more than 10 cm)
• Heterogeneous hypodense mass
• Central decreased attenuation (scar)
• CECT
o Small hemangiomas ("capillary"): Less than 2 cm
• Arterial & venous phases: Show homogeneous
enhancement ("flash filling")
o Typical hemangiomas: 2-10 cm in diameter
• Arterial phase: Early peripheral, nodular or
globular, discontinuous enhancement
• Venous phase: Progressive centripetal
enhancement to uniform filling, still isodense to
blood vessels
• Delayed phase: Persistent complete filling
o Giant hemangioma: More than 10 cm in diameter
• Arterial phase: Typical peripheral nodular or
globular enhancement
• Venous & delayed phases: Incomplete centripetal
filling of lesion (scar does not enhance)
o Atypical hemangioma: Inside to outside pattern

DDx: Focal lesion with Persistent or Delayed Enhancement

/'-

()

,
Cholangiocarcinoma
""'" e
Cholangiocarcinoma
4

Metastases Metastases
 HEPATIC CAVERNOUS HEMANGIOMA
Key Facts
Terminology
• Benign tumor composed of multiple vascular
channels lined by a single layer of endothelial cells
supported by a thin fibrous stroma
Imaging Findings
• Best diagnostic clue: Peripheral nodular enhancement
on arterial phase (AP) scan with slow progressive
centripetal enhancement isodense to vessels
• Most common benign tumor of liver
• Second most common liver tumor after metastases
• May be multiple in up to 50% of cases
Top Differential Diagnoses
• Peripheral (intrahepatic) cholangiocarcinoma
• Hypervascular metastases
• Arterial phase: No significant enhancement
• Venous & delayed phases: Gradual enhancement
from center to periphery (centrifugal filling)
o Hyalinized (sclerosed) hemangioma
• Shows minimal enhancement
• Cannot be diagnosed with confidence by imaging
o Hemangioma in cirrhotic liver
• Flash-filling of small lesions
• May lose characteristic enhancement pattern
• Capsular retraction
• Decrease in size over time
MR Findings
• TlWI
o Small & typical hemangiomas
• Well marginated
• Isointense to blood or hypointense
o Giant hemangioma
• Hypointense mass
• Central cleft like area of marked decreased
intensity (scar or fibrous tissue)
• T2WI
o Small & typical hemangiomas
• Hyperintense, similar to CSF
o Giant hemangioma
• Hyperintense mass
• Marked hyperintense center (scar or fibrosis)
• Hypointense internal septa
• T1 C+
o Small hemangiomas (less than 2 cm)
• Homogeneous enhancement in arterial & portal
phases
o Typical & giant hemangiomas
• Arterial phase: Peripheral, nodular discontinuous
enhancement
• Venous phase: Progressive centripetal filling
• In both phases: Isointense to blood
• Central scar: No enhancement & remains
hypointense
Ultrasonographic Findings
• Real Time
o Small hemangioma
1
Pathology 103
• Hemangiomas occur sporadically
• Associated with focal nodular hyperplasia (FNH)
Clinical Issues
• Usually asymptomatic
• More common in postmenopausal age group
Diagnostic Checklist
• Small hepatocellular carcinomas & hypervascular
metastases can mimic small hemangiomas by their
uniform homogeneous enhancement pattern
• Hemangiomas: Remain isodense to blood vessels on
portal venous & delayed phases of enhancement
• Other benign & malignant liver masses: Usually
become hypodense to blood vessels & liver (except
cholangiocarcinoma)
• Well-defined, hyperechoic lesion
• Size: Less than 2 cm
o Typical hemangioma
• Homogeneous hyperechoic mass with acoustic
enhancement
• Size: 2-10 cm
o Giant hemangioma
• Lobulated heterogeneous mass with echoic border
• Size: More than 10 cm
o Atypical hemangioma
• Well-defined
• Iso-/hypoechoic mass with echoic rim
• Color Doppler
o Show filling vessels in periphery of tumor
o No significant color Doppler flow in center of lesion
• Power Doppler
o May detect flow within hemangiomas
o Flow pattern is nonspecific
o Similar flow pattern may be seen in hepatocellular
carcinoma & metastases
Angiographic Findings
• Conventional
o Dense opacification of lesion
o "Cotton wool" appearance
• Pooling of contrast medium within hemangioma
o Normal-sized feeders
o No neovascularity
o No arteriovenous shunting
o Typically retain contrast beyond venous phase
Nuclear Medicine Findings
• Tc-99m labeled RBC scan with SPECT (95% accuracy)
o Early dynamic scan: Focal defect or less uptake
o Delayed scans (over 30-50 min): Persistent filling
o Vascular tumors (adenoma, HCC & FNH)
• All exhibit early uptake rather than a defect
• May have persistent uptake on delayed scan
o Rarely angiosarcomas exhibit hemangioma pattern
• Early defect & late uptake of isotope
Imaging Recommendations
• Best imaging tool: Helical NE + CECT or MR
• Protocol advice: Arterial, venous & delayed scans
 HEPATIC CAVERNOUS HEMANGIOMA
1 I DIFFERENTIAL DIAGNOSIS • Diagnosis
o Helical CECT, CEMR or RBC scan with SPECT
104 Peripheral (intrahepatic) imaging are highly diagnostic
o Atypical hemangioma
cholangiocarcinoma • Percutaneous or fine needle aspiration biopsy
• Delayed persistent enhancement, "fill in" may mimic
hemangioma Demographics
• Often heterogeneous, not isodense with vessels on CT • Age
• Not bright on T2WI o All age groups
• Often invades/obstructs vessels & bile ducts o More common in postmenopausal age group
o Uncommonly diagnosed in children
Hypervascular metastases • Gender: M:F = 1:5
• Usually multiple
• Hyperdense in late arterial phase images Natural History & Prognosis
• Hypo-or isodense on NECT & portal venous phase • Complications (extremely rare)
• Treated metastases may mimic hemangioma on o Spontaneous rupture
imaging (e.g., breast) o Abscess formation
• Not isodense to vessels on NECT or CECT • Prognosis: Usually good
• Examples: Islet cell, carcinoid, thyroid, renal o Often show slow growth
carcinomas, pheochromocytoma & some breast
cancers Treatment
• Asymptomatic: Usually ignore
• Symptomatic large lesions: Surgical resection
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
• Etiology
o Hemangiomas occur sporadically Consider
o No well-defined predisposing factors • Small hepatocellular carcinomas & hypervascular
• Epidemiology metastases can mimic small hemangiomas by their
o Incidence uniform homogeneous enhancement pattern
• Ranging from 5-20% of population • Hemangiomas: Remain isodense to blood vessels on
• Increases with multiparity portal venous & delayed phases of enhancement
o Prevalence: Uniform worldwide • Other benign & malignant liver masses: Usually
• Associated abnormalities become hypodense to blood vessels & liver (except
o Associated with focal nodular hyperplasia (FNH) cholangiocarcinoma)
o Kasabach-Merritt syndrome Image Interpretation Pearls
• Hemangioma with thrombocytopenia
• Peripheral nodular or globular enhancement on
Gross Pathologic & Surgical Features arterial phase & centripetal enhancement on venous
• Solitary, well-defined, blood-filled, soft nodule phase is a useful discriminating feature of
o Size ranging from 2·20 cm hemangiomas from other lesions
• Cut section: Giant hemangioma
o Areas of fibrosis, necrosis & cystic spaces
I SELECTED REFERENCES
Microscopic Features 1. Danet 1Met a1:Giant hemangioma of the liver: MR
• Large vascular channels lined by single layer of imaging characteristics in 24 patients. Magn Reson
endothelial cells separated by thin fibrous septa Imaging. 21(2):95-101, 2003
• No bile ducts 2. Brancatelli G et a1: Hemangioma in the cirrhotic liver:
• Thrombosis of vascular channels resulting Diagnosis and natural history. Radiology. 219: 69-74, 2001
o Fibrosis & calcification 3. Kim T et al: Discrimination of small hepatic hemangiomas
from hypervascular malignant tumors smaller than 3 em
with three-phase helical CT. Radiology. 219: 699-706, 2001
4. Jeong MG et al: Hepatic cavernous hemangioma: temporal
ICLINICALISSUES peritumoral enhancement during multiphase dynamic MR
imaging. Radiology. 216(3):692-7, 2000
Presentation 5. Vilgrain V et al: Imaging of atypical hemangiomas of the
• Most common signs/symptoms liver with pathologic correlation. RadioGraphies. 20:
o Small & typical hemangioma 379-97, 2000
• Usually asymptomatic 6. Leslie DF et al: Distinction between cavernous
• Commonly seen on routine examination & hemangiomas of the liver and hepatic metastases on CT:
autopsy value of contrast enhancement patterns. AJRAm J
Roentgenol. 164(3):625-9, 1995
o Giant hemangioma
• Asymptomatic
• Liver enlargement, abdominal discomfort & pain
• Lab data: Normal liver function tests
 HEPATIC CAVERNOUS HEMANGIOMA

I IMAGE GALLERY 1
105
Typical
(Left) Axial NECT shows
large mass in lateral segment,
most of which is isodense to
blood except for hypodense
foci of scar. (Right) Axial
CECT in venous
parenchymal phase shows
cloud-like peripheral
enhancement that is
isodense to vessels.

Typical
(Left) Sagittal sonogram
shows uniformly
hyperechoic lesion in
peripheral right lobe. (Right)
Axial CECT in venous phase
shows typical large
hemangioma with nodular
peripheral enhancement and
nonenhancing scar (arrow).
Capillary hemangioma
(curved arrow) isodense to
vessels in all phases.

Variant
(Left) Axial CECT in
venous-parenchymal phase
shows nodular peripheral
enhancement and
nonenhancing central scar
(arrow) in a very large
hepatic cavernous
hemangioma. (Right) Axial
T2WI MR shows large mass
with central hyperintense
scar (black arrow) that is
even more intense. Several
other hemangiomas were
noted (white arrow).
 FOCAL NODULAR HYPERPLASIA
1
106

Homogeneous vascular; nonencapsulated mass with Axial CECT in arterial phase shows homogeneous
central scar and thin radiating septa dividing mass into hypervascular mass with central scar and thin radiating
hyperplasuc nodules. Otherwise normal liver. septa.

o Usually a solitary lesion (80%); multiple in 20%

ITERMINOLOGY o Multiple FNHs associated with multiorgan vascular
Abbreviations and Synonyms malformations and with certain brain neoplasms
• Focal nodular hyperplasia (FNH) CT Findings
Definitions • NECT: Isodense or hypodense to normal liver
• Benign tumor of liver caused by hyperplastic response • CECT
o Hepatic arterial phase (HAP) scan
to a localized vascular abnormality
• Transient intense hyperdensity
o Portal venous phase (PVP) scan
• Hypodense or isodense to normal liver
IIMAGING FINDINGS o Delayed scans
General Features • Mass: Isodense to liver
• Best diagnostic clue: Brightly and homogeneously • Central scar: Hyperdense
enhancing mass in arterial phase CT or MR with • Scar visible in 2/3rd of large & 1/3rd of small FNH
delayed enhancement of central scar MR Findings
• Location
o More common in right lobe • TlWI
o Mass: Isointense to slightly hypointense
o Right lobe to left lobe: 2:1 o Central scar: Hypointense
o Usually subcapsular & rarely pedunculated
• T2WI
• Size o Mass: Slightly hyperintense to isointense
o Majority are smaller than 5 cm (85%)
o Central scar: Hyperintense
o Mean diameter at time of diagnosis is 3 cm
• T1 C+
• Key concepts o Arterial Phase: Hyperintense (homogeneous)
o 2nd most common benign tumor of liver
o Portal Venous: Isointense
o Benign congenital hamartomatous malformation
o Delayed phase
o Accounts for 8% of primary hepatic tumors in
• Mass: Isointense
autopsy series
• Scar: Hyperintense

DDx: Uniformly Hypervascular liver Mass

Hepatic Adenoma Small Hemangioma HCC Metastases

 FOCAL NODULAR HYPERPLASIA

Terminology
• Benign tumor of liver caused by hyperplastic
to a localized vascular abnormality
Imaging Findings
• Best diagnostic clue: Brightly and homogeneously
enhancing mass in arterial phase CT or MR with
delayed enhancement of central scar
• Usually subcapsular & rarely pedunculated
Top Differential Diagnoses
• Hepatic adenoma
• Cavernous hemangioma
• Fibrolamellar carcinoma
• Hepatocellular carcinoma
• Hypervascular metastasis
Key Facts
1
Pathology 107
response • Oral contraceptives don't cause FNH, but have
trophic effect on growth
• No intratumoral calcification, hemorrhage or necrosis
• Size: Less than 5 cm (in 85%)
Clinical Issues
• Often asymptomatic (in 50-90% incidental finding)
• 3rd-4th decades of life
Diagnostic Checklist
• Classic FNH looks like a cross-section of an orange
(central "scar", radiating septa)
• Radiologically FNH may mimic fibrolamellar HCC,
which is usually a large lesion (> 12 cm), has evidence
of calcification (in 68%) & metastases in 70% cases

• Specific hepatobiliary MR contrast agents
o T2WI with superparamagnetic iron oxide (SPIO)
• FNH shows decreased signal due to uptake of iron
oxide particles by Kupffer cells within lesion
• Degree of signal loss in FNH is greater than other
focal liver lesions (metastases, adenoma & HCC)
o Gadobenate dimeglumine (Gd-BOPTA)
• Bright homogeneous enhancement of FNH
• Prolonged enhancement of FNH on delayed scan
(due to malformed bile ductules)
• Delayed scan: Significant enhancement of scar
o Only FNH has both Kupffer cells & bile ductules
o Almost PATHOGNOMONIC in 60% of cases
• Tc-HIDA scan (hepatic iminodiacetic acid)
o Normal or increased uptake
o Prolonged enhancement (80%)
• Tc 99m-Tagged RBC scan (not useful)
o Early isotope uptake & late defect
Imaging Recommendations
• Helical CT or MR (multi-phase studies); Tc-99m-sulfur
colloid scan

Ultrasonographic Findings
• Real Time I DIFFERENTIAL DIAGNOSIS
o Well-demarcated liver lesion
o Mass: Mostly homogeneous & isoechoic to liver Hepatic adenoma
• Occasionally hypoechoic or hyperechoic • Large tumor
o Central scar: Hypoechoic • Symptomatic due to hemorrhage in 50%, scar atypical
o Prominent draining veins or displacement of vessels • Usually heterogeneous due to hemorrhage, necrosis or
• Color Doppler fat
o "Spoke-wheel" pattern
• Large central feeding artery with multiple small Cavernous hemangioma
vessels radiating peripherally • Only small ones with rapid enhancement simulate
o Large draining veins at tumor margins FNH
o High-velocity Doppler signals • NECT: Isodense with blood vessels
• Due to increased blood flow or arteriovenous • CECT: Peripheral enhanced areas stay isodense with
shunts blood vessels

Angiographic Findings Fibrolamellar carcinoma

• Conventional • Large (more than 12 cm) heterogeneous mass
o Arterial phase • Biliary, vascular & nodal invasion
• Tumor: Hypervascular • Metastases (70% of cases)
• Scar: Hypovascular • Fibrous scar
• Enlargement of main feeding artery with a o Large & central or eccentric with fibrous bands &
centrifugal blood supply calcification (68%)
• "Spoke-wheel" pattern" as on color-Doppler o Hypointense scar on T2WI
o Venous phase: Large draining veins
Hepatocellular carcinoma
o Capillary phase
• Heterogeneous mass within cirrhotic liver
• Intense & nonhomogeneous stain
• Necrosis & hemorrhage
• No avascular zones
• Vascular & nodal invasion
Nuclear Medicine Findings
Hypervascular metastasis
• Technetium Sulfur Colloid
o Normal or increased uptake • Multiple lesions; older patient
 FOCAL NODULAR HYPERPLASIA
1 • Hypodense during portal venous phase
108
I PATHOLOGY
General Features
• Genetics
o In genetic hemochromatosis patients, FNH cells
were homozygous for Cys282Tyr mutation
o Ki-67 antigen positive in 4% of FNH hepatocytes
• Etiology
o Ischemia caused by an occult occlusion of
intrahepatic vessels
o Localized arteriovenous shunting caused by
anomalous arterial supply
o Hyperplastic response to an abnormal vasculature
o Oral contraceptives don't cause FNH, but have
trophic effect on growth
• Epidemiology
o 4% of all primary hepatic tumors in pediatric
population
o 3-8% in adult population
• Associated abnormalities
o Hepatic hemangioma (in 23%)
o Multiple lesions of FNH are associated with
• Brain neoplasms: Meningioma, astrocytoma
• Vascular malformations of various organs
Gross Pathologic & Surgical Features
• Localized, well-delineated, usually solitary (80%),
subcapsular mass
• No true capsule, frequently central fiprous scar
• No intratumoral calcification, hemorrhage or necrosis
• Multiple masses (in 20%), rarely pedunculated
• Size: Less than 5 em (in 85%)
Microscopic Features
• Normal hepatocytes with large amounts of fat,
triglycerides & glycogen
• Thick-walled arteries in fibrous septa radiating from
center to periphery
• Proliferation & malformation of bile ducts lead to
slowing of bile excretion
• Absent portal triads & central veins
• Difficult differentiation from regenerative cirrhotic
nodule & liver adenoma
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Often asymptomatic (in 50-90% incidental finding)
o Vague abdominal pain (10-15%) due to mass effect
o Other signs/symptoms
• Hepatomegaly & abdominal mass (very rare)
o Lab data: Usually normal liver function tests
o Diagnosis .
• Characteristic imaging findings
• Core needle biopsy (include central scar)
Demographics
• Age
o Common in young to middle-aged women
• 3rd-4th decades of life
oRange: 7 months to 75 years
• Gender: M:F = 1:8
Natural History & Prognosis
• Excellent
Treatment
• Discontinuation of oral contraceptives
• FNH seldom requires surgery
I DIAGNOSTIC CHECKLIST
Consider
• To rule out other benign & malignant liver lesions
particularly fibrolamellar hepatocellular carcinoma
Image Interpretation Pearls
• Immediate, intense, homogeneously enhancing lesion
on arterial phase followed rapidly by isodensity on
venous phase with delayed enhancement of scar
• Classic FNH looks like a cross-section of an orange
(central"scar", radiating septa)
• Radiologically FNH may mimic fibrolamellar HCC,
which is usually a large lesion (> 12 em), has evidence
of calcification (in 68%) & metastases in 70% cases
• Atypical FNH (telangiectatic FNH): Lack of central scar,
heterogeneous lesion, hyperintense on T1WI,
markedly hyperintense on T2WI & has persistent
contrast-enhancement on delayed CECT & T1 C+
o Probably can not make this diagnosis by imaging
I SELECTED REFERENCES
1. Attal P et al: Telangiectatic focal nodular hyperplasia: US,
CT, and MR imaging findings with histopathologic
correlation in 13 cases. Radiology. 228(2):465-72, 2003
2. Vilgrain V et al: Prevalence of hepatic hemangioma in
patients with focal nodular hyperplasia: MR imaging
analysis. Radiology. 229(1):75-9, 2003
3. Brancatelli G et al: Focal nodular hyperplasia: CT findings
with emphasis on multiphasic helical CT in 78 patients.
Radiology. 219: 61-8, 2001
4. Grazioli L et al: Focal nodular hyperplasia: Morphologic
and functional information from MR imaging with
gadobenate dimeglumine. Radiology. 221: 731-9,2001
5. Casillas VJ et al: Imaging of nontraumatic hemorrhagic
hepatic lesions. Radiographies. 20(2):367-78, 2000
6. Leconte I et al: Focal nodular hyperplasia: Natural course
observed with CT and MRI. Journal of computer assisted
tomography. 24(1): 61-6, 2000
7. Horton KM et al: CT and MR imaging of benign hepatic
and biliary tumors. Radiographies. 19(2):431-51, 1999
8. Buetow PC et al: Focal nodular hyperplasia of the liver:
radiologic-pathologic correlation. Radiographies.
16(2):369-88, 1996
9. Caseiro-Alves F et al: Calcification in focal nodular
hyperplasia: a new problem for differentiation from
fibrolamellar hepatocellular carcinoma. Radiology.
198(3):889-92, 1996
 FOCAL NODULAR HYPERPLASIA

I IMAGE GALLERY 1
109
Typical
(Left) Axial CECT in arterial
phase shows intense
homogeneous enhancement
of mass with central scar
with thin radiating septa.
Typical FNH appearance
resembles cross section of an
orange. (Right) Axial CECT in
venous phase shows mass
(arrow) isodense to liver
with delayed enhancement
of central scar.

Typical
(Left) Axial T1 C+ MR in
arterial phase shows intense
homogeneous enhancement
of mass with central
hypointense scar. (Right)
Axial T2WI MR shows mass
is minimally hyperintense,
scar (arrow) is hyperintense.

Variant
(Left) Axial CECT in arterial
phase shows atypical
heterogeneous, rather than
homogeneous, enhancement
of mass. Note central scar
and large draining vein
(arrow). (Right) Tc-HIDA
scan shows normal liver
uptake and biliary excretion.
Gallbladder (open arrow).
Large peripheral exophytic
FNH (arrow) shows
increased uptake and
delayed excretion of
radiotracer.
 HEPATIC ADENOMA
1
110

Graphic shows hypervascular mass in posterior right Axial CECT in venous phase shows hepatic subcapsular
lobe and spontaneous subcapsular bleeding. hematoma and heterogeneous mass (arrow) which
bled spontaneously.

o Associated with a number of diseases & etiologic

ITERMINOlOGY factors
Abbreviations and Synonyms CT Findings
• Hepatocellular adenoma (HCA) or liver cell adenoma
• NECT
Definitions o Well-defined, spherical mass
• Benign tumor that arises from hepatocytes arranged in o Isodense to hypodense (due to lipid)
cords that occasionally form bile o Hemorrhage: Intratumoral, parenchymal or
subcapsular
o Fat or calcification seen (less often than on MR)
I IMAGING FINDINGS • CECT
o Arterial phase
General Features • Heterogeneous, hyperdense enhancement
• Best diagnostic clue: Heterogeneous, hypervascular o Portal venous phase
mass with hemorrhage in a young woman • Less heterogeneous
• Location • Hyper-, iso-, hypodense to liver
o Subcapsular region of right lobe of liver (75%) o Delayed phase (10 min)
o Intraparenchymal or pedunculated (10%) • Homogeneous, hypodense
• Size • Enhancement does not persist (due to
o Varies between 6-30 cm arteriovenous shunting)
o Average size: 8-10 cm • Pseudocapsule: Hyperattenuated to liver &
• Key concepts adenoma
o Rare benign neoplasm o Large adenomas
o Second most frequent hepatic tumor in young • More heterogeneous than smaller lesions
women after FNH due to use of oral contraceptive MR Findings
steroids
o Usually single (adenoma)i rarely multiple • TlWI
o Mass: Heterogeneous signal intensity
(adenomatosis)

DDx: Heterogeneously Hypervascular liver Mass

, J
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,
HCC Fibmlamellar HCC
"
Atypical FNH Metastases
 HEPATIC ADENOMA
Key Facts
1
Pathology 111
Terminology
• Hepatocellular adenoma (HCA) or liver cell adenoma • Hemorrhage, necrosis & fatty change
• Benign tumor that arises from hepatocytes arranged • No scar within tumor
in cords that occasionally form bile • t Risk in oral contraceptives & anabolic steroid users

Imaging Findings Clinical Issues

• Best diagnostic clue: Heterogeneous, hypervascular
mass with hemorrhage in a young woman
• Subcapsular region of right lobe of liver (75%)
• Average size: 8-10 cm
Top Differential Diagnoses
• Hepatocellular carcinoma (HCC)
• Fibrolamellar hepatocellular carcinoma
• Focal nodular hyperplasia (FNH)
• Hypervascular metastases
• RUQ pain (40%): Due to hemorrhage
• May be mistaken clinically/pathologically for HCC
• Clinical profile: Woman on oral contraceptives
Diagnostic Checklist
• Rule out other benign & malignant liver tumors
which have similar imaging features, particularly
HCCor FNH
• Check for history of oral contraceptives & glycogen
storage disease (in case of multiple adenomas)

• Increased signal intensity (due to fat & recent

hemorrhage), more evident on MR than CT Angiographic Findings
• Decreased signal intensity (necrosis, calcification, • Conventional
old hemorrhage) o Hypervascular mass with centripetal flow
o Rim (fibrous pseudocapsule): Hypointense o Enlarged hepatic artery with feeders at tumor
• T2WI periphery (50%)
o Mass: Heterogeneous signal intensity o Hypovasculari avascular regions
• Increased signal intensity (old • Due to hemorrhage & necrosis
hemorrhage/necrosis) Nuclear Medicine Findings
• Decreased signal intensity (fat, recent
• Technetium Sulfur Colloid
hemorrhage)
o Usually "cold" (photopenic): In 80%
o Rim (fibrous pseudocapsule): Hypointense
o Uncommonly "warm": In 20%
• T1 C+
• Due to uptake in sparse Kupffer cells
o Gadolinium arterial phase
• HIDA scan
• Mass: Heterogeneous enhancement
o Increased activity
o Delayed phase
• Gallium Scan
• Pseudocapsule: Hyperintense to liver & adenoma
o No uptake
• Superparamagnetic iron oxide (SPIO)
o No uptake in adenoma Imaging Recommendations
o Few cases take up SPIO (due to active Kupffer cells) • T2WI; T1WI with dynamic enhanced multiphasic;
• Resulting in a decreased signal on T2WI GRE in - and opposed-phase images
• Indistinguishable from FNH for these cases
• Uptake varies based on number of Kupffer cells
• Gd-BOPTA (gadobenate dimeglumine)
o Hepatocellular-specific contrast agent
I DIFFERENTIAL DIAGNOSIS
o Adenoma Hepatocellular carcinoma (HCC)
• No substantial uptake • May have identical imaging features as hepatic
• Hypointense even on delayed images adenoma
Ultrasonographic Findings • Histologically: May be difficult to distinguish
well-differentiated HCC from adenoma
• Real Time • Biliary, vascular, nodal invasion & metastases establish
o Well-defined, solid, echo genic mass
that lesion is malignant
o Complex hyper & hypoechoic heterogeneous mass
with anechoic areas Fibrolamellar hepatocellular carcinoma
• Due to fat, hemorrhage, necrosis & calcification • Large, lobulated mass with scar & septa
• Color Doppler • Vascular, biliary, nodal invasion common
o Hypervascular tumor • Heterogeneous on all imaging
o Large peripheral arteries & veins
o Intratumoral veins present Focal nodular hyperplasia (FNH)
• Absent in FNH • No malignant degeneration or hemorrhage
• Useful discriminating feature for HCA • T2WI: Scar is typically hyperintense
• Arterial phase
o FNH is homogeneously enhancing mass
 HEPATIC ADENOMA
1 o Central scar: Hypoattenuating/hypointense
• Delayed phase
o May be mistaken clinically/pathologically
• Clinical profile: Woman on oral contraceptives
for HCC

112 o Central scar: Hyperdense/hyperintense • Lab data: Usually normal liver function tests
• Small (:0; 3 em) FNH without scar indistinguishable • Diagnosis: Biopsy & histology
from adenoma
Demographics
Hypervascular metastases • Age
• Usually multiple & look for primary tumors o Young women of childbearing age group
o Breast, thyroid, kidney and endocrine o Predominantly in 3rd & 4th decades
• Arterial phase: Heterogeneous enhancement • Gender
• Portal & delayed phases: Iso-/hypodense o 98% seen in females (M:F = 1:10)
• T1WI: Hypointense o Not seen in males unless on anabolic steroids or
• T2WI: Markedly hyperintense with glycogen storage disease
Natural History & Prognosis
• Complications
\ PATHOLOGY o Hemorrhage: Intrahepatic or intraperitoneal (40%)
General Features o Rupture: Increased risk in pregnancy
• General path comments o Risk of malignant transformation
o HCA: Surrounded by a fibrous pseudocapsule • When size is more than 10 cm (in 10%)
• Due to compression of adjacent liver tissue • Prognosis
o High incidence of o Usually good
• Hemorrhage, necrosis & fatty change • After discontinuation of oral contraceptives
o No scar within tumor • After surgical resection of large/symptomatic
o Poor
• Etiology
o 1 Risk in oral contraceptives & anabolic steroid users • Intraperitoneal rupture
o Pregnancy • Rupture during pregnancy
• Increase tumor growth rate and tumor rupture • Adenomatosis (> 10 adenomas)
o Diabetes mellitus • Malignant transformation
o Von-Gierke type la glycogen storage disease Treatment
• Multiple adenomas: 60% • Adenoma less than 6 cm
• Epidemiology o Observation & discontinue oral contraceptives
o Estimated incidence in oral contraceptive users • Adenoma more than 6 cm & near surface
• 4 Adenomas per 100,000 users o Surgical resection
Gross Pathologic & Surgical Features • Pregnancy should be avoided due to increased risk of
• Well-circumscribed mass on external surface of liver rupture
• Soft, pale or yellow tan
• Frequently bile-stained nodules
• Large areas of hemorrhage or infarction \DIAGNOSTIC CHECKLIST
• "Pseudocapsule" & occasional"pseudopods" Consider
Microscopic Features • Rule out other benign & malignant liver tumors which
• Sheets or cords of hepatocytes have similar imaging features, particularly HCC or
• Absence of portal & central veins & bile ducts FNH
• Increased amounts of glycogen & lipid • Check for history of oral contraceptives & glycogen
• Scattered, thin-walled, vascular channels storage disease (in case of multiple adenomas)

Staging, Grading or Classification Criteria Image Interpretation Pearls

• Typical hepatocellular adenoma (HCA)
o Type I: Estrogen associated HCA
o Type II: Spontaneous HCA in women
o Type III: Spontaneous HCA in men
o Type IV: Spontaneous HCA in children
o Type V: Metabolic disease associated HCA
• Anabolic steroid-associated HCA
• Multiple hepatocellular adenomas (adenomatosis)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o RUQ pain (40%): Due to hemorrhage
o Asymptomatic (20%)
• Spherical well-defined hypervascular & heterogeneous
mass due to hemorrhage & fat, most evident on MR
• Adenomas with uptake of SPIO agent mimic FNH on
T2WI as decreased signal
I SELECTED REFERENCES
1. Grazioli L et al: Hepatic adenomas: Imaging and pathologic
findings. RadioGraphies. 21: 877-94, 2001
2. Ichikawa T et al: Hepatocellular adenoma: Multiphasic CT
and histopathologic findings in 25 patients. Radiology.
214: 861-8, 2000
3. Grazioli L et al: Liver adenomatosis: Clinical, pathologic
and imaging findings in 15 patients. Radiology. 216:
395-402, 2000
 HEPATIC ADENOMA

I IMAGE GALLERY
1
113
Typical
(Left) Axial CECT shows
hypervascular mass (arrow)
with nonenhancing foci.
Hepatic adenoma which
required surgical resection.
(Right) Axial T2WI MR
shows large encapsulated
hepatic mass with
heterogeneously increased
signal intensity.

(Left) Axial T1WI MR shows

hypointense encapsulated
mass with hyperintense foci
(hemorrhage or fat). (Right)
Axial T2WI MR shows mass
nearly isointense to liver with
central focus of
hyperintensity (hemorrhage).

Variant
(Left) Axial NECT shows very
large, mostly homogeneous
mass with small focus of
hemorrhage (arrow). (Right)
Axial CECT in arterial phase
shows hypervascularity with
enlarged vessels within and
on surface of tumor.
 BILIARY HAMARTOMA
1
114

Axial NECT in an asymptomatic patient with biliary Axial CECT of same patient. Many of the hamartomas
hamartomas shows innumerable subcentimeter are now isodense with liver.
hypodense foci in both lobes of the liver.

o Solitary or multiple small, well-defined nodules of

!TERMINOLOGY varied density
Abbreviations and Synonyms • Multiple hamartomas: Seen as an aggregation of
• Von Meyenburg complexes tiny lesions
• Multiple biliary hamartomas o Density of lesions: Depending on predominant
• Multiple bile duct hamartomas cystic/solid component
• Predominantly cystic: Water density
Definitions • Predominantly solid (fibrous stroma): Decreased
• Rare benign malformations of biliary tract attenuation
o Distribution & size
• Both uniform & nonuniform in distribution
IIMAGING FINDINGS • Relatively uniform compared to nonuniform
metastases
General Features • This pattern is nonspecific
• Best diagnostic clue: Multiple near water • Biopsy is needed to diagnose
density/intensity liver lesions < 1.5 cm diameter • Varied size: 2-15 mm
• Location • CECT
o Subcapsular or intraparenchymal in location o Varied enhancement based on
o Scattered throughout both lobes of liver • Cystic or solid components of lesions
• Size: Varies from less than 1.0 to 1.5 cm o Predominantly cystic (water density) lesions
• Key facts • No enhancement
o Typically well-circumscribed but not encapsulated o Predominantly solid (fibrous stroma) lesions
• May occur as • Enhance & become isodense with liver tissue
o Multiple lesions (more common) or
o Isolated solitary lesion MR Findings
• TlWI: Hypointense (both cystic & solid lesions)
CT Findings • T2WI
• NECT o Hyperintense (cystic lesions)
o Intermediate intensity (solid lesions)

DDx: Multiple Hepatic "Cystic" lesions

, 5

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e
it
\
ADPLD "
Hepatic Cysts Caroli Disease Liver Metastases
 BILIARY HAMARTOMA
1
Key Facts
Terminology Pathology 115
• Von Meyenburg complexes • Biliary hamartomas are one of the variants of
• Multiple biliary hamartomas congenital hepatic fibropolycystic disease
• Multiple bile duct hamartomas • Other variants include: Polycystic liver disease &
• Rare benign malformations of biliary tract Caroli disease
• Congenital (embryological/developmental)
Imaging Findings malformation
• Best diagnostic clue: Multiple near water
density/intensity liver lesions < 1.S cm diameter Clinical Issues
• Subcapsular or intraparenchymal in location • Varies; asymptomatic - RUQ pain
• Malignant transformation to cholangiocarcinoma
Top Differential Diagnoses (very rare)
• Autosomal dominant polycystic disease (ADPLD)
• Multiple simple hepatic cysts Diagnostic Checklist
• Caroli's disease • No further evaluation needed when seen as isolated
• Multiple/solitary small metastatic lesions finding in a healthy non oncologic patient

o Heavily T2WI o ERCP: Endoscopic retrograde

• Signal intensity increases further (equal to fluid) cholangiopancrea tography
• Tl C+ o PTC: Percutaneous trans hepatic cholangiography
o Predominantly cystic lesions: No enhancement • Usually have cysts in kidneys & other organs
• ± Thin rim-enhancement on early & late • ± Family history
post-gadolinium images
• Compressed surrounding liver tissue & Multiple simple hepatic cysts
inflammatory cell infiltrate • Multiple well-defined, round, nonenhancing,
o Predominantly solid lesions: Enhancement seen water-density lesions
• Due to fibrous stroma • Cysts are usually seen in a normally appearing liver
• MR Cholangiography (MRC) • Usually solitary, can be multiple as in ADPLD
o Markedly hyperintense nodules • US: Anechoic lesion with through transmission; no
o Typically measuring less than 1.S cm mural nodularity
o No communication with biliary tree
Caroli's disease
Ultrasonographic Findings • Inherited as an autosomal recessive pattern
• Real Time • Multiple small rounded hypodense/hypointense
o Small & well-circumscribed lesions saccular dilatation of IHBDs
o Scattered throughout liver • "Central dot" sign on CECT
o Hypo-/hyperechoic or mixed-echoic o Enhancing tiny dots (portal radicles) within dilated
• Based on solid, cystic or both contents cystic intrahepatic ducts
• ERCP & microscopically: Communicating bile duct
Angiographic Findings abnormality
• Conventional: Lesions show abnormal vascularity:
Grape-like clusters of small rings Multiple/solitary small metastatic lesions
• Metastases: Usually nonuniform in size & distribution
Imaging Recommendations of lesions
• Best imaging tool: High-resolution CT or MRC • Biopsy & histology: Distinguishes hamartomas from
• Protocol advice metastases
o Helical CT
• S mm collimated scans reconstructed every 2.S
mm I PATHOLOGY
o MR: Heavily T2WI!MRC
General Features
• General path comments
I DIFFERENTIAL DIAGNOSIS o Rare benign malformations of bile ducts
o Usually diagnosed
Autosomal dominant polycystic disease • At autopsy or imaging as an incidental finding
(ADPlD) o Biliary hamartomas are one of the variants of
• Usually large & numerous hepatic cysts congenital hepatic fibropolycystic disease
• Do not communicate with each other or biliary tract o Other variants include: Polycystic liver disease &
• Do not opacify at Caroli disease
o Increase prevalence of bile duct hamartomas in
polycystic liver disease cases
 BILIARY HAMARTOMA
1 o Malignant transformation: Hamartoma to • Usually occur as multiple lesions (more common)
cholangiocarcinoma (very rare) • Scattered throughout both lobes of liver
116 • Genetics: No genetic predisposition • Follow-up imaging usually shows no change in
• Etiology appearance
o Congenital (embryological/developmental) • Complications
malformation o Infection: Microabscess formation (rare)
• Due to failure of involution of embryonic bile o Malignant transformation to cholangiocarcinoma
ducts (very rare)
o Same pathogenesis for polycystic liver disease • Prognosis: Usually good
• Prevalence of bile duct hamartomas is greater in
patients with polycystic liver disease than in those Treatment
without • Options
• These two entities may coexist o Asymptomatic: No treatment
• Epidemiology: Very rare; autopsy incidence (0.69%) o Symptomatic: Exploratory laparotomy & surgical
resection
Gross Pathologic & Surgical Features
• Multiple grayish white nodules of varying sizes
ranging from solid to cystic I DIAGNOSTIC CHECKLIST
• Subcapsular or intraparenchymal
• Biliary dilatation within hamartomas: Consider
Narrow/mild/prominent • MRC to differentiate biliary hamartomas abnormalities
o From other biliary
Microscopic Features (communicating/non communicating)
• Proliferation of dilated bile ducts (bile duct
duplication) Image Interpretation Pearls
• Surrounded by dense, hyalinized fibrous stroma • No further evaluation needed when seen as isolated
• No communication exists with biliary system finding in a healthy nononcologic patient
• Cystic hamartomas: Lined by cuboidallflattened • Possibility of misdiagnosing as multiple small liver
epithelium metastases

Staging, Grading or Classification Criteria

• Classification based on degree of hamartoma I SELECTED REFERENCES
consistency/biliary dilatation (not widely used) Orii T et al: Cholangiocarcinoma arising from preexisting
1.
o Class 1: Predominantly solid lesions + narrow bile biliary hamartoma of liver--report of a case.
channels Hepatogastroenterology. 50(50):333-6, 2003
o Class 2: Intermediate lesions (both solid & cystic 2. Mortele B et al: Hepatic bile duct hamartomas (von
foci) + mild dilatation Meyenburg Complexes): MR and MR cholangiography
o Class 3: Predominantly cystic lesions + prominent findings. J Comput Assist Tomogr. 26(3):438-43, 2002
dilated bile channels 3. Allgaier HP et al: Ampullary hamartoma: A rare cause of
biliary obstruction. Digestion. 60(5):497-500, 1999
4. von Schweinitz D et al: Mesenchymal hamartoma of the
liver--new insight into histogenesis. J Pediatr Surg.
I CLINICAL ISSUES 34(8):1269-71, 1999
5. Semelka RC et al: Biliary hamartomas: solitary and multiple
Presentation lesions shown on current MR techniques including
• Most common signs/symptoms gadolinium enhancement. J Magn Reson Imaging.
o Asymptomatic; occasionally dull pain & fullness in 10(2):196-201, 1999
right upper quadrant (RUQ) 6. Principe A et al: Bile duct hamartomas: diagnostic
o Abdomen: Soft/non tender/no palpable masses or problems and treatment. Hepatogastroenterology.
hepatosplenomegaly 44(16):994-7, 1997
7. Cheung YC et al: MRI of multiple biliary hamartomas. BrJ
• Clinical profile Radiol. 70(833):527-9, 1997
o Varies; asymptomatic ~ RUQ pain 8. Iha H et al: Biliary hamartomas simulating multiple
o Lab data hepatic metastasis on imaging findings. Kurume Med J.
• Normal liver function tests 43(3):231-5, 1996
• Normal bilirubin assays 9. Lev-Toaff AS et al: The radiologic and pathologic spectrum
• Normal carcinoembryonic antigen (CEA) levels of biliary hamartomas. AJRAm J Roentgenol.
o Diagnosis: Wedge or core-needle biopsy & histologic 165(2):309-13, 1995
10. Powers C et al: Primary liver neoplasms: MR imaging with
exam
pathologic correlation. Radiographies. 14(3):459-82, 1994
Demographics
• Age: Any age group (usually incidental finding)
• Gender: M = F
Natural History & Prognosis
• Rare congenital, non communicating bile duct
abnormality
 BILIARY HAMARTOMA
I IMAGE GALLERY 1
117
Typical
(Left) Axial NECT shows
multiple small hypodense
lesions in liver. (Right) Axial
CECT of same patient shows
multiple small nonenhancing
lesions of varied sizes
distributed predominantly in
the right lobe of liver;
consistent with biliary
hamartomas.

(Left) Axial T1 C+ MR shows

numerous small
nonenhancing hypointense
lesions diffusely involving the
liver. (Right) Axial T2WI MR
in the same patient
demonstrates multiple small
hyperintense foci of varied
sizes throughout both lobes
of liver (biliary hamartomas).

Typical
(Left) Axial NEeT in a
cirrhotic liver shows multiple
subcentimeter hypodense
lesions (biopsy proven biliary
hamartomas). (Right)
Transverse US of the liver
shows diffuse replacement of
normal parenchyma with
small hyperechoic foci
consistent with biliary
hamartomas.
 HEPATIC ANGIOMYOLIPOMA
1
118

Axial TlWI MR shows heterogeneous hepatic mass with Axial T2WI MR shows heterogeneous mass with fatty
some hyperintense foci indicating fat component. component nearly isointense to subcutaneous fat; other
tumor foci are typically hyperintense.

o Well-defined mass with heterogeneous attenuation

ITERMINOlOGY values due to presence of fat & soft tissue densities
Abbreviations and Synonyms o May be predominantly low density mass
• Hepatic angiomyolipoma (AML), benign liver • CECT
hamartoma o Arterial phase: Prominent enhancement of lesion
o Portal phase: Lesion shows hypoattenuation
Definitions o On early phase of dynamic study, enhancement is
• Benign mesenchymal tumor composed of variable higher than on late phase, but it has prolonged
amounts of smooth muscle (myoid), fat (lipoid), & enhancement (due to proliferation of blood vessels)
proliferating blood vessels (angioid) components • CTA: Central vessels within lesion
MR Findings
I IMAGING FINDINGS • TlWI
o Hypointensity or hyperintensity on Tl WI
General Features o Fatty component of tumor results in hyperintense
• Best diagnostic clue: Well-circumscribed fatty maSSi (high signal) foci on Tl WI
successful diagnosis relies on identification of o Relative loss of signal intensity on opposed-phase
intra tumoral fat at imaging images compared with in-phase; qualitative
• Location assessment of relatively small amounts of lipid
o Liver: Second most common site (first: Kidney) o Frequency-selective fat saturation techniques useful
o In liver: Right lobe is most common site • T2WI
• Size: Variablei range from 0.3-36 cm in diameter o High signal intensity of fatty components
• Key concepts o Heterogeneous hyperintensity
o Round or lobulated solitary mass or as multiple • Tl C+: Soft tissue elements within lesion enhance
lesions with variable shape Ultrasonographic Findings
CT Findings • Real Time
o Homogeneous or heterogeneous echogenic mass due
• NECT
to fat

DDx: Fat Containing liver Mass

Focal Steatosis Hepatic Adenoma Metastatic Teratoma

 HEPATIC ANGIOMYOLIPOMA

Key Facts .
1
Terminology • On early phase of dynamic study, enhancement IS 119
higher than on late phase, but it has prolonged
• Benign mesenchymal tumor composed of variable
enhancement (due to proliferation of blood vessels)
amounts of smooth muscle (myoid), fat (lipoid), &
proliferating blood vessels (angioid) components Pathology
Imaging Findings • Associated with tuberous sclerosis
• Best diagnostic clue: Well-circumscribed fatty mass; Clinical Issues
successful diagnosis relies on identification of • Complication: Spontaneous hemorrhage or rupture
intra tumoral fat at imaging

o If muscle, vascular elements or hemorrhage

predominate, lesion may be hypoechoic
I CLINICAL ISSUES

Angiographic Findings Presentation

• Heterogeneously hypervascular tumor • Asymptomatic & discovered incidentally at imaging
• Pain results from intra tumoral hemorrhage
Imaging Recommendations
• Best imaging tool: MR; fat suppression ± opposed
Natural History & Prognosis
phase GRE imaging • Complication: Spontaneous hemorrhage or rupture
• No malignant potential
Treatment
I DIFFERENTIAL DIAGNOSIS • Conservative, embolization, surgical resection
Hepatocellular carcinoma (HCC)
• Fat within tumor may be localized or show a diffusely
scattered or a mosaic pattern I DIAGNOSTIC CHECKLIST
• Fat is usually a minor component of HCC tumor mass Consider
Focal steatosis • Small fat-density hepatic mass in tuberous sclerosis
• Usually poorly defined & not well circumscribed patient is benign
• Shows blood vessels traversing lesion
Hepatic adenoma I SELECTED REFERENCES
• Well defined as surrounded by capsule 1. Takayama Y et al: Hepatic angiomyolipoma: radiologic and
• Hypoattenuating mass with heterogeneous histopathologic correlation. Abdom Imaging. 27(2):180-3,
attenuation; areas of hemorrhage & infarction within 2002
2. Yoshimura H et al: Angiomyolipoma of the liver with least
Hepatic lipoma amount of fat component: imaging features of CT, MR,
• No enhancement on incremental bolus dynamic CT and angiography. Abdom Imaging. 27(2):184-7, 2002
3. Balci NC et al: Hepatic angiomyolipoma: demonstration by
Metastases (teratoma or liposarcoma) out of phase MRI. Clin Imaging. 26(6):418-20, 2002
• Fat containing; ± fluid, calcification in teratoma
• Most liposarcomas are large, well-circumscribed;
vascular structures with soft tissue attenuation I IMAGE GALLERY

I PATHOLOGY
General Features
• Associated with tuberous sclerosis
Gross Pathologic & Surgical Features
• Fat content varies; < 10% to > 90% of tumor volume
• Usually yellow-to-light tan, secondary to fat content
Microscopic Features
• Epithelioid smooth muscle cells, admixture of mature
fat cells & proliferating blood vessels
(Left) Axial NECT shows large hepatic mass with foci of fat density
(arrow); angiomyolipoma. (Right) Axial CECT in patient with
tuberous sclerosis (prior right nephrectomy for angiomyolipoma).
Almost pure fat density liver mass (arrow).
 HEPATOCELLULAR CARCINOMA
1
120

Graphic shows heterogeneous vascular mass invading Axial CECT in venous phase shows hypodense mass in
portal vein. Surrounding liver is cirrhotic with fibrosis. cirrhotic liver which invades, and occludes the posterior
Varices and ascites. branch of right portal vein (arrows). Nonocclusive
thrombus in main portal vein.

o Most frequent primary visceral malignancy in world

[TERMINOLOGY • Accounts 80-90% of all primary liver malignancies
Abbreviations and Synonyms o 2nd most common malignant liver tumor in
• Hepatocellular carcinoma (HCC); hepatoma or children after hepatoblastoma
primary liver cancer o Growth patterns of HCC: Three major types
• Solitary, often large mass
Definitions • Nodular or multi focal
• Most common primary malignant liver tumor usually • Diffuse or cirrhotomimetic
arising in cirrhotic liver due to chronic viral hepatitis
CT Findings
(HBV/HCV) or alcoholism
• NECT
o In non cirrhotic liver
[IMAGING FINDINGS • Solitary HCC: Large hypo dense mass; ± necrosis,
fat, calcification
General Features • Multifocal HCC: Multiple hypodense lesions
• Best diagnostic clue: Large heterogeneous rarely with a central necrotic portion
hypervascular mass with portal vein invasion • Dominant hypodense mass with decreased
• Location attenuation satellite nodules
o Most often right lobe of liver (solitary) • Encapsulated HCC: Well-defined, rounded,
o Both hepatic lobes (multicentric small nodular) hypodense mass
o Throughout liver in a diffuse manner (diffuse small o In cirrhotic liver
foci) • Iso-/hypodense mass
• Size • Nodular cirrhotic liver
o Small tumors: Less than 3 cm • Ascites & varices
o Large tumors: More than 5 cm • CECT
o Diffuse or cirrhotomimetic: Subcentimeter to few o Hepatic arterial phase (HAP) scan
cms • Heterogeneous enhancement
• Key concepts

DDx: Hypervascular Mass(es) in Dysmorphic liver

Choalgiocarcinoma Metastases Small Hemangioma

 HEPATOCELLULAR CARCINOMA

Key Facts
1
Terminology Pathology 121
• Most common primary malignant liver tumor usually • Invasion: Vascular (common) & biliary (uncommon)
arising in cirrhotic liver due to chronic viral hepatitis • Clear cell carcinoma: HCC with large amounts of fat
(HBV!HCV) or alcoholism • Cirrhosis (60-90%): Due to chronic viral hepatitis
(HBY,HCV) or alcoholism
Imaging Findings • Worldwide highest incidence is in Japan (4.8%)
• Best diagnostic clue: Large heterogeneous • North America: 40% of HCC in non-cirrhotic livers
hypervascular mass with portal vein invasion
• Most often right lobe of liver (solitary) Clinical Issues
• Clinical profile: Elderly patient with history of
Top Differential Diagnoses
cirrhosis, ascites, weight loss, RUQ pain & t AFP
• Cholangiocarcinoma
• Hypervascular metastases Diagnostic Checklist
• Focal nodular hyperplasia (FNH) • HCC: Hypervascular mass invading portal vein
• Small hepatic hemangioma • Small HCC may mimic hemangioma or metastasis in
cirrhotic liver

• Wedge-shaped areas of increased density on HAP: o Increases sensitivity of MR in diagnosing

Perfusion abnormality due to portal vein • Small HCCs in cirrhotic livers
occlusion by tumor thrombus & increased arterial • Mangafodipir trisodium (Mn-DPDP)
flow o Tl WI: Increased signal in well-differentiated HCC
o Portal venous phase (PVP) scan o Differentiates HCC from nonhepatocellular tumors
• Decreased attenuation with heterogeneous areas
of contrast accumulation Ultrasonographic Findings
o Delayed scan: Hypodense to surrounding liver • Real Time
o Small hypervascular HCC o Mixed echogenicity due to tumor necrosis &
• Early & late arterial phases: Hyperattenuating, hypervascularity
more on late phase o Hypoechoic: Due to solid tumor
• CT hepatic arteriography: Lesions show intense o Hyperechoic: Due to fatty metamorphosis
enhancement • Small hyperechoic HCC simulate hemangioma
• CT during arterial portography: No enhancement o Capsule in encapsulated HCC
• Thin hypoechoic band
MR Findings • Color Doppler
• Variable intensity depending on degree of fatty o Shows hypervascularity & tumor shunting
change, fibrosis, necrosis o Small HCC: Indistinguishable from small
• TlWI hemangiomas & metastases
o Noncirrhotic liver
• Hypointense; iso-/hyperintense Angiographic Findings
o Cirrhotic liver • Conventional
• HCC: Hypointense o Hypervascular tumor
• Cirrhotic nodules: Increased signal intensity • Marked neovascularity & AV shunting
• T2WI • Large hepatic artery & vascular invasion
o Noncirrhotic liver: Slightly hyperintense o "Threads & streaks" sign
o Cirrhotic liver • Sign of tumor thrombus in portal vein
• HCC: Hyperintense
• Cirrhotic nodules: Iso to hypointense
Nuclear Medicine Findings
o HCC arising within a siderotic nodule • Hepato Biliary Scan
• "Nodule within a nodule" pattern o Uptake in 50% of lesions
• HCC appears as a small focus of increased signal • Technetium Sulfur Colloid
intensity within decreased signal intensity nodule o HCC in a cirrhotic liver: Seen as a defect
• Tl C+ (gadolinium) o HCC in a noncirrhotic liver: Heterogeneous uptake
o Large HCC in noncirrhotic liver: Nonspecific • Gallium Scan
• Central or peripheral enhancement o HCC is gallium avid in 90% of cases
• Homogeneous or rim-enhancement Imaging Recommendations
o HCC nodules (hypervascular)
• Helical triphasic CT (NE, arterial & venous phases) or
• Arterial phase: Hyperintense MR & CEMR; angiography
• SPIO (superparamagnetic iron oxide)
o FLASH& long TR sequences
o HCC: Higher signal than surrounding liver
• Liver takes up SPIO more than lesion
 HEPATOCELLULAR CARCINOMA
1 I DIFFERENTIAL DIAGNOSIS I CLINICAL ISSUES
122 Cholangiocarcinoma Presentation
• Peripheral tumor often obstructs bile ducts • Clinical profile: Elderly patient with history of
• Capsular retraction; volume loss cirrhosis, ascites, weight loss, RUQ pain & 1 AFP
• Delayed enhancement • Lab data: Increased alpha-fetoprotein (AFP) & LFTs
• Diagnosis: Biopsy & histology
Nodular regenerative hyperplasia (as in
Budd-Chiari syndrome) Demographics
• Called "large regenerative nodules" • Age
o Low incidence areas: 6th-7th decade
• Small nodules: Not detectable
o High incidence areas: 30-45 years
• Large nodules: Homogeneously hypervascular
• Usually 1-4 cm in size • Gender
o Low incidence areas (M:F = 2.5:1)
Hypervascular metastases o High incidence areas (M:F = 8:1)
• Mimic small nodular or multifocal HCC Natural History & Prognosis
• Less likely to invade portal vein
• Complications
Focal nodular hyperplasia (FNH) o Spontaneous rupture & massive hemoperitoneum
• Homogeneous hypervascular mass with central scar • Prognosis
• On non enhanced & delayed CECT & CEMR almost o More than 90% mortality rate; 17% resectability rate
isodense/isointense to liver o 6 Months average survival time; 30% 5 year survival

Small hepatic hemangioma Treatment

• Well-defined, spherical mass isodense to blood
• CECT: "Flash filling" (still isodense with blood)
• On US: Usually hyperechoic nodule
• Angiography: Characteristic "cotton wool" appearance
I PATHOLOGY
General Features
• General path comments
o Soft tumor; may have necrosis & hemorrhage
o Invasion: Vascular (common) & biliary (uncommon)
o Clear cell carcinoma: HCC with large amounts of fat
• Genetics: HBV DNA integrated into host's genomic
DNA in tumor cells
• Etiology
o Cirrhosis (60-90%): Due to chronic viral hepatitis
(HBV, HCV) or alcoholism
o Carcinogens
• Aflatoxins, siderosis, thorotrast, androgens
o ()(-l-antitrypsin deficiency, hemochromatosis
o Wilson disease, tyrosinosis
• Epidemiology
o High incidence: Africa & Asia (HBV & aflatoxins)
o Low incidence: Western hemisphere
o Worldwide highest incidence is in Japan (4.8%)
o HCC in cirrhosis due to hepatitis C virus
• United States: 30-50% of cases of HCC
• Japan: 70% of cases of HCC
o North America: 40% of HCC in non-cirrhotic livers
Gross Pathologic & Surgical Features
• Solitary, nodular or multifocal, diffuse, encapsulated
• Soft tumor with or without necrosis, hemorrhage,
calcification, fat, vascular invasion
Microscopic Features
• Histologic appearances: Solid (cellular) or acinar
• Increased fat & glycogen in cytoplasm
• Radiofrequency & alcohol ablation
o Small isolated tumors
• Intraarterial chemoembolization
o Multifocal unresectable tumor
• Surgical resection
o Limited by inadequate hepatic reserve
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
• HCC: Hypervascular mass invading portal vein
• Small HCC may mimic hemangioma or metastasis in
cirrhotic liver
I SELECTED REFERENCES
1. Laghi A et al: Hepatocellular carcinoma: detection with
triple-phase multi-detector row helical CT in patients with
chronic hepatitis. Radiology. 226(2):543-9, 2003
2. Brancatelli G et al: Hepatocellular carcinoma in
non cirrhotic liver: CT, clinical and pathologic findings in
39 D.S residents. Radiology. 222: 89-94, 2002
3. Kim T et al: Discrimination of small hepatic hemangiomas
from hypervascular malignant tumors smaller than 3 cm
with three-phase helical CT. Radiology. 219(3):699-706,
2001
4. Murakami T et al: Hyper vascular hepatocellular
carcinoma: Detection with double arterial phase
multi-detector row helical CT. Radiology. 218: 763-7, 2001
5. Peterson MS et al: Pretransplantation surveillance for
possible hepatocellular carcinoma in patients with
cirrhosis: Epidemiology and CT-based tumor detection rate
in 430 cases with surgical pathologic correlation.
Radiology. 217: 743-9, 2000
6. Oliver JH 3rd et al: Detecting hepatocellular carcinoma:
value of unenhanced or arterial phase CT imaging or both
used in conjunction with conventional portal venous
phase contrast-enhanced CT imaging. AJR. 167(1):71-7,
1996
 HEPATOCELLULAR CARCINOMA

I IMAGE GALLERY 1
123
Typical
(Left) Axial CECT in arterial
phase shows hypervascular
mass in right lobe, cirrhotic
liver. (Right) Axial CECT in
portal venous phase. Mass is
isodense to liver; hyperdense
capsule delineates tumor.

Typical
(Left) Axial T7 C+ MR in
arterial phase shows
heterogeneous hypervascular
mass. (Right) Axial T2WI MR
barely detects mass as subtle
hyperintense lesion.

Variant
(Left) Axial CECT in arterial
phase shows heterogeneous
mass in right lobe with
enhancing tumor thrombus
in IVC (arrow) and right
hepatic vein. The liver mass
had foci of fat most evident
on NECT. (Right) Axial CECT
in arterial phase shows large
heterogeneous hypervascular
mass that occupies the right
lobe, and causes intrahepatic
biliary obstruction (arrow).
 FIBROLAMELLAR HCC
1
124

Graphic shows large heterogeneous hypervascular mass Axial CECT shows large heterogeneous hypervascular
with central scar and porta hepatis lymphadenopathy. mass with large calcified central scar and cardiophrenic
lymphadenopathy.

• Eosinophilic malignant hepatocytes containing

ITERMINOlOGY prominent nuclei
Abbreviations and Synonyms o Absence of pathologic markers like inclusions of
alpha-fetoprotein bodies which are present in
• Fibrolamellar carcinoma (FLC) of liver
typical HCC
Definitions o Better prognosis than conventional HCC, but still
• Uncommon malignant hepatocellular tumor with locally invasive and frequently metastatic
distinct clinical, histopathologic & imaging differences
CT Findings
from conventional hepatocellular carcinoma (HCC)
• NECT
o Mass
• Well-defined contour
IIMAGING FINDINGS • Hypoattenuating and heterogeneous
General Features o Central scar & septa: Markedly hypodense
• Best diagnostic clue: Heterogeneously-enhancing, o Calcification & necrosis are common
large, lobulated mass with hypointense central scar o Hemorrhage is rarely seen
and radial septa on T2WI • CECT
o Arterial phase
• Location
o Intrahepatic (80%) • Mass: Heterogeneous & hyperattenuating
o Pedunculated (20%) o Portal phase
• Size: Vary from 5-20 em (mean 13 em) • Mass: Iso-/hypoattenuating
o Delayed phase (10 min)
• Key concepts
o Slow-growing tumor that usually arises in a normal • Mass: Isodense
liver • Scar/septa/capsule: Hyperdense
o In less than 5% cases it may occur with underlying o Malignant features
cirrhosis • Biliary & vessel invasion
o Satellite nodules are often present • Nodal metastases (2/3rd cases)
o Characteristic microscopic pattern • Lung metastases

DDx: Hepatic Mass with Central Scar

1":;:\ .
~;:
':.'
....-., "- ...
(L;~~~· •
r~~:r ~.
?- ..·~''-8~'
tW

o~.;.::
\,~·T....~'..
\.,: ..
....... .
FNH HCC Giant Hemangioma Cholangiocarcinoma
 FIBROLAMELLAR HCC

Key Facts
Terminology
• Fibrolamellar carcinoma (FLC) of liver
• Uncommon malignant hepatocellular tumor with
distinct clinical, histopathologic & imaging
differences from conventional hepatocellular
carcinoma (HCC)
Imaging Findings
• Best diagnostic clue: Heterogeneously-enhancing,
large, lobulated mass with hypointense central scar
and radial septa on T2WI
• Size: Vary from 5-20 cm (mean 13 cm)
Top Differential Diagnoses
• Focal nodular hyperplasia (FNH)
• Conventional (HCC)
• Cavernous hemangioma
1
• Intrahepatic cholangiocarcinoma 125
Pathology
• No specific risk factors
• FLC accounts for 1-9% of HCC overall
Clinical Issues
• Usually ()(-fetoprotein levels are normal
Diagnostic Checklist
• FLC: Bigger, more heterogeneous mass frequently
with calcified central/eccentric scar & features of
malignancy (vessel/biliary obstruction, nodal
invasion & lung metastases)
• Conventional HCC can be differentiated from FLC by
underlying cirrhosis (more common) & lack of scar

• Tagged red blood cell scan (not useful)

MR Findings o FLC: Early uptake & late defect
• TlWI o Hemangioma: Early defect & late uptake
o Mass: Homogeneous & slightly hypointense
oScar & septa: Hypointense Imaging Recommendations
• T2WI • Multiphasic helical CT (NECT plus arterial and
o Mass: Heterogeneous & hyperintense venous) or multiphasic MR
oScar & septa: Hypointense
• T1 C+
o Arterial & portal phases I DIFFERENTIAL DIAGNOSIS
• Mass: Intense heterogeneous enhancement
• Scar: No enhancement Focal nodular hyperplasia (FNH)
o Delayed phase • Marked homogeneous enhancement on arterial phase
• Mass: More homogeneous enhancement CT or MR
• Scar & septa: Delayed partial enhancement • Scar: Hyperintense on T2WI
• Nonencapsulated & no calcification
Ultrasonographic Findings • Substantial uptake of SPIO
• Real Time • Tc-99m-Iabeled colloid: Highly specific for FNH
o Mass • Usually asymptomatic
• Large, solitary, well-defined & lobulated • Microscopic pattern
• Variable echotexture o Normal hepatocytes; disorganized bile ductules
o Central scar: Hyperechoic
Conventional (HCC)
Angiographic Findings • Usually underlying cirrhosis is seen
• Conventional • Vascular, nodal & visceral invasion: Common
o Mass • Intratumoral hemorrhage, necrosis, calcification & fat
• Hypervascular (neovascularity) (more common)
• Enlarged feeding arteries • May be multifocal
• Dense tumor blush • Conventional HCC in noncirrhotic liver mimics FLC
• No A-V or A-P shunting • Pathologic markers
o Septa o Inclusions of alpha-fetoprotein bodies are present
• Multiple serpiginous hypovascular areas
o Central scar: Avascular Cavernous hemangioma
o Satellite nodule • Giant hemangioma
• May be seen in capillary phase o Heterogeneous hypodense mass
o Central decreased attenuation (scar)
Nuclear Medicine Findings o Size: Usually more than 10 cm
• Tc-99m-Iabeled sulfur colloid o Arterial phase: Peripheral nodular or globular
o Solitary enhancement
• Single photopenic defect o Venous & delayed phases
o Multifocal • Incomplete centripetal filling of lesion
• Multiple defects • No enhancement of scar
• Difficult to differentiate from multifocal • Typical hemangioma: Well-circumscribed, spherical to
HCC/metastases ovoid mass isodense to blood on both NECT & CECT
 FIBROLAMELLAR HCC
1 Intrahepatic cholangiocarcinoma o Rarely marked increase in levels (10,000 ng/~l)
similar to conventional HCC
126 • May be central (hilar) or peripheral • Diagnosis: Biopsy & histology
• Peripheral: Hypodense solitary or satellite lesions
• Intrahepatic bile duct dilatation seen in both Demographics
• No central scar but extensive fibrosis • Age
• Early rim enhancement with progressive, central and o Adolescents/young adults
persistent patchy enhancement • Age range of 5-69 years (mean 23 years)
• Often causes hepatic volume loss and capsular o Most patients present in 2nd/3rd decade of life
retraction • Gender: M:F = 1:1
Natural History & Prognosis
I PATHOLOGY •
•
Resectability rate: 48%
FLC is frequently recurrent
General Features • Average survival time: 32 months
• Etiology • 5-year survival: 67%
o No specific risk factors • Better prognosis compared to conventional HCC
o Usually no underlying cirrhosis or liver disease
Treatment
o Occasionally hepatitis & cirrhosis may be present
• In less than 5% of cases • Localized tumor
o Surgical resection of hepatic mass & regional nodes
• Epidemiology
o May resect isolated lung metastases
o FLC
• Inoperable cases: Chemotherapy
• Increased prevalence in US
• Less common in Europe
• Rare in Japan & China
o FLC accounts for 1-9% of HCC overall I DIAGNOSTIC CHECKLIST
o Represents 35% of HCC under 50 years of age Consider
Gross Pathologic & Surgical Features • Differentiate FLC from FNH & conventional HCC
• Large, single, well-demarcated, lobulated, • FLC simulates FNH due to presence of central scar in
non encapsulated mass both tumors
• Cut section: Tan, brown or brownish green with Image Interpretation Pearls
streaks of fibrous tissue • FLC: Bigger, more heterogeneous mass frequently with
• Infiltrating fibrous septa; central scar (45-60%) calcified central/eccentric scar & features of
• Rarely encapsulated malignancy (vessel/biliary obstruction, nodal invasion
• Solitary mass (80-90%) & lung metastases)
• Peripheral satellite lesions (10-15%) • Scar on T2WI: Hypointense (FLC); hyperintense (FNH)
• Intrahepatic (80%); pedunculated (20%) • Conventional HCC can be differentiated from FLC by
• Size: Average size is 13 em, most vary from 5-20 em underlying cirrhosis (more common) & lack of scar
Microscopic Features
• Large eosinophilic, polygonal cells
o Arranged in sheets/cords/trabeculae I SELECTED REFERENCES
o Separated by parallel sheets of fibrous tissue (Le., 1. Ichikawa T et al: Fibrolamellar hepatocellular carcinoma:
lamellae) Pre- and posttherapy evaluation with CT and MR imaging.
• Large nuclei with prominent nucleoli Radiology. 217: 145-51,2000
2. Mclarney J et al: Fibrolamellar carcinoma of the liver:
• Granular-appearing cytoplasm
Radiologic-pathologic correlation. RadioGraphics. 19:
453-71, 1999

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Pain, hepatomegaly, palpable RUQ mass, cachexia
o Occasionally jaundice when invades biliary tract
o Symptoms
• Usually present for 3-12 months before diagnosis
o Rarely present with
• Metastatic disease, fever, gynecomastia
• Venous thrombosis (hepatic, portal & IVC)
• Clinical profile: Healthy young adult with large liver
mass
• Laboratory data
o Usually (){-fetoprotein levels are normal
o In 10% cases: Mild increase in levels « 200 ng/~l)
3. Ichikawa T et al: Fibrolamellar hepatocellular carcinoma:
Imaging and pathologic findings in 31 recent cases.
Radiology. 213: 352-61, 1999
4. Schlitt HJ et al: Recurrence patterns of hepatocellular and
fibrolamellar carcinoma after liver transplantation. J Clin
Oncol. 17(1):324-31, 1999
5. Stevens WR et al: Fibrolamellar hepatocellular carcinoma:
stage at presentation and results of aggressive surgical
management. AJRAm J Roentgenol. 164(5):1153-8, 1995
6. Brandt DJ et al: Imaging of fibrolamellar hepatocellular
carcinoma. AJRAm J Roentgenol. 151 (2):295-9, 1988
7. Titelbaum DS et al: Fibrolamellar hepatocellular carcinoma:
pitfalls in nonoperative diagnosis. Radiology. 167(1):25-30,
1988
 FIBROLAMELLAR HCC
1
I IMAGE GALLERY
127

(Left) Axial T1 C+ MR in
arterialphase shows
dominant and satellite
masses that are
well-demarcated, lobulated,
heterogeneous and
hypervascular. (Right) Axial
T2WI MR shows hypointense
eccentric scar (white arrow)
and hyperintense foci of
necrosis (black arrow).

(Left) Axial NECT shows in

77 year old male with
palpable epigastric mass.
Large hypodense mass with
calcified central scar. (Right)
Axial CECT in arterial phase
shows heterogeneous,
hypervascular enhancement.
Note radiating septa
converging at central
calcified scar.

Typical
(Left) Cut section of resected
tumor shows
well-demarcated,
heterogeneous tumor with
bile staining and
central/eccentric fibrous
scars (arrows). (Right) Axial
CECT in venous phase shows
large, heterogeneous,
lobulated mass with calcified
central scar.
 CHOlANGIOCARCINOMA (PERIPHERAL)
1
128

Axial CECT in portal venous phase shows Axial CECT in delayed phase shows increased and
heterogeneous infiltrative mass with intrahepatic biliary persistent enhancement of the tumor due to its fibrous
obstruction and volume loss with capsular retraction. stroma.

• Multicentricity, especially around main tumor

!TERMINOlOGY o Periductal-infiltrating CC: Grows along bile ducts &
Abbreviations and Synonyms is elongated, spiculated, or branch-like
o Intraductal-growing CC: Small, sessile, or polypoid,
• Cholangiocellular carcinoma, intrahepatic
often spreading superficially along mucosal surface
cholangiocarcinoma, peripheral cholangiocarcinoma
& resulting in multiple tumors (papillomatosis)
(PCe)
along various segments of bile ducts
Definitions
CT Findings
• Cholangiocarcinoma (Ce) is an adenocarcinoma that
arises from bile duct epithelium • NECT
o Well-defined, single, predominantly homogeneous
• Tumor that arises peripheral to secondary bifurcation
hypodense mass
of left or right hepatic duct is considered to be
• With lobular margins
peripheral cholangiocarcinoma (PCe)
• Hypodense satellite, daughter nodules (65%)
• Punctate, stippled, chunky calcifications (18%)
o Intrahepatic bile duct dilatation (IHBD) peripheral
IIMAGING FINDINGS to tumor
General Features • May not be a constant finding even if tumor arises
from one of the intrahepatic ducts
• Best diagnostic clue: Infiltrative hepatic mass with
capsular retraction and delayed persistent • CECT
enhancement (CECT and MR) o Mass-forming PCC
• Thin or thick, rim-like enhancement frequently
• Location: Originates from interlobular bile ducts (Le.,
seen around periphery of tumor on arterial phase
bile ducts distal to second-order branches)
• Size: Mass-forming PCC is usually large, 5-15 cm in images
• Progressive, gradual & concentric filling
diameter
(centripetal) on delayed phase images (usually not
• Key concepts
isodense to blood, unlike cavernous hemangioma)
o Mass-forming PCC: Well-circumscribed with
• Substantial delayed enhancement (Le., greater
lobulated margins
than that of liver parenchyma) is common (74%)

DDx: Hepatic Mass with Biliary Obstruction

I --"
~ ---
.) -- .•..
,

(~~
Metastases Fibrolamellar HCC Fibrolamellar HCC
 CHOlANGIOCARCINOMA
Key Facts
Terminology
• Cholangiocarcinoma (CC) is an adenocarcinoma that
arises from bile duct epithelium
• Tumor that arises peripheral to secondary bifurcation
of left or right hepatic duct is considered to be
peripheral cholangiocarcinoma (PCC)
Imaging Findings
• Best diagnostic clue: Infiltrative hepatic mass with
capsular retraction and delayed persistent
enhancement (CECT and MR)
• Location: Originates from interlobular bile ducts (i.e.,
bile ducts distal to second-order branches)
• Size: Mass-forming PCC is usually large, 5-15 cm in
diameter
• Best imaging tool: Helical CT/MR + MRC/ERCP
• Fibrotic component within contributes to delayed
tumoral contrast-enhancement
• Homogeneous hyperattenuating enhancement
• Entire mass may be enhanced only on delayed
phase images
• Only evidence of tumor may be on delayed
images; it may be missed without them
• Delayed imaging demonstrates tumor margins
more optimally
• Tumors with delayed enhancement tend to be
fibrous; however degree of contrast material
retention does not always correlate with fibrous
content of tumors at histopathology
• ± Capsular retraction (frequent), with
parenchymal atrophy of liver segments peripheral
to tumor
o Periductal-infiltrating CC: Bile ducts proximal to
PCC are dilated & involved bile ducts are diffusely
narrow or obliterated without an identifiable mass
• Ill-defined, branch-like, low-attenuation
o Intraductal-growing CC: Presents with focal or
segmental bile duct dilatation
• Tumor may not be depicted when it is small &
isoattenuating to adjacent hepatic parenchyma or
when complex orientation of dilated bile ducts
obscures presence of mass
MR Findings
• T1WI: Large central heterogeneous hypointense mass
• T2WI
o Hyperintense periphery (cellular tumor) + large
central hypointensity (fibrosis)
o Hyperintense foci in center may represent necrosis,
mucin
• T1 C+
o Central hypointense areas exhibiting homogeneous,
heterogeneous, or no enhancement
• Regions of fibrosis display enhancement, whereas
those of coagulative necrosis, cell debris & mucin
show no enhancement
o Dynamic MR: Minimal or moderate rim
enhancement with progressive & concentric filling
with contrast material
(PERIPHERAL)
1
Top Differential Diagnoses 129
• Metastases
• Hepatocellular carcinoma (HCC)
• Fibrolamellar hepatocellular carcinoma
Pathology
• Large, firm, white tumor with dense fibrosis, irregular
margins & capsular retraction
Clinical Issues
• PCC presents as large mass because tumor does not
cause clinical symptoms in its early stages
Diagnostic Checklist
• Hepatocellular-cholangiocarcinoma should be
considered when a hepatic tumor has features of both
• Intratumoral fibrous stroma display marked or
prolonged enhancement on delayed phase scans
• Some cases of PCC exhibiting little fibrosis may
show early enhancement on dynamic studies
• MRA: Displacement or encasement of adjacent vessels
Ultrasonographic Findings
• Real Time
o Mass forming PCC: Homogeneous or heterogeneous
mass with irregular borders & satellite nodules
• Hyperechoic (75%); iso- and/or hypoechoic (14%)
mass
o IHBD of involved hepatic segment may contain
calculi or intraductal mass (echogenic): Mucin is
echo-free
Angiographic Findings
• Avascular, hypoc or hypervascular mass
• Stretched, encased arteries (frequent); neovascularity
in 50%; venous invasion (rarely)
Nuclear Medicine Findings
• Cold lesion on sulfur colloid scans
• May show uptake on gallium scan
Other Modality Findings
• ERCP/percutaneous transhepatic cholangiography:
o Periductal-infiltrating CC: Lumen of bile duct may
be completely obstructed or stringlike, severely
narrowed bile duct may be seen
o Intraductal CC: Biliary tree is dilated (partial
obstruction); diffusely, lobarly, or segmentally, or
aneurysm ally
Imaging Recommendations
• Best imaging tool: Helical CT/MR + MRC/ERCP
• Protocol advice: Delayed contrast-enhanced images,
obtained 5-20 minutes after contrast injection; ideal
timing of delayed images has not been established
 CHOlANGIOCARCINOMA
1 I DIFFERENTIAL DIAGNOSIS
130 Metastases
• Hepatic colorectal metastases: Metastatic
adenocarcinoma histologically same as PCC, mimic
mass-forming PCC on imaging
• Look for IRBD/bile duct disease:
Clonorchiasis/sclerosing cholangitis; may indicate
PCC
Hepatocellular carcinoma (HCC)
• Hypervascularity on arterial phase, hypodense on
portal venous & delayed-phase CT
• Delayed enhancement within fibrous capsule
• Satellite lesions, venous invasion, IHBD, regional
lymphadenopathy
Fibrolamellar hepatocellular carcinoma
• Large, lobulated, heterogeneous mass with central scar
• Delayed partial enhancement of fibrous scar & septa
• Calcification (scar), lymphadenopathy are common
I PATHOLOGY
General Features
• Etiology: Associated with several etiological factors:
Primary sclerosing cholangitis, bile stasis, repeated
cholangitis, clonorchiasis, congenital cystic disease of
liver, hepatolithiasis, Thorotrast deposition
• Epidemiology
o 8-13% of all CC are peripheral/intrahepatic
o PCC is relatively rare cancer; world wide it accounts
for an estimated 15% of liver cancer
Gross Pathologic & Surgical Features
• Large, firm, white tumor with dense fibrosis, irregular
margins & capsular retraction
Microscopic Features
• 90% are adenocarcinomas
• Tendency to spread between hepatocyte plates, along
duct walls, & adjacent to nerves
• Mucin production is often abundant
• Most of mass-forming PCC are poorly-differentiated;
most periductal-infiltrating are well-differentiated;
most intraductal are papillary adenocarcinomas
Staging, Grading or Classification Criteria
• Based on growth characteristics: Mass-forming
(exophytic/nodular); peri ductal-infiltrating
(sclerosing); intraductal-growing (polypoid/papillary)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Abdominal pain (84%), weight loss (77%), painless
jaundice (28%), palpable mass (18%), fatigue
o pcc presents as large mass because tumor does not
cause clinical symptoms in its early stages
• Clinical profile
o Lab data: Moderate anemia, leucocytosis, mild ! AST
& ALT, t carcinoembryonic antigen
(PERIPHERAL)
o Diagnosis: Biopsy; delayed enhancement can be
helpful as target for CT-guided biopsy
Demographics
• Age: 50-60 years, rarely occurring in younger than 40
Natural History & Prognosis
• Tumoral spread
o Local extension along duct
o Local infiltration of liver substance
o Metastases to regional lymph nodes
• Vascular or lymphatic invasion
• Perineural invasion
• Prognosis: Poor; < 20% resectable; 30% 5 year survival
Treatment
• Surgical resection remains primary treatment
• Palliative: Biliary catheter drainage, biliary stenting
• Adjuvant: Radiation & chemotherapy
• Liver transplantation (not considered appropriate in
most cases, high recurrence)
I DIAGNOSTIC CHECKLIST
Consider
• Hepatocellular-cholangiocarcinoma should be
considered when a hepatic tumor has features of both
• Delayed tumoral contrast-enhancement is typical
feature of PCC & may aid in detection &
characterization, however, delayed images must be
interpreted in conjunction with clinical information,
as well as unenhanced & dynamic contrast-enhanced
images, as specificity of these findings is uncertain
Image Interpretation Pearls
• In suspected cholangiocarcinoma (e.g., history of PSC,
or mass with capsular retraction, biliary obstruction)
obtain delayed enhanced scans
I SELECTED REFERENCES
1. Ebied 0 et al: Hepatocellular-cholangiocarcinoma: helical
computed tomography findings in 30 patients. J Comput
Assist Tomogr. 27(2):117-24, 2003
2. Um JH: Cholangiocarcinoma: morphologic classification
according to growth pattern and imaging findings. AJRAm
J Roentgenol. 181(3):819-27, 2003
3. Maetani Y et al: MR imaging of intrahepatic
cholangiocarcinoma with pathologic correlation. AJRAm J
Roentgenol. 176(6):1499-507,2001
4. Lacomis JM et al: Cholangiocarcinoma: delayed CT
contrast enhancement patterns. Radiology. 203(1):98-104,
1997
5. Kim TK et al: Peripheral cholangiocarcinoma of the liver:
two-phase spiral CT findings. Radiology. 204(2):539-43,
1997
6. Soyer P et al: Imaging of intrahepatic cholangiocarcinoma:
1. Peripheral cholangiocarcinoma. AJRAm J Roentgenol.
165(6):1427-31, 1995
 CHOlANGIOCARCINOMA (PERIPHERAL)

I IMAGE GALLERY
1
131
Typical
(Left) Axial CECT shows a
heterogeneous infiltrative
mass causing intrahepatic
biliary obstruction. (Right)
Catheter cholangiogram
shows long segmental
stenosis of left main bile duct
with occlusion of multiple
side branches and dilatation
of peripheral biliary ducts
from intrahepatic
cholangiocarcinoma.

(Left) Axial T1 C+ MR shows

large but subtle
heterogeneous mass
(arrows). Capsular retraction
(open arrows). (Right) Axial
T2WI MR shows mild
hyperintensity within hepatic
mass. Dilated intrahepatic
ducts (arrows).

Typical
(Left) Axial T1 C+ GRE MR
shows large hepatic mass
with ring enhancing
components (arrows) along
with low intensity,
nonenhancing
(fibrotic/necrotic) areas.
(Right) Axial T2WI MR
scarcely shows right hepatic
lobe mass, although absence
of normal ducts and vessels
within mass is a clue.
 EPITHELIOID HEMANGIOENDOTHELIOMA
1
132

Cut section of explanted liver shows multifocal Axial CECT in venous phase shows multifocal confluent
confluent masses with extensive fibrous stroma, causing masses, "target" enhancement (arrow) and capsular
volume 1055 and capsular retraction in right lobe. retraction.

o Other primary malignant vascular tumors of liver

ITERMINOlOGY • Angiosarcoma (2% of all primary malignant liver
Abbreviations and Synonyms tumors)
• Epithelioid hemangioendothelioma (EHE); hepatic • Kaposi sarcoma: Metastatic vascular tumor in
epithelioid hemangioendothelioma AIDS and transplant patients
o All hepatic malignant vascular tumors share
Definitions • Histologic characteristics
• Primary malignant tumor of liver arising from vascular • Grow around & into vessels
elements of mesenchymal tissue • Multifocal
o Clinical course
• Between benign cavernous hemangiomas &
I IMAGING FINDINGS malignant angiosarcomas
• Variable & unpredictable
General Features • Identical to that of extrahepatic EHE
• Best diagnostic clue: Coalescent peripheral hepatic • Metastatic in 40% cases (spleen, mesentery, lymph
nodules with target appearance & capsular retraction nodes, lung, bone)
• Location
o Liver: Periphery (more than 75% of lesions) with CT Findings
extension to capsule • Spectrum of growth in lesions may be seen
o Locations other than liver o Nodular form: Multiple nodules (more common)
• Soft tissues, bone & lung • Multiple liver nodules coalesce to form large
• Lung: Diagnosed as "intravascular confluent masses
bronchioalveolar tumor" o Diffuse or extensive form (very rare)
• Size: Varies from small tumor nodules to large • Usually located at periphery with extension to capsule
confluent masses • Typical "capsular retraction" (due to tumor fibrosis &
• Key concepts ischemia) or flattening
o Rare primary malignant (low grade) vascular tumor • Occasionally calcification within tumor
of liver in adults • Compensatory hypertrophy
o Uninvolved liver (predominantly left lobe)

DDx: Hepatic Mass with Capsular Retraction

Cholangiocarcinoma Treated Metastases Confluent Fibrosis Hepatic Hemangioma

 EPITHELIOID HEMANGIOENDOTHELIOMA

Key Facts
1
Terminology Pathology 133
• Epithelioid hemangioendothelioma (EHE)i hepatic • Slowly progressing low-grade malignant vascular
epithelioid hemangioendothelioma tumor of liver
• Primary malignant tumor of liver arising from • Must not be confused with infantile
vascular elements of mesenchymal tissue hemangioendothelioma
• Exact etiology: Unknown
Imaging Findings
• Best diagnostic clue; Coalescent peripheral hepatic Clinical Issues
nodules with target appearance & capsular retraction • Abdominal pain, jaundice, hepatosplenomegaly
• Size: Varies from small tumor nodules to large • Gender: Females more than males
confluent masses
Diagnostic Checklist
Top Differential Diagnoses • Rule out other hepatic lesions that typically cause
• Cholangiocarcinoma (peripheral) "capsular retraction"
• Treated malignancy (HCC or metastases) • Differentiated from other lesions by tumor cells
• Focal confluent fibrosis staining positive for factor VIll-related antigen
• Hemangioma (especially in cirrhotic liver)

• May have metastatic lesions & ascites o Invasion or occlusion of intrahepatic portal &
• NECT hepatic veins
o Tumor nodules
• Foci of homogeneous decreased attenuation (due Imaging Recommendations
to myxoid stroma) compared to normal liver • Best imaging tool: Helical NE + CECT, or MR + CEMR
parenchyma • Protocol advice: Multiphasic CT or MR
o Conspicuity & extent of lesions
• Superior on NECT than CECT
• CECT I DIFFERENTIAL DIAGNOSIS
o "Target" like enhancement pattern of tumor
• Nonenhancing central part of tumor (myxoid & Cholangiocarcinoma (peripheral)
hyalinized stroma) • Heterogeneous mass with capsular retraction
• Enhancing (hyperemic) peripheral inner rim • Satellite lesions may be seen
(increased vascularity) • Often invades or obstructs vessels & bile ducts
• Nonenhancing peripheral outer rim or halo • Intrahepatic bile duct dilatation
(avascular rim)
Treated malignancy (HCC or metastases)
MR Findings • Capsular retraction
• TlWI • Heterogeneous enhancement pattern
o Hypointense centrally • History of ablation or chemotherapy for liver tumor
o Peripheral thin hypointense rim • Treated metastatic nodules may show
• T2WI o Cystic or necrotic changes
o Hyperintense centrally • Debris, mural nodularity
o Peripheral thin hypointense rim • Thick septa & wall enhancement
• Tl C+ Focal confluent fibrosis
o Target pattern; Three concentric layers of alternating
• Common in advanced cirrhosis
signal intensity (analogous to CECT)
• Central: Hypointense • NECT: Areas of lower attenuation than adjacent liver
• CECT: Isoattenuating or minimally
• Peripheral: Thick enhancing inner rim & thin
hypo- /hypera ttenua ting
nonenhancing outer rim
• MR: Hypointense on Tl WIi hyperintense on T2WI
Ultrasonographic Findings • TIC+: Isointense or delayed enhancement
• Real Time • Associated volume loss seen
o Tumor nodules show varied echogenicity pattern o Capsular retraction adjacent to lesion
• Predominantly: Hypoechoic o Segmental or lobar shrinkage
• Occasionally: Hyperechoic or iso-/hypoechoic • Shape & location: Usually wedge shaped lesions
lesions relative to liver radiating from porta affecting anterior & medial
• Hyper-/isoechoic lesions may have peripheral segments
hypoechoic rims
Hemangioma (especially in cirrhotic liver)
Angiographic Findings • Capsular retraction seen in large lesion with scari
• Conventional hyalinization
o Hypervascular, hypovascular or avascular lesions • Decrease in size over time as cirrhosis progresses
• Based on degree of sclerosis & hyalinization • Rest of liver shows cirrhotic changes
 EPITHELIOID HEMANGIOENDOTHELIOMA
1 I PATHOLOGY
134 General Features
• General path comments
o Slowly progressing low-grade malignant vascular
tumor of liver
• Histologically: Composed of epithelioid-appearing
endothelial cells
• Abundant matrix of myxoid & fibrous stroma
• Positive factor VIII-associated antigen staining
• Malignant cells infiltrate into hepatic sinusoids
• Compress surrounding hepatocytes
• Capsular retraction
• Invade hepatic & portal veins
• Infarction of tumor & central fibrosis
o Must not be confused with infantile
hemangioendothelioma
• Histologically: Benign primary vascular liver
tumor
• Seen in infants & young children
• Resolves spontaneously in many cases
• Etiology
o Exact etiology: Unknown
o Possibly associated with oral contraceptives or
exposure to vinyl chloride
• Epidemiology
o Rare vascular tumor of liver
• Exact incidence is not known
Gross Pathologic & Surgical Features
• Multiple solid nodules
o Tan, white, firm, varied size
o Coalesce more peripherally
• Tumor nodules with hyperemic rim
• Lesions close to capsule cause retraction
Microscopic Features
• Dendritic spindle-shaped or epithelioid cells
• Matrix: Myxoid or fibrous stroma
• Epithelioid cells
o Stain positive for factor VIII-related antigen
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Abdominal pain, jaundice, hepatosplenomegaly
o Occasionally asymptomatic
o Rarely hemoperitoneum & Budd-Chiari syndrome
• Due to hepatic vein invasion
• Clinical profile: Middle aged patient with history of
RUQ pain, hepatomegaly & tumor cells stained
positive for factor VIII-related antigen
• Lab data
o Liver enzymes mildly increased
o (){-Fetoprotein & CEA levels: Normal
• Diagnosis
o Tumor cells staining positive for factor VIII-related
antigen
Demographics
• Age: 25-58 yrs (average age 45 yrs)
• Gender: Females more than males
Natural History & Prognosis
• Complications
o Rupture & hemoperitoneum
o Budd-Chiari syndrome
o Liver failure
• Prognosis
o Most patients survive 5-10 years after diagnosis
o 20% die within first 2 years after diagnosis
o 20% survive for 5-28 years
• With or without treatment
Treatment
• Radical resection or liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other hepatic lesions that typically cause
"capsular retraction"
• Differentiated from other lesions by tumor cells
staining positive for factor VIII-related antigen
Image Interpretation Pearls
• Usually located at periphery with extension to capsule
• Typical "capsular retraction" of peripheral tumor (due
to fibrosis & ischemia)
• Target appearance on CECT or MR
I SELECTED REFERENCES
1. Uchimura K et al: Hepatic epithelioid
hemangioendothelioma. J Clin Gastroenterol. 32(5):431-4,
2001
2. Kehagias DT et al: Hepatic epithelioid
hemangioendothelioma: MR imaging findings.
Hepatogastroenterology. 47(36): 1711-3, 2000
3. Lauffer JM et al: Epithelioid hemangioendothelioma of the
liver. A rare hepatic tumor. Cancer. 78(11):2318-27, 1996
4. Buetow PC et al: Malignant vascular tumors of the liver:
radiologic-pathologic correlation. Radiographies.
14(1):153-66; quiz 167-8, 1994
5. Furuta K et al: Epithelioid hemangioendothelioma of the
liver diagnosed by liver biopsy under laparoscopy. Am J
Gastroenterol. 87(6):797-800, 1992
6. Miller WJ et al: Epithelioid hemangioendothelioma of the
liver: imaging findings with pathologic correlation. AJR
Am J Roentgenol. 159(1):53-7, 1992
7. Van Beers B et al: Epithelioid hemangioendothelioma of
the liver: MR and CT findings. J Comput Assist Tomogr.
16(3):420-4, 1992
8. Furui S et al: Hepatic epithelioid hemangioendothelioma:
report of five cases. Radiology. 171(1):63-8, 1989
9. Radin DR et al: Hepatic epithelioid
hemangioendothelioma. Radiology. 169(1):145-8, 1988
10. Scoazec JY et al: Epithelioid hemangioendothelioma of the
liver. Diagnostic features and role of liver transplantation.
Gastroenterology. 94(6):1447-53,1988
11. Marino IR et al: Treatment of hepatic epithelioid
hemangioendothelioma with liver transplantation. Cancer.
62(10):2079-84, 1988
 EPITHELIOID HEMANGIOENDOTHELIOMA

I IMAGE GALLERY 1
135
Typical
(Left) Cut section of
explanted liver shows
- ~. ~ . multifocal tumor nodule with
extensive fibrous stroma,
target appearance and
... '~:. ~:. t' ,
capsular retraction. (Right)
/J ._. ~ Axial CECT in venous phase
~
. ~
.
shows multifocal tumor

---.
f) .~'
nodules, some with target
A.:~ . .;
.. ~
; ~ .... appearance and capsular

, ..
retraction.

/ ,: . ,:•... ~
, 'F _

.. ;."

Typical
(Left) Axial T7WI MR shows
extensive confluent
hypointense tumor in
peripheral liver causing
volume loss. Compensatory
hypertrophy of uninvolved
liver, including caudate lobe.
(Right) Axial T2WI MR
shows heterogeneous,
hyperintense confluent liver
tumor.

Typical
(Left) Axial CECT in venous
phase shows classic target
appearance of EHE, with
hypodense center, inner rim
of hypervascular
enhancement, and outer rim
of avascular hypodensity.
(Right) Axial T2WI MR
shows target appearance of
EHE with central
hyperintensity, inner rim of
hypointensity and outer rim
of hyperintensity.
 BILIARY CYSTADENOCARCINOMA
1
136

Graphic shows lobulated complex cystic mass with Axial CECT shows complex cystic mass with lobulated
vascularized wall and septa. margins, enhancing wall and septa.

• Typically solitary tumor, usually multilocular, but

ITERMINOLOGY sometimes unilocular
Abbreviations and Synonyms • Tumor is well-encapsulated
• Bile duct cystadenocarcinoma, cystadenoma • Usually seen in middle-aged women
• May recur after excision
Definitions o Benign biliary cystadenoma
• Rare malignant or premalignant, unilocular or • Probably congenital in origin due to presence of
multilocular cystic tumor that may arise from IHBD aberrant bile ducts
within liver (common site) & very rarely from • May recur after excision
extrahepatic biliary tree or gallbladder • Malignant potential to develop into
cystadenocarcinoma even after years of stability
o Benign & malignant lesions together account for
I IMAGING FINDINGS only 5% of all intrahepatic lesions of bile duct origin
o " Microcystic" cystadenoma variant
General Features • Composed of multiple small cysts
• Best diagnostic clue: Complex multiloculated cystic • Glycogen rich cystadenoma
mass in liver with septations & mural calcifications • Typical papillary & mesenchymal stromal features
• Location are not seen
o Right lobe (55%), left lobe (29%), both lobes (16%) • Lined by a single layer of cuboidal epithelial cells
o Intrahepatic biliary ducts (IHBD): 83% • Resembles serous microcystic adenoma of
o Extrahepatic bile ducts: 13% pancreas in pathology & on imaging
o Gallbladder: 0.02%
• Size: Varies from 1.5-25 cm in diameter CT Findings
• Key concepts • NECT
o Biliary cystadenocarcinoma o Large, well-defined, homogeneous, hypodense,
• Malignant transformation of benign biliary water density mass
cystadenoma o Large, well-defined, heterogeneous mass (cystic &
hemorrhagic areas)
o Cystadenocarcinoma: Septations & nodularity

DDx: Complex Cystic Mass

Hemorrhagic Cyst Pyogenic Abscess Cystic Metastases

 BILIARY CYSTADENOCARCINOMA
Key Facts
Terminology
• Bile duct cystadenocarcinoma, cystadenoma
• Rare malignant or premalignant, unilocular or
multilocular cystic tumor that may arise from IHBD
within liver (common site) & very rarely from
extrahepatic biliary tree or gallbladder
Imaging Findings
• Best diagnostic clue: Complex multiloculated·cystic
mass in liver with septations & mural calcifications
• Size: Varies from 1.5-25 cm in diameter
Top Differential Diagnoses
• Hemorrhagic or infected hepatic cyst
• Hepatic pyogenic abscess
• Cystic metastases
• Hydatid cyst
o Cystadenoma: Septations without nodularity
o Fine mural or septal calcifications
o Biliary dilatation (due to pressure effect)
• CECT
o Multilocular tumor
• Nonenhancing cystic spaces (decreased
attenuation)
• Enhancement of internal septa, capsule & nodules
• Enhancement of mural & septal nodules
• Enhancement of papillary excrescences
• Fine mural or septal calcifications
• Less commonly, honeycomb or sponge
appearance (micro cystic variant)
• Mayor may not show metastases or adenopathy
o Unilocular tumor
• Large or small non enhancing cystic space
• Enhancement of outer capsule & papillary
excrescences
• Fine mural calcifications
MR Findings
• TlWI
o Variable signal intensity locules depending on
content of cystic fluid
o Increased signal intensity (mucoid fluid)
o Decreased signal intensity (serous fluid)
o Septal or mural calcifications: Hypointense
• T2WI
o Decreased signal intensity (mucoid flUid)
o Increased signal intensity (serous fluid)
o Septations are well-delineated
o Septal or mural calcifications: Hypointense
• Tl C+: Enhancement of capsule & septa
Ultrasonographic Findings
• Real Time
o Large, well-defined, multiloculated, anechoic mass
o Highly echogenic septations
o Tumor nodules or papillary growths
o Mural or septal calcifications or fluid levels
o Complex fluid: Areas of anechoic + internal echoes
(cystic + hemorrhagic)
1
Pathology 137
• Varying degrees of mural & septal nodularity or
thickening seen
• Papillary excrescences project into cystic spaces
• Cystadenocarcinomas: Thick, coarse, mural & septal
calcifications
• Cystadenomas: Fine septal calcifications
• Malignant transformation of benign biliary
cystadenoma by invasion of capsule
Diagnostic Checklist
• Rule out other "complex cystic" masses of liver
• Large well-defined homogeneous or heterogeneous
"complex cystic" mass with septations & nodularity
• May mimic hemorrhagic or infected hepatic cyst
Angiographic Findings
• Conyentional
o Avascular mass with small clusters of peripheral
abnormal vessels
o Stretching & displacement of vessels
Imaging Recommendations
• NE + CECT or MR + CEMR
I DIFFERENTIAL DIAGNOSIS
Hemorrhagic or infected hepatic cyst
• Complex heterogeneous cystic mass
• Multiple thick or thin septations
• May show mural nodularity & fluid-level
• Calcification mayor may not be seen
• No enhancement on CECT
Hepatic pyogenic abscess
• Simple pyogenic abscess
o Well-defined, round, hypodense mass (0-45 HU)
• "Cluster" sign: Small abscesses aggregate, sometimes
coalesce into a single big septated cavity
• Complex pyogenic abscess: "Target" lesion
o Hypodense rim
o Isodense periphery & decreased HU in center
• Often associated with diaphragmatic elevation,
atelectasis & right side pleural effusion
Cystic metastases
• Usually from ovarian cystadenocarcinoma &
metastatic sarcoma
• Show debris & mural nodularity
• May have thick septa & wall enhancement
Hydatid cyst
• Large well-defined cystic liver mass
• Often has numerous peripheral daughter cysts or
scolices of different density or intensity
• May show curvilinear or ring-like pericyst calcification
• Occasionally dilated intrahepatic bile ducts
o Due to pressure effect or rupture into ducts
 BILIARY CYSTADENOCARCINOMA
1 I PATHOLOGY
138 General Features
• General path comments
o Varying degrees of mural & septal nodularity or
thickening seen
o Papillary excrescences project into cystic spaces
o Cystic cavities are filled with mucinous or serous or
necrotic or blood content
o Cystadenocarcinomas: Thick, coarse, mural & septal
calcifications
o Cystadenomas: Fine septal calcifications
• Etiology
o Malignant transformation of benign biliary
cystadenoma by invasion of capsule
o Biliary cystadenoma
• Probably derived from ectopic nests of primitive
biliary tissue
• Epidemiology
o Very rare malignant biliary tumor
o Incidence: 5% of all intrahepatic cystic masses of
biliary origin
Gross Pathologic & Surgical Features
• Multiloculated cystic tumor with well-defined thick
capsule containing
o Serous, mucinous, bilious, hemorrhagic or mixed
fluid
• Surface is shiny, smooth or bosselated
• Polypoid excrescences & septations may be seen
Microscopic Features
• Single layer of cuboidal or tall columnar biliary type
epithelium with papillary projections
• Malignant epithelial cells line the cysts
• Subepithelial stroma resembles that of ovary
• Usually mucinous, but serous type is also seen
• Goblet cells, Paneth cells & argyrophilic endocrine
cells may be seen
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Abdominal pain, obstructive jaundice, nausea,
vomiting
o Abdominal swelling with palpable mass (90%)
• Diagnosis: Fine needle aspiration & cytology
Demographics
• Age: Peak incidence in 5th decade
• Gender
o Usually occur in middle aged women
o M:F = 1:4
• Ethnicity: Predominantly seen in Caucasians
Natural History & Prognosis
• Complications
o Rupture into peritoneum or retroperitoneum
o Recurrence common
• Prognosis
o Tumors with ovarian stroma found in women have
an indolent course & good prognosis
o Tumors without ovarian stroma found in both sexes
have an aggressive clinical course & poor prognosis
Treatment
• Surgical resection
I DIAGNOSTIC CHECKLIST
Consider
• Rule Qut other "complex cystic" masses of liver
Image Interpretation Pearls
• Large well-defined homogeneous or heterogeneous
"complex cystic" mass with septations & nodularity
• May mimic hemorrhagic or infected hepatic cyst
I SELECTED REFERENCES
1. Levy AD et al: Benign tumors and tumorlike lesions of the
gallbladder and extrahepatic bile ducts:
Radiologic-pathologic correlation. RadioGraphies. 22:
387-413,2002
2. Mortele KF et al: Cystic focal liver lesions in the adult:
Differential CT and MR imaging features. Radiographies.
21: 895-910, 2001
3. Hwang IK et al: Huge biliary cystadenoma mimicking
cholecystic lymphangioma in subhepatic space. ] Comput
Assist Tomogr. 24(4):652-4, 2000
4. Gabata T et al: Biliary cystadenoma with mesenchymal
stroma of the liver: correlation between unusual MR
appearance and pathologic findings. ] Magn Reson
Imaging. 8(2):503-4, 1998
5. Singh Y et al: Multiloculated cystic liver lesions:
Radiologic-pathologic differential diagnosis.
RadioGraphies. 17: 219-24, 1997
6. Buetow PC et al: Biliary cystadenoma and
cystadenocarcinoma: clinical- imaging-pathologic
correlations with emphasis on the importance of ovarian
stroma. Radiology. 196(3):805-10, 1995
Devaney K et al: Hepatobiliary cystadenoma and
7.
cystadenocarcinoma. A light microscopic and
immunohistochemical study of 70 patients. Am] Surg
Pathol. 18(11):1078-91, 1994
8. Wang Y] et al: Primary biliary cystic tumors of the liver.
Am] Gastroenterol. 88(4):599-603,1993
9. Agildere AM et al: Biliary cystadenoma and
cystadenocarcinoma. A]R Am] Roentgenol. 156(5):1113,
1991
10. Korobkin M et al: Biliary cystadenoma and
cystadenocarcinoma: CT and sonographic findings. A]R
Am] Roentgenol. 153(3):507-11, 1989
11. Choi BI et al: Biliary cystadenoma and
cystadenocarcinoma: CT and sonographic findings.
Radiology. 171(1):57-61, 1989
12. Genkins SM et al: Biliary cystadenoma with mesenchymal
stroma: CT and angiographic appearance. ] Comput Assist
Tomogr. 12(3):527-9, 1988
13. Kokubo T et al: Mucin-hypersecreting intrahepatic biliary
neoplasms. Radiology. 168(3):609-14, 1988
14. Forrest ME et al: Biliary cystadenomas:
sonographic-angiographic-pathologic correlations. A]R Am
] Roentgenol. 135(4):723-7, 1980
 BILIARY CYSTADENOCARCINOMA

I IMAGE GALLERY 1
139

(Left) Axial T1WI MR shows

non-communicating
hypointense cystic spaces
within liver mass.
Heterogeneous hypointensity
is due to fluid and mucin
content. (Right) Axial T2WI
MR shows multiloevlated
cystic liver mass.

Typical
(Left) Axial CECT in a young
female who had prior left
hepatectomy shows
recurrent multiloculated
mass with large cystic
spaces, visible wall and
septa. Biliary
Cystadenocarcinoma. (Right)
Transverse sonogram shows
anechoic cystic spaces
separated by thin and thick
septa.

Variant
(Left) Cut surface of resected
mass shows innumerable
small cystic spaces with a
honeycomb or sponge
appearance. "Microcystic"
variant of biliary
cystadenocarcinoma. (Right)
Axial CECT of microcystic
cystadenocarcinoma shows'
innumerable tiny cystic
spaces in honeycomb or
sponge appearance.
 ANGIOSARCOMA, LIVER
1
140

Axial CECT shows mass in lateral segment with central Axial CECT shows multifocal tumors, some with nodular
and peripheral, progressive enhancement, isodense with central or peripheral enhancement.
vessels; simulating hemangioma.

o Multifocal or multinodular (more common: 71%),

ITERMINOlOGY
Abbreviations and Synonyms
• Angiosarcoma (AGS)
• Hemangioendothelial sarcoma, hemangiosarcoma,
Kupffer-cell sarcoma
Definitions
• Angiosarcoma (AGS) is a malignant spindle cell tumor
of endothelial cell derivation that can form poorly
organized vessels, grow along preformed vascular
channels, be arranged in sinusoidal or large cavernous
spaces or form solid nodules or masses
I IMAGING FINDINGS
General Features
• Best diagnostic clue: MR imaging demonstrates
hemorrhagic, heterogeneous, & hypervascular nature
• Location: Skin, soft tissue, breast, liver & spleen
• Size
o Variable; as lesion can be micro nodular (few mm) or
massive (several centimeters in diameter) or diffuse
• Vascular channels within: Variable size from
capillary to cavernous
• Key concepts
or large solitary mass, or as diffusely infiltrating
lesion
Radiographic Findings
• Radiography: If Thorotrast exposure: Localized areas of
t (metallic) density in patchy or circumferential
pattern
CT Findings
• NECT
o Single or multiple hypodense masses
• Hyperdense areas of fresh hemorrhage
o Reticular pattern of deposition of Thorotrast in liver,
spleen, mesenteric, celiac lymph nodes
o Circumferential displacement of Thorotrast in
periphery of a nodule is characteristic
• CECT
o Heterogeneous pattern of enhancement (typical)
likely represents heterogeneity of microscopic
vascular patterns within each tumor
o Peripheral nodular enhancement with centripetal
progression in a dominant mass (less typical)
o Usually hypo intense with nodular enhancement
• Bizarre or ring-enhancement possible
o Vascular channels show persistence of contrast
o Portal & delayed phase demonstrate progressive
enhancement over time

DDx: Heterogeneous Hypervascular Liver Mass(es)

Hemangioma Metastases HCC Hepatic Adenomas

 ANGIOSARCOMA,
Key Facts
Terminology
• Angiosarcoma (AGS)is a malignant spindle cell
tumor of endothelial cell derivation that can form
poorly organized vessels, grow along preformed
vascular channels, be arranged in sinusoidal or large
cavernous spaces or form solid nodules or masses
Imaging Findings
• Heterogeneous pattern of enhancement (typical)
likely represents heterogeneity of microscopic
vascular patterns within each tumor
• Bizarre or ring-enhancement possible
• Portal & delayed phase demonstrate progressive
enhancement over time
Top Differential Diagnoses
• Hemangioma
• CTA: ± Portal vein thrombus by malignant endothelial
cells/intrahepatic arterial encasement
MR Findings
• T1WI
o Large mass, multiple nodules of low signal intensity
o Areas of hemorrhage: Irregular hyperintense regions
o Thorotrast does not produce recognizable MR signal,
may be easily missed
o Micronodular diffusely infiltrative pattern (less
common), seen as diffuse signal heterogeneity
throughout liver
• T2WI
o Heterogeneous or compartmentalized appearance:
Predominantly high signal on T2 with central
septum-like or rounded areas of low signal
• Areas of low signal intensity may reflect
hemosiderin, fibrosis ,or fresh hemorrhage
• Areas of high intensity represent hemorrhage or
necrosis
• Fluid-fluid levels on T2WI is another finding
which reflects hemorrhagic nature of AGS
• T1 C+
o Dynamic enhancement of dominant mass:
Heterogeneous enhancement on early phase images
o Progressive enhancement on delayed images
o May show peripheral nodular enhancement which
progresses centripetally
o Center of lesion remaining unenhanced may
represent fibrous tissue or deoxyhemoglobin
o Areas of abundant, freely anastomosing vascular
channels enhance quickly & contrast persists while
dilated cavernous vascular spaces may show slowly
progressive enhancement
Ultrasonographic Findings
• Hyperechoic masses or nodules
• Heterogeneous echotexture; due to hemorrhage of
various ages
Angiographic Findings
• Conventional
o Moderately hypervascular tumor, diffuse puddling
of contrast material that persists into venous phase
LIVER
1
• Hepatic metastases 141
• Hepatocellular carcinoma (HCC)
• Hepatic adenoma(s)
• Focal nodular hyperplasia (atypical)
Clinical Issues
• Rapid & early metastatic spread: Spleen (16%), lung,
bone marrow, porta hepatis nodes, peritoneum
• Tend to be multifocal, to recur, & to metastasize
• Complication: Rupture & acute hemoperitoneum
Diagnostic Checklist
• Multiphasic contrast-enhanced helical CT & dynamic
MR showing progressive enhancement of multiple
heterogeneous liver lesions
o Fed by large peripheral vessels, & centripetal flow
o If rupture: Demonstrate bleeding/hemoperitoneum
Nuclear Medicine Findings
• Tagged RBC: Early as well as late persistent uptake
• Gallium scan: Increased gallium uptake
Imaging Recommendations
• Best imaging tool: Triphasic helical CT & dynamic MR
• Protocol advice: TI & T2WI, using fast spin-echo with
dynamic TI weighted three-dimensional fast
spoiled-gradient echo technique
I DIFFERENTIAL DIAGNOSIS
Hemangioma
• Centripetal nodular enhancement that approximates
density of contrast-opacified blood in aorta or hepatic
artery during all phases & unenhanced imaging
• Inhomogeneity on T2WI; seen in AGS, may not be
seen in typical hemangiomas (homogeneously
hyperintense)
• Hemangiomas are more often solitary, & when
multiple, are rarely as numerous as seen with AGS
Hepatic metastases
• Multiple; scattered randomly throughout liver
• Hypervascular lesions: Hyperdense in lak arterial
phase; may have internal necrosis without uniform
hyperdense enhancement
Hepatocellular carcinoma (HCC)
• Heterogeneous hypervascular mass(es); vascular +
nodal invasion; necrosis + hemorrhage
Hepatic adenoma(s)
• Blush of homogeneous enhancement in arterial phase
& nearly isointense in later phases of dynamic scans
• Heterogeneous due to hemorrhage, necrosis or fat
Focal nodular hyperplasia (atypical)
• Immediate, intense, hyperdensity on arterial phase,
followed rapidly by isodensity on portal phase
 ANGIOSARCOMA,
1 • May show heterogeneous enhancement, but not
isodense to vessels on multiphasic CT or MR
142
!PATHOLOGY
General Features
• General path comments
o Growth pattern: Multi-nodular, massive (large
dominant mass), mixed (multi-nodular & massive)
& diffuse infiltrative micronodular
o Compartmentalization within tumor
• Etiology
o Environmental carcinogens:
• Polyvinyl chloride (plastic resin), 45-fold i risk of
AGS, latent period: 4-28 years
• Thorotrast (radio contrast used from 1928 to
1950), 38.3% of Thorotrast-related hepatic
malignancies are AGS, latent period: 15-37 years
• Arsenicals (in some pesticides)
o Drugs: Cyclophosphamide, anabolic steroids
o Post radiation (median latency: 74 months)
o Most of AGS occur either in absence of known risk
factors or with cirrhosis, cause is not apparent
• Epidemiology
o Prevalence varies from 0.14 to 0.25 per million
o Most common mesenchymal tumor of liver
o Up to 2% of all primary malignant liver tumors
o 30 times less common than HCC
o Approximately 10 to 20 new cases are diagnosed
every year in United States
• Associated abnormalities
o Hemochromatosis
o Von Recklinghausen disease
Gross Pathologic & Surgical Features
• Begin as small, well-demarcated red nodules evolving
into fleshy, grey-white, soft tissue masses
• Dominant large mass without capsule & containing
large cystic areas filled with bloody debris & necrosis
Microscopic Features
• Malignant endothelial cells lining vascular channels
• Vascular channels with varied patterns
• Fibrosis & hemosiderin in solid portions of tumor
• Thorotrast particles can be found within malignant
endothelial cells in case of Thorotrast induced AGS
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Weakness, weight loss, abdominal pain,
hepatomegaly, ascites, jaundice
• Anemia (62%),thrombocytopenia (54%),
disseminated intravascular coagulation (31%), &
microangiopathic hemolytic anemia (23%)
• Clinical profile
o Rapid & early metastatic spread: Spleen (16%), lung,
bone marrow, porta hepatis nodes, peritoneum
• Portal vein invasion/ hemorrhagic ascites
o Lab data: Elevation of serum neuron-specific
enolase; no elevation of (){-fetoprotein
LIVER
Demographics
• Age: Commonly 60-70 years of age, can occur in
younger patients & in childhood
• Gender: Strong male predominance, M:F = 4:1
Natural History & Prognosis
• Tend to be multifocal, to recur, & to metastasize
• Complication: Rupture & acute hemoperitoneum
• Prognosis is poor
• Most patients die within one year of diagnosis
• Median survival time is 6 months
Treatment
• Surgical resection: Primary modality when tumor
confined to one lobe of liver without metastases
o Outcome is poor, 5 year survival rate is 37%
• Systemic or hepatic arterial chemotherapy:
Antiangiogenic therapy
• Liver transplantation abandoned as treatment because
recurrence rate 64%, & low survival rate
I DIAGNOSTIC CHECKLIST
Consider
• Pleomorphic histopathology of AGS correlates with
various patterns of tumor enhancement
• Multiphasic helical CT, faster scanning techniques &
temporal assessment of lesion enhancement relative to
blood vessels (aorta), helps differentiate AGS from
hemangioma
• Environmental exposure is now rare, detection of
Thorotrast accumulation on CT will become
increasingly rare, with few additional
Thorotrast-induced tumors detected
Image Interpretation Pearls
• Multiphasic contrast-enhanced helical CT & dynamic
MR showing progressive enhancement of multiple
heterogeneous liver lesions
I SELECTED REFERENCES
1. Kitami M et al: Diffuse hepatic angiosarcoma with a portal
venous supply mimicking hemangiomatosis. ] Com put
Assist Tomogr. 27(4):626-9, 2003
2. Yu R et al: Hepatic angiosarcoma: CT findings. Chin Med]
(Engl). 116(2):318-20,2003
3. Koyama T et al: Primary hepatic angiosarcoma: findings at
CT and MR imaging. Radiology. 222(3):667-73, 2002
4. Peterson MS et al: Hepatic angiosarcoma: findings on
multiphasic contrast-enhanced helical CT do not mimic
hepatic hemangioma. A]RAm] Roentgenol. 175(1):165-70,
2000
5. White PG et al: The computed tomographic appearances of
angiosarcoma of the liver. Clin Radial. 48(5):321-5, 1993
 ANGIOSARCOMA, LIVER

I IMAGE GALLERY
1
143
Typical
(Left) Axial CECT in venous
phase shows multifocal
tumors, many with central,
progressive enhancing
channels nearly isodense to
vessels. (Right) Axial CECT
shows multifocalliver and
splenic tumors, many of
which had similar nodular
enhancement patterns.

Typical
(Left) Axial CECT shows
heterogeneous mass that
encases the right hepatic
artery and the anterior
branch of the right portal
vein. (Right) Axial CECT
shows heterogeneous masses
with foci of hypervascularity
within the tumors, and
markedly hypervascular
lymphadenopathy in the
porta hepatis.

Variant
(Left) Axial T7 C+ MR in
arterial phase shows diffuse
micronodular hypervascular
liver tumor (arrows) in a
patient with underlying
cirrhosis. (Right) Axial T2WI
MR shows diffuse
micronodular hyperintense
tumor (arrow) in underlying
cirrhotic liver.
 HEPATIC METASTASES AND LYMPHOMA
1
144

Axial Tl C+ MR shows large heterogeneous Axial T2WI MR shows heterogeneous intensity within
hypointense mass. Metastatic colon cancer. the metastatic mass (colon primary) and hyperintensity
of surrounding parenchyma, perhaps due to edema
and/or compression of liver.

• Transplant recipients & AIDS patients (high risk)

ITERMINOLOGY o Types of lymphoma
Abbreviations and Synonyms • Hodgkin disease (HD)
• Hodgkin disease (HD), non-Hodgkin lymphoma (NHL) • Non-Hodgkin lymphoma (NHL)
o Liver metastases
Definitions • Most common malignant tumor of liver
• Lymphoma: Neoplasm of lymphoid tissues • Compared to primary malignant tumors: 18:1
• Metastases: Malignant spread of neoplasm to hepatic • Liver is second only to regional lymph nodes as a
parenchyma site of metastatic disease
• Autopsy studies reveal 55% of oncology patients
have liver metastases
I IMAGING FINDINGS • May be hypovascular or hypervascular

General Features CT Findings

• Best diagnostic clue • NECT
o Lymphoma: Lobulated low density masses o May be normal
o Metastases: Multiple hypo- or hyperdense lesions o Primary lymphoma
scattered throughout liver in random distribution • Isodense or hypodense to liver
• Location o Secondary lymphoma
o Both lobes of liver • Multiple well-defined, large, homogeneous
o Lymphoma (HD & NHL) arises in periportal areas lobulated low density masses
due to high content of lymphatic tissue • Diffuse infiltration: Indistinguishable from normal
• Size: Variable; from few millimeters to centimeters liver or steatosis
• Key concepts o Metastases
o Hepatic lymphoma • Isodense, hypodense or hyperdense
• Primary (rare) • Calcified: Mucinous adenocarcinoma (colon),
• Secondary (more common): Seen in more than treated metastases (breast), malignant teratoma
50% of patients with HD or NHL o Cystic metastases (less than 20 HU)
• Fluid levels, debris, mural nodules

DDx: Multiple Heterogeneous Hypodense Focal Masses

.~
, .. .,-..-r.
,

·~·DQI
,
..
.'

~
..,."

-.
,
"
': ;:
'~.' ..... '

'.;, .
,<

1: ~

"Hepatic Cysts
V Pyogenic Abscesses Hemangiomas Cholangiocarcinoma
 HEPATIC METASTASES AND LYMPHOMA
Key Facts
Imaging Findings
• Lymphoma: Lobulated low density masses
• Metastases: Multiple hypo- or hyperdense lesions
scattered throughout liver in random distribution
• Calcified: Mucinous adenocarcinoma (colon), treated
metastases (breast), malignant teratoma
• "Light bulb" sign: Very high signal intensity (e.g.,
cystic & neuroendocrine metastases)
Top Differential Diagnoses
• Multiple hepatic cysts
• Multiple liver abscesses
• Multiple hemangiomas
• Multifocal fatty infiltration (steatosis)
• Multifocal HCC or cholangiocarcinoma (CC)
o Cystic metastases (less than 20 HU)
• Fluid levels, debris, mural nodules
• Thickened walls or septations may be seen
• Usually cystadenocarcinoma or sarcoma
(pancreatic, Gl or ovarian primaries)
• CECT
o Lymphoma
• Homogeneous low density discrete masses
• More common in immunosuppressed patients
• Examples: AIDS & organ transplant recipients
o Hypovascular metastases
• Low attenuation center with peripheral rim
enhancement (e.g., epithelial metastases)
• Indicates vascularized viable tumor in periphery &
hypovascular or necrotic center
• Rim enhancement may also be due to compressed
normal parenchyma
o Hypervascular metastases
• Hyperdense in late arterial phase images
• May have internal necrosis without uniform
hyperdense enhancement
• Hypo- or isodense on NECT & portal venous
phase images
• Examples: Islet cell, carcinoid, thyroid, renal
carcinomas & pheochromocytoma
MR Findings
• TIWI
o Lymphoma: Hypointense lesions
o Metastases: Multiple low signal lesions
• T2WI
o Lymphoma: Hyperintense
o Metastases
• Moderate to high signal
• "Light bulb" sign: Very high signal intensity (e.g.,
cystic & neuroendocrine metastases)
• Mimic cysts or hemangiomas due to high signal
"light bulb" appearance
• Tl C+
o Hypovascular metastases
• Similar with gadolinium enhancement to CECT
• Low signal in center and peripheral
rim-enhancement
1
Pathology 145
• Metastases: Depends on underlying primary tumor
• Epidemiology: Over 50,000 deaths per year in US
alone due to metastatic spread of colorectal cancer
• Usually AIDS in lymphoma
Clinical Issues
• Asymptomatic, RUQ pain, tender hepatomegaly
• Weight loss, jaundice or ascites
• 20-40% have good 5 year survival rate if resectable
Diagnostic Checklist
• Rule out other multiple liver lesions like hepatic
cysts, abscesses, hemangiomas which can mimic
metastases
• Epithelial metastases: Vascular rim-enhancement
o Hypervascular metastases
• Hyperintense enhancement on arterial phase
• Superparamagnetic iron oxide (SPIO)
o Metastases: Bright signal on T2WI
• Free of reticuloendothelial system (RES)
o Rest of normal liver: Decreased signal
• Due to SPIO particles phagocytized by RESof liver
Ultrasonographic Findings
• Real Time
o Hepatic lymphoma
• Multiple well-defined hypoechoic lesions
• Diffuse form: May detect innumerable
subcentimeter hypoechoic foci or
indistinguishable from normal liver
o Hypoechoic metastases
• Usually from hypovascular tumors
o Hyperechoic metastases
• GI tract malignancy
• Vascular metastases from islet cell tumors,
carcinoid, choriocarcinoma & renal cell carcinoma
o "Bull's eye" or "target" metastatic lesions
• Alternating layers of hyper- & hypoechoic tissue
• Solid mass with hypoechoic rim or halo
• Usually from aggressive primary tumors
• Example: Bronchogenic carcinoma
o Cystic metastases
• Cystadenocarcinoma of pancreas & ovary
• Treated metastases, sarcomas & squamous cell
carcinoma
o Calcified metastases
• Markedly echo genic with acoustic shadowing
• Example: Mucinous adenocarcinoma of colon,
treated metastases (e.g., breast) malignant
teratoma
Nuclear Medicine Findings
• PET
o Metastases
• Multiple increased metabolic foci
• Fluorodeoxyglucose (18-FDG) avid
o Hepatic lymphoma
• Good concordance with CT & MR
 HEPATIC METASTASES AND LYMPHOMA
1 Imaging Recommendations
146 • NE + CECT or MR + CEMR
• MR with liver specific contrast agents (e.g., SPIO or
mangafodipir) if resection or ablation of metastases is
considered
I DIFFERENTIAL DIAGNOSIS
Multiple hepatic cysts
• No peripheral rim or central enhancement
• May have increased density or intensity due to prior
bleed or infection (e.g., polycystic liver)
• No mural nodules or debris
Multiple liver abscesses
• "Cluster sign" on CT for pyogenic abscesses
• Often with atelectasis & right pleural effusion
• Typical systemic signs of infection
Multiple hemangiomas
• Typical peripheral nodular discontinuous
enhancement on CECT or CEMR
• Isodense with blood vessels on NECT & CECT
• Markedly hyperintense on T2WI
• Uniformly hyperechoic on US
Multifocal fatty infiltration (steatosis)
• Focal signal dropout on opposed-phase T1 GRE MR
• Vessels course through "lesions" without disruption
Multifocal HCC or cholangiocarcinoma (CC)
• HCC: Cirrhotic liver, vascular invasion
• CC: Capsular retraction, delayed enhancement
I PATHOLOGY
General Features
• General path comments
o Lymphoma
• Early disease: Miliary lesions
• Late disease: Multiple nodules
o Metastases: Depends on underlying primary tumor
• Etiology
o Hypovascular liver metastases
• Lung, GI tract, pancreas & most breast cancers
• Lymphoma, bladder & uterine malignancy
o Hypervascular liver metastases
• Endocrine tumors, renal & thyroid cancers
• Some breast cancers, sarcomas & melanoma
• Epidemiology: Over 50,000 deaths per year in US
alone due to metastatic spread of colorectal cancer
• Associated abnormalities
o Primary malignant tumor for metastases
o Usually AIDS in lymphoma
Gross Pathologic & Surgical Features
• Lymphoma: Miliary, nodular or diffuse form
• Metastases
o Vary in size, consistency & vascularity
o Nodular, infiltrative, expansile or miliary
Microscopic Features
• Hodgkin disease (HD)
o Typical Reed-Sternberg cells
• Non-Hodgkin lymphoma (NHL)
o Follicular small cleaved-cells (most common)
o Small noncleaved cells (Burkitt lymphoma - rare)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, RUQ pain, tender hepatomegaly
o Weight loss, jaundice or ascites
• Lab data: Elevated LFTsi normal in 25-50% of patients
• Diagnosis: Imaging, core biopsy and FNA
Demographics
• Age: Usually middle & older age group
• Gender: Depends on underlying primary tumor
Natural History & Prognosis
• Depends on primary tumor site
• 20-40% have good 5 year survival rate if resectable
• In patients with metastatic colon cancer
o 3 year survival rate
• In 21 % of patients with solitary lesions
• In 6% with multiple lesions in one lobe
• In 4% with widespread disease
Treatment
• Resection or ablation for colorectalliver metastases
• Chemoembolization: Carcinoid/endocrine metastases
• Chemotherapy for all others
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other multiple liver lesions like hepatic cysts,
abscesses, hemangiomas which can mimic metastases
Image Interpretation Pearls
• Liver metastases: Hypovascular or hypervascular
• Epithelial metastases: Vascular rim-enhancement
• "Light bulb" sign on T2WI: Cystic & neuroendocrine
metastases
I SELECTED REFERENCES
1. Valls C et al: Hepatic metastases from colorectal cancer:
preoperative detection and assessment of resectability with
helical CT. Radiology. 218(1):55-60, 2001
2. Heimberger T et al: new contrast agents for imaging the
liver. Magn Reson Imaging Clin N Am. 9(4):745-66, 2001
3. Blake SP et al: Liver metastases from melanoma: detection
with multiphasic contrast-enhanced CT. Radiology.
213(1):92-6, 1999
4. Nazarian LN et al: Size of colorectalliver metastases at
abdominal CT: comparison of precontrast and postcontrast
studies. Radiology. 213(3):825-30, 1999
5. Paulson EKet al: Carcinoid metastases to the liver: role of
triple-phase helical CT. Radiology. 206(1):143-50, 1998
 HEPATIC METASTASES AND LYMPHOMA
I IMAGE GALLERY 1
147
Typical
(Left) Axial CECT shows
multiple hypodense masses;
some almost isodense to liver
in a patient with AIDS and
diffuse lymphoma. (Right)
Axial CECT shows necrotic
metastasis with shaggy
enhancing wall causing
extrinsic compression and
obstruction of left lobe bile
ducts. Metastatic colon
carcinoma.

Typical
(Left) Axial CECT in arterial
phase shows hypervascular
mass (arrow) adjacent to
IVC. Metastatic carcinoid
tumor. (Right) Axial CECT in
venous parenchymal phase.
Mass adjacent to IVC is
nearly isodense to liver and
difficult to recognize.

(Left) Axial T2WI MR shows

bright signal ("light bulb") in
center of two cystic/necrotic
metastases from sarcoma of
gastrointestinal tract. (Right)
Axial NECT shows several
focal masses with
amorphous calcification,
characteristic of mucinous
adenocarcinoma (colon
primary).
 RADIATION HEPATITIS
1
148

Axial NECT following resection of primary sarcoma of Axial NECT several months after surgical resection and
IVC and prior to radiation therapy. Note synthetic graft radiation therapy for sarcoma of IVC. Band of low
(arrow). attenuation and volume loss in left lobe corresponds to
radiation port.

o If hepatic congestion is severe, patchy congestion

ITERMINOlOGY
Abbreviations and Synonyms
• Radiation-induced liver disease (RILD)
Definitions
• Radiation-induced liver disease (RILD),often called
radiation hepatitis, is a syndrome characterized by
development of anicteric ascites approximately 2
weeks to 4 months after hepatic irradiation
• RILDis a form of veno-occlusive disease due to fibrous
obliteration of terminal hepatic venules leading to
postsinusoidal obstruction
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Sharp line of demarcation
between normal & abnormal parenchyma corresponds
to radiation port or vascular distribution of yttrium -
90 microspheres
CT Findings
• NECT
o CT performed several months after radiation therapy
shows sharply defined band of low attenuation
corresponding to treatment port
• Due to localized edema or hepatic congestion
simulating tumor nodules may be seen
o In patients with fatty infiltration of liver, irradiated
area may appear as a region of increased attenuation
• May be due to loss of fat in irradiated hepatocytes
or regional edema, with water content
demonstrating higher attenuation than fatty liver
o Over a period of weeks, the initially sharp borders of
irradiated zone become more irregular & indistinct
(peripheral parenchyma regenerates)
o Eventually, irradiated area may be atrophic
• CECT
o Enhancement pattern of irradiated liver may vary
depending on pre-existing hepatic pathology
o Intense enhancement of irradiated parenchyma
compared with normal; attributed to 1 arterial flow
secondary to reduced portal flow; seen in patients
treated for hepatocellular carcinoma (HCC)
o Region of radiation damage is hypodense on portal
venous phase & becomes hyperdense with marked
prolonged enhancement on delayed phase
• Due to ~vascular perfusion & ~ hepatic venous
drainage & subsequent stasis of contrast medium
o Narrowing & irregularity of hepatic vessels
(sinusoidal congestion & perisinusoidal edema)
MR Findings
• T1WI: GeographiC areas of low signal on T1WI

DDx: Segmental or Geographic Hypodense liver

r.
Focal Steatosis Focal Steatosis Hepatic Infarction Hepatic Infarction
 RADIATION HEPATITIS

Key Facts
1
Terminology Top Differential Diagnoses 149
• Radiation-induced liver disease (RILD) • Focal steatosis
• Hepatic infarction
Imaging Findings
• CT performed several months after radiation therapy Pathology
shows sharply defined band of low attenuation • Veno-occlusive disease
corresponding to treatment port
• Best imaging tool: NECT and CECT; or MR T1WI GRE Clinical Issues
with in- and out-of-phase • Usually presents 2-16 weeks after treatment

Imaging Recommendations Natural History & Prognosis

• Best imaging todl: NECT and CECT; or MR TlWI GRE • Complete clinical recovery is typically seen within 60
with in- and out-of-phase days, but there may be permanent hepatocyte loss, fat
deposition, fibrosis & obliteration of central veins
• Chronic changes: Atrophy of involved segments &
I DIFFERENTIAL DIAGNOSIS rarely cirrhosis

Focal steatosis
• May be geographic, band or wedge-shaped
• Preservation of enhancing vessels within "lesion"
• Suppression of signal on opposed-phase GRE MR
Hepatic infarction
• Segmental or geographic hypodense area with straight
margins with absent or heterogeneous enhancement
I DIAGNOSTIC CHECKLIST
Consider
• Variability in liver damage influenced by factors:
Irradiated liver volume, radiation fraction size,
cytotoxic agents & nutritional status
• Modification of arterial enhancement pattern in
irradiated region by concomitant HCC

I PATHOLOGY
General Features I SELECTED REFERENCES
• Etiology 1. Mori H et al: Radiation-induced liver injury showing low
o Unintentional & occurs when liver is unavoidably intensity on T2-weighted images noted in Budd-Chiari
syndrome. Radiat Med. 20(2):69-76, 2002
included in the treatment portal designed to
2. Willemart S et al: Acute radiation-induced hepatic injury:
encompass tumors & adjacent organs evaluation by triphasic contrast enhanced helical CT. Br J
o Patients who receive a single 1200-rad dose of Radiol. 73(869):544-6, 2000
external beam radiation or a 4000- to SSOO-rad 3. Unger EC et al: CT and MR imaging of radiation hepatitis. J
fractionated dose over 6 weeks can develop RILD Comput Assist Tomogr. 11(2):264-8, 1987
o Investigational use of hepatic arterial administration
of Yttrium-90 glass microspheres
• Emit radiation to perfused hepatic area persisting I IMAGE GALLERY
for 64 hour half-life
• Epidemiology: Now more commonly seen with advent
of three-dimensional treatment planning & bone
marrow transplantation with total body radiation
Microscopic Features
• Veno-occlusive disease
• Massive panlobar congestion, hyperemia, hemorrhage,
& mild proliferative change in sublobar central veins
o Stasis secondary to injury of these veins

I CLINICAL ISSUES
(Left) Axial CECT shows heterogeneous mass, (arrow) enhancing
Presentation during arterial phase; hepatocellular carcinoma (HCC). (Right) Axial
• Hepatomegaly/ascites/fatigue/rapid weight gain CECT in patient with HCC, following hepatic arterial embolization of
• Usually presents 2-16 weeks after treatment Yttrium-90 microspheres. Wedge of hypodensity in segments 5 + 8
• May present as late as 7 months represents radiation hepatitis.
SECTION 2: Biliary System

Introduction and Overview

Biliary System Anatomy and Imaging Issues 11-2-2

Congenital
Caroli Disease 11-2-6
Choledochal Cyst 11-2-10

Infection
Accending Cholangitis 11-2-14
Recurrent Pyogenic Cholangitis 11-2-16
Pancreato- Biliary Parasites 11-2-20
AIDS Cholangiopathy 11-2-22

Inflammation
Choledocholithiasis 11-2-26
Mirizzi Syndrome 11-2-30
Hyperplastic Cholecystoses 11-2-32
Cholecystitis 11-2-34
Porcelain Gallbladder 11-2-38
Milk of Calcium Bile 11-2-40
Primary Sclerosing Cholangitis 11-2-42

Neoplasm, Primary
Gallbladder Carcinoma 11-2-46
Cholangiocarcinoma 11-2-50
Ampullary Carcinoma 11-2-54
IPMT, Biliary 11-2-58

Treatment Related
Chemotherapy Cholangitis 11-2-60
 BILIARY SYSTEM ANATOMY AND IMAGING ISSUES

2
2

Graphic shows normal biliary tree, with detailed view of Coronal view of mangafodipir-enhanced MR showing
papilla of Vater. (See text for abbreviation keys). normal biliary tree. Some branches are not in this plane
of section.

• CBD usually joins pancreatic duct (PD) as a common

I TERMINOLOGY channel within the duodenal wall
Abbreviations and Synonyms • Sphincter of Oddi (ampullary sphincter)
• Gallbladder (GB) o Smooth muscle sheath surrounding the common
• Common hepatic duct (CHD) channel (CBD + PD)
• Right ventral - cephalic duct (RVC) • Sphincter of Boyden
• Right dorsal - caudal duct (RDC) o Choledochal sphincter; surrounds distal CBD
• Left lateral duct (LL) and medial duct (LM) o Contraction of sphincter of Oddi or Boyden can
• Duodenal papilla (DP) simulate CBD stricture or stone, though usually
• Choledochal sphincter (CDS) transient
• Pancreatic duct sphincter (PDS)
• Portal Vein (PV)
• Ultrasound (US) I ANATOMY-BASED IMAGING ISSUES I
• MR cholangiopancreatography (MRCP)
Imaging Approaches
• Current role of oral and intravenous (IV)
cholecystography
I IMAGING ANATOMY o Largely replaced by newer modalities
Location o IV contrast agents may be used to augment CT or
• GB is located in GB fossa, indentation on undersurface MR cholangiography (e.g., mangafodipir; IV
of liver manganese-based contrast agent that has
o "Intrahepatic" in < 10% of cases hepatobiliary excretion and can increase sensitivity
o Attached to liver by short veins and bile ducts (of and specificity of hepatic and biliary MR studies)
Lushka) and covered by parietal peritoneum • Role of cholescintigraphy
• GB fundus o IV administration of Tc-99m iminodiacetic acid
o Rounded distal tip; projects below edge of liver compounds ("Tc-HIDA"scans)
o "Phrygian cap"; fundus partially septated and folded o Undergoes rapid uptake by liver and excretion into
upon itself bile
o Allows visualization of CBD and gallbladder
• GB body
o Midportion; often in contact with duodenum and o Main uses: To confirm Dx of acute cholecystitis
hepatic flexure of colon (nonfilling GB) and bile leaks (e.g., after surgery or
trauma)
• GB neck
o Lies between GB body and cystic duct • Role of ultrasonography
o Bears a constant relationship to the main interlobar o Primary imaging modality for most GB + CBD
fissure (hepatic) and undivided right PV lesions
• Cystic duct o Detection of gallstones (near 100% accuracy)
o 2 to 4 cm long o Acute cholecystitis (- 95% accuracy)
o Contains tortuous spiral folds (valves of Heister) • Based on finding stones and focal tenderness
o Highly variable point of entry into CHD ("sonographic Murphy sign"), ± GB wall
thickening
 BILIARY SYSTEM ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
Tumor Classification Tumor-like lesions
• Adenomatous hyperplasia
Epithelial tumors
• Cholesterol polyp
• Adenoma • Inflammatory polyp
• Cystadenoma • Xanthogranulomatous cholangitis
• Papillomatosis • Primary sclerosing cholangitis 2
• Adenocarcinoma
• Cystadenocarcinoma Diffuse GB wall thickening 3

Nonepithelial tumors • Common

• Stromal (leiomyoma, lipoma, hemangioma, • ~ Cholecystitis
lymphangioma) • ~ Hepatitis
• Granular cell tumor • ~ Cardiac, renal, liver failure
• Neurofibroma, ganglioneuroma • Uncommon
• Lymphoma/metastasis • ~ Tumor (primary or metastatic)
• Carcinoid • ~ Adenomatous hyperplasia
• ~ Xanthogranulomatous cholecystitis

o Complications of cholecystitis (perforation,
gangrene)
o GB mass lesions (polyps, primary and metastatic
tumor)
• Role of computed tomography (CT)
o Less sensitive than US in detecting gallstones (stone
attenuation varies from less than water to densely
calcified, by chemical composition of stone)
o Accurate in diagnosis of complicated cholecystitis
(emphysematous, gangrenous, perforated, abscess)
o Primary role in diagnosis and staging of GB
carcinoma
• Role of magnetic resonance (MR)
o MRCP now a primary tool in evaluation of biliary
obstruction (calculi, intrinsic and extrinsic masses)
o Utilizes heavily T2 weighted sequences to show bile
as bright signal fluid
o Can be combined with other sequences to yield
comprehensive evaluation of liver, biliary tree, and
pancreas
o Produces images of intra- and extrahepatic bile ducts
(+ pancreatic duct) that rival those of endoscopic or
transhepatic cholangiograms
• Direct cholangiography
o Percutaneous trans hepatic cholangiography (PTC)
• For known or suspected biliary obstruction,
especially when endoscopic techniques are
unsuccessful (e.g., following prior biliary
diversion) or to diagnose and treat intrahepatic or
proximal extrahepatic biliary obstruction (e.g.,
Klatskin tumor)
o Endoscopic retrograde cholangiopancreatography
(ERCP)
• For known or suspected biliary obstruction,
especially distal obstruction (e.g., pancreatic
carcinoma) that may require endoscopic
placement of biliary stent, endoscopic retrieval of
stones, or biopsy/brushing of biliary tumors
• Also modality of choice for diagnosis and
treatment of traumatic/post-surgical bile leaks;
will usually resolve following endoscopic
placement of a biliary stent
o Post-operative (T-tube) cholangiography
• When CBD has been manipulated at surgery (e.g.,
choledocholithiasis, liver transplantation) aT-tube
is usually left in place within the CBD, allowing
safe access to the external limb
• Allows convenient and safe diagnosis of retained
stones, leaks, or strictures
I CLINICAL IMPLICATIONS
Clinical Importance
• Imaging goals (jaundice, abnormal LFTs)
o Determine presence, level, and cause of biliary
obstruction
• Bile duct dilation criteria
o Intrahepatic: Continuous arborization
• Or> 40% diameter of adjacent vein
o Extrahepatic
• CHD at porta, > 6 mm
• CBD, 8 to 10 mm
• 1 With aging, post-cholecystectomy
• Level of biliary obstruction
o Intrahepatic
• Primary sclerosing cholangitis (PSC)
• Liver mass
o Porta hepatic
• Cholangiocarcinoma
• PSC
• GB carcinoma
• Metastases
• Iatrogenic (e.g., lap. cholecystectomy)
o Intrapancreatic
• Pancreatic carcinoma
• Pancreatitis (usually chronic)
• CBD stones
• Ampullary stenosis
• Ampullary or duodenal carcinoma
• Cholangiocarcinoma
• Criteria for malignant obstruction
o Abrupt transition (dilated to stricture)
o Eccentric duct wall thickening
o Mass in or around duct (e.g., pancreatic cancer)
o Presence of nodes, metastases, vessel invasion
 BILIARY SYSTEM ANATOMY AND IMAGING ISSUES

Graphic shows common variations of cystic duct entry ERe? following cholecystectomy shows leak of bile
into common duct. (contrast) from cystic duct remnenat (arrow). Leak
resolved following placement of biliary stent.

• Criteria for benign obstruction • Uncommon

o Tapered transition o Emphysematous cholecystitis
• E.g., chronic pancreatitis, PSC o Parasitic biliary infestation
o Concentric wall thickening o CBD entry into duodenal diverticulum
o Presence of ductal calculi o Fistula from gut to CBD or GB
• Crohn disease
• Tumor of GB, gut, pancreas
I EMBRYOLOGY • Gallstone erosion into duodenum (origin of
"gallstone ileus")
Embryologic Events
• Bile ducts develop from embryologic ductal plate Sonographic echogenic and shadowing
which surrounds the PV lesion in GB wall
o Errors that occur during involution/remodeling of • Adherent or impacted gallstone
ductal plate lead to variety of fibrocystic • Spiral valve folds
abnormalities of the liver • Cholesterol polyp
• Polycystic liver (autosomal recessive and • Stone or crystal in Rokitansky-Aschoff sinus
dominant) • Intramural gas
• Caroli disease • Gas in duodenum
• Biliary hamartomas • Refraction from folds in GB neck
• Choledochal cyst
• Congenital hepatic fibrosis
o Often associated with similar fibrocystic diseases of I SELECTED REFERENCES
the kidney
1. Krause D et al: MRI for evaluating congenital bile duct
abnormalities.] Comput Assist Tomogr. 26(4):541-52, 2002
2. Gore RM: Gallbladder and Biliary Tract: Differential
I CUSTOM DIFFERENTIAL DIAGNOSISI Diagnosis. In Gore RM, Levine MS (eds) Textbook of
Gastrointestinal Radiology. 2nd ed. Philadelphia, WB
Bile duct stricture Saunders. 1408-14,2000
• Primary sclerosing cholangitis 3. Dohke M et al: Anomalies and anatomic variants of the
• Iatrogenic (e.g., laparoscopic surgery) biliary tree revealed by MR cholangiopancreatography. A]R
• Cholangiocarcinoma Am] Roentgenol. 173(5):1251-4, 1999
• AIDS cholangiopathy 4. Koeller KK, et al (eds): Radiologic pathology: 2nd ed.
• Chemotherapy cholangitis Washington, DC, Armed Forces Institute of Pathology.
2003
• Recurrent pyogenic (Oriental) cholangitis
5. Dahnert W: Radiology review manual 4th ed, Philadelphia,
Gas in biliary tree Lippincott, Williams and Wilkins. 2000
6. Eisenberg RL: Gastrointestinal radiology: A pattern
• Common approach: 3rd ed. Philadelphia,]B Lippincott. 1996
o Sphincterotomy; post-surgical 7. Schulte S]: Embryology, Normal Variation and Congenital
o Biliary stent Anomalies of the Gallbladder and Biliary Tract. In: Freeny
o Patulous sphincter . PC, Stevenson GW (eds): Alimentary Tract Radiology. 5th
• Elderly, prior CBD stone ed. St. Louis, Mosby 1994
I BILIARY SYSTEM ANATOMY AND IMAGING ISSUES
I IMAGE GALLERY
Typical
(Left) MRCP in coronal plane
shows multiple calculi in
dilated CBO. (Right) ERCP
shows irregular arborization
2
of intrahepatic ducts with
multiple strictures due to 5
primary sclerosing
cholangitis (PSG Polypoid
mass (arrow) in CBO is
cholangiocarcinoma.

Typical
(Left) Sagittal sonogram in
40 year old woman with
pain shows distended CB
and multiple small stones.
(Right) Tc-HIDA scan shows
flow of radiotracer from liver
into CBO (arrow) and bowel
(open arrow), but not the
CB. Acute cholecystitis.

(Left) MRCP shows dilated

intrahepatic ducts, normal
CBO (arrow); Klatskin
tumor. (Right) Transhepatic
cholangiogram shows dilated
intrahepatic ducts, complete
obstruction at confluence of
main ducts; Klatskin tumor.
 CAROLI DISEASE

2
6

ERCP shows saccular dilatation of intrahepatic bile Axial CECT shows massive dilatation of intrahepatic bile
ducts. ducts. Note the "central" or "eccentric" dot within
many of the cystic structures, representing portal
radides (arrows).

ITERMINOlOGY • Type V: Represents Caroli disease

• Cystic dilatation of intrahepatic bile ducts
Abbreviations and Synonyms o Caroli disease is of two types
• Communicating cavernous biliary ectasia • Simple type
• Periportal fibrosis type
Definitions • Frequently associated with renal tubular ectasia
• Caroli disease: Congenital, multifocal, segmental, • Have an autosomal recessive inheritance pattern
saccular dilatation of intrahepatic bile ducts (IHBD) o Usually manifests in adolescence, also seen in
• Caroli syndrome: Cystic bile duct dilatation plus newborns & infants
hepatic fibrosis
Radiographic Findings
• Endoscopic retrograde cholangiopancreatogram
IIMAGING FINDINGS (ERCP) findings .
o Saccular dilatations of IHBDs, stones, strictures
General Features o May show communicating hepatic abscesses
• Best diagnostic clue: "Central dot" sign: Strong,
CT Findings
enhancing, tiny dots (portal radicles) within dilated
intrahepatic bile ducts on CECT • NECT: Multiple, rounded, hypodense areas inseparable
from dilated IHBD
• Location: Liver: Diffuse, lobar, or segmental
• Size: Varies from few millimeters to few centimeters • CECT: Enhancing tiny dots (portal radicles) within
dilated IHBD
• Morphology
o One of the variants of fibropolycystic disease MR Findings
o Other variants of fibropolycystic disease • Tl WI: Multiple, small, hypointense, saccular
• Congenital hepatic fibrosis dilatations of IHBD
• Autosomal dominant polycystic liver disease • T2WI: Hyperintense
• Biliary hamartomas • Coronal half-Fourier rapid acquisition with relaxation
• Choledochal cyst enhancement (RARE)
o Based on Todani classification

DDx: Hepatic Cysts with or without Dilated Ducts

ADPLD Biliary Hamartomas Sclerosing Cholangitis RPC

 CAROLI DISEASE
Key Facts
Terminology
• Communicating cavernous biliary ectasia
• Caroli disease: Congenital, multifocal, segmental,
saccular dilatation of intrahepatic bile ducts (IHBD)
• Caroli syndrome: Cystic bile duct dilatation plus
hepatic fibrosis
Imaging Findings
• Best diagnostic clue: "Central dot" sign: Strong,
enhancing, tiny dots (portal radicles) within dilated
intrahepatic bile ducts on CECT
• Frequently associated with renal tubular ectasia
Top Differential Diagnoses
• Polycystic liver disease
• Biliary hamartomas
• Primary sclerosing cholangitis (PSC)
o Kidney: Multiple fluid-containing foci in papillae
(e.g., medullary sponge kidney or renal tubular
ectasia)
• T1 C+
o Enhancement of portal radicles within dilated IHBD
• MR Cholangiopancreatography (MRCP)
o Multiple hyperintense oval-shaped structures
o Shows continuity with biliary tree
o Luminal contents of bile ducts appear hyperintense
in contrast to portal vein, which appears as signal
void
Ultrasonographic Findings
• Real Time
o Dilated intrahepatic bile ducts
o May show intraductal calculi
o Echogenic septa completely or incompletely
traversing dilated lumen of bile ducts (referred to as
intraductal bridging)
o Small portal venous branches partially or completely
surrounded by dilated IRBD
Nuclear Medicine Findings
• Hepato Biliary Scan
o Unusual pattern of retained activity throughout
liver
• Technetium Sulfur Colloid
o Multiple cold defects
Imaging Recommendations
• Best imaging tool: ERCP or 3D MRCP
I DIFFERENTIAL DIAGNOSIS
Polycystic liver disease
• Hepatic cysts
o Numerous (> 10; usually hundreds)
o Do not communicate with each other or biliary tract
o Not associated with biliary ductal dilatation
o Do not opacify at ERCP or percutaneous
transhepatic cholangiography (PTC)
• Patients with this disease often harbor renal cysts - not
confined to medulla
• Ascending cholangitis
• Recurrent pyogenic cholangitis (RPC)
Pathology
• Genetics: Inherited as an autosomal recessive pattern
• Simple type
• Periportal fibrosis type
2
Clinical Issues 7
• Recurrent attacks of cholangitis, fever & jaundice
Diagnostic Checklist
• Rule out other liver diseases which have hepatic cysts
with or without dilated bile ducts
• ERCP: Saccular dilatations show communication with
IHBD which differentiates Caroli from other variants
of fibropolycystic disease
Biliary hamartomas
• One of the variants of fibropolycystic disease
• Rare benign, congenital malformation of bile ducts
• Location: Intraparenchymal
• Innumerable subcentimeter nodules in liver
• Varied enhancement
o Cystic: No enhancement
o Solid: Enhance & become isodense with liver
Primary sclerosing cholangitis (PSC)
• Strictures of both intra- & extrahepatic bile ducts
• Ductal dilatation is not as great as Caroli disease & not
saccular type
• PSC often shows isolated obstructions of IHBD; Caroli
disease does not
• Usually more prominent findings
o Strictures & ductal irregularity
• Often progresses to cirrhosis
Ascending cholangitis
• Intrahepatic abscesses communicate with bile ducts
o Mimics Caroli disease
• Margins of abscesses are irregular
• Extrahepatic bile duct dilatation
o Noted due to an obstructing stone or tumor
Recurrent pyogenic cholangitis (RPC)
• Dilatation of both intra- & extrahepatic bile ducts;
usually of cylindrical and not saccular type
• Biliary calculi of RPC
o Cast-like
o Often fill ductal lumen
I PATHOLOGY
General Features
• General path comments
o Embryology-anatomy
• Ductal plate malformation: Incomplete
remodeling of ductal plate leads to persistence of
embryonic biliary ductal structures
• Genetics: Inherited as an autosomal recessive pattern
 CAROLI DISEASE
• Etiology o Hepatic lobectomy or segmentectomy
o Simple type • Diffuse disease
• Malformation of ductal plate of large central IHBD o Conservative
• More common in adults o Decompression of biliary tract: External drainage or
o Periportal fibrosis type biliary-enteric anastomoses are effective
• Malformation of ductal plates of central IHBD & o Extracorporeal shock wave lithotripsy
smaller peripheral bile ducts, latter leading to o Oral bile salts
2 development of fibrosis o Liver transplantation
• More common in infants & children
8 • Epidemiology: Rare disease
• Associated abnormalities I DIAGNOSTIC CHECKLIST
o Medullary sponge kidney (renal tubular ectasia)
o Autosomal dominant polycystic kidney disease Consider
• Rule out other liver diseases which have hepatic cysts
Gross Pathologic & Surgical Features with or without dilated bile ducts
• Saccular dilatations of intrahepatic bile ducts
• Diffuse, lobar or segmental Image Interpretation Pearls
• Cholangiography: Bulbous dilatations of peripheral
Microscopic Features intrahepatic bile ducts
• Simple type • ERCP: Saccular dilatations show communication with
o Dilatation of segmental IHBD IHBD which differentiates Caroli from other variants
o Normal hepatic parenchyma of fibropolycystic disease
• Periportal fibrosis type
o Segmental dilatation of IHBD
o Proliferation of bile ductules & fibrosis I SELECTED REFERENCES
1. Guy F et al: Caroli's disease: magnetic resonance imaging
features. Eur Radiol. 12(11):2730-6, 2002
I CLINICAL ISSUES 2. Levy AD et al: Caroli's disease: radiologic spectrum with
pathologic correlation. AJRAm J Roentgenol.
Presentation 179(4):1053-7,2002
• Most common signs/symptoms 3. Krause D et al: MRI for evaluating congenital bile duct
o Simple type abnormalities. J Comput Assist Tomogr. 26(4):541-52, 2002
• RUQpain 4. Fulcher AS et al: Case 38: Caroli disease and renal tubular
• Recurrent attacks of cholangitis, fever & jaundice ectasia. Radiology. 220(3):720-3, 2001
o Periportal fibrosis type 5. Mortele KJ et al: Cystic focal liver lesions in the adult:
• Pain, hepatosplenomegaly differential CT and MR imaging features. Radiographies.
21(4):895-910,2001
• Hematemesis (due to varices) 6. Akin 0 et al: An unusual sonographie finding in Caroli's
o Can be asymptomatic at an early stage disease. AJRAm J Roentgenol. 171(4):1167, 1998
• Lab data 7. Gorka W et al: Value of Doppler sonography in the
o May show elevated liver enzymes & bilirubin levels assessment of patients with Caroli's disease. J Clin
• Diagnosis Ultrasound. 26(6):283-7, 1998
o ERCP 8. Asselah T et al: Caroli's disease: a magnetic resonance
o MRCP cholangiopancreatography diagnosis. Am J Gastroenterol.
93(1):109-10, 1998
Demographics 9. Pavone P et al: Caroli's disease: evaluation with MR
cholangiography. AJRAm J Roentgenol. 166(1):216-7, 1996
• Age 10. Miller WJ et al: Imaging findings in Caroli's disease. A]R
o Childhood and 2nd-3rd decade
Am J Roentgenol. 165(2):333-7, 1995
o Occasionally in infancy 11. Zangger P et al: MRI findings in Caroli's disease and
• Gender: M:F = 1:1 intrahepatic pigmented calculi. Abdom Imaging.
20(4):361-4, 1995
Natural History & Prognosis 12. Rizzo RJ et al: Congenital abnormalities of the pancreas
• Complications and biliary tree in adults. Radiographies. 15(1):49-68; quiz
o Simple type 147-8, 1995
• Stone formation (95%): Calcium bilirubinate 13. Choi BI et al: Caroli disease: central dot sign in CT.
• Recurrent cholangitis Radiology. 174(1):161-3, 1990
• Hepatic abscesses 14. Murphy BJ et al: The CT appearance of cystic masses of the
o Periportal fibrosis type liver. Radiographies. 9(2):307-22, 1989
15. Marchal GJ et al: Caroli disease: high-frequency US and
• Cirrhosis & portal hypertension pathologic findings. Radiology. 158(2):507-11, 1986
• Varices & hemorrhage
o Cholangiocarcinoma in 7% of patients
• Prognosis
o Long-term prognosis for Caroli disease is poor
Treatment
• Localized to lobe or segment
 CAROLI DISEASE

I IMAGE GALLERY

(Left) Axial NECT shows

cystic and fusiform dilatation
of IHBDs, worse in right 2
lobe. Note calcified stones in
dependent position (arrow).
(Right) Sagittal sonogram 9
shows cystic lesions in liver;
some with hyperechoic
calculi (arrow).

(Left) Axial T2WI MR shows

cystic and fusiform dilatation
of IHBD. (Right) MRCP
shows communication
between the saccular
hyperintense spaces and the
biliary tree. Gallbladder
(open arrow).

(Left) Axial T2WI MR shows

saccular dilatation of IHBD,
many containing
hypointense calculi (arrows).
(Right) Percutaneous
transhepatic cholangiogram
shows generalized saccular
dilatation of IHBD.
 CHOLEDOCHAL· CYST

2
10

Type IV-a choledochal cyst. Axial CECT shows marked Type tv-a choledochal cyst. ERCP shows fusiform
dilatation of common hepatic duct and moderate dilatation of common duct, dilated IHBD, and near
dilatation of IHBD. perpendicular entrance of CBD into pancreatic duct
(arrow).

ITERMINOLOGY • Type III: Intraduodenal diverticulum;

Definitions
• Congenital segmental aneurysmal dilatation of any
portion of bile ducts, most commonly main portion of
common duct (CD)
I IMAGING FINDINGS
General Features
• Best diagnostic clue: MRCP: Type I choledochal cyst -
hyperintense fusiform dilatation of extrahepatic bile
duct
• Location: Usually extrahepatic (80-90%); CD
• Size: Varies from 2-15 cm
• Morphology
o Most common congenital lesion of large bile ducts
o Maintains continuity with biliary tree
o Often co-exist with other cystic & fibrotic disorders
of liver
o Rare & usually manifest in infancy and childhood
o Increased female predominance
o Todani classification of choledochal cysts
• Type I: Solitary fusiform - extrahepatic (80-90%);
CD
• Type II: Extrahepatic supraduodenal diverticulum
choledochocele
• Type IVa: Fusiform & intrahepatic cysts
• Type IVb: Multiple extrahepatic cysts
• Type V: Multiple intrahepatic cysts; Caroli disease
Radiographic Findings
• Radiography
o Upper gastrointestinal series may show
• Anterior displacement of second part of
duodenum & antrum
• Inferior displacement of duodenum
• Widening of duodenal sweep
• ERCP
o Demonstrates all types of choledochal cysts
o Cystic or fusiform dilatation of common duct (CD)
o Shows CD mucosal diaphragm & aberrant insertion
of CBD into pancreatic duct
CT Findings
• NECT
o Well-defined water density cystic lesion along course
of bile ducts
o May show dilatation of intra-/extrahepatic bile ducts
• CECT
o Nonenhancing hypodense cystic lesion
o May show dilatation of intra-/extrahepatic bile ducts

DDx: Grossly Dilated Bile Ducts or Cysts Simulating Dilated Bile Ducts

(j
..
I? .', \
\".
l;

.•. .
.\

\,' '*
'

I
~;. :.",i .~
Pancreatic Carcinoma Caroli Disease Pancreatic Pseudocyst RPC
 CHOLEDOCHAL CYST

Key Facts
Terminology Pathology
• Congenital segmental aneurysmal dilatation of any • Due to ductal plate malformation of large bile ducts
portion of bile ducts, most commonly main portion • Anomalous junction of CBD & pancreatic duct
of common duct (CD) proximal to duodenal papilla forming long common
channel strongly associated with type I & IV
Imaging Findings choledochal cysts of Todani classification 2
• Best diagnostic clue: MRCP: Type I choledochal cyst - • Incidence: Asians more than Western countries
hyperintense fusiform dilatation of extrahepatic bile 11
duct Clinical Issues
• Size: Varies from 2-15 cm • Triad: Recurrent RUQ pain, jaundice, palpable mass
• May show dilatation of intra-/extrahepatic bile ducts
Diagnostic Checklist
Top Differential Diagnoses • Rule out other pathologies which can cause "marked
• Malignant CBD obstruction biliary dilatation"
• Caroli disease • MRCP or ERCP: Large, solitary cystic or fusiform
• Pancreatic pseudocyst dilatation of CBD with intra- & extrahepatic bile duct
• Recurrent pyogenic cholangitis (RPC) dilatation

• Dilatation of both intra- & extrahepatic bile ducts

MR Findings
• Tl WI: Hypointense
• T2WI: Hyperintense
• Tl C+: Nonenhancing lesion
• MRCP (MR cholangiopancreatography)
o Bile appears hyperintense in contrast to portal vein
• Type I: Solitary cystic or fusiform dilatation of
common duct (CD)
• Type II: Extrahepatic supraduodenal diverticulum
• Type III: Bulbous dilatation of intramural segment
of distal CD
• Type IVa: Marked dilatation of entire extrahepatic
bile duct plus central intrahepatic bile ducts
(IHBD) on coronal oblique images
Ultrasonographic Findings
• Real Time
o Transverse scan
• Large anechoic cyst in subhepatic area
o Oblique scan: Large, marked cystic & fusiform
dilatation of IHBD plus CBD (type IVa cyst)
o Obstetric-ultrasound
• Earliest diagnosis at 25 weeks of pregnancy
• Right-sided large cyst in fetal abdomen plus
adjacent dilated hepatic ducts
• DDx: Duodenal atresia; cyst of liver, mesentery,
omentum or ovary
Nuclear Medicine Findings
• Hepato Biliary Scan
o Large photopenic area in liver
o Shows late filling & prolonged stasis of isotope
o Excludes all other differential diagnoses
Imaging Recommendations
• ERCP or MRCP (coronal & oblique views)
• NE + CECT (coronal reconstruction images)
I DIFFERENTIAL DIAGNOSIS
Malignant CBD obstruction
• Extrahepatic: Short stricture or small polypoid mass
proximal to obstruction
• NECT
o Small growth: Poorly sensitive
o Large growth: Hypodense mass & IHBD dilatation
• CECT
o Persistent enhancing tumor (due to fibrous stroma)
• ERCP
o Exophytic intraductal tumor mass (2-5 mm)
o Infiltrating type
• Frequently long & rarely short focal stricture
Caroli disease
• Inherited as an autosomal recessive pattern
• Due to ductal plate malformation of large intrahepatic
bile ducts
• Multiple small rounded hypodense or hypointense
saccular dilatations of intrahepatic bile ducts
• CECT: "Central dot" sign
o Enhancing tiny dots (portal radicles) within dilated
cystic intrahepatic bile ducts
• ERCP,MRCP & microscopic finding
o Communicating bile duct abnormality
• According to Todani classification Caroli disease
represents type V
Pancreatic pseudocyst
• No communication with bile ducts
• Cystic mass with infiltration of peripancreatic fat
planes
• NECT
o Round hypodense lesion of near water density
o Lobulated, heterogeneous, mixed density lesion (due
to hemorrhage or infection)
• CECT
o Enhancement of thin rim of fibrous capsule
• MRCP: Hyperintense cyst contiguous with dilated
pancreatic duct
• ERCP: Pseudocyst communicating with pancreatic
duct seen in 70% cases
Recurrent pyogenic cholangitis (RPC)
• Dilatation of both intra- & extrahepatic bile ducts
• Stones, sludge, pneumobilia & abscess
 CHOLEDOCHAL CYST
• Biliary calculi: Cast-like & often fill ductal lumen • Abdominal pain (fusiform); palpable mass or
• MRCP: Skip dilatations, strictures of intra- & jaundice (cystic)
extrahepatic bile ducts o Infants: Intermittent jaundice & abdominal mass
• Etiology: Parasites, stones, gram negative bacteria o Children & adults: Intermittent fever, vomiting,
• Most commonly seen in Asians jaundice, pain & pruritus
Demographics
2 I PATHOLOGY • Age
o Usually seen in infancy & childhood
12 General Features o Can present from birth to old age
• General path comments o 25% detected before age 1
o Embryology-anatomy o 80% diagnosed in childhood
• Due to ductal plate malformation of large bile • 60% of patients present before age 10
ducts o 20% in adults
• Faulty budding of primitive pancreatic duct • Gender: M:F = 1:4
• Etiology
o Anomalous junction of CBD & pancreatic duct
Natural History & Prognosis
proximal to duodenal papilla forming long common • Complications
channel strongly associated with type I & IV o Calculi, cholangitis, pancreatitis
choledochal cysts of Todani classification o Rupture, bile peritonitis, abscess & hemorrhage
o Higher pressure in pancreatic duct & absent ductal o Rarely malignant degeneration
sphincter • Cholangiocarcinoma
o Free reflux of enzymes into CBD causes weakening • Prognosis
of CBD wall & dilatation o Usually good after surgical repair
• Epidemiology o Poor: Rupture, peritonitis & malignant degeneration
o Prevalence: 1:13,000 admissions Treatment
o Incidence: Asians more than Western countries
• Surgical excision & reconstruction by Roux-en-Y
• Associated abnormalities hepaticojejunostomy
o Gallbladder: Aplasia or double gallbladder
o Biliary anomalies
• Biliary atresia or stenosis
• Congenital hepatic fibrosis
I DIAGNOSTIC CHECKLIST
o Annular pancreas Consider
Gross Pathologic & Surgical Features • Rule out other pathologies which can cause "marked
biliary dilatation"
• Cystic/fusiform dilated sac with bile, stones or sludge
• Long ectatic common channel with pancreatic duct Image Interpretation Pearls
o Normal length: 0.2-1.0 cm; average: 0.5 cm • MRCP or ERCP: Large, solitary cystic or fusiform
Microscopic Features dilatation of CBD with intra- & extrahepatic bile duct
dilatation
• Widespread ulceration & a denuded mucosa in dilated
common bile duct (CBD)
• Thickened ductal wall consists of chronic
inflammatory cells & fibrous tissue I SELECTED REFERENCES
1. Dohke M et al: Anomalies and anatomic variants of the
Staging, Grading or Classification Criteria biliary tree revealed by MR cholangiopancreatography. A]R
• Classification of anomalous pancreaticobiliary ductal Am] Roentgenol. 173(5):1251-4, 1999
junction (APBD) 2. Govil S et al: Choledochal cysts: evaluation with MR
o Type (P-B): Perpendicular insertion of pancreatic cholangiography. Abdom Imaging. 23(6):616-9, 1998
duct into CBD (fusiform) 3. Irie H et al: Value of MR cholangiopancreatography in
evaluating choledochal cysts. A]RAm] Roentgenol.
o Type (B-P): Perpendicular insertion of CBD into 171(5):1381-5, 1998
pancreatic duct (cystic) 4. Matos C et al: Choledochal cysts: comparison of findings at
o Two major duct unions are associated with type I MR cholangiopancreatography and endoscopic retrograde
choledochal cyst cholangiopancreatography in eight patients. Radiology.
• Classification according to angle of ductal union 209(2):443-8, 1998
o Right-angled union: Cystic dilatation of CBD 5. Kim OH et al: Imaging of the choledochal cyst.
o Acute angled union: Fusiform dilatation of CBD Radiographies. 15(1):69-88, 1995
6. Rizzo R] et al: Congenital abnormalities of the pancreas
and biliary tree in adults. Radiographies. 15(1):49-68; quiz
147-8, 1995
I CLINICAL ISSUES 7. Ebel KD et al: Choledochal cysts: classification and
cholangiographic appearance. A]RAm] Roentgenol.
Presentation 159(3):674-5, 1992
• Most common signs/symptoms
o Triad: Recurrent RUQ pain, jaundice, palpable mass
 CHOLEDOCHAL CYST

I IMAGE GALLERY
Typical

,/ - I
, .
(Left) Todani classification of
choledochal cysts. (Right)
Type !Va choledochal cyst. 2
,
I \ I ,. i .. MRCP shows fusiform
dilatation of intra- and
r .1/ f /' f, /' extrahepatic bile ducts and
abnormal entrance of CBD
13
• into pancreatic duct (arrow).

•
I, II III Gallbladder (open arrow).

..
•• • •
,. -
, j { ,
J ( ,
"
f J/ f 1
\ . '/ , /'

IVa IVb V
Typical
(Left) Axial CECT shows
markedly dilated extra- and
intrapancreatic CBD. Type I
choledochal cyst. (Right)
Coronal reformation of CECT
shows fusiform dilatation of
common duct. Type I
choledochal cyst.

Variant
(Left) Axial CECT shows a
spherical cystic lesion within
the medial wall of the
duodenum, while the rest of
the biliary tree was normal.
Type III; choledochocele.
(Right) Axial CECT shows
Type I choledochal cyst with
cholangiocarcinoma. Dilated
common duct with irregular
wall thickening and a mass
(arrow) invading adjacent
liver.
 ASCENDING CHOLANGITIS

2
14

Axial CECT shows dilated IHBO with indistinct margins. ERC? shows dilated left IHBO with abnormal
arborization and tapering.

ITERMINOLOGY • AIDS-related cholangitis

• Chemotherapy-induced cholangitis
Abbreviations and Synonyms • Ischemic cholangitis
• Ascending (bacterial) cholangitis o Secondary nonsclerosing cholangitis
• Malignant or benign liver/biliary pathology
Definitions o Based on onset, classified into acute & chronic
• Inflammation of intra-/extrahepatic bile duct walls,
usually due to ductal obstruction and infection Radiographic Findings
• Cholangiography
o Ascending (bacterial) cholangitis
IIMAGING FINDINGS • Stone: Radiolucent filling defect
• Irregular & thick bile duct lumen/wall
General Features • Ductal stricture, obstruction & proximal dilatation
• Best diagnostic clue: Irregular contour, branching • IHBD may show communicating hepatic abscesses
pattern & dilatation of bile ducts o Secondary sclerosing & nonsclerosing may mimic
• Location: Intra-/extrahepatic bile ducts primary sclerosing cholangitis at cholangiography
• Morphology
CT Findings
o Usually secondary to gallstones & infection in
industrialized countries • Obstructing stone: Calcific/soft tissue/water density
o Often due to poor nutrition & parasitic infestation • "Bull's eye" sign: Rim of bile surrounding a stone
in developing countries • Dilatation of intra-/extrahepatic bile ducts
o Classification of cholangitis (etiology/pathogenesis) • High density intraductal material (purulent bile)
• Primary sclerosing cholangitis (PSC) • Communicating small hepatic abscesses may be seen
• Secondary sclerosing cholangitis MR Findings
• Secondary nonsclerosing cholangitis
• TIWI
o Secondary sclerosing cholangitis o Stones (hypointense); bile (hypointense)
• Ascending (bacterial) cholangitis o Dilatations, strictures, thickening of bile duct wall
• Recurrent pyogenic (parasitic) cholangitis (RPC) • T2WI: Stones (hypointense); bile (hyperintense)

DDx: Irregularly Dilated Bile Ducts

Sclerosing Cholangitis RPC AIDS Cholangiopathy Cherno. Cholangitis

 ASCENDING CHOLANGITIS

Key Facts
Terminology Top Differential Diagnoses
• Ascending (bacterial) cholangitis • Primary sclerosing cholangitis (PSC)
• Recurrent pyogenic (RPC), AIDS, chemotherapy
Imaging Findings
cholangitis
• Best diagnostic clue: Irregular contour, branching
pattern & dilatation of bile ducts
• "Bull's eye" sign: Rim of bile surrounding a stone
Diagnostic Checklist 2
• Correlate with clinical & lab data to achieve an
• High density intraductal material (purulent bile) accurate cholangiographic interpretation 15
• Communicating small hepatic abscesses may be seen

• MRCP • Gender: M:F = 1:1

o Low signal filling defects (stones) within increased
signal bile Natural History & Prognosis
o Irregular strictures, proximal dilatation of bile ducts • Complications: Small liver abscesses & sepsis
• Prognosis: 100% mortality if not decompressed
Ultrasonographic Findings
• Real Time Treatment
o Dilatation, stenosis & thickening of bile duct walls • Antibiotics to cover gram negative organisms
o Purulent bile: Intraluminal echogenic material • Interventional management of stones/strictures
o Gallbladder: Thickened wall with or without calculi
Imaging Recommendations I DIAGNOSTIC CHECKLIST
• Ultrasonography & MRCP
• Cholangiography (T-tube, retrograde, PTC) Consider
• Correlate with clinical & lab data to achieve an
accurate cholangiographic interpretation
I DIFFERENTIAL DIAGNOSIS Image Interpretation Pearls
Primary sclerosing cholangitis (PSC) • Cholangiography: Strictures, dilatations, intraluminal
• Segmental strictures, beaded and pruned ducts filling defects due to stones
• Involves both intrahepatic & extrahepatic ducts
• End-stage: Liver (lobular, hypertrophy & atrophy)
I SELECTED REFERENCES
Recurrent pyogenic (RPC), AIDS,
1. Arai K et al: Dynamic CT of acute cholangitis: early
chemotherapy cholangitis inhomogeneous enhancement of the liver. AJRAm J
• Clinical setting supports diagnosis Roentgenol. 181(1):115-8, 2003
2. Song HH et al: Eosinophilic cholangitis: US, CT, and
cholangiography findings. J Comput Assist Tomogr.
21(2):251-3, 1997
I PATHOLOGY 3. Balthazar EJ et al: Acute cholangitis: CT evaluation. J
Comput Assist Tomogr. 17(2):283-9, 1993
General Features
• Etiology
o Due to bile duct calculi, stricture & papillary stenosis
o Pathogenesis: Stone/obstruction/bile stasis/infection
I IMAGE GALLERY
• Epidemiology: Most common type of cholangitis in
Western countries
• Associated abnormalities: Usually gallstones

ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Charcot triad (pain,
fever, jaundice)
• Lab data
o Increased WBC count & bilirubin levels
o Increased alkaline phosphatase
(Left) Axial CECT shows an abscess in right hepatic lobe due to
o Positive blood cultures in toxic phase ascending cholangitis. (Right) ERCP shows moderate dilatation of bile
Demographics ducts, extravasation into a liver abscess (arrow). CBO stone was
extracted endoscopically earlier.
• Age: 20-50 years
 RECURRENT PYOGENIC CHOLANGITIS

2
16

Graphic demonstrates marked dilation of intrahepatic Axial CECT of recurrent pyogenic cholangitis
bile ducts with multiple common bile duct and demonstrates marked intrahepatic biliary dilatation with
intrahepatic stones. numerous intrahepatic stones (arrows).

o Rapid tapering of dilated intrahepatic ducts with

ITERMINOLOGY "arrowhead" configuration
Abbreviations and Synonyms o Non-filling of biliary ductal segments due to
• Recurrent pyogenic cholangitis (RPC), oriental strictures of intrahepatic ducts
cholangitis, oriental cholangiohepatitis CT Findings
Definitions • NECT: Biliary stones may be high attenuation or
• Intra- and extrahepatic biliary pigment stones isodense to liver
occurring in patients and immigrants from SE Asia • CECT
o Dilated intra- and extrahepatic biliary ducts within
involved segments on CECT
I IMAGING FINDINGS o CBD may be markedly enlarged
o May be associated with low attenuation pyogenic
General Features liver abscesses, fatty liver atrophy of segments with
• Best diagnostic clue: Intra- and extrahepatic biliary chronic biliary obstruction
stones without stones in gallbladder (GB) MR Findings
• Location: Confined to left lobe (often lateral segment)
• T1WI: Hypointense dilated ducts and intermediate
or involving all biliary ductal segments & common
intensity biliary calculi; may have hyperintense rim
bile duct (CBD)
• T2WI: Hyperintense bile within obstructed ducts and
• Size: Stones are typically 1-4 cm in size
low signal calculi
• Morphology: Combination of pigment stones and
• T1 C+: Hypointense dilated bile ducts with low to
biliary sludge
intermediate signal biliary calculi
Radiographic Findings • MRCP
o Dilated intra- and extrahepatic ducts with low signal
• ERCP
o Dilated intra- and extrahepatic bile ducts filling defects representing stones; intrahepatic ducts
o Common duct stones and intrahepatic duct stones taper rapidly ("arrowhead sign")
without stones in gallbladder

'., ..
DDx: Spectrum of Biliary lesions Mimicking

.~'"'"
.:'
RPC

~~~. '..~
..• 'f ..0,
.....;.t ~",; 1

~..~"r.,j .
.•. , .....
~-. -

Hepatic Stones Cholangitis Bact Cholangitis Cholangiocarcinoma

 RECURRENT PYOGENIC CHOLANGITIS

Key Facts
Terminology • Cholangiocarcinoma
• Intra- and extrahepatic biliary pigment stones Pathology
occurring in patients and immigrants from SE Asia
• Intraductal pigment calculi within intra- and
Imaging Findings extrahepatic ducts, proliferative fibrosis of CBD walls,
• Best diagnostic clue: Intra- and extrahepatic biliary periductal abscesses
• Associated with biliary parasitic infection with
2
stones without stones in gallbladder (GB)
• Location: Confined to left lobe (often lateral Clonorchis sinensis and/or ascaris lumbricoides 17
segment) or involving all biliary ductal segments & Clinical Issues
common bile duct (CBD)
• RUQ pain, recurrent fevers, jaundice
• Dilated intra- and extrahepatic biliary ducts within • Leukocytosis, elevated alkaline phosphatase and
involved segments on CECT bilirubin
Top Differential Diagnoses Diagnostic Checklist
• Intrahepatic stones secondary to biliary stricture • Intra- & extrahepatic bile duct dilatation/stones in SE
• Sclerosing cholangitis Asian patients
• Bacterial cholangitis

Ultrasonographic Findings Bacterial cholangitis

• Real Time: Dilated intrahepatic ducts with echogenic • Dilated intra- and extrahepatic ducts
debris; calculi may/may not cause acoustic shadowing • Stones, sludge and pus in bile ducts
• Color Doppler: No flow within dilated bile ducts • Intra- or extrahepatic strictures
Nuclear Medicine Findings Cholangiocarcinoma
• WBC Scan • Associated with sclerosing cholangitis, choledochal
o Positive for cholangitic liver abscesses cyst, RPC, clonorchiasis
• Infiltrative type at confluence of right and left ducts
Other Modality Findings most common
• Cholangiographic findings: Dilated intra- and • Ductal dilatation of involved segments with or
extrahepatic ducts with filling defects (stones) without parenchymal mass
o "Arrowhead" deformity of rapidly tapering • CECT demonstrates delayed contrast-enhancement
intrahepatic ducts within parenchymal mass components
o Similar to ERCP
Imaging Recommendations
• Best imaging tool: CECT
I PATHOLOGY
• Protocol advice General Features
o 150 ml IV contrast at 2.5 ml/sec for CECT • General path comments
• 5 mm collimation and reconstruction at 5 mm o Intraductal pigment calculi within intra- and
intervals extrahepatic ducts, proliferative fibrosis of CBD
o Heavily T2WI!MRCP and gadolinium-enhanced walls, periductal abscesses
breathheld GRE o End stage biliary cirrhosis
• Genetics: No known genetic predisposition
• Etiology
I DIFFERENTIAL DIAGNOSIS o Associated with biliary parasitic infection with
Intrahepatic stones secondary to biliary Clonorchis sinensis and/or ascaris lumbricoides
o Associated with E. coli infection of bile ducts
stricture • Bacterial production of beta-glucuronidase
• Stricture may be due to prior surgery, trauma or • Leads to hydrolysis of bilirubin, development of
chemotherapy calcium bilirubinate stones within intra- &
• Non-Asian patient extrahepatic bile ducts
• Similar clinical presentation with RUQ pain, fever and o Associated with poor general nutrition
chills • Epidemiology
o Primarily within SE Asia and immigrants from SE
Sclerosing cholangitis
Asia
• Diffuse thickening of CBD o Endemic in SE Asian patients and immigrants from
• Multiple intrahepatic strictures SE Asia (China, Vietnam, Philippines)
• Stones form distal to strictures • Associated abnormalities: Poor nutrition
• Associated with inflammatory bowel disease
 RECURRENT PYOGENIC CHOLANGITIS
Gross Pathologic & Surgical Features
• Dilated bile ducts with brown mud-like pigment
stones, pus
• May have parasitic infection in biliary ducts with
Clonorchis or ascaris
2 Microscopic Features
• Periductal inflammatory changes with infiltration of
18 periportal spaces with inflammatory cells leading to
periductal fibrosis and ultimately biliary cirrhosis
o Inflammatory cells leading to periductal fibrosis and
ultimately biliary cirrhosis
• Localized segmental hepatic atrophy
• Fatty changes in liver
Staging, Grading or Classification Criteria
• Classification based on distribution of affected biliary
segment
o May be isolated to left lobe, particularly lateral
segment
o May involve all biliary segments, as well as CBD
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o RUQ pain, recurrent fevers, jaundice
o Other signs/symptoms
• Hypotension, shaking, chills
• Related to gram-negative septicemia
• Clinical profile
o Leukocytosis, elevated alkaline phosphatase and
bilirubin
o Diagnosis by CT, US or cholangiography
Demographics
• Age: Over 40
• Gender: Affects males and females equally
• Ethnicity: Chinese and SE Asian population
Natural History & Prognosis
• Repeated episodes of cholangitis
• Complications
o Benign: Liver abscesses, biliary stricture and biliary
stones
• Long-term repeated episodes of cholangitis &
stricture formation lead to biliary cirrhosis
o Malignant: Cholangiocarcinoma
Treatment
• Options, risks, complications
o Endoscopic sphincterotomy
o Surgical
• Biliary drainage with hepatico-jejunostomy
• Subcutaneous jejunal ostomy for biliary access
• Left hepatic lobe resection if isolated left lobe
disease
o Interventional radiology
• Percutaneous biliary drainage of affected segments
• Basket and removal of pigment stones
• Balloon dilation of biliary strictures
• Repeated percutaneous procedures to clear
pigment stones & mud-like biliary debris
o Medical therapy
• Long-term suppressive antibiotic therapy
I DIAGNOSTIC CHECKLIST
Consider
• CECT, MRCP
Image Interpretation Pearls
• Intra- & extrahepatic bile duct dilatation/stones in SE
Asian patients
• Massive CBD dilatation
• Rapid tapering of intrahepatic ducts ("arrowhead" sign)
I SELECTED REFERENCES
1. Lee W] et al: Radiologic spectrum of cholangiocarcinoma:
emphasis on unusual manifestations and differential
diagnoses. Radiographics. 21 Spec No:S97-S116, 2001
2. Park MS et al: Recurrent pyogenic cholangitis: comparison
between MR cholangiography and direct cholangiography.
Radiology. 220(3):677-82, 2001
3. Kim M] et al: MR imaging findings in recurrent pyogenic
cholangitis. A]R Am] Roentgenol. 173(6):1545-9, 1999
4. Cosenza CA et al: Current management of recurrent
pyogenic cholangitis. Am Surg. 65(10):939-43, 1999
5. Harris HW et al: Recurrent pyogenic cholangitis. Am] Surg.
176(1):34-7, 1998
6. Leow CK et al: Re: Biliary access procedure in the
management of oriental cholangiohepatitis. Am Surg.
64(1):99, 1998
7. Lo CM et al: The changing epidemiology of recurrent
pyogenic cholangitis. HKM]. 3(3):302-4, 1997
8. Lee DW et al: Biliary infection. Baillieres Clin
Gastroenterol. 11(4):707-24, 1997
9. Sperling RM et al: Recurrent pyogenic cholangitis in Asian
immigrants to the United States: natural history and role of
therapeutic ERCP.Dig Dis Sci. 42(4):865-71, 1997
10. Kirby CL et al: US case of the day. Oriental
cholangiohepatitis. Radiographics. 15(6):1503-6, 1995
11. Mack E: Pathogenesis and Clinical Presentation of Bile
Duct Calculi. Semin Laparosc Surg. 2(2):76-84, 1995
12. Reynolds WR et al: Oriental cholangiohepatitis. Mil Med.
159(2):158-60, 1994
13. Enriquez G et al: Intrahepatic biliary stones in children.
Pediatr Radiol. 22(4):283-6, 1992
14. Kusano S et al: Oriental cholangiohepatitis: correlation
between portal vein occlusion and hepatic atrophy. A]R
Am] Roentgenol. 158(5):1011-4, 1992
15. Goldberg HI et al: Diagnostic and interventional
procedures for the biliary tract. Curr Opin Radiol.
3(3):453-62, 1991
16. Lim ]H: Oriental cholangiohepatitis: pathologic, clinical,
and radiologic features. A]R Am] Roentgenol. 157(1):1-8,
1991
17. Schulte S] et al: CT of the extrahepatic bile ducts: wall
thickness and contrast enhancement in normal and
abnormal ducts. A]R Am] Roentgenol. 154(1):79-85, 1990
18. Kashi H et al: Recurrent pyogenic cholangiohepatitis. Ann
R Coli Surg Engl. 71(6):387-9, 1989
19. vanSonnenberg E et al: Oriental cholangiohepatitis:
diagnostic imaging and interventional management. AJR
Am] Roentgenol. 146(2):327-31, 1986
20. Federle MP et al: Recurrent pyogenic cholangitis in Asian
immigrants. Use of ultrasonography, computed
tomography, and cholangiography. Radiology.
143(1):151-6, 1982
 RECURRENT PYOGENIC·CHOLANGITIS
I IMAGE GALLERY
(Left) Axial NEeT of
intrahepatic stones in
recurrent pyogenic
cholangitis. Note high
attenuation stones in right
2
lobe (arrow). (Right) Axial 19
NECT of intrahepatic stones
in recurrent pyogenic
cholangitis. Note high
attenuation stones in left
lobe (arrow).

Typical
(Left) Axial CECT of
recurrent pyogenic
cholangitis. Note dilated
intrahepatic ducts (arrow)
with numerous right lobe
intrahepatic calculi (open
arrow). (Right) Coronal
CECT reformation of
recurrent pyogenic
cholangitis demonstrates lack
of stones in gallbladder but
numerous intrahepatic
stones (arrows).

Typical
(Left) Axial CECT
demonstrates massive
dilatation of common bile
duct due to stones from
recurrent pyogenic
cholangitis. Note large
pigment stone in common
bile duct (arrow). (Right)
ERCP of common bile duct
in recurrent pyogenic
cholangitis demonstrates
massive filling defect in
common bile duct due to
stone (arrows).
 PANCREATO-BILIARY PARASITES

2
20

ERCP of ascaris in main pancreatic duct (arrow). Ascaris retrieved from bile duct with multiple stones.

ITERMINOLOGY CT Findings
• CECT: Intraductal increased density areas 2° to biliary
Abbreviations and Synonyms worms, fluids; peripancreatic inflammation 2° to
• Biliary ascariasis, biliary clonorchiasis pancreatitis
Definitions MR Findings
• Biliary & pancreatic duct involvement with parasitic • Tl WI: Low signal branching dilated bile ducts
infection from ascariasis, clonorchiasis • T2WI: High signal fluid 2° to dilated ducts with low
signal filling defects
• MRCP
IIMAGING FINDINGS o Dilated bile ducts with low signal linear or rounded
General Features filling defects within high signal bile
• Best diagnostic clue: Longitudinal filling in bile or Ultrasonographic Findings
pancreatic ducts on ERCP in ascariasis • Real Time
• Location: Gallbladder (GB), common bile duct (CBD), o US: "Bull's eye" appearance 2° to echogenic filling
intrahepatic or pancreatic ducts defect
• Size: 2-10 cm • Motility of worms may be evident; central
• Morphology: Linear or rounded anechoic area (digestive tract of worm)
Radiographic Findings Nuclear Medicine Findings
• ERCP • Hepato Biliary Scan: Lack of GB filling in
o Linear, elliptical, rounded filling defects on ERCP ascariasis-related cholecystitis
• Ascariasis may involve entire biliary tract &
pancreatic duct Imaging Recommendations
• Clonorchiasis typically involves peripheral • Best imaging tool: US, ERCP
intrahepatic ducts, not GB or CBD

DDx: Biliary /Pancreati'cl-~sion~Mil"l1ickingParasites

Biliary Lithiasis Cholangiocarcinoma Bact Cholangitis RPC

 PANCREATO-BILIARY PARASITES

Key Facts
Terminology Pathology
• Biliary & pancreatic duct involvement with parasitic • General path comments: Cholangitis, periductal
infection from ascariasis, clonorchiasis fibrosis, acute pancreatitis
Imaging Findings Clinical Issues
• Linear, elliptical, rounded filling defects on ERCP
• us: "Bull's eye" appearance 2 to echogenic filling
0
• RUQ pain, jaundice, anorexia 2
defect 21

I DIFFERENTIAL DIAGNOSIS • Ethnicity: Higher incidence of clonorchiasis in Asian

patients
Biliary lithiasis Natural History & Prognosis
• Echogenic ductal foci with acoustic shadowing on US
• May be asymptomatic with early infection; medication
Cholangiocarcinoma therapy generally effective
• Polypoid intraductal mass Treatment
Bacterial cholangitis • Options, risks, complications
• Low-level echoes (pus) in bile ducts o Pyrantel embonate 90% effective for ascariasis
o Praziquantel moderately effective for clonorchiasis;
Recurrent pyogenic cholangitis (RPC) 20% cure rate with single dose
• Marked biliary dilatation with multiple intraductal
pigment stones
I DIAGNOSTIC CHECKLIST
I PATHOLOGY Consider
• Cholangiocarcinoma
General Features
Image Interpretation Pearls
• General path comments: Cholangitis, periductal
fibrosis, acute pancreatitis • Linear or rounded intraductal filling defects in
• Etiology: Ascaris infestation: Ova ingested, pass pancreatic or common bile ducts
through liver, lungs; worms develop in small bowel
• Epidemiology
o Ascariasis most prevalent helminth infection I SELECTED REFERENCES
worldwide 1. Haseeb AN et al: Evaluation of excretory/secretory Fasciola
o Clonorchiasis endemic in Asia, present in Western (Fhes) antigen in diagnosis of human fascioliasis. J Egypt
world 2 to travel, immigration
0 Soc Parasito!. 33(1):123-38, 2003
2. Amjad N et al: An unusual presentation of acute
• Associated abnormalities: Cholangio CA w/Clonorchis
cholecystitis: biliary ascariasis. Hosp Med. 62(6):370-1,
Gross Pathologic & Surgical Features 2001
3. Capallo DV et al: Biliary ascariasis. South Med J.
• Worms & flukes in biliary tree, cholangitis, acute
77(9):1201-2, 1984
cholecystitis, acute pancreatitis
Microscopic Features
• Periductal round cell infiltrate & fibrosis; pancreatic I IMAGE GALLERY
edema & infiltration

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o RUQ pain, jaundice, anorexia
o Weight loss, diarrhea, palpitations; small bowel
obstruction common with ascariasis
• Clinical profile: Patient with poor nutrition
Demographics
• Age: Ascariasis common in children (Left) ERCP demonstrates left duct dilatation and filling defects from
intrahepatic flukes. (Right) Magnified view of ERC? demonstrates
rounded filling defects in left ducts from Clonorchis.
 AIDSCHOLANGIOPATHY

2
22

ERep of AIDS cholangitis demonstrates multiple Sagittal US of common bile duct demonstrates diffuse
intrahepatic biliary strictures and irregular ductal thickening representing edema from AIDS cholangitis,
contours (arrows),

ITERMINOlOGY CT Findings
Abbreviations and Synonyms • CECT
o Ductal dilatation of CBD and intrahepatic ducts
• AIDS cholangitis, AIDS-related sclerosing cholangitis o Ductal dilatation may asymmetrically involve
Definitions intrahepatic bile ducts
• Spectrum of biliary inflammatory lesions caused by MR Findings
AIDS-related opportunistic infections leading to biliary
• T1WI: Low signal dilated bile ducts
stricture/obstruction or cholecystitis
• T2WI: High signal dilated bile ducts and CBD; GB wall
thickening
• Tl C+
I IMAGING FINDINGS o Dilated bile ducts and CBD; GB wall thickening;
General Features perich olecystic inflammatory changes
• Best diagnostic clue: AIDS patient with multiple • Patients with acute acalculous cholecystitis
intrahepatic strictures, ampullary stenosis} or • MRCP
o High signal dilated ducts; intra- and extrabiliary
gallbladder (GB) wall thickening from cholecystitis
strictures on MRCP
• Location
o GB, common bile duct (CBD) Ultrasonographic Findings
o Intrahepatic ducts may be involved • Real Time
• Size: Varies from short focal biliary strictures to longer o Diffuse GB thickening; peri ductal hyper- or
segment strictures hypoechoic areas; dilated intrahepatic ducts; diffuse
• Morphology: Irregular intrahepatic strictures mimic CBD thickening on US
sclerosing cholangitis • Due to edema and ulceration of CBD
Radiographic Findings • Color Doppler: No flow within dilated intrahepatic
ductal segments
• ERCP
o Ampullary stenosis with CBD dilation, CBD
ulcerations, mult intrahepatic strictures on ERCP

,-
DDx: Spectrum of Biliary lesions Mimicking AIDS Cholangitis

( ..
i'"
I I

... : .1 Ampullary Stenosis Cholangiocarcinoma

"
 AIDS CHOLANGIOPATHY

Key Facts
Terminology • us protocol: Parasagittal view of CBD to demonstrate
• Spectrum of biliary inflammatory lesions caused by mural thickening
AIDS-related opportunistic infections leading to • MRC protocol: Axial and coronal SSFSE;obliques,
biliary stricture/obstruction or cholecystitis heavily weighted T2WI

Imaging Findings Pathology

• Best diagnostic clue: AIDSpatient with multiple
intrahepatic strictures, ampullary stenosis, or
gallbladder (GB) wall thickening from cholecystitis
• Ampullary stenosis with CBD dilation, CBD
ulcerations, mult intrahepatic strictures on ERCP
• High signal dilated ducts; intra- and extrabiliary
strictures on MRCP
• Diffuse GB thickening; peri ductal hyper- or
hypoechoic areas; dilated intrahepatic ducts; diffuse
CBD thickening on US
• General path comments: Opportunistic infection of
2
GB, bile ducts from cryptosporidium & CMV; 23
periductal inflammation, acalculous cholecystitis
Clinicallss,ues
• Poor prognosis due to advanced AIDS presentation
Diagnostic Checklist
• AIDS patient with distal ampullary stenosis,
intrahepatic strictures or acalculous cholecystitis

• Associated with primary sclerosing cholangitis (PSC),

Imaging Recommendations recurrent pyogenic cholangitis (RPC), choledochal cyst
• Best imaging tool • Infiltrates along ductal epithelium, invades hepatic
o US parenchyma
o MRCP • Delayed CECT useful to demonstrate intrahepatic
o ERCP component
• Protocol advice
o US protocol: Parasagittal view of CBD to
demonstrate mural thickening I PATHOLOGY
o MRC protocol: Axial and coronal SSFSE;obliques,
heavily weighted T2WI General Features
• General path comments: Opportunistic infection of
GB, bile ducts from cryptosporidium & CMV;
I DIFFERENTIAL DIAGNOSIS periductal inflammation, acalculous cholecystitis
• Genetics: No known predisposition
Sclerosing cholangitis • Etiology
• Beading of CBD o AIDS-related opportunistic infection of biliary tract
• Pseudodiverticula of CBD o Cryptosporidium, cytomegalovirus (CMV) most
• Multiple intrahepatic strictures with asymmetric common pathologies
involvement o CMV has been implicated in vasculitis of central
• Long segment stricture of CBD nervous system (CNS), retinal or gastrointestinal
• Involvement of cystic duct characteristic (GI) tract
Ampullary stenosis • Biliary strictures may be secondary to vasculitis
associated with CMV
• Distal CBD fibrous stricture not associated with
• Epidemiology: Late stage AIDS patients (CDC stage IV
infection
AIDS based on T4 counts)
• May result from passage of CBD stones or chronic
• Associated abnormalities
pancreatitis
o Other AIDS-related GI infections
• Smooth stricture not associated with ulceration or
• Enteritis and colitis from CMV, cryptosporidium,
irregularities
other opportunistic infections
Acalculous cholecystitis • MAl causing ileo-cecal inflammation and necrotic
• GB wall thickened by sterile inflammation low attenuation mesenteric nodes
• Not associated with AIDS or opportunistic infection • Hepatic and splenic microabscesses from fungal
• Often secondary to ischemic injury from low flow infections (Candida, cryptococcus)
state o AIDS-related malignancies
o Post-cardiac surgery • Non-Hodgkin lymphoma of liver, stomach,
• Percutaneous cholecystomy for poor operative risk spleen, mesentery or retroperitoneal nodes
patients • Kaposi sarcoma of retroperitoneal nodes
• Late in clinical course Gross Pathologic & Surgical Features
o Secondary to bacterial invasion
• Acalculous cholecystitis, biliary strictures involving
Cholangiocarcinoma CBD, intrahepatic duct
• Progressive biliary obstruction • Biliary strictures involving CBD, intrahepatic duct
 AIDSCHOl.ANGIOPATHY
Microscopic Features I SELECTED REFERENCES
• CMV inclusions, cryptosporidium organisms may be 1. Ko WF et al: Prognostic factors for the survival of patients
found on biopsy with AIDScholangiopathy. Am] Gastroenterol.
• Fibrotic strictures of CBD, intrahepatic ducts 98(10):2176-81,2003
2. Enns R: AIDScholangiopathy: "an endangered disease". Am
Staging, Grading or Classification Criteria ] Gastroenterol. 98(10):2111-2, 2003
3. Chen XM et al: Cryptosporidiosis and the pathogenesis of
2 • Type I: Distal CBD stricture from ampullary stenosis
o 15-20% of patients AIDS-cholangiopathy. Semin Liver Dis. 22(3):277-89, 2002
4. Kumar KSet al: Isolated intrahepatic biliary dilatation in a
24 • Type II: Diffuse intrahepatic biliary strictures patient with acquired immune deficiency syndrome
o 20% of patients (AIDS):AIDScholangiopathy versus incidental unilobar
• Type III: Combined types I, II Caroli's disease.] Clin Gastroenterol. 32(1):79-81, 2001
o 50% of patients 5. Mukhopadhyay S et al: AIDScholangiopathy as initial
• Type IV: Long segment stricture of CBD with possible presentation of HIV infection. Trop Gastroenterol.
ulceration 22(1):29-30, 2001
o 15% of patients 6. Mahajani RV et al: Cholangiopathy in HIV-infected
• Type V: Acalculous cholecystitis, GB wall thickening, patients. Clin Liver Dis. 3(3):669-84, x, 1999
7. Cello ]P: AIDS-Related biliary tract disease. Gastrointest
pericholecystic inflammation
Endosc Clin N Am. 8(4):963, 1998
8. Kumar M et al: AIDS associated cholangiopathy. Trop

I CLINICAL ISSUES 9.
Gastroenterol. 19(4):155-6, 1998
Boige N et al: Hydrops-like cholecystitis due to
cryptosporidiosis in an HIV-infected child. ] Pediatr
Presentation Gastroenterol Nutr. 26(2):219-21, 1998
• Most common signs/symptoms 10. Wilcox CM et al: Hepatobiliary diseases in patients with
o Fever AIDS:focus on AIDS cholangiopathy and gallbladder
o RUQpain disease. Dig Dis. 16(4):205-13, 1998
11. Misra A et al: AIDScholangiopathy. Indian] Gastroenterol.
o Jaundice
17(3):104-5, 1998
• Clinical profile 12. Keaveny AP et al: Hepatobiliary and pancreatic infections
o Elevated alkaline phosphatase in AIDS:Part II. AIDS Patient Care STDS. 12(6):451-6, 1998
o Cryptosporidium in stool or duodenum 13. Castiella A et al: AIDS-associated cholangiopathy in a series
of ten patients. Rev Esp Enferm Dig. 90(6):419-30, 1998
Demographics 14. Keaveny AP et al: Hepatobiliary and pancreatic infections
• Age: > 20 yrs in AIDS:Part one. AIDSPatient Care STDS. 12(5):347-57,
• Gender: M < F 1998
• Ethnicity 15. Fulcher ASet al: Magnetic resonance
o Parallel demographics for AIDS patients cholangiopancreatography: a new technique for evaluating
• Incidence has decreased substantially with newer the biliary tract and pancreatic duct. Gastroenterologist.
6(1):82-7, 1998
anti-retroviral therapy
16. Lefkowitch ]H: The liver in AIDS. Semin Liver Dis.
Natural History & Prognosis 17(4):335-44,1997
17. Liberman E et al: Foamy macrophages in acquired
• Sphincterotomy provides some pain relief immunodeficiency syndrome cholangiopathy with
o Doesn't alter intrahepatic disease Encephalitozoon intestinalis. Arch Pathol Lab Med.
• Anti-CMV therapy not effective 121(9):985-8, 1997
• Poor prognosis due to advanced AIDS presentation 18. Castiella A et al: Ursodeoxycholic acid in the treatment of
AIDS-associated cholangiopathy. Am] Med. 103(2):170-1,
Treatment 1997
• Options, risks, complications 19. Daly CA et al: Sonographic prediction of a normal or
o Asymptomatic abnormal ERCPin suspected AIDSrelated sclerosing
• Observation cholangitis. Clin Radiol. 51(9):618-21, 1996
o Symptomatic 20. Cacciarelli AG et al: Biliary fistula in a patient with HIV
cholangiopathy. Gastrointest Endosc. 44(3):345-8, 1996
• Sphincterotomy for pain relief
21. Willson R et al: Human immunodeficiency virus
I-associated necrotizing cholangitis caused by infection
with Septata intestinalis. Gastroenterology. 108(1):247-51,
I DIAGNOSTIC CHECKLIST 1995
22. Cello]P et al: Long-term follow-up of endoscopic
Consider retrograde cholangiopancreatography sphincterotomy for
• Sclerosing cholangitis patients with acquired immune deficiency syndrome
papillary stenosis. Am] Med. 99(6):600-3, 1995
Image Interpretation Pearls 23. Carmody E et al: Cytomegalovirus cholangitis after renal
• AIDS patient with distal ampullary stenosis, transplantation. Can Assoc Radiol]. 45(6):473-5, 1994
intrahepatic strictures or acalculous cholecystitis 24. Chung C] et al: Hepatobiliary abnormalities on sonography
in children with HIV infection. ] Ultrasound Med.
13(3):205-10, 1994
 AIDS CHOLANGIOPATHY
I IMAGE GALLERY

(Left) Sagittal US of common

bile duct demonstrates
irregular thickening (arrow)
from AIDS cholangitis.
2
(Right) Axial US of common
bile duct shows irregular 25
thickening (arrow) from
AIDS cholangitis.

Typical
(Left) Sagittal US
demonstrates diffuse
gallbladder wall thickening
(arrow) in CMVacalculous
cholecystitis. (Right) Axial
CECT demonstrates
gallbladder wall edema and
pericholecystic inflammatory
changes (arrow) in CMV
acalculous cholecystitis.

Typical
(Left) Sagittal US of distal
common bile duct
demonstrating focal mural
thickening (arrow) in
ampullary stenosis from
cryptosporidium in AIDS
cholangitis. (Right) ERCP of
patient with ampullary
stenosis from
cryptosporidium in AIDS
cholangitis demonstrates
distal common bile duct
stricture (arrow).
 CHOLEDOCHOLITHIASIS

2
26

Coronal graphic shows multiple small nonobstructive MRCP shows multiple hypointense calculi (arrows)
stones in distal CBO and gallbladder. within a dilated CBO.

o Secondary duct stones due to passage of gallstones

ITERMINOLOGY into CBD
Abbreviations and Synonyms • Most common duct stones in Western countries
• Cholangiolithiasis or biliary calculi • Accounts 95% of CBD stones in USA
• Within CBD anywhere between porta hepatis &
Definitions ampulla of Vater
• Intra- &/or extrahepatic stones or calculi • Major composition: Cholesterol 70-80%; pigment
20-30% in Western countries & calcium
bilirubinate in Eastern countries
IIMAGING FINDINGS • 15% of gallstone patients also have CBD stones
• 95% of patients with CBD stones have or have
General Features had gallstones
• Best diagnostic clue: MRC: Discrete low signal filling • 15-25% acute calculous cholecystitis patients have
defects within bile ducts CBD stones
• Location: Intra- & extrahepatic bile ducts (more • 12-15% who undergo cholecystectomy have
common in CBD) choledocholithiasis
• Size: Varies from 1-15 mm
• Morphology Radiographic Findings
o Most frequent cause of biliary obstruction without • ERCP (both diagnostic & therapeutic)
ductal dilatation o Opacification of both extra- & intrahepatic duct
o Classified into two types based on etiology system + pancreatic duct
• Primary duct stones o Stones: Seen as radiolucent filling defects
• Secondary duct stones • Intraoperative & postoperative (T tube)
o Primary duct stones: Form within bile ducts cholangiography
• Accounts only 5% of CBD stones in USA o Direct tests for detection of CBD stones
• Major composition: Pigment o A meniscus of contrast material clearly outlines
• Far less common than secondary stones in margins of stones
Western countries

DDx: Obstrucion of Common Bile Duct

Pancreatic Carcinoma Chronic Pancreatitis Cholangiocarcinoma Papillary Stenosis

 CHOLEDOCHOLITHIASIS
Key Facts
Terminology
• Intra- &/or extrahepatic stones or calculi
Imaging Findings
• Best diagnostic clue: MRC: Discrete low signal filling
defects within bile ducts
• "Bull's eye" sign: Rim of bile surrounding a stone
within duct
• Thin meniscus of water density bile around stone
posteriorly
Top Differential Diagnoses
• Pancreatic or ampullary cancer
• Chronic pancreatitis
• Cholangiocarcinoma
• Papillary stenosis or dysfunction
• Primary sclerosing cholangitis (PSC)
CT Findings
• NECT
o Attenuation of calculi varies from less than water
density, through soft tissue, to dense calcification
o Mixed stones: Predominantly cholesterol & calcium
bilirubinate (usually calcified rim or central nidus)
• Increased attenuation (75-85% stones due to
sufficient Ca++ bilirubinate)
• "Bull's eye" sign: Rim of bile surrounding a stone
within duct
• Most accurate sign (60-80% sensitivity) for bile
duct stones on NECT
• Thin meniscus of water density bile around stone
posteriorly
• Some stones are isodense to soft tissue
o Pure cholesterol stones are rare
• Isodense with bile (indistinguishable)
o Abrupt termination of CBD (complete obstruction
by a large stone)
• Stone isodense to bile or pancreas (DDx:
Malignant stricture & carcinoma of ampulla)
• Less accurate than "bull's eye" sign
o CBD &/or IHBD dilatation: Varies based on
• Stone size, degree & duration of obstruction
• Water density tubular branching structures
MR Findings
• MRC (MR cholangiography)
o Bile: Very bright signal
o Ductal stones: Decreased signal intensity foci
o Low-signal filling defects within increased signal
intensity bile
Ultrasonographic Findings
• Real Time
o Echogenic focus with posterior acoustic shadowing
• Stone within CBD
o 10% stones: No acoustic shadow
• Small size, soft & porous composition
• DDx: Intraductal clot, infection, sludge ball,
tumor, parasite
o CBD/intrahepatic bile duct dilatation (IHBD) based
on stone size, degree & duration of obstruction
Pathology
• Obstruction, dilatation, sclerosis, stricture
• Bile stasis/infection: Bilirubinate stone formation
• Primary duct stones (5%): Form within bile ducts
• Secondary duct stones (95%): Gallstones into CBD
• Approximately 25 million Americans have gallstones 2
• Associated abnormalities: Gall stones
27
Clinical Issues
• Acute RUQ pain, pruritus, jaundice, pancreatitis
• Increased alkaline phosphatase & direct bilirubin
• Gender: Females (middle age) more than males
Diagnostic Checklist
• Rule out other causes of "CBD obstruction"
• MRC & ERCP: Discrete filling defects or obstruction &
prestenotic dilatation of CBD/intrahepatic bile ducts
• CBD: 4-6 mm (normal size); 6-7 mm (equivocal);
more than 8 mm (dilatation)
• Common hepatic duct: 4-5 mm (normal size)
• IHBD: 1-2 mm (usually not visible)
Nuclear Medicine Findings
• Hepato-biliary scan (HIDA)
o Diagnose early, low grade obstruction
o Stones with intermittent obstruction
• Retention & delayed passage of isotope
Imaging Recommendations
• Best imaging tool
o ERCP: Gold standard for detection of CBD stones in
the absence of T tube
o MRC: Sensitivity: 81-100%; specificity: 85-100%
• Protocol advice
o MRC: Using two techniques
• RARE:Single-slab rapid acquisition with relaxation
enhancement
• HASTE:Multislice half-Fourier acquisition
single-shot turbo spin-echo
• Helical NECT: Using thin sections (:0:: 5 mm)
o Sensitivity: 75-85%
• Ultrasonography: High frequency transducer (5 MHz)
sensitivity 50-60%
o Proximal CBD: Parasagittal scan in supine/left
posterior oblique position
o Distal CBD
• Semierect (approx. 60° to vertical)
• Right posterior oblique (45°) position in transverse
plane provides a good acoustic window
o If gas obscures; have patient drink 6-12 oz of water
• Keep patient in right decubitus position for 2-3
minutes & rescan in semierect position
o Postcholecystectomy patients with persistent RUQ
pain: CBD imaged
• After a fast & 45 mins to 1 hr after a fatty meal
• CBD dilates more than 2 mm above baseline in
partial stone obstruction
 CHOLEDOCHOLITHIASIS
I DIFFERENTIAL DIAGNOSIS
Pancreatic or ampullary cancer
• Hypodense mass in head of pancreas or ampulla
• "Double duct" sign
o Obstruction & dilatation of pancreatic duct/CBD
2 • Heterogeneous poorly enhancing mass
• Contiguous organ invasion may be seen
28 o Duodenum, stomach & mesenteric root
• Duodenal distention with water: Helpful for
visualization with CT
Chronic pancreatitis
• Focal or diffuse atrophy of gland; enlarged head
• Dilated main pancreatic duct with ductal calculi
• Distal CBD long stricture causes prestenotic dilatation
• Thickening of peripancreatic fascia & fat necrosis
Cholangiocarcinoma
• Extrahepatic: CBD growth (stricture or polypoid mass)
• Obstruction & dilatation of CBD/IHBD
• ERCP: Depicts stricture or intraductal tumor mass
Papillary stenosis or dysfunction
• Causes dilatation of CBD & intrahepatic bile ducts
• No mass or filling defect
Primary sclerosing cholangitis (PSC)
• Idiopathic or autoimmune reaction or genetic
• CBD always involved; IHBD & extrahepatic (68-89%)
• ERCP: Classic "beaded appearance"
o Alternating segments of dilatation & focal strictures
I PATHOLOGY
General Features
• General path comments
o Mechanism of stones in CBD & IHBD
• Obstruction, dilatation, sclerosis, stricture
• Bile stasis/infection: Bilirubinate stone formation
• Infection: E. coli, Klebsiella & other gram negative
organisms with S-glucuronidase activity
• Etiology
o Primary duct stones (5%): Form within bile ducts
• Chronic hemolytic disease, recurrent cholangitis
• Congenital anomalies of bile ducts (e.g., Caroli
disease)
• Motor disorder of sphincter of Oddi
• Low fat & protein diet, foreign body (suture
material)
• Parasites: Clonorchis sinensis & ascaris (major
causes in Asia)
o Secondary duct stones (95%): Gallstones into CBD
• Obesity, Crohn disease & ileal resection
• Hemolytic anemias (sickle cell anemia &
hereditary spherocytosis)
• Increased triglycerides, hyperalimentation, Native
American heritage
• Epidemiology
o Approximately 25 million Americans have gallstones
o Secondary duct stones
• Accounts for 95% of choledocholithiasis in USA
• Associated abnormalities: Gall stones
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute RUQ pain, pruritus, jaundice, pancreatitis
o Small stones spontaneously pass with/without pain
• Clinical profile: Fat, fertile, forty year old female with
history of acute or intermittent RUQ pain & jaundice
• Lab data
o Increased alkaline phosphatase & direct bilirubin
o Late phase: Increased AST & ALT levels
• Diagnosis
o Cholangiography: MRC, ERCP or
intra-operative/post-operative T tube
Demographics
• Age: Usually adults, can be seen in any age group
• Gender: Females (middle age) more than males
Natural History & Prognosis
• Complications: Cholangitis, obstructive jaundice,
pancreatitis, secondary biliary cirrhosis
• Prognosis
o Cholecystectomy patients
• Undetected duct stones left behind in 1-5% cases
Treatment
• Stones < 3 mm: Usually spontaneously pass
• Stones 3-10 mm: Endoscopic sphincterotomy
o Stone retrieval balloon to sweep duct
o Basket to snare stones
• Stones more than 10-15 mm
o Require fragmentation by mechanical lithotripsy
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of "CBD obstruction"
Image Interpretation Pearls
• MRC & ERCP: Discrete filling defects or obstruction &
prestenotic dilatation of CBD/intrahepatic bile ducts
I SELECTED REFERENCES
1. Kim TK et al: Diagnosis of intrahepatic stones: superiority
of MR cholangiopancreatography over endoscopic
retrograde cholangiopancreatography. AjR Am J
Roentgenol. 179(2):429-34, 2002
2. Soto JA et al: Detection of choledocholithiasis with MR
cholangiography: comparison of three-dimensional fast
spin-echo and single- and multisection half-Fourier rapid
acquisition with relaxation enhancement sequences.
Radiology. 215(3):737-45, 2000
3. Vitellas KM et al: MR cholangiopancreatography of bile
and pancreatic duct abnormalities with emphasis on the
single-shot fast spin-echo technique. RadioGraphies. 20:
939-957, 2000
4. Fulcher AS et al: MR cholangiography: technical advances
and clinical applications. Radiographies. 19(1):25-41;
discussion 41-4, 1999
 CHOLEDOCHOLITHIASIS
I IMAGE GALLERY

(Left) ERCP shows multiple

calculi (filling defects) within
cystic and common bile
ducts. (Right) ERCP
2
following endoscopic
papillotomy shows a wire 29
basket being used to
fragment, snare and extract
biliary calculi.

Typical
(Left) Axial CECT shows
calcific density within the
dependent portion of the
gallbladder. Dilated CBD
(arrow) from obstructing
biliary calculus. (Right) Axial
CECT shows soft tissue
density stone (arrow)
causing abrupt obstruction of
CBD. Note thin meniscus of
bile around stone, within
CBD.

(Left) Oblique sagittal

sonogram shows dilated
CBD (arrow) and obstructing
stone (open arrow). Note
acoustic shadow behind
stone. (Right) Axial CECT
following attempted ERCP
extraction of CBD stone. The
stone (arrow) remains within
the distal CBD. Extraluminal
gas in retroperitoneum and
peritoneal cavity due to
duodenal perforation.
 MIRIZZISYNDROME

2
30

Mirizzi syndrome. Graphic depicts large cystic duct ERCP of Mirizzi syndrome. Note smooth extrinsicmass
stone (arrow) causing dilatation of common hepatic effect by a stone compressing common hepatic duct
duct. and non-fillingof gallbladder (arrow).

o High signal dilated intrahepatic bile ducts

ITERMINOlOGY o Low signal filling defects representing gallstones in
Definitions GB, cystic duct
• Partial or complete obstruction of common hepatic • T1 C+: Low signal dilated intrahepatic bile ducts,
duct (CHD) due to gallstone impacted in cystic duct or gallstones in cystic duct, normal distal CBD
infundibulum of gallbladder (GB) • MRCP
o Dilated intrahepatic ducts, filling defect in CHD
Ultrasonographic Findings
I IMAGING FINDINGS • Real Time: Gallstone impacted in cystic duct; dilated
General Features intrahepatic ducts on US
• Best diagnostic clue: Impacted cystic duct stone on US Nuclear Medicine Findings
with proximal dilatation of intrahepatic duct • Hepato Biliary Scan
• Location: Gallstone in cystic duct o Non-filling GB, dilated intrahepatic ducts
• Size: Gallstones typically 1-3 cm
• Morphology: Smooth extrinsic filling defect on ERCP Imaging Recommendations
at level of CHD • Best imaging tool: US, CECT, ERCP
• Protocol advice: Color Doppler to detect dilated
Radiographic Findings intrahepatic bile ducts
• ERCP:Extrinsic narrowing of CHD; dilated
intrahepatic ducts; lack of GB filling
CT Findings I DIFFERENTIAL DIAGNOSIS
• CECT: Dilated intrahepatic bile ducts; stone in neck of Cystic duct stones
GB; normal distal CBD; acute/chronic cholecystitis
• No dilation of intrahepatic ducts
with pericholecystic inflammatory changes • Biliary colic or acute cholecystitis
MR Findings Common hepatic obstruction from nodes
• T2WI • Porta hepatis nodes obstructing dilated ducts

DDx: LesionsMimicking Mirizzi Syndrome

Cystic Duct Stones Porta Hepatis Node Cholangiocarcinoma

 MIRIZZI SYNDROME
Key Facts
Terminology
• Partial or complete obstruction of common hepatic
duct (CHD) due to gallstone impacted in cystic duct
or infundibulum of gallbladder (GB)
Imaging Findings
• Best diagnostic clue: Impacted cystic duct stone on
US with proximal dilatation of intrahepatic duct
• ERCP: Extrinsic narrowing of CHD; dilated
intrahepatic ducts; lack of GB filling
• CECT: Dilated intrahepatic bile ducts; stone in neck
of GB; normal distal CBD; acute/chronic cholecystitis
with pericholecystic inflammatory changes
• Real Time: Gallstone impacted in cystic duct; dilated
intrahepatic ducts on US
2
31
Diagnostic Checklist
• Porta hepatis obstruction from nodes

• Hypoechoic masses without shadowing

I DIAGNOSTIC CHECKLIST
Cholangiocarcinoma
• Soft tissue mass porta hepatis obstructing bile ducts Consider
• Dilated intrahepatic ducts • Porta hepatis obstruction from nodes
Image Interpretation Pearls
• Stone impacted in cystic duct with dilated ducts
I PATHOLOGY
General Features
• General path comments
I SELECTED REFERENCES
o Acute/chronic cholecystitis 1. Abou-Saif A et al: Complications of gallstone disease:
o May have cholecysto-choledochal fistula Mirizzi syndrome, cholecystocholedochal fistula, and
o Repeated episodes of cholangitis lead to biliary gallstone ileus. Am J Gastroenterol. 97(2):249-54, 2002
2. Haritopoulos KN et al: Mirizzi syndrome: a case report and
cirrhosis
review of the literature. Int Surg. 87(2):65-8, 2002
• Etiology 3. Gomez G: Mirizzi Syndrome. CUff Treat Options
o Cystic duct anatomically oriented parallel to CHD Gastroenterol. 5(2):95-99, 2002
o Impaction of stone in cystic duct or GB neck 4. Xiaodong H et al: Diagnosis and treatment of the Mirizzi
o Partial or complete obstruction of CHD syndrome. Chin Med Sci]. 14(4):246-8, 1999
• Epidemiology: Tracks prevalence of gallstones
Gross Pathologic & Surgical Features I IMAGE GAllERY
• Acute/chronic cholecystitis
• May have cholecysto-choledochal fistula
Staging, Grading or Classification Criteria
• Type I: Extrinsic compression of CHD due to cystic
duct stone
• Type II: Cholecystobiliary fistula < 1/3 circumference
of ductal wall
• Type III: Cholecystobiliary fistula with 2/3 of ductal
wall involvement
t; • Type IV: Cholecystobiliary fistula with entire ductal
: wall circumference involvement

(Left) Sagittal US of neck of gallbladder demonstrates impacted stone

I CLINICAL ISSUES (arrow) with dilation of common hepatic duct (open arrow). (Right)
Sagittal US of right lobe of liver. Note dilated intrahepatic duct
Presentation (arrow).
• Most common signs/symptoms: Fever, jaundice, RUQ
pain
Demographics
• Age: Adults> 40 yrs
• Gender: M:F = 1:2
Natural History & Prognosis
• Jaundice & cholangitis if not treated promptly
Treatment
• Cholecystectomy with careful dissection of cystic duct
to avoid injury to CHD
 HYPERPLASTICCHOLECYSTOSES

2
32

Schematic drawing of adenomyomatosis. Note Transverse sonogram of adenomyomatosis. Note

thickened gallbladder wall with multiple intramural thickened gallbladder wall (open arrow) and multiple
cystic spaces (arrow). echogenic foci with "comet tail" reverberation artifacts
(arrow).

• Location: GB wall
ITERMINOLOGY • Size: Polyps typically 5-10 mm
Abbreviations and Synonyms Radiographic Findings
• Cholesterolosis; strawberry gallbladder (GB), • Radiography: OCG: "Pearl necklace" GB with multiple
cholesterol polyp; adenomyomatosis contrast-filled intramural diverticula
Definitions CT Findings
• General: Idiopathic non-neoplastic & • CECT: Thickened GB wall (segmental or diffuse); often
non-inflammatory proliferative disorder resulting in brisk enhancement of wall post-contrast; cystic
GB wall thickening
nonenhancing spaces within GB wall corresponding to
• Adenomyomatosis: Mural GB wall thickening 2° to intramural diverticula
exaggeration of normal luminal epithelial folds
(Rokitansky-Aschoff sinuses) in conjunction with MR Findings
smooth muscle proliferation • T2WI: High signal cystic spaces within thickened GB
• Cholesterolosis: Deposition of foamy cholesterol-laden wall
histiocytes in subepithelium of GB; numerous small • T1 C+: Nonenhancing cystic spaces within thickened
accumulations (strawberry GB) or larger polypoid GB wall
deposit (cholesterol polyp)
Ultrasonographic Findings
• Real Time
I IMAGING FINDINGS o Adenomyomatosis: Focal or diffuse GB wall
thickening; intramural high amplitude echoes with
General Features "comet tail" artifacts
• Best diagnostic clue o Cholesterolosis: Multiple small « 10 mm) polyps
o Adenomyomatosis: Fundal, diffuse or mid-body GB • Color Doppler: Avascular or hypervascular areas
wall thickening with intramural high amplitude
echoes & "comet tail" reverberation artifacts
Imaging Recommendations
o Cholesterolosis: Multiple GB polyps • Best imaging tool: US, CECT

DDx: Spectrum of GB Disease Mimicking Cholecystoses

Cholecystitis Gallbladder CA GB Polyps

 HYPERPLASTIC CHOLECYSTOSES

Key Facts
Imaging Findings •. Best imaging tool: US, CECT
• Adenomyomatosis: Fundal, diffuse or mid-body GB Clinic::allssLJes
wall thickening with intramural high amplitude
• Mostcommon signs/symptoms: Most often
echoes & "comet tail" reverberation artifacts
asymptomatic, but may present with RUQ pain
• Cholesterolosis: Multiple GBpolyps
• CECT: Thickened GB wall (segmental or diffuse); Diag~o~ticChecklist 2
often brisk enhancement of wall post-contrast; cystic • Consider chronic cholecystitis>
nonenhancing spaces within GB wall corresponding 33
• "Comet tail" reverberation artifacts within thickened
to intramural diverticula wall

I DIFFERENTIAL DIAGNOSIS Treatment

• Options, risks, complications: Cholecystectomy only if
Chronic:: cholecystitis
symptomatic
• Generalized wall thickening; gallstones; no mural
"comet tail" artifact
GB carcinoma I DIAGNOSTIC CHECKLIST
• Polypoid mass> 2 cm; diffuse asymmetric GB wall
Consider
thickening; hepatic invasion
• Consider chronic cholecystitis
Adenomatous polyp
Image Interpretation Pearls
• Polypoid mass 5-15 mm
• "Comet tail" reverberation artifacts within thickened
wall
I PATHOLOGY
General Features I SELECTED REFERENCES
• General path comments: Adenomyomatosis: Diffuse or 1. Owen CC et al: Gallbladder polyps, cholesterolosis,
segmental GB wall thickening with multiple cystic adenomyomatosis, and acute acalculous cholecystitis.
spaces Semin Gastrointest Dis. 14(4):178-88,2003
2. Ghersin E et al: Twinkling artifact in gallbladder
• Etiology: Idiopathic
adenomyomatosis. J Ultrasound Med. 22(2):229-31, 2003
• Epidemiology: Occurs in 5-25% of resected GB patients
3. Yoshimitsu K et al: Radiologic diagnosis of
• Associated abnormalities: Gallstones in 25-75% of adenomyomatosis of the gallbladder: comparative study
patients among MRI, helical CT, and transabdominal US. J Com put
Assist Tomogr. 25(6):843-50, 2001
Gross Pathologic::& Surgic::alFeatures
• Focal or diffuse GB wall thickening without
inflammatory changes
I IMAGE GALLERY
Microscopic:: Features
• Adenomyomatosis
o Mural thickening 2° to smooth muscle proliferation
& exaggerated folds of Rokitansky-Aschoff sinuses
• Cholesterolosis
o Subepithelium deposition of cholesterol-laden
histiocytes with villus-like mucosal protrusions; may
coalesce into polyps

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Most often (Left) Sagittal sonogram of segmental adenomyomatosis demonstrates
asymptomatic, but may present with RUQ pain focal gallbladder wall thickening in mid-body ("hourglass deformity"
- arrow). Note gallstones (open arrow). (Right) ERe? demonstrates
Demographic::s focal narrowing ("hourglass deformity" - arrow), multiple intramural
diverticula (open arrow), and gallstones (curved arrow).
• Age: > 35 yrs
• Gender: M:F = 1:3
Natural History & Prognosis
• Usually incidental finding; little clinical importance
 CHOI.EC¥STITIS

2
34

Transverse color Doppler sonogram demonstrates Acute cholecystitis on biliary scintigram. Note isotope
marked hyperemia (arrow) and gallbladder wall filling common bile duct (arrow) and small bowel (open
thickening from acute cholecystitis, arrow) without filling of gallbladder.

o May document common bile duct (CBD) stones in

ITERMINOlOGY patients with associated cholangitis
Abbreviations and Synonyms o No filling of gallbladder
• Acute calculous cholecystitis, acute acalculous CT Findings
cholecystitis
• NECT
Definitions o Distended GB
• Acute inflammation of gallbladder (GB), 95% 2° to o Edematous pericholecystic fat with stranding
calculus obstructing cystic duct; acalculous in 5% 2° to o Calcified gallstones (15%)
ischemia, secondary inflammation/infection • CECT
o Uncomplicated cholecystitis
• GB wall thickening
I IMAGING FINDINGS • Increased mural enhancement
• Pericholecystic fat stranding
General Features • Cholesterol stones typically not visible
• Best diagnostic clue o Complicated cholecystitis
o Impacted gallstone in cystic duct • Intramural or pericholecystic abscesses leading to
o Positive sonographic Murphy sign asymmetric GB wall thickening
o Gallbladder wall thickening • Gas in lumen and/or wall of gallbladder
• Location: Stone impacted in GB neck or cystic duct • High attenuation gallbladder hemorrhage
• Size: Distended GB (> 5 cm transverse diameter) • Focal interruption of GB wall due to necrosis
• Morphology: Distended GB more rounded in shape • Adherent omentum
than normal "pear-shaped" configuration MR Findings
Radiographic Findings • T2WI
• Radiography: Calcified stones in only 15-20% of o Distended GB with stones
patients with cholecystitis o High signal pericholecystic fat
• ERCP • T1 C+

DDx: Spectrum of Diseases Mimicking Cholecystitis

--
lI;~j
---- ...•

'. A·
\.; -.
.~'" ,

~
'
•...
..
'

'

'.

'- y
Pancreatitis Diverticulitis
- .
Liver Abscess
 CHOLECYSTITIS

Key Facts
Terminology • Protocol advice: Longitudinal and transverse images
• Acute inflammation of gallbladder (GB), 95% 2° to of GB, para sagittal images of GB neck region & cystic
calculus obstructing cystic duct; acalculous in 5% 2° duct in LPO position to detect impacted gallstones
to ischemia, secondary inflammation/infection (I.e., immobile)

Imaging Findings Clinical Issues 2

• Uncomplicated: Gallstones; positive sonographic • Most common signs/symptoms: Acute RUQ pain,
Murphy sign; gallstone impacted in neck of GB or fever 35
cystic duct; thickened GB wall (> 4 cm) on US • May progress to gangrenous cholecystitis and
• Complicated: Gallstones; pericholecystic perforation if untreated
fluid/abscess; intraluminal membranes; gas in GB • Excellent prognosis in uncomplicated cases or with
wall/lumen; sonographic Murphy sign absent in 1/3 prompt surgery
of patients; asymmetric wall thickening on US Diagnostic Checklist
• Best imaging tool: US or biliary scintigraphy • Perforated ulcer or pancreatitis with secondary GB
wall thickening

o "Rim sign" of increased hepatic enhancement in

patient with gangrenous cholecystitis Acute pancreatitis
o Focal interruption of enhancement • Enlarged pancreas
• Peripancreatic fluid or inflammatory changes
Ultrasonographic Findings • Nonenhancing areas of necrosis
• Real Time
o Uncomplicated: Gallstones; positive sonographic Hepatic flexure diverticulitis
Murphy sign; gallstone impacted in neck of GB or • Colonic diverticula
cystic duct; thickened GB wall (> 4 cm) on US • Pericolonic inflammation
o Complicated: Gallstones; pericholecystic • Fecalith
fluid/abscess; intraluminal membranes; gas in GB liver abscess
wall/lumen; sonographic Murphy sign absent in 1/3
• CECT
of patients; asymmetric wall thickening on US o "Cluster sign" of multiloculated pyogenic abscesses
Nuclear Medicine Findings o Air-fluid level from gas-forming organism
• Hepato Biliary Scan • US: Irregular, hypo echoic mass with little
o 99m Tc iminodiacetic acid derivatives enhancement through transmission
o Non-visualization of GB at 4 hours has 99%
specificity
o Increased uptake in gallbladder fossa during arterial I PATHOLOGY
phase due to hyperemia in 80% of patients
General Features
o "Rim sign" seen in 34% of patients is due to
• General path comments
increased uptake in gallbladder fossa
o Distended GB
• Positive predictive value of 57% for gangrenous
o Thickened, inflamed GB wall
cholecystitis
o Pericholecystic adhesions to omentum
Imaging Recommendations • Genetics
• Best imaging tool: US or biliary scintigraphy o Increased incidence of gallstones in selected
• Protocol advice: Longitudinal and transverse images of population
GB, parasagittal images of GB neck region & cystic • Hispanics, Pima Indians
duct in LPO position to detect impacted gallstones • Etiology
(I.e., immobile) o 95% calculous
• Obstructing stone in cystic duct
o 5% acalculous
I DIFFERENTIAL DIAGNOSIS • Ischemia with secondary inflammation/infection
• AIDS patients have opportunistic GB infection
Peptic ulcer disease (PUD) • Epidemiology
• Thickened duodenum o Incidence parallels prevalence of gallstones
• Ectopic gas if perforated ulcer • 25 million Americans have gallstones
• Periduodenal inflammatory changes in anterior • M:F = 1:3
pararenal space
• Secondary GB wall thickening
Gross Pathologic & Surgical Features
• Gallstones in gallbladder neck or cystic duct
• Thickened GB wall with hyperemia of wall
 CHOlECYSTITIS
• Omental adhesions I SELECTED REFERENCES
Microscopic Features 1. Yusoff IF et al: Diagnosis and management of cholecystitis
• Lumen: Gallstones, sludge and cholangitis. Gastroenterol Clin North Am.
• GB mucosa: Ulcerations 32(4):1145-68,2003
• GB wall: Acute polymorphonuclear (PMN) infiltration 2. Bennett GL et al: Ultrasound and CT evaluation of
emergent gallbladder pathology. Radiol Clin North Am.
• Bacterial cultures positive in 40-70% of patients
2 Staging, Grading or Classification Criteria 3.
41(6):1203-16,2003
Browning JD et al: Gallstone disease and its complications.
Semin Gastrointest Dis. 14(4):165-77,2003
36 • Non-perforated
4. Barie PS et al: Acute acalculous cholecystitis. Curr
o GB wall intact on CT and/or US Gastroenterol Rep. 5(4):302-9, 2003
• Gangrenous 5. Pazzi P et al: Biliary sludge: the sluggish gallbladder. Dig
o US: Perich ole cystic fluid, intraluminal membranes, Liver Dis. 35 Suppl 3:S39-45, 2003
asymmetric GB wall thickening 6. Roth T et al: Acute acalculous cholecystitis associated with
• Perforated aortic dissection: report of a case. Surg Today. 33(8):633-5,
o CECT: Pericholecystic abscess, GB wall necrosis with 2003
lack of enhancement 7. Cheema S et al: Timing of laparoscopic cholecystectomy in
acute cholecystitis. Ir J Med Sci. 172(3):128-31,2003
8. Ozaras R et al: Acute viral cholecystitis due to hepatitis A
virus infection. J Clin Gastroenterol. 37(1):79-81, 2003
I CLINICAL ISSUES 9. Ko CW et al: Gastrointestinal disorders of the critically ill.
Biliary sludge and cholecystitis. Best Pract Res Clin
Presentation Gastroenterol. 17(3):383-96, 2003
• Most common signs/symptoms: Acute RUQ pain, fever 10. Gandolfi L et al: The role of ultrasound in biliary and
• Clinical profile pancreatic diseases. Eur J Ultrasound. 16(3):141-59,2003
o Increased WBC 11. Trowbridge RL et al: Does this patient have acute
o May have mild elevation in liver enzymes cholecystitis? JAMA. 289(1):80-6, 2003
12. Fayad LM et al: Functional magnetic resonance
Demographics cholangiography (fMRC) of the gallbladder and biliary tree
• Age: Typically> 25 y with contrast-enhanced magnetic resonance
cholangiography. J Magn Reson Imaging. 18(4):449-60,
• Gender: M:F = 1:3
2003
Natural History & Prognosis 13. Pedrosa I et al: The interrupted rim sign in acute
cholecystitis: a method to identify the gangrenous form
• May progress to gangrenous cholecystitis and
with MRI. J Magn Reson Imaging. 18(3):360-3, 2003
perforation if untreated
14. Oh KYet al: Limited abdominal MRI in the evaluation of
• Excellent prognosis in uncomplicated cases or with acute right upper quadrant pain. Abdom Imaging.
prompt surgery 28(5):643-51,2003
• Complications 15. Gore RM et al: Imaging benign and malignant disease of
o Mirizzi syndrome and Bouveret syndrome (gallstone the gallbladder. Radiol Clin North Am. 40(6):1307-23, vi,
erodes into duodenum causing obstruction) 2002
16. Bingener-Casey J et al: Reasons for conversion from
Treatment laparoscopic to open cholecystectomy: a 10-year review. J
• Prompt cholecystectomy Gastrointest Surg. 6(6):800-5, 2002
o Laparoscopic surgery for uncomplicated cases 17. Kitano S et al: Laparoscopic cholecystectomy for acute
cholecystitis. J Hepatobiliary Pancreat Surg. 9(5):534-7,
• Percutaneous cholecystectomy
2002
o Useful for poor operative risk patients with GB 18. Indar AA et al: Acute cholecystitis. BM]. 325(7365):639-43,
empyema 2002
• Percutaneous drainage 19. Merchant SS et al: Staphylococcus aureus cholecystitis: a
o Well-defined, well-localized pericholecystic abscesses report of three cases with review of the literature. YaleJ
BioI Med. 75(5-6):285-91,2002
20. Tanaka M: Bile duct clearance, endoscopic or laparoscopic?
I DIAGNOSTIC CHECKLIST 21.
J Hepatobiliary Pancreat Surg. 9(6):729-32, 2002
Abou-Saif A et al: Complications of gallstone disease:
Consider Mirizzi syndrome, cholecystocholedochal fistula, and
gallstone ileus. Am J Gastroenterol. 97(2):249-54, 2002
• Perforated ulcer or pancreatitis with secondary GB wall 22. Adusumilli S et al: MR imaging of the gallbladder. Magn
thickening Reson Imaging Clin N Am. 10(1):165-84,2002
23. Kalimi R et al: Diagnosis of acute cholecystitis: sensitivity
Image Interpretation Pearls of sonography, cholescintigraphy, and combined
• Stone impacted in cystic duct sonography-cholescintigraphy. J Am ColI Surg.
• Sonographic Murphy sign must be unequivocal to be 193(6):609-13, 2001
considered positive
 CHOLECYSTITIS
I IMAGE GALLERY
Typical
(Left) Transverse sonogram
of calculous cholecystitis.
Note cystic duct stone
(arrow) and marked
2
gallbladder wall thickening
(open arrow). (Right) Axial 37
CECT of cholecystitis. Note
pericholecystic stranding
(arrow) and focal
interruption of enhancement
of gallbladder wall (open
arrow).

Typical
(Left) Sagittal US of
gangrenous cholecystitis
demonstrates intraluminal
membranes from fibrinous
debris (arrow). (Right)
Sagittal US of gangrenous
cholecystitis demonstrating
sloughed mucosa (arrow).

Typical
(Left) Axial CECT of
pericholecystic abscess
demonstrates distended
gallbladder with edematous
wall (arrow). (Right) Axial
CECT of perich ole cystic
abscess demonstrates
walled-off abscess from
fundal perforation (arrow).
 PORCELAIN GJt.LLBlADDER

2
38

Anteroposterior radiography shows thin rim of Coronal CECT shows rim of interrupted calcifications
calcification (arrows) in right upper quadrant (arrow) in the lateral aspect of the gallbladder wall.
conforming to the shape of the gallbladder.

• CECT: Calcification in GB wall

ITERMINOlOGY
Ultrasonographic Findings
Abbreviations and Synonyms
• Real Time
• Calcified gallbladder (GB), calcifying cholecystitis,
o Echogenic curvilinear structure in GB fossa with
cholecystopathia chronica calcarea acoustic shadowing
Definitions o Coarse foci of calcification with acoustic shadowing
• Calcification of the gallbladder wall in GB wall
Imaging Recommendations
• Best imaging tool: CT
[IMAGING FINDINGS • Protocol advice: Thin-sections may facilitate detection
General Features and localization of calcifications
• Best diagnostic clue: Rim of calcification in right upper
quadrant conforming to shape of the gallbladder
• Location: Gallbladder wall IDIFFERENTIAl DIAGNOSIS
• Size: May involve all or part of the gallbladder wall Large gallstone
• Morphology: Two patterns: Selective mucosal
• Mimics porcelain GB on all modalities
calcification and diffuse intramural calcification
Emphysematous cholecystitis (ultrasound)
Radiographic Findings
• Echogenic crescent in gallbladder with acoustic
• Radiography shadowing on ultrasound mimics GB wall calcification
o Curvilinear or granular calcification in GB wall
• CT can best distinguish gas from calcium
o May involve entire wall or just a segment
• Fluoroscopy: Usually non-functional GB on oral Iatrogenic
cholecystograms • Iodized oil in GB wall following hepatic
chemo-embolization
CT Findings
• NECT: Calcification in GB wall

DDx: Mimics of Porcelain Gallbladder

Large Gallstone Large Gallstone Gallbladder Gas Chemoem boli za tion

 PORCELAIN GALLBLADDER

Key Facts
Imaging Findings Pathology
• Curvilinear or granular calcification in GB wall • General path comments: Risk factor for gallbladder
• May involve entire wall or just a segment carcinoma
• Associated abnormalities: Gallstones in 90-95%
Top Differential Diagnoses
• Large gallstone Clinical Issues 2
• Emphysematous cholecystitis (ultrasound) • Options, risks, complications: Prophylactic
cholecystectomy is current consensus 39
recommendation

o Flecks of calcium in gallbladder mucosa ~

I PATHOLOGY significant (5%) risk of cancer
General Features Treatment
• General path comments: Risk factor for gallbladder • Options, risks, complications: Prophylactic
carcinoma cholecystectomy is current consensus
• Etiology recommendation
o Several theories of pathogenesis
• Intermittently obstructed GB ~ supersaturated
bile ~ calcium carbonate accumulates ~ I DIAGNOSTIC CHECKLIST
precipitates in wall
• Intramural hemorrhage from cholecystitis ~ Consider
mural calcification • Look for gallbladder mass on CT if porcelain GB
• Dystrophic mural calcification related to chronic identified
inflammation of GB wall
• Epidemiology: Rare: 0.06-0.8% of cholecystectomy Image Interpretation Pearls
specimens • Wall-echo-shadow (WES) sign on ultrasound can
• Associated abnormalities: Gallstones in 90-95% differentiate gallstones from porcelain GB

Gross Pathologic & Surgical Features

• Brittle gallbladder wall with bluish discoloration I SELECTED REFERENCES
• Fibrotic wall and brittle consistency makes
1. Opatrny L: Porcelain gallbladder. CMAJ. 166(7): 933, 2002
laparoscopic cholecystectomy technically challenging 2. Gore RM et al: Imaging benign and malignant disease of
Microscopic Features the gallbladder. Radiol Clin North Am. 40(6): 1307-23, vi,
2002
• Two histopathologic forms 3. Stephen AE et al: Carcinoma in the porcelain gallbladder: a
o Coarse plaques of calcium in muscularis of GB wall relationship revisited. Surgery. 129(6): 699-703, 2001
o Punctate foci of mucosal calcification 4. Towfigh S et al: Porcelain gallbladder is not associated with
gallbladder carcinoma. Am Surg. 67(1): 7-10, 2001
S. Rybicki PJ:The WES sign. Radiology. 214(3): 881-2, 2000
I CLINICAL ISSUES
Presentation I IMAGE GAllERY
• Most common signs/symptoms
o Usually asymptomatic
o Other signs/symptoms
• Right upper quadrant pain
• Palpable right upper quadrant mass
Demographics
• Age: Occurs in 6th decade; mean age = 54 years
• Gender: M:F = 1:5
Natural History & Prognosis
• Incidence of cancer in porcelain GB usually quoted as
12-62% based on retrospective data from 1950s-1960s
• More recent reviews suggest much weaker association:
(Left) Axial NECT shows thick rind of calcification in gallbladder wall
0-5% incidence
(open arrow). Note layer of calcified stones in dependent aspect of
• Risk of gallbladder cancer may depend on pattern of gallbladder. (Right) Axial CECT shows curvilinear calcification in GB
calcification wall (black arrow) and low attenuation area in adjacent liver
o Diffuse intramural calcification ~ no risk of cancer representing gallbladder carcinoma (white arrows) (Courtesy M.
Nino-Murcia, MD).
 MILK OF CALCIUM BILE

2
40

Axial NECT of milk of calcium bile in asymptomatic Axial CECT of milk of calcium bile in asymptomatic
patient. Note high attenuation liquid layering posteriorly patient. Note normal gallbladder wall (arrow) & lack of
in gallbladder (arrow). inflammatory changes.

• CECT: GB wall may enhance normally or be thickened

ITERMINOlOGY in patients with associated cholecystitis
Abbreviations and Synonyms MR Findings
• Limy bile syndrome, calcium carbonate bile
• T2WI: Low signal calcium carbonate layers
Definitions dependently in GB
• Calcium carbonate precipitate within gallbladder (GB) • Tl C+: GB wall may enhance normally or be thickened
lumen in cholecystitis
• MRCP
o Low signal fluid layering dependently in GB
IIMAGING FINDINGS Ultrasonographic Findings
General Features • Real Time: Medium- to low-level echogenic fluid
within GB with acoustic shadowing; may have
• Best diagnostic clue: Identification of calcified liquid
within GB associated gallstones, thickened GB wall on US
• Location: Within GB • Color Doppler: Hyperemia of GB wall in patients with
acute cholecystitis
Radiographic Findings
Nuclear Medicine Findings
• Radiography: Calcified liquid in GB, fluid-fluid level
on upright film • Hepato Biliary Scan
o Non-filling of GB if cystic duct obstruction
• ERCP
o Milk of calcium may obstruct cystic duct, prevent Imaging Recommendations
filling of GB • Best imaging tool: US, NECT
CT Findings • Protocol advice: Tissue harmonic US may improve
visualization of acoustic shadowing
• NECT: High attenuation (> 150 HU) liquid in
gallbladder; rarely occurring in common bile duct
(CBD)

DDx: Gallbladder lesions Mimicking Milk of Calcium Bile

Vicarious Excretion Biliary Sludge GB Heme Gallbladder CA

 MILK OF CALCIUM BILE
Key Facts
Terminology • Real Time: Medium- to low-level echogenic fluid
• Calcium carbonate precipitate within gallbladder within GB with acoustic shadowing; may have
(GB) lumen associated gallstones, thickened GB wall on US
• Best imaging tool:· US, NECT
Imaging Findings • Protocol advice: Tissue harmonic US may improve
• NECT: High attenuation (> 150 HU) liquid in viSualization of acoustic shadowing 2
gallbladder; rarely occurring in common bile duct
(CBD) Clinical Issues 41
• Incidental finding; may be asymptomatic

I DIFFERENTIAL DIAGNOSIS Treatment

• Options, risks, complications
Vicarious excretion of contrast
o No treatment
• Hepatic excretion of iodinated contrast o Laparoscopic or open cholecystectomy
• Normally seen 24 hours post-contrast administration o Bile leak, abscess, hematoma related to
GB sludge cholecystectomy
• Echogenic luminal debris, no distal acoustic
enhancement
• May be "congested" as tumefactive sludge I DIAGNOSTIC CHECKLIST
• Avascular with color Doppler Consider
G B hemorrhage • GB sludge or hemorrhage
• High attenuation (> 30 HU) fluid in GB Image Interpretation Pearls
• Echogenic on ultrasound, no distal acoustic
• Echogenic fluid similar to sludge but with acoustic
shadowing
shadowing
GB carcinoma
• Polypoid hypoechoic mass> 1 cm arising from GB
mucosa with internal flow on color Doppler I SELECTED REFERENCES
• Large hypoechoic mass infiltrating GB fossa & 1. Itoh H: Management of limy bile syndrome: no therapy,
invading liver laparotomy or endoscopic treatment? Intern Med.
• Associated with gallstones, chronic cholecystitis, 42(1):1-2,2003
porcelain GB 2. Moreaux J et al: Limy bile. A surgical experience in 16
patients. Gastroenterol Clin BioI. 18(6-7):550-5, 1994
3. Fowler CL et al: Limy bile syndrome. J Pediatr Surg.
28(12):1568-9, 1993
!PATHOLOGY
General Features
• Etiology: GB stasis
I IMAGE GALLERY
• Epidemiology: Incidence of 0.27% in patients
undergoing cholecystectomy
Gross Pathologic & Surgical Features
• Calcium carbonate in bile, thickened GB wall

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: RUQ pain, but may be
asymptomatic
Demographics (Left) Axial US of milk of calcium bile shows echogenic material with
• Age: 42-66 yrs acoustic shadowing. (Right) Axial NEeT reveals high attenuation milk
• Gender: M < F of calcium bile in gallbladder.

Natural History & Prognosis

• Incidental finding; may be asymptomatic
• Calcium carbonate, associated gallstones with cystic
duct obstruction & acute/chronic cholecystitis
 PRIMARYS.CLEROSINGCHOLANGITIS

2
42

Advanced PSC induced liver disease.Axial CECT shows Typical cholangiographic findings of PSc. ERCP shows
dysmorphic liver with caudate hypertrophy and abnormal arborization of IHBO with multifocal
peripheral atrophy. IHBO are irregularly dilated, strictures, marked dilatation of left hepatic ducts.
especially in left lobe.

ITERMINOLOGY • Contour is grossly lobulated; caudate lobe

hypertrophy (98%)
Abbreviations and Synonyms o Accentuated lobulation of hepatic contour is result
• Primary sclerosing cholangitis (PSC) of asymmetric atrophy & marked focal hypertrophy

Definitions Radiographic Findings

• Primary sclerosing cholangitis (PSC) is a chronic
idiopathic inflammatory process of bile ducts
I IMAGING FINDINGS
General Features
• Best diagnostic clue
o Classic "beaded appearance"- alternating segments of
dilatation & focal circumferential strictures
• Combined findings of multifocal short strictures,
beading, pruning, diverticula, & mural irregularity
• Location
o CBD (almost always involved); IRBD + extrahepatic
(68-89%); Intrahepatic ducts only (11-25%);
extrahepatic ducts only (2-3%); cystic duct (15-18%)
o Most severely affected segments of biliary tree in
PSC are main right & left bile ducts
• Morphology
o Liver in patients with PSC-induced end-stage
cirrhosis is markedly deformed
• ERCP
o Multifocal strictures, mural irregularity, diverticula
o Biliary ductal dilatation is a typical finding (> 80%)
CT Findings
• NECT
o Scattered, dilated intrahepatic ducts with no
apparent connection to main bile ducts
o Skip dilatations, stenosis, beading, pruning &
thickening of duct wall
o Periportal fibrosis: Low-attenuation soft tissue
adjacent to major ducts & portal vein branches
o In~rahepatic biliary calculi: Areas of calcific density
adJacent to portal veins (8%)
o End-stage PSC: Abnormal, rounded, lobular liver
contour; atrophy of lateral & posterior segments
• Low-attenuation rind-like appearance of right lobe
in presence of hypertrophy of caudate lobe
• Rind accentuates hypertrophied (&
higher-attenuation) caudate lobe; creating effect
of pseudotumor (seen best on NECT)

DDx: Irregularly Dilated Bile Ducts

Ascending Cholangitis AIDS Cholangiopathy Cholangiocarcinoma Liver Mets (Gastric CA)

 PRIMARY SCLEROSING CHOLANGITIS
Key Facts
Terminology
• Primary sclerosing cholangitis (PSC) is a chronic
idiopathic inflammatory process of bile ducts
Imaging Findings
• Classic "beaded appearance"- alternating segments of
dilatation & focal circumferential strictures
• Combined findings of multifocal short strictures,
beading, pruning, diverticula, & mural irregularity
• Periportal fibrosis: Low-attenuation soft tissue
adjacent to major ducts & portal vein branches
• Intrahepatic biliary calculi: Areas of calcific density
adjacent to portal veins (8%)
• End-stage PSC: Abnormal, rounded, lobular liver
contour; atrophy of lateral & posterior segments
• Result of different amounts of fibrosis & hepatic
parenchyma in the two regions; atrophic right
lobe contains more fibrosis & is thus of lower
attenuation than hypertrophied caudate lobe
o Cholangiocarcinoma (CC): Progressive biliary
dilatation; periportal soft tissue hypo attenuating
mass of 1.5 cm or greater, suspicious for malignancy
• Mural thickening of extrahepatic ducts of 5 mm
or greater is presumptive evidence of CC
• CECT
o Mural enhancement of bile ducts (may be seen in
normal ducts & in other diseases as well)
o Endstage PSC: Cirrhotic liver; hypertrophy of
caudate & deep right lobe; atrophy of peripheral
liver
o CC: Delayed contrast-enhancement
MR Findings
• TIWI
o t Signal intensity along bile ducts & portal veins
(periportal fibrosis)
o t Signal intensity-periportal infiltrating soft tissue
mass (CC)
• T2WI
o 1 Signal intensity (periportal fibrosis)
o 1 Signal intensity soft tissue mass (CC)
• Peripheral wedge-shaped areas of parenchymal
atrophy consistent with confluent fibrosis
• Biliary ductal dilatation
• May see focal areas of parenchymal edema &
hyperperfusion secondary to focal inflammation (also
seen in other inflammatory bile duct diseases:
Infectious/recurrent pyogenic cholangitis)
• MRCP: Irregular strictures with segmental dilatations
of intra- & extrahepatic ducts
Ultrasonographic Findings
• Dilatation + stenosis + irregular fibrous thickening of
walls of bile ducts (2-5 mm)
• Brightly echogenic portal triads
• Gallbladder abnormalities (40%): Gallstones (26%),
adenoma & adenocarcinoma (4%)
• Low-attenuation rind-like appearance of right lobe in
presence of hypertrophy of caudate lobe
Clinical Issues
• Age: 70% are younger than 45 years
• 70% of patients are male
• Multicentric nature & chronic obstruction of bile
2
ducts leads to cirrhosis in many patients 43
• Complications: Biliary cirrhosis, portal hypertension,
cholangiocarcinoma (6-15%)
Diagnostic Checklist
• Pseudotumor of caudate lobe in PSC should be
recognized as such & not misinterpreted as neoplasm
o Direct involvement of gallbladder wall (15%):
Asymmetric, uniform, symmetric thickening of wall
• Echogenic biliary casts punctate coarse calcifications
along portal vein
Other Modality Findings
• Cholangiography: Multiple segmental strictures
involving both intra- & extrahepatic bile ducts
o "Beaded appearance" - alternating segments of
dilatation & focal circumferential strictures
o Stricture length: Can vary from 1-2 mm (band
strictures) to several cms (commonly 1-1.5 cm)
• "Pruned-tree" appearance: Opacification of central
ducts+ obliterated peripheral smaller radicles
• Diverticular outpouchings (25%): 1-2 mm to 1 cm;
saccular; herniations adjacent to strictures, mucosal
extensions of thickened duct wall
• Mural irregularity (50%): Fine, brush-border to coarse,
shaggy or frankly nodular appearance
• Intraluminal filling defects (5-10%): Small, 2-5 mm
diameter; 1 cm or larger occur in 50% of PSC
complicated by cholangiocarcinoma
• Cholangiographic findings which suggest malignant
degeneration include markedly dilated ducts or ductal
segments (100% in PSC + CC vs. 24% in PSC alone)
o Polypoid mass (46% in PSC + CC vs. 7% in PSC
alone); 1 cm or greater in diameter with CC
o Progressive stricture formation or ductal dilatation:
New stricture + lengthening of strictures between 6
months & 6 years « 20%)
o Dominant stricture: Often near hilum, more severely
strictured than remainder of ducts (25%)
• Gallbladder carcinoma: Discrete or infiltrative mass
Imaging Recommendations
• Best imaging tool
o Helical NE + CECT, MR + MRCP, cholangiography
(T-tube & retrograde)
o Diagnosis is usually established with ERCP & less
often with percutaneous transhepatic
cholangiography (PTC)
• Protocol advice
 PRIMARY SCI.ERDSINGCHDLANGITIS
o Coronal-oblique half-Fourier RARE MR
cholangiogram using of both thin- & thick-section
• Thin-section (5 mm): Useful in depicting focal
strictures & subtle irregularities of ductal segments
on multiple images
• Thick-section (40 mm): Provides comprehensive
view of entire biliary tract on single image
2 o 70% of patients have ulcerative colitis
44 I DIFFERENTIAL DIAGNOSIS
Ascending cholangitis
• Presence of bile duct stones; prior sphincterotomy
AIDS cholangiopathy
• ERCP findings closely simulate PSC
• Strictures of distal CBD and IHBD
Sclerosing cholangiocarcinoma
• Intraductal mass> 1 em; ductal dilatation proximal to
dominant stricture
• Progressive cholangiographic changes within 0.5-1.5
years of initial diagnosis
Extrinsic compression
• Cirrhosis: Peripheral dilated bile ducts & irregular
strictures; centrally obstructed by large nodules
• Liver masses: Hepatic metastases + lymph nodes in
porta hepatis cause extrinsic compression of ducts
Chemotherapy cholangitis
• Segmental strictures of variable length
• Duct beading & intrahepatic duct involvement are
relatively less common in chemotherapy cholangitis
• Gallbladder & cystic duct are usually more severely
involved in chemotherapy cholangitis than in PSC
I PATHOLOGY
General Features
• Etiology: Idiopathic; hypersensitivity reaction (genetic
& immunologic)
• Associated abnormalities: Ulcerative colitis (70%),
Crohn disease (13%), pancreatitis, sicca complex,
Riedel struma, retroperitoneal/mediastinal fibrosis
Gross Pathologic & Surgical Features
• Dilated + stenotic bile ducts, periportal fibrosis,
intraductal calculi
• Endstage PSC: Lobular liver, atrophy (periphery liver),
caudate hypertrophy
Microscopic Features
• Nonsuppurative, non granulomatous destruction of
bile ducts
• Portal hepatitis or cholangitis (stage 1); periportal
hepatitis or fibrosis (stage 2); septal fibrosis or bridging
necrosis or both (stage 3); cirrhosis (stage 4)
I CLINICAL ISSUES
Presentation
• Progressive chronic & intermittent obstructive
jaundice
• Fatigue, pruritus, RUQ pain, hepatosplenomegaly
• Lab: Elevation of serum alkaline phosphatase
Demographics
• Age: 70% are younger than 45 years
• Gender
o 70% of patients are male
Natural History & Prognosis
• Variable natural history; usually progressive downhill
• Involvement of small intrahepatic bile ducts (so-called
small-duct PSC) may be the only &/or earliest
manifestation of PSC
o Classic obliterative fibrosis of medium-sized & large
ducts (so-called large-duct PSC) occurs at later stage
• Results in cholestasis with progression to
secondary biliary cirrhosis & hepatic failure
o Multicentric nature & chronic obstruction of bile
ducts leads to cirrhosis in many patients
• Biliary calculi develop in obstructed ducts as a
consequence of biliary stasis & possible secondary
infection
• Atrophy/hypertrophy complex may even occur in
absence of cirrhosis
• Parenchymal atrophy: Due to chronic obstruction of
segmental bile ducts, & is a sequela of parenchymal
necrosis & fibrosis
• Regional hypertrophy: In areas with absent or less
severe biliary obstruction; in response to I hepatic
function that accompanies parenchymal atrophy
• Complications: Biliary cirrhosis, portal hypertension,
cholangiocarcinoma (6-15%)
• Prognosis: 5 year survival is 88%; median survival is
11.9 years from time of diagnosis
Treatment
• Liver transplantation
I DIAGNOSTIC CHECKLIST
Consider
• History of ulcerative colitis; identification of
morphologic findings seen more frequently in PSC
induced end-stage cirrhosis & known ductal
abnormalities of PSC may allow one to strongly
suggest PSC as cause of cirrhosis
• Pseudo tumor of caudate lobe in PSC should be
recognized as such & not misinterpreted as neoplasm
I SELECTED REFERENCES
1. Bader TR et al: MR imaging features of primary sclerosing
cholangitis: patterns of cirrhosis in relationship to clinical
severity of disease. Radiology. 226(3):675-85, 2003
2. Campbell WL et al: Using CT and cholangiography to
diagnose biliary tract carcinoma complicating primary
sclerosing cholangitis. AJRAm J Roentgenol.
177(5): 1095-100, 2001
3. Fulcher AS et al: Primary sclerosing cholangitis: evaluation
with MR cholangiography-a case-control study. Radiology.
215(1):71-80,2000
 PRIMARY SCLEROSING CHOLANGITIS
I IMAGE GALLERY

(Left) Catheter
cholangiogram shows
multifocal strictures, 2
moderate dilatation of right
IHBD. Tight stricture of main
left hepatic duct (arrow) 45
with partial opacification of
very dilated left IHBD.
(Right) Oblique sagittal
sonogram shows thickened,
hyperechoic wall of common
duct (arrow) in patient with
PSc.

(Left) Axial CECT shows

irregular dilatation of IHBD
and abnormal arborization
with no apparent connection
to the central biliary tree.
(Right) Axial CECT in patient
with PSC and ulcerative
colitis shows thick-walled
recto-sigmoid colon with fat
density proliferation in
submucosa (arrow).

Other
(Left) PTC shows multifocal
strictures and dilatations of
right IHBD. There is a long
dominant stricture of the
main left duct and marked
dilatation of the left IHBD
raising concern for
cholangiocarcinoma. (Right)
PTC of patient with liver
transplant for endstage
PSC-induced liver disease
shows markedly irregular
mucosal surface with
multifocal strictures and
filling defects within ducts.
Recurrent PSc.
 GALLBLADDERC.t\RCINOMA

2
46

Schematic drawing of gallbladder carcinoma. Note Axial CECT demonstrates large hypodense mass
gallstones and focal mural mass arising from the infiltratinggallbladder fossa and invading liver (arrows).
gallbladder wall that invades the adjacent liver and Open arrow denotes gallbladder.
obctructs the common hepatic duct.

ITERMINOLOGY • Non-visualization of GB
• Rarely pneumobilia 2° to GB enteric fistula
Definitions • ERCP
• Malignant epithelial neoplasm arising from o Non-visualization of GB
gallbladder (GB) mucosa o Common hepatic duct obstruction
o Dilated intrahepatic ducts
CT Findings
I IMAGING FINDINGS • NECT: Calcification of GB wall (porcelain GB);
General Features calcified gallstones
• Best diagnostic clue • CECT
o Large GB mass infiltrating gallbladder fossa o Hypovascular mass infiltrating GB fossa, invading
extending into liver liver along main lobar fissure; porta hepatis
o Polypoid intraluminal mass adenopathy on CECT
o Diffuse or focal irregular mural thickening mimics o Nodal mets to peripancreatic area may simulate
chronic cholecystitis pancreatic carcinoma
o Invasion of liver and porta hepatis
• Location: GB fundus and body; uncommon in cystic
duct o Calcified stones or porcelain GB
• Size: Variable; smaller polypoid mass in early CA, large MR Findings
infiltrating lesions typical
• TlWI
• Morphology: Large soft tissue mass infiltrating GB o Iso- or hypointense GB fossa mass with increased
fossa; polypoid mucosal mass in GB signal compared to normal liver
Radiographic Findings o Hypovascular after IV gadolinium
• Radiography • T2WI: Mass slightly increased in signal intensity
o Plain abdominal radiographs compared to liver
• Calcified gallstones or porcelain GB • Tl C+: Hypovascular GB fossa mass invading liver
o Oral cholecystogram (OCG) • MRCP

DDx: Spectrum of GB Dise(lsesMirnickingCarcil1oma

Cholecystitis Porta Hepatis Nodes GB Polyps Adenomyomatosis

 GALLBLADDER CARCINOMA

Key Facts
Imaging Findings • GB polyp
• Morphology: Large soft tissue mass infiltrating GB • Adenomyomatosis
fossa; polypoid mucosal mass in GB Pathology
• NECT: Calcification of GB wall (porcelain GB);
• Associated with porcelain GB & chronic
calcified gallstones
• Hypovascular mass infiltrating GB fossa, invading
inflammation 2° to gallstones; malignant
degeneration of adenomatous mucosal polyps
2
liver along main lobar fissure; porta hepatis
• Scirrhous infiltrating mass extending from GB wall to 47
adenopathy on CECT
obliterate GB fossa & invade liver; porta hepatis
• Real Time: Gallstones, calcified GB wall mass adenopathy
infiltrating GB fossa, porcelain GB, moderately
echogenic polypoid mucosal mass (> 1 cm) on US Clinical Issues
• Best imaging tool: US, CECT • Most common signs/symptoms: RUQ pain, weight
loss, jaundice, vomiting
Top Differential Diagnoses
• Complicated cholecystitis
• Metastatic disease to GB fossa

o Dilated bile ducts due to common hepatic duct • Focal thickening of midportion of GB ("hourglass GB")
obstruction • May demonstrate diffuse wall thickening
• Intramural cholesterol crystals as bright echoes with
Ultrasonographic Findings "comet tail" reverberation echoes
• Real Time: Gallstones, calcified GB wall mass
infiltrating GB fossa, porcelain GB, moderately
echo genic polypoid mucosal mass (> 1 cm) on US I PATHOLOGY
• Color Doppler: Areas of increased vascularity
General Features
Nuclear Medicine Findings
• General path comments
• Hepato Biliary Scan o 90% adenocarcinoma
o Non-filling of GB • Early stage: Polypoid mucosal mass
Imaging Recommendations • Late stage: Mass infiltrating GB fossa
• Best imaging tool: US, CECT o 10% squamous or anaplastic
• Protocol advice: Longitudinal & transverse images of • Genetics: No known association
GB fossa with grayscale and color Doppler • Etiology
o Associated with porcelain GB & chronic
inflammation 2° to gallstones; malignant
I DIFFERENTIAL DIAGNOSIS degeneration of adenomatous mucosal polyps
o 75% have gallstones
Complicated cholecystitis o Porcelain GB predisposes to GB carCinoma
• Gallstones • Epidemiology
• Thick-walled GB, pericholecystic abscess o Most common type of biliary cancer
• GB may be contracted • 6500 deaths per year in US
• May be indistinguishable from carcinoma o 75% are women
o Average age of presentation is 70 yrs
Metastatic disease to GB fossa o Fifth most common GI cancer, 9 times more
• Most often nodal distribution around portal vein common than extrahepatic cholangiocarcinoma
• Melanoma may directly metastasize to GB mucosa • Associated abnormalities
• Hepatoma and other hepatic tumors may secondarily o Gallstones in > 65%
spread to GB via duct invasion o Chronic cholecystitis
• Porta hepatis lymphadenopathy o Porcelain GB (4-60%)
o Lymphoma and GI tract carcinoma most common o Ulcerative colitis; rarely Crohn disease
o Primary sclerosing cholangitis
GB polyp o Familial polyposis coli
• Non-shadowing, mucosal mass
o Moderately echogenic without shadowing Gross Pathologic & Surgical Features
• Non-mobile, attached to wall • Scirrhous infiltrating mass extending from GB wall to
• Typically < 1 cm for cholesterol polyp obliterate GB fossa & invade liver; porta hepatis
• No flow on color Doppler adenopathy
• Direct invasion of liver, duodenum, stomach, bile
Adenomyomatosis duct, pancreas, R kidney
• Localized fundal GB wall thickening
• Gallstones
 GAI.I.BI.ADDERCARCI NOMA
• Lymphatic spread to porta hepatis, peri pancreatic & 3. Pandey M: Risk factors for gallbladder cancer: a reappraisal.
retroperitoneal nodes Eur J Cancer Prevo 12(1):15-24,2003
4. Misra S et al: Carcinoma of the gallbladder. Lancet Oncol.
• Intraperitoneal spread common with ascites, omental
4(3):167-76, 2003
nodules & peritoneal implants 5. Goindi G et al: Risk factors in the aetiopathogenesis of
• Hematogenous spread (late in clinical course) to lungs, carcinoma of the gallbladder. Trop Gastroenterol.
liver & bones 24(2):63-5, 2003
• Perineural invasion common 6. Kokudo N et al: Strategies for surgical treatment of
2 Microscopic Features
gallbladder carcinoma based on information available
before resection. Arch Surg. 138(7):741-50; dis 750, 2003
48 • Adenocarcinoma (90%) 7. Yamamoto T et al: Early gallbladder carcinoma associated
• Squamous or anaplastic carcinoma (10%) with primary sclerosing cholangitis and ulcerative colitis. J
Gastroenterol. 38(7):704-6, 2003
Staging, Grading or Classification Criteria 8. Varshney S et al: Incidental carcinoma of the gallbladder.
• Stage I: Carcinoma confined to mucosa Eur J Surg Oncol. 28(1):4-10, 2002
• Stage II: Carcinoma involves mucosa & muscularis 9. Cunningham CC et al: Primary carcinoma of the gall
• Stage III: Carcinoma extends to serosa bladder: a review of our experience. J La State Med Soc.
154(4):196-9, 2002
• Stage IV: Transmural involvement with positive nodes
10. Doty JR et al: Cholecystectomy, liver resection, and
• Stage V: Liver or distant metastases pylorus-preserving pancreaticoduodenectomy for
gallbladder cancer: report of five cases. J Gastrointest Surg.
6(5):776-80, 2002
I CLINICAL ISSUES 11. Corvera CD et al: Role of laparoscopy in the evaluation of
biliary tract cancer. Surg Oncol Clin N Am. 11(4):877-91,
Presentation 2002
• Most common signs/symptoms: RUQ pain, weight 12. Rashid A: Cellular and molecular biology of biliary tract
loss, jaundice, vomiting cancers. Surg Oncol Clin N Am. 11(4):995-1009,2002
13. Gore RM et al: Imaging benign and malignant disease of
• Clinical profile: Elevated bilirubin, elevated alkaline
the gallbladder. Radiol Clin North Am. 40(6):1307-23, vi,
phosphatase with biliary obstruction 2002
Demographics 14. Xu AM et al: Multi-slice three-dimensional spiral CT
cholangiography: a new technique for diagnosis of biliary
• Age: Mean 70 years diseases. Hepatobiliary Pancreat Dis Int. 1(4):595-603,2002
• Gender: M:F = 1:3 15. Towfigh S et al: Porcelain gallbladder is not associated with
gallbladder carcinoma. Am Surg. 67(1):7-10, 2001
Natural History & Prognosis 16. Levy AD et al: Gallbladder carcinoma:
• Spreads by local invasion to liver, nodal spread to radiologic-pathologic correlation. Radiographies.
porta hepatis and para-aortic nodes, hematogeneous 21(2):295-314; questionnaire, 549-55, 2001
spread to liver 17. Dixit VK et al: Aetiopathogenesis of carcinoma gallbladder.
• Very poor prognosis; 4% 5 yr survival rate, 75% of Trop Gastroenterol. 22(2):103-6, 2001
patients have mets at time of diagnosis 18. Tazuma S et al: Carcinogenesis of malignant lesions of the
gall bladder. The impact of chronic inflammation and
Treatment gallstones. Langenbecks Arch Surg. 386(3):224-9, 2001
• Cholecystectomy for lesions confined to GB wall 19. Stewart CJ et al: Brush cytology in the assessment of
pancreatico-biliary strictures: a review of 406 cases. J Clin
without liver invasion
Pathol. 54(6):449-55, 2001
• Radical cholecystectomy and/or partial hepatectomy 20. Pandey M et al: Carcinoma of the gallbladder: a
with regional node dissection for lesions infiltrating retrospective review of 99 cases. Dig Dis Sci. 46(6):1145-51,
porta hepatis 2001
21. Kaushik SP: Current perspectives in gallbladder carcinoma.
J Gastroenterol Hepatol. 16(8):848-54, 2001
I DIAGNOSTIC CHECKLIST 22. Fujii H et al: Small cell carcinoma of the gallbladder: a case
report and review of 53 cases in the literature.
Consider Hepatogastroenterology. 48(42):1588-93, 2001
23. Eriguchi N et al: Xanthogranulomatous cholecystitis.
• Adenomyomatosis with GB wall thickening
Kurume MedJ. 48(3):219-21, 2001
o Benign adenomatous polyp < 2 cm 24. Donohue JH: Present status of the diagnosis and treatment
Image Interpretation Pearls of gallbladder carcinoma. J Hepatobiliary Pancreat Surg.
8(6):530-4, 2001
• Porcelain GB 25. Narula 1M: Historical review of carcinoma of the
• Mass infiltrating GB fossa gallbladder. IndianJ Hist Med. 16:6-11, 1971
• Large polypoid GB mucosal mass with flow

I SELECTED REFERENCES
1. Yun EJ et al: Gallbladder carcinoma and chronic
cholecystitis: differentiation with two-phase spiral CT.
Abdom Imaging. 29(1):102-8, 2003
2. Enomoto T et al: Xanthogranulomatous cholecystitis
mimicking stage IV gallbladder cancer.
Hepatogastroenterology. 50(53):1255-8, 2003
 GALLBLADDER CARCINOMA
I IMAG E GALLERY
Typical
(Left) Axial US of gallbladder
carcinoma. Note large
hypoechoic mass obliterating
gallbladder. Note gallstone
2
(arrow). (Right) Axial US of
gallbladder carcinoma 49
invading liver. Note
hypoechoic mass infiltrating
gallbladder fossa and
invading right and left lobes
of liver (arrows).

(Left) Axial CECT of invasive

gallbladder carcinoma. Note
hypodense mass invading
liver (arrow) infiltrating along
portal vein (open arrow).
(Right) Axial CECT
demonstrates gallbladder
carcinoma as polypoid mass
(arrow).

Typical
(Left) Axial CECT
demonstrates hypodense
fundal mass (arrow). (Right)
Axial CECT demonstrates
invasion of liver (arrow).
 CHOLANGIOCARCINOMA

2
50

Axial graphic shows Klatskin tumor, a small mass at the Cholangiogram shows mass at confluence of main right
confluence of the main right and left bile ducts, that and left ducts with marked dilatation of IHBO.
invades adjacent liver and hepatic vein. Common hepatic duct is involved but cystic and
common bile ducts are not.

o Two types, based on anatomy & radiography

ITERMINOLOGY • Intrahepatic (peripheral/central); extrahepatic
Abbreviations and Synonyms o Intrahepatic
• Cholangiocellular or bile duct adenocarcinoma • Peripheral (intrahepatic bile ducts): May be
exophytic, polypoid or infiltrative
Definitions • Central or hilar (confluence of right & left hepatic
• Malignancy that arises from intrahepatic bile duct ducts and proximal common hepatic duct):
(IHBD) or extrahepatic bile duct epithelium Klatskin tumor (small mass in liver hilus)
o Extrahepatic
• Common duct: Distal common hepatic duct
IIMAGING FINDINGS (CBD)
• May arise as short stricture or small polypoid mass
General Features
• Best diagnostic clue: Klatskin tumor - small hilar mass Radiographic Findings
obstructing bile ducts on CT or ERCP • Cholangiography (PTC/ERCP)
• Location o Exophytic intraductal tumor mass (2-5 mm in
o Distal CBD (30-50%); common hepatic duct diameter)
(14-37%) o Infiltrating type: Frequently long, rarely short
o Proximal CBD (15-30%); confluence of hepatic ducts concentric focal stricture
(10-26%) o Ductal wall irregularities; prestenotic diffuse/focal
o Left & right hepatic duct (8-13%); cystic duct (6%) biliary dilatation
• Size: Intrahepatic mass (5-20 cm); extrahepatic - o Hilar strictures (due to Klatskin tumor): Proximal
smaller bile duct dilatation
• Morphology CT Findings
o 2nd most common primary hepatic tumor after
hepatoma • NECT
o Intrahepatic
o Manifests with various histologic types and growth
patterns • Peripheral-hypodense solitary or satellite lesions &
IHBD dilatation

DDx: Obstruction of Extrahepatic Bile Duct

Pancreatic Carcinoma Chronic Pancreatitis Choledocholithiasis Sclerosing Cholangitis

 CHOLANGIOCARCINOMA

Key Facts
Terminology Pathology
• Cholangiocellular or bile duct adenocarcinoma • Pre-existing bile duct diseases
• Malignancy that arises from intrahepatic bile duct • Inflammatory bowel disease (lOx increased risk)
(IHBD) or extrahepatic bile duct epithelium • One third of all malignancies originating in the liver
Imaging Findings
•
•
Obstructive type: U-IV-shaped obstruction (70-85%)
Ductal sclerosing adenocarcinoma (2/3 cases)
2
• Best diagnostic clue: Klatskin tumor - small hilar mass
obstructing bile ducts on CT or ERCP Diagnostic Checklist 51
• Intrahepatic (peripheral/central); extrahepatic • Rule out other biliary & pancreatic pathologies that
• Capsular retraction may be seen can mimic cholangiocarcinoma by obstructing
extrahepatic bile duct
Top Differential Diagnoses • Cholangiography & MRCP: Usually long & rarely
• Pancreatic carcinoma short focal stricture, irregular ductal wall, stenosis &
• Chronic pancreatitis prestenotic biliary ductal dilatation
• Choledocholithiasis • Klatskin tumor: Small tumor at confluence of
• Primary sclerosing cholangitis (PSC) right/left hepatic & proximal common hepatic ducts
• Porta hepatis tumor

• Central (hilar)-hypodense mass at confluence & o Poor or absent tumor stain

IHBD dilatation o Hepatic artery & portal vein
• Capsular retraction may be seen • Displacement, encasement or occlusion
o Extrahepatic: Common duct
Nuclear Medicine Findings
• Small growth (poorly sensitive)
• Large growth (seen as hypodense mass) & IHBD • Hepato Biliary Scan
dilatation o Cold lesion
• Technetium Sulfur Colloid
• CECT o Cold lesion
o Arterial phase
• Early rim-enhancement with progressive, central • Gallium Scan
patchy enhancement & IHBD dilatation o May show uptake
o Portal phase Imaging Recommendations
• Minimal enhancement of irregular thickened bile • Best imaging tool: MRCP or ERCP
duct wall • Protocol advice: MRCP three-dimensional maximum
• Invasion of portal vein seen with intrahepatic type intensity projection (MIP) reconstruction images
• Enlarged portal lymph nodes may be seen
o Delayed phase
• Persistent enhancing tumor (due to fibrous
stroma)
I DIFFERENTIAL DIAGNOSIS
Pancreatic carcinoma
MR Findings
• Arises from ductal epithelium of exocrine pancreas
• Tl WI: Iso-/hypointense • Irregular, heterogeneous, poorly enhancing mass
• T2WI: Hyperintense periphery (viable) & central
• Abrupt obstruction of pancreatic and/or distal CBD
hypointensity (fibrosis)
o Distal CBD block mimics cholangiocarcinoma
• Tl C+: Superior to CT in detecting small hilar tumors,
• Dilated pancreatic duct & obliteration of
intrahepatic and periductal tumor infiltration
retropancreatic fat
• Tl WI fat suppressed image
• Location: Head (60%), body (20%), tail (15%)
o Shows tumor of intrapancreatic portion of CBD as
• 65% of patients present with advanced local disease &
low signal intensity against high signal intensity
distant metastases
head of pancreas
• MRCP Chronic pancreatitis
o Reveals site & extension of tumor growth
• NECT
o Shows location of obstruction & IHBD dilatation o Focal or diffuse atrophy of gland; calcification
o Dilated main pancreatic duct (MPD) & intraductal
Ultrasonographic Findings
calculi
• Real Time o Intra & peri pancreatic cysts
o Mixed echoic, homo-/heterogeneous mass & dilated
o Thickening of peri pancreatic fascia
IHBD
o Small hypodense focal masses (fat & fibrosis)
o Dilated intra & extrahepatic bile ducts if lesion is in
o Splenic vein thrombosis, splenomegaly, varices
CBD
• CECT: Heterogeneous enhancement
Angiographic Findings • MRCP: Dilated MPD plus radicles; may show long
• Conventional tapered stricture of distal CBD & dilated bile ducts
o Avascular, hypo-/hypervascular
 CHOl4NGIOC~RCINOM4

Choledocholithiasis ICLINICAL ISSUES

• Intra-/extrahepatic bile duct stones
Presentation
• 60-70% stones, increased attenuation (Ca++); 20-30%,
less than water or soft tissue density • Most common signs/symptoms
• "Bullis-eye" sign: Rim of bile surrounding a calcified o Varies with location
stone within duct • Intrahepatic: Pain, palpable mass, weight loss,
2 o Most accurate sign on NECT
• CBD obstruction & intrahepatic duct dilatation
painless jaundice
• Extrahepatic: Pain, enlarged tender liver,
obstructive jaundice, anorexia
52
Primary sclerosing cholangitis (PSC) • Lab data
• Dilatation of both intra- & extrahepatic bile ducts o Increased bilirubin & alkaline phosphatase
• PSC often shows isolated obstructions of IHBDs
Demographics
• ERCP: Skip dilatations, strictures, beading, pruning &
thickening of ductal wall • Age: Peak age: 6-7th decade
• PSC strictures indistinguishable from scirrhous • Gender: M:F = 3:2
infiltrating cholangiocarcinoma Natural History & Prognosis
Porta hepatis tumor • Intrahepatic
• Bulky primary (HCC) & secondary liver tumors o 5 year survival (30%)
• HCC & metastases may invade or obstruct IHBD • Extrahepatic
o Median survival of 5 months
o 5 year survival (1.6%)
I PATHOLOGY • Recurrence after transplantation: Quite common

General Features Treatment

• General path comments
o Almost all cholangiocarcinomas are
adenocarcinomas arising from bile duct epithelium
o Tumor types
• Exophytic intrahepatic masses
• Scirrhous infiltrating neoplasms: Cause stricture
• Polypoid neoplasms of ductal wall: Bulge into bile
duct lumen
o Patterns of dissemination
• Local extension along duct
• Local infiltration of liver
• Spread to lymph nodes
• Etiology
o Pre-existing bile duct diseases
• E.g., biliary lithiasis, clonorchiasis, recurrent
pyogenic cholangitis & psc
o Inflammatory bowel disease (lOx increased risk)
o Caroli disease, Thorotrast exposure
o Familial polyposis & choledochal cyst
• Epidemiology
o More frequent in Asia
o One third of all malignancies originating in the liver
Gross Pathologic & Surgical Features
• Intrahepatic: Mass (5-20 cm)
• Satellite nodules in 65%, biliary calculi
• Extrahepatic (from common duct): Growth pattern
o Obstructive type: U-IV-shaped obstruction (70-85%)
o Stenotic type: Strictured rigid lumen with irregular
margins (10-25%)
o Polypoid or papillary type
• Intraluminal filling defect (5-6%)
Microscopic Features
• Desmoplastic reaction (fibrosis)
• Mucin; glandular & tubular structures
• Most common histologic type
o Ductal sclerosing adenocarcinoma (2/3 cases)
• Surgical resection (less than 20% resectable)
• Radiation; laser therapy & biliary stenting
• Liver transplantation (controversial)
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other biliary & pancreatic pathologies that
can mimic cholangiocarcinoma by obstructing
extrahepatic bile duct
Image Interpretation Pearls
• Cholangiography & MRCP: Usually long & rarely short
focal stricture, irregular ductal wall, stenosis &
prestenotic biliary ductal dilatation
• Klatskin tumor: Small tumor at confluence of right/left
hepatic & proximal common hepatic ducts
I SELECTED REFERENCES
1. Kim YH: Extrahepatic cholangiocarcinoma associated with
clonorchiasis: CT evaluation. Abdom Imaging. 28(1):68-71,
2003
2. Han JK et al: Cholangiocarcinoma: pictorial essay of CT
and cholangiographic findings. Radiographies.
22(1):173-87,2002
3. Campbell WL et al: Using CT and cholangiography to
diagnose biliary tract carcinoma complicating primary
sclerosing cholangitis. AJRAm J Roentgenol.
177(5):1095-100,2001
4. Lee WJ et al: Radiologic spectrum of cholangiocarcinoma:
emphasis on unusual manifestations and differential
diagnoses. Radiographies. 21 Spec No:S97-S116, 2001
5. Maetani Y et al: MR imaging of intrahepatic
cholangiocarcinoma with pathologic correlation. AJRAm J
Roentgenol. 176(6):1499-507,2001
6. Han JK et al: Hilar cholangiocarcinoma: thin-section spiral
CT findings with cholangiographic correlation.
Radiographies. 17(6):1475-85, 1997
 CHOLANGIOCARCINOMA

I IMAGE GALLERY

(Left) Axial CECT shows a

mass (arrows) arising near
the confluence of the main 2
right and left bile ducts. A
biliary stent (open arrow) is
in place, but the IHBD are 53
still dilated. (Right) Klatskin

,-:-:~, :.~"'"
"':;.
}"~~.
,,; C.~",',··· tumor. Cholangiogram
through left hepatic
internal-external stent shows
" marked dilatation of the left
,._~ f' .,:~~~ ~ .~. ,;c:. ~ biliary ductal system, no
filling of the right and normal

",:i\\ .~ ~
~__ 111III' ~:-1 cystic and common bile
ducts.

V ~~ ~~t·

Typical
(Left) Klatskin tumor. Axial
T2WI MR shows massively
dilated ducts ending abruptly

.
- ..
.

~
i:.,.),.".
J~~
'
Ill. -
~
at a hypointense mass
(arrow) at the confluence of
the main hepatic bile ducts.
(Right) Klatskin tumor.

y. ~
MRCP in coronal plane
shows dilated IHBD, normal
CBD (arrow) and pancreatic
duct (open arrow).
•

Typical
(Left) Sagittal oblique
sonogram shows markedly
dilated CBD (arrow)
obstructed by a mass (open
arrow) with homogeneous
echogenicity and no acoustic
shadowing. (Right) ERCP
shows an "apple core"
stricture (arrow) of the
common duct due to
cholangiocarcinoma.
 AMPULLARY CARCINOMA

2
54

Endoscopic image of ampulla demonstrates soft tissue Axial CECT with water distension of duodenum
ampullary carcinoma. demonstrates lobulated, round soft tissue mass (arrow)
arising from ampulla.

ITERMINOlOGY CT Findings
Definitions • CECT: Ampullary mass w/variable attenuation (most
often hypodense) distinct from pancreas w/dilated
• Malignant epithelial neoplasm (adenocarcinoma)
CBD & PD; nodal or liver mets in advanced cases
arising from ampulla of Vater
MR Findings
• T1WI: Isointense with pancreas; low signal on
I IMAGING FINDINGS fat-saturated T1 compared to normal pancreas
General Features • T2WI
o Intermediate signal ampullary mass
• Best diagnostic clue o Dilated main PD and CBD
o Soft tissue mass involving ampulla
• T1 C+: Enhancing soft tissue mass of lower signal than
o "Double duct" sign with obstruction of both pancreas
common bile duct (CBD) and pancreatic duct (PD)
• MRCP
o Lesion best visualized on CECT when duodenum o Dilated PD and CBD
distended with water • Fat suppressed T1Wls
• Location: Ampulla of Vater; medial wall of duodenum o Adenocarcinomas low signal-intensity
• Size: 1-4 cm in diameter; mean 2.7 cm o Low signal-intensity compared to normal-enhancing
• Morphology: Often lobulated mass pancreas following gadolinium administration and
Radiographic Findings breathheld gradient echo imaging
• Fluoroscopy: UGI: Filling defect in second part of Ultrasonographic Findings
duodenum in region of ampulla of Vater • Real Time
• ERCP o Dilated CBD and PD
o Visible ampullary mass o Ampullary mass usually not visible
o Obstruction of CBD and PD oLiver mets in advanced cases
o Useful for biopsy • Color Doppler: No flow in dilated hypoechoic CBD
andPD

DDx: Spectrum of Ampullary

.,. lesions Mimicking Carcinoma

,: ,,~
~
.
·A

:J.
.

"., '..
,,
' , ...
..

w
Pancreatic CA Duodenal Adenoma Amp Schwan noma CBD Stone
 AMPlJLLARY CARCINOMA

Key Facts
Terminology Pathology
• Malignant epithelial neoplasm (adenocarcinoma) • Associated abnormalities: Familial adenomatosis coli,
arising from ampulla of Vater Gardner syndrome, colon cancer
Imaging Findings Clinical Issues
• Soft tissue mass involving ampulla • Jaundice (71%), weight loss (61%), back pain (46%) 2
• "Double duct" sign with obstruction of both common are most common symptoms
bile duct (CBD) and pancreatic duct (PD) • Prognosis: 5-year survival rate 38% in resected 55
• CECT: Ampullary mass w/variable attenuation (most patients
often hypodense) distinct from pancreas w/dilated
CBD & PD; nodal or liver mets in advanced cases Diagnostic Checklist
• Duodenal distension with water on CECT key to
Top Differential Diagnoses identifying lesion; ampullary mass and double duct
• Pancreatic head carcinoma invading ampulla sign also key indicators
• Adenoma of ampulla • Perform dedicated pancreatic protocol when
• Mesenchymal tumor of ampulla ampullary lesion suspected
• Duodenal carcinoma (adenocarcinoma)

• Endoscopic US
o Useful for staging and biopsy Mesenchymal tumor of ampulla
o Detection of nodal mets • May be hypervascular ampullary mass on CT if
neurogenic, i.e., schwannoma or carcinoid
Angiographic Findings • High T2 signal on MR if neurogenic origin
• Conventional: Superselective injection of
gastroduodenal artery demonstrates hypovascular Duodenal carcinoma (adenocarcinoma)
mass • Soft tissue mass arising from duodenal mucosa
secondarily invading ampulla and/or pancreas
Nuclear Medicine Findings • May not have "double duct" sign
• PET:May demonstrate liver mets • May present with gastrointestinal bleeding
• Hepato Biliary Scan
o Dilated bile ducts
Imaging Recommendations
I PATHOLOGY
• Best imaging tool: CECT with dedicated biphasic General Features
pancreatic protocol • General path comments: Lobulated or infiltrating
• Protocol advice mass (adenocarcinoma) arising from ampulla of Vater
o Patient to drink 16 oz water immediately prior to CT • Genetics: No known association
o Arterial phase acquisition: Rapid bolus injection of • Etiology: Unknown adenocarcinoma arising from
150 ml IV contrast (4-5 ml!sec); 1.25 mm adenomatous epithelium of ampulla
collimation after 10 sec delay • Epidemiology
o Venous phase acquisition at 70 seconds with 5 mm o Associated with history of smoking (30%) and
collimation diabetes (17%)
o Reconstruct pancreas images with 20 cm field of o Rare tumor representing 0.2% of GI tract
view malignancies
o Additional reformations including curved planar • Associated abnormalities: Familial adenomatosis coli,
reformat of PD and CBD useful Gardner syndrome, colon cancer
Gross Pathologic & Surgical Features
I DIFFERENTIAL DIAGNOSIS • Lobulated soft tissue mass arising from ampulla of
Vater
Pancreatic head carcinoma invading ampulla
• Hypoattenuating mass on late arterial phase CECT Microscopic Features
• Obstructed CBD and PD • Malignant ductal epithelial cells; varying degrees of
• Mass on fat-saturated T1 and after IV gadolinium differentiation and necrosis
• Intestinal type
Adenoma of ampulla o Simple and cribriform glands present with
• Indistinguishable from carcinoma on CT pseudo stratified oval nuclei with varying degrees of
• "Double duct" sign with dilated CBD and PD nuclear atypia
• Variable in size, from 1-5 cm • Pancreatobiliary type
o Similar histology to ductal adenocarcinoma with
single layer of round markedly atypical nuclei,
micropapillary areas
 AMPULLAR}' CARCINOMA
• Spectrum of histology: Dysplasia, CA in situ, frank
adeno CA
Staging, Grading or Classification Criteria
• TNM staging system related to nodal and distant
metastases
o Nodal metastases outside of peripancreatic region
ISELECTED REFERENCES
1. Martin]A et al: Ampullary adenoma: clinical
manifestations, diagnosis, and treatment. Gastrointest
Endosc Clin N Am. 13(4):649-69,2003
2. Duffy ]P et al: Improved survival for adenocarcinoma of
the ampulla of Vater: fifty-five consecutive resections. Arch

2 considered M1lesion
• T1: Lesion confined to ampulla
3.
Surg. 138(9):941-8; discussion 948-50, 2003
Clements WM et al: Ampullary carcinoid tumors: rationale
for an aggressive surgical approach. ] Gastrointest Surg.
56 • T2: Tumor invading duodenal wall 7(6):773-6,2003
• T3: Pancreatic invasion < 2 cm deep 4. Smith TR et al: Prolapse of the common bile duct with
• T4: Pancreatic invasion> 2 cm deep small ampullary villous adenocarcinoma into third part of
the duodenum. A]R Am] Roentgenol. 181(2):599-600,
2003
I CLINICAL ISSUES 5. Lindell G et al: Management of cancer of the ampulla of
Vater: does local resection playa role? Dig Surg.
Presentation 20(6):511-5,2003
6. Nakano K et al: Combination therapy of resection and
• Most common signs/symptoms intraoperative radiation for patients with carcinomas of
o Jaundice (71%), weightloss (61%), back pain (46%) extrahepatic bile duct and ampulla of Vater: prognostic
are most common symptoms advantage over resection alone? Hepatogastroenterology.
o Other signs/symptoms 50(52):928-33, 2003
• Gastrointestinal bleeding with heme positive stool 7. Trimbath]D et al: Attenuated familial adenomatous
• Clay-colored stool polyposis presenting as ampullary adenocarcinoma. Gut .
• Fever and chills from cholangitis 52(6):903-4,2003
• Clinical profile 8. Handra-Luca A et al: Adenomyoma and adenomyomatous
hyperplasia of the Vaterian system: clinical, pathological,
o Elevated bilirubin and alkaline phosphatase
and new immunohistochemical features of 13 cases. Mod
o May have both elevated CEA or CA19-9 tumor Pathol. 16(6):530-6,2003
markers 9. Kim]H et al: Differential diagnosis of periampullary
• Elevated pre-operative tumor markers associated carcinomas at MR imaging. Radiographies. 22(6):1335-52,
with poor outcome 2002
10. Kaiser A et al: The adenoma-carcinoma sequence applies to
Demographics epithelial tumours of the papilla of Vater. Z Gastroenterol.
• Age: Mean age 6S years 40(11):913-20, 2002
• Gender: M:F = 2: 1 11. Rodriguez C et al: How accurate is preoperative diagnosis
• Ethnicity: No known ethnic predilection by endoscopic biopsies in ampullary tumours? Rev Esp
Enferm Dig. 94(10):585-92, 2002
Natural History & Prognosis 12. Irie H et al: MR imaging of ampullary carcinomas.]
• Depends on nodal and distal metastases at time of Comput Assist Tomogr. 26(5):711-7, 2002
13. Jordan PH]r et al: Treatment of ampullary villous
presentation
adenomas that may harbor carcinoma. ] Gastrointest Surg.
• Prognosis: S year survival rate 38% in resected patients 6(5):770-5,2002
o Best survival in patients with negative surgical 14. Skordilis P et al: Is endosonography an effective method
margins, negative nodes and well-differentiated for detection and local staging of the ampullary
tumors carcinoma? A prospective study. BMC Surg. 2(1):1, 2002
15. Yoshida T et al: Hepatectomy for liver metastasis from
Treatment ampullary cancer after pancreatoduodenectomy.
• Pancreatoduodenal resection (Whipple procedure) in Hepatogastroenterology. 49(43):247-8, 2002
good operative risk patients 16. Eriguchi N et al: Carcinoma of the ampulla of Vater
• Local resection prone to recurrence of tumor associated with other organ malignancies. Kurume Med].
48(4):255-9, 2001
17. Nikfarjam M et al: Local resection of ampullary

I DIAGNOSTIC CHECKLIST
adenocarcinomas of the duodenum. ANZ] Surg.
71(9):529-33,2001
18. Hirata S et al: Periampullary choledochoduodenal fistula in
Consider ampullary carcinoma. ] Hepatobiliary Pancreat Surg.
• Primary pancreatic cancer invading ampulla 8(2):179-81,2001
19. Wittekind C et al: Adenoma of the papilla and
Image Interpretation Pearls ampulla--premalignant lesions? Langenbecks Arch Surg.
• Duodenal distension with water on CECT key to 386(3):172-5,2001
identifying lesion; ampullary mass and double duct 20. Wagle PK et al: Pancreaticoduodenectomy for
sign also key indicators periampullary carcinoma. Indian] Gastroenterol.
20(2):53-5, 2001
• Perform dedicated pancreatic protocol when
21. Yeo C] et al: Periampullary adenocarcinoma. Ann Surg
ampullary lesion suspected
227(6): 821-31, 1998
22. Talamini MA et al: Adenocarcinoma of the ampulla of
Vater. Ann Surg 225(5): 590-600, 1997
 AMPULLARY CARCINOMA
I IMAGE GALLERY

(Left) Coronal CECT curved

planar reformation of
common bile duct shows
ampullary CA invading head
of pancreas. Note
2
hypodense mass obstructing 57
pancreatic and common bile
ducts (arrow). (Right)
Coronal CECT minimum
intensity image of ampullary
CA invading head of
pancreas demonstrates
hypodense mass (arrow).

Typical
(Left) Anteroposterior spot
film from upper CI series
demonstrates rounded filling
defect from ampullary CA
(arrow). (Right) Coronal
CECT curved planar
reformation of pancreatic
duct demonstrates very small
ampullary tumor (arrow)
obstructing pancreatic &
common bile ducts.

(Left) Axial CECT thick slab

image demonstrates
ampullary CA with an
unusual degree of increased
vascularity (arrow). (Right)
Coronal CECT of ampullary
CA demonstrates marked
common bile duct
obstruction secondary to
mass (arrow).
 IPMT,BILIARY

2
58

Schematic drawing of biliary /PMT. Note segmental Axial CECT of biliary /PMT. Note marked distended
dostension of right lobe intra-hepatic ducts filled with intrahepatic ducts with "aneurysmal" dilatation of left
mucin and containing mucosal mass arising from ductal intrahepatic duct (arrow) distended with mucin.
epithelium.

ITERMINOlOGY CT Findings
Abbreviations and Synonyms • CECT: Markedly dilated intra- or extrahepatic bile
ducts with intraluminal enhancing fungating mass
• Intraductal papillary mucinous tumor of biliary ducts
(IPMT) MR Findings
Definitions • T2WI: Markedly dilated high signal bile duct with low
signal intraluminal filling defects
• Mucin-producing papillary neoplasm of biliary mucosa
• MRCP
o Diffuse or segmental biliary ductal dilatation
o Intraductal filling defects
I IMAGING FINDINGS
Ultrasonographic Findings
General Features
• Real Time
• Best diagnostic clue: Diffuse segmental "aneurysmal" o Complex "mass" of aneurysm ally dilated bile ducts
dilation of bile ducts with polypoid or nodular o Echogenic intraductal masses; anechoic mucin
intraductal mass • Color Doppler: No flow in dilated bile ducts
• Location: Intra- or extrahepatic bile ducts
• Size: Marked dilation of bile ducts Imaging Recommendations
• Morphology: "Aneurysmal" dilatation of • Best imaging tool: CECT, MRCP, US
mucin-distended ducts • Protocol advice: 150 ml contrast injected at 2.5 ml!sec;
5 mm collimation; 5 mm reconstruction; use 1.25-2.5
Radiographic Findings mm reconstructions for improved reformations
• ERCP
o Dilated ducts with intraluminal filling defects
representing mucin plugs or tumor
o Segmental ductal obstruction
I DIFFERENTIAL DIAGNOSIS
Cholangiocarcinoma
• Polypoid intraductal mass; biliary stricture; not mucin
producing

DDx: Hepato-Biliary lesions Mimicking IPMT

.. .

...,,'A·
-~ '

Cholangiocarcinoma RPC
~
- .
Liver Abscess Bact Cholangitis
 IPMT, BILIARY

Key Facts
Terminology • CECT: Markedly dilated intra- or extrahepatic bile
• Mucin-producing papillary neoplasm of biliary ducts with intraluminal enhancing fungating mass
mucosa • Protocol advice: 150 ml contrast injected at 2.5
ml/seci 5 mm collimationi 5 mm reconstructioni use
Imaging Findings 1.25-2.5 mm reconstructions for improved
• Best diagnostic clue: Diffuse segmental "aneurysmal" reformations 2
dilation of bile ducts with polypoid or nodular
intraductal mass Clinical Issues 59
• Most common signs/symptoms: Intermittent
abdominal pain, fever, chills, jaundice

Recurrent pyogenic cholangitis (RPC) Natural History & Prognosis

• Intrahepatic & common bile duct (CBD) stonesi
markedly dilated intra- or extrahepatic ducts
Pyogenic liver abscess
• "Cluster sign" of low attenuation locules of abscessi no
dilated bile ducts, mucin production or intraductal
mass
• Repeated episodes of cholangitis
• Good prognosis for adenomas or dysplasiai invasive
CA prognosis depends on nodal status, presentation
Treatment
• Surgical resection of involved lobe or segment curative
for adenoma of dysplasia

Bacterial cholangitis
• Pus within dilated bile ductsi low-level echoes in CBDi I DIAGNOSTIC CHECKLIST
thickened CBD walls
Consider
• Cholangiocarcinoma
I PATHOLOGY Image Interpretation Pearls
General Features • Aneurysmal dilation of segmental bile ducts with
• General path comments nodular enhancing intraductal tumor
o Extensive mucin formation in dilated ducts
o Nodular intraductal tumors
• Genetics: No known genetic disposition I SELECTED REFERENCES
• Etiology: Unknown 1. Lim JH et al: Intraductal papillary mucinous tumor of the
• Epidemiology: Adult East Asians bile ducts. Radiographies. 24(1):53-66; discussion 66-7,
• Associated abnormalities: Rupture with intraperitoneal 2004
mucinous mass 2. Sugiyama M et al: Magnetic resonance
cholangiopancreatography for postoperative follow-up of
Gross Pathologic & Surgical Features intraductal papillary-mucinous tumors of the pancreas. Am
• Markedly distended mucin-filled bile ducts with J Surg. 185(3):251-5,2003
frond-like papilloma 3. Oshikiri T et al: Mucin-secreting bile duct
adenoma--clinicopathological resemblance to intraductal
Microscopic Features papillary mucinous tumor of the pancreas. Dig Surg.
• Mucinous papillary tumor of bile duct mucosa 19(4):324-7, 2002
o Columnar epithelial cells arranged in innumerable
papillary fronds distending bile ducts with mucin
• Spectrum: Adenomatous dysplasia to frank invasive I IMAGE GALLERY
adenocarcinoma
• Multiple tumors: Papillomatosis

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Intermittent
abdominal pain, fever, chills, jaundice
• Clinical profile: Asian patient with recurrent episodes
of abdominal pain and fever
Demographics
• Age: Fifth through seventh decade (Left) Left bile duct IPMT on gadolinium-enhanced GRE axial image.
• Ethnicity: Eastern Asia Note marked dilatation of left bile ducts. (Right) Left bile duct IPMT
on transverse sonogram. Note distended bile duct with echogenic
mucin (arrow).
 CHEMOTHERAPY CHOLANGITIS
2
60
Transhepatic cholangiogram in patient who had
intra-arterial chemotherapy through infusion pump
catheter (open arrow); dilated right IHBD with multiple
central biliary strictures (arrow).
ITERMINOlOGY
Definitions
• Iatrogenic cholangitis following intra-arterial
chemotherapy for treatment of primary hepatic
tumors and metastases
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Clinical setting; if tumor
progression excluded by CT, MR, US or other means
• Location
o Strictures of common hepatic duct are characteristic
o Strictures frequently involve biliary bifurcation
o Intrahepatic duct strictures are less common
o Sparing of distal common bile duct; hallmark
o Gallbladder & cystic duct may be involved
o Distribution of strictures in biliary tree reflects
hepatic arterial supply to bile ducts
• Morphology: Extrahepatic biliary sclerosis; common
hepatic duct stenosis or complete obstruction;
intrahepatic bile duct (IHBD) dilatation without a
well-recognized obstruction
Radiographic Findings
• ERCP
DDx: Irregularly Dilated Bile Ducts
Sclerosing Cholangitis Ascending Cholangitis
Axial CECT in patient who had right hepatectomy for
metastatic colon cancer. Mildly dilated IHBD are noted
while some focally dilated ducts (arrows) could be
mistaken for metastases.
o Abnormalities range from minimal duct wall
irregularity to marked duct wall thickening with
near obliteration of lumen
CT Findings
• CT may be necessary to differentiate cholangitis from
extrinsic duct compression by lymph nodes or tumor
• Shows mildly dilated IHBD
Other Modality Findings
• Cholangiography: Segmental strictures of variable
length (similar to those seen in primary sclerosing
cholangitis)
Imaging Recommendations
• Best imaging tool: CT & ERCP; may be used in concert
to differentiate this entity from other causes of
jaundice (hepatic replacement by tumor, porta hepatis
adenopathy & chemotherapy hepatotoxicity)
I DIFFERENTIALDIAGNOSIS
Primary sclerosing cholangitis (PSC)
• Skip dilatations, stenosis, beading, pruning, irregular
thickening of wall of intra- & extrahepatic bile ducts
o Duct beading & intrahepatic duct involvement are
relatively less common in chemotherapy cholangitis
Iatrogenic Biliary Injury Metastases
 CHEMOTHERAPY CHOLANGITIS
Key Facts
Terminology Top Differential Diagnoses
• Iatrogenic cholangitis following intra-arterial • Primary sclerosing cholangitis (PSC)
chemotherapy for treatment of primary hepatic • Ascending cholangitis
tumors and metastases • Iatrogenic biliary injury
Imaging Findings
• Extrinsic compression (liver masses)
2
• Strictures of common hepatic duct are characteristic Pathology
• Strictures frequently involve biliary bifurcation • Ischemic cholangiopathy 61
• Sparing of distal common bile duct; hallmark • Floxuridine (FUDR): Offending agent in most cases

• Gallbladder & cystic duct are usually more severely

involved in chemotherapy cholangitis than in PSC
Ascending cholangitis
• Presence of bile duct stones; prior sphincterotomy
Iatrogenic biliary injury
• Such as common hepatic duct injury during
laparoscopic cholecystectomy
Extrinsic compression (liver masses)
• May compress common hepatic duct
I PATHOLOGY
General Features
• Etiology
o Ischemic cholangiopathy
o Direct chemical toxic effects of drug on duct &/or
occlusion of peribiliary vascular plexus with
resultant biliary fibrosis
o Floxuridine (FUDR): Offending agent in most cases
• Epidemiology: Cholangiographic abnormalities
reported in 7-30% of patients undergoing intra-arterial
chemotherapy
• Associated abnormalities: Aneurysm or
pseudoaneurysm of hepatic artery at infusion site,
pancreatitis, cholecystitis, gallbladder perforation
Treatment
• Immediate cessation of intra-arterial FUDR; surgical or
percutaneous drainage of biliary tree; balloon dilation
of stricture
• In rare cases liver transplantation may be indicated
despite a history of metastasizing carcinoma
I DIAGNOSTIC CHECKLIST
Consider
• When clinical signs of hepatic dysfunction occur in
absence of tumor progression, biliary sclerosis from
chemotherapy must be suspected
I SELECTED REFERENCES
1. Aldrighetti L et al: Extrahepatic biliary stenoses after
hepatic arterial infusion (HAl) of floxuridine (FUdR) for
liver metastases from colorectal cancer.
Hepatogastroenterology. 48(41):1302-7, 2001
Shea W] ]r et al: Sclerosing cholangitis associated with
2.
hepatic arterial FUDR chemotherapy:
radiographic-histologic correlation. A]R Am] Roentgenol.
146(4):717-21,1986
3. Botet]F et al: Cholangitis complicating intraarterial
chemotherapy in liver metastasis. Radiology. 156(2):335-7,
1985

I CLINICAL ISSUES I IMAGE GALLERY

Presentation
• Obstructive, severe, persistent jaundice
• Lab: Hyperbilirubinemia with progressively elevated
serum glutamic-pyruvic transaminase & alkaline
phosphatase
Natural History & Prognosis
• Extrahepatic biliary stenosis may represent primary
event leading to secondary intrahepatic biliary damage
that does not correlate with specific floxuridine
toxicity but results from bile stasis & infection,
recurrent cholangitis & eventually biliary sclerosis
• Evolution to biliary cirrhosis is sometimes possible (Left) Right transhepatic cholangiogram shows dilated IHBD. High
• Complications: Ischemic cholecystitis, if grade central biliary strictures developed after intra-arterial
chemoembolization particles injected "upstream" from chemotherapy with FUDR for metastatic colon carcinoma to the liver.
origin of cystic artery (Right) Axial CECT shows centrally dilated IHBD (arrow) in patient
• Severe complications: Acute hepatic failure or death with metastatic colon carcinoma (curved arrow) who had undergone
intra-arterial chemotherapy.
• Mortality rate: 1%
 SECTION 3: Pan«:reas

Introduction and Overview

Pancreas Anatomy and Imaging Issues 11-3-2

Normal Variants and Congenital

Agenesis of Dorsal Pancreas 11-3-6
Annular Pancreas 11-3-8
Pancreas Divisum 11-3-12
EctopicPancreatic Tissue 11-3-16

Inflammation
Cystic Fibrosis, Pancreas 11-3-18
Acute Pancreatitis 11-3-20
Pancreatic Pseudocyst 11-3-24
Chronic Pancreatitis 11-3-28

Trauma
Traumatic Pancreatitis 11-3-32

Neoplasm, Benign
Serous Cystadenoma of Pancreas 11-3-36
Pancreatic Cysts 11-3-40

Neoplasm, Malignant
Mucinous Cystic Pancreatic Tumor 11-3-42
IPMT, Pancreas 11-3-46
Pancreatic Ductal Carcinoma 11-3-50
Pancreatic Islet Cell Tumors 11-3-54
Solid and Papillary Neoplasm 11-3-58
Pancreatic Metastases 11-3-60
 PANCREAS ANATOMY AND IMAGING ISSUES
3
2
Graphic shows the major venous anatomy relevant to
the pancreas. The splenic vein lies in a groove on the
dorsal surface of the pancreas. LGV + SGV = left and
short gastric veins.
I IMAGING ANATOMY
Location
• Pancreas lies in the anterior pararenal space (APRS)
• Size of pancreas varies among population and in
individuals
o Tends to atrophy with age> 70, and pancreatic duct
dilates
o Pancreatic head is widest part; neck narrowest
(ventral to mesenteric vessels); gentle taper to tail
I ANATOMY-BASED IMAGING ISSUES I
Key Concepts or Questions
• Advantages and disadvantages of CT vs. MR for
pancreatic evaluation
o MR and CT have similarly high accuracy in
experienced hands
o MR does not require iodinated contrast
administration (but should be performed with bolus
injection of gadolinium contrast with multiphasic
imaging of liver and pancreas
o MR allows easy evaluation of common bile duct and
pancreatic duct (using MRCP sequences)
o CT shows calcifications, generally less prone to
technical and interpretive errors, faster, more
available, more practical for acutely ill patients
• Which MR sequences are most useful for evaluation of
a known or suspected pancreatic tumor?
o Volume-acquired 3D fat-suppressed gradient echo
sequences; with and without bolus dynamic
Gd-enhancement
• What CT protocol should be used for
known/suspected pancreatic tumor?
o Nonenhanced images through liver and pancreas:
Can aid detection of some hypervascular tumors and
calcifications
o Pancreatic phase images (35-45 sec delay after
initiation of bolus of 125 ml contrast at 4 ml/sec
Axial pancreatic (top) and hepatic (bottom) phase
CECT shows hypervascular islet cell tumor with liver
metastases (arrows), much less obvious on hepatic
phase image.
• Through liver and pancreas: Allows detection of
hypervascular (e.g., islet cell) and hypovascular
(e.g., pancreatic ductal) carcinomas
• With multidetector-row CT, acquire 1 to 2 mm
thick sections to facilitate CT angiography and 3D
displays of ductal anatomy: Display axial section
as 5 mm thick
o Hepatic parenchymal phase images (70 sec delay)
• 5 mm thick images; best for hypovascular liver
metastases, venous and nodal anatomy
o Water as oral contrast medium
• Facilitates detection of hypervascular tumor in or
near duodenum (e.g., gastrinoma), and improves
quality of 3D reformations, because dense oral
contrast medium would interfere with vessel
display
• What CT protocol should be used for "acute
pancreatitis"?
o The same "pancreatic tumor protocol" can be used,
but it is usually sufficient to use an "acute abdomen
protocol" (iodinated oral contrast medium; all scans
obtained after IV administration of 125 ml of
contrast at 3 ml/sec; 5 mm thick contiguous
sections, 70 sec delay)
• Is there a role for ultrasonography in patient suspected
of having pancreatic tumor?
o Ultrasonography, especially endoscopic US, can
reveal some small tumors that are not apparent on
CT or MR
o Intra-operative US is often useful to detect small islet
cell tumors and to minimize surgical manipulation
of the pancreas
o Ultrasonography is often used to guide percutaneous
(or endoscopic) biopsy of pancreatic tumors
• What criteria can be used to distinguish pancreatitis
from carcinoma?
 PANCREAS ANATOMY AND IMAGING ISSUES
DIFFERENTIAL DIAGNOSIS
Tumors of exocrine pancreas
Common
• Ductal adenocarcinoma
Uncommon
• Mucinous cystic neoplasm
• Intraductal papillary mucinous tumor (IPMT)
• Serous (microcystic) adenoma
Rare
• Cystic and solid (pseudopapillary) neoplasm
• Acinar cell carcinoma
• Pancreatoblastoma
• Mature cystic teratoma
o Pancreatic ductal carcinoma is a scirrhous
hypovascular mass best detected on bolus enhanced
CT (or MR) and typically causes narrowing or
obstruction of vessels and ducts, and extends
dorsally to the celiac and superior mesenteric arterial
origins
o Acute pancreatitis causes fluid exudation and fat
infiltration, extending ventrally and laterally into
the mesentery and anterior pararenal space
o Necrotizing pancreatitis may result in a
nonenhancing pancreatic "mass", but there are
multiple other radiographic and clinical signs of
pancreatitis in these patients
o Chronic pancreatitis, like carcinoma, may result in
pancreatic head enlargement, ductal dilation, and
(rarely) infiltration of perivascular planes: The
presence of lymph node or liver metastases or
biopsy may be necessary for diagnosis of carcinoma
• How vigorously and by what means should a cystic
pancreatic mass be investigated?
o If clinical signs, symptoms, and lab tests support a
diagnosis of pseudocyst, merely monitor (CT or
sonography) for stability and resolution
o If incidental, small « 2 cm) lesion in elderly patient
or patient with limited « 10 years) life expectancy,
can usually ignore as insignificant
o If lesion has characteristic features of serous
cystadenoma and causes few symptoms, continued
surveillance at 6 to 12 month intervals (CT, MR or
US)
o If lesion has characteristic features of side-branch
IPMT (intraductal papillary mucinous tumor),
confirm with ERCP and surveillance with CT or MR
o If pancreatic cysts are asymptomatic and are
discovered as part of a multisystem syndrome
disorder (e.g., polycystic disease, von Hippel
Lindau), no further evaluation unless cyst is
complex or growing
• Consider possibility of pseudoaneurysm and
obtain color Doppler US, contrast-enhanced CT or
MR, or angiography
• Consider the possibility of metastases in patient
with a known tumor
Tumors of endocrine pancreas
• Islet cell (neur?endocrine) tumor
• Small cell carCInoma
Nonepithelial tumors
• Soft tissue tumors (neurofibroma, etc.)
• Lymphoma
Hypervascular pancreatic mass(es)
• Islet cell (neuroendocrine) tumor
• Metastases
• => Especially renal cell carcinoma, melanoma
3
3
o Most other pancreatic cystic masses should be
regarded as malignant or premalignant and surgical
exploration should be considered
CLINICAL IMPLICATIONS
Clinical Importance
• Location in the anterior pararenal space
o Duodenum also located n APRS:Pancreatitis and
pancreatic carcinoma may result in duodenal wall
thickening, luminal narrowing or obstruction, or an
intramural mass (tumor or pseudocyst)
o Ascending and descending colon also located in
APRS:Lateral spread of inflammation from
pancreatitis may result in "colon cut-off sign" due to
spasm of the proximal descending colon: Rightward
spread of inflammation can affect the ascending
colon and clinically simulates acute appendicitis or
colitis
• Location adjacent to lesser sac: Pancreas is separated
from the lesser sac (omental bursa) by only the
posterior parietal peritoneum
o Acute pancreatitis often results in fluid exudation
into lesser sac which may be mistaken for a
pseudocyst: These acute fluid collection usually
resolve quickly, while pseudocysts take longer to
develop and resolve, and have a fibro-inflammatory
wall
• Position of pancreatic tail within the splenorenal
ligament
o The tail of the pancreas constitutes only the distal
few centimeters of gland and lies intraperitoneally:
Acute inflammation of the pancreatic tail may result
in an intra splenic pseudocyst and pancreatic ascites
• Islet cell tumors may be "functioning" (excess
hormone-secreting) or not
o Nonfunctioning tumors remain asymptomatic until
large, and are frequently invasive and metastatic
o Functioning tumors result in symptoms and permit
earlier diagnosis
o Insulinoma (solitary, small, symptomatic
hypoglycemia)
 PANCREAS ANATOMY AND IMAGING ISSUES

3
4

Graphic shows ventral pancreatic anlagen developing
as an outpouching of the hepatic-biliary diverticulum.
As the stomach and duodenum elongate, the ventral
pancreas and bile ducts rotate clockwise and posteriorly
to fuse with the dorsal pancreas.
Axial CECT shows notmal variant, ventral pancreas
(head/uncinate) with greater fatty lobulation than dorsal
pancreas (body/tail), simulating a pancreatic head
mass.

o Gastrinoma (Zollinger-Ellison syndrome) o Necrotic pancreatic ductal carcinoma

o Duodenal diverticulum
• Uncommon
EMBRYOLOGY o Simple cyst
o Congenital polycystic conditions
Embryologic Events • von Hippel Lindau, polycystic disease, CF
• The body-tail segment of the pancreas developed from o Cystic and solid (pseudopapillary) tumor
the embryologic dorsal pancreatic bud, while the o Lymphangioma
head-uncinate segment develops from the o Metastases (cystic, squamous or sarcoma)
embryologic ventral bud which also gives rise to the o Choledochal cyst
biliary tree
• During normal development, the ventral bud migrates Hypovascular pancreatic mass
clockwise around the fetal duodenum and eventually • Common
merges with the dorsal bud to form the pancreas with o Pancreatic ductal carcinoma
the branching pancreatic and bile ducts • Uncommon
o Metastases/lymphoma
Practical Implications o Chronic pancreatitis
• Failure or anomalies of rotation and fusion may result o Islet cell tumor (usually hypervascular)
in annular pancreas, pancreas divisum, agenesis of • Rare
dorsal pancreas o Pancreaticoblastoma
• The ventral (head-uncinate) and dorsal (body-tail) o Acinar cell carcinoma
segments may have a different "texture" in adults that o Small cell carcinoma
may be misinterpreted as pancreatic pathology o Stromal tumors (neurofibroma, etc.)

I CUSTOM DIFFERENTIAL DIAGNOSISI SELECTED REFERENCES

Dilated pancreatic duct 1. Koeller KK,et al (eds): Radiologic Pathology (2nd ed).
Washington, D.C., Armed Forces Institute of Pathology,
• Chronic pancreatitis 2003
• Pancreatic ductal carcinoma 2. McNulty NJ et al: Multi--detector row helical CT of the
• Ampullary tumor pancreas: effect of contrast-enhanced multiphasic imaging
• Intraductal papillary mucinous tumor (IPMT) on enhancement of the pancreas, peripancreatic
• Distal common duct stone vasculature, and pancreatic adenocarcinoma. Radiology.
• Advanced age 220(1):97-102,2001
3. Diihnert W: Radiologic Review Manual (4th ed).
Cystic pancreatic mass Philadelphia. Lippincott, Williams and Wilkins, 2000
• Common 4. Fukuoka K et al: Complete agenesis of the dorsal pancreas.
o Pseudocyst J Hepatobiliary Pancreat Surg. 6(1):94-7, 1999;6(1):94-7
o Mucinous cystic tumor 5. Gore RM: Pancreas: Differential Diagnosis. In Gore RM,
Levine MS (eds) Textbook of Gastrointestinal Radiology
o IPMT
(2nd ed.) Philadelphia, WB Saunders, 2000, pp 1836-41
o Serous cystadenoma
 PANCREAS ANATOMY AND IMAGING ISSUES

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
dilated pancreatic duct and
glandular atrophy due to
IPMT. Note bulging papilla
(arrow). (Right) Axial CECT
shows dilated PO, glandular
atrophy, and ductal calculi,
due to chronic pancreatitis.

3
5

Typical
(Left) Axial CECT shows
lobulated, septated cystic
mass in pancreatic body due
to mucinous cystic tumor.
(Right) Axial CECT shows
hypervascular pancreatic
masses (arrows) due to
metastatic renal cell
carcinoma.

Typical
(Left) Axial CECT shows
heterogeneous hypovascular
mass in pancreatic head and
perivascular invasion;
pancreatic carcinoma.
(Right) Axial CECT shows
heterogeneous hypovascular
mass in pancreatic head and
calcifications; chronic
pancreatitis.
 AGENESIS OF DORSAL PANCREAS

3
6

Axial CECT shows pancreatic head with focal ERCPshows fillingof ventralpancreatic duct within the
calcifications indicative of chronic pancreatitis. head and uncinate process only. Note normal tapering
Glandular tissue and duct were completely absent in of the pancreatic duct and its side branches.
the body-tail segment.

• Visualized ducts: Taper normally, small in caliber,

ITERMINOLOGY confined to head of pancreas
Abbreviations and Synonyms CT Findings
• Complete or partial pancreatic agenesis; pancreatic • Short pancreas; normal pancreatic head; absence of
aplasia/hypoplasia tail with complete or partial absence of body
Definitions MR Findings
• Agenesis of dorsal pancreas is a rare congenital • MRCP: In complete agenesis of dorsal pancreas,
anomaly which results from defective pancreas accessory & dorsal duct system are not observed
formation
Ultrasonographic Findings
• Pancreatic head in normal location with
I IMAGING FINDINGS nonvisualization of body & tail
• Limited value: Pancreatic nonvisualization may be due
General Features to overlying bowel gas or other technical factors
• Best diagnostic clue: Absence of pancreatic tissue in
expected location of neck, body & tail Angiographic Findings
• No feeding arteries for pancreatic body or tail
Radiographic Findings
• ERCP: Filling of ventral duct without identifiable Imaging Recommendations
dorsal ducts or minor papilla in agenesis of entire • Best imaging tool: CT & MRCP or ERCP
dorsal pancreas
• Hypoplasia: Main pancreatic duct is shortened, some
dorsal ducts remain, as evidenced by filling of I DIFFERENTIAL DIAGNOSIS
accessory duct of Santorini
o Minor papilla may be present in these cases; Pancreatic carcinoma
indicates only portions of dorsal pancreas failed to • Abrupt obstruction of pancreatic duct
develop • Irregular, nodular, rat-tail eccentric obstruction
• Localized encasement with prestenotic dilatation

DDx: Obstruction of Pancreatic Duct

Pancreatic Carcinoma Pancreas Divisum Pancreas Divisum Chronic Pancreatitis

 AGENESIS OF DORSAL PANCREAS
Key Facts
Terminology
• Complete or partial pancreatic agenesis; pancreatic
aplasia/hypoplasia
Imaging Findings
• Best diagnostic clue: Absence of pancreatic tissue in
expected location of neck, body & tail
• Short pancreas; normal pancreatic head; absence of
tail with complete or partial absence of body
Pancreas divisum
• Contrast injection into major papilla demonstrates
only short ventral duct with normal tapering
• Contrast injection into minor papilla fills dorsal duct
• No communication between ventral + dorsal ducts
Chronic pancreatitis
• Dilated & beaded main pancreatic duct + radicles;
intraductal calculi
I PATHOLOGY
General Features
• General path comments: Familial occurrence of
agenesis of dorsal pancreas suggests hereditary
mechanisms may playa role in pathogenesis
• Etiology
o Defect of dorsal pancreatic bud (anlage)
o Dorsal bud develops from foregut & extends into
dorsal mesentery; forms body, tail, anterior head
• Duct of dorsal pancreas becomes main pancreatic
duct & distal to site of fusion, it becomes
accessory duct & drains through minor papilla
• Epidemiology
o Complete agenesis of dorsal pancreas is rare
o Few reported cases of dorsal agenesis or hypoplasia
• Associated abnormalities
o Complete pancreatic agenesis (very rare); related
with stillbirth or early neonatal death
o Polysplenia syndrome (7 cases reported)
o Associated absence of uncinate process
Microscopic Features
• Biopsy specimens from head show normal pancreatic
tissue but only fat from expected position of body
• Absence of recognizable islets of Langerhans
I CLINICAL ISSUES
Presentation
• May be asymptomatic, incidental finding
• Diabetes mellitus & recurrent abdominal pain
• May present with jaundice/steatorrhea
Demographics
• Age: Mean: 31.6; range: 0-56 years
Top Differential Diagnoses
• Pancreatic carcinoma
• Pancreas divisum
• Chronic pancreatitis
Diagnostic Checklist
• May radiologically mimic acquired atrophy of
pancreatic body & tail (pseudo-agenesis)
Natural History & Prognosis
3
• Complicated by pancreatitis, insulin-requiring diabetes 7
mellitus, duodenal papillary dysfunction
I DIAGNOSTIC CHECKLIST
Consider
• May radiologically mimic acquired atrophy of
pancreatic body & tail (pseudo-agenesis)
o In pancreatic atrophy due to pancreatic carcinoma
or chronic pancreatitis, there may be a mass with
area of architectural distortion; some portion of
body or tail should remain, often containing dilated
duct
o Traumatic transection of pancreatic duct may also
cause proximal atrophy, but there should be some
remaining pancreas & history of trauma
I SELECTED REFERENCES
1. Fukuoka K et al: Complete agenesis of the dorsal pancreas.
J Hepatobiliary Pancreat Surg. 6(1):94-7, 1999
2. Oldenburg B et al: Pancreatitis and agenesis of the dorsal
pancreas. Eur J Gastroenterol Hepatol. 10(10):887-9, 1998
3. Wildling R et al: Agenesis of the dorsal pancreas in a
woman with diabetes mellitus and in both of her sons.
Gastroenterology. 104(4):1182-6, 1993
4. Shah KK et al: CT diagnosis of dorsal pancreas agenesis. J
Comput Assist Tomogr. 11(1):170-1, 1987
I IMAGE GALLERY
(Left) Axial CECT shows no pancreatic tissue or duct in the expected
position of the body-tail segments. (Right) Axial CECT shows normal
appearance of pancreatic head and uncinate in a patient with dorsal
agenesis; initially misdiagnosed as a pancreatic head mass which
prompted an unnecessary biopsy.
 ANNULAR PANCREAS

3
8

Graphic shows concentric duodenal luminal narrowing Axial CECT shows pancreatic tissue completely
by encircling pancreatic tissue. The small pancreatIc encirclingthe second part of the duodenum (arrow).
head duct also encircles the descending duodenum.
Note proximal duodenal dilatation.

• Pancreatitis seen in 15-30% of patients

ITERMINOlOGY
Definitions
• Ring of pancreatic tissue that encircles second part of
duodenum
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Pancreatic duct encircles the
endoscope or duodenum on ERCP
• Location: 2nd part of duodenum (85%); 1st or 3rd
parts (15%)
• Morphology
o Uncommon congenital anomaly of pancreas
o Usually manifests in neonates (52% cases) or
asymptomatic until adulthood (48% cases)
o Three theories in development of annular pancreas
• Persistence or hypertrophy plus abnormal
migration of ventral left pancreatic bud to right of
duodenum rather to left (more common)
• Hypertrophy of both dorsal & ventral ducts leads
to complete ring
• Adherence of ventral duct to duodenum before
rotation
o Acquired associated pathology
• Gastric & duodenal ulcers in 26-48% of cases
Radiographic Findings
• Plain x-ray abdomen
o Narrowing of descending duodenum
o Classic "double-bubble" sign
• Large proximal bubble: Dilated stomach
• Small distal bubble: Dilated duodenal bulb
• Fluoroscopic guided Barium Study (UGI)
o Extrinsic, eccentric defect on medial margin of
second part of duodenum
o Concentric narrowing of second part of duodenum
& dilated proximal duodenum
o Reverse peristalsis; duodenal ulcer (2nd part) may be
seen
o Periampullary duodenal ulcer in an adult
• Diagnosis: Annular pancreas or Zollinger-Ellison
syndrome (ZES)
• ERCP
o Normal main pancreatic duct in body & tail
communicating with small duct of pancreatic head
encircling duodenum
o Small duct of pancreatic head seen originating on
right anterior surface of duodenum, passing
posteriorly around duodenum & entering main duct
CT Findings
• Often nonspecific

DDx: Narrowed Duodenal lumen

Duodenal Carcinoma Postbulbar Ulcer Pancreatic Carcinoma Duodenal Hematoma

 ANNULAR PANCREAS

Key Facts
Terminology Pathology
• Ring of pancreatic tissue that encircles second part of • Congenital anomaly
duodenum • Persistence of left ventral pancreatic bud & abnormal
migration to right of duodenum than to left
Imaging Findings • Associated congenital anomalies in up to 75% cases
• Best diagnostic clue: Pancreatic duct encircles the • Intestinal malrotationi imperforate anus
endoscope or duodenum on ERCP • Esophageal atresiaj duodenal atresia/stenosis
• Location: 2nd part of duodenum (85%)i 1st or 3rd
parts (15%) Clinical Issues
• Gastric & duodenal ulcers in 26-48% of cases • Nausea, vomiting, epigastric pain, jaundice
• Pancreatitis seen in 15-30% of patients
Diagnostic Checklist
• Classic "double-bubble" sign
.• Differentiate other causes from annular pancreas 3
Top Differential Diagnoses which can produce "narrow duodenal lumen"
• Duodenal carcinoma (descending part) 9
• Postbulbar peptic ulcer • Periampullary ulcer in an adult suggests diagnosis of
• Pancreatic carcinoma annular pancreas or Zollinger-Ellison syndrome (ZES)

• Large pancreatic head & central area of increased o Annular lesion at second part of duodenum:
attenuation representing contrast within narrowed Indistinguishable from annular pancreas
duodenal segment • MRCP or ERCP: Depicts course & drainage pattern
• Gastric & duodenal dilatation plus circumferential of pancreatic duct
thickening of wall • On CT: Discrete mass or thickening of duodenal wall
• Obstructing band of pancreatic tissue & dilated main • Hypotonic duodenography differentiates
pancreatic duct periampullary & ampullary tumors causing narrow
• Dilated CBD & intrahepatic bile ducts (IHBD) duodenal lumen
MR Findings Postbulbar peptic ulcer
• Fat-suppressed T1WI • Location: Usually medial wall of proximal descending
o Normal pancreatic tissue encircling duodenum duodenum above ampulla of Vater
• MRCP • On barium studies
o Depicts course & drainage pattern of pancreatic duct o Ulcer on medial wall of second part of duodenum
o Folds radiate toward ulcer crater
Ultrasonographic Findings oEdema & spasm: Result in smooth, rounded
• Real time indentation on lateral wall
o Nonspecific enlargement of pancreatic head o "Ring stricture" with eccentric narrowing of
Angiographic Findings postbulbar duodenum due to scarring & fibrosis of
ulcer
• Celiac angiography
o Edema with spasm & ring stricture causes narrow
o Anomalous branch from posterior
duodenal lumen
pancreaticoduodenal artery that courses in a right &
• Mimics narrow duodenal lumen of annular
inferior direction supplying annular moiety
pancreas
Imaging Recommendations
Pancreatic carcinoma
• MR + MRCPj ERCPibarium study
• Irregular, heterogeneous, poorly enhancing mass
• Location: Head (60% of cases)
• Pancreatic duct and/or CBD show abrupt obstruction
I DIFFERENTIAL DIAGNOSIS & dilatation
Duodenal carcinoma • Obliteration of retropancreatic fat
• Rare malignant tumor • Extensive local invasion & regional metastases
• Accounts for 1% of all GIT neoplasms • Invasion: Medial part of duodenal sweep, narrowing
• Location: Postbulbar portion of duodenum lumen may be seen
o At or below level of ampulla of Vater • Barium UGI finding
• Increased incidence reported in patients with o "Inverted 3" contour: Medial part of duodenal sweep
Gardener syndrome & celiac disease • ERCP
• Occasionally associated with Crohn disease & o Irregular eccentric obstruction of CBD & pancreatic
neurofibromatosis duct
• On barium studies • Trousseau sign: Migratory thrombophlebitis
o Polypoid, ulcerated or annular lesions o Characteristic of cancer
o Narrowed duodenal lumen • 65% of patients present with advanced local disease &
distant metastases
 ANNULAR PANCREAS
Duodenal hematoma
• Results from trauma or anticoagulation
• Narrowed lumen, folds often thickened in "picket
fence" pattern
I PATHOLOGY
General Features
• General path comments
o Embryology-anatomy: Normal pancreas
• Normal development: From ventral anlage
• Hepatic diverticulum: Forms right & left ventral
3 pancreatic buds
• Right ventral bud persists to form head &
10 uncinate process
• Left ventral bud: Atrophies
• Etiology
o Congenital anomaly
o Most common pathogenesis
• Persistence of left ventral pancreatic bud &
abnormal migration to right of duodenum than to
left
• Ring of pancreatic tissue encircles 2nd part
• Duodenal narrowing leads to vomiting
o Ventral anlage undergoes 180 counter clockwise
0 I SELECTED REFERENCES
rotation while duodenum undergoes 90 clockwise
0
rotation, so that ventral anlage contiguous with
dorsal anlage, medial to duodenum
• Epidemiology: Uncommon in adults
• Associated abnormalities
o Associated congenital anomalies in up to 75% cases
• Intestinal malrotation; imperforate anus
• Esophageal atresia; duodenal atresia/stenosis
• Down syndrome & cardiac anomalies
Gross Pathologic & Surgical Features
• Concentric narrowing of second part of duodenum
encircled by band of tissue
Microscopic Features
• Normal pancreatic tissue & ductal epithelium
• No inflammatory cells
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Children & adults
• Nausea, vomiting, epigastric pain, jaundice
o Neonates: Persistent vomiting since first day of life
• History of polyhydramnios in utero
• Other manifestations of GIT obstruction
Demographics
• Age: Neonates (52%); children & adults (48%)
• Gender: Males more than females
Natural History & Prognosis
• Complications
o Gastric & duodenal ulcers (26-48%)
o Pancreatitis (15-30%)
• Prognosis
o Good: After surgical correction
Treatment
• Pediatric population
o Retrocolic duodenojejunostomy/gastrojejunostomy
• Adult population
o Surgical & interventional endoscopic procedures
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate other causes from annular pancreas
which can produce "narrow duodenal lumen"
(descending part)
• Periampullary ulcer in an adult suggests diagnosis of
annular pancreas or Zollinger-Ellison syndrome (ZES)
Image Interpretation Pearls
• ERCP: Small pancreatic duct encircling second part of
duodenum with narrow lumen
• Barium UGI: Concentric narrowing of second part of
duodenum with proximal dilatation & reverse
peristalsis
1. Yamaguchi Y et al: Annular pancreas complicated by
carcinoma of the bile duct: diagnosis by MR
cholangiopancreatography and endoscopie
ultrasonography. Abdom Imaging. 28(3):381-3, 2003
2. Harthun NL et al: Duodenal obstruction caused by
intraluminal duodenal diverticulum and annular pancreas
in an adult. Gastrointest Endosc. 55(7):940-3, 2002
3. Shan YSet al: Annular pancreas with obstructive jaundice:
beware of underlying neoplasm. Pancreas. 25(3):314-6,
2002
4. Benya EC: Pancreas and biliary system: imaging of
developmental anomalies and diseases unique to children.
Radiol Clin North Am. 40(6):1355-62, 2002
5. McCollum MO et al: Annular pancreas and duodenal
stenosis.] Pediatr Surg. 37(12):1776-7, 2002
6. Kamisawa T et al: A new embryologic hypothesis of
annular pancreas. Hepatogastroenterology. 48(37):277-8,
2001
7. ]ayaraman MV et al: CT of the duodenum: an overlooked
segment gets its due. Radiographies. 21 Spec No:SI47-60,
2001
8. Fulcher AS et al: MR pancreatography: a useful tool for
evaluating pancreatic disorders. Radiographies. 19(1):5-24;
discussion 41-4; quiz 148-9, 1999
9. ]advar H et al: Annular pancreas in adults: imaging features
in seven patients. Abdom Imaging. 24(2):174-7,1999
10. Berrocal T et al: Congenital anomalies of the upper
gastrointestinal tract. Radiographies. 19(4):855-72, 1999
11. Weiss H et al: Ultrasonography of fetal annular pancreas.
Obstet Gynecol. 94(5 Pt 2):852, 1999
12. Lecesne R et al: MR cholangiopancreatography of annular
pancreas.] Comput Assist Tomogr. 22(1):85-6, 1998
13. Kallen B et al: Major congenital malformations in Down
syndrome. Am] Med Genet. 65(2):160-6,1996
14. Rizzo R] et al: Congenital abnormalities of the pancreas
and biliary tree in adults. Radiographies. 15(1):49-68; quiz
147-8, 1995
15. Reinhart RD et al: MR imaging of annular pancreas. Abdom
Imaging. 19(4):301-3, 1994
 ANNULAR PANCREAS

I IMAGE GALLERY

(Left) Upper CI series shows

circumferential narrowing of
the lumen of the duodenum
by annular pancreas. (Right)
ERC? shows small pancreatic
head duct originating on
right anterior surface of
duodenum, encircling the
duodenum and emptying
into the main pancreatic
duct near the ampulla. 3
11

Typical
(Left) Axial CECT shows
pancreatic tissue completely
encircling the second part of
the duodenum (arrow).
(Right) Axial CECT shows
pancreatic head tissue
encircling duodenum.
Infiltrated mesentery due to
pancreatitis related to
annular pancreas.

Typical
(Left) ERC? shows
pancreatic duct branch
encircling endoscope within
duodenum. (Right) ERC?
shows a portion of annular
pancreatic duct that has a
beaded appearance due to
chronic pancreatitis.
 PANCREAS DIVISUM

3
12

ERCP with cannulation of major papilla shows MRCP in a patient with recurrent episodes of
opacification of the ventral pancreatic duct. The duct is pancreatitis shows faintly the ventral duct (open arrow)
small and short with normal tapering. Note adjacent with a pancreas divisum configuration of the dorsal
acinarization of pancreatic head. pancreatic duct.

ITERMINOlOGY Radiographic Findings

Definitions
• Failure of fusion of ventral & dorsal pancreatic buds
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Normal branching of short
ventral duct (head), not communicating with long
dorsal pancreatic duct (body & tail) on ERCP
• Morphology
o Most common congenital anatomic variant of
pancreas
o Most common variant of pancreatic ductal fusion &
drainage anomalies
o Seen in 12-26% of patients with idiopathic recurrent
pancreatitis
o Drainage consequences of anatomical variant
• Head & uncinate process of pancreas: Drained by
ventral pancreatic duct of Wirsung via major
papilla
• Body & tail of pancreas: Drained by dorsal
pancreatic duct of Santorini via minor papilla
o Clinically: Most cases asymptomatic or may
contribute to pancreatitis
• ERCP
o Cannulation of major papilla
• Opacification of short, tapered ventral pancreatic
(Wirsung) duct
o Cannulation of minor or accessory papilla
(technically difficult)
• Opacification of long ± dilated dorsal pancreatic
(Santorini) duct
o No communication between dorsal (long) & ventral
(short) pancreatic ducts
CT Findings
• Abnormal contour of pancreatic head & neck
• Large pancreatic head
• Two distinct pancreatic moieties separated by a fat
cleft
• Unfused ductal system on thin collimation scans
• Changes of pancreatitis (acute or chronic)
o Focal or diffuse enlargement of gland
o Fluid collections or pseudocyst
o Infiltration or obliteration of peri pancreatic fat
o Intra-/peripancreatic cysts or atrophy of gland
MR Findings
• T2* GRE: Depicts pancreatic ductal anomaly
• MRCP

DDx: Obstruction of Pancreatic Duct

Pancreatic Carcinoma Chronic Pancreatitis Annular Pancreas Dorsal Agenesis

 PANCREAS DIVISUM
Key Facts
Terminology
• Failure of fusion of ventral & dorsal pancreatic buds
Imaging Findings
• Best diagnostic clue: Normal branching of short
ventral duct (head), not communicating with long
dorsal pancreatic duct (body & tail) on ERCP
• Two distinct pancreatic moieties separated by a fat
cleft
• Unfused ductal system on thin collimation scans
• Changes of pancreatitis (acute or chronic)
• Best imaging tool: MRCP or ERCP
Top Differential Diagnoses
• Pancreatic carcinoma
• Chronic pancreatitis
• Annular pancreas
o Shows course & drainage pattern of dorsal & ventral
pancreatic ducts
• Dorsal duct: Long & narrow entering minor
papilla
• Ventral duct: Short; entering major papilla
• No communication between dorsal & ventral
ducts
Ultrasonographic Findings
• Secretin Test
o Performed to identify patients who will benefit from
surgical sphincterotomy
o Secretin mechanism increases HC03 secretion
which overloads a functionally inadequate papilla
o Secretin-induced ductal dilatation occurs in 72% of
symptomatic patients due to stenotic minor or
accessory papilla in pancreas divisum anomaly
o Normal result: No change in size of duct before & 20
minutes after secretin administration
o Grade I response
• 1 mm dilated duct in only one segment of
pancreas
• Result is equivocal & patient probably will not
benefit from surgery
o Grade II response
• More than 2 mm dilated duct in 2 segments of
pancreas
o Grade III response
• More than 2 mm dilated duct in 3 segments of
pancreas
o Grade II & III: Patients benefit from surgery
Imaging Recommendations
• Best imaging tool: MRCP or ERCP
I DIFFERENTIAL DIAGNOSIS
Pancreatic carcinoma
• Heterogeneous, poorly-enhancing mass
• Location: Head of pancreas (more common)
• Abrupt obstruction & dilatation of main pancreatic
duct
Pathology
• On day 37 ventral pancreas rotates posterior to
duodenum & comes in contact with dorsal pancreas,
failure of fusion results in pancreas divisum
• Ventral pancreas: Head & uncinate process (short
duct of Wirsung)
• Dorsal pancreas: Body & tail (long & narrow duct of
Santorini)
Clinical Issues
• Most cases are asymptomatic
Diagnostic Checklist 3
• Rule out other causes of "pancreatic duct obstruction"
• MRCP & ERCP: Demonstrate short ventral & long 13
dorsal pancreatic ducts with lack of communication
between two ducts
• Obliteration of retropancreatic fat
• Extensive local invasion & regional metastases
• Contiguous organ invasion seen:
o Duodenum, stomach & mesenteric root
• ERCP&MRCP
o Main pancreatic duct: Irregular, nodular, rat-tailed
eccentric obstruction & dilatation
Chronic pancreatitis
• Focal or diffuse atrophy of gland showing
heterogeneous enhancement
• Obstruction & dilatation of pancreatic +/- bile ducts
• Intraductal calculi & areas of calcification
• Focal masses (areas of fibrosis & fat necrosis)
• Intra or peri pancreatic cysts
• Thickening of peri pancreatic fascia
• ERCP & MRCP: Obstruction & dilatation of pancreatic
duct/radicles
Annular pancreas
• Congenital anomaly of pancreas
• Ring of pancreatic tissue that encircles second part of
duodenum
• Barium study
o Concentric narrowing of second part with dilated
proximal duodenum
o Reverse peristalsis & duodenal ulcer may be seen
• CT
o Gastric & duodenal dilatation with circumferential
thickening of wall
o Obstructing band of pancreatic tissue & dilated
main pancreatic/bile ducts may be seen
• ERCP &MRCP
o Depicts course & drainage pattern of pancreatic duct
o Pancreatic duct encircling endoscope or second part
of duodenum with narrow lumen
o Obstruction & dilatation of pancreatic/bile ducts
Agenesis of dorsal pancreas
• ERCP appearance may be identical to pancreas
divisum
• Diagnostic key: Absence of pancreatic body-tail on CT
orMR
 PANCREAS DIVISUM
• Prognosis
I PATHOLOGY o Good: After medical & surgical correction in
General Features symptomatic patients
• General path comments Treatment
o Embryology
• Asymptomatic
• On day 37 ventral pancreas rotates posterior to o No specific treatment
duodenum & comes in contact with dorsal
• Symptomatic
pancreas, failure of fusion results in pancreas o Medical
divisum • Conservative measures/pancreatic enzyme therapy
o Anatomy of pancreas divisum o Surgical or endoscopic correction
• Ventral pancreas: Head & uncinate process (short • Sphincteroplasty of minor or accessory papilla
duct of Wirsung)
• Dorsal pancreas: Body & tail (long & narrow duct
3 of Santorini)
o Pathogenesis: Embryological anomaly
I DIAGNOSTIC CHECKLIST
14 o Dorsal pancreas (body & tail) Consider
• Long & narrow duct of Santorini & minor papilla • Rule out other causes of "pancreatic duct obstruction"
• Poor drainage of secretions from body & tail
• Increased stasis & ductal pressure: Pancreatitis Image Interpretation Pearls
• Head (ventral pancreas) is spared • MRCP & ERCP: Demonstrate short ventral & long
o Ventral pancreas (head & uncinate process) dorsal pancreatic ducts with lack of communication
• Alcoholics: Pancreatitis due to reflux of bile via between two ducts
short duct of Wirsung
• Body & tail (dorsal pancreas) is spared due to
pancreas divisum I SELECTED REFERENCES
• Etiology: Congenital anatomical variant: Failure of 1. Khalid A et al: Secretin-stimulated magnetic resonance
fusion of dorsal & ventral pancreatic buds pancreaticogram to assess pancreatic duct outflow
• Epidemiology obstruction in evaluation of idiopathic acute recurrent
o Prevalence pancreatitis: a pilot study. Dig Dis Sci. 48(8):1475-81, 2003
• Seen in 3-6% of general population 2. Mishra D et al: Pancreas divisum: an uncommon cause of
• 4-11 % of autopsy series & 3-4% of ERCP series acute pancreatitis. Indian J Pediatr. 70(7):593-5, 2003
• May be associated with other congenital anomalies 3. Benya EC: Pancreas and biliary system: imaging of
developmental anomalies and diseases unique to children.
Gross Pathologic & Surgical Features Radiol Clin North Am. 40(6):1355-62, 2002
4. Manfredi R et al: Idiopathic chronic pancreatitis in
• Dorsal & ventral pancreatic tissues
children: MR cholangiopancreatography after secretin
• Two separate duct systems administration. Radiology. 224(3):675-82, 2002
Microscopic Features 5. Masatsugu T et al: Serous cystadenoma of the pancreas
associated with pancreas divisum. J Gastroenterol.
• Normal pancreatic tissue & ductal epithelium 37(8):669-73, 2002
• With or without inflammatory cells 6. Vitellas KM et al: MR cholangiopancreatography of bile
and pancreatic duct abnormalities with emphasis on the
single-shot fast spin-echo technique. Radiographics.
I CLINICAL ISSUES 20(4):939-57; quiz 1107-8,1112,2000
7. Morgan DE et al: Pancreas divisum: implications for
Presentation diagnostic and therapeutic pancreatography. AjR Am J
• Most common signs/symptoms Roentgenol. 173(1):193-8, 1999
8. Fulcher AS et al: MR cholangiography: technical advances
o Most cases are asymptomatic
and clinical applications. Radiographies. 19(1):25-41;
o Young patients: Epigastric pain, nausea & vomiting discussion 41-4, 1999
due to pancreatitis 9. Bret PM et al: Pancreas divisum: evaluation with MR
o May be seen in multiple family members cholangiopancreatography. Radiology. 199(1):99-103, 1996
• Clinical profile: Young patient with history of episodes 10. Rizzo RJ et al: Congenital abnormalities of the pancreas
of idiopathic recurrent pancreatitis and biliary tree in adults. Radiographies. 15(1):49-68; quiz
147-8, 1995
Demographics
• Age
o Varies widely at diagnosis
o Common between 30 & SO yrs
• Gender: Males> females
Natural History & Prognosis
• Complications
o Recurrent pancreatitis (mostly in children)
o Pancreaticolithiasis, serous cystadenoma
(speculative)
 PANCREAS DIVISUM

I IMAGE GAllERY
Typical
(Left) Axial CECT shows
dilated pancreatic duct and
small pseudocysts (arrows)
in patient with pancreas
divisum complicated with
recurrent episodes of
pancreatitis. (Right) Axial
CECT shows calculus
(arrow) within duct of
Santorini, which is of normal
caliber "downstream" as it
enters the minor papilla.
3
15

(Left) ERCPin a patient with

chronic pancreatis and
pancreas divisum shows
filling of ventral pancreatic
duct (arrow) and a dilated
common bile duct (open
arrow), following
cannulation of major papilla.
(Right) ERCPshows
dilatation and irregularity of
dorsal pancreatic duct and
small pseudocysts (arrow),
following cannulation of
minor papilla. Pancreas
divisum and chronic
pancreatitis.

Typical
(Left) Axial CECT shows a
fatty cleft (arrow) separating
the ventral and dorsal
pancreatic segments. (Right)
Axial CECTshows a fatty
cleft (arrow) separating the
ventral (head-uncinate
process) and dorsal
(body-tail) segments.
 ECTOPIC PANCREATIC TISSUE

3
16

Upper CI series shows small intramural antral mass with Upper CI series shows a small antral mass with intact
a collection of barium (arrow) marking the rudimentary mucosa. Central "dot" of barium (arrow) fills
duct. rudimentary duct.

o Mainly lies submucosally (73%); can be located in

ITERMINOlOGY muscular layer (17%), or in subserosa (10%)
Abbreviations and Synonyms • Size
• Eptopic pancreatic tissue (EPT) o Nodule: 0.5-2 cm; may be up to 5 cm in diameter
• Pancreatic rests; heterotopic, aberrant, accessory o Pit: May be 5 mm in diameter & 10 mm in length
pancreas • Morphology
o In submucosal layer, appears as well-defined flat or
Definitions nodular projection into gut lumen, with intact
• Pancreatic tissue located outside normal confines of overlying mucosa
pancreas & lacking any anatomic or vascular o In muscularis or subserosallayers, produces smooth
connection with it bulge or area of wall thickening
Radiographic Findings
I IMAGING FINDINGS • Ability of radiographic contrast studies to visualize EPT
depends on size & location of deposit
General Features • Barium study may show narrowed pyloric channel
• Best diagnostic clue with or without a polypoid or sessile mass
o Small intramural gastric mass with central • Characteristic appearance: Well-defined, smoothly
umbilication is diagnostic (45%) marginated, round or oval broad-based mass
• Central umbilication: Orifice of rudimentary duct o In stomach: Typically 1 to 2 cm in diameter, along
into which EPT opens & empties into gut lumen greater curvature or posterior aspect of antrum,
near summit of nodule within 6 cm of pylorus
• Location o Nodule may be larger, have a narrow base & appear
o Those identifiable at imaging are nearly always in polypoid, or located in more proximal antrum
stomach or duodenum • Streaking or central depression; with contrast filling
o Remaining sites: Ileum, Meckel diverticulum, liver, pit in center of mound; specific feature
biliary tract, spleen, omentum, mesentery, lung, o This may be mistaken for an ulcerative lesion
mediastinum, fallopian tube, esophagus, colon • Reflux of contrast into rudimentary duct-like structure
may extend below central pit

DDx: Ulcerated Gastric Polypoid lesion

Gastric Ulcer Gastric Carcinoma Gastric Metastases Gastric Stromal Tumor

 ECTOPIC PANCREATIC TISSUE
Key Facts
Terminology
• Pancreatic tissue located outside normal confines of
pancreas & lacking any anatomic or vascular
connection with it
Imaging Findings
• Small intramural gastric mass with central
umbilication is diagnostic (45%)
CT Findings
• Usually too small to be detected
• Rarely intramural cystic collections in stomach &
duodenum
Other Modality Findings
• Endoscopy: More capable of identifying EPT when
nodule is small & located in duodenum
o Often nonspecific due to submucosal location
o Central umbilication may be visualized, & if
injected, rudimentary duct system may be seen
I DIFFERENTIAL DIAGNOSIS
Gastric ulcer
• Round ulcer, smooth mound of edema, radiating folds
to ulcer edge, Hampton line, ulcer collar
Gastric carcinoma
• Polypoid or circumferential mass, ± ulceration, focal
wall thickening with mucosal irregularity, focal
infiltration of wall
Gastric metastases
• "Bull's eye" sign: Ulceration in center of lesion
• Melanoma, Kaposi sarcoma
Gastric stromal tumor
• Large, lobulated, submucosal mass with ulceration;
requires biopsy, histologic diagnosis
I PATHOLOGY
General Features
• Epidemiology: Incidence of autopsy series: 2-14%
• Seen in organs, like pancreas, derived from endoderm
as a result of heteroplastic differentiation of parts of
embryonic endoderm that do not normally produce
pancreatic tissue
Microscopic Features
• May contain all or only some elements of normal
pancreas; including acini, ducts & islet cells
I CLINICAL ISSUES
Presentation
• Asymptomatic: Incidental finding
• Characteristic appearance: Well-defined, smoothly
marginated, round or oval broad-based mass
Top Differential Diagnoses
• Gastric ulcer
• Gastric carcinoma
• Gastric metastases
• Gastric stromal tumor
• Symptomatic: May simulate duodenal ulcer, 3
gallbladder disease, or appendicitis
• In stomach, symptoms of pyloric obstruction 17
• Periampullary, rare biliary obstruction
Natural History & Prognosis
• Complicated by bleeding or mucosal ulceration
• Acute pancreatitis +/- hemorrhage and necrosis
• Chronic pancreatitis with pseudocyst formation
• Malignancy: Ductal adenocarcinoma
Treatment
• Surgical intervention for obstruction or hemorrhage
• Endoscopic resection if lesion confined to submucosa
• When asymptomatic may be treated expectantly
I DIAGNOSTIC CHECKLIST
Consider
• If central umbilication is absent, lesion may not be
differentiated from other submucosal tumors
I SELECTED REFERENCES
1. Jeong HY et al: Adenocarcinoma arising from an ectopic
pancreas in the stomach. Endoscopy. 34(12):1014-7, 2002
2. Hayes-Jordan A et al: Ectopic pancreas as the cause of
gastric outlet obstruction in a newborn. Pediatr Radiol.
28(11):868-70, 1998
3. Kaneda M et al: Ectopic pancreas in the stomach
presenting as an inflammatory abdominal mass. Am J
Gastroenterol. 84(6):663-6, 1989
I IMAGE GALLERY
(Left) Upper GI series show small intramural mass (arrow) along
greater curvature of antrum with intact mucosa. (Right) Upper GI
series show small mass with central umbilication (arrow).
 CYSTIC FIBROSIS, PANCREAS

3
18

Axial NECT shows lipomatous replacement of entire Axial NECT shows lipomato~s replacement and
pancreas. pseudohypertrophy of enUre pancreas.

• Pancreatic dysfunction in advanced cases; on CT

ITERMINOLOGY demonstrated as fibrosis & marked atrophy
Abbreviations and Synonyms • Pancreatic cysts: Late manifestation; diffuse
• Cystic fibrosis (CF) replacement of pancreas by multiple fluid-filled cysts
• Mucoviscidosis/fibrocystic disease o Related to inspissation of tenacious secretions,
leading to ductal ectasia
Definitions
MR Findings
• Cystic fibrosis is a recessively inherited disorder of
epithelial chloride transport & characterized by • Fat deposition: Hyperintense on T1WI
abnormality of exocrine gland function • Fibrosis: Hypointense on T1 & T2WI
• Pancreatic cysts are relatively common finding; these
true cysts are typically quite small but are well
I IMAGING FINDINGS demonstrated at MR imaging & MRCP
• Pancreatic duct abnormalities also occasionally seen
General Features
Ultrasonographic Findings
• Best diagnostic clue: Lipomatous pseudohypertrophYi
• Diffuse increased echogenicity (fatty replacement &
fatty replacement of pancreas (-90 to -120 HU) on CT
fibrosis)
• Location: Multisystem disease; affecting primarily
• Hypoechoic enlargement of pancreas noted in some
pancreas, lungs, gut, liver & exocrine glands
patients who exhibit clinical signs of pancreatitis
CT Findings • Chronic calcific pancreatitis
• Early: Inhomogeneous attenuation • Macroscopic multiple cysts (dilated acini & ducts)
• Later: Low attenuation with complete fatty infiltration • Pancreas may be barely identifiable or not
& replacement demonstrated at all (advanced disease; atrophy)
• Microcysts may develop (multiple microscopic); some
may become small macroscopic cysts demonstrable
with CT
• ± Scattered foci of calcifications
• Dilatation of pancreatic duct (uncommon)

DDx: Fatty Replacement of Pancreas

Fatty Lobulation Chronic Pancreatitis Chronic Pancreatitis Schwachman-Oiamond

 CYSTIC FIBROSIS, PANCREAS
Key Facts
Terminology
• Cystic fibrosis is a recessively inherited disorder of
epithelial chloride transport & characterized by
abnormality of exocrine gland function
Imaging Findings
• Best diagnostic clue: Lipomatous pseudohypertrophy;
fatty replacement of pancreas (-90 to -120 HU) on CT
I DIFFERENTIAL DIAGNOSIS
Normal fatty lobulation
• Profuse fat with glandular atrophy seen occasionally in
elderly, obese and diabetic
Chronic pancreatitis
• Focal/diffuse enlargement or atrophy; dilated
pancreatic duct; ductal calculi
• Intra/peri pancreatic cysts; thickening of peripancreatic
fascia; heterogeneous enhancement
• Small, hypodense, focal masses (fibrosis & fat necrosis)
Shwachman-Oiamond syndrome
• Pancreatic lipomatosis: Pancreas is completely replaced
by fat; has low attenuation value on CT scans
I PATHOLOGY
General Features
• General path comments
o Cystic fibrosis (CF) is major cause of pancreatic
exocrine failure in childhood
o Primary ductal cell chloride channel abnormality
results in dehydrated protein-rich secretions
obstructing proximal ducts, leading to acinar cell
destruction, fibrosis & exocrine insufficiency
• Genetics
o Autosomal recessive
o CF gene located on long arm of chromosome 7
o Mutations in transmembrane conductance regulator
(CFTR) gene
• Etiology
o Mutations of CFTR gene leads to pancreatic
pathology
o Disruption of chloride ion, bicarbonate & water
transport in duct cells
• Epidemiology
o Prevalence: 1 per 2,000
o Pancreatic abnormalities in 85-90% of CF patients
I CLINICAL ISSUES
Presentation
• Steatorrhea, malabsorption, fat intolerance
• Diabetes mellitus
• Pancreatitis: Susceptivity to infection by
Staphylococcus aureus & Pseudomonas aeruginosa
• Pancreatic dysfunction in advanced cases; on CT
demonstrated as fibrosis & marked atrophy
• Pancreatic cysts: Late manifestation; diffuse
replacement of pancreas by multiple fluid-filled cysts
Pathology
• Mutations in transmembrane conductance regulator
(CFTR) gene
• Chronic pulmonary disease, hepatic fibrosis, intestinal 3
obstruction, infertility in males
• Lab: Positive sweat test 19
• Genotyping has proved useful in identifying gene
carriers; antenatal diagnosis & treatment
Demographics
• Age: Children; patients reaching adulthood represent a
rapidly growing percentage of CF population
• Ethnicity: More common in Caucasians
Natural History & Prognosis
• Pancreatic involvement varies depending on degree of
ductal obstruction by mucus; leading to exocrine
gland atrophy, progressive fibrosis & cyst formation
• Pancreatitis in less than 1% of CF patients
• Predisposes to pancreatic cancer
Treatment
• Aggressive nutritional & pancreatic enzyme therapy
I SELECTED REFERENCES
1. Taylor C] et al: The pancreas in cystic fibrosis. Paediatr
Respir Rev. 3(1):77-81, 2002
2. King L] et al: Hepatobiliary and pancreatic manifestations
of cystic fibrosis: MR imaging appearances. Radiographies.
20(3):767-77,2000
3. Soyer P et al: Cystic fibrosis in adolescents and adults: fatty
replacement of the pancreas--CT evaluation and functional
correlation. Radiology. 210(3):611-5, 1999
I IMAGE GALLERY
(Left) Axial NECT shows hyperaeration of the upper lobes with cystic
bronchiectasis in a patient with cystic fibrosis. (Right) Axial CECT
shows lipomatous replacement of the pancreas and one of several
small cysts (arrow).
 ACUTE PANCREATITIS

3
20

Axial CECT. Extensive infiltration of the peri pancreatic Axial CECT shows extensive peri pancreatic infiltration to
fat planes. The celiac axis and portal vein (arrow) are the perirenal fascia (arrow) and ventrally into the
surrounded, splenic vein (open arrow) is occluded, mesentery (open arrow).
gastric wall is thickened

• Absence of gas distal to splenic flexure caused by

ITERMINOLOGY functional colonic spasm due to spread of
Definitions pancreatic inflammation to proximal descending
• Acute inflammatory process of pancreas with variable colon
involvement of other regional tissues or remote organ • ERCP
systems o Dilated or normal main pancreatic duct (MPD)
o Communication of pseudocyst with MPD (acutely)
o May show narrowed & tapered distal common bile
IIMAGING FINDINGS duct (CBD) with prestenotic biliary dilatation
CT Findings
General Features
• Focal or diffuse enlargement of pancreas
• Best diagnostic clue: Enlarged pancreas, fluid
• Heterogeneous enhancement; non enhancing necrotic
collections & obliteration of fat planes
areas
• Location: Pancreatic & peri pancreatic
• Rim-enhancement of acute fluid collections, abscesses
• Size: Pancreas increased in size (focal or diffuse)
and pseudocysts
• Morphology: Inflammatory disease of pancreas
• Infiltration of peri pancreatic fat; gall stones
producing temporary changes with restoration of
• Pseudoaneurysm: May simulate pseudo cyst; on CECT
normal anatomy and function following resolution
enhances like adjacent blood vessels
Radiographic Findings • Chest: Pleural effusions & basal atelectasis
• Radiography MR Findings
o Duodenal ileus
o Sentinel loop: Mildly dilated, gas-filled segment of • TIWI
o Tl WI gradient-echo image
small bowel with or without air-fluid levels
• Variable decreased signal intensity & enlarged
o "Colon cutoff" sign
gland
• Markedly distended transverse colon with air
• T2WI
o Fat-suppressed T2WI

DDx: Peripancreatic Infiltration

Pancreatic Carcinoma Perf. Ulcer (Duodenal) "Shock" Pancreas Lymphoma

 ACUTE PANCREATITIS
Key Facts
Terminology
• Acute inflammatory process of pancreas with variable
involvement of other regional tissues or remote organ
systems
Imaging Findings
• Best diagnostic clue: Enlarged pancreas, fluid
collections & obliteration of fat planes
• Communication of pseudocyst with MPD (acutely)
• Chest: Pleural effusions & basal atelectasis
Top Differential Diagnoses
• Infiltrating pancreatic carcinoma
• Perforated duodenal ulcer
• "Shock" pancreas
• Lymphoma & metastases
• Fluid collections, pseudocyst, necrotic areas:
Hyperintense
• Gallstones or intraductal calculi: Hypointense
• T1 C+
o Heterogeneous enhancement pattern
• Nonenhancing decreased signal areas
(necrosis/fluid collection/pseudocyst)
• Pancreatic pseudocyst contiguous with MPD
• Vascular occlusions can be easily demonstrated
• MRCP
o All fluid-containing structures: Hyperintense
o Dilated or normal main pancreatic duct (MPD)
o Pseudocyst contiguous with MPD
Ultrasonographic Findings
• Real Time: Enlarged hypoechoic gland/fluid
collection/ abscess/pseudocyst
Angiographic Findings
• Conventional
o Performed when a pseudoaneurysm is suspected
o Useful when pancreatitis due to a vascular cause
• Vasculitis, polyarteritis nodosum, lupus
• Postaortic aneurysm resection
Imaging Recommendations
• NE + CECT; MR (fat-suppressed images); MRCP; ERCP
I DIFFERENTIAL DIAGNOSIS
Infiltrating pancreatic carcinoma
• Irregular, heterogeneous} poorly-enhancing mass
• Abrupt obstruction & dilatation of pancreatic duct
• Obliteration of retropancreatic fat
• No hemorrhage, calcium very rare
• Local tumor extension: Splenic hilum & porta hepatis
• Contiguous organ invasion
o Duodenum, stomach & mesenteric foot
• ERCP: Main pancreatic duct
o Irregular} nodular} rat-tailed eccentric obstruction
o Prestenotic dilatation
• Angiography: Hypovascular mass encasing vessels
Pathology
• Alcohol! gallstones/metabolic/infection/trauma/ drugs
• Pathogenesis: Due to reflux of pancreatic enzymes,
bile, duodenal contents & increased ductal pressure
Clinical Issues
• Clinical profile: Patient with history of alcoholism,
fever & severe mid-epigastric pain radiating to back
• Increased serum amylase & lipase
• Leukocytosis, hypocalcemia (poor prognostic sign)
Diagnostic Checklist
• Rule out other pathologies which can cause 3
"peripancreatic infiltration"
• Bulky, irregularly enlarged pancreas with obliteration 21
of peripancreatic fat planes, fluid collections,
pseudocyst or abscess formation
Perforated duodenal ulcer
• Penetrating ulcers may infiltrate anterior pararenal
space, simulating pancreatitis
• Less than 50% of cases have evidence of extraluminal
gas or contrast medium collections
• Pancreatic head may be involved
"Shock" pancreas
• Infiltration of peripancreatic & mesenteric fat planes
following hypotensive episode (e.g., blunt trauma)
• Pancreas itself looks normal or diffusely enlarged
• Usually presents with "shock bowel" appearance with
mucosal enhancement and submucosal edema
• Quickly resolves following resuscitation
Lymphoma & metastases
• Nodular, bulky, enlarged pancreas due to infiltration
• Retroperitoneal adenopathy
• MPD & side branches
o Show extrinsic mass effect & ductal draping
• Smooth ductal splaying or some narrowing
o Lack of communication with tumor
• Peripancreatic infiltration (obliteration of fat planes)
• Primary may be seen in case of metastatic infiltration
I PATHOLOGY
General Features
• General path comments
o Embryology-anatomy
• Congenital anomalies may cause pancreatitis
• Annular pancreas: Failure of migration of ventral
bud to contact dorsal
• Pancreas divisum: Ventral & dorsal pancreatic
buds fail to fuse; relative block at minor papilla
• Genetics
o Hereditary pancreatitis
• Autosomal dominant & incomplete penetrance
• Etiology
o Alcohol! gallstones/metabolic/ infection/trauma/ drugs
o Pathogenesis: Due to reflux of pancreatic enzymes}
bile, duodenal contents & increased ductal pressure
 ACUTE PANCREATITIS
• MPD or terminal duct blockage
• Edema; spasm; incompetence of sphincter of Oddi
• Periduodenal diverticulum or tumor
• Epidemiology
o In USA: Urban & VA hospitals (alcohol); suburban &
rural (gallstones)
o Incidence in USA
• 0.005 to 0.01 % in general population
Gross Pathologic & Surgical Features
• Bulky pancreas, necrosis, fluid collection & pseudocyst
Microscopic Features
• Acute edematous pancreatitis
3 o Edema, congestion, leukocytic infiltrates
• Acute hemorrhagic pancreatitis
22 o Tissue destruction, fat necrosis & hemorrhage
• Dilated ducts & protein plugs may be seen
Staging, Grading or Classification Criteria
• CT classification: Five grades based on severity
o Grade A: Normal pancreas
o Grade B
• Focal or diffuse enlargement of gland
• Contour irregularities & heterogeneous
attenuation
• No peripancreatic inflammation
o Grade C: Intrinsic pancreatic abnormalities &
associated inflammatory changes in peri pancreatic
fat
o Grade D
• Small & usually single, ill-defined fluid collection
o Grade E
• Two or more large fluid collections
• Presence of gas in pancreas or retroperitoneum
• Most important criterion: Presence & extent of
necrotizing pancreatitis (nonenhancing parenchyma)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Epigastric pain, often radiating to back
o Tenderness, fever, nausea, vomiting
o Grey Turner sign: Bluish discoloration of flanks
o Cullen sign: Periumbilical discoloration
• Clinical profile: Patient with history of alcoholism,
fever & severe mid-epigastric pain radiating to back
• Lab data
o Increased serum amylase & lipase
o Hyperglycemia, increased lactate dehydrogenase
o Leukocytosis, hypocalcemia (poor prognostic sign)
o Fall in hematocrit, rise in blood urea nitrogen (BUN)
Demographics
• Age
o Usually young & middle age group
o Can be seen in any age group
• Gender: Males more than females
Natural History & Prognosis
• Complications
o Pancreatic
• Fluid collections, pseudocyst, necrosis, abscess
o Gastrointestinal
• Hemorrhage, infarction, obstruction, ileus
o Biliary: Obstructive jaundice
o Vascular: Pseudoaneurysm, porto-splenic vein
thrombosis, hemorrhage
o Disseminated intravascular coagulation (DIe)
o Shock due to pulmonary & renal failure
o Cardiac, CNS & metabolic complications
• Prognosis
o Early detection with minor complications: Good
o Late detection with major complications: Poor
o Infected pancreatic necrosis - almost 50% mortality
even with surgical debridement
Treatment
• Conservative
o NPO, gastric tube, atropine, analgesics, antibiotics
• Treat complications of acute pancreatitis
o Infected or obstructing pseudocysts require drainage
o Most resolve spontaneously
o Infected necrosis requires surgery, not catheter
drainage
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other pathologies which can cause
"peri pancreatic infiltration"
Image Interpretation Pearls
• Bulky, irregularly enlarged pancreas with obliteration
of peri pancreatic fat planes, fluid collections,
pseudocyst or abscess formation
I SELECTED REFERENCES
1. Balthazar E]:Acute pancreatitis: assessment of severity with
clinical and CT evaluation. Radiology. 223(3):603-13, 2002
2. Balthazar E]:Staging of acute pancreatitis. Radiol Clin
North Am. 40(6):1199-209, 2002
3. Balthazar E]: Complications of acute pancreatitis: clinical
and CT evaluation. Radiol Clin North Am. 40(6):1211-27,
2002
4. Piironen A: Severe acute pancreatitis: contrast-enhanced
CT and MRIfeatures. Abdom Imaging. 26(3):225-33, 2001
5. Lecesne R et al: Acute pancreatitis: interobserver agreement
and correlation of CT and MR cholangiopancreatography
with outcome. Radiology. 211(3):727-35, 1999
6. Vitellas KMet al: Pancreatitis complicated by gland
necrosis: evolution of findings on contrast-enhanced CT.]
Comput AssistTomogr. 23(6):898-905, 1999
7. Sica GT et al: Comparison of endoscopic retrograde
cholangiopancreatography with MR
cholangiopancreatography in patients with pancreatitis.
Radiology. 210: 605-10, 1999
8. Balthazar E] et al: Imaging and intervention in acute
pancreatitis. Radiology. 193(2):297-306, 1994
 ACUTE PANCREATITIS
I IMAGE GALLERY
Typical
(Left) Mild acute
pancreatitis. Gland is
diffusely enlarged with·
minimal peripancreatic
infiltration (arrow). (Right)
Mild acute pancreatitis. Axial
CECT shows enlarged
pancreatic head with
infiltration of mesenteric fat
(arrow).
3
23

Typical
(Left) Necrotizing
pancreatitis. Axial CECT
shows almost no enhancing
viable pancreatic tissue, only
fluid and necrotic tissue.
(Right) Infected pancreatic
necrosis. Axial CECT shows
no enhancing parenchyma.
The necrotic tissue contains
gas bubbles (arrows)
indicating infection.

(Left) Central pancreatic

necrosis. Axial CECT shows
enhancing viable tissue in
pancreatic tail. The
pancreatic body is necrotic
and a large pseudocyst has
formed (arrow). (Right) Axial
CECT shows viable
enhancing pancreatic head
and pseudocyst (arrow).
 PANCREATIC PSEUDOCYST

3
24

Axial CECT shows dilated main pancreatic duct and Coronal T2WI MR shows dilated main pancreatic duct
pseudocyst in and adjacent to pancreatic head. (curved arrow) and contiguous pseudocyst (arrow).
Gallbladder (open arrow).

o In contrast to true cysts, pseudocysts lack a true

ITERMINOLOGY epithelial lining
Definitions Radiographic Findings
• Collection of pancreatic fluid & inflammatory exudate
• ERCP
encapsulated by fibrous tissue o Communication of pseudocyst with pancreatic duct
seen in 70% of cases (decreases over time)
I IMAGING FINDINGS CT Findings
• NECT
General Features o Round or oval, homogeneous, hypodense lesion
• Best diagnostic clue: Cystic mass with infiltration of with a near water density ("mature" pseudocyst)
peripancreatic fat planes o Hemorrhagic, infected pseudocyst: Lobulated,
• Location heterogeneous, mixed density lesion
o Two thirds within pancreas: Usually in lesser sac o ± Pancreatic calcification; main pancreatic duct
• Body & tail (85%); head (15%) (MPD) & common bile duct (CBD) dilatation
o One third • CECT
• ]uxtasplenic, retroperitoneum & mediastinum o Enhancement of thin rim of fibrous capsule
• Pararenal, left lobe of liver o No enhancement of pseudocyst contents
• Size: Varies from 2-10 cm o Gas within pseudocyst suggests superimposed
• Morphology infection
o One of the complications of acute pancreatitis o Decompression of pseudocyst into pancreatic duct,
• Other pancreatic complications: Fluid collections, stomach or bowel may result in gas within cyst
abscess, infected necrosis, pseudoaneurysm o Pseudoaneurysms can be caused by or simulate a
o Seen in approximately 15% of patients with acute pseudocyst
pancreatitis • CECT shows enhancement like adjacent blood
o Can also be seen with chronic pancreatitis vessels
o Develop over a period of 4-6 weeks after onset of
acute pancreatitis

DDx: Cystic Pancreatic Mass

Serous Cystadenoma Mucinous Cystic tumor /PMT of Pancreas Congenital Cysts (VHL)
 PANCREATIC PSEUDOCYST

Key Facts
Terminology
• Collection of pancreatic fluid & inflammatory
exudate encapsulated by fibrous tissue
Imaging Findings
• Best diagnostic clue: Cystic mass with infiltration of
peripancreatic fat planes
• Communication of pseudocyst with pancreatic duct
seen in 70% of cases (decreases over time)
• Enhancement of thin rim of fibrous capsule
Top Differential Diagnoses
• Serous (microcystic) cystadenoma
• Mucinous cystic tumor of pancreas
• IPMT of pancreas
• Cystic islet cell tumor
• Congenital cysts
Pathology
• Unabsorbed fluid collections organize & within 4-6
weeks develop a fibrous capsule
• Pseudocyst: Major complication of acute (more
common) & chronic pancreatitis
Clinical Issues
• Clinical profile: Patient with history of chronic
alcoholism, abdominal pain & palpable tender mass
• May persist, resolve or can even continue to grow
• Complications: Pseudocysts larger than 4-5 cm in size
Diagnostic Checklist 3
• Rule out other "cystic lesions of pancreas"
• Consider possibility of pseudoaneurysm, especially if 25
drainage is contemplated

• Angiography: Highly vascular tumor due to extensive

MR Findings
• T1WI: Hypointense
• T2WI
o Hyperintense (fluid)
o Mixed intensity (fluid + debris)
• Tl C+: May show enhancement of fibrous capsule
• MRCP
o Hyperintense cyst contiguous with dilated
pancreatic duct
Ultrasonographic Findings
• Real Time
o Usually solitary unilocular cyst (body or tail)
o Multilocular in 6% of cases
o Fluid-debris level & internal echoes due to autolysis
(blood clot/cellular debris)
o Septations (rare; sign of infection or hemorrhage)
o Dilated pancreatic duct & CBD may be seen
o Calcification of pancreas (chronic pancreatitis)
Angiographic Findings
• Conventional
o To confirm diagnosis of pseudoaneurysm
• Splenic artery is most frequently involved,
followed by inferior & superior
pancreatico-duodenal arteries
Imaging Recommendations
• NE + CECT, MRCP, ERCP, us
I DIFFERENTIAL DIAGNOSIS
Serous (microcystic) cystadenoma
• Synonym: Glycogen-rich cystadenoma of pancreas
• Benign pancreatic tumor (arises from acinar cells)
• Slowly growing tumor & may become quite large
• Most frequently seen in middle-aged females
• CECT
o Honeycomb or sponge appearance
o Enhancement of septa delineating small cysts
o Enhancement of cyst wall seen
o May have calcification in central scar
capillary network within septa
Mucinous cystic tumor of pancreas
• CT: Multiloculated hypodense mass
• CT: Enhancement of thin internal septa & wall
• T2WI MR
o Cysts: Hyperintense
o Internal septations: Hypointense
• Location: Tail of pancreas (more common)
• Sonography: Multiloculated cystic mass with
echogenic internal septa
• Angiography: Predominantly avascular
• Multilocularity or mural nodules favor tumor
• Often indistinguishable from pseudocyst by imaging
alone
• More common in middle-aged females
• Most consider this tumor as premalignant
IPMT of pancreas
• IPMT: Intraductal papillary mucinous tumor
• Cystic lesion contiguous with dilated MPD sometimes
indistinguishable from pseudocyst
• Low grade malignancy arises from main pancreatic
duct (MPD) or branch pancreatic duct (BPD)
• Side branch type usually arises in BPD of pancreatic
head/uncinate, resembling cluster of grapes or small
tubular cysts
• Main duct type causes gross dilatation of MPD +/-
cystic spaces
• Maybe be indistinguishable from chronic pancreatitis
& pseudocyst
Cystic islet cell tumor
• Usually non-insulin producing & nonfunctioning
• Tumor: Cystic on NECT & non enhancing on CECT
o No pancreatic ductal dilatation
• Angiography: Hypervascular primary & secondary
Congenital cysts
• Associated with von Hippel-Lindau (VHL) & ADPKD
• Rare, usually small & multiple nonenhancing cysts
• No pancreatic ductal dilatation
 PANCREATIC PSEUDOCYST
I PATHOLOGY
General Features
• General path comments
o Fluid collection
• Rupture of pancreatic duct
• Release of enzymes & pancreatic juice
• Exudation of fluid from surface of pancreas due to
activation of enzymes within gland
• Usually absorbed within 2-3 weeks
• Seen in up to 50% of patients with acute
pancreatitis; does not constitute pseudocyst
o Pseudocyst
3 • Unabsorbed fluid collections organize & within
4-6 weeks develop a fibrous capsule
26 • Etiology
o Pseudocyst: Major complication of acute (more
common) & chronic pancreatitis
• Chronic alcoholism (75%)
• Abdominal trauma (13%): Major cause in children
• Cholelithiasis, pancreatic carcinoma, idiopathic
• Epidemiology
o Pseudocysts form during initial attack of pancreatitis
in 1-3% of patients
o Pseudocysts develop after several episodes of
alcoholic pancreatitis in 12% of patients
• Associated abnormalities: Acute or chronic pancreatitis
Gross Pathologic & Surgical Features
• Collection of fluid, tissue, debris, pancreatic enzymes
& blood covered by a thin rim of fibrous capsule
Microscopic Features
• Inflammatory cells, necrosis, hemorrhage
• Absence of epithelial lining
• Walls consist of necrotic, granulation or fibrous tissue
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Clinical significance is related to its size &
complications
• Abdominal pain with or without radiation to back
(common complaint)
• Palpable, tender mass in middle or left upper
abdomen
• Signs of hemorrhage: Increase in size, bruit over
mass, decrease in hemoglobin level & hematocrit
• Clinical profile: Patient with history of chronic
alcoholism, abdominal pain & palpable tender mass
• Lab data
o Acute pancreatitis
• Increased serum amylase & lipase
o Chronic pancreatitis
• Secretin test: Decreased amylase & bicarbonate
Demographics
• Age: More common in young & middle age group
• Gender: Males> females
Natural History & Prognosis
• Natural history: Difficult to predict
o May persist, resolve or can even continue to grow
o Spontaneous resolution of pseudocyst can occur by
• Drainage into pancreatic duct
• Erosion into adjacent hollow organ (stomach,
small bowel, colon)
• Rupture with spillage into peritoneal cavity
• Complications: Pseudocysts larger than 4-5 cm in size
o Compression of adjacent bowel or bile duct
• Obstruction, severe pain, jaundice
o Spontaneous rupture into peritoneal cavity
• Ascites, peritonitis
o Secondary infection: Infected pseudocyst not as
lethal as infected pancreatic necrosis
o Erosion into adjacent vessel
• Hemorrhage or pseudo aneurysm
o Rupture & hemorrhage are prime causes of death
from pseudocyst
• Prognosis
o Spontaneous resolution in 25-40% of patients
o Percutaneous drainage cure in 90% of cases
• Complications in 5-10% of cases
Treatment
• Conservative therapy
o Infected pseudocyst
o Asymptomatic or decrease in size on serial scans
• Percutaneous drainage
o Size more than 4-5 cm
o Symptomatic or increase in size
o Requires long-term catheter if pseudocyst still
communicates with pancreatic duct
o Drainage routes
• Retroperitoneal, transperitoneal
• Transgastric, transhepatic or duodenal
• Surgical therapy: Internal (usually into stomach) or
external drainage of cyst
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other "cystic lesions of pancreas"
• Consider possibility of pseudo aneurysm, especially if
drainage is contemplated
Image Interpretation Pearls
• Correlate with ancillary imaging findings and clinical
setting of prior pancreatitis to confirm diagnosis and
avoid mismanagement
I SELECTED REFERENCES
1. Morgan DE et al: Pancreatic fluid collections prior to
intervention: Evaluation with MR imaging compared with
CT and US. Radiology. 203: 773-8, 1997
2. Lee MJ et al: Acute complicated pancreatitis: Redefining
the role of inter ventiona I radiology. Radiology. 183: 171-4,
1992
3. Sonnenberg EV et al: Percutaneous drainage of infected
and noninfected pancreatic pseudocysts: Experience in 101
cases. Radiology. 170: 757-61, 1989
 PANCREATIC PSEUDOCYST

I IMAGE GALLERY
Typical
(Left) Axial CECTshows
large pseudocyst displacing
stomach anteriorly. (Right)
Axial CECT in patient who
had large retrogastric
pseudocyst. High density
material within small
pseudocyst (arrow) is oral
contrast medium, indicating
spontaneous rupture of
pseudocyst into stomach. 3
27

Typical
(Left) Axial CECT shows
large pseudocyst resulting
from central pancreatic
necrosis. Pancreatic tail is
intact; pancreatic duct is
disrupted and empties into
pseudocyst. (Right) Axial
T2WI MR shows
hyperintense pseudocyst
within lesser sac.

Typical
(Left) Axial NECT shows
hyperdense clotted blood
within pseudocyst (arrow);
parenchymal calcifications
from chronic pancreatitis.
Ascites around spleen.
(Right) Axial CECT shows
intrasplenic subcapsular
pseudocyst resulting from
retroperitoneal extension of
inflammation from pancreas
into splenic hilum.
 CHRONIC PANCREATITIS

3
28

Axial CECT shows glandular atrophy, dilated main Axial CECT shows parenchymal atrophy, calcifications
pancreatic duct and intraductal calculi. and small pseudocysts.

• Pancreatic calcification
ITERMINOLOGY • Small, irregular calcifications (local or diffuse)
Definitions • Barium (UGI series)
• Irreversible inflammatory damage of pancreas usually o Changes seen in second part of duodenum
evident on imaging or functional testing • Varying degrees of atony
• Thickened, irregular & spiculated mucosal folds
• Stricture & proximal dilatation
I IMAGING FINDINGS • Enlarged papilla of Vater (Poppel papillary sign)
o Frostberg sign: Inverted-3 configuration of duodenal
General Features loop (seen occasionally)
• Best diagnostic clue: Atrophy of gland, dilated main • ERCP
pancreatic duct (MPD), intraductal calculi o Dilated & beaded MPD plus radicles
• Size: Pancreas usually decreased in size (atrophy) o MPD filling defects: Intraductal calculi
• Morphology o CBD may appear dilated with distal narrowing
o Inflammatory disease of pancreas characterized by CT Findings
irreversible damage to morphology & function
o Pancreatic calcification • NECT
o Gland
• Almost diagnostic of chronic pancreatitis
• Atrophy (more common)
• In 40-60% of patients with alcoholic pancreatitis
• Focal or diffuse enlargement (occasionally)
• Approximately 90% of calcific pancreatitis is
o Dilated MPD with ductal calculi
caused by alcoholism
o Intra & peripancreatic cysts
• Other features
o Thickening of peri pancreatic fascia
o In USA 75% of cases are due to alcoholism
o Splenic vein thrombosis, splenomegaly, varices
o Developing countries: Malnutrition & alcoholism
o Hypodense focal mass (fibrosis & fat necrosis)
Radiographic Findings • Usually in pancreatic head
• Radiography • May simulate neoplasm
o Plain x-ray abdomen • CECT
o Heterogeneous enhancement of pancreas

DDx: Dilated Main Pancreatic Duct and Gland Atrophy

Pancreatic Carcinoma Pancreatic Carcinoma IPMT of Pancreas IPMT of Pancreas

 CHRONIC PANCREATITIS
Key Facts
Imaging Findings
• Best diagnostic clue: Atrophy of gland, dilated main
pancreatic duct (MPD), intraductal calculi
• Pancreatic calcification
• Dilated MPD with ductal calculi
• Thickening of peri pancreatic fascia
• Splenic vein thrombosis, splenomegaly, varices
• Hypodense focal mass (fibrosis & fat necrosis)
• Heterogeneous enhancement of pancreas
• Pseudocyst contiguous with MPD
• CBD may be dilated with smooth distal tapering
Top Differential Diagnoses
• Pancreatic carcinoma
• IPMT of pancreas
o Mass due to chronic pancreatitis: Varied
enhancement due to presence or absence of fibrosis
• Fibroinflammatory mass: Common in pancreatic
head
• Hypoenhanced mass: Due to fibrosis
• Isodense enhancing mass: Lack of fibrosis
MR Findings
• TlWI GEl
o Decreased or loss of signal intensity
• Fat-suppressed T2WI
o Pseudocyst, necrotic areas: Hyperintense
o Gallstones, intraductal calculi: Hypointense
• Tl C+ GEl
o Heterogeneous enhancement pattern
• Nonenhancing decreased signal areas: Necrosis,
pseudocyst
• Pancreatic pseudocyst contiguous with dilated
MPD is well depicted
• Vascular occlusions can be demonstrated
• MRCP
o Fluid-containing structures are well depicted
• Dilated MPD plus radicles
• Pseudocyst contiguous with MPD
• CBD may be dilated with smooth distal tapering
Ultrasonographic Findings
• Real Time
o Atrophic gland
o Dilated MPD
• Tubular anechoic structure
• Seen in up to 90% of cases
o Echogenic foci (calcifications) with posterior
acoustic shadowing
o Pseudocyst
• Unilocular, anechoic & sharply defined
Imaging Recommendations
• NE + CECT; MR (fat-suppressed images); MRCP; ERCP
Pathology
• Chronic pancreatitis usually caused by alcohol abuse
Clinical Issues
• Clinical profile: Patient with history of chronic
alcoholism, recurrent attacks of mid-epigastric pain
radiating to back, jaundice, steatorrhea & diabetes
• Elevated serum amylase & lipase
Diagnostic Checklist
• Differentiate from other conditions which can cause
"MPD dilatation & glandular atrophy"
• Glandular atrophy with dilated MPD, ductal calculi, 3
thickened peri pancreatic fascia ± pseudocyst are best
signs for chronic pancreatitis 29
I DIFFERENTIAL DIAGNOSIS
Pancreatic carcinoma
• Irregular, heterogeneous, poorly enhancing mass
• Location: Head (60% of cases); body (20%); tail (15%)
• Atrophy of parenchyma may be seen
• Main pancreatic duct and/or common bile duct
o Obstruction & dilatation
• Obliteration of retropancreatic fat
• Extensive local invasion & regional metastases
o Local invasion to medial wall of duodenal sweep;
narrows lumen
• ERCP
o Irregular, nodular, rat-tailed eccentric obstruction
• 65% of patients present with advanced local disease &
distant metastases
• Some cases of chronic pancreatitis & pancreatic cancer
are impossible to differentiate without surgical
excision & histology
IPMT of pancreas
• IPMT: Intraductal papillary mucinous tumor
• Low grade malignancy arises from main pancreatic
duct (MPD) or branch pancreatic duct (BPD)
• Involvement of main pancreatic duct may simulate
chronic pancreatitis clinically & on CT/MR
• Dilated MPD and parenchymal atrophy
• ERCP: Best diagnostic tool
o Visualizes mucus ± polypoid lesions within MPD
I PATHOLOGY
General Features
• General path comments
o Embryology-anatomy
• Congenital anomalies may cause pancreatitis
• Pancreas divisum: Ducts too small to adequately
drain pancreatic secretions leading to chronic
stasis
• Annular pancreas: Pancreatic ductal obstruction
and stasis of secretions
o Chronic calcifying pancreatitis (alcoholism)
 CHRONIC PANCREATITIS
• Lesions are diffuse
o Chronic obstructive pancreatitis (gallstones)
• Lesions are more prominent in head of pancreas
• Pattern does not have a lobular distribution
• Genetics
o Hereditary pancreatitis
• Autosomal dominant & incomplete penetrance
• Etiology
o Chronic pancreatitis usually caused by alcohol abuse
o Gallstones, hyperlipidemia, trauma, drugs often
cause acute but rarely chronic pancreatitis
o Pathogenesis: Due to chronic reflux of pancreatic
enzymes, bile, duodenal contents & increased ductal
pressure
3 • MPD or terminal duct blockage
• Edema, spasm or incompetent sphincter of Oddi
30
• Periduodenal diverticulum or tumor
• Epidemiology: More common in developing countries
Gross Pathologic & Surgical Features
• Hard atrophic pancreas with intraductal calculi &
dilated MPD
• Areas of multiple parenchymal calcifications
• Pseudo cysts may be seen
Microscopic Features
• Atrophy & fibrosis of acini with dilated ducts
• Mononuclear inflammatory reaction
• Occasionally squamous metaplasia of ductal
epithelium
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Recurrent attacks of mid-epigastric pain, typically
radiates to back
o Jaundice, steatorrhea & diabetes mellitus
o Endocrine & exocrine deficiencies due to progressive
destruction of gland & stricture of common bile
duct (CBD)
o Mid-epigastric pain & weight loss
o Continued consumption of alcohol for a period of
3-12 years is usually required to develop
manifestations of chronic pancreatitis
• Clinical profile: Patient with history of chronic
alcoholism, recurrent attacks of mid-epigastric pain
radiating to back, jaundice, steatorrhea & diabetes
• Lab data
o Elevated serum amylase & lipase
o Increased blood glucose levels & fat in stool
o Secretin test: Decreased amylase & bicarbonate
Demographics
• Age: Usually middle age group
• Gender: Males more than females
Natural History & Prognosis
• Complications
o Diabetes
o Malabsorption
o Biliary obstruction; jaundice
o GI bleeding & splenic vein thrombosis
o Significant increase in pancreatic cancer incidence
• Prognosis
o Poor
Treatment
• Surgical or endoscopic intervention
o Ductal & GI obstruction; GI bleeding
o Large pseudocyst or persistently symptomatic
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other conditions which can cause
"MPD dilatation & glandular atrophy"
• May be very difficult to distinguish chronic
pancreatitis with a focal fibrotic mass (in head) from
pancreatic carcinoma
Image Interpretation Pearls
• Glandular atrophy with dilated MPD, ductal calculi,
thickened peri pancreatic fascia ± pseudocyst are best
signs for chronic pancreatitis
I SELECTED REFERENCES
1. Remer EM et al: Imaging of chronic pancreatitis. Radiol
Clin North Am. 40(6):1229-42, v, 2002
2. Manfredi R et al: Idiopathic chronic pancreatitis in
children: MR cholangiopancreatography after secretin
administration. Radiology. 224(3):675-82, 2002
3. Varghese JC et al: Value of MR pancreatography in the
evaluation of patients with chronic pancreatitis. Clin
Radiol. 57(5):393-401, 2002
4. Matos C et al: MR imaging of the pancreas: a pictorial tour.
Radiographies. 22(I):e2, 2002
5. Remer EM et al: Imaging of chronic pancreatitis. Radiol
Clin North Am. 40(6):1229-42, v, 2002
6. Varghese JC et al: Value of MR pancreatography in the
evaluation of patients with chronic pancreatitis. Clin
Radiol. 57(5):393-401, 2002
7. Matos C et al: MR imaging of the pancreas: a pictorial tour.
Radiographies. 22(1):e2, 2002
8. Ichikawa T et al: Duct-penetrating sign at MRCP:
usefulness for differentiating inflammatory pancreatic mass
from pancreatic carcinomas. Radiology. 221(1):107-16,
2001
9. Kim T et al: Pancreatic mass due to chronic pancreatitis:
correlation of CT and MR imaging features with pathologic
findings. A]R Am] Roentgenol. 177(2):367-71,2001
10. Manfredi R et al: Severe chronic pancreatitis versus
suspected pancreatic disease: dynamic MR
cholangiopancreatography after secretin stimulation.
Radiology. 214(3):849-55, 2000
11. Johnson PT et al: Pancreatic carcinoma versus chronic
pancreatitis: dynamic MR imaging. Radiology.
212(1):213-8, 1999
12. Sica GT et al: Comparison of endoscopic retrograde
cholangiopancreatography with MR
cholangiopancreatography in patients with pancreatitis.
Radiology. 210(3):605-10, 1999
13. Fulcher AS et al: MR pancreatography: a useful tool for
evaluating pancreatic disorders. Radiographies. 19(1):5-24;
discussion 41-4; quiz 148-9, 1999
14. Sica GT et al: Comparison of endoscopic retrograde
cholangiopancreatography with MR
cholangiopancreatography in patients with pancreatitis.
Radiology. 210(3):605-10, 1999
 CHRONIC PANCREATITIS
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
pancreatic calculi,
perisplenic and perigastric
varices (arrows) due to
obstruction of splenic vein.
(Right) MRCP shows long
tapered narrowing of CBD
(arrow) as it passes through
head of pancreas. Irregular
strictures and dilatation of
pancreatic duct (curved
arrow). Callbladder (open 3
arrow).
31

(Left) MRCP shows dilated

main pancreatic duct
(arrow) and adjacent
pseudocyst (open arrow).
(Right) ERCPshows irregular
dilatation of main pancreatic
duct and side branches.

(Left) Axial CECT shows

marked parenchymal
atrophy with fatty
replacement and a dilated
main pancreatic duct
containing calculi (arrow).
(Right) Chronic pancreatitis.
Axial CECT shows a large
heterogeneous mass in the
pancreatic head with
parenchymal calculi. No
tumor was found after
surgical resection.
 TRAUMATIC PANCREATITIS

3
32

Pancreatic transection. Axial CECT shows fracture plane Pancreatic transection. Axial CECT shows fracture plane
through neck of pancreas and peripancreatic edema through neck of pancreas. Pancreatic duct was
and hemorrhage. disrupted and body-tail of pancreas was resected at
surgery.

• Irregularity of pancreatic contour

ITERMINOLOGY • Edemalfluid in peri pancreatic fat
Abbreviations and Synonyms o Loss of normal fat plane: Peripancreatic infiltration
• Traumatic pancreatic injury • Heterogeneous parenchymal attenuation
• Thickening of anterior renal fascia
Definitions • Peripancreatic soft tissue changes of traumatic
• Inflammatory disease of pancreas secondary to trauma pancreatitis are often subtlei becoming more evident
within 24-48 hours
• Laceration
I IMAGING FINDINGS o Area of low attenuation (actual size of laceration
difficult to visualize)
General Features o Linear cleft, usually oriented anteroposteriorly
• Best diagnostic clue: Enlargement of gland, • Pancreatic fracture or transection
heterogeneous parenchyma, peripancreatic fluid o Ill-defined low density area
collections & history of trauma o Results in clear separation of two ends of gland
• Morphology: Spectrum of pancreatic injuries ranges o Nearly always extends through pancreatic neck
from acute pancreatitis, contusions, deep lacerations & • Lacerations/fractures may produce subtle changes in
fractures with ductal disruption parenchymal densityi may be undetectable on CT
• Extrapancreatic fluid collections: Perivascular,
Radiographic Findings transverse mesocolon, pararenal space, lesser sac,
• ERCP juxtasplenic, root of mesentery
o Normal in cases of pancreatic "contusion" • Pancreatic contusion/hematoma: Range from apparent
o Transection of pancreatic duct: Abrupt duct contour deformity of pancreas to rounded-mass
termination or contrast extravasation several centimeters in diameter
o Communication of pseudocyst with pancreatic duct o Peripancreatic hematoma may mimic fluid-filled
o May cause pancreatitis proximal small bowel loop
CT Findings o Often have concomitant injury to liver (left lobe)
• Focal/diffuse pancreatic enlargement and bowel

DDx: Peripancreatic Infiltration, Following Trauma

"Shock" Pancreas "Shock" Pancreas Duodenal Hematoma Duodenal Perforation

 TRAUMATIC PANCREATITIS
Key Facts
Imaging Findings
• Best diagnostic clue: Enlargement of gland,
heterogeneous parenchyma, peripancreatic fluid
collections & history of trauma
• Morphology: Spectrum of pancreatic injuries ranges
from acute pancreatitis, contusions, deep lacerations
& fractures with ductal disruption
• Irregularity of pancreatic contour
• Edema/fluid in peri pancreatic fat
• Rupture of main pancreatic duct (MPD) (23%)
• Protocol advice: 24-48 hours delayed scans may
uncover findings not present earlier
Top Differential Diagnoses
• "Shock" pancreas
• Intramural duodenal hematoma ± duodenal rupture
• Pancreatitis caused by ERCP (+/- papillotomy, etc.)
usually more severe in & around pancreatic head
MR Findings
• T1WI: Variable decreased signal intensity
• T2WI: Fluid collections & pseudocyst: Hyperintense
on fat suppressed T2WI
• T1 C+
o Heterogeneous enhancement pattern
o Nonenhancing hypointense areas (fluid collection,
pseudocyst, necrosis)
• MRCP
o Rupture of main pancreatic duct (MPD) (23%)
o Pseudocyst contiguous with MPD
Ultrasonographic Findings
• us (& CT) findings in traumatic pancreatitis may be
similar to those of non traumatic pancreatitis
• Not as sensitive as CT in diagnosing acute injury
Imaging Recommendations
• Best imaging tool
oCT: More accurate method of detecting
extrapancreatic fluid collections, pancreatic
lacerations or fractures
o Emergency ERCP to investigate pancreatic injuries
when CT shows injury, but status of pancreatic duct
is uncertain
• Protocol advice: 24-48 hours delayed scans may
uncover findings not present earlier
I DIFFERENTIAL DIAGNOSIS
"Shock" pancreas
• In severe injury; "hypoperfusion complex"
• Abnormally intense contrast-enhancement of
pancreas, bowel wall & kidneys
• Moderate to large peritoneal fluid collections
• Decreased caliber of aorta & inferior vena cava
• Diffuse dilatation of intestine with fluid
• Mesenteric and peripancreatic fat planes are infiltrated
• Findings resolve spontaneously within 24 hours of
fluid resuscitation
Pathology
• Penetrating/blunt trauma
• Trauma to pancreas is uncommon, accounts for
3-12% of all abdominal injuries
Clinical Issues
• Complications: Recurrent pancreatitis, pseudocyst,
hemorrhage, pseudoaneurysm, fistula & abscess
• Mortality from pancreatic injuries is nearly 20%
Diagnostic Checklist
• CT diagnosis of pancreatic trauma may be difficult in
selected patients who are scanned soon after injury 3
• CT signs of traumatic pancreatitis become more
evident after 24-48 hours 33
Intramural duodenal hematoma ± duodenal
rupture
• Hematoma
o Focal high attenuation thickening of duodenal wall
o Hemorrhage can cause a picket-fence appearance
o Smooth intramural mass causing incomplete bowel
obstruction
• Rupture
o Air/fluid level in adjacent extraperitoneal space
o Gas or fluid tracking into anterior pararenal space
o Extravasation of oral contrast into anterior pararenal
space
• May simulate or coexist with pancreatic injury
I PATHOLOGY
General Features
• Etiology
o Penetrating/blunt trauma
• Gunshot (45%), blunt (37%), stab wound (18%)
o Mechanism in blunt trauma
• Compression against vertebral column with shear
across pancreatic neck
• Relatively fixed position of pancreas anterior to
spine
o In children: Trauma from a bicycle handlebar,
motor vehicle accident, child abuse
o Lacerations usually accompany midline compression
injury, which may also involve left hepatic lobe,
duodenum, central renal vascular pedicle
o Endoscopic procedures:
• ERCP,especially with papillotomy, stone
extraction, stent placement
o Surgery: Billroth II resections, splenectomy, biliary
surgery, aortic graft surgery
• Epidemiology
o Trauma to pancreas is uncommon, accounts for
3-12% of all abdominal injuries
o Acute post traumatic pancreatitis is an infrequent
disease; representing 0.4% of acute pancreatitis with
pseudocyst formation
 TRAUMATIC PANCREATITIS
o Pancreatic penetrating injuries more common than o Superficial lesions not affecting major pancreatic
blunt trauma duct can be managed nonoperatively
o Combined injury of other organs seen in 80% of • Surgical: For lacerations or fractures
patients o Surgical drainage
o Accounts for about 5% of all abdominal injuries in o Partial pancreatectomy
childhood o Persistently elevated serum amylase levels &
o Trauma is most common cause of pseudocyst in increasing cyst size are indications for surgical
children (often related to child abuse) intervention
Staging, Grading or Classification Criteria
• Grade 1: Contusion/hematoma; pancreatic duct intact I DIAGNOSTIC CHECKLIST
• Grade 2: Parenchymal injury; pancreatic duct intact
• Grade 3: Major ductal injury Consider
• Grade 4: Severe crush injury • CT diagnosis of pancreatic trauma may be difficult in
3 • Grade of pancreatic injury is an independent predictor selected patients who are scanned soon after injury
of both pancreatic complications & mortality • Thickening of anterior renal fascia on CT of trauma
34 • Grade 1 & 2: Conservative management patient should prompt critical evaluation of the
• Grade 3 & 4: Require surgery within 24 hours pancreas
• CT signs of traumatic pancreatitis become more
evident after 24-48 hours
ICLINICAL ISSUES
Presentation I SELECTED REFERENCES
• Most common signs/symptoms
o History of traumatic injury 1. Kao LS et al: Predictors of morbidity after traumatic
pancreatic injury. J Trauma. 55(5):898-905, 2003
o Upper abdominal pain 2. Mayer JM et al: Pancreatic injury in severe trauma: early
o Postprandial vomiting, abdominal distention diagnosis and therapy improve the outcome. Dig Surg.
• Clinical profile 19(4):291-7; discussion 297-9,2002
o Serum amylase/lipase levels 3. Akhrass R et al: Pancreatic trauma: a ten-year
• Elevated in 90% of patients multi-institutional experience. Am Surg. 63(7):598-604,
• May be normal immediately after trauma 1997
o Leukocytosis, hyperamylasemia 4. Portis M et al: Traumatic pancreatitis in a patient with
o Diagnosis: Exploratory laparotomy pancreas divisum: clinical and radiographic features.
Abdom Imaging. 19(2):162-4, 1994
o Patients with penetrating trauma generally undergo
5. Lewis G et al: Traumatic pancreatic pseudocysts. Br J Surg.
immediate laparotomy 80(1):89-93, 1993
o Blunt injuries to pancreas may be clinically occult & 6. Jeffrey RBJr et al: Computed tomography of pancreatic
may go unrecognized on initial evaluation trauma. Radiology. 147(2):491-4, 1983
7. Ivancev K et al: Value of computed tomography in
Demographics traumatic pancreatitis in children. Acta Radiol Diagn
• Gender: More frequently seen in males (69%) (Stockh). 24(6):441-4, 1983
8. Chintapalli K et al: Renal fascial thickening in pancreatitis.
Natural History & Prognosis J Comput Assist Tomogr. 6(5):983-6, 1982
• Complications: Recurrent pancreatitis, pseudocyst,
hemorrhage, pseudo aneurysm, fistula & abscess
• Subcutaneous fat necrosis & polyarthritis secondary to
post-traumatic pancreatitis is reported in less than 1%
of patients with pancreatic disease
• Cerebral fat embolism is a rare possible complication
of traumatic pancreatitis
• Mortality from pancreatic injuries is nearly 20%
• Morbidity (42%)
o Pancreatic fistula (11 %)
o Pancreatitis (7%)
o Pancreatic pseudocyst (3%)
o Intra-abdominal abscesses (8%)
o Associated liver or intestinal injuries (> 80%)
• Morbidity is higher with external drainage compared
to exploration without drainage
Treatment
• Conservative
o Total parenteral nutrition
o Somatostatin or octreotide
o More recently, endoscopic with pancreatic stenting
 TRAUMATIC PANCREATITIS
I IMAGE GALLERY
Typical
(Left) Pancreatic contusion.
Axial CECTshows
heterogeneous mass effect at
pancreatic neck, fluid
between splenic vein and
pancreatic body. (Right)
Pancreatic contusion. Axial
CECTshows infiltration of fat
planes anterior and posterior
to pancreatic neck.
3
35

Typical
(Left) Pancreatic transection.
Initial axial CECT shows
subtle fracture plane and
hematoma in body of
pancreas (arrow). (Right)
Pancreatic transection. Axial
CECT 48 hours after trauma
shows "pseudocyst" in lesser
sac.

Typical
(Left) Pancreatic transection.
Axial CECTshows fracture
plane completely through
pancreatic neck. (Right)
Iatrogenic (post ERCP)
pancreatitis. Axial CECT
shows extensive infiltration of
fat planes and spaces around
pancreatic head, while
pancreatic body and tail are
uninvolved:
 SEROUS CYSTADENOMA OF PANCREAS

3
36

Graphic shows mass with a sponge or honeycomb Serous cystadenoma. Axial CECT shows a large mass in
appearance in the pancreatic head. Innumerable small the pancreatic head having a sponge appearance with a
cysts, a central scar and no obstruction of the pancreatic central scar. No pancreatic or biliary obstruction was
or bile duct. present.

o Tumors show increased frequency in patients with

ITERMINOLOGY von Hippel-Lindau disease
Abbreviations and Synonyms o Based on WHO subclassification: Two types
• Glycogen-rich or micro-/macrocystic serous adenoma • Serous micro cystic adenomas (more common)
• Serous oligocystic ("macrocystic" variant)
Definitions adenoma
• Benign pancreatic tumor that arises from acinar cells
Radiographic Findings
• ERCP
o Displacement, narrowing & dilatation of adjacent
I IMAGING FINDINGS MPD and/or CBD
General Features
CT Findings
• Best diagnostic clue: Honeycomb or sponge-like mass
in pancreatic head (micro cystic serous cystadenoma) • CECT
o Microcystic adenoma: Honeycomb pattern
• Location • Enhancement of septa delineating small cysts
o Head of pancreas (more common)
o Capsular enhancement is noted
o Can be seen in any part of pancreas
o Calcification within central scar may be seen
• Size o CBD and/or pancreatic duct dilatation may be seen
o Large cystic lesion varies from 5-10 cm
o Atrophy of pancreas distal to tumor (rarely)
o Innumerable small cysts (1-20 mm) within large
• Macrocystic serous cystadenoma (usually unilocular)
cystic lesion
o Location: Usually in pancreatic head
• Morphology o One or few cystic components (locules)
o Found incidentally in 10-30% of cases
o Thin non enhancing imperceptible wall
o Slowly growing tumors & may become quite large
o Otherwise indistinguishable from mucinous cystic
masses
tumor
o Calcification is more common in serous than
mucinous tumor (38:16%) MR Findings
o Most frequently seen in middle-aged/elderly women • TlWI

DDx: Cystic Pancreatic Mass

Pseudocyst Mucinous Cystic Pancreatic Carcinoma IPMT Pancreas

 SEROUS CYSTADENOMA OF PANCREAS

Key Facts
Terminology
• Glycogen-rich or micro-/macrocystic serous adenoma
• Benign pancreatic tumor that arises from acinar cells
Imaging Findings
• Best diagnostic clue: Honeycomb or sponge-like mass
in pancreatic head (microcystic serous cystadenoma)
• Enhancement of septa delineating small cysts
• Capsular enhancement is noted
• Macrocystic serous cystadenoma (usually unilocular)
• Thin nonenhancing imperceptible wall
Top Differential Diagnoses
• Pseudocyst
• Mucinous cystadenoma of pancreas
• Pancreatic carcinoma
• Intraductal papillary mucinous tumor (IPMT)
• Congenital pancreatic cysts
Pathology
• Etiology: Uncertain
• Associated abnormalities: Seen with increased
frequency in Von Hippel-Lindau disease patients
Clinical Issues
• Asymptomatic, epigastric pain, palpable mass
• Carcinoembryonic antigen level (CEA): Negative
• Gender: M < F (M:F = 1:4)
Diagnostic Checklist
• Rule out other "cystic pancreatic masses"
3
• Large, well-demarcated, lobulated cystic lesion
37
composed of innumerable small cysts (1-20 mm)
separated by thin septa located in head of pancreas

o Tumor: Hypointense o Round/oval, hypodense (near water HU)

o Blood within cysts: Varied intensity o Lobulated, mixed density lesion
o Central scar & calcification: Hypointense • Hemorrhagic or infected
• T2WI o Acute pancreatitis: Enlarged pancreas
o Tumor: Hyperintense o Chronic pancreatitis: Gland atrophy, dilated MPD &
o Central scar & calcification: Hypointense intraductal calculi
• T1 C+ • CECT: Rim- or capsule-enhancement
o Capsular enhancement • T1WI: Hypointense
o Enhancement of septa delineating small cysts • T2WI
o Central scar: Enhancement on delayed scan o Hyperintense (fluid); mixed intensity (fluid/debris)
• MRCP • MRCP: Hyperintense pseudocyst contiguous with MPD
o Depict pancreatic duct & CBD dilatation
Mucinous cystadenoma of pancreas
Ultrasonographic Findings • Most consider this tumor as premalignant
• Real Time • Location: Tail of pancreas (more common)
o Tumor with tiny cysts • CECT: Multiloculated cystic mass
• Hyperechoic mass but with through transmission o Enhancement of thin internal septa & wall
o Tumor with large cysts • T2WI
• Discrete anechoic areas with thin walls o Cysts: Hyperintense
o Calcification o Internal septations: Hypointense
• Highly reflective echoes with acoustic shadowing • Sonography: Multiloculated cystic mass with
echogenic internal septa
Angiographic Findings • Angiography: Predominantly avascular
• Conventional • Multilocularity or mural nodules favor tumor
o Highly vascular tumor due to extensive capillary • May be indistinguishable from macrocystic serous
network within septa cystadenoma of pancreas by imaging alone
o Neovascularity & dense tumor blush • Gross pathology: Multiloculated cystic mass with septa
o Large cysts: Produce lucent regions o Cysts
o Dilated feeding arteries • Fewer than 6 in number, larger than 2 cm
o Prominent draining veins • Peripheral calcification may be seen
Imaging Recommendations Pancreatic carcinoma
• NE + CECT, MR + CEMR, us • May appear cystic due to necrosis/fibrosis
Intraductal papillary mucinous tumor (IPMT)
I DIFFERENTIAL DIAGNOSIS • Low grade malignancy, arises from
o Main pancreatic duct (MPD)
Pseudocyst o Branch pancreatic duct (BPD) or combined
• Collection of pancreatic fluid encapsulated by fibrous • CECT
tissue o BPD lesion: Lobulated "multicystic" lesion
• Location: More common in body or tail • May show ring enhancement
• Usually unilocular, key is history of pancreatitis o MPD lesion: Markedly dilated & tortuous MPD
• Cystic mass with infiltration of peripancreatic fat o Combined type
• NECT • Cystic lesion in head & grossly dilated MPD
 SEROUS CYSTADENOMA OF PANCREAS
• MR
o T1WI: Hypointense BPD ± dilated, tortuous MPD
o T2WI: Hyperintense BPD cysts ± dilated MPD
o MRCP: Hyperintense cysts, dilated MPD
• BPD & combined type lesions of IPMT may simulate
serous microcystic adenoma due to presence of dilated
small branch ducts in pancreatic head
o Appear as "grape-like" clusters or small cysts
Congenital pancreatic cysts
• Examples: von Hippel-Lindau & ADPKD
• Rare, usually small & multiple non enhancing cysts
• No pancreatic ductal dilatation
3 Cystic islet cell tumor
• Usually non-insulin producing & nonfunctioning
• Tumor: Nonenhancing cyst contents
38
o No pancreatic ductal dilatation
• Angiography: Hypervascular primary & secondary
I PATHOLOGY
General Features
• General path comments
o Cell of origin: Centro acinar cell
o Positive staining for tumor cells is found with
epithelial membrane antigen & cytokeratins of low
and high molecular weights
o Tumor shares both clinical & pathologic
characteristics of biliary & ovarian tumors
o Tumors: Composed of smaller cysts (1-20 mm)
o Serous cystadenomas: No malignant potential
• Etiology: Uncertain
• Epidemiology
o Cystic pancreatic neoplasms are rare
o Accounts 10-15% of all pancreatic cysts
o Accounts only 1% of all pancreatic neoplasms
• Associated abnormalities: Seen with increased
frequency in von Hippel-Lindau disease patients
Gross Pathologic & Surgical Features
• Well-circumscribed, round/ovoid, cystic, multilocular
• Lobulated edges secondary to bulging cysts
• On cut section
o Honeycombed or spongy appearance (due to small,
innumerable cysts)
o Fluid in cysts
• Typically clear with no mucoid plugs
• Hemorrhagic (rarely)
o Thin fibrous septa radiating from central scar
o Dystrophic calcification within central scar
Microscopic Features
• Cysts are lined by cuboidal/flat epithelial cells
separated by fibrous septa
• Cells are glycogen-rich
• No cytologic atypia nor mitotic figures
• Areas of calcium; cholesterol clefts
• Hemosiderin-laden macrophages
• Pancreatic tissue adjacent to tumor is normal or
focally atrophic
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, epigastric pain, palpable mass
o Weight loss, jaundice, diabetes mellitus
o Other signs/symptoms of mass effect on adjacent
structures (stomach & bowel)
• Lab data
o Carcinoembryonic antigen level (CEA): Negative
• Diagnosis
o Endoscopic US with cyst aspiration & cytology
Demographics
• Age
o Middle & elderly age group (more common)
o Mean age 65 years
• Gender: M < F (M:F = 1:4)
Natural History & Prognosis
• Complications
o Due to mass effect
• Bowel (second part of duodenum) obstruction
• CBD obstruction (jaundice)
• Atrophy of pancreatic gland distal to tumor
• Prognosis
o Completely excised: Good prognosis
o No malignant potential
Treatment
• Asymptomatic & small tumors
o No surgical excision if confidently diagnosed
• Symptomatic & large tumors
o Complete surgical excision & follow-up
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other "cystic pancreatic masses"
Image Interpretation Pearls
• Large, well-demarcated, lobulated cystic lesion
composed of innumerable small cysts (1-20 mm)
separated by thin septa located in head of pancreas
I SELECTED REFERENCES
1. Goldsmith JD: Cystic neoplasms of the pancreas. Am J Clin
Pathol. 119 Suppl:S3-16, 2003
2. Sheth S et al: Imaging of uncommon tumors of the
pancreas. Radiol Clin North Am. 40(6):1273-87, vi, 2002
3. Yeh HC et al: Microcystic features at US: a nonspecific sign
for microcystic adenomas of the pancreas. Radiographies.
21(6):1455-61,2001
4. Curry CA et al: CT of primary cystic pancreatic neoplasms.
AJR. 175:99-103, 2000
5. Procacci C et al: Characterization of cystic tumors of the
pancreas: CT accuracy. Journal of Computer Assisted
Tomography. 23(6): 906-12,1999
6. Healy JC et al: CT of microcystic (serous) pancreatic
adenoma. J Comput Assist Tomogr. 18(1):146-8, 1994
7. BuckJL et al: From the Archives of the AFIP.Microcystic
adenoma of the pancreas. Radiographies. 10(2):313-22,
1990
 SEROUS CYSTADENOMA OF PANCREAS
[IMAGE GALLERY
Typical
(Left) Axial GCT shows
large sponge-like microcystic
serous cystadenoma in
pancreatic head. Small
calcifications are present in
the central scar and septa
(arrow). (Right) Axial CECT
shows a sponge-like
microcystic serous
cystadenoma in the
pancreatic head. 3
39

Variant
(Left) Axial T2WI MR shows
a unilocular macrocystic
serous cystadenoma in the
pancreatic head (Courtesy V.
Vilgrain, MO). (Right) MRCP
shows unilocular
macrocystic serous
cystadenoma in pancreatic
head (open arrow).

Typical
(Left) Axial CECT shows
multiloculated tumor in the
region of the pancreatic
head. Note fibrous septa
surrounding cystic structures
within tumor. (Right) Axial
CECT shows both
microcystic and macrocystic
components within this
serous cystadenoma.
 PANCREATIC CYSTS

3
40

Axial CECT shows a water density thin-walled cyst Endoscopic sonography shows a 2 cm diameter simple
(arrow) in the pancreatic neck. cyst in the neck of the pancreas.

ITERMINOlOGY CT Findings
• Imaging modalities show cystic nature of lesion
Abbreviations and Synonyms
• Round/oval homogeneous hypodense lesion with near
• Congenital/true cysts water density
Definitions o Thin imperceptible wall
• Congenital true pancreatic cyst is a very rare cause of MR Findings
cystic lesion of pancreas • Tl WI: Low signal intensity
• Refers to non-neoplastic, non-inflammatory cysts • T2WI: Very high signal intensity
Ultrasonographic Findings
I IMAGING FINDINGS • Anechoic; usually devoid of internal echoes
• Trauma & internal hemorrhage can cause a more
General Features complex appearance
• Best diagnostic clue: Differential diagnosis of true cyst
from other cystic lesions of pancreas is usually based Imaging Recommendations
on histology • Best imaging tool: Endoscopic US is diagnostic
• Size: Usually quite small; giant cyst as large as 15 cm procedure of choice
in diameter reported
• Morphology
o Round or oval shape, smooth thin wall, absence of I DIFFERENTIAL DIAGNOSIS
internal structures
o Usually unilocular Pseudocyst
o Solitary or multiple (associated with cystic • Usually more complex; history of pancreatitis
syndromes)
Serous cystadenoma
Radiographic Findings • Honeycomb-like microcystsi sponge-like mass with
• ERCP: No connection between cyst & pancreatic innumerable small cysts in pancreatic head
ductal system • Unilocular or macrocystic serous adenoma

DDx: "Pseudocyst"

Pseudocyst Serous Cystadenoma Mucinous Tumor Lymphangioma

 PANCREATIC CYSTS

Key Facts
Terminology Top Differential Diagnoses
• Refers to non-neoplastic, non-inflammatory cysts • Pseudocyst
• Serous cystadenoma
Imaging Findings • Mucinous cystic neoplasm
• Usually unilocular • Intraductal papillary mucinous tumor (IPMT)
• Round/oval homogeneous hypodense lesion with
near water density Diagnostic Checklist
• Thin imperceptible wall • May be impossible to distinguish from macrocystic
• Anechoic; usually devoid of internal echoes serous cystadenoma

• May be indistinguishable from true cyst except by

I DIAGNOSTIC CHECKLIST 3
histology of wall
Consider 41
Mucinous cystic neoplasm
• Septated mass, usually in body/tail of pancreas • Possibility of pseudocyst if history of pancreatitis
• Mutilocularity or mural nodules favor tumor • Excision for symptomatic or "complex" cystic
neoplasms
Intraductal papillary mucinous tumor (IPMT) • May be impossible to distinguish from macrocystic
• Dilated main pancreatic duct & adjacent cystic lesions serous cystadenoma
o Both are benign & of minimal clinical significance
lymphangioma
• Multiseptated mesenteric cystic mass Image Interpretation Pearls
• Imaging characteristics of simple pancreatic cysts on
CT & endosonography are uncommonly similar to
!PATHOLOGY those of benign cystic neoplasms

General Features
• Epidemiology
o Isolated cysts are very rare
o Comprise less than 1% of all pancreatic cysts
• Syndromes account for most non-neoplastic cysts
o von Hippel Lindau disease
o Autosomal dominant polycystic kidney (ADPKD)
o Beckwith-Wiedemann syndrome
Gross Pathologic & Surgical Features
• True epithelial lining (absent in pseudocysts)
Microscopic Features
• Cyst wall: Cuboidal epithelium
• High amylase & lipase contents of cyst
• Fluid of cyst does not contain any mucus
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, painless, epigastric mass
o Incidental finding; no history of pancreatic disease
Natural History & Prognosis
• "Simple" cyst :S 2 cm in asymptomatic adult is rarely
of any clinical significance
• Can follow with imaging, especially in elderly
Treatment
• Complete excision if symptomatic
• Laparotomy may be performed with presumptive
diagnosis of cystic tumor of pancreas
I SELECTED REFERENCES
1. Cohen-Scali F et al: Discrimination of unilocular
macrocystic serous cystadenoma from pancreatic
pseudocyst and mucinous cystadenoma with CT: initial
observations. Radiology. 228(3):727-33, 2003
2. Bergin D et al: Simple pancreatic cysts: CT and
endosonographic appearances. AJRAm J Roentgenol.
178(4):837-40,2002
3. Takahashi 0 et al: Solitary true cyst of the pancreas in an
adult: report of a case. lnt J Gastrointest Cancer.
30(3):165-70, 2001
4. Mao C et al: Solitary true cyst of the pancreas in an adult.
lnt J Pancreatol. 12(2):181-6, 1992
I IMAGE GALLERY
(Left) Axial NECT in patient with ADPKD shows multiple renal cysts,
some with calcified walls. Also present are several pancreatic cysts
(arrow). (Right) Axial CECT in a patient with von Hippel Lindau
syndrome shows several small cysts in pancreatic tail.
 MUCINOUS CYSTIC PANCREATIC TUMOR

3
42

Mucinous cystic tumor. Graphic show a multiseptated Axial CECT shows a mucinous cystic tumor in
mucin-filled cystic mass in the pancreatic tail that pancreatic tail with multiple septations and
displaces the pancreatic duct. displacement of the pancreatic duct.

ITERMINOLOGY Radiographic Findings

• ERCP
Abbreviations and Synonyms
o Displacement & narrowing of main pancreatic duct
• Mucinous macro cystic neoplasm, macro cystic adjacent to tumor
adenoma, mucinous cystadenoma or
cystadenocarcinoma CT Findings
Definitions • NECT
o Hypodense unilocular or multilocular cyst
• Thick-walled, uni-/multilocular low grade malignant o Focal calcifications may be seen (16% of cases)
tumor composed of large, mucin-containing cysts
• Location: Wall, septum or peripheral
• CECT
o Multilocular cystic lesion
I IMAGING FINDINGS • Enhancement of thin internal septa & cyst wall
General Features o Unilocular cystic lesion
• Enhancement of cyst wall
• Best diagnostic clue: Enhancing multiseptated mass in
body or tail of pancreas MR Findings
• Location: Tail of pancreas (more common) • T1WI
• Size: Varies from 2-12 cm in diameter o Variable signal intensity based on cyst content
• Morphology • Fluid-like material: Hypointense
o Classified under pancreatic mucinous tumors along • Proteinaceous or hemorrhagic: Hyperintense
with intraductal papillary mucinous tumor (IPMT) o Focal calcifications: Hypointense
of pancreas • T2WI
o Mucin producing tumors must be considered when o Cysts: Hyperintense
cystic lesions of pancreas are found o Internal septations: Hypointense
o Most consider this tumor as premalignant, if not o Focal calcifications: Hypointense
low grade malignancy
• T1 C+
o Fat suppression sequence

DDx: Cystic Pancreatic Mass

Pseudocyst Serous Cystadenoma IPMT Simple Cyst

 MUCINOUS CYSTIC PANCREATIC TUMOR

Key Facts
Terminology Pathology
• Thick-walled, uni-/multilocular low grade malignant • May be related to germ cell migration during 1st 8
tumor composed of large, mucin-containing cysts weeks of gestation
• Etiology: Is uncertain
Imaging Findings
• Best diagnostic clue: Enhancing multi septated mass Clinical Issues
in body or tail of pancreas • Asymptomatic, epigastric pain, palpable mass
• Variable signal intensity based on cyst content • Cyst fluid levels of CA 72-4 (more than 4 U/ml)
• Predominantly avascular mass
Diagnostic Checklist
Top Differential Diagnoses • Differentiate from other "cystic pancreatic lesions"
• Pseudocyst • Cyst aspiration & check for mucin/tumor markers
• Serous cystadenoma of pancreas • Large, multiloculated cystic mass with enhancing 3
• IPMT of pancreas septa & cyst wall in pancreatic body or tail
• Cystic islet cell tumor 43
• Congenital pancreatic cysts

• Enhancement of septations & cyst wall o Serous lesion usually has thinner wall
• MRCP
o Depicts displacement, narrowing & prestenotic IPMT of pancreas
dilatation of pancreatic duct • IPMT: Intraductal papillary mucinous tumor
• Low-grade malignancy arises from
Ultrasonographic Findings o Main pancreatic duct (MPD)
• Real Time o Branch pancreatic duct (BPD) or combined
o Multiloculated cystic mass with echogenic internal • BPD or combined type of IPMT may simulate
septa mucinous cystic neoplasm due to presence of dilated
o Unilocular anechoic mass cystic branch ducts in pancreatic tail
Angiographic Findings Cystic islet cell tumor
• Conventional • Usually non-insulin producing & nonfunctioning
o Predominantly avascular mass • Tumor: Cystic on NECT & non enhancing cyst
o Cyst wall & solid component contents
• Show small areas of vascular blush & o Cyst wall shows enhancement
neovascularity • No pancreatic ductal dilatation
o Displacement of surrounding arteries & veins by • Angiography: Hypervascular primary & secondary
cysts
Congenital pancreatic cysts
Imaging Recommendations • Examples: van Hippel-Lindau disease & autosomal
• MR + T1 C+; CECT dominant polycystic kidney disease (ADPKD)
• Rare, usually small & multiple nonenhancing cysts
• No pancreatic ductal dilatation
I DIFFERENTIAL DIAGNOSIS Variant of ductal adenocarcinoma
Pseudocyst • Mucinous colloid adenocarcinoma or
• Inflammatory changes in peri pancreatic fat mucin-hypersecreting cancer
• Pancreatic calcifications & temporal evolution of • Pancreatic ductal obstruction & dilatation
lesion • Local invasion & regional metastases
• Communicate with pancreatic duct (70% of cases)
lymphangioma
• Clinical history of pancreatitis or alcoholism
• Often extends from or into retroperitoneal soft tissues
• Lab data: Increased levels of amylase
• Water density; imperceptible wall; thin septations
• Simulates unilocular mucinous cystic tumor
Serous cystadenoma of pancreas
• Large, well-defined, encapsulated, sponge-like mass in !PATHOLOGY
pancreatic head
• Innumerable small cysts separated by thin septa General Features
• Central scar with calcification • General path comments
• Calcification more common in serous than mucinous o Embryology-anatomy
pancreatic neoplasms (38:16%) • May be related to germ cell migration during 1st 8
• Macrocystic variant of serous cystadenoma weeks of gestation
o Difficult to distinguish from mucinous tumor
 MUCINOUS CYSTIC PANCREATIC TUMOR
o A neoplasm with number of cysts less than 6 &
more than 2 em in diameter seen in 95% cases
o Stromal component is must for diagnosis of
mucinous cystic neoplasm
o Tumor shares both clinical & pathologic
characteristics of biliary & ovarian tumors
o Great propensity for invasion of adjacent organs
o Hypovascular mass with sparse neovascularity
• Etiology: Is uncertain
• Epidemiology
o Uncommon primary tumor of pancreas
o Frequency: 10% of pancreatic cysts & 1% of
pancreatic neoplasms
3 Gross Pathologic & Surgical Features
• Large encapsulated mass by thick fibrous capsule (2-12
44 em in diameter)
• Smooth & round; a lobulated surface may be seen
• Cut section: Multi-/unilocular large cysts
o More than 2 em; thin septa less than 2 mm thick
• Cystic cavity may be filled with thick mucoid
material/clear/green/blood-tinged fluid
• Solid papillary projections protrude into interior of
tumor (sign of cancer)
Microscopic Features
• Tall, mucin-producing columnar cells
• Subtended by a densely cellular mesenchymal stroma
• Characteristic ovarian-type stroma with spindle cells
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, epigastric pain, palpable mass
o Symptoms of mass effect on adjacent structures
(stomach/bowel)
o Rarely tumor may manifest with local
invasion/distant metastases
o Very rarely present with systemic manifestations
caused by tumor production of gastrin/VIP
• Laboratory data
o Increased levels of serum CEA
o Increased cyst fluid levels of CA 19-9 (80% of cases)
o Cyst fluid levels of CA 72-4 (more than 4 U/ml)
• 80% sensitivity & 95% specificity for tumor
• Diagnosis
o Endoscopic ultrasound with cyst aspiration/cytology
o Tumor markers
o Surgical resection
Demographics
• Age
o Mean age: 50 years (range of 20-95 years)
o 50% between 40-60 years
• Gender: Females more than males (M:F = 1:9)
Natural History & Prognosis
• Complications
o Due to mass effect
• Bowel obstruction
• Pancreatic duct narrowing or extrinsic obstruction
• Prognosis
o Completely excised: Good prognosis
o Incompletely excised, marsupialized or drained
• Poor prognosis
o 5 year survival rate with malignancy regardless of
surgery (74.3%)
Treatment
• Complete surgical excision
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other "cystic pancreatic lesions"
• Cyst aspiration & check for mucin/tumor markers
Image Interpretation Pearls
• Large, multiloculated cystic mass with enhancing
septa & cyst wall in pancreatic body or tail
I SELECTED REFERENCES
1. Maire F et al: Benign inflammatory pancreatic mucinous
cystadenomas mimicking locally advanced
cystadenocarcinomas. Presentation of 3 cases.
Pancreatology. 2(1):74-8, 2002
2. Hara T et al: Mucinous cystic tumors of the pancreas. Surg
Today. 32(11):965-9, 2002
3. Oshikawa 0 et al: Dynamic sonography of pancreatic
tumors: comparison with dynamic CT. AJR Am J
Roentgenol. 178(5):1133-7,2002
4. Yamaguchi K et al: Radiologic imagings of cystic neoplasms
of the pancreas. Pancreatology. 1(6):633-6, 2001
5. Balci NC et al: Radiologic features of cystie, endocrine and
other pancreatic neoplasms. Eur J Radiol. 38(2):113-9, 2001
6. Friedman AC et al: CT of primary cystic pancreatic
neoplasms: nihilism may be unwarranted. AJR Am J
Roentgenol. 177(2):469-70,2001
7. Megibow AJ et al: Cystie pancreatic masses: cross-sectional
imaging observations and serial follow-up. Abdom
Imaging. 26(6):640-7, 2001
8. Grogan J et al: Making sense of mucin-producing
pancreatic tumors. AJR. 176: 921-9, 2001
9. Sarr MG et al: Clinical and pathologic correlation of 84
mucinous cystic neoplasms of the pancreas: can one
reliably differentiate benign from malignant (or
premalignant) neoplasms? Ann Surg. 231(2):205-12, 2000
10. Lundstedt C et al: Serous and mucinous
cystadenoma/cystadenocarcinoma of the pancreas. Abdom
Imaging. 25(2):201-6, 2000
11. Taouli B et al: Intraductal papillary mucinous tumors of
the pancreas: helical CT with histopathologic correlation.
Radiology. 217(3):757-64, 2000
12. de Lima JE Jr et al: Mucinous cystic neoplasm of the
pancreas. Radiographies. 19(3):807-11, 1999
13. Le Borgne J et al: Cystadenomas and cystadenocarcinomas
of the pancreas: a multiinstitutional retrospective study of
398 cases. French Surgical Association. Ann Surg.
230(2):152-61, 1999
14. Buetow PC et al: From the Archives of the AFIP. Mucinous
cystic neoplasms of the pancreas: radiologic-pathologic
correlation. Radiographies. 18(2):433-49, 1998
 MUCINOUS CYSTIC PANCREATIC TUMOR

I IMAGE GALLERY
Typical
(Left) Axial CECT shows a
cystic mucinous tumor in the
pancreatic tail, with a few
large cystic spaces separated
by visible septa and focal
calcifications. (Right) Axial
CECT shows a cystic
mucinous tumor in the
pancreatic body containing a
few cystic spaces and septa.
Pancreatic duct is
compressed and dilated.
3
45

Typical
(Left) Axial CECT shows a
unilocular mucinous cystic
tumor in the pancreatic tail.
Visible non-calcified wall.
(Right) Coronal MRCP shows
cystic mucinous tumor in
pancreatic tail with a few
cystic spaces and septa.
Pancreatic duct is deviated
but otherwise normal.

Typical
(Left) Mucinous cystic
tumor. CECT in 20 year old
woman thought to have
pseudocysts. Lack of
resolution led to cyst
aspiration yielding thick
mucinous material with a
low amylase and high CA
79-9 levels. (Right) Axial
CECT shows a 7.5 cm cystic
mass (arrow) in the
pancreatic tail. Confirmed
mucinous cystic tumor at
surgical resection.
 IPMT, PANCREAS

3
46

Graphic shows combined main and branch type IPMT Axial CECT shows combined main and branch type
with gross dilatation of all ducts by mucin, which pours IPMT, with dilatation of all pancreatic ducts and
out of a bulging papilla into the duodenum. pancreaUc parenchymal auophy
Parenchyma in head is atrophic.

• Branch pancreatic duct (BPD) type: Focal

ITERMINOlOGY lobulated "multicystic" dilatation of branch ducts
Abbreviations and Synonyms • Main pancreatic duct (MPD) type: Diffuse
• Intraductal papillary mucinous tumor (IMPT) dilatation of main pancreatic duct
• Intraductal mucin-hypersecreting neoplasm, • Combined type: Dilatation of both BPD & MPD
ductectatic mucinous cystadenoma/carcinoma o Most common type is combined variety
• BPD plus MPD
Definitions
• Low grade malignancy that arises from epithelial
Radiographic Findings
lining of main pancreatic duct (MPD) and/or branch • ERCP
pancreatic ducts (BPD) with excessive mucin o Cystic BPDs, dilated MPD
production o Thick intraductal mucinous secretions
• Elongated or band-like filling defects in MPD
o Mucin & papillary tumors clearly detected as
jlMAGING FINDINGS nodular filling defects
o Diagnostic of IPMT of pancreas
General Features • Real-time visualization of patulous ampulla &
• Best diagnostic clue: "Multicystic" lesion in uncinate increased mucus production
process/head contiguous with dilated MPD on CECT
CT Findings
• Location
• BPD type: Lobulated "multicystic" lesion ("grape-like"
o BPD lesion: Uncinate process & head
clusters or tubes & arcs)
o MPD lesion: Usually body or tail
o Thin, irregular, peripheral ring-enhancing
• Size: BPD cysts: 5-20 mm
"multicystic" lesion
• Morphology
o Bulging ampulla at duodenal sweep with thin rim
o IPMT is a subdivision of mucin producing tumors
enhancement
along with mucinous macrocystic neoplasm
• MPD type: Markedly dilated tortuous MPD
o Classified into three types
o May visualize polypoid lesions lining MPD
o Punctate calcifications may be seen

DDx: Dilated Main Pancreatic Duct with or without Cyst

Chronic Pancreatitis Pancreatic Carcinoma Mucinous Cystic

 IPMT, PANCREAS
Key Facts
Terminology
• Intraductal papillary mucinous tumor (IMPT)
• Low grade malignancy that arises from epithelial
lining of main pancreatic duct (MPD) and/or branch
pancreatic ducts (BPD) with excessive mucin
production
Imaging Findings
• Best diagnostic clue: "Multicystic" lesion in uncinate
process/head contiguous with dilated MPD on CECT
• BPD type: Lobulated "multicystic" lesion ("grape-like"
clusters or tubes & arcs)
• MPD type: Markedly dilated tortuous MPD
• May visualize polypoid lesions lining MPD
• Combined type: "Multicystic" lesion in uncinate
process contiguous with grossly dilated MPD
• Combined type: "Multicystic" lesion in uncinate
process contiguous with grossly dilated MPD
• Atrophy of gland may be seen distal to tumor
MR Findings
• TlWI
o Axial: Hypointense branch duct cysts ± dilated MPD
o Coronal: Clustered cystic lesion with thin septa
• T2WI
o Hyperintense dilated branch duct cysts ± dilated
MPD
o May show papillary excrescence in cystic lesion of
pancreatic head or along MPD
• MRCP
o Lobulated clustered cysts, dilated MPD
o Reveals communication between cystic lesions/ducts
o Intraductal mucin & papillary tumors: May be
detected as nodular filling defects
Ultrasonographic Findings
• Real Time
o Septated cystic lesion
o Dilated MPD
o Punctate calcifications (occasionally)
o Intra-operative or endoscopic sonography affords
superior visualization
Imaging Recommendations
• MR, MRCP, ERCP & CT coronal & oblique
reconstructed images
• Thin-sections facilitate quality reformations
I DIFFERENTIAL DIAGNOSIS
Chronic pancreatitis
• Focal or diffuse atrophy of gland
• Obstruction & dilatation of pancreatic/bile ducts
• Intraductal calculi & areas of calcification
• Thickening of peripancreatic fascia
• Pseudocyst: Round, homogeneous, hypodense cystic
lesion
• ERCP &MRCP
o Obstruction & dilatation of pancreatic duct/radicles
• Reveals communication between cystic lesions/ducts
Top Differential Diagnoses
• Chronic pancreatitis
• Pancreatic pseudocyst
• Mucinous cystic neoplasm
• Serous cystadenoma
Diagnostic Checklist
• Rule out other cystic pancreatic pathologies
associated with dilated main pancreatic duct (MPD)
• Markedly dilated MPD & adjacent cystic lesions in
uncinate process or head of pancreas
• MRCP shows communication between cystic lesions
3
& dilated ductal system 47
o Pseudocyst contiguous with pancreatic duct
Pancreatic carcinoma
• Hypovascular mass with abrupt obstruction of ducts
Pancreatic pseudocyst
• Collection of pancreatic fluid encapsulated by fibrous
capsule
• Round/oval, homogeneous, hypodense cystic lesion
• Inflammatory changes in peripancreatic fat
• MRCP: Communicate with dilated pancreatic duct
(70% of cases)
• Clinical history of pancreatitis or alcoholism
• Sometimes mimics combined type of IPMT lesion
Mucinous cystic neoplasm
• Most consider this tumor as premalignant
• Septated globular mass
• Location: Tail of pancreas (more common)
• Enhancement of thin septa & wall
• Multilocularity or mural nodules favor tumor
• ERCP or MRCP: Displacement, narrowing & dilatation
of pancreatic duct adjacent to tumor
• Angiography: Predominantly avascular
• Gross pathology: Multiloculated cystic mass with septa
o Cysts
• Fewer than 6 in number, larger than 2 cm
• Peripheral calcification may be seen
Serous cystadenoma
• Glycogen-rich cystadenoma of pancreas
• Benign pancreatic tumor (arises from acinar cells)
• Slowly growing tumor & may become quite large
• Most frequently seen in middle-aged females
• Sponge-like mass of innumerable small cysts in head
o May simulate BPD type of IPMT in head
• ERCP or MRCP
'0 Displacement, narrowing & dilatation of pancreatic
duct adjacent to tumor
o No communication of lesion to pancreatic duct
• Calcification within central scar may be seen
• Enhancement of septa & cyst wall
• Angiography: Highly vascular tumor
 IPMT, PANCREAS
o Recurrent attacks of acute & chronic pancreatitis
[PATHOLOGY o Biliary disease
General features • Prognosis
o Localized lesion
• General path comments
o Tumors are typically papillary lesions • After resection have better prognosis than ductal
o Ranging in size from a few millimeters to panductal adenocarcinoma & mucinous cystadenocarcinoma
o Varying proportions of dysplasia & in situ cancer o Invasive carcinoma: Poor prognosis
• Etiology Treatment
o Uncertain • In older & less symptomatic cases
o Pathogenesis: Sequence of events in IPMT o Periodic monitoring of head/uncinate BPD variant
• Hyperplasia of columnar epithelial cells lining • In younger & more symptomatic cases
ducts o Complete surgical excision & frozen section analysis
• Dysplasia & proliferation to form papillary
3 projections

48
• Papillary projections protrude into & expand BPD
&MPD
I DIAGNOSTIC CHECKLIST
• Excessive mucin production, obstruction & Consider
dilatation of BPD/MPD • Rule out other cystic pancreatic pathologies associated
• Malignant transformation over many years with dilated main pancreatic duct (MPD)
• Epidemiology: Rare pancreatic cystic neoplasm
Image Interpretation Pearls
Gross Pathologic & Surgical features • Markedly dilated MPD & adjacent cystic lesions in
• BPD uncinate process or head of pancreas
o Cystically dilated branch ducts with rough internal • MRCP shows communication between cystic lesions &
surface dilated ductal system
o Few/multiple intraductal elevated papillary tumors
(adenomas)
o Communicating channels between cystic spaces I SELECTED REFERENCES
• MPD Sugiyama M et al: Predictive factors for malignancy in
1.
o Dilated MPD filled with mucin
intraductal papillary-mucinous turn ours of the pancreas. Br
o Flat elongated tumor (hyperplasia/malignancy) with J Surg. 90(10):1244-9, 2003
a rough surface 2. SaiJK et al: Management of branch duct-type intraductal
papillary mucinous tumor of the pancreas based on
Microscopic features magnetic resonance imaging. Abdom Imaging. 28(5):694-9,
• Simple hyperplasia, papillary adenomas, dysplasia, in 2003
situ carcinoma 3. Prasad SRet al: Intraductal papillary mucinous tumors of
• Innumerable papillary projections covered with the pancreas. Abdom Imaging. 28(3):357-65, 2003
columnar epithelial cells 4. Taouli B et al: Intraductal papillary mucinous tumors of
• Intervening septa that separate mucin-filled lacuna the pancreas: features with multimodality imaging. J
Comput AssistTomogr. 26(2):223-31, 2002
5. Peters HE et al: Magnetic resonance
cholangiopancreatography (MRCP)of intraductal
I CLINICAL ISSUES papillary-mucinous neoplasm (IPMN)of the pancreas: case
report. Magn Reson Imaging. 19(8):1139-43,2001
Presentation 6. LimJH et al: Radiologic spectrum of intraductal papillary
• Most common signs/symptoms mucinous tumor of the pancreas. Radiographies.
o BPD & MPD types 21(2):323-37; discussion 337-40, 2001
• Pain, weight loss, diarrhea, attacks of pancreatitis 7. SilasAM et al: Intraductal papillary mucinous tumors of
& diabetes the pancreas. AJRAm J Roentgenol. 176(1):179-85,2001
o Most specific predictive signs of malignancy 8. Taouli B et al: Intraductal papillary mucinous tumors of
the pancreas: helical CT with histopathologic correlation.
• Diabetes mellitus Radiology. 217(3):757-64, 2000
• Solid mass on imaging 9. Fukukura Y et al: Intraductal papillary mucinous tumors of
• Dilated MPD: More than 10 mm the pancreas: thin-section helical CT findings. AJRAmJ
• Diffuse/multifocal involvement Roentgenol. 174(2):441-7,2000
• Attenuating/calcified intraluminal content 10. Procacci C et al: Intraductal papillary mucinous tumor of
• Laboratory data the pancreas: a pictorial essay. Radiographies.
o Increased serum & urinary amylase 19(6):1447-63, 1999
o Altered pancreatic function tests 11. Ariyama J et al: Endoscopic ultrasound and intraductal
ultrasound in the diagnosis of small pancreatic tumors.
Demographics Abdom Imaging. 23(4):380-6, 1998
12. Koito K et al: Mucin-producing pancreatic tumors:
• Age: Onset between 60 & 80 years
comparison of MR cholangiopancreatography with
• Gender: M > F endoscopic retrograde cholangiopancreatography.
Natural History & Prognosis Radiology. 208(1):231-7, 1998
• Complications
 IPMT, PANCREAS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
combined branch pancreatic
duct (SPD) and main
pancreatic duct (MPD)
IPMT. Dilated main
pancreatic duct, glandular
atrophy, no mass. (Right)
Combined branch pancreatic
duct (SPD) and main
pancreatic duct (MPD)
IPMT. "Cystic" dilatation of 3
branch ducts in pancreatic
head and uncinate.
49

Typical
(Left) Coronal MRCP in
combined SPD and MPD
IPMT. Pancreatic duct (open
arrow) and common bile
duct (curved arrow) are
dilated. Cluster of dilated
mucin-filled branch ducts in
pancreatic head (arrow).
(Right) Endoscopic view
showing mucin pouring out
of a patulous papilla.

Typical
(Left) Malignant main
pancreatic duct (MPD)
IPMT. The main pancreatic
duct (open arrow) is grossly
distended. Solid papillary
tumor nodules are evident
(arrows). (Right) Malignant
main pancreatic duct (MPD)
IPMT. The MPD (open
arrow) is grossly dilated.
Solid papillary tumor nodules
are evident (arrows).
 PANCREATIC DUCTAL CARCINOMA

3
50

Graphic shows scirrhous mass in pancreatic head that Axial CECT (venous phase) shows hypodense mass
partially obstructs the common bile and pancreatic (arrow) in pancreatic head that encases splenoportal
ducts. The mesenteric vessels are encased by tumor; confluence & SMA (open arrow); occludes SMV. Body
celiac lymphadenopathy ispresent. & tailare atrophic; duct is dilated.

o Small & ill-defined or large tumor, with extensive

ITERMINOLOGY local invasion & regional metastasis
Abbreviations and Synonyms Radiographic Findings
• Pancreatic adenocarcinoma, pancreatic cancer
• Barium (UGI) study
Definitions o "Frostberg 3" sign
• Malignancy that arises from ductal epithelium of • "Inverted 3" contour to medial part of duodenal
exocrine pancreas sweep
o Spiculated duodenal wall, traction & fixation
o "Antral padding"
I IMAGING FINDINGS • Extrinsic indentation of posteroinferior margin of
antrum
General Features • ERCP
• Best diagnostic clue: Irregular, heterogeneous, o Irregular, nodular, rat-tailed eccentric obstruction
poorly-enhancing mass with abrupt obstruction of o Localized encasement with prestenotic dilatation
pancreatic and/or common bile duct ("double duct o "Double duct" sign: Obstruction of pancreatic and
sign") common bile duct at same level
• Location: Head (60%), body (20%), diffuse (15%), tail
CT Findings
(5%)
• NECT
• Size
o Isodense mass, no hemorrhage, Ca++ very rare
o Varies; average diameter is 2-3 cm
o Dilated pancreatic duct & obliteration of
o Large tumor can be up to 8-10 cm
retropancreatic fat
• Morphology
o Most common primary malignant tumor of exocrine • CECT
o Heterogeneous, poorly-enhancing mass
pancreas
o Parenchymal atrophy distal to tumor may be seen
o Accounts more than 75% of pancreatic tumors
o Pancreatic ductal dilatation distal to tumor
o Rarely resectable for cure
o Lesion in head may also cause CBD obstruction &
dilatation of bile ducts

DDx: Pancreatic Mass with or without Dilated Main Pancreatic Duct

Chronic Pancreatitis Islet Cell Tumor Metastases Lymphoma

 PANCREATIC DUCTAL CARCINOMA
Key Facts
Terminology
• Malignancy that arises from ductal epithelium of
exocrine pancreas
Imaging Findings
• Best diagnostic clue: Irregular, heterogeneous,
poorly-enhancing mass with abrupt obstruction of
pancreatic and/or common bile duct ("double duct
sign")
• Location: Head (60%), body (20%), diffuse (15%), tail
(5%)
• Small & ill-defined or large tumor, with extensive
local invasion & regional metastasis
• Parenchymal atrophy distal to tumor may be seen
• Hypovascular tumor
o Local tumor extension into splenic hilum & porta
hepatis
o Contiguous organ invasion
• Duodenum, stomach & mesenteric root
o Vascular invasion: "Tear drop" shaped SMV
• Encasement of more than half circumference of
vessel, narrowing or occlusion
• Mesenteric collateral veins may be seen
o Distant metastases
• Liver, peritoneum & regional nodes (common)
• Adrenals, bones, lungs & pleura (rare)
MR Findings
• TlWI
o Low signal intensity relative to normal parenchyma
due to fibrous nature of tumor
o Fat-suppressed Tl WI
• Hypointense lesion compared to high signal
intensity of normal pancreatic parenchyma
• T2WI: Variable signal intensity
• Tl C+
o Poor or no enhancement on dynamic study
o No significant diagnostic improvement over CT
• T2 GRE & Tl WI spin-echo sequences
o Detects vascular invasion
• MRCP: Show level & degree of ductal obstruction
Ultrasonographic Findings
• Real Time
o Hypoechoic mass & contour deformity of gland
o Pancreatic ductal dilatation distal to tumor
Angiographic Findings
• Conventional
o Hypovascular tumor
o Effective in detecting carcinoma of body & tail
• Demonstrates vascular narrowing
• Displacement or occlusion by tumor
Imaging Recommendations
• Helical CT
o With thin collimation (3-5 mm)
o Rapid IV bolus contrast injection
• CT&MR
Top Differential Diagnoses
• Chronic pancreatitis
• Islet cell carcinoma
• Metastases
• Lymphoma
Clinical Issues
• Usually asymptomatic until late in its course
• Obstructive jaundice, pain & weight loss
• Elevated levels of tumor markers: CEA, CA19-9
Diagnostic Checklist
• Differentiate from other solid pancreatic masses with 3
or without main pancreatic duct dilatation
• Irregular heterogeneous mass in head of pancreas 51
with eccentric ductal obstruction/dilatation &
extensive local invasion & regional metastases
o High predictive value (near 100%) for tumor
unresectability
o Less predictive value (75-85%): Resectable tumor
• ERCP; PET combined CT; endoscopic US
I DIFFERENTIAL DIAGNOSIS
Chronic pancreatitis
• Focal or diffuse atrophy of gland, fibrotic mass in head
• Dilated main pancreatic duct with ductal calculi
• Parenchymal calcification is also seen
• Distal CBD long stricture causes prestenotic dilatation
• Thickening of peripancreatic fascia & fat necrosis
• May be indistinguishable from cancer on imaging
Islet cell carcinoma
• Hypervascular primary & secondary tumors
• Ring enhancement seen in insulinoma
• No pancreatic ductal dilatation
• Usually functioning tumors are small in size &
non-functioning tumors are large in size
Metastases
• Hypovascular metastases (e.g., lung, colon)
• Hypervascular metastases (e.g., renal, melanoma)
• Rarely obstruct pancreatic and biliary ducts
Lymphoma
• May show focal or diffuse glandular enlargement
• Rarely obstructs ducts & widely disseminated in nodes
I PATHOLOGY
General Features
• General path comments
o Produces mucin & dense, collagenous desmoplastic
stroma
o Spread: Local, peri pancreatic, perivascular,
perineural & lymphatic invasion
• Genetics
o Mutations in K-ras genes & p16INK4 gene on
chromosome 9p21
 PANCREATIC DUCTAL CARCINOMA
o Abnormal high levels of p53 gene
• Etiology: Increased risk factors: Cigarette smoking,
diabetes mellitus, chronic pancreatitis, high-fat diet
• Epidemiology
o Fourth leading cause of cancer deaths in US
o 11th most common cancer in US
o Accounts 2-3% of all cancers
• Associated abnormalities
o Heritable syndromes
• Hereditary pancreatitis, ataxia telangiectasia
• Familial colon cancer, Gardner syndrome
• Familial aggregation of pancreatic cancer
Gross Pathologic & Surgical Features
3 • Hard nodular mass obstructing pancreatic duct/CBD
• Hypovascular, locally invasive, desmoplastic response
52
Microscopic Features
• White fibrous lesion, dense cellularity, nuclear atypia
• Most ductal cancers are mucinous adenocarcinomas
Staging, Grading or Classification Criteria
• Stage I
o Confined to pancreas (or)
o Extension into peri pancreatic tissues
• Stage II: Stage I plus regional lymph node metastases
• Stage III: Stage I & II plus distant metastases
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Usually asymptomatic until late in its course
o Head of pancreas
• Obstructive jaundice, pain & weight loss
o Body & tail
• Weight loss & massive metastases to liver
o Courvoisier law suggests pancreatic cancer
• Painless jaundice with a palpable gallbladder
o At presentation
• 65% patients: Advanced local disease/metastases
• 21%: Localized disease with spread to regional
lymph nodes
• 14%: Tumor confined to pancreas
o Lab data
• Elevated levels of tumor markers: CEA, CA19-9
Demographics
• Age
o Mean age at onset: 55 years
o Peak age: 7th decade
• Gender: M:F = 2:1
• Ethnicity: Blacks more than whites
Natural History & Prognosi"s
• Complications: Venous thrombosis, GI hemorrhage
• Prognosis
o 1 & 5 year survival (poor, even with surgery)
o With surgery (pancreaticoduodenectomy)
• 5-year survival rate is approximately 20%
o Without surgery
• 5-year survival rate is less than 5%
o Tumor markers used for prognosis are
• CEA, CA19-9 & CA 242
Treatment
• Complete surgical resection for potentially curative
tumor « 15%)
• Pancreaticoduodenectomy ("Whipple resection")
• Radiotherapy: External beam radiation
• Chemotherapy
• Endoscopic stenting-palliates obstructive jaundice
• Gastric bypass-palliates duodenal obstruction
• Chemical splanchnicectomy or celiac nerve block to
palliate abdominal pain
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other solid pancreatic masses with
or without main pancreatic duct dilatation
Image Interpretation Pearls
• Irregular heterogeneous mass in head of pancreas with
eccentric ductal obstruction/dilatation & extensive
local invasion & regional metastases
I SELECTED REFERENCES
1. Yusoff IF et al: Preoperative assessment of pancreatic
malignancy using endoscopic ultrasound. Abdom Imaging.
28(4):556-62,2003
2. Roche CJ et al: CT and pathologic assessment of
prospective nodal staging in patients with ductal
adenocarcinoma of the head of the pancreas. AJR Am J
Roentgenol. 180(2):475-80, 2003
3. Ly IN et al: MR imaging of the pancreas: a practical
approach. Radiol Clin North Am. 40(6):1289-306, 2002
4. Rodallec M et al: Helical CT of pancreatic endocrine
tumors. J Comput Assist Tomogr. 26(5):728-33, 2002
5. McNulty N et al: Multi-detector row helical CT of pancreas:
Effect of contrast enhanced multiphasic imaging on
enhancement of pancreas,peripancreatic vasculature, and
pancreatic adenocarcinoma. Radiology. 220: 97-102, 2001
6. Johnson D: Pancreatic carcinoma: Developing a protocol
for multi-detector row CT. Radiology. 220: 3-4, 2001
7. Jadvar H et al: Evaluation of pancreatic carcinoma with
FDG PET. Abdom Imaging. 26(3):254-9, 2001
8. Brizi MG et al: Staging of pancreatic ductal
adenocarcinoma with spiral CT and MRI. Rays.
26(2):151-9,2001
9. Ros PR et al: Imaging features of pancreatic neoplasms.
JBR-BTR. 84(6):239-49, 2001
10. Nishiharu T et al: Local extension of pancreatic carcinoma:
Assessment with thin-section helical CT versus with
breath-hold fast MR imaging-ROC analysis. Radiology. 212:
445-52, 1999
11. Demachi H et al: Histological influence on
contrast-enhanced CT of pancreatic ductal
adenocarcinoma. J Comput Assist Tomogr. 21(6):980-5,
1997
12. Ichikawa T et al: Pancreatic ductal adenocarcinoma:
preoperative assessment with helical CT versus dynamic
MR imaging. Radiology. 202(3):655-62, 1997
13. Soyer P et al: Involvement of superior mesenteric vessels
and portal vein in pancreatic adenocarcinoma: detection
with CT during arterial portography. Abdom Imaging.
19(5):413-6, 1994
 PANCREATIC DUCTAL CARCINOMA

I IMAGE GALLERY
Typical
(Left) CECT shows cancer
arising from pancreatic
head/uncinate with extensive
encasement of SMA (open
arrow) and splenoportal
confluence. Perigastric
collaterals (arrow) indicate
splenic vein occlusion.
(Right) Axial CECT shows
hypovascular mass in
pancreatic body (open
arrow) that occludes the 3
splenic artery and vein, with
perigastric varices (arrow). 53
Note liver metastases.

Typical
(Left) Transverse abdominal
sonogram shows hypoechoic
mass (cursors) within
pancreatic head (arrow).
Gastric antrum (open
arrow). (Right) Axial T1 C+
MR shows hypointense mass
(open arrow) within
pancreatic head. Note
teardrop shape of SMV
(arrow) indicating tumor
invasion.

Typical
~ , .. -
•••
(Left) Coronal TIWI MR
shows dilated biliary tree
obstructed by hypointense
mass (arrow) within
pancreatic head (Courtesy V.
Vilgrain, MO). (Right)
(j .' . Coronal MRCP shows
" J' . "double duct" sign:
-' .
. __:~5J
~ ~ Common bile duct and
pancreatic duct (arrow) are
obstructed at the same point

fJ~J; .•... ~ . '

#:",.
."")
by invasive pancreatic ductal
carcinoma.

, ,
 PANCREATIC ISLET CELL TUMORS

3
54

Axial CECT in arterial phase shows an 8 mm Insulinoma. Intra-operative sonography shows 8 mm

hyper vascular insulinoma (arrow) in pancreatic head hypoechoic mass (arrow) in pancreatic head, just lateral
that was not detected on portal venous phase CT. to superior mesenteric vein (SMV) (open arrow).
Opacified SMA & unopacified SMV (open arrow).

o May be hormonally functional (85%) or

ITERMINOlOGY
Abbreviations and Synonyms
• Pancreatic or gastroenteropancreatic neuroendocrine
tumor (NET)
Definitions
• Tumors arising from pancreatic endocrine
of Langerhans)
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Hypervascular mass(es) in
pancreas (primary) & liver (metastases)
• Location
o Pancreas (85%); ectopic (15%)
• Ectopic: Duodenum, stomach, nodes, ovary
o Gastrinoma: Gastrinoma triangle
• Superiorly: Cystic & common bile duct (CBD)
• Inferiorly: 2nd & 3rd parts of duodenum
• Medially: Pancreatic neck & body
• Size: Varies from few millimeters to 10 centimeters
• Morphology
o Rare compared to tumors of exocrine pancreas
o Benign or malignant
o Single or multiple (with different cell types)
nonfunctional
o Functioning tumors: Often secrete multiple
pancreatic hormones, with dominant single
defining clinical presentation
• Insulinoma, glucagonoma, gastrinoma,
somatostatinoma, VIPoma (vasoactive intestinal
cells (islets polypeptide), PPoma (pancreatic polypeptide),
APUDoma (carcinoid clinical syndromes)
o Nonfunctioning tumors
• Hypofunctioning or clinically silent large tumors
• Larger than functioning tumors at diagnosis
• Cystic islet cell tumor: Usually non-insulin
producing & non functioning
CT Findings
• Functioning tumors
o NECT
• Small or large in size; calcification may be seen
• Small lesions: Usually undetectable
• Cystic & necrotic areas (usually non-insulin
tumors)
o CECT: Arterial phase (AP) & portal venous phase
• Most are hypervascular (hyperdense on AP)
• Delayed scan: Solid/ring-enhancement
(insulinoma)
• Enhancing metastases (AP) in liver & nodes
• Nonfunctioning tumors
o NECT

DDx: Pancreatic Mass

Pancreatic Carcinoma Mucinous Cystic tumor Metastases (Rena/) Metastases (Rena/)

 PANCREATIC ISLET CEll TUMORS

Key Facts
Terminology
• Pancreatic or gastroenteropancreatic neuroendocrine
tumor (NET)
• Tumors arising from pancreatic endocrine cells (islets
of Langerhans)
Imaging Findings
• Best diagnostic clue: Hypervascular mass(es) in
pancreas (primary) & liver (metastases)
• Pancreas (85%); ectopic (15%)
• Cystic & necrotic areas (usually non-insulin tumors)
• Liver metastases often extensive even in relatively
healthy patient
Top Differential Diagnoses
• Pancreatic ductal adenocarcinoma
• Mucinous cystic tumor of pancreas
• Metastases
• Serous cystadenoma of pancreas
Pathology
• Insulinoma: Most common islet cell tumor
Clinical Issues
• Palpitations, sweating, tremors, headache, coma
• Can live with metastases for many years
Diagnostic Checklist
• Differentiate from other solid, cystic, vascular tumors
• Correlate with clinical & biochemical information
• Hypervascular pancreatic tumor & liver metastases
3
suggests islet cell/neuroendocrine tumor
55
• Large functioning & nonfunctioning tumors:
Hypervascular, complex & highly malignant

• Mixed density; usually large & complex • Detects very small lesions; sensitivity (75-100%)
• Cystic & necrotic areas (seen in large tumors)
• Calcification
Angiographic Findings
o CECT • Conventional
• Usually hypervascular o Functioning & nonfunctioning tumors
• Nonenhancing cystic or necrotic areas • Hypervascular (primary & secondary)
• Enhancing viable tumor o Hepatic venous sampling after intra-arterial
• Enhancing metastases (AP) stimulation of pancreas
• Liver metastases often extensive even in relatively • Functioning tumors: Elevated levels of hormones
healthy patient • Nonfunctioning: Decreased levels or absent
• Large functional & nonfunctional tumors: Highly Imaging Recommendations
malignant
• NE+ CECT
o Calcification; local invasion
• MR & T1 C+ (including fat-suppressed delayed images)
o Early invasion of portal vein leads to liver metastases
• Endoscopic ultrasound (EUS)
MR Findings • PET study with 68Ga(DFO)-octreotide
" Functional tumors o Complimentary in cases with disseminated disease
o Fat-saturated Tl WI: Hypointense
o T2WI SE & STIRsequences: Hyperintense (both
primary & secondaries) I DIFFERENTIAL DIAGNOSIS
o T1 C+
Pancreatic ductal adenocarcinoma
• Tl WI: Solid or ring-enhancement (insulinoma)
• Fat-saturated delayed enhanced TIWI SE: • Hypovascular tumor; pancreatic ductal obstruction
Hyperintense (solid enhancing lesions) • Location: Head (60%)
• Nonfunctioning tumors • Obliteration of retropancreatic fat
o T1WI SE image • Extensive local invasion & regional metastases
• Small tumors: Isointense • ERCP
• Large tumors: Heterogeneous (cystic & necrotic) o Irregular, nodular, rat-tailed eccentric obstruction
o T2WI SE image Mucinous cystic tumor of pancreas
• Small tumors: Isointense • Can be similar to cystic/necrotic islet cell tumor
• Large tumors: Hyperintense (cystic & necrotic) • Location: Tail of pancreas (more common)
o Tl C+
• NECT: Multiloculated hypodense mass
• Fat-saturated delayed enhanced Tl WI SE: • CECT: Enhancement of thin internal septa & wall
Hyperintense (small) • T2WI: Cysts (hyperintense); septations (hypointense)
• Nonenhancing (cystic + necrotic areas) & • Angiography: Predominantly avascular
increased enhancing viable tumor
Metastases
Ultrasonographic Findings • Examples: Renal cell carcinoma & melanoma
• Real Time • Small, well-defined, round hypervascular lesions
o Endoscopic ultrasound (EUS) • Indistinguishable from islet cell tumor metastases
• Detects small islet cell tumors
• Homogeneously hypo echoic mass Serous cystadenoma of pancreas
o Intra-operative US • Benign, glycogen-rich cystadenoma of pancreas
 PANCREATIC ISLET CELL TUMORS
• Honeycomb or sponge appearance
• Location: Head of pancreas (more common)
• Enhancement of septa delineating small cysts
• Cyst wall enhances; angiography (highly vascular)
• Macrocystic type: Thin wall/septa than cystic islet cell
I PATHOLOGY
General Features
• General path comments
o Embryology-anatomy
• Originate from embryonic neuroectoderm
• Etiology
3 o Arise from APUD cells
• APUD: Amine precursor uptake & decarboxylation
56 o Pathogenesis
• Insulinoma: B-cell tumor ~ hyperinsulinemia
hypoglycemia
• Gastrinoma: Islet cell tumor ~ increased gastrin ~
increased gastric acid ~ peptic ulcer
• Glucagonoma: (X-celltumor ~ increased
glucagon ~ erythema migrans & diabetes mellitus
• Nonfunctioning: Derived from (X& B cells
• Epidemiology
o Insulinoma: Most common islet cell tumor
• Solitary benign (90%); malignant (10%)
o Gastrinoma: 2nd common
• Multiple & malignant (60%); MEN 1(20-60%)
o Nonfunctioning: 3rd common
• Accounts 20-45% of all islet cell tumors
• Malignant (80-100%)
• Associated abnormalities
o Gastrinoma (Zollinger-Ellison syndrome)
• Associated with MEN type I
Gross Pathologic & Surgical Features
• Small tumor: Encapsulated & firm
• Large tumor: ± Cystic, necrotic, calcified
Microscopic Features
• Sheets of small round cells, uniform nuclei/cytoplasm
o Electron microscopy: Neuron specific enolase
("neuro-endocrine")
jClINICAL ISSUES
Presentation
• Most common signs/symptoms
o Insulinoma: Whipple triad (hypoglycemia + low
fasting glucose + relief by IV glucose)
• Palpitations, sweating, tremors, headache, coma
o Gastrinoma (Zollinger-Ellison syndrome)
• Peptic ulcer, increased acidity & diarrhea
o Glucagonoma: Necrolytic erythema migrans,
diarrhea, diabetes, weight loss
o Nonfunctional
• Mostly asymptomatic
• Pain, jaundice, variceal bleeding
Demographics
• Age
o Insulinoma: 4th-6th decade
o Gastrinoma: 4th-5th decade
• Gender
o Insulinoma: M < F
o Gastrinoma: M > F
Natural History & Prognosis
• Complications
o Glucagonoma: Deep venous thrombosis (DVT) &
pulmonary embolism
• Prognosis
o Insulinoma (good); gastrinoma (poor)
o Nonfunctional
• 3 year survival (60%), 5 year survival (44%)
• Can live with metastases for many years
Treatment
• Acute phase: Octreotide (potent hormonal inhibitor)
• Insulinoma: Surgery curative
~ • Gastrinoma
o Medical: Omeprazole, 5-fluorouracil
o Surgery curative in 30% cases
• Nonfunctional: Resection/embolization
• Transarterial chemoembolization: Liver metastases
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other solid, cystic, vascular tumors
• Correlate with clinical & biochemical information
Image Interpretation Pearls
• Hypervascular pancreatic tumor & liver metastases
suggests islet cell/neuroendocrine tumor
• Solid/ring-enhancement (insulinoma): Delayed scans
• Large functioning & non functioning tumors:
Hypervascular, complex & highly malignant
I SELECTED REFERENCES
1. Dromain C et al: MR imaging of hepatic metastases caused
by neuroendocrine tumors: comparing four techniques.
AJRAmJ Roentgenol. 180(1):121-8,2003
2. Marcos HB et al: Neuroendocrine tumors of the pancreas in
von Hippel-Lindau disease: spectrum of appearances at CT
and MR imaging with histopathologic comparison.
Radiology. 225(3):751-8, 2002
3. Ichikawa T et al: Islet cell tumor of the pancreas: biphasic
CT versus MR imaging in tumor detection. Radiology.
216(1):163-71,2000
4. Thoeni RF et al: Detection of small, functional islet cell
tumors in the pancreas: selection of MR imaging sequences
for optimal sensitivity. Radiology. 214(2):483-90, 2000
5. Stafford-Johnson DB et al: Dual-phase helical CT of
nonfunctioning islet cell tumors. J Comput Assist Tomogr.
22(2):335-9, 1998
6. Sohaib SA et al: Cystic islet cell tumors of the pancreas. AJR
AmJ Roentgenol. 170(1):217, 1998
7. Stafford Johnson DB et al: Dual-phase helical CT of
nonfunctioning islet cell tumors. J Com put Assist Tomogr.
22(1):59-63, 1998
8. Buetow PC et al: Islet cell tumors of the pancreas: clinical,
radiologic, and pathologic correlation in diagnosis and
localization. Radiographies. 17(2):453-72; quiz 472A-472B,
1997
 PANCREATIC ISLET CELL TUMORS

I IMAGE GALLERY
(Left) Axial CECT during
arterialphase, in patient with
metastatic glucagonoma.
Solid and ring-enhancing
hypervascular liver
metastases. (Right)
Malignant glucagonoma.
Axial CECT (arterial phase)
shows hyper vascular mass in
pancreatic tail (arrow) with
hyper vascular liver
metastases.
3
57

Typical
(Left) Malignant
non-functional NET. Axial
CECT (portal venous phase)
shows hypovascular mass in
pancreatic body with direct
invasion of splenic vein
(arrow). Hypodense liver
metastases. (Right)
Malignant non-functional
NET. Axial CECT (portal
venous phase) shows
hypodense mass in
pancreatic tail with focal
calcification (arrow). Liver
metastases.

(Left) Malignant
non-functioning NET. Axial
CECT (arterialphase) shows
large hypervascular mass in
pancreatic head. Note
absence of pancreatic ductal
dilatation. (Right) Benign
gastrinoma. Axial T2 WI MR
shows 7 cm hyperintense
mass (arrow) in pancreatic
head, just medial to
duodenum (open arrow).
 SOLID AND PAPILLARY NEOPLASM

3
58

Graphic shows large mass arising from pancreatic tail, Axial CECT in a 15 year old girl shows a large solid and
having prominent solid and cystic/hemorrhagic cystic mass in the pancreatic body/tail (Courtesy B.
components. Jeffrey, M 0).

o Thick capsule enhancement

ITERMINOLOGY
MR Findings
Abbreviations and Synonyms
• TlWI
• Solid and papillary epithelial neoplasm; papillary o Large well-demarcated mass with central areas of
epithelial neoplasm; papillary cystic carcinoma; solid low and high signal intensity
and cystic tumor of the pancreas o High signal intensity secondary to hemorrhage
Definitions o Capsule appears as rim of low intensity
• Pancreatic mass of low malignant potential with solid Ultrasonographic Findings
and cystic features • Fluid-debris levels; posterior enhancement
Angiographic Findings
I IMAGING FINDINGS • Avascular/hypovascular; depends on degree of necrosis
General Features Imaging Recommendations
• Best diagnostic clue: Well-demarcated large mass with • Best imaging tool: Helical CT
solid and cystic areas in pancreatic tail region in CT • Protocol advice
• Location: Commonly in body and/or tail of pancreas o Helical CT: Pancreatic mass protocol
• Size: Average 10 em, range of 2.5-20 em • 125 ml IV at 4 ml per second; water for oral
contrast
CT Findings
• NECT
o Encapsulated, hypodense mass I DIFFERENTIAL DIAGNOSIS
o ± Calcification (rare)
o ± Metastases to liver, lymph nodes Mucinous cystic pancreatic tumor
• CECT • Most common in middle age to elderly women
o Well-defined heterogenous large mass • Usually a spherical mass in body or tail of the pancreas
o Low density areas of variable size within the lesion; with several cystic spaces separated by thin septa
depends on degree of hemorrhage and necrosis

DDx: Cystic Pancreatic Mass

Serous Cystadenoma Muc. Cystic Tumor Gastric GIST Ovarian Cancer

 SOLID AND PAPILLARY NEOPLASM
Key Facts
Imaging Findings • Serous cystadenoma of pancreas
• Best diagnostic clue: Well-demarcated large mass with • Exophytic gastric mass
solid and cystic areas in pancreatic tail region in CT • Pancreatic pseudocyst
• Location: Commonly in body and/or tail of pancreas • Pancreatic metastases
• Low density areas of variable size within the lesion; Diagnostic Checklist
depends on degree of hemorrhage and necrosis
• Palpable pancreatic mass in a young
Top Differential Diagnoses African-American female
• Mucinous cystic pancreatic tumor • Encapsulated mass with prominent cystic and solid
components

Serous cystadenoma of pancreas Demographics

3
• CT • Age: < 35 years of age 59
o Usually located in head of pancreas • Gender: M:F = 1:9.5
o Typically has "sponge" appearance with innumerable • Ethnicity: African-Americans or other non-Caucasian
small cysts groups
o Never has thick soft tissue component like solid and
papillary neoplasm Natural History & Prognosis
• Complications: Hemorrhage, pseudocyst, sepsis, shock
Exophytic gastric mass • Prognosis: Good, after surgical resection; rarely recurs
• GIST may closely simulate solid and papillary
neoplasm Treatment
• Necrotic mass arising from gastric wall • Complete surgical excision
• Usually in older adult
Pancreatic pseudocyst I DIAGNOSTIC CHECKLIST
• CT: Cystic mass in or around pancreas; usually no
mural nodularity Consider
• History or signs of pancreatitis • Palpable pancreatic mass in a young African-American
female
Pancreatic metastases
• Example: Breast, lung, melanoma and ovarian cancer Image Interpretation Pearls
• Usually clinically silent; in older population • Encapsulated mass with prominent cystic and solid
components

I PATHOLOGY
I SELECTED REFERENCES
General Features
1. Madan AK et al: Solid and papillary epithelial neoplasm of
• General path comments the pancreas. J Surg Oncol. 85(4):193-8, 2004
o 0.13-2.7% of all pancreatic tumors 2. Buetow PC et al: Solid and papillary epithelial neoplasm of
o Low malignant potential the pancreas: imaging-pathologic correlation on 56 cases.
Radiology. 199(3):707-11, 1996
Gross Pathologic & Surgical Features 3. Choi BI et al: Solid and papillary epithelial neoplasms of
• Areas of central hemorrhage and necrosis surrounded the pancreas: CT findings. Radiology. 166(2):413-6, 1988
by solid and pseudopapillary structure
• Thick, fibrous, hypervascular capsule surrounding a
mixture of solid and cystic areas I IMAGE GALLERY
Microscopic Features
• Homogeneous, small epithelioid cells present singly,
in aggregates, small sheets or papillary structures

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic or abdominal pain
o Palpable abdominal mass
• Lab-Data: Normal
(Left) Axial CECT shows poorly defined solid and cystic mass in
pancreatic body. (Right) Axial NECT shows a solid and cystic
pancreatic tumor in an adolescent girl.
 PANCREATIC METASTASES AND LYMPHOMA

3
60

Axial CECT shows primary malignancy in left kidney Axial CECT in patient with new diagnosis of renal cell
renal cell carcinoma. carcinoma shows hypervascular metastases in
pancreatic tail (arrow) and a retroperitoneal lymph
node (open arrow).

• Hyperattenuation: Heterogenous (60%) or

ITERMINOLOGY homogenous (15%)
Definitions • Hypoattenuation (20%)
• Metastases from primary cancer of other sites • Isoattenuation (5%)
• Lymphoma: Malignant tumor of B lymphocytes o Concomitant intraabdominal metastases
• Liver (36%)
• Lymph nodes (30%)
I IMAGING FINDINGS • Adrenal glands (30%)
o Dilatation of the pancreatic duct or biliary tree not
General Features common (33%)
• Best diagnostic clue: Mass(es) in pancreas without o Encasement of the major peri pancreatic vascular
pancreatic or biliary ductal obstruction structure (rare)
• Other general features • Pancreatic lymphoma
o Pancreatic metastases o Homogeneous soft tissue mass
• Types of spread: Hematogenous, lymphatic, direct o Minimal enhancement
• Hematogenous spread: Most common o Focal and circumscribed single or multiple masses
o Pancreatic lymphoma o Diffuse enlargement of pancreas with infiltrating
• Secondary: Direct extension from peripancreatic tumor ± peri pancreatic fat involvement; may
lymphadenopathy simulate acute pancreatitis
• May be only apparent site of involvement o Peripancreatic lymph node enlargement
o ± Disseminated lymph nodes
CT Findings o Lymphadenopathy below level of renal veins;
• Pancreatic metastases feature of lymphoma
o Solitary (78%) or multiple (17%) o Dilatation of the pancreatic duct (uncommon);
o May cause diffuse infiltration distinguish feature from adenocarcinoma
o Discrete mass(es) more common o Encasement of the peripancreatic vessels
o Enhancement pattern is variable; mimics primary
tumor MR Findings
• Pancreatic metastases

DDx: Solid Pancreatic Mass

Pancreatic Cancer Ductal Carcinoma Islet Cell Tumor Mucinous Tumor

 PANCREATIC METASTASES AND LYMPHOMA

Key Facts
Imaging Findings
• Best diagnostic clue: Mass(es) in pancreas without
pancreatic or biliary ductal obstruction
• May cause diffuse infiltration
• Focal and circumscribed single or multiple masses
• Diffuse enlargement of pancreas with infiltrating
tumor ± peripancreatic fat involvement; may
simulate acute pancreatitis
• Peripancreatic lymph node enlargement
• Encasement of the peri pancreatic vessels
• Best imaging tool: Helical CT
Top Differential Diagnoses
• Pancreatic ductal carcinoma
• Pancreatic Islet cell tumors
• Mucinous cystic pancreatic tumor
Pathology
• Most common lymphoma is non-Hodgkin B cell
subtype
• Epidemiology: Increased diagnosis by incidental
findings by CT
Clinical Issues
• Asymptomatic or abdominal pain, jaundice, weight
loss
• Age: Usually in middle age and elderly
Diagnostic Checklist
• Check for history of primary cancer 3
• Overlapping radiographic features of pancreatic
metastases, lymphoma and primary carcinoma 61
• Consider renal cell metastases to pancreas even years
after resection of primary tumor

o TlWI • Most common in middle age to elderly women

• Hypointense ± fat-saturation
o Tl C+
• Rim of enhancement in larger tumors I PATHOLOGY
• Homogenous enhancement in smaller tumors
o T2WI General Features
• Slightly heterogenous and moderately • General path comments
hyperintense o Pancreatic metastases
• Diffuse, enlarged metastases may appear as • 3-10% at autopsy, less common "clinically"
hypo intense nodules o Pancreatic lymphoma
• Most common lymphoma is non-Hodgkin B cell
Imaging Recommendations subtype
• Best imaging tool: Helical CT • Primary: < 1% of pancreatic neoplasms
• Etiology
o Pancreatic metastases
I DIFFERENTIAL DIAGNOSIS • Renal cell carcinoma (30%)
• Bronchogenic carcinoma (23%)
Pancreatic ductal carcinoma • Breast carcinoma (12%)
• Location: Head (60%) • Soft tissue sarcoma (8%)
• CT • Colonic carcinoma (6%)
o Heterogenous, poorly-enhancing mass • Melanoma (6%)
o Abrupt pancreatic and/or common bile duct • Prostate carcinoma
obstruction • Ovarian carcinoma
o Encasement of vessels • Other gastrointestinal tumors
o Obliteration of retropancreatic fat o Pancreatic lymphoma
o Extensive local invasion & regional metastases • Primary (rare)
Pancreatic Islet cell tumors • Secondary (30% of patients with widespread
lymphoma)
• CT • Epidemiology: Increased diagnosis by incidental
o Ring-enhancement seen in insulinoma
o No pancreatic ductal dilatation findings by CT
o Functioning tumors usually small in size • Associated abnormalities
o Pancreatic lymphoma
o Non-functioning tumors usually large in size
• Immunocompromised patients, particularly HIV
• Hypervascular primary and secondary tumors
Mucinous cystic pancreatic tumor Gross Pathologic & Surgical Features
• Location: Body/tail of pancreas (more common) • Solitary or multiple; polypoid masses
• CT Microscopic Features
o Multiloculated hypodense mass • Pancreatic metastases: Varies based on primary cancer
o Enhancement of thin internal septa & wall • Pancreatic lymphoma: Lymphoepitheliallesions
• MR: T2WI
o Hyperintense cysts
o Hypointense internal septations
• Treated as malignant or premalignant
 PANCREATIC METASTASES AND LYMPHOMA
agents and multidetector technology. ] Com put Assist
I CLINICAL ISSUES Tomogr. 27 Suppl1:S17-22, 2003
3. Schima W et al: Evaluation of focal pancreatic masses:
Presentation comparison of mangafodipir-enhanced MR imaging and
• Most common signs/symptoms contrast-enhanced helical CT. Eur Radiol.
o Pancreatic metastases 12(12):2998-3008, 2002
• Asymptomatic or abdominal pain, jaundice, 4. Sheth S et al: Imaging of uncommon tumors of the
weight loss pancreas. Radiol Clin North Am. 40(6):1273-87, vi, 2002
• Acute pancreatitis (uncommon) 5. Hanbidge AE: Cancer of the pancreas: the best image for
early detection--CT, MRI, PET or US? Can] Gastroenterol.
o Pancreatic lymphoma
16(2):101-5,2002
• Abdominal pain 6. Scatarige]C et al: Pancreatic parenchymal metastases:
• Obstructive jaundice & hyperbilirubinemia observations on helical CT. A]R Am] Roentgenol.
(uncommon) 176(3):695-9,2001
• Diagnosis 7. Tamm E et al: Pancreatic cancer: current concepts in
3 o Pancreatic lymphoma
• Percutaneous or endoscopic biopsy
imaging for diagnosis and staging. Cancer]. 7(4):298-311,
2001
8. Salvatore]R et al: Primary pancreatic lymphoma: a case
62
Demographics report, literature review, and proposal for nomenclature.
• Age: Usually in middle age and elderly Med Oncol. 17(3):237-47,2000
9. Merkle EM et al: Imaging findings in pancreatic
• Gender
lymphoma: differential aspects. A]R Am] Roentgenol.
o Pancreatic metastases
174(3):671-5,2000
• M:F = 1:1 10. Kassabian A et al: Renal cell carcinoma metastatic to the
o Pancreatic lymphoma pancreas: a single-institution series and review of the
• M:F = 1.4:1 literature. Urology. 56(2):211-5, 2000
11. Ng CS et al: Metastases to the pancreas from renal cell
Natural History & Prognosis carcinoma: findings on three-phase contrast-enhanced
• Pancreatic metastases helical CT. A]R Am] Roentgenol. 172(6):1555-9, 1999
o Few months to several years after primary tumor; 12. Bouvet M et al: Primary pancreatic lymphoma. Surgery.
usually widespread 123(4):382-90, 1998
o Renal cell carcinoma 13. Klein KAet al: CT characteristics of metastatic disease of
the pancreas. Radiographies. 18(2):369-78, 1998
• Occasionally presents 5-10 years after diagnosis of
14. Merkle EM et al: Metastases to the pancreas. Br] Radiol.
primary tumor 71(851):1208-14, 1998
• May be isolated only to pancreas 15. Cario E et al: Diagnostic dilemma in pancreatic lymphoma.
• Prognosis Case report and review. Int] Pancreatol. 22(1):67-71,1997
o Pancreatic metastases 16. Ferrozzi F et al: Pancreatic metastases: CT assessment. Eur
• Very poor Radiol. 7(2):241-5, 1997
o Pancreatic lymphoma 17. Jones WF et al: AIDS-related non-Hodgkin's lymphoma of
• Poor, 30% cure rate after treatment the pancreas. Am] Gastroenterol. 92(2):335-8, 1997
18. Miller FH et al: Pancreaticobiliary manifestations of AIDS.
Treatment A]R Am] Roentgenol. 166(6):1269-74, 1996
19. Oas OK et al: Ultrasound guided percutaneous fine needle
• Pancreatic metastases
aspiration cytology of pancreas: a review of 61 cases. Trop
o Mostly palliative treatment only Gastroenterol. 16(2):101-9, 1995
o Surgical resection if metastases is only isolated to 20. Keogan MT et al: Computed tomography and magnetic
pancreas resonance imaging in the assessment of pancreatic disease.
• Pancreatic lymphoma Gastrointest Endosc Clin N Am. 5(1):31-59, 1995
o Chemotherapy 21. Friedman AC et al: Rare pancreatic malignancies. Radiol
Clin North Am. 27(1):177-90, 1989

I DIAGNOSTIC CHECKLIST
Consider
• Check for history of primary cancer

Image Interpretation Pearls

• Overlapping radiographic features of pancreatic
metastases, lymphoma and primary carcinoma
• Consider renal cell metastases to pancreas even years
after resection of primary tumor

I SELECTED REFERENCES
1. Fenchel S et al: Multislice helical CT of the pancreas and
spleen. Eur] Radiol. 45 Suppl1:S59-72, 2003
2. Merkle EM et al: Helical computed tomography of the
pancreas: potential impact of higher concentrated contrast
 PANCREATIC METASTASES AND LYMPHOMA
I IMAGE GAllERY
Typical
(Left) Axial CECT shows
large heterogenous mass in
pancreatic head and dilation
of the pancreatic duct due to
lymphoma, (Right) Axial
CECT in a patient with NH
lymphoma shows a large
heterogeneous pancreatic
head mass without
pancreatic or biliary ductal
dilation. The vessels are also 3
encased but not obstructed.

63

(Left) Axial CECT shows a

hypodense mass in
pancreatic tail due to
metastatic sarcoma. (Right)
Axial CECT shows multiple
hypodense masses in the
liver and pancreas due to
lung cancer.

••
(Left) Axial CECT in a patient
12 years post nephrectomy
for renal cell carcinoma
(RCC), Multiple pancreatic
hypervascular masses
(arrow) are proven RCC
. metastases, (Right) Axial

.• -
• CECT shows hypervascular
'~.'
, ' I~ , -,- .....• metastases to head of

..-.,
pancreas from renal cell

.-.: ~
' ~,_ ' ",' '. __ I, -. carcinoma, 12 years after
'
' .
:" , ~
,

.. ,

nephrectomy.

0«_
,.
.
: "
.
,.

..
,

'.,
~
~
.
.
"

..

~
.....

~
I. .•
.

, ••

~--
I

:1.,.,.
-
•••

•
-
 PART III
Genitourinary and Retroperitoneum

Retroperitoneum [I]
Adrenal []]

Kidney and Urinary Tract III

Ureter GJ 6
Bladder []]

Genetal Tract (Male) []]

SECTION 1: Retroperitoneum

Introduction and Overview

Retroperitoneum Anatomy and Imaging Issues 11I-1-2

Normal Variants
Duplications and Anomalies of Ive 11I-1-6

Inflam mation
Retroperitoneal Fibrosis 11I-1-10
Pelvic Lipomatosis 11I-1-14

Trauma
Retroperitoneal Hemorrhage 11I-1-16

Neoplasm
Retroperitoneal Sarcoma 11I-1-20
Retroperitoneal Lymphoma 11I-1-24
Retroperitoneal Metastases 11I-1-28

Treatment Related
Retroperitoneal Lymphocele 11I-1-32
 RETROPERITONEUM ANATOMY AND IMAGING ISSUES
1
2

Graphic shows 3 main divisions of retroperitoneum, the Axial NEeT in pancreatitis shows extensive infiltration of
anterior pararenal (yellow), perirenal (purple), and APS, limited laterally by the lateroconal fascia, and
posterior pararenal space (blue). Duodenum (not posteriorly by the renal fascia. Duodenum (open
shown) lies in APS. arrow); colon (arrow).

• Renal and lateroconal fascia are laminated planes and

I TERMINOLOGY can form spaces as pathways of spread for rapidly
Abbreviations and Synonyms expanding fluid collections or inflammatory processes
• Parietal peritoneum (PP) (e.g., hemorrhage, or pancreatitis)
• Anterior renal (Gerota) fascia (ARP) • Anterior renal fascia can "split" into a retromesenteric
• Posterior renal fascia (PRP) plane, which is continuous across the midline
• Lateroconal plane (fascia) (LCP) abdomen (green in graphic above)
• Retromesenteric plane (RMP) • Posterior renal fascia splits into retrorenal plane
• Retrorenal plane (RRP) • Lateroconal fascia splits into lateroconal plane
• All three of these planes communicate at the junction
of the lateroconal and renal fascia
I IMAGING ANATOMY Internal Structures-Critical Contents
Location • Sympathetic nerves and ganglia
o Extend in parallel paraspinal chains along spine
• Basic compartments
o Potential site of neural tumors
o Anterior pararenal space (APS)
• Inferior vena cava (IVe)
o Perirenal space (PS)
o Major conduit for thrombi from legs and pelvis to
o Posterior pararenal space (PPS)
lungs (pulmonary emboli)
• Compartments communicate inferiorly
o Major conduit for tumor emboli to lungs
• APS contains
(pulmonary metastases)
o Colon (ascending and descending)
• Kidneys, liver and adrenals are major organs of
o Pancreas
origin
o Duodenum (2nd and 3rd portions)
o Uncommon site of primary tumor
• Perirenal space contains
• Sarcoma of IVC
o Kidneys
o Adrenals • Aorta
o Atherosclerotic occlusive disease and aneurysm are
o Proximal ureters
major diseases
• PPS contains
• Rupture of aneurysm can cause hemorrhage into
o No solid organs
any or all of retroperitoneal compartments
Anatomic Relationships • Extension along renal hilum into perirenal space
• Parietal peritoneum separates peritoneal cavity from can simulate primary renal pathology
APS o Dissection
• Anterior renal fascia (Gerota) separates perirenal from • Rarely starts in abdomen, unless iatrogenic (e.g.,
APS angiography, placement of stent graft)
• Posterior renal fascia (Zuckerkandl) separates perirenal • Often spreads into abdominal aorta and its
from PPS branches from thoracic aortic dissection
• Lateroconal fascia separates from APS from PPS and o Inflammation
marks the lateral extent of the APS • Aortitis ("peri-aneurysmal fibrosis") can simulate
retroperitoneal fibrosis
 RETROPERITONEUM ANATOMY AND IMAGING ISSUES
DIFFERENTIAL DIAGNOSIS
Retroperitoneal neoplastic masses
• Lymphoma
• Metastatic lymphadenopathy
: Primary (benign and m~lignant)
=> Mesenchymal (e.g., lIposarcoma)
• => Neurogenic (e.g., paraganglioma)
• => Germ cell (e.g., teratoma)
Retroperitoneal non-neoplastic masses
: Hemato~?
PancreatitIs
• => ~E.g.,pseudocyst)
• Unnoma
: => (E.g., ~ost obs~ructi:ve, trauma)
Retropentoneal fibroSiS
• Extramedullary hematopoiesis
I CLINICAL IMPLICATIONS
Clinical Importance
• Disease within APS is common
o Pancreatic disease> duodenal> colonic
o Pancreatitis often spreads throughout APS to affect
duodenum and colon
• Duodenal effects: Spasm, fold thickening,
stricture, intramural pseudocyst, fistula
• Colonic effects: Same processes, affecting
ascending or descending colon; "colon cut off"
sign due to pancreatitis causing spasm or stricture
of anatomic splenic flexure
o Perforated duodenal ulcer
• May result in fluid and gas in APS plus
intraperitoneal
o Colonic inflammation
• Colitis, diverticulitis, epiploic appendagitis
• Disease within PS is common
o Any inflammatory or neoplastic process of kidney or
adrenal
o Renal fascia is very strong
• Effective in containing most primary renal
pathology within PS
• Effective in excluding most other pathology from
PS
o Perirenal space can be breached through renal hilum
or through opening in inferior cone of renal fascia
• Perirenal fluid
o Blood
• Trauma (including iatrogenic), tumor,
anticoagulation, abdominal aortic aneurysm,
vasculitis
• Attenuation: SO to 80 HU acutely
o Pus or inflammation
• Renal and perirenal abscess (or less commonly,
abscess originating outside kidney, such as
diverticulitis)
• Pancreatitis (may result in perirenal pseudocyst;
must breach renal fascia or enter through renal
hilum)
• Attenuation: Approximately 10 to 20 HU
1
Fat-containing retroperitoneal mass 3
• Liposarcoma
• Teratoma
• Primary or metastatic (testicular or ovarian)
• Myelolipoma
• => Adrenal
• Angiomyolipoma
• => Kidney
Retroperitoneal neurogenic tumors
• Usually paraspinal (sometimes intra-psoas)
• Often elongated, smooth, encapsulated
• Usually benign
• May enlarge neural foramina, extend intraspinally
• May be multiple (neurofibromatosis)
o Urine
• Trauma with parenchymal laceration into
collecting system or uretero-pelvic disruption
• Acute extravasation (common with ureteral
calculus; results in perirenal "stranding", not much
fluid)
• Chronic extravasation (results in urinoma, can
wall off into a "uriniferous pseudocyst")
• Attenuation: Varies from water to density of
contrast-opacified urine
• Retroperitoneal tumors
o Liposarcoma: Most common mesenchymal tumor
• Women slightly more than men
• Peak incidence between fourth and sixth decades
• Does not arise from a lipoma or normal
retroperitoneal fat
• Insidious symptoms, large size at diagnosis
• 90% have enough fat to be recognizable on CT
• Poorly differentiated or myxoid tumors (or parts
of a larger tumor) may have soft tissue or water
attenuation
• Complete removal often impossible; recurrence is
common
• Leiomyosarcoma
o Second most common primary mesenchymal tumor
o Most common in women, fifth or sixth decade
o May arise from within'IVe or retroperitoneal
smooth muscle
o Symptoms may reflect obstruction of IVC (e.g.,
Budd-Chiari syndrome, lower extremity edema)
o Like other sarcomas, often has a vascular periphery
and a necrotic core
• Malignant fibrous histiocytoma
o Does not contain fat or involve IVC
o Otherwise indistinguishable from other
retroperitoneal tumors
• Nerve sheath tumors
o Occur in younger patients
o May be isolated mass or extensive
(neurofibromatosis)
o May be benign or malignant
• Indistinguishable by imaging
 RETROPERITONEUM ANATOMY AND IMAGING ISSUES
1
4

Coronal reformauon of NECT in acute pancreatitis Sagittal reformation of NECT shows fat in perirenal
shows infilitrauon of fat in the APS outlining the fat in the space outlined by extensive infiltration of the APS.
perirenal space, separated by the renal fascia. Below the perirenal space, the anterior and posterior
pararenal spacesjoin (arrow).

o Key findings: Bilobed, dumbbell-shaped mass with

one component extending into neural foramen or I CUSTOM DIFFERENTIAL DIAGNOSISI
eroding spine or rib Retroperitoneal mesenchymal neoplasms
• Neuroblastoma, ganglioneuroblastoma,
ganglioneuroma (benign and malignant)
o Derived from sympathetic ganglion cells • Common
o Can arise anywhere along chain of sympathetic o Lipoma, liposarcoma (most common)
ganglia • Uncommon or rare
• Paraspinal or pre-sacral in location o Leiomyoma, sarcoma
o Neuroblastoma: Malignant tumor; children o Malignant fibrous histiocytoma
o Ganglioneuroblastoma: Variable age and biologic o Lymphangioma
behavior o Hemangioma
o Ganglioneuroma: Benign o Hemangiopericytoma
• Paraganglioma o Angiosarcoma
o Derived from paraganglionic cells nears sympathetic
chain
o Diagnosed at age 35-45, typically I SELECTED REFERENCES
o Most are hormonally active (identical to 1. Nishino M et al: Primary retroperitoneal neoplasms: CT
pheochromocytoma; catecholamines) with same and MRimaging findings with anatomic and pathologic
symptoms (headache, hypertension, palpitations, diagnostic clues. Radiographics.23(1):45-57,2003
sweating) 2. KoellerKKet al: Radiologicpathology. 2nd ed.
o Key findings: Very bright on T2WI MR; brightly Washington, DC, Armed ForcesInstitute of Pathology,
531-7,2003
enhancing; well-defined mass < 7cm 3. VivasI et al: Retroperitoneal fibrosis:typical and atypical
• Teratoma manifestations. BrJ Radiol. 73(866):214-22,2000
o Most common primary retroperitoneal tumor arising 4. BassJE et al: Spectrum of congenital anomalies of the
from an embryonic rest inferior vena cava: cross-sectionalimaging findings.
o Diagnosed in infancy, with second peak in early Radiographies.20(3):639-52,2000
adulthood 5. HeikenJP et al: Textbookof gastrointestinal radiology:
o Are mature, benign: Displace, rather than invade Peritoneal cavity and retroperitoneum: normal anatomy
o Key findings: Fat, calcification and soft tissue and examination techniques. 2nd ed. Philadelphia, WB
Saunders. 39-57, 2000
components 6. AizensteinRIet al: Interfascialand perinephric pathways
o Imaging may resemble liposarcoma, but teratoma is in the spread of retroperitoneal disease:refined concepts
more common in childhood based on CTobservations. AJRAmJ Roentgenol.
• Disease within the PPS is uncommon 168(3):639-43,1997
o Due to absence of viscera 7. MeyersMA:Dynamic radiology of the abdomen: normal
o Common site of hemorrhage due to coagulopathy and pathologic anatomy. 3rd ed. New York,Springer
o May be site of primary retroperitoneal sarcoma -Verlag,1-100, 1988
o Disease originating in other space can "invade" PPS
(e.g., diverticulitis)
 RETROPERITONEUM ANATOMY AND IMAGING ISSUES
I IMAGE GALLERY
1
5

(Left) Axial CECT shows

large aortic aneurysm with
bleeding into perirenal and
anterior pararenal spaces,
distending the
retromesenteric plane.
(Right) Axial CECT shows
spontaneous
"retroperitoneal"
hemorrhage (heparin) into
iliopsoas compartment.
Hematocrit effect (arrow).

(Left) Axial CECT shows

shows a liposarcoma with
fatty and myxoid elements
displacing kidney and bowel.
(Right) Axial CECT shows
huge liposarcoma, mostly
myxoid, displacing bowel
and obstructing ureters.

(Left) Axial CECT shows

retroperitoneal fibrosis as a
mantle of soft tissue
surrounding aorta and IVC,
and obstructing left ureter.
(Right) Axial CECT shows
large soft tissue mass
surrounding aorta + IVC but
causing no obstruction;
metastatic testicular
carcinoma.
 DUPLICATIONS AND ANOMALIES OF IVC
1
6

Graphic shows transposition of IVC (on left picture); Graphic shows circumaortic left renal vein, with smaller
duplication of IVC (right). Note that duplicated IVC ventral vein crossing cephalad to dorsal vein (left). Right
continues as left iliac vein and empties into left renal graphic shows completely retroaortic renal vein.
vein.

o Usually, left Ive ends at left renal vein, which

ITERMINOlOGY crosses anterior to aorta in normal fashion to join
Definitions right Ive
• Congenital anomalies of inferior vena cava (lve) o Left and right Ive may have significant asymmetry
in size
• Left Ive
IIMAGING FINDINGS o Left Ive ends at left renal vein, which crosses
anterior to aorta in normal fashion, uniting with
General Features right renal vein to form normal right suprarenal Ive
• Best diagnostic clue: Malposition or duplication of Ive o t Enhancement of right renal vein relative to left
inferior to renal vein renal vein (dilution from unenhanced venous return
• Other general features from lower extremities)
o Types of Ive anomalies • Azygos continuation of the Ive
• Duplication of or double Ive o Ive passes posterior to diaphragmatic crus to enter
• Left Ive thorax as azygos vein
• Azygos continuation of the Ive o Azygos vein joins superior vena cava at normal
• eircumaortic left renal vein location in right peribronchial location
• Retroaortic left renal vein o Hepatic veins drain directly into right atrium
• Duplication of Ive with retroaortic right renal o Enlarged azygos vein is similar in attenuation to
vein and hemiazygos continuation of the Ive superior vena cava
• Duplication of Ive with retroaortic left renal vein o Gonadal veins drain to ipsilateral renal veins
and azygos continuation of the Ive • Duplication of Ive with retroaortic right renal vein
• eircumcaval or retrocaval ureter and hemiazygos continuation of the Ive
• Absence of infrarenal or entire Ive o Left and right Ive inferior to renal vein
o Right Ive ends at right renal vein, which crosses
CT Findings posterior to aorta to join left Ive
• Duplication of Ive o Suprarenal Ive passes posterior to diaphragmatic
o Left and right Ive inferior to renal vein crus to enter thorax as hemiazygos vein

DDx: Paraaortic Soft Tissue "Mass" Simulating IVC

Lymphadenopathy IVC Stenois, Collaterals Col/aterals Cirrhosis, Varices

 DUPLICATIONS AND ANOMALIES OF IVC

Key Facts
1
Terminology Pathology 7
• Congenital anomalies of inferior vena cava (lVC) • Duplication of IVC: 0.2-3% of general population
Imaging Findings Clinical Issues
• Best diagnostic clue: Malposition or duplication of • Asymptomatic
IVC inferior to renal vein • Usually diagnosed incidentally by imaging
• Usually, left IVC ends at left renal vein, which crosses • Prognosis: Very good
anterior to aorta in normal fashion to join right IVC • Usually no treatment
• Left and right IVC may have significant asymmetry in
size Diagnostic Checklist
• Best imaging tool: CT; consider multiplanar • Duplication of IVC should be suspected in recurrent
reformations pulmonary embolism following IVC filter placement
• Pre-operative imaging may be important in planning
Top Differential Diagnoses abdominal surgery, liver or kidney transplantation or
• Retroperitoneal lymphadenopathy interventional procedures (e.g., IVC filters, varicocele
• Varices/Collaterals sclerotherapy, venous renal sampling)
• Gonadal vein

o In thorax, collateral pathways for hemiazygos vein • Right renal artery crossing anteriorly to azygos vein;
include may demonstrate azygos continuation of the IVC
• Crosses posterior to aorta at T8-9 to join azygos
vein Angiographic Findings
• Continues superiorly to join coronary vein of • Most accurate diagnostic method
heart via persistent left superior vena cava Imaging Recommendations
• Accessory hemiazygos continuation to left
• Best imaging tool: CT; consider multi planar
brachiocephalic vein
reformations
• Duplication of IVC with retroaortic left renal vein and
azygos continuation of the IVC
o Mixture of findings previously mentioned
• Circumaortic left renal vein (common variant)
I DIFFERENTIAL DIAGNOSIS
o 2 left renal veins Retroperitoneal lymphadenopathy
• Superior renal vein joined by left adrenal vein and • E.g., metastases and lymphoma, granulomatous
crosses aorta anteriorly disease
• Inferior renal vein, 1-2 cm below to superior renal • Left sided paraaortic adenopathy; may mimic
vein, joihS by left gonadal vein and crosses aorta duplication of or left IVC
posteriorly o Differentiate by renal vein drainage or
• Retroaortic left renal vein contrast-enhancement of IVC
o 1 left renal vein, crosses aorta posteriorly • Retrocrural adenopathy; may mimic enlarged azygos
• Circum caval ureter vein in retrocrural space
o Proximalureter courses posterior IVC, emerges to o Differentiate by tubular structure of azygos vein
right of aorta and lies anterior to right iliac vessels extending from diaphragm to azygos arch
• Absence of infrarenal or entire IVC o Retrocrural adenopathy lacks enhancement
o External and internal iliac veins join to form • Retroperitoneal adenopathy; may mimic circumaortic
enlarged ascending lumbar veins left renal vein
o Venous return from lower extremities to azygos and
hemiazygos vein via anterior paravertebral collateral Varices/Collaterals
veins • E.g., cirrhosis, IVC obstruction
o ± Suprarenal IVC formed by left and right renal
veins Gonadal vein
o May be acquired abnormality following thrombosis • May appear as para aortic soft tissue "mass" or mimic
of IVC left sided IVC
• Follow inferiorly; does not "join" left iliac vein
MR Findings
• Flow voids or flow-related enhancement; may
distinguish aberrant vessels from masses I PATHOLOGY
Ultrasonographic Findings General Features
• Infrahepatic IVC ends with azygos or hemiazygos • General path comments
continuation o Embryology
• Hepatic veins drain directly into right atrium
 DUPLICATIONS AND ANOMALIES OF IVC
1 • 6-8th gestational weeks: lnfrahepatic IVC develops
from appearance and regression of three paired
8 embryonic veins; postcardinal, subcardinal and
supra cardinal veins
• Normal IVC comprised of hepatic, suprarenal,
renal and infrarenal segments
• Hepatic segment develops from vitelline vein
• Suprarenal segment develops from
sub cardinal-hepatic anastomosis
• Renal segment develops from right
supra-subcardinal and post-subcardinal
anastomoses
• lnfrarenal segment develops from right
supracardinal vein
• In thorax, supracardinal veins form to azygos and
hemiazygos veins
• In abdomen, sub cardinal and supracardinal veins
progressively replace postcardinal veins
• In pelvis, postcardinal veins form common iliac
veins
• Etiology
o Congenital
o Risk factor: First degree relatives
o Pathogenesis
• Duplication of IVC: Persistence of both
supracardinal veins
• Left IVC: Regression of right supracardinal vein
with persistence of left supracardinal vein
• Azygos continuation of the IVC: Failure to form
right sub cardinal-hepatic anastomosis, resulting
atrophy of right sub cardinal vein
• Circumaortic left renal vein: Persistence of dorsal
limb of embryonic left renal vein and of dorsal
arch of renal collar (intersupracardinal
anastomosis)
• Retroaortic left renal vein: Persistence of dorsal
arch of renal collar and regression of ventral arch
(intersubcardinal anastomosis)
• Duplication of IVC with retroaortic right renal
vein and hemiazygos continuation of the IVC:
Persistence of left lumbar and thoracic
supracardinal vein and left suprasubcardinal
anastomosis, with failure to form right
sub cardinal-hepatic anastomosis
• Duplication of IVC with retroaortic left renal vein
and azygos continuation of the IVC: Persistence of
left supracardinal vein and dorsal limb of the
renal collar with regression of ventral arch, with
failure to form sub cardinal-hepatic anastomosis
• Epidemiology
o Prevalence
• Duplication of IVC: 0.2-3% of general population
• Left IVC: 0.2-0.5%
• Azygos continuation of the IVC: 0.6%
• Circumaortic left renal vein: 8.7%
• Retroaortic left renal vein: 2.1 %
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Circum caval ureter: Partial right ureteral obstruction
or recurrent urinary tract infections
o Absence of infrarenal or entire IVC: Venous
insufficiency of lower extremities or idiopathic deep
venous thrombosis
• Diagnosis
o Usually diagnosed incidentally by imaging
Demographics
• Age: Any age
Natural History & Prognosis
• Prognosis: Very good
Treatment
• Usually no treatment
• Circum caval ureter: Surgical relocation of ureter
anterior to IVC
I DIAGNOSTIC CHECKLIST
Consider
• Duplication of IVC should be suspected in recurrent
pulmonary embolism following IVC filter placement
Image Interpretation Pearls
• Pre-operative imaging may be important in planning
abdominal surgery, liver or kidney transplantation or
interventional procedures (e.g., IVC filters, varicocele
sclerotherapy, venous renal sampling)
I SELECTED REFERENCES
1. Yilmaz E et al: Interruption of the inferior vena cava with
azygos/hemiazygos continuation accompanied by distinct
renal vein anomalies: MRAand CT assessment. Abdom
Imaging. 28(3):392-4, 2003
2. BasileA et al: Embryologic and acquired anomalies of the
inferior vena cava with recurrent deep vein thrombosis.
Abdom Imaging. 28(3):400-3, 2003
3. Brochert A et al: Unusual duplication anomaly of the
inferior vena cava with normal drainage of the right IVC
and hemiazygous continuation of the left IVe.] Vase
Interv Radiol. 12(12):1453-5,2001
4. Bass]E et al: Spectrum of congenital anomalies of the
inferior vena cava: cross-sectional imaging findings.
Radiographies. 20(3):639-52, 2000
5. Bass]E et al: Absence of the infrarenal inferior vena cava
with preservation of the suprarenal segment as revealed by
CT and MRvenography. A]RAm] Roentgenol.
172(6):1610-2, 1999
6. Mayo] et al: Anomalies of the inferior vena cava. A]RAm]
Roentgenol. 140(2):339-45, 1983
7. Breckenridge]W et al: Azygos continuation of inferior vena
cava: CT appearance.] Comput AssistTomogr. 4(3):392-7,
1980
8. Garris]B et al: Ultrasonic diagnosis of infrahepatic
interruption of the inferior vena cava with azygos
(hemiazygos) continuation. Radiology. 134(1):179-83, 1980
9. Royal SAet al: CT evaluation of anomalies of the inferior
vena cava and left renal vein. A]RAm] Roentgenol.
132(5):759-63, 1979
10. Faer M] et al: Inferior vena cava duplication:
demonstration by computed tomography. Radiology.
130(3):707-9, 1979
 DUPLICATIONS AND ANOMALIES OF IVC
I IMAGE GALLERY 1
9
Typical
(Left) Axial CECT shows
duplicated IVC at level of
renal vein which is dilated
(arrow) as it receives the
left-sided IVC. (Right) Axial
GCT shows duplicated IVC
(arrows) with a smaller
left-sided vessel.

Typical
(Left) Axial CECT shows
duplicated IVC with equal
size vessels. (Right) Axial
GCT shows duplicated IVC.
The left common iliac vein
appears normal, but does
not cross to join the right
iliac vein.

Typical
(Left) Axial CECT shows
polysplenia syndrome with
absent IVC and continuation
of dilated azygous vein
(arrow). (Right) Axial CECT
shows polysplenia syndrome
with absent IVC and
continuation of dilated
azygous vein (arrow).
 RETROPERITONEAL FIBROSIS
1 .- I

~('*
10

,,~
r
~

.@2~i
~

=->-)

Graphic shows encasement

,'"'- and displacement of Retrograde pyelogram shows encasement and
mid-ureters by a band of fibrous tissue. Hydronephrosis. displacement of mid-ureters by retroperitoneal fibrosis.

• May involve root of mesentery & adjacent organs

ITERMINOLOGY • Extend via crus of diaphragm ~ thorax as fibrous
Definitions mediastinitis
• Chronic inflammatory process in lumbar • Fibrotic mass in true pelvis involving iliac vessels
retroperitoneum + lower ureters
o 15% cases associated with fibrotic process elsewhere
Radiographic Findings
I IMAGING FINDINGS • Excretory urography (IVU)
General Features o Classic triad
• Upper ureteral hydronephrosis (above L4/5)
• Best diagnostic clue: Mantle of soft tissue encasing
• Medial deviation of ureters in middle third,
aorta, inferior vena cava (lVe) & ureters
typically bilateral
• Location: Lower lumbar region & extends into pelvis
• Gradual tapering of ureters, extrinsic compression
• Morphology
• Retrograde pyelography (RGP)
o Exuberant mass of woody fibrous tissue that usually
o Pyelocaliectasis & ureterectasis to the level of L4-5
encases abdominal aorta, IVC & ureters
o Medial deviation of ureters
o Pinkish or glistening
o Valuable study to assess location, extent & severity
• Other general features
of ureteral obstruction
o Two types based on etiology
• Fluoroscopic guided barium enema
• Primary or idiopathic (common): 2/3 cases
o Retroperitoneal fibrosis involving pelvic cavity may
• Secondary: 1/3 cases
show extrinsic compression, displacement of rectum
o Classification based on pathology & radiology
or rectosigmoid loop
• Limited (common) & extensive (rare)
• Malignant & nonmalignant CT Findings
o Limited type (common form) • Fibrous plaque
• Common manifestation: Isolated plaque over o Soft tissue density fibrotic plaque or mantle of
lower lumbar spine variable thickness
o Extensive type (rare form)

l
, ,
,
DDx: Mantle of Tissue Around Aorta

..
o,;~:
.
.•
'"
)I

,. :~

"Aortitis
~
Testicular Mets.
~.

Leaking AAA Retroperitoneal Bleed

 RETROPERITONEAL FIBROSIS
Key Facts
Terminology
• Chronic inflammatory process in lumbar
retroperitoneum
Imaging Findings
• Best diagnostic clue: Mantle of soft tissue encasing
aorta, inferior vena cava (IVe) & ureters
• ± Displacing structures anteromedially/anterolaterally
• Rarely invades aorta, IVC & ureters
• 1 Enhancement: Active inflammatory process
• I Enhancement: Well-organized fibrous tissue
Top Differential Diagnoses
• Aortitis
• Retroperitoneal metastases & lymphoma
• Ruptured abdominal aortic aneurysm (AAA)
• Retroperitoneal hemorrhage
o Plaque may extend from crus of diaphragm to
common iliac vessels
• Most commonly extends from renal hilum to
pelvic brim
• Rarely extends to involve kidneys, pancreas,
spleen, mediastinum
• Plaque may be asymmetrical, sharply localized or
very extensive
o Usually surrounds aorta, IVC & ureters; ± caudal
extension to iliac vessels
• ± Displacing structures
anteromedially / an terolaterally
• ± Compression or narrowing, proximal dilatation
• Rarely invades aorta, IVC & ureters
• No tissue plane visible between fibrosis & muscles
o Contrast enhancement varies depending on
maturity of fibrous tissue
• 1 Enhancement: Active inflammatory process
• I Enhancement: Well-organized fibrous tissue
o Difficult to differentiate a confluent malignant
retroperitoneal adenopathy & fibrosis
MR Findings
• Tl WI: Low-medium homogeneous signal intensity
• T2WI
o Low-moderate heterogeneous signal intensity
o Signal intensity, less than fat but more than muscle
Ultrasonographic Findings
• Real Time: Hypoechoic "halo": Irregularly contoured
periaortic soft tissue mass
Imaging Recommendations
• NE + CECT; IVU or RGP
I DIFFERENTIAL DIAGNOSIS
Aortitis
• Severe degree is known as inflammatory abdominal
aortic aneurysm or perianeurysmal fibrosis
• Cause: Unknown, may be due to hypersensitivity to
antigens in atheromatous plaques
• Gross pathologic finding
1
Pathology 11
• Primary (idiopathic): Probably autoimmune disease
with antibodies ~ stimulate desmoplastic reaction
• Drugs: Methysergide, B blocker, hydralazine,
ergotamine, LSD
• Epidemiology: Prevalence, 1 in 200,000 population
• Other associated inflammatory fibrotic processes
• Mass of woody fibrous tissue; pinkish & glistening
Clinical Issues
• Extrinsic ureteral obstruction ~ renal failure
• Great vessels (aorta & IVe) obstruction
Diagnostic Checklist
• Check for underlying malignancy, AAA
• Mantle or rind of soft tissue density encircling great
vessels & ureters in retroperitoneum
o Thick cuff of firm, fibrous granulation tissue around
aorta encompassing adjacent structures
• Morphology & histology are identical to idiopathic
retroperitoneal fibrosis
• Imaging
o NECT
• Perivascular, irregular mantle of tissue with
variable attenuation
• Isodense or hypodense to aorta
o CECT
• Enhancing fibrotic rind of variable attenuation
• Ureters: Normal; displaced or obstructed
o Multiplanar CT & MR imaging: Demonstrate
relationship of perianeurysmal mass to aneurysm,
ureters & psoas muscle
o MRA: Evaluates blood flow in affected vessels
o Aortography: Determines extent of aneurysmal
disease & its relationship to peripheral vessels
Retroperitoneal metastases & lymphoma
• Metastases
o E.g., prostate, cervix, breast & lung carcinoma
o May cause mantle of tissue + desmoplastic response
similar to retroperitoneal fibrosis
o Additional pelvic + retroperitoneal nodes usually
seen
o Usually more discrete or asymmetrical
• Lymphoma
o May simulate retroperitoneal fibrosis, especially after
treatment
o Rarely obstructs ureters
o Usually involves higher nodes as well
Ruptured abdominal aortic aneurysm (AAA)
• Location of AAA
o Usually infrarenal with extension to iliac arteries
• NECT
o Thickened diaphragmatic crura
o "Enlarged" left psoas muscle
o Aortic outline is obscured by soft tissue density
• CECT
o Saccular or fusiform dilatation of aorta
o Indistinct focal area of aortic wall
 RETROPERITONEAL
1 o Anterior displacement of kidney
o Extravasation of contrast material
12 o Fluid collection (hematoma) within posterior
pararenal + perirenal spaces
Retroperitoneal hemorrhage
• Most cases are iatrogenic
o Usually due to over anticoagulation
• Rupture of abdominal aortic aneurysm
o Second most common cause of retroperitoneal bleed
• Spontaneous tumor bleed is 3rd most common cause
o Kidney: Renal cell carcinoma & angiomyolipoma
o Adrenal: Carcinoma or myelolipoma
• CT findings
o Acute: High density fluid collection or hematoma
o Chronic: Low density (organized clot)
o Associated renal, adrenal tumors or aortic aneurysm
• MR findings
o Varied signal intensity (evolution of blood products)
o Hyperacute phase: Due to oxyhemoglobin
• Tl WI; slightly hypointense; T2WI; hyperintense
o Acute phase: Iso-/hypointense on Tl WI & markedly
I signal on T2WI due to deoxyhemoglobin
o Chronic phase: I Signal (TlWI); 1 signal (T2WI)
• Diagnosis: History of anticoagulation; imaging
evidence of tumor or aneurysm
!PATHOLOGY
General Features
• Etiology
o Primary (idiopathic): Probably autoimmune disease
with antibodies ~ stimulate desmoplastic reaction
• Ormond speculation: Retroperitoneal fibrosis is
similar to collagen vascular disease, supported by
coexistence with other inflammatory processes
o Secondary
• Drugs: Methysergide, B blocker, hydralazine,
ergotamine, LSD
• Diseases that stimulate desmoplastic reaction:
Malignant tumors, metastases, Hodgkin, carcinoid
tumor, hematoma, radiation, retroperitoneal
injury, surgery, infection, urinary extravasation
• Epidemiology: Prevalence, 1 in 200,000 population
• Associated abnormalities
o Other associated inflammatory fibrotic processes
• Pseudotumor of orbit; Reidel thyroiditis
• Sclerosing cholangitis; chronic fibrosing
mediastinitis
Gross Pathologic & Surgical Features
• Mass of woody fibrous tissue; pinkish & glistening
• Encases vessels & ureters
Microscopic Features
• Early: Collagen, plasma cells, histiocytes, giant cells
• Late: Acellular fibrosis
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Pain: Back, flank, abdomen
FIBROSIS
o Renal insufficiency, HTN, leg edema, anemia
• Lab data: 1 ESR; I hematocrit; ± azotemia
Demographics
• Age: Usually above 40 years
• Gender: M:F = 2:1
Natural History & Prognosis
• Complications
6 Extrinsic ureteral obstruction ~ renal failure
o Great vessels (aorta & IVe) obstruction
• Prognosis
o Good; may require surgery
Treatment
• Withdrawal of possible causative agent
• Corticosteroids; ureteral stent; ureterolysis
I DIAGNOSTIC CHECKLIST
Consider
• Check for underlying malignancy, AAA
Image Interpretation Pearls
• Mantle or rind of soft tissue density encircling great
vessels & ureters in retroperitoneum
I SELECTED REFERENCES
1. Fukukura Y et al: Autoimmune pancreatitis associated with
idiopathic retroperitoneal fibrosis. AJRAm J Roentgenol.
181(4):993-5, 2003
2. Hamano H et al: Hydronephrosis associated with
retroperitoneal fibrosis and sclerosing pancreatitis. Lancet.
359(9315):1403-4,2002
3. Nishimura H et al: MR imaging of soft-tissue masses of the
extraperitoneal spaces. Radiographies. 21(5):1141-54, 2001
4. Sung MS et al: Myxoid liposarcoma: appearance at MR
imaging with histologie correlation. Radiographies.
20(4):1007-19,2000
5. Vivas I et al: Retroperitoneal fibrosis: typical and atypical
manifestations. Br J Radiol. 73(866):214-22,2000
6. Rominger MB et al: Perirenal involvement by
retroperitoneal fibrosis: the usefulness of MRI to establish
diagnosis. Urol Radiol. 13(3):173-6, 1992
7. Amis Jr ES: Retroperitoneal fibrosis. AJR 157: 321-9, 1991
8. Arrive L et al: Malignant versus nonmalignant
retroperitoneal fibrosis: differentiation with MR imaging.
Radiology. 172(1):139-43, 1989
' 9. Mulligan SA et al: CT and MR imaging in the evaluation of
retroperitoneal fibrosis. J Com put Assist Tomogr.
13(2):277-81, 1989
10. Arger PH et al: Retroperitoneal fibrosis: An analysis of the
clinical spectrum and roentgenographic signs. AJR 119:
812, 1973
 RETROPERITONEAL FIBROSIS

I IMAGE GALLERY 1
13
Typical
(Left) Axial CECT shows
mantle of soft tissue around
aorta. Stent (arrow) in left
ureter to bypass obstruction.
(Right) Axial CECT shows
mantle of soft tissue
surrounding the aortic
bifurcation and left ureteral
stent (arrow).

(Left) Coronal T2WI MR

shows mantle of soft tissue
(arrows) surrounding aorta
and We. (Right) Coronal
T2WI MR shows
hydronephrosis, worse on
left.

Variant
(Left) Axial CECT shows
unusually massive
retroperitoneal fibrosis in
September, 2003.
Obstructed left kidney.
(Right) Axial CECT in
November, 2003, shows
slight decrease in fibrotic
mass following steroid
therapy. and relief of
obstruction by stent (arrow).
 PELVIC LIPOMATOSIS
1
14

Air-contrast barium enema (BE) shows smooth, long Air-contrast BE shows extrinsic compression of rectum
stricture of rectum due to pelvic lipomatosis. by pelvic lipomatosis.

ITERMINOLOGY CT Findings
• Nonencapsulated fatty mass surrounding pelvic organs
Abbreviations and Synonyms
symmetrically
• Pelvic lipomatosis (PL) • Fat density attenuation (-80 to -120 HU); may contain
Definitions strands with higher attenuation than fat
• Nonmalignant overgrowth of nonencapsulated fatty MR Findings
tissue in perirectal and perivesical spaces of pelvis • Cephalad displacement of bladder base
• Elevation of prostate gland
• Elongation of bladder neck and posterior urethra
I IMAGING FINDINGS • Medial and superior displacement of seminal vesicles
General Features • Medial or lateral displacement of ureters
• t Distance between prostate gland & rectum by fat
• Best diagnostic clue: Classic triad 1) pelvic
radiolucency, 2) elevation of an intact rectosigmoid Ultrasonographic Findings
and 3) elevation of urinary bladder with symmetric • Real Time
inverted pear shape o Tubular or cigar shaped urinary bladder compressed
by extensive echogenic perivesical tissue
Radiographic Findings
o Bladder "floats" in pelvic fat and unable to distend
• Radiography normally to the pelvic side walls
o t Radiolucency of the perivesical area
o High positioned, pear-shaped or inverted teardrop Imaging Recommendations
bladder • Best imaging tool: CT: Definitive diagnosis
o Tubular narrowing of rectosigmoid colon
• IVP: Dilated, tortuous and medially displaced ureters
• Fluoroscopic-guided barium enema I DIFFERENTIAL DIAGNOSIS
o "Lower rectum": Elongation & symmetrical extrinsic
compression of rectum and cephalad displacement Proctitis
of sigmoid colon • E.g., radiation, lymphogranuloma venereum

DDx: Narrowed Rectum +/or Urinary Bladder

Proctitis Narrow Pelvis Lymphadenopathy

 PELVIC LIPOMATOSIS

Key Facts
1
Imaging Findings • Ulcerative colitis 15
• Best diagnostic clue: Classic triad 1) pelvic • Post operation
radiolucency, 2) elevation of an intact rectosigmoid Pathology
and 3) elevation of urinary bladder with symmetric • General path comments: Nonmalignant condition
inverted pear shape
Diagnostic Checklist
Top Differential Diagnoses
• Close follow-up to monitor ureteral obstruction
• Proctitis • Use CT to differentiate fatty infiltration from other
• Normal variant conditions

Normal variant Treatment

• Large pelvic muscles with narrow bony pelvis • Urinary diversion to relieve obstruction
• Pear-shaped bladder caused by large iliopsoas muscles • Role of surgical resection is unclear
Ulcerative colitis
• Pancolitis with decreased haustration & multiple I DIAGNOSTIC CHECKLIST
ulcerations
• Diffuse & symmetric wall thickening of colon Consider
• Chronic phase ~ lead pipe colon • Close follow-up to monitor ureteral obstruction
Post operation Image Interpretation Pearls
• E.g., proctosigmoid resection/re-anastomosis • Use CT to differentiate fatty infiltration from other
conditions

I PATHOLOGY
General Features
I SELECTED REFERENCES
• General path comments: Nonmalignant condition 1. Heyns CF: Pelvic lipomatosis: a review of its diagnosis and
management. J Urol. 146(2):267-73, 1991
• Etiology: Unknown 2. Demas BE et al: Pelvic lipomatosis: diagnosis and
• Epidemiology: 0.6-1.7 per 100,000 hospital admissions characterization by magnetic resonance imaging. Urol
• Associated abnormalities: Cystitis glandularis Radiol. 10(4):198-202, 1988
3. Klein FA et al: Pelvic lipomatosis: 35-year experience. J
Gross Pathologic & Surgical Features Urol. 139(5):998-1001, 1988
• Abnormal deposition of mature adipose tissue 4. Clark WM et al: Ultrasonographic features of pelvic
lipomatosis. Urol Radiol. 1(3):183-6, 1980
Microscopic Features 5. Chang SF: Pear-shaped bladder caused by large iliopsoas
• Fat overgrowth without fibrosis or inflammation muscles. Radiology. 128(2):349-50, 1978
6. Ambos MA et al: The pear-shaped bladder. Radiology.
122(1):85-8, 1977
I CLINICAL ISSUES 7. Moss AA et al: Pelvic lipomatosis: a roentgenographic
diagnosis. Am J Roentgenol Radium Ther Nucl Med.
Presentation 115(2):411-9, 1972
• Most common signs/symptoms
o Asymptomatic
o Compressed urinary system (Le., 1 frequency, I IMAGE GALLERY
dysuria, nocturia, and hematuria)
o Compressed intestinal tract (Le., constipation, rectal
bleeding, tenesmus, ribbon-like stools with mucus)
o Compressed vasculature (Le., edema of lower
extremities)
Demographics
• Age: 9-80 years old, peak 25-60 years old
• Gender: M:F = 10:1
• Ethnicity: African-Americans
Natural History & Prognosis
• Complications: Hydroureteronephrosis, renal failure,
ureteral obstruction, obstruction of inferior vena cava (Left)Axial NECT shows markedly narrowed recto-sigmoid colon
or pelvic veins (arrow) + displacement of bladder (open arrow). (Right) Axial NECT
shows displacement + narrowing of colon and bladder by pelvic
lipomatosis.
 RETROPERITONEAL HEMORRHAGE
1
16

Axial CECT shows spontaneous hemorrhage into Axial NECT shows multifocal collections in the right
multiple sites in the retroperitoneum and body wall in retroperitoneum and body wall, plus a fluid-fluid level
an anticoagulatedpatient. ("hematocrit effect") (arrow) in an anticoagulated
patient.

• ± Hematoma within perihepatic, perisplenic, perirenal,

ITERMINOlOGY pararenal and/or pelvic spaces
Abbreviations and Synonyms • ± Mass effect: Displacement of spleen anteriorly,
• Hemoretroperitoneum kidney anteriorly and/or midline
• ± Extension of hematoma: Superiorly to diaphragm,
Definitions inferiorly to pelvis
• Bleeding in the retroperitoneum or posterior o Extensive swelling of psoas & iliac muscle
abdominal wall muscles • Signs of bleeding from coagulopathy or
anticoagulation
o Hematocrit effect
I IMAGING FINDINGS • Fluid-fluid level within "mass"
o Bleeding into several anatomic spaces (e.g.,
General Features retroperitoneum, body wall muscles)
• Best diagnostic clue: High density collection in o Bleeding out of proportion to injury
retroperitoneal space with fluid-fluid level • Associated abdominal aortic aneurysm
• Location: Localized to area of bleeding; blood vessel or o NECT
specific organ • Located infrarenal; extension to iliac arteries
• Other general features • Thickened diaphragmatic crura
o Associated findings depend on etiology • "Enlarged" left psoas muscle
• Aortic outline is obscured by soft tissue density
CT Findings o CECT
• Active bleeding • Saccular or fusiform dilatation of aorta
o Linear or flame-like appearance isodense to • Indistinct focal area of aortic wall
enhanced vessels • Blood tracks along perirenal fascia ~ can follow
o Extravasation of vascular contrast (80-370 HU) renal hilum into perirenal space; simulates renal
• Acute (60-80 HU): High attenuating fluid collection or source of bleeding
hematoma • Anterior displacement of kidney
• Chronic (20-40 HU): Low density (organized clot) • Extravasation of contrast
• Mixed-density mass (acute and chronic)

DDx: Retroperitoneal Mass or Fluid

Perforated Duod. Ulcer Testicular Ca Mets Liposarcoma Retroperit. Fibrosis

 RETROPERITONEAL HEMORRHAGE
Key Facts
1
Terminology Pathology 17
• Bleeding in the retroperitoneum or posterior • Anticoagulation (most common): Warfarin,
abdominal wall muscles low-molecular weight heparin
• Abdominal aortic aneurysm (2nd most common)
Imaging Findings • Spontaneous tumor rupture (3rd most common)
• Best diagnostic clue: High density collection in
retroperitoneal space with fluid-fluid level Clinical Issues
• ± Hematoma within perihepatic, perisplenic, • Abdominal, back or flank pain; pain radiating to
perirenal, pararenal and/or pelvic spaces groin and anteromedial thigh
• ± Mass effect: Displacement of spleen anteriorly,
kidney anteriorly and/or midline Diagnostic Checklist
• Most common cause is iatrogenic; discuss with
Top Differential Diagnoses referring physician
• Retroperitoneal abscess • Hematocrit effect and bleeding into several spaces
• Retroperitoneal tumor indicates anticoagulation
• Asymmetrical muscles • Spontaneous perirenal hemorrhage: Look for
underlying tumor

• Associated renal or adrenal tumors • Etiology

o Spherical heterogeneous mass replacing part of o Affected adjacent segment of bowel
kidney • Example: Retrocecal appendicitis
o Renal cell carcinoma o Penetrating trauma
• Most common neoplastic cause of spontaneous o Inflammatory bowel disease
hemorrhage o Perforated duodenal ulcer
o Angiomyolipoma o Perforated colonic carcinoma
• Contains visible fat in most cases • Radiography
• May be obscured by hemorrhage or distorted o Mottled gas collection
anatomy o Increased density at area of abscess
o Adrenal carcinoma, pheochromocytoma or o Obliteration of psoas outline
metastases o Obliteration of renal outline and fat within the
• Distorted anatomy due to bleeding may obscure perirenal space (perirenal abscess)
tumor o Renal"halo" sign: Fluid in pararenal space and
• Trauma outside the renal fascia outlines perirenal fat
o Significant retroperitoneal bleeding usually due to • Fluoroscopic-guided barium studies
renal traumatic injury o Displacement of organs due to abscess (e.g., medial
displacement of kidney due to perirenal abscess)
MR Findings
o ± Obstruction (distal colon)
• Varied signal intensity (evolution of blood products)
• CT
• Hyperacute phase (due to oxyhemoglobin) o Gas and fluid within enclosed space
o T1WI: Slightly hypointense o ± Extravasation of enteric contrast
o T2WI: Hyperintense
• Acute phase (due to deoxyhemoglobin) Retroperitoneal tumor
o Tl WI: Isointense or slightly hypointense • Example: Retroperitoneal sarcoma
o T2WI: Markedly hypointense oCT
• Chronic phase • Usually located peri-/pararenal region; > 10 cm
o Tl WI: Hypointense • Large heterogeneous mass of fat and soft tissue
o T2WI: Hyperintense attenuation displacing retroperitoneal structures
or viscera
Imaging Recommendations
• Poorly or sharply marginated and encapsulated
• Best imaging tool: Helical CT mass; ± calcification
• Protocol advice • Displacement, compression, distortion of adjacent
o Helical CT
structures (kidneys, bowel, colon)
• Injection rate:::: 3 ml per second; 70 seconds delay • Liver metastases (necrotic or cystic)
• Diaphragm to symphysis using 5 mm collimation o MRI: Variable signal intensities depending on fat,
solid, cystic, necrotic, hemorrhagic components
I DIFFERENTIAL DIAGNOSIS Asymmetrical muscles
• Examples: Polio or amputation
Retroperitoneal abscess • CT: Asymmetrical, enlarged iliopsoas muscle ~
• Less likely to extend far from site of origin versus indentation or compression of bladder dome
peritoneal abscesses (asymmetrical pear-shaped bladder)
 RETROPERITONEAL HEMORRHAGE
1 I PATHOLOGY I DIAGNOSTIC CHECKLIST
18 General Features Consider
• Etiology • Most common cause is iatrogenic; discuss with
o Iatrogenic referring physician
• Anticoagulation (most common): Warfarin,
low-molecular weight heparin Image Interpretation Pearls
• Femoral vein cannulization or arteriography • Hematocrit effect and bleeding into several spaces
• Percutaneous nephrostomy or renal biopsy indicates anticoagulation
• Translumbar aortography • Spontaneous perirenal hemorrhage: Look for
o Arterial aneurysms underlying tumor
• Abdominal aortic aneurysm (2nd most common)
• Renal artery aneurysm
• Inferior adrenal artery aneurysm I SELECTED REFERENCES
• Lumbar artery aneurysm 1. Harris AC et al: Ct findings in blunt renal trauma.
• Iliac artery aneurysm Radiographies. 21 Spec No:S201-14, 2001
• Ovarian artery aneurysm 2. Lindner A et al: Images in clinical medicine.
Retroperitoneal hemorrhage. N Engl] Med. 344(5):348,
• Uterine artery aneurysm
2001
• Inferior pancreaticoduodenal artery aneurysm
3. Amano T et al: Retroperitoneal hemorrhage due to
o Spontaneous tumor rupture (3rd most common) spontaneous rupture of adrenal myelolipoma. Int] Urol.
• Renal cell carcinoma 6(11):585-8, 1999
• Angiomyolipoma 4. Patten RM et al: Traumatic laceration of the liver limited to
• Adrenal carcinoma the bare area: CT findings in 25 patients. A]R Am]
• Adrenal myelolipoma Roentgenol. 160(5):1019-22, 1993
• Adrenal pheochromocytoma 5. Trerotola SO et al: Bleeding complications of femoral
catheterization: CT evaluation. Radiology. 174(1):37-40,
• Renal or adrenal cyst
1990
o Trauma Wilms G et al: Embolization of iatrogenic pelvic and
6.
• Abdominal wall, renal or adrenal trauma retroperitoneal hemorrhage.] BeIge Radiol. 72(4):279-82,
• Trauma to postero-superior region of segment VII 1989
(liver bare area) 7. Sclafani S] et al: Lumbar arterial injury: radiologic diagnosis
o Other disease and management. Radiology. 165(3):709-14, 1987
• Pancreatitis 8. Weinbaum PI et al: The accuracy of computed tomography
in the diagnosis of retroperitoneal blood in the presence of
Gross Pathologic & Surgical Features abdominal aortic aneurysm.] Vase Surg. 6(1):11-6, 1987
• Contusion, laceration and/or ischemia of organ 9. Ralls PW et al: Renal biopsy-related hemorrhage: frequency
and comparison of CT and sonography. ] Com put Assist
Microscopic Features Tomogr. 11(6):1031-4, 1987
• Contusion, laceration and/or ischemia of tissue 10. Illescas FF et al: CT evaluation of retroperitoneal
hemorrhage associated with femoral arteriography. A]RAm
] Roentgenol. 146(6):1289-92, 1986
11. Cronan JJ et al: Retroperitoneal hemorrhage after
I CLINICAL ISSUES percutaneous nephrostomy. A]R Am] Roentgenol.
144(4):801-3, 1985
Presentation 12. Rosen A et al: CT diagnosis of ruptured abdominal aortic
• Most common signs/symptoms aneurysm. A]R Am] Roentgenol. 143(2):265-8, 1984
o Abdominal, back or flank pain; pain radiating to 13. Federle MP: Computed tomography of blunt abdominal
groin and anteromedial thigh trauma. Radiol Clin North Am. 21(3):461-75, 1983
o Hypotension, nausea, vomiting 14. Samuels son L et al: Ruptured aneurysm of the internal iliac
artery.] Comput Assist Tomogr. 6(4):842-4, 1982
o Abdominal distention, peritoneal signs
15. Jeffrey RB et al: Computed tomography of splenic trauma.
o Weakness and I sensation of lower extremities Radiology. 141(3):729-32, 1981
16. Amendola MA et al: Evaluation of retroperitoneal
Demographics hemorrhage by computed tomography before and after
• Age: Any age, increase likelihood with age translumbar aortography. Radiology. 133(2):401-4, 1979
• Gender: M > F 17. Cisternino S] et al: Diagnosis of retroperitoneal
hemorrhage by serial computed tomography. ] Comput
Natural History & Prognosis Assist Tomogr. 3(5):686-8, 1979
• Complications: Paresis, shock and death 18. Sagel SSet al: Detection of retroperitoneal hemorrhage by
• Prognosis: Poor, but dependent on cause computed tomography. A]R Am] Roentgenol.
129(3):403-7, 1977
Treatment 19. Stephens DH et al: Computed tomography of the
• Stop medication ± protamine retroperitoneal space. Radiol Clin North Am. 15(3):377-90,
• Infusion of blood products (Le., fresh frozen plasma, 1977
packed red blood cells) 20. Einarsson GV: Spontaneous retroperitoneal hemorrhage as
• Transcatheter or angiographic embolization for active first presentation of carcinoma of the kidney. A case report.
Scand] Urol Nephrol. 20(3):235-6, 1986
bleeding
• Surgical repair and resection (Le., tumor, organ injury)
 RETROPERITONEAL HEMORRHAGE

[IMAGE GALLERY 1
19
Typical
(Left) Axial CECT shows peri-
and pararenal hemorrhage
following renal biopsy.
(Right) Axial CECT shows a
large rectus sheath
hematoma with foci of active
bleeding (arrow) in an
anticoagulated patient.

Typical
(Left) Axial CECT shows a
ruptured abdominal aortic
aneurysm (AAA) with blood
tracking throughout all three
retroperitoneal
compartments. (Right) Axial
CECT shows a ruptured AAA
with blood tracking
throughout the right
retroperitoneal spaces.

Typical
(Left) Axial CECT shows
perirenal hemorrhage that
occurred spontaneously from
underlying angiomyolipoma
(arrows). (Right) Axial CECT
shows peri- and pararenal
hemorrhage following blunt
abdominal trauma. Note
active arterial bleeding
(arrow).
 RETROPERITONEAL SARCOMA
1
20

Axial CECT shows predominantly fatty mass that Axial CECT shows liposarcoma with heterogeneous
displaces bowel, including descending colon (arrow); areas whose attenuation is similar to fat or soft tissue
liposarcoma. (arrows), probably representing myxoid stroma.

• Most common primary retroperitoneal malignant

ITERMINOLOGY tumor
Definitions • 2nd Most common adult soft tissue sarcoma after
• Malignant primary retroperitoneal tumor arising from malignant fibrous histiocytoma
various elements of primitive mesenchyme, urogenital • 15% of all soft tissue tumors, 10-20% of them in
ridge, embryonic remnants retroperitoneum
• Malignant from inception; rarely arises from
lipomas
I IMAGING FINDINGS • Usually grow slowly & attain a large size before
they are detected
General Features • Histologically: 5 types (well-differentiated, myxoid
• Best diagnostic clue: Large heterogeneous mass of fat + 40-50% (most common), round cell, pleomorphic,
soft tissue attenuation displacing retroperitoneal mixed)
structures or viscera o Leiomyosarcoma: Smooth muscle tumor (vessels &
• Location: Usually peri-/paranephric region embryonic remnants)
• Size: Usually large at diagnosis(> 10 cm) • 2nd Most common primary retroperitoneal tumor
• Key concepts after liposarcoma
o 80% of primary retroperitoneal tumors: Malignant • Accounts for 11% of all retroperitoneal malignant
• Lymphomas (most common); retroperitoneal tumors
sarcomas • Leiomyosarcomas are more common than benign
o Most malignant retroperitoneal tumors are of leiomyomas
mesodermal origin o Fibrosarcoma & malignant fibrous histiocytoma:
o Classified into four types based on tissue of origin Connective tissue tumors
• Liposarcoma • Fibrosarcoma: 3% of all retroperitoneal malignant
• Leiomyosarcoma tumors
• Fibrosarcoma • Malignant fibrous histiocytoma: Most common
• Rhabdomyosarcoma soft tissue sarcoma in adults, 15% of these arise in
o Liposarcoma: Adipose tissue tumor abdominal cavity & retroperitoneum

DDx: Large Mass in the Retroperitoneum

.... _,~

" ··r·
... ~
..
Renal AML Adrenal Myeolipoma Retroperineal Bleed
•y
Retroperitoneal
'-

Bleed
 RETROPERITONEAL SARCOMA
Key Facts
1
Terminology Top Differential Diagnoses 21
• Malignant primary retroperitoneal tumor arising • Renal angiomyolipoma (AML)
from various elements of primitive mesenchyme, • Retroperitoneal hemorrhage
urogenital ridge, embryonic remnants
Pathology
Imaging Findings • Mesodermal origin: Most adult malignant
• Best diagnostic clue: Large heterogeneous mass of fat retroperitoneal tumors
+ soft tissue attenuation displacing retroperitoneal • Large, encapsulated mass
structures or viscera
• Location: Usually peri-/paranephric region
Clinical Issues
• Size: Usually large at diagnosis (> 10 cm) • Abdominal, back, flank, radicular pain
• Poorly or sharply marginated + encapsulated mass ± • Palpable maSSi GI & urinary tract symptoms
calcification . Diagnostic Checklist
• Displacement, compression, distortion of adjacent
• Differentiate from adrenal & renal tumors
structures (kidneys, bowel, colon)
• Large mixed attenuation mass of fat & soft tissue
• Liver metastases (necrotic or cystic)
density displacing retroperitoneal structures

o Rhabdomyosarcoma: Striated muscle tumor • Solid & necrotic extraluminal tumor

• Embryonal rhabdomyosarcomas: 60% of striated • Enhancing intravascular component
muscle tumors o Intramural tumor: Extremely rare
o Angiosarcoma: Malignant tumor of vascular
endothelium (rare) MR Findings
o Lymphangiosarcoma (lymph vessels) & • Variable signal intensities depending on amount of
myxosarcoma (mesenchyme) fat, solid, cystic, necrotic, hemorrhagic components

Radiographic Findings Ultrasonographic Findings

• Fluoroscopic guided barium study • Real Time
o Show displacement of stomach, small bowel, colon o Liposarcoma: Large well-defined solid mass with
• Excretory urography findings internal echoes (fat)
o Demonstrate displacement of kidney, ureter, bladder o Leiomyosarcoma: Large solid mass + hypoechoic
± hydronephrosis cystic & necrotic content
o Usually medial displacement of ureters (most tumors Angiographic Findings
arise laterally)
• Conventional
CT Findings o Show hypo-/hypervascularity; displacement of great
• Liposarcoma: 3 CT patterns based on amount & vessels
distribution of fat in tumor (often coexist) o Liposarcoma: Hypovascular tumor
o Solid pattern: Attenuation values> +20 HU o Leiomyosarcoma: Hypervascular + feeding vessels
o Mixed pattern: Discrete fatty areas with HU < -20 & • Inferior venacavography
areas> +20 HU o For primary or secondary involvement of IVC
o Pseudocystic pattern: Homogeneous density Imaging Recommendations
between +20 & -20 HU
• NE + CECT; MR; angiography
o Poorly or sharply marginated + encapsulated mass ±
calcification
o Displacement, compression, distortion of adjacent
structures (kidneys, bowel, colon) I DIFFERENTIAL DIAGNOSIS
o ± Invasion of adjacent structures Renal angiomyolipoma (AMl)
o CECT: Hetero-/homogeneous enhancementi usually • Composed of blood vessels, muscle & fat tissue
lack prominent vessels
• Hamartomatous lesion (indicates a benign tumor)
• Leiomyosarcoma • 20% of renal angiomyolipoma patients have tuberous
o Extravascular (62%) sclerosis
• Large retroperitoneal mass ± necrotic or cystic • 80% of tuberous sclerosis patients have renal
degeneration angiomyolipomas
• Liver metastases (necrotic or cystic) • Large angiomyolipoma may simulate retroperitoneal
o Intravascular mass (6%) liposarcoma (both contain fat)
• Solid mass within IVC + dilatation or obstruction o Renal parenchymal defect & enlarged vessels favor
• Proximal IVC mass: Show dilated hepatic veins + angiomyolipoma (AML)
portal radicles
o Smooth compression of kidney & extension beyond
• CECT: Heterogeneous enhancement perirenal space favor liposarcoma
o Extra + intravascular mass (33%)
 RETROPERITONEAL SARCOMA
1 Retroperitoneal hemorrhage Natural History & Prognosis
22 • Most are iatrogenic (over anticoagulation) • Liposarcoma
• Abdominal aortic aneurysm rupture is 2nd most o 5 year survival rate of 32%
common cause of retroperitoneal bleed • Leiomyosarcoma
• Spontaneous tumoral bleed: 3rd most common cause o High mortality within 5 years
o Kidney: Renal cell carcinoma, angiomyolipoma o Local recurrence: 40-70% cases
o Adrenal: Carcinoma or myelolipoma
• CT findings
Treatment
o Acute: High density fluid collection or hematoma • Complete resection, radiotherapy, chemotherapy
o Chronic: Low density (organized clot) • Follow-up imaging & excision of local recurrence
o Associated renal, adrenal tumors or aortic aneurysm
• MR findings
o Varied signal intensity (evolution of blood products) I DIAGNOSTIC CHECKLIST
o Hyperacute phase: Due to oxyhemoglobin
Consider
• Tl WI: Slightly hypointense
• T2WI: Hyperintense • Differentiate from adrenal & renal tumors
o Acute phase: Due to deoxyhemoglobin Image Interpretation Pearls
• Tl WI: Isointense or slightly hypointense • Large mixed attenuation mass of fat & soft tissue
• T2WI: Markedly hypointense density displacing retroperitoneal structures
o Chronic phase: !Signal (Tl WI); t signal (T2WI)
• Diagnosis
o History of anticoagulation
o Imaging evidence of tumor or aneurysm
I SELECTED REFERENCES
1. Nishino M et al: Primary retroperitoneal neoplasms: CT
and MR imaging findings with anatomic and pathologic
diagnostic clues. Radiographies. 23(1):45-57, 2003
I PATHOLOGY 2. Tateishi U et al: Primary dedifferentiated liposarcoma of
the retroperitoneum. Prognostic significance of computed
General Features tomography and magnetic resonance imaging features. ]
• General path comments Comput Assist Tomogr. 27(5):799-804, 2003
o Embryology/anatomy 3. Bellin MF et al: Evaluation of retroperitoneal and pelvic
• Mesodermal origin: Most adult malignant lymph node metastases with MRI and MR
retroperitoneal tumors lymphangiography. Abdom Imaging. 28(2):155-63, 2003
4. Israel GM et al: CT differentiation of large exophytic renal
• Etiology: Unknown
angiomyolipomas and perirenal liposarcomas. AJR. 179:
• Epidemiology: 1 in 11,800 admissions 769-73,2002
Gross Pathologic & Surgical Features 5. Grubnic S et al: MR evaluation of normal retroperitoneal
and pelvic lymph nodes. Clin Radiol. 57(3):193-200;
• Liposarcoma discussion 201-4, 2002
o Large, encapsulated mass 6. Gupta AK et al: CT of recurrent retroperitoneal sarcomas.
o White or yellow glistening, brain-like A]R Am] Roentgenol. 174(4):1025-30, 2000
• Leiomyosarcoma 7. Heslin M] et al: Imaging of soft tissue sarcomas. Surg Oncol
o Lobulated, encapsulated tumor Clin N Am. 8(1):91-107, 1999
o Cystic, necrotic, blood & Ca++ components 8. Kurosaki Y et al: Well-differentiated liposarcoma of the
retroperitoneum with a fat-fluid level: US, CT, and MR
Microscopic Features appearance. Eur Radiol. 8(3):474-5, 1998
• Liposarcoma 9. Radin R et al: Adrenal and extra-adrenal retroperitoneal
o Myxoid (mucinous + fibrous tissue + fat < 10%)
ganglioneuroma: imaging findings in 13 adults. Radiology.
202(3):703-7, 1997
• Leiomyosarcoma 10. Engelken]D et al: Retroperitoneal MR imaging. Magn
o Smooth muscle + atypical giant cells Reson Imaging Clin N Am. 5(1):165-78, 1997
o Invasion of vessels 11. Kim T et al: CT and MR imaging of abdominal
liposarcoma. A]R. 166: 829-33, 1996
12. Mesurolle B et al: Retroperitoneal extramedullary
I CLINICAL ISSUES hematopoiesis: sonographic, CT, and MR imaging
appearance. A]R Am] Roentgenol. 167(5):1139-40, 1996
Presentation 13. Kim T et al: CT and MR imaging of abdominal
liposarcoma. A]R Am] Roentgenol. 166(4):829-33, 1996
• Most common signs/symptoms
14. Miyazaki T et al: Retroperitoneal leiomyosarcoma. Its MR
o Abdominal, back, flank, radicular pain manifestations. Clin Imaging. 17(3):207-9, 1993
o Palpable mass; GI & urinary tract symptoms 15. Lane RH et al: Primary retroperitoneal neoplasms: CT
o Leg edema, varicosities, hypoglycemia findings in 90 cases with clinical & pathologic correlation.
A]R. 152: 83-9, 1989
Demographics
• Age: 40-60 y
• Gender
o Liposarcoma: M > F
o Leiomyosarcoma: M < F
 RETROPERITONEAL SARCOMA

I IMAGE GALLERY 1
23
Typical
(Left) Axial CECT shows a
low density mass that
displaces right kidney,
pancreas, ete.
Leiomyosarcoma. (Right)
Axial CECT shows
leiomyosarcoma that invades
or displaces right psoas,
aorta, and other structures.

(Left) Radiograph shows

massive displacement of
kidneys and ureters, marked
by ureteral stents (arrows);
liposarcoma in 30 year-old
man. (Right) Axial CECT in a
30 year-old man with
liposarcoma shows
displacement of all pelvic
(and abdomina/) contents,
including left iliac vessels
(arrow).

(Left) Axial CECT shows a

primary sarcoma of the IVC
(arrow). Note collateral
vessels. (Right) Axial CECT
shows liposarcoma arising in
the mesentery, which
bridges the intraperitoneal
and retroperitoneal spaces.
 RETROPERITONEAL LYMPHOMA
1
24

Axial CECT with splenic and nodal involvement from Axial CECT of retroperitoneal nodes from Hodgkin
Hodgkin disease. Note multiple low-density lesions lymphoma. Note enlarged para-aortic and aortocaval
(arrow) with celiac (open arrow) and retroperitoneal nodes (arrows).
(curved arrow) involvement.

o Enlarged nodes (> 1.5 cm in short axis) involving

ITERMINOLOGY bilateral retroperitoneal nodal chains
Definitions o Confluent soft tissue mantle of nodes surrounding
aorta and IVC
• Hodgkin (HD) and non-Hodgkin (NHL) lymphoma
o Nodes may displace aorta from spine, unusual for
involving retroperitoneal nodes
other types of nodal mets
o Attenuation of nodes similar to muscle
o In untreated patients nodes are rarely calcified «
IIMAGING FINDINGS 1%) or cystic
General Features o Nodes involved with NHL typically greater in size
• Best diagnostic clue: Mantle of soft tissue adenopathy than HD and demonstrate skip areas
surrounding aorta, inferior vena cava (lVe) o 25% of newly-diagnosed HD patients have positive
• Location: Para-aortic, aortocaval and retrocaval nodal para-aortic nodes at presentation, compared to 50%
groups with NHL
• Size: Nodes> 1.5 cm in short axis o HD nodes typically involve upper para-aortic nodes
• Morphology: Confluent soft tissue mantle of first without skip areas of involvement
adenopathy surrounding aorta and IVC o HD nodes may be normal in size but contain
microscopic tumor leading to false~negative CT
Radiographic Findings o Involvement of peripancreatic nodes from NHL
• Radiography results in large peri pancreatic mass (pancreatic
o Lymphangiography (LAG) lymphoma)
• Used for HD to detect involvement in o Involvement of mesenteric nodes more commmon
normal-sized nodes with NHL (> 50%) than HD « 5%)
• Positive nodes have "foamy appearance" or focal
filling defects
MR Findings
• Tl WI: Low signal para-aortic lymphadenopathy
CT Findings • T2WI: High signal para-aortic lymphadenopathy
• CECT • Tl C+: Nodes enhance similar to muscle without
necrosis

DDx: Retroperitoneal Disorders Mimicking lymphoma

Carcinoma Testicular CA Liposarcoma Neurogenic mass

 RETROPERITONEAL LYMPHOMA

Key Facts
1
Terminology • Neurogenic mass 25
• Hodgkin (HD) and non-Hodgkin (NHL) lymphoma • Tuberculosis (TB)
involving retroperitoneal nodes • Retroperitoneal fibrosis

Imaging Findings Clinical Issues

• Best diagnostic clue: Mantle of soft tissue adenopathy
surrounding aorta, inferior vena cava (IVe)
• Location: Para-aortic, aortocaval and retrocaval nodal
groups
• Best imaging tool: PET-CTwith FDG; CECT
• Protocol advice: Combining PET with CT reduces
false-positive and false-negative rates
Top Differential Diagnoses
• Carcinoma
• Sarcoma
• Testicular metastases
• Nodal mass; fever; night sweats; weight loss
• Clinical profile: Young patient with palpable nodal
mass
• Age: HDbimodal; 2 peak incidences: 20-24, 80-84
• HD: 80% cure with chemotherapy and/or radiation,
or stem cell transplant
• NHL: Depends on grade and stage; in general, worse
than HD, worst in AIDS-related NHL; poor prognosis
if patients don't respond to initial therapy
Diagnostic Checklist
• Metastatic nodes from carcinoma

• Low attenuation myxomatous element

Ultrasonographic Findings
• Real Time: Hypoechoic nodes without enhanced
through sound transmission
Nuclear Medicine Findings
• PET
o Hypermetabolic nodes with FDG
o Sensitivity 90-95%, compared to 80-85% for CECT
o More sensitive for thoracic lymphoma (> 90%) than
abdominal or pelvic disease (> 75%)
o Detects more lesions than CT, may result in
changing stage of patients in 10-40% of cases
o Modifies therapy in 25% of cases
o Useful to determine early response to chemotherapy
o Helpful to distinguish residual lymphoma vs.
fibrosis post-radiation
o Not useful for B-celllymphoma or for
mucosa-associated lymphoid tissue (MALT)
Imaging Recommendations
• Best imaging tool: PET-CTwith FDG; CECT
• Protocol advice: Combining PET with CT reduces
false-positive and false-negative rates
I DIFFERENTIAL DIAGNOSIS
Carcinoma
• Enlarged nodes that are discrete but not "confluent"
adenopathy, or "mantle" appearance of soft tissue
retroperitoneal mass
• Calcification for mucinous adenocarcinoma or serous
carcinoma of the ovary
Sarcoma
• Typically unilateral large retroperitoneal mass
• Displaces organs such as colon or kidney
• Often heterogeneous enhancement on CECT
• Fat attenuation in liposarcoma
Testicular metastases
• Nodes enlarged along gonadal vein as it drains into
IVC; left renal hilus and retrocaval on right
Neurogenic mass
• Paraganglioma, ganglioneuroma typically paraspinous
in location
• Widening of neural foramen
• Heterogeneous enhancement on CECT and MRI
Tuberculosis (TB)
• Low attenuation nodes
• Associated TB peritonitis includes mesenteric or
omental nodules, ascites & ileo-colic mural thickening
Retroperitoneal fibrosis
• Usually limited to mantle of tissue surrounding aorta
and IVC from mid-lumbar level through common iliac
Miscellaneous
• Kaposi sarcoma: Hypervascular retroperitoneal nodes
in AIDS patient
• Castleman disease (angiofollicular lymphoid
hyperplasia): Hypervascular retroperitoneal nodes
• Growing teratoma syndrome: Water- or fat-density
nodal mass due to development of mature teratoma
following chemotherapy for testicular
nonseminomatous tumor
I PATHOLOGY
General Features
• General path comments: Rubbery enlarged nodes
without necrosis or calcification
• Genetics: Several types of NHL associated with
oncogenes bcll, 2, 3, and 6
• Etiology: Unknown
• Epidemiology
o 5% of newly-diagnosed cancer in USA
• HD: 8,000 cases/year in USA
• NHL: 50,000 cases/year in USA
o Incidence has increased due to AIDS-related NHL
and organ transplantation
o Third most common cancer death
 RETROPERITONEAL
1 Gross Pathologic & Surgical Features
26 • Excisional biopsy or core biopsy preferred for HD
subtyping
• Fine needle aspiration (FNA) adequate for recurrent or
NH lymphoma
Microscopic Features
• HD
o Few malignant cells within background of normal
T-lymphocytes and inflammatory cells
o Malignant cells: Reed-Sternberg cells and variants
• Lacunar cells, mononuclear and pleomorphic
variants
o Distorted nodal architecture
o Histologic subtypes: Nodular sclerosing, lymphocyte
predominant, mixed cellularity, lymphocyte
depletic
• NHL
o Classification currently based on World Health
Organization criteria
o Cell type origin determines subtype (B-cell, T-cell or
natural killer cell) as well as immune phenotype
Staging, Grading or Classification Criteria
• Ann Arbor staging classification
o Stage I: Single lymph node region/extralymphatic
site
o Stage II: Two or more nodal regions on same side of
diaphragm (including single extralymphatic site)
o Stage Ill: Nodal group involvement above and below
diaphragm
o Stage IV: Diffuse involvement of one or more
extralymphatic sites (liver, lungs, marrow)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Nodal mass; fever; night sweats; weight loss
o Other signs/symptoms: Axillary, neck or groin mass
• Clinical profile: Young patient with palpable nodal
mass
Demographics
• Age: HD bimodal; 2 peak incidences: 20-24, 80-84
• Gender: Slight increase in males for HD
• Ethnicity: HD: Higher in Caucasians
Natural History & Prognosis
• HD: 80% cure with chemotherapy and/or radiation, or
stem cell transplant
• NHL: Depends on grade and stage; in general, worse
than HD, worst in AIDS-related NHL; poor prognosis if
patients don't respond to initial therapy
Treatment
• Options, risks, complications
o HD: Initial chemotherapy or radiation; stem cell
transplant for treatment failure or relapse
o NHL: Initial treatment with chemotherapy; stem cell
transplant for failure or recurrence
LYMPHOMA
I DIAGNOSTIC CHECKLIST
Consider
• Metastatic nodes from carcinoma
Image Interpretation Pearls
• Confluent soft tissue mass surrounding aorta and IVC
I SELECTED REFERENCES
1. Morgan PB et al: Uncommon presentations of Hodgkin's
disease. Case 1. Hodgkin's disease of the jejunum. J Clin
Oncol. 22(1):193-5, 2004
2. O'Malley ME et al: US of gastrointestinal tract
abnormalities with CT correlation. Radiographics.
23(1):59-72, 2003
3. Sheth S et al: Mesenteric neoplasms: CT appearances of
primary and secondary tumors and differential diagnosis.
Radiographics. 23(2):457-73; quiz 535-6, 2003
4. Ahmad A et al: Gastric mucosa-associated lymphoid tissue
lymphoma. AmJ Gastroenterol. 98(5):975-86, 2003
5. Vinnicombe SJ et al: Computerised tomography in the
staging of Hodgkin's disease and non-Hodgkin's
lymphoma. Eur J Nucl Med Mol Imaging. 30 Suppl
1:S42-55, 2003
6. Elis A et al: Detection of relapse in non-Hodgkin's
lymphoma: role of routine follow-up studies. Am J
Hematol. 69(1):41-4, 2002
7. Apter S et al: Calcification in lymphoma occurring before
therapy: CT features and clinical correlation. AJRAm J
Roentgenol. 178(4):935-8,2002
8. Kurosawa H et al: Burkitt lymphoma associated with large
gastric folds, pancreatic involvement, and biliary tract
obstruction. J Pediatr Hematol Oncol. 24(4):310-2, 2002
9. Boni L et al: Primary pancreatic lymphoma. Surg Endosc.
16(7): 1107-8, 2002
10. Jerusalem G et al: Whole-body positron emission
tomography using 18F-fluorodeoxyglucose compared to
standard procedures for staging patients with Hodgkin's
disease. Haematologica. 86(3):266-73, 2001
11. Daskalogiannaki M et al: Splenic involvement in
lymphomas. Evaluation on serial CT examinations. Acta
Radiol. 42(3):326-32, 2001
12. Sheth S et al: Non-Hodgkin lymphoma: pattern of disease
at spiral CT. Crit Rev Diagn Imaging. 42(6):307-56, 2001
13. Fields S et al: CT-guided aspiration core needle biopsy of
gastrointestinal wall lesions. J Com put Assist Tomogr.
24(2):224-8, 2000
14. Jung G et al: Abdominal lymphoma staging: is MR imaging
with T2-weighted turbo-spin-echo sequence a diagnostic
alternative to contrast-enhanced spiral CT? J Comput
Assist Tomogr. 24(5):783-7, 2000
15. Hwang K et al: Imaging of malignant lymphomas with
F-18 FDG coincidence detection positron emission
tomography. Clin Nucl Med. 25(10):789-95, 2000
16. Oh YKet al: Stages I-III follicular lymphoma: role of CT of
the abdomen and pelvis in follow-up studies. Radiology.
210(2):483-6, 1999
17. Chang DK et al: Lymph node involvement rate in
low-grade gastric mucosa-associated lymphoid tissue
lymphoma--too high to be neglected.
Hepatogastroenterology. 46(28):2694-700, 1999
18. Zinzani PL et al: The role of positron emission tomography
(PET) in the management of lymphoma patients. Ann
Oncol. 10(10):1181-4, 1999
19. Kessar P et al: CT appearances of mucosa-associated
lymphoid tissue (MALT)lymphoma. Eur Radiol. 9(4):693-6,
1999
 RETROPERITONEAL LYMPHOMA

I IMAGE GAllERY 1
27
Typical
(Left) Axial CECT of typical
confluent "mantle" of nodal
disease in non-Hodgkin
lymphoma. Note soft tissue
mass (arrow) surrounding
aorta and inferior vena cava.
(Right) Axial CECT of nodal
disease in non-Hodgkin
lymphoma. Note nodal mass
surrounding inferior vena
ca va (arrows).

Typical
(Left) Pancreatic
non-Hodgkin lymphoma on
axial CECT Note bulky mass
in region of head of pancreas
(arrow). (Right) Pancreatic
non-Hodgkin lymphoma on
axial CECT Note lack of
encasement of mesenteric
vessels (arrow).

Typical
(Left) Axial CECT after
lymph angiogram (LAC) for
Hodgkin disease. Note high
attenuation LAC contrast in
some nodes (arrow) but not
others (open arrow). (Right)
Fused coronal PET-CT image
in patient with Hodgkin
disease. Note FOC avid right
para-aortic and iliac nodes
(arrows).
 RETROPERITONEAL METASTASES
1
28

Axial CECT shows lobulated, homogenous bulky left Axial CECTshows heterogeneous retroperitonealnodal
para-aortic lymphadenopathy in patient with left mass encasing vessels in patient with
testicularseminoma. non-seminomatous germ cell tumor. A stent has been
placed in the rightureter (arrow).

ITERMINOlOGY • Size
o Para-aortic, aortocaval, pelvic lymphadenopathy: >
Abbreviations and Synonyms 10 mm short axis
• Retroperitoneal (RP) metastases (mets) o Retrocrural nodes: > 6 mm short axis diameter
• Morphology
Definitions o Focal or diffusely infiltrating masses
• Spread of malignant cells to retroperitoneal lymph o Round nodes (max diameter < l.Sx min diameter)
nodes and surrounding tissues suggest malignancy
Radiographic Findings
I IMAGING FINDINGS • Radiography: Lymphangiography no longer indicated
for detection of retroperitoneal adenopathy
General Features
CT Findings
• Best diagnostic clue
• NECT: Calcification in metastatic nodes from ovarian
o Enlarged RP lymph nodes in patient with known
cancer, mucin-producing tumors
malignancy
o Cluster of small RP nodes in patient with • CECT
o Testicular cancer: Bulky RP nodes in seminomas,
nonseminomatous testicular cancer
small nodes in nonseminomatous germ cell tumors
• Location (GCT)
o RP lymphadenopathy: Periaortic, interaortocaval,
o Discrete or confluent nodal masses
pericaval
• Low density nodes can be seen in untreated
• Testicular cancers: Lymphatic drainage follows
testicular CA
gonadal vessels; nodal mets at ipsilateral renal
• Growing low density RP mass in treated
hilum
nonseminomatous GCTs: Residual teratoma
• Pelvic cancers: Obturator nodes may be first site of
o Discrete masses in retroperitoneal fat
involvement
o Malignant retroperitoneal fibrosis: Confluent soft
o Perinephric and properitoneal fat
tissue mass; anterior displacement of aorta
o Iliopsoas muscle compartment

DDx: Retroperitoneal Masses

Lymphoma Hemorrhage Tuberculosis LAM

 RETROPERITONEAL METASTASES
Key Facts
1
Imaging Findings Top Differential Diagnoses 29
• Testicular cancers: Lymphatic drainage follows • Retroperitoneal lymphoma
gonadal vessels; nodal mets at ipsilateral renal hilum • Primary retroperitoneal tumors
• Para-aortic, aortocaval, pelvic lymphadenopathy: > 10 • Infection: Tuberculosis, AIDS
mm short axis • Retroperitoneal fibrosis
• Retrocrural nodes: > 6 mm short axis diameter • Lymphangioleiomyomatosis (LAM)
• Round nodes (max diameter < 1.Sx min diameter) • Retroperitoneal hemorrhage
suggest malignancy
• Testicular cancer: Bulky RP nodes in seminomas, Pathology
small nodes in nonseminomatous germ cell tumors • Lymphatic or hematogenous spread: Testicular
(GCT) cancer, melanoma, ovarian, prostate, lung, breast
• Low density nodes can be seen in untreated testicular • Direct extension from primary intra-abdominal
CA neoplasms: Pancreas, GI cancers
• Growing low density RP mass in treated • Testicular germ cell tumors: Nodes at level of
nonseminomatous GCTs: Residual teratoma ipsilateral renal hilum

• Nodes may displace aorta anteriorly from spine

MR Findings
• TlWI Primary retroperitoneal tumors
o RP lymphadenopathy: Low-intermediate SI nodes • Mesenchymal tumors: Liposarcoma, leiomyosarcoma,
o Metastatic melanoma may have high signal malignant fibrous histiocytoma
intensity • Usually large size
• T2WI o Malignant: Average size 11-20 cm
o Tumor usually high signal intensity o Benign: Average size 4-7 cm
o Low signal intensity masses after treatment may be
fibrosis Infection: Tuberculosis, AIDS
• Tl C+: Metastatic disease in nodes may enhance like • TB: Often have low density adenopathy
primary tumor • AIDS: Adenopathy common but enlargement usually
not massive
Ultrasonographic Findings
Retroperitoneal fibrosis
• Real Time
o Round or oval lymph nodes with ratio • 70% idiopathic; may be immune response to
longitudinal/transverse diameter < 2 atherosclerotic disease
o Narrow or absent echogenic fatty hilum • 8-10% of cases are due to metastatic foci inciting
o Echogenic and heterogeneous; more echogenic than desmoplastic response
lymphoma Lymphangioleiomyomatosis (LAM)
• Color Doppler • Proliferation of smooth muscle cells in lymph vessels
o Malignant nodes may have increased vascularity
causes obstruction
o Displacement of normal nodal hilar vessels
• Low density masses surround and displace
Nuclear Medicine Findings retroperitoneal vessels
• PET Retroperitoneal hemorrhage
o May be more sensitive and specific than CT for
• High attenuation on noncontrast CT
certain RP mets depending on primary
• Clinical setting of anticoagulation, trauma and/or
o Non-seminomatous GCTs with well-differentiated
dropping hematocrit
components not very FDG-avid
Cirrhosis/hepatitis
Imaging Recommendations
• Upper abdominal lymphadenopathy common with
• Best imaging tool: CT
biliary and chronic viral etiologies
• Protocol advice
o Oral contrast may be helpful in thin patients to Sarcoidosis
distinguish bowel from RP nodes • Enlarged abdominal lymph nodes in 30%
'0 Obtain non-contrast scans if hemorrhage suspected

!PATHOlOGY
I DIFFERENTIAL DIAGNOSIS
General Features
Retroperitoneal lymphoma • General path comments
• Confluent soft tissue mantle of adenopathy or o Lymphatic or hematogenous spread: Testicular
enlarged bilateral nodes (> 1.S cm short axis) cancer, melanoma, ovarian, prostate, lung, breast
 RETROPERITONEAL
1 o Direct extension from primary intra-abdominal
neoplasms: Pancreas, GI cancers
30 • Etiology
o Malignant RP lymphadenopathy
• Testicular germ cell tumors: Nodes at level of
ipsilateral renal hilum
• Pelvic malignancies: Ovarian, bladder, prostate,
uterus, colorectal
• Patients with poorly differentiated cancer of
unknown primary affecting primarily
retroperitoneal-mediastinal nodes considered to
have extragonadal germ-cell syndrome
o Malignant retroperitoneal fibrosis: Desmoplastic
reaction to cancer cells
• Carcinomas: Breast, stomach, colon, lung
• Lymphoma, melanoma, carcinoid, sarcomas
• Associated abnormalities: Ureteral obstruction with
bulky retroperitoneal disease
Gross Pathologic & Surgical Features
• Seminoma: May have bulky retroperitoneal masses
• Non-seminomatous GCTs: Often have metastases in
normal sized nodes
Microscopic Features
• Testicular GCTs: Metastases may have different
histology from primary testicular lesion
o Embryonal CA: May show teratomatous histology in
nodes
o Teratoma may show choriocarcinoma in the lymph
nodes
Staging, Grading or Classification Criteria
• Retroperitoneal lymphadenopathy makes testicular
cancer stage II
ICLINICALISSUES
METASTASES
I DIAGNOSTIC CHECKLIST
Consider
• Lymph node enlargement> 1.5 em is unusual in the
AIDS complex alone; should prompt CT-guided biopsy
to rule out lymphoma, Kaposi sarcoma or MAl
Image Interpretation Pearls
• Nonseminomatous GCTs: Any lymph nodes at
ipsilateral hilum should be considered suspicious
regardless of size
• RP nodes> 4 mm located anterior to mid-portion of
aorta suspicious for metastatic nonseminomatous GCT
I SELECTED REFERENCES
1. Haaga JR et al: CT and MR imaging of the whole body, 4th
ed. St. Louis, Mosby, 1684-1714,2003
2. Nishino M et al: Primary retroperitoneal neoplasms: CT
and MR imaging findings with anatomic and pathologic
diagnostic clues. Radiographies. 23(1):45-57, 2003
3. Pallisa E et al: Lymphangioleiomyomatosis: pulmonary and
abdominal findings with pathologic correlation.
Radiographies. 22 Spec No:S185-98, 2002
4. Avila NA et al: Lymphangioleiomyomatosis:
abdominopelvic CT and US findings. Radiology.
216(1):147-53, 2000
5. Vesselle HJ et al: FDG PET of the retroperitoneum: normal
anatomy, variants, pathologic conditions, and strategies to
avoid diagnostic pitfalls. Radiographies. 18(4):805-23;
discussion 823-4, 1998
6. Hilton S et al: CT detection of retroperitoneal lymph node
metastases in patients with clinical stage I testicular
nonseminomatous germ cell cancer: assessment of size and
distribution criteria. AjR Am J Roentgenol. 169(2):521-5,
1997
7. Jeffrey RBJr et al: Abdominal CT in acquired
immunodeficiency syndrome. AJRAm J Roentgenol.
146(1):7-13, 1986

Presentation
• Most common signs/symptoms
o Retroperitoneal disease often asymptomatic
o Other signs/symptoms
• Back pain, hematuria, obstructive uropathy
• Clinical profile
o RP lymphadenopathy: Known lymphoma, testicular,
renal cell, cervical, prostate, or ovarian cancer
o RP fat metastases: Melanoma, small cell lung cancer
Demographics
• Age: Any; depends on primary cancer
• Gender: Both; depends on primary cancer
Natural History & Prognosis
• Testicular cancer: Excellent prognosis even with RP
lymphadenopathy
Treatment
• Options, risks, complications
o Testicular germ-cell tumors: 3 possibilities for
residual masses
• Residual cancer: Rx with chemotherapy
• Residual benign teratoma following chemotherapy
("growing teratoma syndrome"): Rx with surgery
• Fibrosis: No Rx needed
 RETROPERITONEAL METASTASES

I IMAGE GALLERY 1
31

(Left) Axial CECT shows

heterogeneous pericaval
nodal mass (arrow)
representing metastatic
prostate CA. Sclerotic bony
metastases are also present.
(Right) Axial CECT shows left
para-aortic and pericaval
nodes (arrows) in patient
with metastatic rectal cancer.

Variant
(Left) Axial CECT shows
para-aortic and paracaval
nodes (arrows) in patient
with ovarian CA. Mesenteric
nodes (open arrows),
ascites, and peritoneal
implants (curved arrows) are
also present. (Right) Axial
CECT shows tumor implant
in perinephric fat (arrow) in
patient with metastatic
neuroendocrine tumor of the
cervix. Pancreatic metastases
are also present (open
arrows).

Variant
(Left) Axial CECT shows
heterogeneously enhancing
enlarged pelvic lymph nodes
(open arrows) in patient with
metastatic ovarian cancer. A
peritoneal drop metastasis is
also noted (arrow). (Right)
Axial CECT shows masses in
the right retroperitoneal fat
(arrows) representing
metastatic leiomyosarcoma.
 RETROPERITONEAL LYMPHOCELE
1
32

Axial CECT shows bilaterallymphoceles (arrows) along Axial CECT shows Iymphocele (arrow) along right iliac
the iliac vessels in a woman following bilateral vesselsin a patient following pancreatic transplantation
salpingo-oophorectomy and pelvic lymph node (open arrow). Note high attenuation clips along
dissection. periphery of Iymphocele.

ITERMINOlOGY MR Findings
• Tl WI: Round or ovoid mass with low signal intensity
Abbreviations and Synonyms
• T2WI: Round or ovoid mass with high signal intensity
• Retroperitoneal (RP) lymphocele, lymph cyst
• Tl C+: Low signal masses without enhancement
Definitions
Ultrasonographic Findings
• Pseudocyst formed when lymph leaks from disrupted
• Real Time
lymphatics
o Anechoic or hypoechoic mass
o Increased through transmission
o May have dependent debris, septations
I IMAGING FINDINGS
Imaging Recommendations
General Features • Best imaging tool: CECT
• Best diagnostic clue: Low density retroperitoneal fluid • Protocol advice: Delayed imaging (> 15 minutes)
collection in post-surgical patient useful to detect filling of urinoma or bladder
• Location: Along iliac vessels, para-aortic diverticulum
retroperitoneum, inguinal area
• Size: Variable
• Morphology I DIFFERENTIAL DIAGNOSIS
o Unilocular or multilocular
o Round or oval Other pelvic cystic masses
CT Findings • Urinoma: Should fill with contrast on delayed imaging
• Abscess: Rim-enhancement
• NECT • Hematoma, endometrioma: High attenuation;
o Low density fluid collection when uncomplicated fluid-fluid levels
o Calcification is rare
• CECT:Imperceptible wall; does not enhance with Bladder (BI.) diverticulum
contrast • Typically near ureteral insertions
• Fill with contrast on delayed imaging

DDx: Cystic Pelvic Masses

Pelvic Hematoma Endometrioma 81. Diverticulum

 RETROPERITONEAL LYMPHOCELE
Key Facts 1
Terminology • CECT: Imperceptible wall; does not enhance with 33
• Pseudocyst formed when lymph leaks from disrupted contrast
lymphatics • Anechoic or hypoechoic mass

Imaging Findings Clinical Issues

• Best diagnostic clue: Low density retroperitoneal fluid • Most lymphoceles are asymptomatic
collection in post-surgical patient • May compromise function of renal transplants by
• Location: Along iliac vessels, para-aortic obstructing blood supply or ureter
retroperitoneum, inguinal area

• May see narrow neck connecting to bladder

Natural History & Prognosis
lymphangioma • Most lymphoceles are asymptomatic
• More often multiloculated • May compromise function of renal transplants by
• May have calcification obstructing blood supply or ureter
• May become secondarily infected

I PATHOLOGY Treatment
• Options, risks, complications
General Features o Smalllymphoceles: Often resolve spontaneously
• General path comments: Common after surgery near o Percutaneous aspiration: Not definitive
large lymphatic trunks o Long-term catheter drainage: Success in 50-87%
• Etiology o Sclerotherapy: Success in 79-94%
o Lymphatics have slow flow and normally collapse o Transplants: Surgicallymphocele fenestration
after being disrupted
o Persistent lymphatic leakage from divided lymphatic
channels ~ lymph pseudocyst I DIAGNOSTIC CHECKLIST
• Epidemiology
o Up to 30% incidence post radical pelvic Image Interpretation Pearls
lymphadenectomy • Enhancing thick wall suggests superinfection
o 0.6-20% incidence post renal transplant
• 3-4% symptomatic
I SELECTED REFERENCES
Gross Pathologic & Surgical Features
1. Fuller TF et al: Management of lymphoceles after renal
• Tan, dark yellow or brown fluid transplantation: laparoscopic versus open drainage. J Urol.
169(6):2022-5,2003
Microscopic Features 2. Chow CC et al: Complications after laparoscopic pelvic
• Fat globules, lymphocytes, few red blood cells lymphadenectomy: CT diagnosis. AJR Am J Roentgenol.
• Fluid with high protein content 163(2):353-6, 1994
3. vanSonnenberg E et al: Lymphoceles: imaging
characteristics and percutaneous management. Radiology.
I CLINICAL ISSUES 161(3):593-6, 1986

Presentation
• Most common signs/symptoms I IMAGE GAllERY
o Only 5-7% of patients symptomatic
o Renal transplant patients
• Iliac vein compression: Unilateral leg edema
• Swelling over transplant
• Elevated creatinine due to ureteral compression
o Abdominal distention, pain, secondary infection
• Clinical profile
o Post-operative patient: RP node dissection; groin
surgery
o Renal transplant patient
o Typical presentation is several weeks after surgery
Demographics
• Age: Any (Left) Axial NECT shows right sided Iymphoce/e with thin rim of
• Gender: Both calcification (arrow). (Right) Sagittal CECT shows Iymphoce/e
(arrow) anterior to internal iliac vessels.Note adjacent surgical clips.
 SECTION 2: Adrenal

Introduction and Overview

Adrenal Anatomy and Imaging Issues 11I-2-2

Infection
Adrenal TB and Fungal Infection 11I-2-6

Vascular - Traumatic
Adrenal Hemorrhage 11I-2-8

Metabolic
Adrenal Hyperplasia 11I-2-12
Adrenal Insufficiency 11I-2-16

Neoplasm, Benign
Adrenal Cyst 11I-2-18
Adrenal Adenoma 11I-2-20
Adrenal Myelolipoma 11I-2-24
Pheochromocytoma 11I-2-26

Neoplasm, Malignant
Adrenal Carcinoma 11I-2-30
Adrenal Metastases and Lymphoma 11I-2-34
Adrenal Collision Tumor 11I-2-38
 ADRENAL ANATOMY AND IMAGING ISSUES
2
2
Graphic shows adrenal anatomy. Note multiple arterial
sources. Right adrenal vein drains directly into the IVc,
while the left enters the renal vein.
I TERMINOLOGY
Abbreviations and Synonyms
• Tuberculosis (TB)
• Adrenal corticotrophic hormone (ACTH)
• Corticotropin releasing hormone (CRH)
• Antidiuretic hormone (ADH)
I IMAGING ANATOMY
• Location
o Adrenal glands lie in the perirenal space (within the
perirenal fascia), usually cephalic to the kidneys
o Right adrenal is suprarenal, lies lateral to
diaphragmatic crus, medial to liver, and touches the
back of the IVC
o Left adrenal is usually ventral to upper pole of left
kidney, dorsal to the splenic vein and pancreas
I ANATOMY-BASED IMAGING ISSUES I
Key Concepts or Questions
• Adrenal cortex and medulla are essentially different
organs within the same structure
o Cortex is an endocrine gland secreting primarily
cortisol, aldosterone, and androgenic steroids (all
derived from cholesterol, which contributes to the
high lipid content characteristic of adrenals)
o Medulla is derived from neural crest and secretes
epinephrine and norepinephrine
• Adrenal physiology is controlled by elaborate
interaction between the hypothalamus, pituitary, and
adrenals
o Stress results in release of epinephrine (and
cytokines with fever, trauma, etc.) which cause the
pituitary to secrete ACTH, which stimulates adrenal
release of cortisol
Graphic shows stimulation of the anterior pituitary by
epinephrine and cytokines, or by hypothalamic release
of CRH or ADH, resulting in release of ACTH causing
adrenal to secrete cortisol.
o Elevated serum cortisol has a suppressing effect on
the hypothalamus and pituitary, reducing further
release of ACTH
• Cushing syndrome (due to excess cortisol)
o Characterized by truncal obesity, hirsutism,
amenorrhea, hypertension, weakness, abdominal
striae
o Causes
• Adrenal hyperplasia (75-80%)
• Adrenal adenoma (20-25%)
• Adrenal carcinoma « 5%)
• Exogenous corticosteroid medication
o Diagnostic tests for Cushing syndrome
• Serum: High cortisol level
• Urine: High 17-hydroxycorticoid level
• Serum: Elevated ACTH = hyperplasia; low ACTH =
autonomous adenoma
• Dexamethasone suppression test: To distinguish
pituitary from "ectopic" source of excess ACTH
• Conn syndrome (excess aldosterone)
o Characterized by hypertension, hypokalemic
alkalosis, muscle weakness, cardiac dysfunction
o Causes
• Adrenal adenoma (65-70%)
• Adrenal hyperplasia (25-30%)
• Adrenal carcinoma « 1%)
• Addison syndrome (disease) (adrenal insufficiency)
o Causes of slow onset insufficiency
• Autoimmune (most common in developed
countries)
• TB or systemic fungal or granulomatous disease
• Metastases
• AIDS (acquired immunodeficiency syndrome)
o Causes of abrupt onset insufficiency
• Adrenal hemorrhage (sepsis, shock, septicemia,
anticoagulation, vasculitis)
• Post-partum pituitary necrosis (Sheehan
syndrome)
• Withdrawal of long term steroid medication
• What is the likelihood of a small « 1.5 cm) adrenal
mass to be an adenoma?
 ADRENAL ANATOMY AND IMAGING ISSUES
DIFFERENTIAL DIAGNOSIS
Adrenal neoplasms
• Adenoma
• Metastases
• Lymphoma
• Pheochromocytoma
• Carcinoma
• Myelolipoma
• Hemangioma
Non-neoplastic adrenal masses
• Hemorrhage
• Infection
• Hyperplasia
• Cyst
• Pseudocyst
• (Gastric diverticulum; vessels)
o In the absence of a known malignancy, close to
100%
• (At least 2% of the general population have a
non-hyperfunctioning adrenal adenoma)
o In a patient with cancer, most small adrenal masses
are still adenomas
• If an adrenal mass is of indeterminate attenuation (>
15 HU) on nonenhanced CT, will a subsequent MR
evaluation be of value in distinguishing adenoma
from metastasis?
o Probably not because both non enhanced CT and
MR rely on imaging evidence of high lipid content
to diagnose adenoma
• "Lipid-poor" adenomas are best evaluated by
"adrenal protocol CT" (including delayed phase)
• What is a practical imaging approach for diagnosing
pheochromocytoma?
o Diagnosis should be established with reasonable
certainty prior to imaging (symptoms of headache,
palpitations and excessive sweating; urine assay for
catecholamine metabolites, metanephrine and
vanillylmandelic acid)
o For CT, include lower thorax to symphysis
• 98% of pheochromocytomas arise in the abdomen
• 90% arise in the adrenals
• Pericardium is rare site, but easily included on CT
o For MR, include at least adrenals through aortic
bifurcation
• T2WI and gadolinium-enhanced sequences are
best
• Is there an important distinction between the lipid
content, characteristic of adenoma, and fat content,
characteristic of myelolipoma?
o The lipid in adenoma is intra- and intercellular and
not in macroscopic deposits
• NECT detects this usually as near-water density (-5
to +15 HU)
• MR detects this best with a combination of
in-phase and opposed-phase sequences; the latter
designed to suppress signal from tissue with an
even admixture of lipid and water protons
o The fat in myelolipomas is usually macroscopic,
mature
Incidental adrenal mass
• Adenoma
• Nodular hyperplasia
• Myelolipoma
•
•
(Pseudomass)
Hematoma, hemorrhage 2
• Metastasis
• Ganglioneuroma 3
• Pheochromocytoma
• Carcinoma
Neural crest tumors
• Neuroblastoma (malignant, children)
• Ganglioneuroblastoma
• Ganglioneuroma (benign, adults)
• Pheochromocytoma (usually benign, adults)
• NECT detects this usually as fat-density (-80 to
-100 HU) deposits: Thin sections and pixel
analysis may be necessary to detect small foci of
fat
• Opposed-phase MR will not suppress signal from
large foci of fat
• Is it necessary and safe to give LV. contrast material
for CT or MR evaluation of pheochromocytoma?
o Because most sporadic pheochromocytomas are
large (3 to 5 cm) and are easily recognized in an
adrenal site, contrast administration is usually
unnecessary
o In the setting of "syndromic " pheochromocytoma
(e.g., von Hippel Lindau, multiple endocrine
neoplasia), pheochromocytomas are often multiple,
small, and extra-adrenal, and LV. contrast may be
necessary
o It is extremely rare for LV. administration of
nonionic iodinated or gadolinium-based contrast
media to induce a hypertensive crisis in patients
with pheochromocytoma
o Most authorities consider it unnecessary to give
prophylactic ()(and B-adrenergic blockade prior to a
contrast-enhanced CT or MR exam
• What is the role of MIBG in diagnosis of
pheochromocytoma?
o MIBG (metaiodobenzylguanidine) has a sensitivity
of nearly 90% and a specificity of 99%
o It is generally available only in large academic
referral centers and is very expensive
o Main roles are in patients with familial
pheochromocytoma syndromes, and in patients
with strong clinical evidence of pheochromocytoma
in whom CT or MR have failed to identify the tumor
• What is the role of adrenal biopsy?
o Rarely necessary with the proper use and
interpretation of CT or MR
o Dangerous and usually contraindicated for
pheochromocytoma
• What is the role of PET or PET-CTfor evaluation of
adrenal cancer (primary or metastatic)?
o Usually unnecessary
o Unproven in large clinical trials to date
 ADRENAL ANATOMY AND IMAGING ISSUES

2
4

Graphic shows adrenals on axial section. Note that the Axial CECT shows one of the typical appearances of
left adrenal lies in front of upper renal pole and behind normal adrenal glands (arrows).
splenic vein, while the right is suprarenal and touches
the IVC.

o Probably accurate and useful for diagnosing adrenal • Sepsis (Waterhouse-Friderichsen)

metastases as part of a "whole body" PET-CT exam • Surgery
for recognized indications (e.g., lymphoma, • Tumor
melanoma) • Blunt abdominal trauma
Ectopic (nonpituitary) sources of ACTH
• Oat cell carcinoma of lung
I CUSTOM DIFFERENTIAL DIAGNOSISI • Liver cancer
Calcified adrenal lesion • Bronchial or thymic carcinoid
• Infection • Bronchia adenoma
o TB, histoplasmosis • Pancreatic islet cell tumor
• Hemorrhage ("old") • Medullary carcinoma (thyroid)
• Carcinoma (small foci) • Thymoma
• Pheochromocytoma • Pheochromocytoma
o Plus heterogeneous hypervascularity
• Myelolipoma
o Plus foci of fat density I SELECTED REFERENCES
• Metastases 1. Caoili EM et al: Adrenal masses: characterization with
o Rare combined unenhanced and delayed enhanced CT.
Radiology. 222(3):629-33, 2002
Pheochromocytoma associated diseases 2. Dunnick NR et al: Imaging of adrenal incidentalomas:
• Multiple endocrine neoplasia (type II and III) current status. AJRAm J Roentgenol. 179(3):559-68, 2002
o Plus medullary thyroid carcinoma and parathyroid 3. Mayo-Smith WW et al: State-of-the-art adrenal imaging.
adenoma (Sipple syndrome) Radiographies. 21(4):995-1012, 2001
o Mucosal neuroma syndrome (type III) 4. Krebs TL et al: MR imaging of the adrenal gland:
radiologic-pathologic correlation. Radiographies .
• Medullary thyroid cancer and intestinal
18(6):1425-40, 1998
ganglioneuromatosis s. Cirillo RLJr et al: Pathology of the adrenal gland: imaging
• Neurofibromatosis features. AJRAm J Roentgenol. 170(2):429-35, 1998
• von Hippel Lindau 6. Dunnick NR et al: Adrenal radiology: distinguishing benign
o In some families, the predominant finding from malignant adrenal masses. AJRAm J Roentgenol.
o Usually with CNS hemangioblastomas, renal and 167(4):861-7, 1996
pancreatic cysts and tumors 7. Korobkin M et al: Delayed enhanced CT for differentiation
• Carney syndrome of benign from malignant adrenal masses. Radiology.
200(3):737-42, 1996
o Plus pulmonary chondromas, GI stromal tumors
• Tuberous sclerosis
o Plus neurological lesions, renal cysts and
angiomyolipomas
Adrenal hemorrhage in adults: Etiology
• Anticoagulant therapy
• Stress
 ADRENAL ANATOMY AND IMAGING ISSUES
IMAGE GALLERY
Typical
(Left) Graphic shows
location and function of
adrenal cortex and medulla. 2
(Right) Graphic shows
adrenal CT protocol and
criteria for diagnosing 5
adenoma.

~.lL'J'lt~~,~
~~

CORTEX

Cortisol
Aldosterone Steroids
Androgenic

(all derived from

cholesterol)

Typical
(Left) Graphic shows the
Calculating Washout formula for calculating
on Adrenal CT adrenal washout on CT.
(Right) Adrenal protocol CT.
ROI over lesion. expressed In Hounsfleld units (HU I NECT (A) shows adrenal
mass (arrow) measuring 30
Rel~tlve HU. The mass measures 60
HU on CECT (8), and 40
Enhanced CT(HU) - Delayed(HU)
------------- x 100% HU on delayed CT (C).
Enhanced CT(HU)
Lipid-poor adenoma.
A.bsolute( True I

Enhanced CT(HU) - Delayed(HU) x 100%

Enhanced CT(HU) - Unenhanced(HU)

Relative Washout> 40""} =Adenoma

Absolute Washout> 60""

Typical
(Left) Graphic indicates
potential sites for
pheochromocytoma (blue)
and paraganglioma (green)
organ of Zuckerkandl at
aortic bifurcation. (Right)
Axial CECT shows
hypervascular mass (arrow)
in pelvis; paraganglioma
with clinical symptoms
identical to those of
pheochromocytoma.
 ADRENAL TO AND FUNGAL INFECTION

2
6

Axial NECT shows nodular calcification and atrophy of Axial NECT shows nodular calcification and atrophy of
the adrenal glands. the adrenal glands.

[TERMINOLOGY • Chronic: Atrophic glands with calcifications

MR Findings
Definitions
• Tl WI: Signal intensity similar to spleen
• Tuberculosis and fungal infections in adrenal glands
• T2WI: Signal intensity similar to or higher than fat
• DWI: Granulomas show little enhancement
I IMAGING FINDINGS Ultrasonographic Findings
• Real Time: Homo-/heterogeneous, hypoechoic masses
General Features
• Best diagnostic clue: Calcifications of adrenal glands Imaging Recommendations
• Location: Bilateral> unilateral • Best imaging tool: CT
• Other general features
o Stages of infection: Acute, chronic
Radiographic Findings
I DI.FFERENTIAL DIAGNOSIS
• Radiography: Suprarenal calcifications Adrenal adenoma
• Mass with low attenuation « 10 HU)
CT Findings
• Focal mass without diffuse enlargement
• Acute • Enhancing mass that "de-enhances" rapidly
o Homo-/heterogenous masses
o Mild to marked symmetrical enlargement of adrenal Pheochromocytoma
glands with preserved contour • > 3 em, hypervascular mass
o Central areas of low attenuation (caseous necrosis) • Bilateral in multiple endocrine neoplasia (MEN)
o Enhancement in the peripheral rim
o Calcifications Adrenal metastases and lymphoma
• Faint flecks of calcium • Metastases
• Focal low attenuation nodules o Unilateral or bilateral, invasive, enhancing masses
• Large areas of necrosis or dense calcification o Central necrosis ± hemorrhage
• Enlargement of lymph nodes o Focal masses without diffuse enlargement

DDx: Adrenal Mass +/- Calcification

Lymphoma Metastases Hyperplasia Hemorrhage

 ADRENAL TO AND FUNGAL INFECTION

Key Facts
Imaging Findings • Pheochromocytoma
• Best diagnostic clue: Calcifications of adrenal glands • Adrenal metastases and lymphoma
• Mild to marked symmetrical enlargement of adrenal • Adrenal hemorrhage
glands with preserved contour • Adrenal hyperplasia
• Adrenal myelolipoma
• Enlargement of lymph nodes
• Best imaging tool: CT Diagnostic Checklist
2
Top Differential Diagnoses • Infection in patients with acute adrenal insufficiency 7
• Bilateral enlargement with preserved contour
• Adrenal adenoma

• Lymphoma • Gender: M = F
o Usually bilateral, triangular shaped masses
Natural History & Prognosis
Adrenal hemorrhage • Complications: Adrenal insufficiency (Addison
• Heterogenous hyperdense mass (50-90 HU) disease), sepsis
• Prognosis: Good, if recognized and treated
Adrenal hyperplasia
• Width of adrenal gland limb> 10 mm Treatment
• Contour of adrenal gland preserved • Tuberculosis: Antituberculosis therapy
• No discrete mass or nodule • Fungal infections: Antifungal therapy
Adrenal myelolipoma
• Small, asymptomatic, well-defined suprarenal mass I DIAGNOSTIC CHECKLIST
• Mass with fat attenuation (-30 to -115 HU)
Consider
• Infection in patients with acute adrenal insufficiency
I PATHOLOGY
Image Interpretation Pearls
General Features • Bilateral enlargement with preserved contour
• Etiology
o Tuberculosis (most common in underdeveloped
countries) I SELECTED REFERENCES
o Histoplasmosis (most common in Southeastern, 1. Kumar N et al: Adrenal histoplasmosis: clinical
South Central U.S.) presentation and imaging features in nine cases. Abdom
o Blastomycosis (South American, North American) Imaging. 28(5):703-8, 2003
o Coccidioidomycosis 2. Wilson DA et al: Histoplasmosis of the adrenal glands
o Cryptococcosis studied by CT. Radiology. 150(3):779-83, 1984
o Paracoccidioidomycosis 3. Wilms GE et al: Computed tomographic findings in
o Risk factors: Immunocompromised patients bilateral adrenal tuberculosis. Radiology. 146(3):729-30,
1983
• Associated abnormalities: Pulmonary or other
4. Morgan HE et al: Bilateral adrenal enlargement in
disseminated infections Addison's disease caused by tuberculosis.
Nephrotomographic demonstration. Radiology.
Gross Pathologic & Surgical Features
115(2):357-8, 1975
• Calcifications; caseous necrosis
Microscopic Features
• Granulomatous or fungal organisms I IMAGE GALLERY

I CLINICAL ISSUES •• I, ~ •••.• _~

Presentation ~:~,~~
, 'J .
• Most common signs/symptoms: Fever, night sweats, ..~
.. ,
weight loss, lethargy, weakness
• Diagnosis
o Tuberculosis: Positive purified protein derivative
(PPD) skin test
: 1&,'
o Percutaneous aspiration with cytology (e.g., acid fast
bacilli (AFB)staining) for definitive diagnosis """'~ ..
Demographics (Left) Axial NECT shows focal calcifications and atrophy of the
• Age: Any age; 20-40 years; most common adrenal glands. (Right) Axial NECT shows shows focal calcifications
and atrophy of the adrenal glands .
 ADRENAL HEMORRHAGE

2
8

Axial CECT shows hemorrhage around right adrenal Axial CECT shows nonspecific left adrenal mass;
followingmotor vehicle crash. hematoma followingmotor vehicle crash.

ITERMINOLOGY o Bilateral AH in 15% of individuals who die of shock

o Manifest with adrenal insufficiency when 90% of
Abbreviations and Synonyms adrenal tissue is destroyed
• Adrenal hemorrhage (AH) o Neonatal adrenal hemorrhage
• Most common cause of adrenal mass in infancy
Definitions • Usually seen during first week of life
• Hemorrhage within adrenal gland or tumor • Incidence ranges from 1.7-3% per 1000 births
• Gland, hypervascular & weighs twice of adults

IIMAGING FINDINGS CT Findings

General Features
• Best diagnostic clue: Hyperdense lesion within adrenal
gland on NECT
• Key concepts
o Relatively uncommon condition but potentially
catastrophic event seen in patients of all ages
• More common in neonates than children & adults
o Secondary to traumatic & non traumatic causes
• Traumatic more common than non traumatic
• May be unilateral or bilateral
o Traumatic hemorrhage: Blunt abdominal trauma
• Unilateral in 80% of cases: Right (85%), left (15%)
• Bilateral in 20% of cases
o Nontraumatic hemorrhage (often bilateral): Causes
are classified into 5 categories
• Stress, hemorrhagic diathesis or coagulopathy
• Neonatal stress, adrenal tumors, idiopathic
• Unilateral or bilateral adrenal hematomas
• ± Associated adrenal or renal vein thrombosis
• Acute or subacute hematoma
o Round or oval mass of high attenuation (50-90 HU)
o Asymmetric enlargement of adrenal glands
o Homogeneous & no enhancement with contrast
o Distortion of normal adrenal gland shape
o Inflammatory stranding of periadrenal fat
o Thickening of adjacent diaphragmatic crura
o ± Periadrenal hemorrhage + perinephric extension
o ± Upper abdominal traumatic findings
• Pneumothorax, hydropneumothorax, rib fracture
• Contusion of lung, liver, spleen or pancreas
• Chronic hematoma
o Mass with a hypoattenuating center (pseudocyst)
o Lack of enhancement confirms cystic nature of mass
o Calcifications (usually seen after 1 year in adults)
• Neonates: Seen within 1-2 weeks after trauma
• Hematomas j in size, attenuation over a period of time

DDx: Adrenal Mass

Bilateral Adenomas Bilateral Lymphoma Bilateral Metastases Hyperplasia

 ADRENAL HEMORRHAGE
Key Facts
Imaging Findings
• Best diagnostic clue: Hyperdense lesion within
adrenal gland on NECT
• ± Associated adrenal or renal vein thrombosis
• Round or oval mass of high attenuation (50-90 HU)
• Homogeneous & no enhancement with contrast
• Distortion of normal adrenal gland shape
• ± Underlying large adrenal mass (cyst, myelolipoma)
• Tl & T2WI: Varied signal based on age of hematoma
Top Differential Diagnoses
• Adrenal adenoma
• Adrenal metastases & lymphoma
• Adrenal hyperplasia
• Adrenal infection
• Adrenal carcinoma
• ± Underlying large adrenal mass (cyst, myelolipoma)
o Intracystic or intratumoral hemorrhage
• Adrenal cyst: Mass of water density
• Adrenal myelolipoma: Heterogeneous fatty mass
MR Findings
• Tl & T2WI: Varied signal based on age of hematoma
• Acute hematoma (less than 7 days after onset)
o T1WI: Isointense or slightly hypointense
o T2WI: Markedly hypointense
o Due to high concentration of intracellular
deoxyhemoglobin ~ T2 proton relaxation
• Subacute hematoma (7 days to 7 weeks after onset)
o Tl WI: Hyperintense
• Due to free methemoglobin (Fe3+), produced by
oxidation of hemoglobin (Fe2+) as hematoma ages
o T2WI: Markedly hyperintense
• Due to serum & clot lysis products
o Large hematoma: Varied signal (irregular clot lysis)
• Multilocular, fluid-fluid levels
• Chronic hematoma (beyond 7 weeks after onset)
o Tl & T2WI: Hyperintense hematoma
• Due to persistence of free methemoglobin
o TIWI & T2WI: Hypointense rim·
• Due to hemosiderin deposition in fibrous capsule
• Adrenal or renal vein thrombosis
o Clot: i Signal on both T1 & T2WI
• Extension of thrombus into IVe can be seen by MR
• Underlying large adrenal mass (cyst or myelolipoma)
o Varied signal based on content of mass lesion
• Gradient-echo imaging
o Demonstrate "blooming" effect (magnetic
susceptibility) due to hemosiderin deposition
• Detect blood & monitor hemorrhage as it
progresses from methemoglobin to hemosiderin
• Useful in large hematomas which exhibit slower
clot evolution
Ultrasonographic Findings
• Real Time
o Acute hematoma: Hyperechoic mass-like lesion
o Subacute hematoma
• Mixed echogenicity + central hypoechoic area
Pathology
• Anticoagulation therapy (most common)
• Antiphospholipid antibody syndrome & disseminated
intravascular coagulopathy
• Stress: Surgery, sepsis, burns, hypotension, steroids
• Metastases: Lung cancer & malignant melanoma 2
• Blunt abdominal trauma (Rt gland> Lt gland)
9
• Adrenal vein thrombosis; adrenal tumor
• Meningococcal septicemia (Waterhouse-Friderichsen
syndrome)
• Stress or adrenal tumor ~ i ACTH ~ i arterial blood
flow + limited venous drainage ~ hemorrhage
Diagnostic Checklist
• Check for history of trauma, anticoagulant therapy,
coagulopathies, malignancies, stress, adrenal tumor
o Chronic hematoma
• Anechoic & cyst-like lesion; wall calcification
• Color Doppler: Shows avascular nature of mass
Angiographic Findings
• Conventional
o Usually not recommended in adrenal hemorrhage
o Adrenal hemorrhage & pseudocyst: Avascular
o Adrenal mass: Neovascularity seen
Nuclear Medicine Findings
• Tc99m dimercaptosuccinic acid study (DMSA)
o Adrenal hematoma: Photopenic suprarenal mass
with inferior displacement of kidney
Imaging Recommendations
• Best imaging tool: Helical CT & MR
• Protocol advice
oCT: 3 mm thick section at 3 mm intervals or less
o MR: Spin-echo & gradient-echo imaging
I DIFFERENTIAL DIAGNOSIS
Adrenal adenoma
• NECT: Lipid rich adenoma « 10 HU)
• CECT: Washout of adenoma 15 min post LV. > 40%
washout
• Tl W out-af-phase
o Marked signal "drop-out" (lipid rich adenoma)
Adrenal metastases & lymphoma
• Adrenal metastases
o Lung cancer: Hemorrhagic; enhancing adrenal mass
o Malignant melanoma: Hypervascular metastases
• Adrenal lymphoma
o Primary (rare); secondary (non-Hodgkin, common)
o Often bilateral; retroperitoneal disease usually seen
o Discrete or diffuse mass, shape is maintained
Adrenal hyperplasia
• Adrenal glands are often symmetrically enlarged
• Width of adrenal gland limbs> 10 mm (diagnostic)
• No discrete mass or nodule seen as a rule
 ADRENAL HEMORRHAGE
Adrenal infection
• Granulomatous infection: Tuberculosis, histoplasmosis
• Adrenal calcification seen on CT imaging
Adrenal carcinoma
• Rare, unilateral, invasive & enhancing mass
2 • More than 6 cm when initially diagnosed
10
!PATHOlOGY
General Features
• Etiology
o Bilateral adrenal hemorrhage
• Anticoagulation therapy (most common)
• Antiphospholipid antibody syndrome &
disseminated intravascular coagulopathy
• Stress: Surgery, sepsis, bums, hypotension, steroids
• Pheochromocytoma; rarely trauma
• Metastases: Lung cancer & malignant melanoma
o Unilateral adrenal hemorrhage
• Blunt abdominal trauma (Rt gland> Lt gland)
• Adrenal vein thrombosis; adrenal tumor
o Neonates
• Difficult labor or delivery; renal vein thrombosis
• Asphyxia or hypoxia; hemorrhagic disorders
• Meningococcal septicemia
(Waterhouse-Friderichsen syndrome)
o Pathogenesis (non-traumatic)
• Stress or adrenal tumor ~ 1 ACTH ~ 1 arterial
blood flow + limited venous drainage ~
hemorrhage
• Stress or tumor ~ 1 catecholamines ~ adrenal
vein spasm ~ stasis ~ thrombosis ~ hemorrhage
• Coagulopathies ~ 1 venous stasis ~ thrombosis ~
hemorrhage
• Epidemiology
o Autopsy studies: 0.3-1.8% of un selected cases
o 15% of individuals who die of shock
o 2% of orthotopic liver transplantation cases
• Associated abnormalities
o Adrenal or renal vein thrombosis
o Adrenal tumor, hemorrhagic disorders
Gross Pathologic & Surgical Features
• Hematoma, enlarged gland, peri adrenal stranding
Microscopic Features
• Necrosis of all 3 cortical layers + medullary cells
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Nonspecific: Abdominal, lumbar, thoracic pain
o Fever, tachycardia, hypotension
o Acute adrenal insufficiency
• Fatigue, anorexia, nausea & vomiting
o Acute abdomen
• Guarding, rigidity, rebound tenderness
o Confusion, disorientation, shock in late phase
o Symptoms of associated underlying conditions
o Rarely, asymptomatic; incidental finding (imaging)
o Waterhouse-Friderichsen syndrome: Skin rash,
cough, headache, dizziness, arthralgias & myalgias
• Lab-data
o I Hematocrit or hemoglobin; 1 WBC
o Hyponatremia, hyperkalemia & prerenal azotemia
o I Serum cortisol, aldosterone, androgens & 1 ACTH
Demographics
• Age
o Any age group
• More common in neonates than children & adults
o Nontraumatic (40-80 years); traumatic (20-30 years)
• Gender: M:F=2:1
Natural History & Prognosis
• Complications
o Prerenal azotemia, adrenal abscess, shock
• Prognosis
o Prognosis depends on etiology rather than extent of
adrenal hemorrhage
o Unilateral adrenal hemorrhage (e.g., from blunt
trauma or liver transplantation)
• Rarely of clinical concern, resolve on its own &
adrenal functions normally
o Overall, AH is associated with a 15% mortality rate
• Waterhouse-Friderichsen syndrome: 55-60%
o Adults-adrenal crisis; neonate- death (> blood loss)
Treatment
• Medical
o Correct fluid, electrolytes & treat underlying cause
• Surgical: Adrenalectomy (open or laparoscopic)
o Surgery not required, except in adrenal tumors
I DIAGNOSTIC CHECKLIST
Consider
• Check for history of trauma, anticoagulant therapy,
coagulopathies, malignancies, stress, adrenal tumor
Image Interpretation Pearls
• NECT: Hyperdense lesion within adrenal gland
• MR: Varied signal intensity based on age of hematoma
I SELECTED REFERENCES
1. Dunnick NR et al: Imaging of adrenal incidentalomas:
Current status. AJR. 179:559-68, 2002
2. Caoili EM et al: Adrenal masses: characterization with
combined un enhanced and delayed enhanced CT.
Radiology. 222(3):629-33, 2002
3. Vella A et al: Adrenal hemorrhage: a 25-year experience at
the Mayo Clinic. Mayo Clin Proc. 76(2):161-8, 2001
4. Mayo-Smith WW et al: State-of-the-art adrenal imaging.
Radiographies. 21(4):995-1012, 2001
5. Khati NJ et al: Adrenal adenoma and hematoma mimicking
a collision tumor at MR imaging. Radiographies.
19(1):235-9, 1999
6. Kawashima A et al: Imaging of nontrilUmatic hemorrhage
of the adrenal gland. Radiographies. 19(4):949-63, 1999
7. Krebs TL et al: MR imaging of the adrenal gland:
radiologic-pathologic correlation. Radiographies.
18(6):1425-40, 1998
 ADRENAL HEMORRHAGE
I IMAGE GALLERY

Typical
(Left) Axial NECT shows high
density hemorrhage in the
dependent part of a large
cystic mass; proven adrenal
hemorrhage in a post-partum
2
31 year old woman. (Right) 11
Axial CECT shows
heterogeneous right adrenal
mass; postpartum
hemorrhage,

Typical
(Left) Axial CECT shows
bilateral adrenal hemorrhage
due to shock from pelvic
fractures. (Right) Axial CECT
shows pelvic fractures in a
patient with bilateral adrenal
hemorrhage.

Typical

,
(Left) Axial CECT shows a
right adrenal hematoma due
to trauma from recent liver
, transplantation, (Right) Axial

. ~ . CECTshows a right adrenal

'II> hematoma due to recent
liver transplantation.
~-
j ~
.;:. ~',:.

\ '6,
~~:'

t·' ..
,,'- ~"
,
.

•••
 ADRENAL HYPERPLASIA
2
12
Axial CECT shows enlargement of both adrenal glands
but preservation of normal shape.
ITERMINOlOGY
Definitions
• Bilateral adrenal gland hyperfunction ± enlargement
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Enlarged limbs of one or both
adrenal glands> 10 mm width on CT
• Location: Suprarenal
• Key concepts
o Width of adrenal gland limbs> 10 mm is consistent
with hyperplasia
o Glands may appear normal size or multinodular
o Adrenal gland shape is maintained
o Clinical syndromes caused by adrenal hyperplasia
• Cushing syndrome (hypercortisolism)
• Conn syndrome (hyperaldosteronism)
o Bilateral adrenocortical hyperplasia
• ACTH dependent Cushing syndrome
• Conn syndrome (primary hyperaldosteronism)
• PPNAH or PPNAD: Primary pigmented nodular
adrenocortical hyperplasia or dysplasia
• AIMAH: ACTH-independent macro nodular
adrenocortical hyperplasia
• Congenital adrenal hyperplasia (CAH)
DDx: Bilateral Adrenal Enlargement
Adenomas Lymphoma
Axial CECT shows enlargement of both adrenal glands
but preservation of normal shape.
o Cushing syndrome (hypercortisolism)
• Adrenocorticotrophic hormone (ACTH)
dependent (80-85%)
• Adrenocorticotrophic hormone (ACTH)
independent (15-20%)
o ACTH dependent Cushing syndrome: Bilateral
adrenal hyperplasia in 80-85% of cases
• ACTH-secreting anterior pituitary adenoma:
Cushing disease (75-85% of cases)
• Ectopic ACTH-secreting tumors (15%)
• Hypothalamic tumor ~ t corticotropin releasing
factor ~ t pituitary ACTH (rare)
o ACTH independent Cushing syndrome (15-20%)
• Usually due to adrenal adenoma> carcinoma
• Rarely caused by PPNAH or PPNAD & AIMAH
o Conn syndrome (primary hyperaldosteronism)
• 20% of cases are due to adrenal gland hyperplasia
• 80% of cases are due to adrenal adenoma
o Congenital adrenal hyperplasia (CAH)
• Autosomal recessive; due to enzyme deficiencies
• Decreased secretion of cortisol, aldosterone or
both with compensatory increase in ACTH
• Cause most cases of adrenogenital syndrome
• Usually present in children, rarely in adults
• Associated with testicular & ovarian tumors that
arise from ectopic adrenal cortical rests
o Bilateral adrenal medulla hyperplasia
• Nodular or diffuse
Metastases Hemorrhage
 ADRENAL HYPERPLASIA
Key Facts
Imaging Findings
• Best diagnostic clue: Enlarged limbs of one or both
adrenal glands> 10 mm width on CT
• Adrenal glands are often symmetrically enlarged
• Usually both adrenal glands involved
• No discrete mass or nodule seen as a rule
• Cushing syndrome can show nodular hyperplasia
• Up to 30% of cases may show normal glands
Top Differential Diagnoses
• Adrenal adenoma
• Adrenal metastases & lymphoma
• Adrenal hemorrhage
• Pheochromocytoma
Pathology
• Congenital (due to enzyme deficiencies)
• May cause hypertensive symptoms similar to
pheochromocytoma
• Associated with multiple endocrine neoplasia
(MEN 2b/3), duodenal carcinoid
CT Findings
• ACTH dependent Cushing syndrome (80-85% of cases)
o Adrenal glands are often symmetrically enlarged
o Limbs of adrenal gland> 10 mm makes this
diagnosis
o Usually both adrenal glands involved
o No discrete mass or nodule seen as a rule
o Cushing syndrome can show nodular hyperplasia
o Up to 30% of cases may show normal glands
• Conn syndrome (primary hyperaldosteronism)
o Findings similar to Cushing syndrome
o Adrenal glands may show nodular hyperplasia
• PPNAH or PPNAD
o Normal sized adrenal glands
o Small discrete nodules between 2-5 mm in size
o Moderate enhancement of nodular glands
• AIMAH
o Massively enlarged bilateral adrenal glands
o Normal adrenal shape is retained
o Adrenal limb width & nodule size 30 mm
o Marked enhancement of hyperplastic nodular
glands, predominantly at periphery
MR Findings
• Adrenal hyperplasia due to Cushing, Conn syndromes
o Tl WI define both adrenals within retroperitoneal fat
o Increase in width of adrenal gland limbs> 10 mm
o Adds no new information compared to CT findings
• TIWI
o PPNAH or PPNAD
• Hyperintense relative to muscle & spleen
• Isointense relative to liver
o AIMAH
• Isointense relative to spleen & hypointense
relative to liver
o ACTH-dependent (hyperplasia) Cushing syndrome
• Out of phase sequence: 35-40% signal dropout
within glands indicating intracellular lipid
• Cushing disease: Pituitary adenoma - t ACTH -
adrenal gland hyperplasia
• Cushing syndrome: Adrenal adenoma/carcinoma,
iatrogenic, ectopic ACTH production
• Conn syndrome (primary hyperaldosteronism)
Clinical Issues
2
• Moon facies, truncal obesity, buffalo hump 13
• Hypertension & hypokalemia
• Lab data: t Levels of ACTH, cortisol, aldosterone
• Diagnosis: Clinical, biochemical, imaging, histology
Diagnostic Checklist
• Correlate clinical, biochemical & imaging findings
• Enlarged limbs of adrenal glands (> 10 mm) on CT
• Multinodular hyperplasia difficult to distinguish from
adenoma by CT
• T2WI
o PPNAH or PPNAD
• Hyperintense relative to muscle & liver
• Isointense relative to spleen
o AIMAH
• Hyperintense relative to both liver & spleen
• Tl C+
o PPNAH or PPNAD: Moderate nodular enhancement
o AIMAH: Marked homogeneous enhancement of
hyperplastic nodular glands
Nuclear Medicine Findings
• Adrenocortical scintigraphy
o NP-59 is a cholesterol analog that binds to low
density lipoprotein receptors of adrenal cortex
o Normal NP-59: When both adrenal glands are seen
on day 5 after injection or thereafter
o Adrenal gland hyperplasia: Bilateral early adrenal
visualization before day 5 after injection
o Adenoma (adrenal): Unilateral early adrenal
visualization before day 5 after injection
Imaging Recommendations
• Abdominal CT best imaging tool for this diagnosis
• Contiguous :s; 3 mm CT images best technique
I DIFFERENTIAL DIAGNOSIS
Adrenal adenoma
• Intracellular lipid makes CT density -20 to +10 HU
• Focal mass, not diffuse enlargement
• Over 40% washout of contrast on a 15 minute delayed
CT scan is diagnostic of an adenoma
Adrenal metastases & lymphoma
• Adrenal metastases
o Invasive, enhancing mass in adrenal gland
o Focal mass, loss of adrenal shape
• Adrenal lymphoma
o Usually spread to adrenal gland of retroperitoneal
o Bilateral primary lymphoma (non-Hodgkin) can
simulate hyperplasia; usually more mass-like
o Hypovascular; moderate enhancement with contrast
 ADRENAL HYPERPLASIA
Adrenal hemorrhage
• Adults
o Septicemia, burns, trauma, severe stress or
hypotension
o Excessive anticoagulation, thrombocytopenia
o Disseminated intravascular coagulation,
2 anti phospholipid antibody syndrome
• Neonates: Meningococci, pneumococci & gonococci
14 • Adrenal hematomas usually appear round in shape
• Some poorly marginated & show periadrenal stranding
• CT
o Acute: High density fluid collection (40-60 HU)
o Chronic: Low density (clot of 20-30 HU)
Pheochromocytoma
• Tumor of adrenal medulla & usually more than 3 em
• Highly vascular; prone to hemorrhage & necrosis
• Tumors are very hyperintense on T2WI
• Bilateral adrenal tumors in MEN IIA & lIB syndromes
I PATHOLOGY
General Features
• General path comments
o Normal adrenal anatomy
• Classically described as inverted Y,V or T-shaped
• Width of limb (4-9 mm); gland weight (4-6 grams)
• Etiology
o Congenital (due to enzyme deficiencies)
o Cushing disease: Pituitary adenoma ~ 1 ACTH ~
adrenal gland hyperplasia
o Cushing syndrome: Adrenal adenoma/carcinoma,
iatrogenic, ectopic ACTH production
o Conn syndrome (primary hyperaldosteronism)
• 1 Aldosterone ~ 1 preservation of sodium & loss
of potassium at renal tubular level
• 1 Sodium ~ water retention ~ 1 extracellular
volume ~ hypertension ~ I renin production
• Hypokalemia causes hypokalemic alkalosis
• Associated abnormalities
o Pituitary adenoma in ACTH-dependent hyperplasia
o PPNAH associated with Carney complex
• Cushing disease, spotty skin pigmentation
• Cutaneous & cardiac myxomas
• Sertoli cell tumors of testis in males
• Multiple myxoid fibroadenomas of breast, females
o Adrenal medulla hyperplasia: MEN 2b/3, duodenal
carcinoid
Gross Pathologic & Surgical Features
• Bilateral diffusely enlarged adrenal glands
• Nodular, macronodular or pigmented nodular glands
Microscopic Features
• Thickened zona glomerulosa, fasciculata, reticularis
• Fasciculata may show excess lipid, lipid depleted or
atypical cells with hyperchromatic nuclei in nodules
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic incidental CT finding
o Cushing syndrome (hypercortisolism)
• Moon facies, truncal obesity, buffalo hump
o Conn syndrome (hyperaldosteronism)
• Hypertension & hypokalemia
• Lab data: 1 Levels of ACTH, cortisol, aldosterone
• Diagnosis: Clinical, biochemical, imaging, histology
Demographics
• Age
o Adults (70-80%); children (15-20%)
• Cushing syndrome: 25-40 years
• Conn syndrome: 30-50 years
• Gender: Cushing syndrome (M:F = 1:5); Conn
syndrome (M:F = 1:2)
Natural History & Prognosis
• Complications: Untreated adrenal crisis ~ death
• Prognosis: Usually good with treatment
Treatment
• Adrenal hyperplasia without symptoms: Follow-up
• Adrenal hyperplasia with symptoms: Surgical resection
of pituitary adenoma or source of hormone
I DIAGNOSTIC CHECKLIST
Consider
• Correlate clinical, biochemical & imaging findings
Image Interpretation Pearls
• Enlarged limbs of adrenal glands (> 10 mm) on CT
• Multinodular hyperplasia difficult to distinguish from
adenoma by CT
I SELECTED REFERENCES
1. Rockall AG et al: CT and MR imaging of the adrenal glands
in ACTH-independent cushing syndrome. Radiographies.
24(2):435-52, 2004
2. Imaki T et al: Adrenocortical hyperplasia associated with
ACTH-dependent Cushing's syndrome: comparison of the
size of adrenal glands with clinical and endocrinological
data. Endocr]. 51(1):89-95, 2004
3. Caoili EM et al: Adrenal masses: characterization with
combined unenhanced and delayed enhanced CT.
Radiology. 222(3):629-33, 2002
4. Mayo-Smith WW et al: State-of-the-art adrenal imaging.
Radiographics. 21(4):995-1012, 2001
5. Doppman JL et al: Adrenocorticotropin-independent
macronodular adrenal hyperplasia: an uncommon cause of
primary adrenal hypercortisolism. Radiology.
216(3):797-802, 2000
6. Sohaib SA et al: CT appearance of the adrenal glands in
adrenocorticotrophic hormone-dependent Cushing's
syndrome. AJRAmJ Roentgenol. 172(4):997-1002, 1999
7. Kawashima A et al: Spectrum of CT findings in
nonmalignant disease of the adrenal gland. Radiographies.
18(2):393-412, 1998
8. Krebs TL et al: MR imaging of the adrenal gland:
radiologic-pathologic correlation. Radiographies.
18(6):1425-40, 1998
9. Doppman JL et al: Cushing syndrome due to primary
pigmented nodular adrenocortical disease: findings at CT
and MR imaging. Radiology. 172(2):415-20, 1989
 ADRENAL HYPERPLASIA
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
multiple small nodules in
both adrenal glands, one
manifestation of adrenal
2
hyperplasia. (Right) Axial
CECT shows multiple small 15
nodules in both adrenal
glands, one manifestation of
adrenal hyperplasia.

(Left) Axial CECT shows

prominent adrenal glands
without obvious hypertrophy
or nodularity. (Right) Axial
CECT shows prominent
adrenal glands without
obvious hypertrophy or
nodularity.

(Left) Axial T2WI MR shows

prominent adrenal glands.
(Right) Coronal T2WI MR
shows microadenoma
(arrow) in pituitary; one
potential manifestation of
excess ACTH production
and adrenal hyperplasia.
 ADRENAL INSUFFICIENCY

2
16

Axial CECT shows bilateral adrenal hemorrhage Axial NECT shows splenic and adrenal calcifications
following shock due to motor vehicle crash + pelvic indicating prior fungal or tuberculous infection.
fractures.

o Small, atrophic glands (autoimmune)

ITERMINOlOGY o Dense, chunky calcifications (infection)
Abbreviations and Synonyms Imaging Recommendations
• Primary adrenal insufficiency, Addison disease • Best imaging tool: CT
Definitions
• Inadequate secretion of corticosteroids resulting from
partial or complete destruction of adrenal glands I DIFFERENTIAL DIAGNOSIS
Adrenal metastases and lymphoma
• Metastases
I IMAGING FINDINGS o Unilateral or bilateral, invasive, enhancing masses
General Features o Central necrosis ± hemorrhage
o Focal masses without diffuse enlargement
• Location: Bilateral
o May not cause adrenal insufficiency
• Other general features
• Lymphoma
o Imaging findings depend on course
• Acute, subacute « 2 years), chronic o Usually bilateral, triangular shaped masses
o Rarely causes adrenal insufficiency
Radiographic Findings .
Adrenal hemorrhage
• Radiography: Bilateral calcifications above the kIdneys
• Heterogenous enhancing mass (40-80 HU)
CT Findings
Adrenal infection
• Acute: Addisonian crisis, adrenal apoplexy
o Enlarged adrenal glands • Unilateral or bilateral enlarged adrenal glands
o Heterogenous enhancement (hemorrhage) Adrenal adenoma
• Subacute • May be bilateral
o Enlarged glands with normal adrenal contours
o Reduced density due to caseation or necrosis
• Chronic

DDx: Adrenal Masses or Atrophy

Lymphoma Metastases Hyperplasia

 ADRENAL INSUFFICIENCY

Key Facts
Imaging Findings Top Differential Diagnoses
• Location: Bilateral • Adrenal metastases and lymphoma
• Imaging findings depend on course • Adrenal hemorrhage
• Heterogenous enhancement (hemorrhage) • Adrenal infection
• Enlarged glands with normal adrenal contours
• Small, atrophic glands (autoimmune) Diagnostic Checklist 2
• Dense, chunky calcifications (infection) • Aggressive diagnosis & treatment to avoid
complications 17
• Best imaging tool: CT

!PATHOlOGY Natural History & Prognosis

• Complications: Acute: Shock, death
General Features
• Prognosis: Good, if recognized and treated
• Etiology
o Idiopathic autoimmune disorders (80% of cases) Treatment
o Granulomatous diseases: Tuberculosis (most • Acute
common cause in underdeveloped nations), o Glucocorticoid therapy
sarcoidosis o Volume and electrolytes replacement
o Systemic fungal infections: Histoplasmosis (most o Correct any etiology (e.g., antibiotics for infections)
common infection in southeastern, south central • Chronic
U.S.), cryptococcosis, blastomycosis o Glucocorticoid + mineralocortoid replacement
o Adrenal hemorrhage, necrosis or thrombosis: Stress
after surgery, shock, anticoagulation (e.g., warfarin),
sepsis (e.g., Waterhouse-Friderichsen syndrome), I DIAGNOSTIC CHECKLIST
coagulation disorders, antiphospholipid syndrome
o Neoplasms: Metastases (e.g., lung, ovary, breast, Consider
kidney), lymphoma, leukemia • Aggressive diagnosis & treatment to avoid
o Acquired immune deficiency syndrome (AIDS): complications
Opportunistic infections (e.g., cytomegalovirus),
neoplasms (e.g., Kaposi sarcoma) Image Interpretation Pearls
o Other causes: Adrenomyeloneuropathy, familial • Can not diagnose adrenal hyper- or hypofunction by
glucocorticoid deficiency, amyloidosis imaging alone
• Epidemiology: 50 per 1,000,000 persons
Gross Pathologic & Surgical Features I SELECTED REFERENCES
• Enlarged glands with hemorrhagic and necrotic tissues 1. Kawashima A et al: Imaging of non traumatic hemorrhage
• Small, atrophic glands or dense, chunky calcifications of the adrenal gland. Radiographies. 19(4):949-63, 1999
2. Kawashima A et al: Spectrum of CT findings in
Microscopic Features
nonmalignant disease of the adrenal gland. Radiographies.
• Hemorrhagic destruction; infectious organisms 18(2):393-412, 1998
• Lymphocytic, granulomatous or neoplastic infiltration 3. Doppman JL et al: CT findings in Addison's disease. J
Comput Assist Tomogr. 6(4):757-61, 1982

ICLINICAllSSUES
Presentation
I IMAGE GALLERY
• Most common signs/symptoms
o Acute: Fever, abdominal or back pain, hypotension,
weakness, nausea, vomiting, diarrhea ....
·;········
. .
....
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o Chronic: Progressive lethargy, weakness, cutaneous ~ '-~_
•...
f>
"",.' "
pigmentation, weight loss :
-.;,,';-
--.•. , .
• Lab data ....• ~
o Chemistry: Hyponatremia, hyperkalemia, azotemia,
hypercalcemia, hypoglycemia
o Adrenocorticotrophic hormone (ACTH) stimulation
test: Cortisol level fail to rise
o 24-hour urine cortisol: Decreased
Demographics
• Gender: M:F = 1:1; autoimmune: 1:2-3
~
~
;;

- .'
~
~ •
.
- ;

(Left) Axial CECT shows massive adrenal metastases from lung cancer
that resulted in adrenal insufficiency. (Right) Axial CECT shows
massive adrenal metastases from malignant melanoma.
 ADRENAL CYST

2
18

Sagittal sonogram shows sonolucent mass above right Axial T2WI MR shows high intensity left adrenal cyst
kidney; adrenal cyst. (arrow).

• Punctate within intracystic hemorrhage (5%)

ITERMINOlOGY • CECT: No central enhancement ± wall enhancement
Definitions MR Findings
• A cystic mass arising in adrenal gland
• T1WI
o Homogeneous, hypointense mass
o Hyperintense mass (hemorrhage)
I IMAGING FINDINGS • T2WI: Hyperintense mass
General Features Imaging Recommendations
• Best diagnostic clue: Well-defined nonenhancing • Best imaging tool: CT or MR
water-density adrenal mass ± calcifications
• Location
o Suprarenal I DIFFERENTIAL DIAGNOSIS
o Right as common as left
o Unilateral> bilateral (8-10% of cases) Adrenal adenoma
• Size: < 5 cm (50%), up to 20 cm • NECT: Homogeneous, well-defined, ovoid mass with :s;
30 HU density
CT Findings • CECT: Enhancing mass without visible wall or
• NECT peripheral calcifications
o Unilocular or multilocular mass
o Well-defined, round to oval, homogeneous mass Gastric diverticulum
with water (0 HU) or near water density • Abnormal rounded soft tissue shadow, often lies in
o Higher or mixed attenuation mass (hemorrhage, suprarenal location
intracystic debris, crystals) • Air-filled, fluid-filled or contrast-filled mass with no
o 1 Wall thickness, up to 3 mm enhancement of contents
o Calcifications
• Rim-like or nodular (51-69%) Adrenal myelolipoma
• Centrally in intracystic septation (19%) • Usually has foci of fat density

.....
r; :
t.:·.
DDx: "Cystic"- Appearing

~ ~
•
...•...
• t"

'
i;.

.-.,
~~.
Suprarenal Mass

....
. . .

..' L...
- ' .'
."" '

' .
"

.
' ~ ,
,;7 .fI'"

Adenoma Gastric Diverticulum Myelolipoma Metastases

 ADRENAL CYST

Key Facts
Imaging Findings • Adrenal myelolipoma
• Best diagnostic clue: Well-defined nonenhancing • Necrotic adrenal tumor
water-density adrenal mass ± calcifications Clinical Issues
• Unilocular or multilocular mass
• Asymptomatic
• t Wall thickness, up to 3 mm

Top Differential Diagnoses

Diagnostic Checklist 2
• Complicated cyst has t malignant potential
• Adrenal adenoma 19
• Complicated cyst has high attenuation, thick
• Gastric diverticulum
enhancing wall and/or septations

o Cyst with high malignant potential, > 5 cm

Necrotic adrenal tumor
o Patients with symptoms, endocrine abnormalities,
• Primary or metastatic complications

I PATHOLOGY I DIAGNOSTIC CHECKLIST

General Features Consider
• Etiology • Complicated cyst has t malignant potential
o Endothelial lining (45-48%)
• Lymphangioma Image Interpretation Pearls
• Hemangioma • Complicated cyst has high attenuation, thick
o Pseudocyst (39-42%) enhancing wall and/or septations
• Prior hemorrhage (e.g., vascular neoplasm,
primary adrenal mass) or infarction
• Cystic degeneration I SELECTED REFERENCES
o Epithelial lining: True cyst (9-10%)
1. Otal P et al: Imaging features of uncommon adrenal masses
• Glandular or retention cyst with histopathologic correlation. Radiographies.
• Embryonal cyst 19(3):569-81, 1999
• Cystic adenoma 2. Neri LM et al: Management of adrenal cysts. Am Surg.
• Mesothelial inclusion cyst 65(2):151-63, 1999
o Parasitic cyst (7%) 3. Kawashima A et al: Imaging of nontraumatic hemorrhage
• Hydatid or echinococcal cyst of the adrenal gland. Radiographies. 19(4):949-63, 1999
4. Kawashima A et al: Spectrum of CT findings in
• Epidemiology: 1-2 per 10,000 persons
nonmalignant disease of the adrenal gland. Radiographies.
18(2):393-412, 1998
5. Rozenblit A et al: Cystic adrenal lesions: CT features.
I CLINICAL ISSUES Radiology. 201(2):541-8, 1996
6. Tung GA et al: Adrenal cysts: imaging and percutaneous
Presentation aspiration. Radiology. 173(1):107-10, 1989
• Most common signs/symptoms 7. Johnson CD et al: CT demonstration of an adrenal
o Asymptomatic pseudocyst. J Comput Assist Tomogr. 9(4):817-9, 1985
o Abdominal pain, gastrointestinal symptoms,
palpable mass
• Diagnosis I IMAGE GALLERY
o Usually found incidentally on imaging
..•.
Demographics '\..

• Age: Any age; 20-50 years of age most common

• Gender: M:F = 1:3
Natural History & Prognosis
• Complications
~...
,
.,..,,"
\.
,

o Hypertension, infection, rupture, hemorrhage

• Prognosis
o Good r",~.
;_
_¥ \T'll_
'\l.J
JI
Treatment
• Fine-needle aspiration
o Cyst with low malignant potential (Left) Axial CECT shows nonenhancing water density right adrenal
• Surgical resection cyst. (Right) Sagittal reformation of CECT shows nonenhancing right
adrenal cyst (arrow).
o Usually by laparoscopic approach
 ADRENAL ADENOMA

.... ' .~~

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~

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Graphic shows small lipid-rich mass within the adrenal Surgical photograph of resected adenoma shows a 2 cm
gland. lipid-rich nodule (arrows) within the adrenal gland.

o Primary hyperaldosteronism (Conn syndrome)

ITERMINOLOGY
Definitions
• Benign tumor of adrenal gland cell origin
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Well-circumscribed, low density,
small adrenal mass on CT
• Size
o Adenoma of Cushing syndrome: Varies from 2-5 cm
o Adenoma of Conn syndrome: < 2 cm (20% < 1 cm)
• Morphology: Usually round to oval suprarenal mass
• Key concepts " o Lipid poor adrenal adenoma (30% of cases)
o Most common adrenal cortex tumor (10% bilateral)
• Accounts for 90% of all "incidentalomas"
o May occur in up to 9% of general population
o Lipid rich adrenal adenoma: 70% of all adenomas
o Lipid poor adrenal adenoma: 30% of adenomas
o t Incidence in patients with diabetes & HTN
oCT: Study of choice to diagnose adrenal
incidentalomas
o Classified into two types based on function
• Nonhyperfunctioning: Normal hormone levels
• Hyperfunctioning: Primary hyperaldosteronism;
Cushing syndrome; hyperandrogenism
• 80% of cases are due to adrenal adenoma
• 20% of cases are due to adrenal hyperplasia
• Adenomas are often small & difficult to detect
o Cushing syndrome
• 15-25% of cases are due to adrenal adenoma
• 80-85% of cases are due to adrenal hyperplasia
• Adenomas are usually greater than 2.0 cm
CT Findings
• NECT
o Smooth, well-defined, round or oval in shape
o Homogeneous soft tissue mass of 0-20 HU
o Lipid rich adrenal adenoma (70% of cases)
• Low-attenuation (less than 10 HU)
• Characteristic & diagnostic of adenoma
• Attenuation varies from 10-30 HU
• Difficult to differentiate from metastases on NECT
o Cushing syndrome due to adrenal adenoma
• Remainder of ipsilateral gland & contralateral
adrenal gland are atrophic due to I ACTH levels
• t Cortisol: Feedback inhibition on pituitary ACTH
o Conn syndrome due to adrenal adenoma
• Remainder of ipsilateral gland & contralateral
adrenal gland appear normal
o Large adenomas
• More heterogeneous than small adenomas
• ± Hemorrhage, cystic degeneration, calcification

DDx: Adrenal Mass

Metastatic Melanoma Adrenal Carcinoma Pheochromocytoma Gastric Diverticulum

 ADRENAL ADENOMA
Key Facts
Imaging Findings
• Best diagnostic clue: Well-circumscribed, low density,
small adrenal mass on CT
• Smooth, well-defined, round or oval in shape
• Homogeneous soft tissue mass of 0-20 HU
• Enhancing adrenal mass that "de-enhances" rapidly
• Washout of adenoma: 10 min. post injection> 50%
• Enhanced-delayed x 100/enhanced-unenhanced
• T1WI out of phase: 1 Signal "drop-out" (lipid-rich)
• Adrenal adenoma: No increased uptake of FDG
• Adrenal adenoma: Unilateral early adrenal
visualization before day 5 after NP-59 injection
• Washout value of> 50%: Sensitivity (96%), specificity
(near 100%) for adrenal adenoma
• Washout value of < 50%: Indicative of either
metastases or an atypical adenoma
• CECT
o Enhancing adrenal mass that "de-enhances" rapidly
• Enhanced phase: Attenuation varies, 40-50 HU
• 10 min. delayed phase: Attenuation I 20-25 HU
o Washout of adenoma: 10 min. post injection> 50%
• Enhanced-delayed x 100/enhanced-unenhanced
o Washout pattern is diagnostic for adenoma
• No follow-up is required if seen
o Lipid poor adenoma have similar washout pattern to
lipid rich adenoma
MR Findings
• TlWI &T2WI
o Varied signal intensity
• Lipid rich adrenal adenoma
o Tl WI out of phase: 1 Signal "drop-out" (lipid-rich)
o Tl WI in phase: Hyperintense
• T1 C+
o Early phase: Adenoma shows enhancement
o Delayed phase: > 50% washout is seen
Ultrasonographic Findings
• Real Time
o May show a mass lesion in suprarenal area
• Right suprarenal mass seen more clearly than left
side due to acoustic window provided by liver
Angiographic Findings
• Conventional
o Adrenal arteriography
• Catheterization of renal or inferior adrenal arteries
show vascular supply of adrenal tumors
• Adenomas are usually hypovascular to moderately
vascular
• No arterial encasement, venous laking or puddling
which are of malignant vascular features
o Adrenal venography
• Most commonly to obtain adrenal vein samples
• Adrenal adenoma is seen as a filling defect within
adrenal gland displacing adjacent vessels
• Circumferential vein frequently seen around
adrenal adenoma
Top Differential Diagnoses
• Adrenal metastases & lymphoma
• Adrenal carcinoma
• Pheochromocytoma
•
•
Adrenal hyperplasia
Gastric diverticulum 2
Clinical Issues 21
• Asymptomatic incidental CT imaging finding
• Conn syndrome: Hypertension & weakness
• Cushing syndrome: Moon facies, truncal obesity,
purple striae & buffalo hump
• Diagnosis: Clinical, biochemical, imaging, histology
Diagnostic Checklist
• Asymptomatic mass: Usually a non-hyperfunctioning
adenoma, even in a patient with a known cancer
Nuclear Medicine Findings
• FDG-PET
o Adrenal adenoma: No increased uptake of FDG
• Differentiates from malignant lesion (1 uptake)
• Adrenocortical scintigraphy by using NP-59
o NP-59 is a cholesterol analog that binds to
low-density lipoprotein receptors of adrenal cortex
o NP-59 used + dexamethasone: Accentuate uptake in
non-ACTH-dependent adrenal tissues (adenoma)
o Normal NP-59: When both adrenal glands are seen
on day 5 after injection or thereafter
o Adrenal adenoma: Unilateral early adrenal
visualization before day 5 after NP-59 injection
o Adrenal hyperplasia: Bilateral early adrenal
visualization before day 5 after NP-59 injection
Imaging Recommendations
• CT is study of choice to confirm the diagnosis of
adrenal adenoma
o CT technique: 3 mm thick section at 3 mm intervals
• If suspect adrenal adenoma, NECT alone sufficient
o Attenuation value < 10 HU is diagnostic
• If CECT done, assess the following
o If lesion < 37 HU on CECT, call it adenoma
o If lesion> 37 HU, on CECT, get 10 min delayed scan
to determine washout
o Washout value of> 50%: Sensitivity (96%),
specificity (near 100%) for adrenal adenoma
o Washout value of < 50%: Indicative of either
metastases or an atypical adenoma
• MR with in and out of phase imaging
o Diagnostic for lipid-rich adenomas
I DIFFERENTIAL DIAGNOSIS
Adrenal metastases & lymphoma
• Adrenal metastases
o Unilateral or bilateral masses ± central necrosis,
hemorrhage
o Usually known to have malignancy elsewhere
o NECT: Metastases mimic lipid poor adenoma
 ADRENAL/ADENOMA
o CECT: Hypervascular & prolonged washout pattern
• Adrenal lymphoma
o Usually spread to adrenal gland of retroperitoneal
o Unilateral primary lymphoma (non-Hodgkin) can
mimic adenoma
o Hypovascular; moderate enhancement with contrast
2 Adrenal carcinoma
• Rare, unilateral invasive & enhancing mass
22 • More than 6 cm when initially diagnosed
Pheochromocytoma
• Tumor> 3 cm in most cases, T2WI very hyperintense
• Highly vascular tumor prone to hemorrhage, necrosis
• Bilateral adrenal tumors in MEN syndromes
Adrenal hyperplasia
• Adrenal glands are often symmetrically enlarged
• Width of adrenal gland limbs> 10 mm (diagnostic)
• No discrete mass or nodule seen as a rule
• Dominant macronodule of macronodular hyperplasia
mimic small adrenal adenoma
o Cortisol-secreting adenoma: Remainder of ipsilateral
& contralateral glands, atrophic (t ACTH)
o Macronodular hyperplasia: Both glands are enlarged
(due to elevated ACTH levels)
• No obvious enhancement & washout pattern seen
Gastric diverticulum
• Abnormal rounded soft tissue shadow in left
suprarenal area, mimics adrenal mass
• Diverticular contents do not enhance; adenomas do
Adrenal myelolipoma
• Small or large, asymptomatic adrenal mass
• Intramural fatty elements recognized on imaging
Ganglioneuroma
• Younger patients, mean age = 27 Y
• Larger mass with average tumor size of 8 cm
Unilateral adrenal hemorrhage
• Chronic hematoma: Well-defined, round, low density,
mass-like lesion simulating adenoma
!PATHOLOGY
General Features
• General path comments
o Most adrenals with adenoma: Normal function
o Occasionally adenoma causes adrenal hyperfunction
o Normal adrenocortical secretory hormones
• Adrenal cortex: Cortisol, aldosterone, androgens
• Etiology: Exact etiology unknown
• Epidemiology
o Most common adrenal tumor of all incidentalomas
o 1 Incidence in patients with diabetes or HTN
o Occur in up to 9% of population (postmortem data)
• Associated abnormalities: Multiple endocrine
neoplasia syndromes (MEN)
Gross Pathologic & Surgical Features
• Well-delineated, tan-yellow, ovoid mass
Microscopic Features
• 70% of adenomas: High % of intracytoplasmic lipid
• 30% of adenomas: Less % of intracytoplasmic lipid
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic incidental CT imaging finding
o Conn syndrome: Hypertension & weakness
o Cushing syndrome: Moon facies, truncal obesity,
purple striae & buffalo hump
o Virilization in women
o Lab data: 1 Aldosterone, cortisol & androgens
o Diagnosis: Clinical, biochemical, imaging, histology
Demographics
• Age
o Prevalence of adenoma increases with age
• Peak at 60-69 y and decreasing thereafter
Natural History & Prognosis
• Prognosis: Excellent when incidental & non secretory
Treatment
• No treatment when asymptomatic incidental finding
• Laparoscopic removal of gland if hyperfunctioning
I DIAGNOSTIC CHECKLIST
Consider
• Asymptomatic mass: Usually a non-hyperfunctioning
adenoma, even in a patient with a known cancer
Image Interpretation Pearls
• Well-defined, low density « 10 HU) suprarenal mass
• Enhances + wash out pattern of> 50% within 10 min.
• T1W out of phase: 1 Signal"drop-out" lipid-rich mass
I SELECTED REFERENCES
1. Kebapci M et al: Differentiation of adrenal adenomas (lipid
rich and lipid poor) from non adenomas by use of washout
characteristics on delayed enhanced CT. Abdom Imaging.
28(5):709-15, 2003
2. Caoili EM et al: Adrenal masses: characterization with
combined un enhanced and delayed enhanced CT.
Radiology. 222(3):629-33, 2002
3. Dunnick NR et al: Imaging of adrenal incidentalomas:
Current status. A]R. 179:559-68,2002
4. Mayo-Smith WW et al: State-of-the-art adrenal imaging.
Radiographies. 21(4):995-1012, 2001
5. Pena CS et al: Characterization of indeterminate
(lipid-poor) adrenal masses: use of washout characteristics
at contrast-enhanced CT. Radiology. 217(3):798-802, 2000
6. Caoili EM et al: Delayed enhanced CT of lipid-poor adrenal
adenomas. A]RAm] Roentgenol. 175(5):1411-5,2000
7. Kawashima A et al: Spectrum of CT findings in
nonmalignant disease of the adrenal gland. Radiographies.
18(2):393-412, 1998
8. Korobkin M et al: Adrenal adenomas: relationship between
histologic lipid and CT and MR findings. Radiology.
200(3):743-7, 1996
 ADRENAL ADENOMA

I IMAGE GALLERY
(Left) Axial TlWI MR in
phase GRE sequence shows
bilateral small adrenal
masses (arrows). (Right)
2
Axial Tl WI MR opposed
phase GRE sequence shows 23
loss of signal in both
adenomas, confirming
presence of lipid.

(Left) Axial NECT shows

homogeneous low density
right adrenal adenoma
(arrow), normal left adrenal.
(Right) Axial NECT shows
homogeneous low density
adenoma that is larger than
typical and which had grown
slowly over 10 years.

Typical
(Left) Axial NECT shows an
indeterminate left adrenal
mass, proved to be a
lipid-poor adenoma. (Right)
Axial CECT in parenchymal +
delayed phases shows
significant enhancement and
rapid washout. Absolute
washout is 69%, relative is
42%, indicating lipid-poor
adenoma.
 ADRENAL MYELOLIPOMA

2
24

Axial CECT shows heterogeneous, predominantly fatty, Axial CECT shows predominantly fatty left adrenal mass.
right adrenal mass with calcified foci.

• Presence of pure fat within tumor is diagnostic

ITERMINOlOGY
Definitions
• Rare benign tumor composed of mature fat tissue &
hematopoietic elements (myeloid & erythroid cells)
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Heterogeneous fatty adrenal mass
• Location: Suprarenal
• Size: Usually 2-10 cm, rarely 10-20 cm
• Key concepts
o Rare, benign neoplasm of adrenal gland
o Seen in 0.2-0.4% of cases based on autopsy series
o Frequency among all incidentalomas, 7-15%
o Incidental finding on CT in older people
o Typically unilateral & very rarely bilateral
o Large tumor can bleed spontaneously or necrose
o Nonfunctioning (do not secrete any hormones)
o Large myelolipoma can mimic retroperitoneal
lipoma or liposarcoma
• Usually well-defined mass with recognizable capsule
• Punctate calcifications seen (20% of cases)
MR Findings
• MR appearance depends on histologic composition
o Tumor with major fat component
• T1WI in phase: Typically hyperintense
• Fat suppression sequences: Loss of signal
o Bone marrow elements (myeloid & erythroid cells)
• Low signal on T1WI; moderate signal on T2WI
Ultrasonographic Findings
• Real Time
o Well-defined, echogenic mass (I fat tissue)
o Heterogeneous mass (I myeloid cells)
Angiographic Findings
• Conventional: Differentiate myelolipoma from
retroperitoneal liposarcoma by determining origin of
blood supply & vascularity of tumors
Imaging Recommendations
• Helical NECT or MR with fat suppression sequence

CT Findings
• CT appearance depends on histologic composition I DIFFERENTIAL DIAGNOSIS
o Most tumors are heterogeneous fatty adrenal masses
• Low-attenuation of fat density (-30 to -90 HU) Adrenal adenoma
• Lipid rich adenoma: I Attenuation (less than 10 HU)

DDx: Adrenal Mass

Adenoma Metastases Metastases Liposarcoma

 ADRENAL MYELOLIPOMA

Key Facts
Terminology Top Differential Diagnoses
• Rare benign tumor composed of mature fat tissue & • Adrenal adenoma
hematopoietic elements (myeloid & erythroid cells) • Adrenal metastases & lymphoma
• Pheochromocytoma
Imaging Findings
• Most tumors are heterogeneous fatty adrenal masses Diagnostic Checklist 2
• Low-attenuation of fat density (-30 to -90 HU) • Differentiate from other tumors (lipid-rich adenoma)
• T1WI in phase: Typically hyperintense • Key is presence of tumoral fat & benign nature, avoid 25
• Fat suppression sequences: Loss of signal further workup for incidental mass

• No true fat density, unlike adrenal myelolipoma

• CECT: Wash out 10 min. post injection> 50% . Demographics
• Age: Usually elderly age group: 50-70 years
Adrenal metastases & lymphoma
• Adrenal metastases Natural History & Prognosis
o Invasive, enhancing mass in adrenal gland • Complication: Rupture with hemorrhage (rare)
o Higher density on CT than myelolipoma • Prognosis: Excellent
• Adrenal lymphoma Treatment
o Primary (rare); secondary (non-Hodgkin common)
• When diagnosis is certain, surgery not needed
o Often bilateral; retroperitoneal disease usually seen
o Discrete or diffuse mass, shape is maintained
Liposarcoma I DIAGNOSTIC CHECKLIST
• Retroperitoneal primary sarcoma involving perirenal
space may simulate adrenal (or renal) fatty tumor
Consider
• Differentiate from other tumors (lipid-rich adenoma)
Pheochromocytoma • Key is presence of tumoral fat & benign nature, avoid
• Highly vascular; prone to hemorrhage & necrosis further workup for incidental mass
• Hyperintense on T2WI; bilateral in MEN syndromes
Image Interpretation Pearls
Adrenal carcinoma • Well-defined heterogeneous fat density tumor on CT
• Rare, unilateral, invasive & enhancing mass • T1WI hyperintense, signal loss with fat suppression

I PATHOLOGY I SELECTED REFERENCES

1. Dunniek NR et al: Imaging of adrenal incidentalomas:
General Features
current status. AJRAm J Roentgenol. 179(3): 559-68, 2002
• Etiology 2. Mayo-Smith WW et al: State-of-the-Art adrenal imaging.
o Unknown Radiographies. 21(4): 995-1012, 2001
o Best hypothesis: Reticuloendothelial cell metaplasia 3. Rao P et al: Imaging and pathologic features of
of capillaries in adrenal (stress/infection/necrosis) myelolipoma. Radiographies. 17: 1373-85, 1997
o Secondary hypothesis: Myelolipoma represents a site 4. Cyran KM et al: Adrenal myelolipoma. AJR 166: 395-400,
of extramedullary hematopoiesis 1996
• Epidemiology: Autopsy incidence 0.2-0.4%
• Associated abnormalities: Adrenal collision tumors
[IMAGE GALLERY
Gross Pathologic & Surgical Features
• Cut section: Fat & soft tissue components
Microscopic Features
• Mature fat cells & megakaryocytes; no malignant cells

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Usually an incidental finding on CT, MR or US
o "Acute abdomen": Rupture with hemorrhage (rare) (Left) Chemical shift (opposed phase) axial MR shows heterogeneous
• Diagnosis: CT or MR; biopsy prone to sampling error right adrenal mass with signal 1055at fat/50ft tissue interfaces. (Right)
Axial CECT shows a large heterogeneous fatty myelolipoma in right
adrenal (and an adenoma in left gland).
 PHEOCHROMOCYTOMA

2
26

Graphic shows heterogeneous hypervascular adrenal Axial CECT shows 5 cm heterogeneous hyper vascular
pheochromocytoma. left adrenal pheochromocytoma (arrow).

o 10% bilateral, malignant & extra-abdominal

ITERMINOLOGY
Abbreviations and Synonyms
• Paraganglioma or ganglioneuroma (extra-adrenal)
Definitions
• Tumor arising from chromaffin cells of adrenal
medulla or extra-adrenal ectopic tissue
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Very hyperintense 3-5 cm mass
on T2WI with bright heterogeneous enhancement
• Location
o Along sympathetic chain: Neck to urinary bladder
o Subdiaphragmatic (98%); thorax (1-2%)
• Adrenal medulla (90%); extra-adrenal (10%)
• Size
o Usually more than 3 cm in most cases
o Weight ranging from 1 gm to over 4 kg
• Morphology
o Well-circumscribed, encapsulated tumor
• Solitary (sporadic); multiple (familial)
• Key concepts
o Also called tumor with "rule of 10s" or 10% tumors
o 10% extra-adrenal: Paragangliomas/chemodectomas
o 10% familial, pediatric, silent
o 10% have autosomal dominant transmission &
associated with various other dominant conditions
o Extra-adrenal tumors arise from sympathetic ganglia
• Neck, mediastinum, pelvis or urinary bladder
• Aortic bifurcation (organ of Zuckerkandl): Ganglia
at origin of inferior mesenteric artery
o 90% patients present with HTN secondary to release
of catecholamines
o Term pheochromocytoma refers to dusky color
• Tumor "stains" this color when treated with
chromium salts
o Imaging: Difficult to distinguish benign, malignant
• Benign lesions can be locally invasive of IVC &
renal capsule
• Distant metastases indicate malignancy
CT Findings
• NECT
o Well-defined, round, homogeneous (muscle density)
o ± Areas of t density (hemorrhage)
o ± Areas of! density (cystic, necrotic, septate)
o ± Areas of curvilinear or mural calcification
• CECT
o Shows marked homogeneous enhancement
o Heterogeneous enhancement
• Due to tissue necrosis & hemorrhage
o Peripheral enhancement with fluid-levels

DDx: Adrenal Mass

....
.'.• 0_0"
"'" t

'f;.}'
Adenoma with Bleed Adenoma with Bleed Adrenal Metastasis
-G
Myelolop. + Adenoma
 PHEOCHROMOCYTOMA
Key Facts
Terminology
• Paraganglioma or ganglioneuroma (extra-adrenal)
• Tumor arising from chromaffin cells of adrenal
medulla or extra-adrenal ectopic tissue
Imaging Findings
• Best diagnostic clue: Very hyperintense 3-5 cm mass
on T2WI with bright heterogeneous enhancement
• Adrenal medulla (90%); extra-adrenal (10%)
• 10% bilateral, malignant & extra-abdominal
• Well-defined, round, homogeneous (muscle density)
• ± Areas of t density (hemorrhage)
• ± Areas of I density (cystic, necrotic, septate)
• ± Areas of curvilinear or mural calcification
• Markedly hyperintense on T2WI (characteristic)
• After 24-72 hrs.: t Uptake of 1-131 MIBG in tumor
MR Findings
• TlWI
o Isointense to muscle & hypointense to liver
o Heterogeneous signal intensity
• Due to areas of hemorrhage & necrosis
o ± Areas of increased signal intensity
• Due to acute or subacute hemorrhage
• T2WI
o Markedly hyperintense on T2WI (characteristic)
o Long T2 relaxation time
• Due to t water content as a result of necrosis
o Heterogeneous signal intensity (in 33% of cases)
• Due to hemorrhage & necrosis with fluid levels
• Tl C+
o Characteristic salt & pepper pattern (due to
increased tumor vascularity)
• Salt: Represents enhancing parenchyma
• Pepper: Represents flow void of vessels
o Can show marked early as well as prolonged
contrast -enhancement
Ultrasonographic Findings
• Real Time
o Iso-/hypoechoic (77%) & hyperechoic (23%) in
contrast to normal renal parenchyma
o Round & well-circumscribed mass
Angiographic Findings
• Conventional: Hypervascular tumor
Nuclear Medicine Findings
• 1-131 or 123 Metaiodobenzylguanidine (MIBG)
o After 24-72 hrs.: t Uptake of 1-131 MIBG in tumor
o Particularly useful for detecting extra-adrenal tumors
• Metastatic disease in malignant condition
• Recurrent & extra-abdominal tumors
o Sensitivity (80-90%); specificity (90-100%)
Imaging Recommendations
• Helical NE + CECT
o Hypertensive crisis is rare or nonexistent with LV.
administration of nonionic contrast material
Top Differential Diagnoses
• Adrenal adenoma
• Adrenocortical carcinoma
• Adrenal metastases & lymphoma
•
•
Adrenal myelolipoma (myelolop.)
Adrenal hemorrhage
2
• Granulomatous infection
27
Clinical Issues
• Clinical profile: A young patient with paroxysmal
attacks of headache, palpitations, sweating & tremors
• t Levels of vanillylmandelic acid (VMA) 24-hr. urine
Diagnostic Checklist
• Imaging findings + history & labs (usually diagnostic)
• Spherical suprarenal mass, 3-5 cm, very hyperintense
on T2WI MR & brightly enhancing with contrast
• Routine premedication «(){ and ~ blockade) is not
recommended by most authorities
• MR &Tl C+
• MIBG: For ectopic, recurrent & metastatic tumors
I DIFFERENTIAL DIAGNOSIS
Adrenal adenoma
• Most common benign tumor of adrenal gland (cortex)
• Histopathologically: Rich in lipid
• Imaging
o NECT
• Well-defined mass of < 10 HU (lipid rich)
• Well-defined mass of> 10 HU (lipid poor)
o CECT: Enhancing mass that "de-enhances" rapidly
• Washout adenoma: 10 min. post injection (> 50%)
• Washout pattern is diagnostic for adenoma
o MR: Signal suppression on out of phase Tl WI
• Hyperfunctioning: Clinical symptoms, signs; lab data
Adrenocortical carcinoma
• Rare; usually unilateral; rarely bilateral (up to 10%)
• Functioning tumors (small); nonfunctioning (large)
• Imaging
o Large, unilateral adrenal mass with invasive margins
o Large solid mass, areas of necrosis, hemorrhage
o ± Calcification (30% cases); variable enhancement
o Tl WI hypointensei T2WI hyperintense
o Local spread: Renal vein or IVC extension
o Metastatic tumor spread: Lungs, liver, nodes & bone
• Diagnosis: Biopsy & histology
Adrenal metastases & lymphoma
• Adrenal metastases
o E.g., lung, breast, renal cell carcinoma & melanoma
o Unilateral or bilateral; central necrosis ± hemorrhage
o History: Patient usually known to have malignancy
o Lung cancer
• Adrenal metastases (35-38% of cases); usually solid
o Breast carcinoma: Adrenal metastases (50% of cases)
o Renal cell carcinoma (RCe)
• Adrenal metastases (seen in 18-25% of cases)
 PHEOCHROMOCYTOMA
• Usually ipsilateral & hypervascular • ± Cystic, mucoid, serosanguineous, hemorrhage
o Malignant melanoma
• Adrenal metastases (50% cases); usually bilateral Microscopic Features
• CT: Solid or cystic; rim calcification may be seen • Large cells: Granular cytoplasm & pleomorphic nuclei
• MR: Hyperintense on T1WI (melanin pigment) • Chromaffin reaction: Cells stained with chromium salt
• Adrenal lymphoma
o 25% cases of secondary lymphoma; primary (rare)
2 o Non-Hodgkin most common; usually bilateral I CLINICAL ISSUES
o Histologically: Diffuse cell type> nodular type
28 o CECT: Mild enhancement (hypovascular) Presentation
o Diagnosis: Percutaneous aspiration biopsy • Most common signs/symptoms
o Symptoms may be episodic or paroxysmal
Adrenal myelolipoma (myelolop.) o Crisis: Headaches, HTN, palpitations, sweating,
• Rare benign tumor (fat + hematopoietic elements) tremors, arrhythmias, pain
• Unilateral fatty adrenal tumor (-100 to -30 HU) o Classic: Paroxysmal HTN ± visual changes
• T1WI: Typically hyperintense; size varies (2-10 cm) o Atypical: Labile HTN, myocardial infarction, CVA
• Out of phase T1WI: Focal areas of signal loss • Clinical profile: A young patient with paroxysmal
• US: Echogenic mass in adrenal bed attacks of headache, palpitations, sweating & tremors
• Lab data
Adrenal hemorrhage o 1 Levels of vanillylmandelic acid (VMA) 24-hr. urine
• Etiology: Septicemia, burns, trauma, stress, • Normal range of VMA levels: 1.8-6.7 mg/24 hours
hypotension & hematological abnormalities
• CT findings Demographics
o Usually bilateral • Age: 3rd & 4th decades; 1 familial incidence
o Old hemorrhage: Soft tissue attenuation (20-35 HU) • Gender: M = F
o Recent hemorrhage: 1 Attenuation values
Natural History & Prognosis
• MR findings: T1 & T2WI
o Varied signal depending on hematoma age • Complications: During hypertensive crisis
o Subacute phase: Usually 1 signal (methemoglobin) o Cerebrovascular accidents (CVA)
o Perilesional dark ring (hemosiderin or ferritin) o Pregnancy + pheochromocytoma: Mortality (48%)
o Malignancy in 2-14% cases; distant metastases
Granulomatous infection • Prognosis
• E.g., tuberculosis, histoplasmosis, other fungal diseases o Noninvasive & nonmetastatic: Good prognosis
• Usually bilateral, heterogeneous, poorly enhancing o Malignant & metastatic: Poor prognosis
(acute) • 5 year survival rate is < 50%
• Chronic: Small & calcified adrenals
Treatment
• Diagnosis: Clinical history & lab data
• Medical therapy: Before, during, after surgery
o Alpha-adrenergic blockers
jPATHOLOGY • Phenoxybenzamine, phentolamine
o Beta-adrenergic blocker: Propranolol
General Features • Surgical resection: Benign & malignant
• General path comments • Chemotherapy
o Embryology/anatomy o Cyclophosphamide + vincristine + dacarbazine
• Neoplasm of chromaffin cells derived from neural
crest or neuroectoderm
• Etiology I DIAGNOSTIC CHECKLIST
o Chromaffin cells of sympathetic nervous system
Consider
• Adrenal medulla: Pheochromocytoma
• Imaging findings + history & labs (usually diagnostic)
• Extra-adrenal: Paraganglioma
• Epidemiology Image Interpretation Pearls
o Incidence • Spherical suprarenal mass, 3-5 cm, very hyperintense
• 0.13% in autopsy series; 0.1-0.5% of HTN cases on T2WI MR & brightly enhancing with contrast
• Associated abnormalities
o With 10% autosomal dominant variety
• von Hippel-Lindau syndrome I SELECTED REFERENCES
• Type 1 neurofibromatosis
• Multiple endocrine neoplasia syndromes (MEN) 1. Dunnick NR et al: Imaging of adrenal incidentalomas:
current status. AJRAm J Roentgenol. 179(3):559-68, 2002
type lIA & type lIB 2. Mayo-Smith WW et al: State-of-the-art adrenal imaging.
• Tuberous sclerosis; Sturge-Weber syndrome Radiographies. 21(4):995-1012, 2001
• Carney syndrome: Pulmonary chondroma, gastric 3. Kawashima A et al: Spectrum of CT findings in
leiomyosarcoma, pheochromocytoma nonmalignant disease of the adrenal gland. Radiographies.
18(2):393-412, 1998
Gross Pathologic & Surgical Features
• Round, tan-pink to violaceous, encapsulated mass
 PHEOCHROMOCYTOMA
jlMAGE GALLERY

(Left) Axial NECT of brain in

30 year old man with severe
hypertension shows massive 2
hemorrhage. (Right) Axial
T2WI MR in 30 year old man
shows bilateral hyperintense 29
heterogeneous adrenal
pheochromocytomas
(arrows).

Typical
(Left) Coronal T2WI MR
shows large right adrenal
mass that is heterogeneous
and hyperintense. (Right)
Axial T2WI NEMR shows
heterogeneous hyperintense
right adrenal
pheochromocytoma.

Typical
(Left) Axial CECT shows
hypervascular mass (arrow)
adjacent to bladder; pelvic
pheochromocytoma
(paraganglioma). (Right)
Axial CECT shows
hypervascular paraganglioma
in a patient with headache
and palpitation associated
with voiding.
 ADRENAL CARCINOMA

2
30

Graphic shows large, hypervascular adrenal mass Axial CECT shows large heterogeneous adrenal
directly invading the inferiorvena cava. carcinoma with direct invasion of the inferiorvena cava.

• 20% of cases metastatic at presentation

!TERMINOLOGY
Abbreviations and Synonyms
• Adrenocortical carcinoma, adrenal cancer
Definitions
• Malignant growth from one of the adrenal cell lines
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Large, solid, unilateral adrenal
mass with invasive margins (bilateral in 10%)
• Location: Suprarenal, usually unilateral (left> right)
• Size
o Functioning tumors: Usually 5 cm at presentation
o Nonfunctioning tumors: 10 cm or more
• Morphology
o Large suprarenal invasive lesion
o Usually contain hemorrhagic, cystic & calcific areas
• Key concepts
o Rare & highly malignant neoplasm of adrenal cortex
o Accounts for 0.05-0.2% of all cancers in U.S.A
o Mostly unilateral, but bilateral in up to 10% of cases
o Rarely, etiology of asymptomatic incidentaloma
o Local spread: Renal vein, inferior vena cava (lVe)
o Metastatic spread: Lungs, liver, nodes, bone
o Most patients are at stage 3 or 4 at time of diagnosis
o Functioning « 50%); non functioning (:> 50%)
o Clinical syndromes with functioning tumors
• Cushing syndrome most common (30-40%)
• Virilization in females (20-30%)
• Conn syndrome, feminization (males) < common
o Accounts for only 0.002% of all childhood cancers
• Mostly functional, virilization seen in 95% cases
CT Findings
• Solid, well-defined suprarenal mass + invasive margins
• Usually unilateral; may be bilateral in 10% of cases
• ± Areas of necrosis, hemorrhage, calcification, fat
o Calcification within tumor seen in 30% of cases
• Variable enhancement (necrosis & hemorrhage)
• ± Renal vein, IVC, adjacent renal extension
• Metastases to lung bases, liver or nodes
MR Findings
• T1WI: Hypointense adrenal mass compared to liver
• T2WI: Hyperintense adrenal mass compared to liver
• T1 C+: Heterogeneous enhancement (tumor necrosis)
• Multiplanar contrast enhanced imaging
o Renal vein, IVC & adjacent renal parenchymal
invasion well-depicted on MR
o Sagittal imaging helps to evaluate IVC invasion
o Delineate tumor-liver interface if tumor is on right

DDx: Adrenal Mass

Adenoma with Bleed Myelolop. + Adenoma Gastric Diverticulum Adrenal Metastasis

 ADRENAL CARCINOMA
Key Facts
Imaging Findings
• Best diagnostic clue: Large, solid, unilateral adrenal
mass with invasive margins (bilateral in 10%)
• Functioning tumors: Usually 5 cm at presentation
• Nonfunctioning tumors: 10 cm or more
• ± Areas of necrosis, hemorrhage, calcification, fat
• Variable enhancement (necrosis & hemorrhage)
• ± Renal vein, IVC, adjacent renal extension
• Metastases to lung bases, liver or nodes
Top Differential Diagnoses
• Adrenal adenoma
• Adrenal Metastases & lymphoma
• Adrenal myelolipoma (myelolop.)
• Pheochromocytoma
• Renal cell carcinoma (RCC) upper pole
Ultrasonographic Findings
• Real Time
o Variable appearance depending on size & contents
o Small tumors: Echo pattern similar to renal cortex
o Large tumors: Mixed heterogeneous echo pattern
(due to areas of necrosis & hemorrhage)
Angiographic Findings
• Conventional
o Selective catheterization
• Inferior phrenic artery opacifies superior adrenal
artery, which is often predominant arterial supply
• Renal artery opacifies inferior adrenal artery
• Middle adrenal artery arise from aorta
o Enlarged adrenal arteries; minimal neovascularity
o Inferior venacavography: Confirms tumor invasion
Nuclear Medicine Findings
• FDG-PET
o Adrenal carcinoma: Increased uptake of FDG
• Differentiates from adenoma by lack of 1 uptake
• Adrenocortical scintigraphy by using NP-59
o No uptake in either gland with large tumor
• Carcinoma side: Most of the gland is destroyed
• Contralateral side: Carcinoma 1 hormone release
~ pituitary feedback shutdown of normal gland
Imaging Recommendations
• NE + CECT: Study of choice to exclude adenoma
o 3 mm slice sections with 3 mm increments for
precise cephalad extension of tumor thrombus
I DIFFERENTIAL DIAGNOSIS
Adrenal adenoma
• Well-defined, soft tissue adrenal mass of 0-20 HU
o Lipid rich adenoma: Low density « 10 HU) mass
o Lipid poor adenoma: Varied density of 10-20 HU
• Enhancing adrenal mass that "de-enhances" rapidly
• Washout of adenoma: 10 min. post injection> 50%
o Characteristic & diagnostic of adrenal adenoma
o Similar washout pattern: Lipid rich & poor adenoma
Pathology
• May be associated with genetic syndromes
• Beckwith-Wiedemann, Li-Fraumeni, Carney & MEN
type 1
Clinical Issues 2
• Cushing syndrome (30-40%): 1 Cortisol
• Virilization in females (20-30%): 1 Androgens 31
• Conn syndrome (primary hyperaldosteronism)
• Feminization in males: 1 Androgens
Diagnostic Checklist
• Rule out other adrenal tumors especially adenoma
• Large, unilateral adrenal mass with invasive margins
+ venous, nodal invasion + distant metastases
• Precise definition of cephalad extension of tumor
venous thrombus is essential for surgical resection
Adrenal Metastases & lymphoma
• Adrenal metastases
o Unilateral or bilateral masses ± necrosis, hemorrhage
o Size: Usually < 5 cm, may be larger in melanoma
o Mostly hypervascular & prolonged washout pattern
o Usually known to have malignancy elsewhere
• Adrenal lymphoma
o Primary (rare); secondary (non-Hodgkin common)
o Often bilateral; retroperitoneal disease usually seen
o Discrete or diffuse mass, shape is maintained
o Extensive retroperitoneal tumor engulfing adrenal
o Hypovascular, moderate enhancement with contrast
Adrenal myelolipoma (myelolop.)
• Small or large, asymptomatic suprarenal mass
• Intramural fatty elements seen on imaging
Pheochromocytoma
• Tumor> 3 cm in most cases
• Highly vascular tumor prone to hemorrhage, necrosis
• Tumors are very hyperintense on T2WI
• Bilateral adrenal tumors in multiple endocrine
neoplasia (MEN) IIA & IIB syndromes
• Clinical presentation & lab data may be helpful
Renal cell carcinoma (RCC) upper pole
• Large upper pole RCC mimics large adrenal carcinoma
• Angiography
o Selective injection of renal artery: Downward
displacement of kidney with tumor vascularity
o Selective injection of superior or middle adrenal
arteries: Opacifies tumor & confirm adrenal etiology
o Hypervascular (RCC); hypovascular (adrenal cancer)
o Neovascularity: Predominant with RCC than adrenal
Adrenal hemorrhage
• Adrenal hematomas usually appear round in shape
• Some poorly marginated & show periadrenal stranding
• Acute: High density fluid collection (40-60 HU)
• Chronic: Large, well-defined, low density hematoma
• No enhancement of hematoma on CECT
 ADRENAL CARCINOMA
Ganglioneuroma
• Younger patients, mean age of 27 years
• Larger mass with average tumor size of 8 cm
• Well-circumscribed mass simulating adrenal cancer
2 I PATHOLOGY
32 General Features
• Genetics
o Usually shows loss of heterozygosity at some loci
o More likely to be aneuploid or tetraploid
o Genetic syndromes may 1 incidence of tumor
• Etiology: Unknown for sporadic adrenal carcinoma
• Epidemiology
o 0.05-0.2% of all cancers
o 2 new cases per 1 million population
o 1 per 1500 adrenal tumors are malignant
o 20% have metastatic disease at presentation
• Associated abnormalities
o May be associated with genetic syndromes
• Beckwith-Wiedemann, Li-Fraumeni, Carney &
MEN type 1
Gross Pathologic & Surgical Features
• Usually large & predominantly yellow on cut surface
• Necrotic, hemorrhagic, calcific, lipoid & cystic areas
Microscopic Features
• Well differentiated to markedly anaplastic cells
Staging, Grading or Classification Criteria
• Staging of adrenal carcinoma
o Tl: Diameter ~ 5 cm without local invasion
o T2: Diameter> 5 cm without local invasion
o T3: Any size tumor with local invasion but not
involving adjacent organs
o T4: Any size tumor with local invasion & extension
into adjacent organs, nodes & distant metastases
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Presentation with non-hormonally active
malignancy
• Abdominal pain, fullness or palpable mass
• Incidentally discovered mass on imaging exam
• Metastatic disease in lung, liver ± bone (20% at
presentation)
• 54% of cases nonfunctioning at presentation
• Presentation with hormonally active malignancy
o Cushing syndrome (30-40%): 1 Cortisol
• Moon facies, truncal obesity, purple striae &
buffalo hump
o Virilization in females (20-30%): 1 Androgens
• 95% of children with functioning adrenal
carcinoma present with virilization
o Conn syndrome (primary hyperaldosteronism)
• Hypertension & weakness
o Feminization in males: 1 Androgens
• Other clinical syndromes at presentation
o Hypoglycemia, polycythemia &
nonglucocorticoid-related insulin resistance
Demographics
• Age
o Bimodal distribution
• 1st peak below age 5
• 2nd peak in 4-5th decades of life
• Gender
o Overall, females more than males
• Females account for 65-90% of all cases
• Functioning tumors: More common in females
• Nonfunctioning tumors: More common in males
Natural History & Prognosis
• Rapid growth with local invasion & distant metastases
• Tumor thrombus: IVC & renal vein
• Mean survival 18 months; children better than adults
• 5 year survival for stage 3 disease is under 30%
• Stage 1 & 2: Good prognosis after surgical removal
• Stage 3 & 4: Poor prognosis with or without treatment
Treatment
• Small lesions: Laparoscopic adrenalectomy
• Large lesions with extension: Radical resection of
ipsilateral kidney, adrenal gland, adjacent structures
• Metastatic sites also resected as possible
• Chemotherapy: Mitotane, cisplatin, 5-FU & suramin
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other adrenal tumors especially adenoma
Image Interpretation Pearls
• Large, unilateral adrenal mass with invasive margins +
venous, nodal invasion + distant metastases
• Precise definition of cephalad extension of tumor
venous thrombus is essential for surgical resection
I SELECTED REFERENCES
1. Caoili EM et al: Adrenal masses: characterization with
combined unenhanced and delayed enhanced CT.
Radiology. 222(3):629-33, 2002
2. Dunnick NR et al: Imaging of adrenal incidentalomas:
current status. AJRAm J Roentgenol. 179(3):559-68, 2002
3. Dunnick NR: Adrenal masses. AJRAm J Roentgenol.
179(5):1344,2002
4. Mayo-Smith WW et al: State of the art adrenal imaging.
RadioGraphies. 21:995-1012, 2001
5. Siegelman ES:MR imaging of the adrenal neoplasms. Magn
Reson Imaging Clin N Am. 8(4):769-86, 2000
6. Otal P et al: Imaging features of uncommon adrenal masses
with histopathologic correlation. Radiographies.
19(3):569-81, 1999
7. Dunnick NR et al: Adrenal radiology: distinguishing benign
from malignant adrenal masses. AJRAm J Roentgenol.
167(4):861-7, 1996
8. McLoughlin RF et al: Tumors of the adrenal gland: findings
on CT and MR imaging. AJRAm J Roentgenol.
163(6):1413-8, 1994
9. Fishman EKet al: Primary adrenocortical carcinoma. CT
evaluation with clinical correlation. AJRAm J Roentgenol.
148: 531-5, 1987
 ADRENAL CARCINOMA

I IMAGE GALLERY
Typical
(Left) Coronal T2WI MR
shows heterogeneous,
hypointense, large left
adrenal carcinoma. (Right)
Axial T2WI MR shows
2
heterogeneous, large left 33
adrenal carcinoma.

(Left) Axial CECT shows

large, heterogeneous,
partially calcified right
adrenal carcinoma with
spontaneous retroperitoneal
bleed. (Right) Axial CECT of
30 year old man shows
gynecomastia (arrows), his
presenting complaint, due to
adrenal carcinoma.

(Left) Axial NEMR, opposed

phase eRE, shows no signal
dropout from adrenal mass
(arrow); carcinoma. (Right)
Axial CECTshows an
unusually homogeneous left
adrenal carcinoma.
 ADRENAL METASTASES AND LYMPHOMA

2
34

Axial T1WI MR in phase shows left adrenal mass Axial T1WI MR opposed phase shows no signal
(arrow); metastatic lung cancer. dropout; metastatic lung cancer.

• Lung & breast cancers: 30-40% metastasize to

ITERMINOlOGY
Definitions
• Adrenal metastases from other primary cancer sites
• Adrenal lymphoma: Malignant tumor of
B-Iymphocytes
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Discrete or diffuse suprarenal
masses of soft tissue density
• Key concepts
o Adrenal metastases
• Adrenals are common site of metastatic disease
• Adrenal glands are 4th most common site of
metastases after lungs, liver & bone
• Most common primary sites: Lung, breast, skin
(melanoma), kidney, thyroid & colon cancers
• Indicates stage IV of distant metastatic disease
• Seen in 27% of autopsy cases of epithelial origin
• May be unilateral or bilateral; small or large
• Mostly, discrete intraparenchymal masses
• Direct contiguous extension into adrenals may
occur due to malignancy in surrounding organs
• Malignant melanoma: 50% metastasize to adrenal
glands
adrenal glands
• Renal & GI tract malignancies: 10-20% metastasize
to adrenal glands
• Most often, adrenal metastases are clinically silent
• Occasionally, present with adrenal insufficiency
o Adrenal lymphoma
• Lymphoma of adrenal gland is unusual
• Autopsy: 25% cases of lymphoma involve adrenals
• Primary lymphoma (rare); secondary (common)
• Non-Hodgkin (common); Hodgkin (rare)
• Non-Hodgkin: 4% cases have adrenal involvement
• Diffuse cell type predominate over nodular type
• Bilateral (50%); usually associated with
retroperitoneal adenopathy
CT Findings
• Adrenal metastases
o Small metastases
• Well-defined, round or oval in shape
• Homogeneous soft tissue density masses
• Unilateral or bilateral
• Adrenal gland contour is maintained
• Necrosis, hemorrhage & calcification (rare)
• May mimic lipid poor adrenal adenoma on NECT
o Large metastases
• Unilateral or bilateral enlarged adrenal glands
• Lobulated or irregular in shape
• Heterogeneous density (necrosis, hemorrhage)

DDx: Adrenal Mass

..•.
•
",-t'

~ "I
• '~1.'_

- A-
I '. ' '~
"-
Adrenal Adenomas Adrenal Carcinoma Adrenal Hemorrhage Pheochromocytomas
 ADRENAL METASTASES AND LYMPHOMA
Key Facts
Imaging Findings Pathology
• Best diagnostic clue: Discrete or diffuse suprarenal • Primary malignant tumors of lung, breast, skin
masses of soft tissue density (melanoma), kidney, thyroid & GI tract
• ± Central necrosis, hemorrhage, calcification • Adrenal lymphoma: Often secondary, non-Hodgkin
• May have thick enhancing rims (metastases)
• Hypervascular or hypovascular Clinical Issues 2
• Prolonged washout pattern on CECT (metastases) • Almost always clinically silent
• Washout value of < 50% after 10-15 min.: Indicates • Addison disease: Weakness, weight loss, anorexia, 35
either metastases or atypical adenoma nausea, vomiting, hypotension, skin pigmentation
• Clinical profile: Patient with history of malignancy
Top Differential Diagnoses elsewhere & manifestations of Addison disease
• Adrenal adenoma • Diagnosis: Imaging & percutaneous needle biopsy
• Adrenal carcinoma
• Adrenal hemorrhage Diagnostic Checklist
• Adrenal pheochromocytoma • Check for history of primary cancer or lymphoma
• Adrenal myelolipoma • Overlapping findings: Adrenal metastases, lymphoma
• Imaging important to suggest & stage malignancy

• Distortion of normal contour of adrenal gland

• ± Central necrosis, hemorrhage, calcification Ultrasonographic Findings
• May have thick enhancing rims (metastases) • Real Time
• Hypervascular or hypovascular o Adrenal metastases
• ± Invasion of contiguous organs such as kidneys • Solid lesions with heterogeneous echogenicity
• Prolonged washout pattern on CECT (metastases) • Usually echogenicity is less than surrounding fat
• Washout value of < 50% after 10-15 min.: (hypoechoic)
Indicates either metastases or atypical adenoma o Adrenal lymphoma
o Direct contiguous adrenal invasion • Relatively homogeneous, hypoechoic lesions
• Tumor growth of kidney, pancreas, stomach, liver • ± Areas of echogenicity within mass lesion
& retroperitoneal sarcoma may be seen • Enlarged suprarenal glands
• Adrenal lymphoma
o Discrete or diffuse mass, shape is maintained Angiographic Findings
o Configuration of adrenal limbs is preserved • Conventional
o Homogeneous soft tissue density on NECT o Adrenal metastases
o Hypovascular, moderate enhancement with contrast • Hypervascular: RCC or sarcoma metastases
• Attenuation values vary between 40-60 HU • Hypovascular: Squamous cell cancer metastases
o Unilateral or bilateral (in 50% of cases) o Adrenal lymphoma
o Usually associated with retroperitoneal adenopathy • Hypovascular adrenal mass
o Extensive retroperitoneal tumor engulfing adrenal • Palisading of vessels may be seen
gland • Infiltration: Encasement & amputation of vessels
o Necrosis is uncommon without prior therapy Nuclear Medicine Findings
• Rapidly growing lymphoma may show necrosis
• FDG-PET
o Adrenal lymphoma may mimic lipid poor adenoma
o Malignant lesions (adrenal metastases & lymphoma)
MR Findings • Increased uptake
• Adrenal metastases o Benign lesion (adrenal adenoma)
o Without necrosis & hemorrhage • No increased uptake
• T1WI: Usually homogeneous & hypointense • [Iodine-131] 6-iodomethyl-19-norcholesterol (NP-59)
• T2WI: Relatively hyperintense (due to fluid o Malignant lesions (adrenal metastases & lymphoma)
content) • Lack of uptake
o With necrosis & hemorrhage o Benign lesion (adrenal adenoma)
• T1 & T2WI: Heterogeneous signal intensity • Increased uptake
o Exception with metastatic malignant melanoma • Unilateral early adrenal visualization before day 5
• Hyperintense on T1WI after NP-59 injection
• Occasionally remain hyperintense on T2WI o Normal adrenal glands or normal NP-59
mimicking pheochromocytoma • When both adrenal glands are seen on day 5 after
• Hyperintense on T1WI out of phase & T1WI injection or thereafter
fat-saturated sequences exclude adenoma Imaging Recommendations
• Adrenal lymphoma
• Best imaging tool: Helical NE + CECT; MR
o Nonspecific MR findings
• Protocol advice
oCT: 3 mm thick section at 3 mm intervals or less
o MR T1WI in & out of phases to exclude adenoma
 ADRENAL METASTASES AND LYMPHOMA
I DIFFERENTIAL DIAGNOSIS
Adrenal adenoma
• NECT
o Lipid rich adenoma: Low attenuation « 10 HU)
• Characteristic & diagnostic of adenoma
2 o Lipid poor adenoma: Attenuation varies, 10-30 HU
• Simulate metastases on NECT
o Large adenoma: Heterogeneous density
36
• Due to hemorrhage, cystic degeneration, Ca++
• CECT
o Lipid rich & poor adenomas
• Washout of adenoma: 10 min. post LV. > 50%
• Washout pattern: Diagnostic for adenoma
• T1WI out of phase
o Lipid rich adenoma: Marked signal"drop-out"
o Lipid poor adenoma: Minimal signal loss
Adrenal carcinoma
• Rare, unilateral, invasive & enhancing mass
• More than 6 cm when initially diagnosed
Adrenal hemorrhage
• Acute: High density fluid collection (40-60 HU)
• Chronic: Low density collection (clot of 20-30 HU)
o May mimic as metastases or lymphoma
Adrenal pheochromocytoma
• Highly vascular; prone to hemorrhage & necrosis
• Tumors are very hyperintense on T2WI
• Bilateral adrenal tumors in MEN IIA & IIB syndromes
Adrenal myelolipoma
• Tumor composed of fat & hematopoietic elements
• Usually are heterogeneous fatty adrenal masses
• Tumor with t fat content
o Fat suppression sequence: Hypointense
o Marked signal drop-out at fat-soft tissue interfaces
I PATHOLOGY
General Features
• General path comments
o Normal anatomy of adrenal glands
• Located between vertebrae T11 & L2, lateral to
body of Ll
• Gland has an anteromedial ridge & 2 limbs
• Maximum body width: 0.79 mm (Rt); 0.6 mm (Lt)
• Length of adrenal limbs varies: May be up to 4 cm
• Width of limbs: < 1 cm (right thinner than left)
• Etiology
o Adrenal metastases
• Primary malignant tumors of lung, breast, skin
(melanoma), kidney, thyroid & GI tract
o Adrenal lymphoma: Often secondary, non-Hodgkin
• Epidemiology
o Adrenal metastases
• Autopsy series: 27% of cases of epithelial origin
o Adrenal lymphoma
• Incidence: 4% of cases with non-Hodgkin
• Autopsy series: 25% of cases with lymphoma
• Associated abnormalities
o Primary malignant tumor in case of metastases
o Generalized adenopathy in case of lymphoma
o Adrenal collision tumor
• Metastases & adenoma in same adrenal gland
Gross Pathologic & Surgical Features
• Discrete or diffuse; unilateral or bilateral
• ± Cystic, necrotic, hemorrhagic, calcific areas
Microscopic Features
• Metastases: Varies based on etiology
• Lymphoma: Lymphoepithelial, Reed-Sternberg cells
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Almost always clinically silent
o Extensive masses: Adrenocortical insufficiency
(Addison disease) when 90% of tissue is damaged
o Addison disease: Weakness, weight loss, anorexia,
nausea, vomiting, hypotension, skin pigmentation
• Clinical profile: Patient with history of malignancy
elsewhere & manifestations of Addison disease
• Lab data
o Adrenocortical insufficiency
• j Cortisol, aldosterone, androgens & i ACTH
• j Na+, Cl & t K+ levels
• Diagnosis: Imaging & percutaneous needle biopsy
Demographics
• Age: Any age group
• Gender: M = F
Natural History & Prognosis
• Complications: Adrenocortical insufficiency
• Prognosis: Usually poor
Treatment
• Chemotherapy & follow-up
I DIAGNOSTIC CHECKLIST
Consider
• Check for history of primary cancer or lymphoma
Image Interpretation Pearls
• Overlapping findings: Adrenal metastases, lymphoma
• Imaging important to suggest & stage malignancy
I SELECTED REFERENCES
1. Dunniek NR et al: Imaging of adrenal incidentalomas:
Current status. AJR. 179:559-68,2002
2. Mayo-Smith WW et al: State-of-the-art adrenal imaging.
Radiographies. 21(4):995-1012, 2001
3. Dunniek NR et al: Adrenal radiology: Distinguishing
benign from malignant adrenal masses. AJR.167:861-867,
1996
4. Alvarez-CastellsA et al: CT of primary bilateral adrenal
lymphoma. J Comput AssistTomogr .17:408-409,1993
5. Glazer HS et al: Non-Hodgkin lymphoma: Computed
tomographic demonstration of unusual extranodal
involvement. Radiology. 149:211-217, 1983
 ADRENAL METASTASES AND LYMPHOMA

I IMAGE GALLERY

(Left) Axial CECT shows

heterogeneous, bilateral
adrenal masses; metastatic
breast cancer. (Right) Axial
CECT shows an unusually
2
hypodense left adrenal 37
metastasis from breast
cancer.

(Left) Axial NECT in renal

transplant patient shows
bilateral adrenal masses of
lymphoma (post-transplant
Iymphoproliferative
disorder). (Right) Axial NECT
shows pelvis in a renal
transplant patient with
bilateral adrenal lymphoma
(PTLD).

(Left) Axial T2WI MR

(nonenhanced) shows a left
adrenal metastasis (arrow)
and a right renal cyst. (Right)
Ax~/CECTshowslarge
adrenal masses due to
metastatic melanoma.
 ADRENAL COLLISION TUMOR

2
38

Axial NECT shows near-water density adenoma + a Axial CECT shows bright enhancement of small nodule
small focus of higher density. (melanoma) within larger mass (adenoma) = collision
tumor.

o May see sharp demarcation between

ITERMINOlOGY
Abbreviations and Synonyms
• Adrenal collision tumor (ACT)
Definitions
• Coexistence of two contiguous but histologically
different tumors within same adrenal gland
I IMAGING FINDINGS
General Features
• Best diagnostic clue
o Histological verification by percutaneous biopsy
with imaging guidance
• Two histologically distinct masses; without
significant tissue admixture
• Location: Unilateral; both within same adrenal gland
• Both tumors may be malignant, or one may be benign
& other malignant; or both benign
o Adrenal masses may be adenomas, metastases, cysts,
myelolipomas, hemangiomas, carcinomas etc.
• Some have characteristic imaging features
myelolipomatous & adenomatous components
• Differentiate benign from malignant lesions
o CT attenuation values; threshold of 10 HU on NECT
• Sensitivity 71% and specificity 98%
o Discriminate adenomas from nonadenomas
• Attenuation values on CECT after 15 minute delay
• Adenomas diagnosed on delayed CECT at
thresholds of 37 HU (specificity, sensitivity; 96%)
MR Findings
• Demonstrates & enables characterization of 2 masses
• Chemical shift MR: Demonstrates lipid; t specificity
o In-phase & opposed-phase gradient-echo technique
• Indeterminate masses at CT or conventional MR
• ACT suspected when there is only focal I in signal
intensity of mass on opposed-phase images
• Opposed-phase image: Adenoma, (quantitative) I
signal; metastatic component t signal intensity
• Myelolipoma, benign cortical mass; show loss of
signal intensity; not metastases, hemorrhage, cyst
o Adrenal-spleen ratio (ASR);quantitative analysis
• Identifies all adenomas with ratio of less than 70
• ASRlower for adenoma than for metastases

CT Findings Nuclear Medicine Findings

• Nature of mass: Cystic; solid; solid fatty lesions • Adrenocortical scintigraphy with NP-59
o Macroscopic fat within mass; may be association of (131 I-6b-iodomethyl-norcholesterol)
solid soft tissue lesion with myelolipoma o Differentiate adenomas from nonadenomas

DDx: Adrenal Mass

Adenoma Adrenal Carcinoma Adrenal Metastases Myelolipoma

 ADRENAL COLLISION TUMOR

Key Facts
Terminology • ACT suspected when there is only focal! in signal
• Coexistence of two contiguous but histologically intensity of mass on opposed-phase images
different tumors within same adrenal gland Top Differential Diagnoses
Imaging Findings • Adenoma
• Histological verification by percutaneous biopsy with •
•
Carcinoma
Lymphoma; metastasis
2
imaging guidance
• Both tumors may be malignant, or one may be • Myelolipoma 39
benign & other malignant; or both benign • Hemorrhage; infection

o Limited in masses < 2 cm in diameter with

indeterminate uptake
Microscopic Features
• 2 neoplasms; without significant tissue admixture
Imaging Recommendations
• Best imaging tool
o NECT, CECT or MR ICLINICALISSUES
• Characterization of separate components of ACT
• Planning & guiding percutaneous needle biopsy Presentation
• Protocol advice • May be asymptomatic; incidental finding at imaging
o Lesions ~ 10 HU; likely benign; no further work-up • Diagnosis: Percutaneous needle biopsy
o Lesions ~ 20 HU; likely malignant; biopsied when o Noninvasive differential diagnosis using CT, MR
result may influence management
Natural History & Prognosis
o For indeterminate NECT: Consider CECT w/delayed
• Variable; depends on histology of each component
scans for washout calculation or chemical shift MR
• ASRthreshold of 70; benign lesion; no work-up
• ASR> 70; biopsy depending on clinical situation
I DIAGNOSTIC CHECKLIST
Consider
I DIFFERENTIAL DIAGNOSIS • ACT suspected on chemical shift MR; only focal ! in
Adenoma signal intensity of mass on opposed-phase images
• Hypointense to liver on T2WI; < 10 HU on NECT
• Rapid washout pattern of contrast material
I SELECTED REFERENCES
Carcinoma 1. Khati NJ et al: Adrenal adenoma and hematoma mimicking
• Large (usually ~ 5 cm); central necrosis & hemorrhage a collision tumor at MR imaging. Radiographies.
• Lung metastases, lymphadenopathy, venous invasion 19(1):235-9, 1999
2. Otal P et al: Imaging features of uncommon adrenal masses
Lymphoma; metastasis with histopathologic correlation. Radiographies.
• Lymphoma: Usually bilateral; hypovascular 19(3):569-81, 1999
• Metastases: Unilateral or bilateral; known malignancy 3. Schwartz LH et al: Collision tumors of the adrenal gland:
o Hyperintense on T2WI; delayed contrast washout demonstration and characterization at MR imaging.
Radiology. 201(3):757-60, 1996
Myelolipoma
• Intramural fatty elements recognized on imaging
Hemorrhage; infection
I IMAGE GALLERY
• Hemorrhage: High density on NECT
• Infection: Granulomatous; AIDS

!PATHOLOGY
General Features
• Epidemiology: Extremely rare; prevalence unknown
• Associated abnormalities: Unusual variant of multiple
endocrine neoplasia syndrome; ACT as component
Gross Pathologic & Surgical Features
• Two neoplastic processes; may replace adrenal gland
(Left) Axial NECT shows heterogeneous left adrenal mass,
myelolipoma + adenoma = collision tumor (Right) Axial NECT shows
heterogeneous left adrenal mass, myelolipoma + adenoma = collision
tumor.
SECTION 3: Kidney and Urinary Tract

Introduction and Overview

Kidney and Urinary Tract Anatomy and Imaging 11I-3-2

Normal Variants and Pseudolesions

Renal Ectopia 11I-3-6
Horseshoe Kidney 11I-3-10
Column of Bertin 11I-3-14

Congenital
AD Polycystic Disease, Kidney 11I-3-16
vonHippel Lindau Disease 11I-3-20
Ureteropelvic Junction Obstruction 11I-3-24

Infection
Pyelonephritis 11I-3-28
Renal Abscess 11I-3-32
Xanthogranulomatous Pyelonephritis 11I-3-36
Emphysematous Pyelonephritis 11I-3-40
HIV Nephropathy 11I-3-42

Inflammation
Glomerulonephritis 11I-3-44
Renal Papillary Necrosis 11I-3-46
Urolithiasis 11I-3-48
Nephrocalcinosis 11I-3-52
Acquired Cystic Disease of Uremia 11I-3-56
Medullary Sponge Kidney 11I-3-60

Vascular
Renal Artery Stenosis 11I-3-64
Renal Infarction 11I-3-68
Renal Vein Thrombosis 11I-3-72

Trauma
Renal Trauma 11I-3-76

Neoplasm, Benign
Renal Cyst 11I-3-80
Renal Oncocytoma 11I-3-84
Renal Angiomyolipoma 11I-3-88
Multilocular Cystic Nephroma 11I-3-92

Neoplasm, Malignant
Renal Cell Carcinoma 11I-3-96
Renal Medullary Carcinoma 11I-3-100
Transitional Cell Carcinoma 11I-3-102
Renal Metastases and Lymphoma 11I-3-106
 KIDNEY AND URINARY TRACT ANATOMY AND IMAGING

3
2

Graphic shows algorithm for analyzing imaging features Graphic shows algorithm for analyzing a renal mass,
of a renal cystic mass, listing the most common causes. showing main differential for an infiltrative mass.

o Scan diaphragm to pubis with 4 min delay

I EMBRYOLOGY
Abbreviations and Synonyms
• Renal cell carcinoma (RCe)
• Transitional cell carcinoma (TCe)
• Intravenous pyelogram (excretory urogram) (IVP)
• Xanthogranulomatous pyelonephritis (XGP)
• Tuberculosis (TB)
I IMAGING ANATOMY
location
• Kidneys lie within the perirenal space (with adrenals)
• Congenital anomalies of location (ectopic, rotational),
fusion, and number (congenital absence) are relatively
common
I ANATOMY-BASED IMAGING ISSUES
Key Concepts or Questions
• Current role of IVP
o Detection of anomalies such as ureteral duplication,
ureterocele
o Complementary to retrograde pyelography and
CT/MR for transitional cell carcinoma
o Alternative (suboptimal) to CT for evaluation of
neatly all etiologies of hematuria ± flank pain
• Calculi, tumor, infection, trauma, vascular
• How can we replace the IVP (urography) with CT
completely?
o By performing CT urography
• How do you perform CT urography?
o 1000 cc water orally 15 to 20 min prior to CT (to
produce diuresis)
o Nonenhanced scans through kidneys (diaphragm to
iliac crest)
o 100 to 125 ml nonionic contrast IV at 3 ml/sec
o After 3 min , roll patient 360 (to opacify all
0
segments of bladder and collecting system)
o May add earlier phase (40 sec delay) if vascular
anatomy is important
o Rarely add 10 min delay in event of high grade
ureteral obstruction
• How can we minimize or deal with the hypodense
renal mass "too small to characterize" on CT?
o Minimize occurrence by
• Obtaining CT sections less than Vz the diameter of
the lesion
• Using adequate volume of IV contrast (~ 100 ml
in average adult)
• Avoid scanning in corticomedullary phase
o Check non enhanced or delayed CT, if available
• No visible enhancement or de-enhancement,
almost alw~ys benign
• If lesion less dense than blood on NECT,almost
always benign
• Consider clinical setting (e.g., septic; oncology
patient; elderly): ~ 2 cm hypodense mass in very
elderly patient usually requires no further
evaluation
• Sonography good at resolving cyst versus solid
• What are the key morphologic features used to
distinguish a benign (Bosniak class I or II) form a
"surgical" (Bosniak III or IV) cyst?
o Bosniak II
• Calcification:Thin, peripheral, milk of calcium
• Hyperdense: Homogeneous, no enhancement
• Septations: < 2 mm thick, no nodularity, no
enhancement
o Bosniak III
• Calcification: Thick (~ 2 mm), central, irregular
• Hyperdense: Heterogeneous, enhancement
• Septations: ;:::2mm, nodularity, enhancement
• Renal calcifications
o Dystrophic
o Nephrocalcinosis
• Cortical
• Medullary
o Calculi (nephrolithiasis)
IKIDNEY AND URINARY TRACT ANATOMY AND IMAGING I
DIFFERENTIAL DIAGNOSIS
Expansile renal mass (benign)
• Cyst
• Angiomyolipoma
• Oncocytoma
• Multilocular cystic nephroma
• Focal severe pyelonephritis)
• Other rare tumors
• => (Leiomyoma, fibroma, etc.)
Expansile renal mass (malignant)
• Renal cell carcinoma
• Metastases
• Lymphoma
Infiltrative renal process/mass (benign)
• Pyelonephritis (including XGP, TB)
• Infarction (acute)
o Vascular, usually arterial
• Dystrophic calcification
o Calcification in abnormal, damaged tissue
• Tumor, infection, infarction
• Cortical nephrocalcinosis
o Often appears as "egg-shell" calcification
o Kidneys usually small with diminished or absent
function
o Typical causes
• Chronic glomerulonephritis
• Acute cortical necrosis (shock, sepsis, toxins)
• Transplant rejection
• Medullary nephrocalcinosis
o Calcification in normal tissue
o Renal size and function are often normal
o Pattern: May conform to renal pyramids
o Often associated with nephrolithiasis (calculi)
• E.g., medullary sponge kidney, hypercalcemic
condition
• Distinguishing among causes of nephrocalcinosis
o Medullary sponge kidney: Cystic dilation of renal
tubules that contain stones +/or fill with contrast on
urography; clustered at papillary tips: Calculi
become obscured by contrast-opacified urine
(unique)
• Papillary blush" is a normal finding; does not
constitute medullary sponge
o Renal tubular (transient dense opacification of
tubules in papilla; in dehydration) acidosis: Dense
and extensive calcification of the medullary portions
of the renal lobes; electrolyte abnormalities
• Calcification is not obscured by contrast-opacified
urine
o Renal papillary necrosis
• Sloughed papilla that may calcify, often as a ring
shape
• May have amorphous calcification of papilla
• Calcifications are not obscured by opacified urine
Infiltrative renal process/mass (malignant)
• Transitional cell carcinoma
• Lymphoma
• Renal cell carcinoma
• Medullary carcinoma
• Squamous cell carcinoma
Multiple renal cysts
• Multiple simple cysts
• Autosomal dominant polycystic disease
• Multicystic dysplastic kidney
• Acquired cystic kidney disease (uremia)*
• Tuberous sclerosis complex* 3
• von Hippel Lindau*
• => *Associated with multiple renal neoplasms
I CLINICAL IMPLICATIONS
Clinical Importance
• Histologic variations and cell of origin of primary
renal carcinomas affect imaging appearance and
approach, as well as prognosis
o Clear cell RCC (hypernephroma)
• Constitutes 70 to 85% of renal carcinomas
• Refers to lipid-rich cytoplasm
• Hypervascular, heterogeneous
• Often aggressive growth and widely metastatic
o Papillary RCC
• Constitutes 10 to 15% of RCC
• Hypovascular, homogeneous, often encapsulated
• Easily mistaken for a cyst on imaging studies
• Slow to metastasize, better prognosis
o Medullary carcinoma
• Arises from epithelium of papilla or distal
collecting duct
• Affects young men with sickle cell anemia
• Very aggressive, infiltrative, metastatic
• Survival < 4 months average
o Transitional cell carcinoma
• Arises from uroepithelium
• Appears as obstruction or filling defect within
collecting system of kidney, ureter or bladder
• May diffusely infiltrate the kidney
o Squamous cell carcinoma
• Due to squamous metaplasia of uroepithelium
caused by chronic irritation (stones, infection)
• Infiltrative, aggressive; survival < 1 year average
• Imaging resembles XGP
• Survival < 1 year average
I CUSTOM DIFFERENTIAL DIAGNOSIS I
Focal or global small, scarred kidney
• Reflux nephropathy (chronic atrophic pyelonephritis)
• Ischemia/infarction
Renal and ischemic infarction
• Embolic more common cause than thrombotic
 I KIDNEY AND URINARY TRACT ANATOMY AND IMAGING I

3
4

Graphic shows algorithm for analyzing imaging features
of a renal mass, showing main differenual for a solid
expansile mass.
Graphic shows effect of parual volume averaging on
appearance of small masses. If a cystic mass (8) fills the
CT secuon, it will appear of water attenuauon. If it fills
only part of the secuon (C, 0), it will appear of higher
density.

• Usually a cardiac source • Contusion

o Prosthetic cardiac valves • Vasculitis
o Endocarditis • Renal vein thrombosis
o Myocardial infarction
o Atrial fibrillation Bilateral persistent nephrograms
o Intracardiac catheters/pacemaker wires • Hypotension/shock
o Cardiac tumors • Obstruction of renal tubules
o Vasculitis o Myoglobin, urate, protein, acute tubular necrosis
o Aortic dissection • Bilateral ureteral, arterial} or venous obstruction
o Atheromatous plaque Medullary nephrocalcinosis causes
• May be asymptomatic; usually causes flank pain, fever}
nausea • Hypercalcemia
o Hyperparathyroidism, milk-alkali, vitamin D
• Key finding: Wedge-shaped or global
toxicity
non enhancement of kidney on CECT (acute)
• Medullary sponge kidney (renal tubular ectasia)
o Cortical rim sign (enhancement of capsule and
• Renal tubular acidosis
peripheral cortex)
• Chronic infection
o (Subacute infarction collateral vessels)
o TB,AIDSorganisms} chronic pyelonephritis
o Focal or global atrophy without caliceal dilation
(chronic)
Reflux nephropathy I SELECTED REFERENCES
• Previously known as chronic atrophic pyelonephritis 1. Koeller KK et al: Radiologic pathology. 2nd ed.
• Usually due to reflux and episodic urinary infection Washington, DC, Armed Forces Institute of Pathology.
beginning in infancy or childhood 487-594,2003
o Often present in young adult with hypertension and 2. Urban BA et al: Three-dimensional volume-rendered CT
renal insufficiency angiography of the renal arteries and veins: normal
anatomy, variants, and clinical applications.
• Can be unilateral or bilateral
Radiographics. 21(2):373-86; questionnaire 549-55, 2001
• Leads to focal or global decrease in renal size 3. Pollack HM et al: Clinical Urography. 2nd ed. Philadelphia,
• Key finding: Cortical loss (scar) over dilated calyx WB Saunders, 2000
4. Dahnert W: Radiology review manual. 4th ed.
Unilateral delayed or persistent nephrogram Philadelphia, Lippincott, Williams and Wilkins. 723-56,
• Slow blood inflow 2000
o Renal artery stenosis 5. Nicolau C et al: Autosomal dominant polycystic kidney
• Slow blood outflow disease types 1 and 2: assessment of US sensitivity for
o Renal vein stenosis/thrombosis diagnosis. Radiology. 213(1):273-6, 1999
• Slow urine outflow 6. Bosniak MA: Diagnosis and management of patients with
o Ureteral obstruction complicated cystic lesions of the kidney. AJR Am J
Roentgenol. 169(3):819-21, 1997
• Decreased nephron function 7. Kawashima A et al: CT of renal inflammatory disease.
Striated nephrogram Radiographies. 17(4):851-66; discussion 867-8} 1997

• Pyelonephritis
I KIDNEY AND URINARY TRACT ANATOMY AND IMAGING I

I IMAGE GALLERY I

(Left) Axial CECT shows

Bosniak /I cyst (arrow) with
thin calcified septum, water
density. (Right) Axial CECT
shows Bosniak 11/cystic mass
(multilocular cystic
nephroma); thick, calcified
septa.

3
5

(Left) Nonenhanced, axial

CT shows vicarious excretion
(dense bile) and persistent
nephrograms due to acute
tubular necrosis; shock
following splenic laceration.
(Right) Axial CECT shows
delayed right nephrogram
due to acute ureteral
obstruction (ureteral
calculus).

(Left) Axial CECT shows

striated nephrogram; acute
pyelonephritis. (Right) Axial
CECT shows extensive
medullary nephrocalcinosis;
primary oxalosis.
 RENAL ECTOPIA

3
6

Graphic shows crossed inferior fused renal ectopia. Axial CECT shows embryologic left kidney in an inferior
crossed ectopic location. Note dilated left renal artery
(arrow) and renal pelvis opening toward the right.

o Crossed RE: Kidney located on opposite side of

ITERMINOLOGY midline from its ureteral orifice
Abbreviations and Synonyms • Size: Ectopic kidneys vary in size
• Renal ectopia (RE) • Other general features
o Caudal RE
Definitions • Unilateral (more common)
• Abnormal location of kidney due to developmental • Involvement of both kidneys (rare)
anomaly • In solitary kidney (least common)
o Crossed RE:With fusion (most common); without
fusion (10-15%); in solitary kidney (least common)
I IMAGING FINDINGS • With fusion (most common)
• Without fusion (10-15%)
General Features • In solitary kidney (least common)
• Best diagnostic clue: Abnormal location of kidney o Classification of unilateral fused kidney or crossed
• Location fused RE
o Kidneys normal location: 1st-3rd lumbar vertebrae • Superior: Kidney crosses over midline; lies superior
o Ipsilateral RE: Kidney on same side of body as orifice to resident kidney
of its attendant ureter • Sigmoid (S-shaped): Crossed kidney lies inferiorly
• Cranial (superior RE): Above normal position; • Unilateral lump kidney: Both kidneys completely
intrathoracic; or below eventrated diaphragm fused; large irregular lump
• Caudal (simple RE): Below normal position; • Unilateral L-shaped: Crossed kidney inferior &
abdominal, iliac or pelvic transverse; resident kidney normally oriented
• Abdominal: Kidney lies above iliac crest, below L2 • Unilateral disc: Each kidney fused to other along
• Iliac: Kidney located opposite iliac crest or in iliac medial concave border
fossa • Inferior RE: Crossed kidney inferior to resident; its
• Pelvic (sacral): Kidney located in true pelvis; below upper pole fused to lower pole of resident kidney
iliopectineal line
Radiographic Findings
• Radiography

DDx: Misplaced Kidney

Renal Transplant Renal Transplant Displaced Kidney Displaced Kidney

 RENAL ECTOPIA
Key Facts
Terminology
• Renal ectopia (RE)
• Abnormal location of kidney due to developmental
anomaly
Imaging Findings
• Best diagnostic clue: Abnormal location of kidney
• Renal outline not visible in expected position
• Soft-tissue outline of kidney may be visible in ectopic
position
• Cranial RE: Kidney residing in thorax; differentiate
from a mediastinal mass
• Abdominal or iliac RE
• Pelvic RE: Differentiate RE from various pelvic masses
• Crossed RE: CT with thin (4-5 mm) slices may show
degree of separation of kidneys
o Chest x-ray (in cranial RE)
• Well-defined posteroinferior mediastinal mass
o Abdominal x-ray (in caudal RE)
• Renal outline not visible in expected position
• Soft-tissue outline of kidney may be visible in
ectopic position
• Malposition of colon
o Abdominal x-ray (in crossed RE)
• Soft-tissue outline of kidney may be visible on
opposite side
• Malposition of colon
• IVP
o Cranial RE
• Kidney lies partially or completely in thorax
• Length of attendant ureter longer than normal
o Abdominal or iliac RE: Kidney in either abdominal
or iliac area
• Can be simple (unilateral, bilateral, or solitary) or
crossed
• Kidney usually smaller & ureter shorter than
normal
• Bizarre pattern of calyces; extrarenal calyces
(common)
• Rotational anomaly: Incomplete, reverse or
nonrotation
o Pelvic kidney
• Left (70%) > right; if bilateral, left usually lower
than right kidney & generally fused
• May see anomalies of rotation
• Ureter is frequently too high as it exits renal pelvis
("high insertion")
• May see ectopic ureter, extrarenal calyces, calyceal
diverticula
o Crossed RE: Distal ureter inserts into trigone on side
of origin
• Both pelvises rotated anteriorly in superior,
unilateral lump & inferior RE
• Sigmoid (S-shaped): Resident kidney pelvis is
medial; lateral in crossed kidney
• Unilateral disc: Resident kidney pelvis is
anteromedial, pelvis of other is anterolateral
o Bilateral crossed RE: Both kidneys on wrong side but
their attendant ureters arise normally
Top Differential Diagnoses
• Renal allograft
• Renal autotransplantation
• Horseshoe kidney
• Acquired renal displacement
Diagnostic Checklist
• Retroperitoneal mass, huge renal cyst, gigantic renal
pelvis secondary to UP] obstruction can force kidney
to opposite side simulating crossed unfused RE (IVP)
• CECT helps in detecting RE & cause of displacement
• On IVP, pelvic kidney may be difficult to locate (due
to sacral superimposition & bowel gas) 3
• Clue to pelvic kidney is ureter, which can be visible
7
even if collecting system & kidney are not visible
• Important not to confuse RE with renal ptosis
CT Findings
• Cranial RE: Kidney residing in thorax; differentiate
from a mediastinal mass
o Adrenal gland may lie above, behind or below
ectopic kidney
• Abdominal or iliac RE
o Adrenal gland in normal place; appears linear on CT
o Colonic flexures, duodenum, loops of small bowel,
spleen, tail of pancreas in abnormal position
• Pelvic RE: Differentiate RE from various pelvic masses
• Crossed RE: CT with thin (4-5 mm) slices may show
degree of separation of kidneys
Ultrasonographic Findings
• Real Time
o Cranial RE
• Kidney lies just below an eventrated diaphragm
• Passing through defect in diaphragm
o Caudal RE (abdominal, iliac, or pelvic)
• Renal sinus echo complex: Eccentric or absent
o Crossed RE
• Separation of kidneys can be demonstrated
• Kidneys move separately from each other during
respiration
o Crossed fused RE
• One or two anterior or posterior notches in renal
parenchyma
• Renal sinuses lie in different planes, run in
different directions & echoes reflect differently
Angiographic Findings
• Conventional
o Cranial RE
• Elongated renal artery from aorta at normal level
• Occasionally, accessory RA from thoracic aorta
o Abdominal or iliac RE
• Renal arteries arise lower in aorta than normal
• Multiple renal arteries (common)
o Pelvic RE
• Renal arteries are often multiple
• Usually arise from distal end or aortic bifurcation
o Crossed RE: Anomalous blood supply arising from
vessels in vicinity
 RENAL ECTOPIA
Nuclear Medicine Findings
• Tc99m-DMSA or Tc99m-glucohepatanate scan
o Detects ectopic kidney by outlining kidney shape
o Crossed fused renal ectopia: Isotope excretion or
localization by a kidney, with no contralateral
isotope excretion or localization
Imaging Recommendations
• Helical CECT; IVP
I DIFFERENTIAL DIAGNOSIS
3 Renal allograft
• Transplanted kidney in iliac fossa
8 • Renal vessels anastomosed to external iliac artery, vein
• Ureter reimplanted into bladder via submucosal
tunnel; variable axis of pelvis
Renal autotransplantation
• Surgically repositioning patient's own kidney
Horseshoe kidney
• Fusion of lower poles of kidneys in low mid-abdomen
Acquired renal displacement
• Due to large liver, splenic or any retroperitoneal tumor
I PATHOLOGY
General Features
• Etiology
o Cranial RE: Kidney herniated into thorax through
lumbocostal triangle or foramen of Bochdalek
o Caudal RE: Diminished ureteral growth; umbilical
arteries block cranial ascent of kidney; asymmetry in
level of development of 2 kidneys
o Crossed RE: Mesonephric ducts & ureteral buds may
stray from normal course
o RE inherited as autosomal recessive trait; reported in
monozygotic twins
• Epidemiology
o Cranial RE: 1 in 15,000 autopsies
o Abdominal or iliac RE: 1 in 600 on IVP
o Pelvic kidney: 1 in 725 live births
o Unilateral crossed fused RE: 1 in 1,300 to 1 in 7,600
• Associated abnormalities
o Genitourinary (50%): Malrotation, hypospadias,
high insertion of ureter into renal pelvis, ectopic
ureter, extrarenal calyces, calyceal diverticula,
bladder extrophy
o Skeletal (40%): Anomalies of ribs, vertebral bodies;
skull asymmetry & absence of radius
o Cardiovascular (40%): Valvular & septal defects
o Gastrointestinal (33%): Anorectal malformations
malrotation. ' 6.
o Ears, lips, palate (33%): Low-set or absent ears; hare
lip; cleft palate
o Hematopoietic (7%): Fanconi anemia
o Cranial RE: Omphalocele
o Pelvic kidney: Vesicoureteral reflux, contralateral
renal agenesis, absent or hypoplastic vagina
o Crossed ectopia: Megaureter, cryptorchidism,
urethral valves, multicystic dysplasia
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o May be asymptomatic, incidental finding
o May present with signs & symptoms of obstruction,
urolithiasis, reflux & infection
Demographics
• Gender: Cranial RE (M > F); crossed fused RE (M < F)
Natural History & Prognosis
• Complications
o Obstruction, urolithiasis, reflux, infection
o Pelvic kidneys: I Function & may obstruct labor
o Aberrant arteries may cross & obstruct ureter
o Abdominal & iliac ectopic kidneys more injury
prone; prone to vascular injury during aortic surgery
• Prognosis
o Recurrent obstruction, reflux, infection: Poor
Treatment
• Treat complications of renal ectopia
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
• Retroperitoneal mass, huge renal cyst, gigantic renal
pelvis secondary to UP] obstruction can force kidney
to opposite side simulating crossed unfused RE (IVP)
• CECT helps in detecting RE & cause of displacement
• On IVP, pelvic kidney may be difficult to locate (due
to sacral superimposition & bowel gas)
o Clue to pelvic kidney is ureter, which can be visible
even if collecting system & kidney are not visible
• Important not to confuse RE with renal ptosis
o Kidney drops further down in abdomen from its
normal position, but attendant ureter of normal
length & renal arteries arise from normal site
I SELECTED REFERENCES
1. Li J et al: Single ureteral ectopia with congenital renal
dysplasia. J Urol. 170(2 Pt 1):558-9, 2003
2. Gu LL et al: Crossed solitary renal ectopia. Urology.
38(6):556-8, 1991
Hawass ND et al: Intrathoracic kidneys: report of 6 cases
3.
and a review of the literature. Eur UroI.14(1):83-7, 1988
4. Goodman JD et al: Crossed fused renal ectopia:
sonographic diagnosis. Urol Radiol. 8(1):13-6, 1986
5. McCarthy S et al: Ultrasonography in crossed renal ectopia.
J Ultrasound Med. 3(3):107-12, 1984
Hertz M et al: Crossed renal ectopia: clinical and
radiological findings in 22 cases. Clin Radiol. 28(3):339-44
1977 '
Rubinstein ZJ et al: Crossed renal ectopia: angiographic
7.
findings in six cases. Am J Roentgenol. 126(5):1035-8, 1976
 RENAL ECTOPIA

I IMAGE GALLERY
Typical
(Left) Axial CECT shows right
kidney in normal position, no
kidney in left renal fossa.
(Right) Axial CECT shows left
kidney in midline, non-fused,
pelvic location.

3
9

Typical
(Left) Axial CECT shows
horseshoe kidney with fusion
of the lower poles across the
midline and low in position.
(Right) Axial CECT shows a
horseshoe kidney with
hydronephrosis. Retro-renal
lymphadenopathy from
prostate metastases.

Typical
(Left) Axial CECT shows
normal right kidney, but no
left. (Right) Axial CECT
shows the left kidney in a
crossed-fused inferior ectopic
location with its pelvis
opening postero-Iateral/y.
 HORSESHOE KIDNEY

3
10

Graphic shows horseshoe kidney. Note multiple renal Axial CECT shows fusion of the lower poles of the
arteries. kidneys across the midline and low position of kidneys.

o Visualize the Isthmus of the 2 kidneys

ITERMINOLOGY
• IVP
Definitions o Midline fusion
• A congenital anomaly of the kidney where 2 kidneys • Hand holding calyces: Lower calyces descend
fused by isthmus at the lower poles toward midline near isthmus
• Nephrogram is U-shaped
• If width of isthmus is < 1/3 length of kidney, renal
I IMAGING FINDINGS pelvis lies between normal 30° anteromedial and
90° direct anterior angle
General Features • If width of isthmus is ~ 1/3 length of kidney,
• Best diagnostic clue: 2 kidneys on opposite sides of the renal pelvis lies between 90° direct anterior and
body with the lower poles fused in midline lateral angle
• Location • Rarely, calyces from opposing kidneys joined to
o Ectopic, lies lower than normal kidney form a common renal pelvis with 1 ureter
o Isthmus usually anterior to aorta and inferior vena o Lateral fusion
cava (IVC) • Lower calyces crosses midline and drain part of
o Rarely, isthmus is posterior or in between aorta renal parenchyma on opposite kidney
(posterior) and IVC (anterior) • Nephrogram is L-shaped
• Morphology • One part crosses midline and lies in transverse
o 2 types of fusion position, renal pelvis lies anteriorly or laterally
• Midline or symmetrical fusion (90% of cases) • Remaining part lies in vertical position, renal
• Lateral or asymmetrical fusion pelvis lies anteriorly or medially
o Large and extrarenal renal pelvis
Radiographic Findings o Renal pelvis often large and flabby; ureter inserts
• Radiography abnormally high in renal pelvis
o Kidney appears too close to the spine o Rarely, kidney is fused at the upper poles (5%)
o Vertical long axis of kidney may be seen, lower poles o Ipsilateral lower calyces medial to ureter; may
lie closer to spine simulate renal malrotation without fusion

DDx: Abnormal Position and Rotation of Kidney

Crossed Ectopia Crossed Ectopia Displacement Displacement

 HORSESHOE KIDNEY

Key Facts
Terminology Top Differential Diagnoses
• A congenital anomaly of the kidney where 2 kidneys • Renal ectopia
fused by isthmus at the lower poles • Renal displacement
Imaging Findings Pathology
• Best diagnostic clue: 2 kidneys on opposite sides of • Epidemiology: 1 in 400 people
the body with the lower poles fused in midline
• Ectopic, lies lower than normal kidney
Clinical Issues
• Isthmus usually anterior to aorta and inferior vena • Asymptomatic or associated abnormalities
cava (lVC) • Any age
• Midline or symmetrical fusion (90% of cases) • Gender: M:F = 2:1
• Kidney appears too close to the spine
• Large and extrarenal renal pelvis
Diagnostic Checklist
• Associated abnormalities and other complications in
3
• Multiple, bilateral renal arteries
imaging, treatment and prognosis 11
• IVP followed by CT or scintigraphy for pre-operative
• Kidney appears U-shaped with isthmus in midline
assessment

o Ureteropelvic (more common) or ureterovesical

junction obstruction with delayed clearing of Nuclear Medicine Findings
contrast • Demonstrate fusion with functional parenchymal
o "Flower-vase" appearance: Each ureter crosses tissue
isthmus and curves laterally and continues medially, Imaging Recommendations
assuming a normal course distally
• Best imaging tool
o Bifid or double ureters drain the kidneys
o IVP followed by CT or scintigraphy for pre-operative
• Voiding Cystourethrography
assessment
o Vesicoureteral reflux
o US for diagnosis in utero
CT Findings • Protocol advice: CTA: Use 3-D volume-rendered CT to
• CECT better define the vessels
o Define structural abnormalities
• Degree and site of fusion: Midline or lateral fusion
• Degree of renal malrotation I DIFFERENTIAL DIAGNOSIS
• Renal parenchymal changes (e.g., scarring, cystic
Renal ectopia
disease)
• Collecting system abnormalities (e.g., duplex • Kidney congenitally in abnormal position
system, hydronephrosis) • Ipsilateral or simple ectopia: Kidney on proper side of
o Differentiate composition of isthmus between body as its ureter
fibrous or normal parenchymal tissue o Abdominal: Kidney lies above iliac crest but below
• CTA L2
o Variant arterial supply o Iliac: Kidney is located opposite iliac crest or in iliac
• Multiple, bilateral renal arteries fossa
• Inferior mesenteric artery always crosses the o Pelvic (sacral): Kidney in true pelvis
isthmus • Crossed renal ectopia: 2 kidneys are on the same side
• Arteries arising from aorta or common iliac, of the body (right side> left)
internal iliac, external iliac or inferior mesenteric o With fusion (90%): 2 fused kidneys lie on the same
arteries side of spine; ureter of crossed kidney crosses
midline to insert into bladder
Ultrasonographic Findings o Without fusion: 2 kidneys lie on the same side of
• Real Time spine without fusion; ureter of crossed kidney
o Isthmus lies anterior to spine and continues with crosses midline to insert into bladder
lower poles of opposite kidneys o Solitary: 1 kidney arises on the wrong side, ureter
o Curved configuration, elongation and poorly crosses midline to insert into bladder
defined lower poles o Bilateral: Left and right kidneys arise on the wrong
o Inverted triangular or pyriform shape (longitudinal side, both ureters crosses midline to insert into
scan) bladder
Angiographic Findings Renal displacement
• Conventional: Variant arterial supply • Thoracolumbar gibbous deformity: Alters the renal
axis ~ pseudohorseshoe kidney
• Displacement by mass: Forcing one kidney to opposite
side of body or into pelvis
 HORSESHOE KIDNEY
o Retroperitoneal masses o Trauma injury: Isthmus lies anteriorly without
o Giant hydronephrosis secondary to ureteropelvic protection by ribs ~ split by hard blow to abdomen
junction (UP]) obstruction o UP] obstruction: High "insertion" of ureter
o Giant renal cyst (e.g., autosomal dominant o Recurrent infections: Vesicoureteral reflux and UP]
polycystic kidney disease) obstruction
o Urolithiasis: 75% metabolic calculi} 25% struvite
calculi
I PATHOLOGY o Wilms tumors in children: 2-8 times more common
o Primary renal carcinoid tumor: 1 Prevalence
General Features • Prognosis
• General path comments: Most common renal fusion o Poor, with associated abnormalities causing
anomaly significant morbidity and mortality
• Genetics: Reported in identical twins, but no clear o Good} without other abnormalities
evidence
3 • Epidemiology: 1 in 400 people Treatment
• Associated abnormalities • Surgical separation in symptomatic patients
12 o Congenital disorders
• Chromosomal abnormalities: Turner syndrome,
trisomy 18 I DIAGNOSTIC CHECKLIST
• Hematological abnormalities: Fanconi anemia,
dyskeratosis congenita with pancytopenia Consider
• Laurence-Biedl-Moon syndrome • Associated abnormalities and other complications in
• Thalidomide embryopathy imaging, treatment and prognosis
o Anomalies (most common to least common) Image Interpretation Pearls
• UP] obstruction • Kidney appears U-shaped with isthmus in midline
• Vesicoureteral reflux
• Unilateral or bilateral duplication
• Megaureter I SELECTED REFERENCES
• Ectopic ureter
• Unilateral triplication 1. Strauss S et al: Sonographic features of horseshoe kidney:
review of 34 patients. J Ultrasound Med. 19(1):27-31, 2000
• Renal dysplasia
2. Pozniac MA et al: Three-dimensional computed
• Retrocaval ureter tomographic angiography of a horseshoe kidney with
• Supernumerary kidney ureteropelvic junction obstruction. Urology 49:267-268,
• Anorectal malformation 1997
• Esophageal atresia 3. Banerjee B et al: Ultrasound diagnosis of horseshoe kidney.
• Rectovaginal fistula Br J Radiol. 64(766):898-900, 1991
• Omphalocele 4. Mesrobian HG et al: Wilms tumor in horseshoe kidneys: a
• Cardiovascular, vertebral, neurological, peripheral report from the National Wilms Tumor Study. J Urol.
skeletal or facial anomalies 133(6):1002-3, 1985
5. Grainger R et al: Horseshoe kidney--a review of the
Gross Pathologic & Surgical Features presentation, associated congenital anomalies and
complications in 73 patients. Ir MedJ. 76(7):315-7, 1983
• Isthmus is composed of normal parenchyma or
6. Evans WP et al: Horseshoe kidney and urolithiasis. J Urol.
connective tissue 125(5):620-1, 1981
7. Pitts WR Jr et al: Horseshoe kidneys: a 40-year experience. J
Urol. 113(6):743-6, 1975
I CLINICAL ISSUES 8. Whitehouse GH: Some urographic aspects of the horseshoe
kidney anomaly-a review of 59 cases. Clin Radiol.
Presentation 26(1):107-14, 1975
• Most common signs/symptoms 9. Boatman DL et al: Congenital anomalies associated with
o Asymptomatic or associated abnormalities horseshoe kidney. J Urol. 107:205-7, 1973
10. Kolin CP et al: Horseshoe kidney: a review of 105 patients.
o Vague abdominal pain, radiating to the back
J Urol. 107(2):203-4, 1972
o Nausea and vomiting 11. SeguraJW et al: Horseshoe kidney in children. J Urol.
o Rovsing sign, palpable abdominal mass 108:333-6, 1972
12. Boatman DL et al: The arterial supply of horseshoe kidneys.
Demographics AmJ Roentgenol Radium Ther Nucl Med. 113(3):447-51,
• Age 1971
o Any age 13. Zondek LH et al: Horseshoe kidney and associated
o Still births> infants> children> adults; j with age congenital malformations. Urol Int. 18:347-56, 1964
because many diagnosed based on associated
abnormalities
• Gender: M:F = 2:1
Natural History & Prognosis
• Complications
 HORSESHOE KIDNEY
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
hydronephrosis of left side of
a horseshoe kidney due to
stones and UP] obstruction.
(Right) Axial CECT shows a
horseshoe kidney with
hydronephrosis, UP]
obstruction and multiple
calculi (arrow).

3
13

Typical
(Left) Axial NECT shows
horseshoe kidney, multiple
calculi, and renal scarring.
(Right) Axial NECT shows
horseshoe kidney, calculi,
and renal scarring.

Typical
(Left) Axial CECT shows a
horseshoe kidney with a
large mass (RCe; replacing
most of the isthmus and left
side. (Right) Axial CECT
shows tumor thrombus
(arrow) within the left renal
vein of a patient with
horseshoe kidney and RCC.
 COLUMN OF BERTIN

3
14

Craphic shows mass-like extension of renal cortex Axial CECT shows mass-like extension of renal cortex
between upper and middle calices. (curved arrow) between upper and middle calices.

ITERMINOLOGY CT Findings
Abbreviations and Synonyms • NECT
o Mass continuous with renal cortex
• Septal cortex, hypertrophied or enlarged column of o Lateral indentation of renal sinus
Bertin, focal cortical hyperplasia, benign cortical rest, o Deformed adjacent calices and infundibula
cortical island, focal renal hypertrophy, junctional
• CECT
parenchyma o Absence of a mass
Definitions o Similar enhancement as normal renal cortex
• Hypertrophic medial bands of cortical tissue that Ultrasonographic Findings
separate the pyramids of the renal medulla
• Real Time
o Normal renal outline
o Isoechoic with renal cortex; hypoechoic to medulla
I IMAGING FINDINGS o Mass with an echogenic linear rim of renal sinus fat
o Splaying of central sinus echoes
General Features
• Best diagnostic clue: Normally enhancing renal cortex Nuclear Medicine Findings
protruding into renal sinus • In equivocal cases, renal scintigraphy can differentiate
• Location normal renal parenchyma from pathologic mass
o Between upper and middle calyces
o Left side> right side Imaging Recommendations
o Unilateral> bilateral (18% of cases) • Best imaging tool: Multiphasic CT
• Protocol advice: CT: Corticomedullary phase best
Radiographic Findings
• IVP
o Mass effect on pelvicaliceal system, always at level of
emerging renal vein
o Splaying and abnormal separation of upper and
lower pole of collecting system

DDx: Renal Mass or Pseudomass

-.- ...

. :~
.;.~;::;:-
.•...•...•.•.
.. ~..-
.

•.',.. .~

Renal Cell Carcinoma Fetal Lobulation Fetal Lobulation Angiomyolipoma

 COLUMN OF BERTIN

Key Facts
Terminology Top Differential Diagnoses
• Hypertrophic medial bands of cortical tissue that • Renal cell carcinoma
separate the pyramids of the renal medulla • Fetal lobulation
• Renal scarring
Imaging Findings • Other renal masses
• Best diagnostic clue: Normally enhancing renal
cortex protruding into renal sinus Diagnostic Checklist
• Isoechoic with renal cortex; hypoechoic to medulla • Pseudotumor, not pathological disease
• Absence of a mass on CECT

I DIFFERENTIAL DIAGNOSIS I DIAGNOSTIC CHECKLIST 3

Renal cell carcinoma Consider 15
• Solitary renal mass with central necrosis, • Pseudotumor, not pathological disease
heterogeneous
• Mass is usually rounded or oval in cortical location Image Interpretation Pearls
and grows by expansion • Absence of a mass on CECT
• Multiple septations, septal thickening and nodularity
• Hypervascular mass
I SELECTED REFERENCES
Fetal lobulation
1. Yeh HC et al: Junctional parenchyma: revised definition of
• Persistent cortical lobulation hypertrophic column of Bertin. Radiology. 185(3):725-32,
'. Fourteen individual lobes with centrilobar cortex 1992
located around calices 2. Seppala RE et al: Sonography of the hypertrophied column
of Bertin. A]R Am] Roentgenol. 148(6):1277-8, 1987
Renal scarring 3. Lafortune M et al: Sonography of the hypertrophied
• Focal scarring from chronic reflux nephropathy, column of Bertin. A]R Am] Roentgenol. 146(1):53-6, 1986
surgery, trauma or infarction 4. Leekam RN et al: The sonography of renal columnar
• Nodular compensatory hypertrophy of areas of hypertrophy.] Clin Ultrasound. 11(9):491-4, 1983
unaffected tissue 5. Mahony BS et al: Septa of Bertin: a sonographic
pseudotumor.] Clin Ultrasound. 11(6):317-9, 1983
Other renal masses 6. Williams ED et al: Kidney pseudotumour diagnosed by
emission computed tomography. Br Med] (Clin Res Ed).
• E.g., renal metastases and lymphoma, renal
285(6352):1379-80, 1982
oncocytoma, renal angiomyolipoma 7. Green WM et al: "Column of Bertin": diagnosis by
nephrotomography. Am] Roentgenol Radium Ther Nucl
Med. 116(4):714-23, 1972
I PATHOLOGY
General Features
• General path comments: Embryology: Unresorbed
I IMAGE GALLERY
polar parenchyma of one or both of two sub-kidneys
that fuse to form a normal kidney

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Asymptomatic
• Diagnosis
o Usually found incidentally on imaging
o Most likely to simulate a mass on sonography
Natural History & Prognosis
• Normal variant (Left) Sonogram shows pseudomass (arrow) in upper pole of kidney
isoechoic to renal cortex. (Right) Sonogram shows pseudomass
• Complications: None
(arrow) in upper pole of kidney; column of Bertin,
• Prognosis: Very good
Treatment
• None
 AD POLYCYSTIC DISEASE, KIDNEY

3
16

Axial NEeT shows massively enlarged kidneys with Sagittal sonogram shows massively enlarged kidney with
innumerable cysts. innumerable cysts.

• Fourth leading cause of chronic renal failure in

ITERMINOLOGY the world
Abbreviations and Synonyms • 50% chance of inheriting mutant gene from
• Autosomal dominant polycystic kidney disease ADPKD parent ~ child
(ADPKD) or adult PKD • Multisystemic disorder with cystic & non-cystic
• Autosomal recessive polycystic kidney disease (ARPKD) manifestations
o Infantile, newborn, childhood, hamartomatous PKD o Cystic manifestations of ADPKD
• Kidneys (100%); liver (75%); pancreas (10%);
Definitions ovaries & testis
• Hereditary disorder characterized by multiple renal o Noncystic manifestations of ADPKD
cysts & various systemic manifestations • Cardiac valvular disorders (26%); hernias (25%);
colonic diverticula
• Aneurysms: Cerebral"berry" aneurysms (5-10%);
IIMAGING FINDINGS aorta & coronary arteries
o ARPKD or infantile PKD
General Features • Very rare cystic renal disease in infancy/childhood
• Best diagnostic clue: Massively enlarged kidneys with • Enlarged polycystic kidneys + hepatic cysts
innumerable cysts • Usually bilateral, often present with renal failure
• Location: Renal cortex, medulla & subcapsular areas & may develop hepatic fibrosis & portal HTN
• Size: Variable in size • Poor prognosis due to early onset of renal failure
• Morphology: Well-defined, round or oval cysts + thin
imperceptible or calcified wall Radiographic Findings
• Other general features • IVP
o PKD: One of the classifications of renal cystic disease o Usually not recommended
o PKD: Two major genetically inherited types (ADPKD o Plain film
& ARPKD) • ± Curvilinear, dystrophic cyst wall calcification
o ADPKD or adult PKD • ± Renal calculi
• One of the most common monogenetic disorders o Mild or markedly enlarged kidneys

DDx: Multiple Cysts in Kidney

,-

-, '
Acquired Cystic Dis. Acquired Cystic Dis. Tuberous Sclerosis
 AD POLYCYSTIC DISEASE, KIDNEY

Key Facts
Terminology Top Differential Diagnoses
• Autosomal dominant polycystic kidney disease • Multiple simple cysts
(ADPKD) or adult PKD • Acquired cystic disease of uremia
• Hereditary disorder characterized by multiple renal • von Hippel-Lindau disease
cysts & various systemic manifestations • Tuberous sclerosis
• Medullary cystic disease
Imaging Findings
• Best diagnostic clue: Massively enlarged kidneys with Clinical Issues
innumerable cysts • Asymptomatic, flank pain
• Cysts: Multiple, well-defined, round or oval in shape; • Hematuria, HTN, renal failure (depends on age)
variable in size; bilateral; ± calculi
• Bosselated kidneys: Multiple cysts projecting beyond Diagnostic Checklist
renal contours • Check for genetic or family history of PKD 3
• Hypodense cysts (water attenuation) + thin wall • Check for associated cystic lesions of liver, pancreas,
• CECT: No enhancement of cysts + normal renal tissue spleen, ovaries & intracranial aneurysms + cardiac 17
enhancement valve defects
• Check for history of dialysis (acquired cystic disease)

o "Swiss cheese" pattern: Smoothly marginated

radiolucencies in cortex & medulla seen on Ultrasonographic Findings
nephrographic phase • Real Time: Multiple well-defined round anechoic areas
o Smooth, bosselated renal contour in both enlarged kidneys
o Normal or effaced collecting system
Angiographic Findings
CT Findings • Conventional
• Cysts: Multiple, well-defined, round or oval in shape; o Arterial phase: Stretching & displacement of
variable in size; bilateral; ± calculi intrarenal vessels around cysts
• Early stage: Kidneys are normal in size & contour o Nephrographic phase: Multiple avascular masses
• Later stage: t In size, number of cysts, renal volume; ± (Swiss cheese pattern) in renal cortex & medulla
asymmetrical kidneys
Nuclear Medicine Findings
• Bosselated kidneys: Multiple Gysts projecting beyond
• Technetium-99m (Tc99m)
renal contours
mercaptoacetylinetriglycerine (MAG3)
• Uncomplicated cysts
o Multiple photopenic masses
o Hypodense cysts (water attenuation) + thin wall
o Delayed clearance of radionuclide
o CECT: No enhancement of cysts + normal renal
tissue enhancement • Obstruction of collecting system due to cysts
• Decreased renal function
• Complicated (hemorrhagic cysts)
• Indium-Ill labeled WBC scan
o Hyperdense cysts (60-90 HU); usually subcapsular
o Detects infected renal cysts
o ± Perinephric large hematomas (due to rupture)
o Increased tracer uptake, markedly at cyst periphery
o ± Curvilinear mural calcification within cysts
o CECT: Hypodense relative to normal renal tissue Imaging Recommendations
• Complicated (infected cysts) • u.s.: Sensitivity
97%; specificity 100%; accuracy 98%
o Hypodense; ± gas within infected cyst o Recommended for screening family members
o Thick irregular cyst wall & adjacent renal fascia • NE + CECT; MR
o CECT: ± Wall enhancement
MR Findings
• TIWI
I DIFFERENTIAL DIAGNOSIS
o Uncomplicated & infected cysts: Hypointense Multiple simple cysts
o Complicated (hemorrhagic cysts)
• Rarely as numerous as in ADPKD
• Varied signal (depending on age of hemorrhage) • Usually not associated with nephromegaly
• Hyperintense (methemoglobin-, paramagnetic + • Normal renal function
short TI relaxation time)
• Differential features favoring ADPKD
• ± Fluid-iron levels (> intense settling layer
o Family history; presence of renal failure
posteriorly-, methemoglobin)
o Cysts (other organs): Liver, pancreas, spleen, ovaries
• T2WI o Intracranial aneurysms
o Uncomplicated: Hyperintense (thin wall)
o Complicated (infected cysts): Hyperintense (marked Acquired cystic disease of uremia
mural thickening) • History: Chronic renal failure with long term dialysis
o Complicated (hemorrhagic cysts); Varied signal or • Early stage; Small kidneys with few cysts
markedly hyperintense • Advanced stage: Large kidneys + multiple small cysts
 AD POLYCYSTIC DISEASE, KIDNEY
yon Hippel-Lindau disease I CLINICAL ISSUES
• Autosomal dominant; multiple renal cysts & cysts in
Presentation
other organs
• Renal cysts are usually less numerous than in ADPKD • Most common signs/symptoms
• Hemangioblastomas: Cerebellar, spinal & retinal o Asymptomatic, flank pain
• Multifocal renal cell carcinomas, pheochromocytomas o Hematuria, HTN, renal failure (depends on age)

Tuberous sclerosis Demographics

• Multiple bilateral renal cysts
• Small fat-containing renal angiomyolipomas (AMLS)
• Cerebral paraventricular calcifications
• Renal cysts have typical histological features in
tuberous sclerosis
3 Medullary cystic disease
• Nephronophthisis or salt wasting nephropathy
18
• Two types based on age related & inherited patterns
o Childhood nephronophthisis: Autosomal recessive +
associated eye, CNS, hepatic, skeletal abnormalities
o Adult form: Autosomal dominant + none associated
• Kidneys are almost invariably small in size
• Clinically, progressive renal failure in young patients
• Imaging
o Renal cysts may be too small to be seen
o Visible cysts occur only in renal medulla
• Cysts in ADPKD involve both cortex & medulla
!PATHOLOGY
General Features
• Genetics
o Autosomal dominant (90%); spontaneous mutations
(10%)
o 50% Chance of inheriting mutant gene from ADPKD
parent to child
o Three types of ADPKD based on gene location
• ADPKD1: Short arm of chromosome 16 (90%)
• ADPKD2: Long arm of chromosome 4 (10%), type
3 gene unknown
• Etiology
o Hereditary: Autosomal dominant
o Pathogenesis: Abnormal gene ~ proliferation of
renal tubular cells~ diverticula of nephrons
(collecting ducts) ~ cystogenesis
• Epidemiology
o Incidence
• 1 in 400-1,000 persons in U.S.
o Prevalence in U.S.: Higher than cystic fibrosis,
hemophilia, sickle cell disease & muscular dystrophy
• Associated abnormalities
o Spectrum of hepatic cystic disease
• Polycystic liver disease; congenital hepatic fibrosis
• Biliary hamartomas; Caroli disease
o Pancreatic & splenic cysts
o Intracranial aneurysms & cardiac valve defects
Gross Pathologic & Surgical Features
• Enlarged kidneys
• Cysts with clear, serous, turbid or hemorrhagic fluid
Microscopic Features
• Cysts lined by simple flattened or cuboidal epithelium
• ± Wall calcification
• Age: Childhood to 8th decade
• Gender: M = F
Natural History & Prognosis
• Complications
o Hemorrhage; infection; rupture
o Renal failure; malignancy (rare)
• Prognosis: Fair following renal transplantation
Treatment
• Treat symptoms & complications
o Hypertension, pain, renal infection
• Renal transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Differentiate from other multiple renal cystic diseases
• Check for genetic or family history of PKD
• Check for associated cystic lesions of liver, pancreas,
spleen, ovaries & intracranial aneurysms + cardiac
valve defects
• Check for history of dialysis (acquired cystic disease)
Image Interpretation Pearls
• Bilateral, multiple cysts + enlarged kidneys
• ± Cystic lesions: Liver, pancreas, spleen, ovaries
• ± Cerebral "berry" aneurysms & cardiac valve defects
I SELECTED REFERENCES
1. Chatha RKet al: Von Hippel-Lindau disease masquerading
as autosomal dominant polycystic kidney disease. Am J
Kidney Dis. 37(4):852-8, 2001
2. Slywotzky CM et al: Localized cystic disease of the kidney.
AJRAmJ Roentgenol. 176(4):843-9,2001
3. Nascimento AB et al: Rapid MR imaging detection of renal
cysts: age-based standards. Radiology. 221(3):628-32, 2001
4. Nicolau C et al: Autosomal dominant polycystic kidney
disease types 1 and 2: assessment of US sensitivity for
diagnosis. Radiology. 213(1):273-6, 1999
5. Perrone RD: Extrarenal manifestations of ADPKD. Kidney
Int. 51(6):2022-36, 1997
6. Fick GM et al: Natural history of autosomal dominant
polycystic kidney disease. Annual Review of Medicine. 45:
23-9, 1994
7. Gabow PA:Autosomal dominant polycystic kidney disease
N. Engl. J. Med., 329(5): 332 - 342, July 29, 1993
8. Parfrey PS et al: The diagnosis and prognosis of autosomal
dominant polycystic kidney disease. N Engl J Med.
323(16):1085-90, 1990
9. Walker FC Jr et al: Diagnostic evaluation of adult
polycystic kidney disease in childhood. AJRAm J
Roentgenol. 142(6):1273-7, 1984
 AD POLYCYSTIC DISEASE, KIDNEY
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
polycystic involvement of
kidneys and liver, with
varying density of cysts +
mural calcification due to
prior hemorrhage. (Right)
Axial CECT (delayed) shows
bilateral polycystic kidneys
and dilated left collecting
system due to obstructing
calculus. 3
19

Typical
(Left) Axial CECT shows
massive cystic enlargement
of kidneys and liver. (Right)
Axial CECT shows extensive
renal and liver involvement.

Variant
(Left) Axial CECT in a 79
year old woman with
ADPKD shows functioning
kidneys with numerous cysts.
(Right) Axial CECT in a 79
year old woman with
ADPKD but preserved renal
function.
 VON HIPPEL LINDAU DISEASE

3
20

Graphic shows muluple cysts in pancreas and kidneys, Axial CECT shows multiple pancreatic and renal cysts
solid masses (renal cell carcinoma) in both kidneys and and a renal cell carcinoma (arrow) in a patient with
a left adrenal pheochromocytoma (arrow). VHL.

• Liver cysts, papillary cystadenoma of epididymis

ITERMINOlOGY & broad ligament (rare)
Abbreviations and Synonyms o Eyes: Retinal hemangioblastomas (RHb)
• von Hippel-Lindau (VHL) syndrome • Most frequent & earliest detected lesions of VHL
• Also called "retinal angiomas" or "hemangiomas"
Definitions • Rarely, optic nerve & chiasm hemangioblastomas
• Rare, autosomal dominant multi-systemic disorder • 45-59% of patients with VHL exhibit RHb
characterized by abnormal growth of tumors • About 50% of cases have bilateral RHb
• Diagnosed in one & two year olds; 5% of cases
detected before age 10
I IMAGING FINDINGS o CNS: Cerebellar hemangioblastomas (CHb)
• One of the most common manifestations of VHL
General Features • CHb occurs in 44-72% of all VHL cases
• Best diagnostic clue: Retinal or cerebellar tumor • Represent 2% of all brain & 7-10% of posterior
(hemangioblastoma) + multiple renal cysts or adrenal fossa tumors (only 5-30% ofthese are due to VHL)
tumor (pheochromocytoma) • Usually occurs at a young age & often multiple
• Other general features • Solitary CHb, considered potentially a VHL case
o Autosomal dominant (AD) familial tumor syndrome • Medullary Hb (5%); spinal Hb (13%) of VHL cases
o Sporadic mutations occur rarely resulting in a o Endolymphatic sac tumors
syndrome indistinguishable from hereditary VHL • Relatively newly recognized complication of VHL
o Manifestations of VHL • Inner ear: End of endolymphatic duct (labyrinth)
• Retinal & CNS (cerebellar, medullary & spinal o Renal cysts & tumors
cord) hemangioblastomas • Renal cysts: 50-75% of VHL cases; simple &
• Endolymphatic sac tumor: Papillary cystadenoma complex; usually bilateral & multiple
• Renal: Cysts & renal cell carcinoma (RCe) • RCC: 28-45% of VHL cases; multicentric &
• Adrenal: Pheochromocytoma bilateral (75% of cases); seen in young age
• Pancreas: Cysts, serous cystadenoma, islet cell • Hereditary cancer syndromes: Bilateral solid clear
tumor, adenocarcinoma, hemangioblastoma cell renal cancers without other VHL features

DDx: Multiple Renal Cysts/Masses

Cysts + RCC AD Polycystic Acquired Cystic Dis. ACKD+ RCC

 VON HIPPEL LINDAU DISEASE
Key Facts
Terminology
• von Hippel-Lindau (VHL) syndrome
• Rare, autosomal dominant multi-systemic disorder
characterized by abnormal growth of tumors
Imaging Findings
• Best diagnostic clue: Retinal or cerebellar tumor
(hemangioblastoma) + multiple renal cysts or adrenal
tumor (pheochromocytoma)
• CHb occurs in 44-72% of all VHL cases
• Simple cysts: Well-defined, rounded, thin-walled,
nonenhancing, near water density lesions
• Multiple, bilateral solid hypovascular or complex
cystic masses + mural nodules, septa (RCe)
• Well-defined, round, suprarenal mass (muscle HU)
• Honeycomb or sponge-like pancreatic head mass
o Adrenal tumors: Pheochromocytomas
• Occur in 7-18% of all patients with VHL
• Some families have t pheochromocytomas
frequency + low frequency of CHb & renal cancers
• 50-80% are bilateral; often multiple & benign
• Malignancy rate, 10-15% with t incidence of
metachronous tumors developing after surgery
• Mostly localized, but 15-18% are extraadrenal
• 20% of VHL families with high prevalence of
pheochromocytoma will develop islet cell tumors
o Pancreatic cysts & tumors
• Frequency of pancreatic involvement, 15-77%
• Simple cysts, 80-90%; serous cystadenoma, 8-12%
• Islet cell (neuroendocrine) tumors (7-12%):
Usually nonfunctional & often multiple
• Combined lesions (11%); ± pheochromocytoma
CT Findings
• Renal masses
o Renal cysts
• Usually bilateral & multiple
• Simple cysts: Well-defined, rounded, thin-walled,
nonenhancing, near water density lesions
• Complex cysts: Irregular, septate, minimally
calcified, slightly t attenuation, no enhancement
o Renal cell carcinoma
• Multiple, bilateral solid hypovascular or complex
cystic masses + mural nodules, septa (RCe)
• Adrenal tumors: Pheochromocytomas
o Well-defined, round, suprarenal mass (muscle HU)
o ± Areas of necrosis, hemorrhage, calcification
o Homogeneous or heterogeneous enhancement
• Pancreatic cysts & tumors
o Pancreatic cysts
• Round or oval shaped water density lesions
• Usually multiple; thin imperceptible wall
o Serous (microcystic) cystadenoma
• Honeycomb or sponge-like pancreatic head mass
• Enhancement of septa delineating small cysts
• Size varies from 5-10 cm + capsular enhancement
• ± Calcification; ± CBD, pancreatic duct dilatation
o Islet cell (neuroendocrine) tumor
• Usually large (nonfunctional) mixed density
Top Differential Diagnoses
• AD polycystic kidney disease (ADPKD)
• Acquired cystic kidney disease of uremia (ACKD)
• Medullary cystic disease
Clinical Issues
• More than one hemangioblastoma in CNS or retina
• One hemangioblastoma + visceral manifestation
• Known family history + one of above manifestation
Diagnostic Checklist
• Annual clinical, biochemical & imaging screening of
VHL patients & at risk family members, relatives 3
• Genetic testing in family members, define their status
• Retinal & CNS Hbs; renal & pancreatic cysts 21
• Bilateral solid renal masses-CT (RCCs); hyperintense
adrenal mass T2WI (pheochromocytoma)
• ± Calcification, cystic & necrotic areas
• Hypervascular, enhancing metastases
MR Findings
• Renal masses
o Renal cysts
• Simple cysts: Hypointense (T1WI); hyperintense
(T2WI); no enhancement (Tl C+)
• Complex cysts: Varied signal (Tl & T2WI), ±
enhancement (T1 C+)
o Renal cell carcinoma
• Multiple solid lesions of varied intensity (T1 &
T2WI); heterogeneous enhancement (T1 C+)
• Adrenal tumors: Pheochromocytomas
o TlWI
• Isointense to muscle & hypointense to liver
• Heterogeneous signal (necrosis & hemorrhage)
o T2WI
• Markedly hyperintense (characteristic)
• Long T2 relaxation time (due to increase water
content as a result of necrosis)
o T1 C+: Brightly enhancing with contrast
• Pancreatic cysts & tumors
o Pancreatic cysts
• Hypointense (TlWI); hyperintense (T2WI)
o Serous (micro cystic) cystadenoma
• Hypointense (TlWI); hyperintense (T2WI)
• T1 C+: Capsular enhancement; enhancement of
septa delineating small cysts
• MRCP: Depict pancreatic duct & CBD dilatation
o Islet cell (neuroendocrine) tumor
• T1WI: Isointense (small); heterogeneous (large)
• T2WI: Isointense (small); hyperintense (large)
• T1 C+: Hyperintense (small); heterogeneous (large)
Ultrasonographic Findings
• Real Time
o Renal & pancreatic cysts: Well-defined & anechoic
o RCC: Hyperechoic, isoechoic or hypoechoic
o Serous cystadenoma of pancreas
• Tumor with tiny cysts: Hyperechoic mass but with
through transmission
o Pancreatic islet cell tumor: Hypoechoic mass
 YON HIPPEL LINDAU DISEASE
o Pheochromocytoma
• Iso-/hypoechoic (77%); hyperechoic (23%)
Nuclear Medicine Findings
• 1-131 or 123 Metaiodobenzylguanidine (MIBG)
o After 24-72 hrs: i Uptake in pheochromocytoma
Imaging Recommendations
• Helical NE + CECT & MR, T1 C+
I DIFFERENTIAL DIAGNOSIS
Renal cyst
3 • May be present in kidney with RCC without VHL
• Cyst and RCC are both common
22
AD polycystic kidney disease (ADPKD)
• Multiple, bilateral renal cysts of VHL mimic ADPKD
• Massively enlarged kidneys with innumerable cysts
• Cystic manifestations of ADPKD
o Kidneys (100%); liver (75%); pancreas; ovaries; testes
Acquired cystic kidney disease of uremia
(ACKD)
• Bilateral small kidneys with multiple small cysts
• History of long term dialysis in endstage renal disease
• Increased prevalence of renal carcinoma
Medullary cystic disease
• Nephronophthisis or salt wasting nephropathy
• Adult form (AD); childhood form (AR)
• Small sized kidneys with cysts in medulla
• Clinically, progressive renal failure in young patients
I PATHOLOGY
General Features
• Genetics
o Gene is located on short arm of chromosome 3
• Due to inactivation of tumor suppressor gene
• 30% of patients have no exact mutation identified
• Etiology: Autosomal dominant; very rarely sporadic
• Epidemiology: Prevalence, one in 39,000-53,000
Gross Pathologic & Surgical Features
• Varies depending on underlying lesion of VHL
Microscopic Features
• Varies depending on tumor of origin
Staging, Grading or Classification Criteria
• Proposed national cancer institute (NCI) classification
o 1. VHL without pheochromocytoma (most common)
• Retinal & CNS Hb, renal cysts & cancers,
pancreatic cysts, no pheochromocytoma
o II. VHL with pheochromocytoma
• A. Retinal & CNS Hbs, islet cell tumors of
pancreas, pheochromocytomas
• B. Retinal & CNS Hbs, renal cell carcinomas,
pancreatic lesions, pheochromocytomas
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Symptoms & signs
varies based on tumor of origin
• Lab data
o CBC to look for polycythemia vera
o Urine: Vanillylmandelic acid (VMA), & hematuria
• Diagnostic criteria
o More than one hemangioblastoma in CNS or retina
o One hemangioblastoma + visceral manifestation
o Known family history + one of above manifestation
Demographics
• Age: Infancy to 7th decade (average age 26 years)
Natural History & Prognosis
• Complications: Varies depending on underlying tumor
• Prognosis
o RCC is the leading cause of death in VHL patients
• 35-75% prevalence in one autopsy series
o 2nd most common cause of morbidity & mortality
in VHL disease is CNS hemangioblastomas
• CHb: Neurologic compromise & death
• Retinal Hb: Retinal detachment or visual loss
Treatment
• Medical: Symptomatic treatment
• Surgical: Organ-sparing surgery
I DIAGNOSTIC CHECKLIST
Consider
• Annual clinical, biochemical & imaging screening of
VHL patients & at risk family members, relatives
• Genetic testing in family members, define their status
• Consider VHL disease in whom retinal or cerebellar
Hb, renal tumors, or pheochromocytoma is diagnosed
Image Interpretation Pearls
• Retinal & CNS Hbs; renal & pancreatic cysts
• Bilateral solid renal masses-CT (RCCs); hyperintense
adrenal mass T2WI (pheochromocytoma)
I SELECTED REFERENCES
1. Taouli B et al: Spectrum of abdominal imaging findings in
von Hippel-Lindau disease. A]RAm] Roentgenol.
181(4):1049-54,2003
2. Marcos HB et al: Neuroendocrine tumors of the pancreas in
von Hippel-Lindau disease: spectrum of appearances at CT
and MR imaging with histopathologic comparison.
Radiology. 225(3):751-8, 2002
3. Couch V et al: von Hippel-Lindau disease. Mayo Clin Proc.
75(3):265-72, 2000
4. Choyke PL et al: von Hippel-Lindau disease: genetic,
clinical, and imaging features. Radiology. 194(3):629-42,
1995
5. Hough OM et al: Pancreatic lesions in von Hippel-Lindau
disease: prevalence, clinical significance, and CT findings.
A]RAm] Roentgenol. 162(5):1091-4, 1994
6. Choyke PL et al: von Hippel-Lindau disease: radiologic
screening for visceral manifestations. Radiology. 174(3 Pt
1):815-20, 1990
 VON HIPPEL LINDAU DISEASE

I IMAGE GALLERY

Typical
(Left) Sagittal MR shows
cerebellar and spinal cord
hemangioblastomas
(arrows). (Right) Sagittal MR
shows multiple spinal
hemangioblastomas
(arrows).

3
23

Typical
(Left) Axial CECT shows a
heterogeneous left adrenal
mass (arrow), a
pheochromocytoma in a
patient with VHL. (Right)
Axial CECT shows numerous
pancreatic cysts. Left
nephrectomy for renal cell
carcinoma.

(Left) Axial CECT shows two

of multiple renal cysts in a
patient with VHL. (Right)
Axial CECT shows a small
renal cell carcinoma (arrow)
in a patient with VHL.
 URETEROPELVIC JUNCTION OBSTRUCTION

3
24

Axial CECT in arterial phase shows relationship of Coronal MIP CT image shows two left renal arteries and
dilated renal pelvis to arteries and veins. the renal vein branches relative to dilated renal pelvis.
Pigtail ureteral stent (arrow).

o Incomplete visualization of normal caliber ureter

!TERMINOLOGY o Increased or decreased renal size due to partial or
Abbreviations and Synonyms complete obstruction, respectively
• Ureteropelvic junction (UP]) obstruction, pelviureteric o "Negative pyelogram" appearance in early films:
junction obstruction, idiopathic, pelvic or congenital Unilateral delayed opacification of collecting system
hydronephrosis o "Calyceal crescents": Thin, semilunar, opacified
collecting tubules in the periphery of dilated calyces
Definitions o Dilated papillary (Bellini) ducts: Small dots of
• Obstructed urine flow from renal pelvis to proximal contrast at medial edge of dilated calyces
ureter ~ pressure increase in renal pelvis o "Puddles" or "Ball" pyelogram: Contrast settles in
dependent portions of dilated calyces
o "Soap bubble" nephrogram: Parenchymal thinning
I IMAGING FINDINGS ~ opacified, thin, overlapping rim of parenchyma
o "Shell" or "rim" nephrogram: Opacify only the
General Features peripheral portion of thin rim
• Best diagnostic clue: Pyelocaliectasis with UP] o Delayed clearing of contrast from collecting system
narrowing o "Linear band" sign: Linear oblique crossing defect in
• Location proximal end of ureter
o Left kidney (2 times) > right kidney o "Short segment" sign: Small amount of contrast
o Unilateral> bilateral obstruction (10-30% of cases) trapped in segment just below the UP]
o Enlarged extrarenal pelvis out of proportion to
Radiographic Findings cali ectasis; may simulate normal variant
• Radiography • Usually flabby or distensible
o Calcification in wall of dilated renal pelvis • Relatively narrow or "closed" outlet
o Staghorn calculus, calculus fragments or "explosion" • Turgid in some phases of IVP
• IVP o Diuresis IVP as adjunct
o Marked pyelocaliectasis; UP] narrowing
o Giant hydronephrosis; may displace, rotate and
obstruct contralateral kidney and ureter

DDx: Mimics of UPJ Obstruction

(I)'
- y -
Urolithiasis Urolithiasis Tee at UP} Renal Pelvic Blood Clot
 URETEROPELVIC JUNCTION OBSTRUCTION

Key Facts
Terminology Top Differential Diagnoses
• Obstructed urine flow from renal pelvis to proximal • Urolithiasis
ureter ~ pressure increase in renal pelvis • Tumor
• Extrinsic indentation
Imaging Findings • Retrocaval ureter
• Best diagnostic clue: Pyelocaliectasis with UP] • Renal pelvis blood clot
narrowing
• Left kidney (2 times) > right kidney Clinical Issues
• Incomplete visualization of normal caliber ureter • Indicated when patient has symptoms, stones,
• Increased or decreased renal size due to partial or infection or renal function is impaired or at risk
complete obstruction, respectively
• "Negative pyelogram" appearance in early films: Diagnostic Checklist
Unilateral delayed opacification of collecting system • Use CT or MR to evaluate potential acquired 3
• Delayed clearing of contrast from collecting system etiologies of UP] obstruction
• Chronic obstruction ~ progressive changes to renal 25
• Best imaging tool: IVP: Adult; US: Neonates and
children pelvis and calyces ~ distinctive urographic signs

• Delayed clearing (> 10 min.) of contrast, • Hydronephrosis ± ureterectasis

pyelocaliectasis and flank pain; suggest
intermittent UP] obstruction
• Voiding cystourethrography
o Exclude severe vesicoureteral reflux in infants
• Retrograde ureteropyelography
o Assess ureter if not visualized in other studies
o "]et" of contrast squirting through narrow UP] into
non-opacified, urine-filled renal pelvis
o Angulated UP] by crossing bands or vessels
CT Findings
• NECT
o Hydronephrosis ± ureterectasis
o Level of obstruction
o ± Acquired etiologies (e.g., crossing vessels,
neoplasm, retroperitoneal inflammatory conditions)
and associated abnormalities (e.g., renal
malformation)
• CTA
o Use 3D reconstruction to better define vessels prior
to endoscopic pyelotomy
o 3D and multi planar reformations
• Useful in planning endopyelotomy
• Show relation of pelvis to adjacent vessels
MR Findings
• MRA: Detect crossing vessels
• MR Urography: ± Acquired etiologies
Ultrasonographic Findings
• Real Time
o Use US in both prenatal and postnatal evaluation
o Prenatal findings
• Anteroposterior (AP) pelvic diameter> 10 mm,
pelvic to AP renal diameter ratio> 0.5, caliectasis:
Fetal hydronephrosis
• Mild pyelectasis (pelvic diameter 4-10 mm in < 20
weeks of gestation and 5-10 mm in 20-24 weeks):
10-15% obstructed
• Pelvic or pyelocalyceal dilatation
• Oligo-, poly- or euhydroamnios
• ± Urine ascites
o Postnatal findings
• Large, medial sonolucent area (renal pelvis)
surrounded by smaller, rounded sonolucent areas
(dilated calyces)
• Assess severity and level of obstruction
• Advanced parenchymal atrophy
• Hypertrophy of normal kidney contralateral to
hydronephrotic kidney
Nuclear Medicine Findings
• Diuresis renography
o Separates obstructive from nonobstructive dilatation
o Localize level of obstruction
o Assess renal function, often pre-operatively
o "Homsy" sign: Delayed double-peak pattern; suggests
intermittent UP] obstruction
Imaging Recommendations
• Best imaging tool: IVP: Adult; US: Neonates and
children
• Protocol advice
o IVP
• Visualize UP] with prone oblique view; left/right
anterior oblique for left/right UP], respectively
• Diuresis IVP: Furosemide LV. 0.5 mg/kg 15-20
min. into IVP; film at 5, 10, 15 min. after
injection
o CTA: Use 3D reconstruction to better define vessels
and their relation to UP]
o US: Serial US should be done several days
postnatally due to relative neonatal oliguria
o Diuresis Renography: Tc99m labeled
mercaptoacetyltriglycine (MAG3) is preferred due to
lower radiation burden
I DIFFERENTIAL DIAGNOSIS
Urolithiasis
• Ureteral filling defects
• Most calculi are markedly hyperdense (> 200 HU)
• Usually acute; rarely cause prolong dilatation and
obliteration of renal pelvis and calyces
 URETEROPELVIC JUNCTION OBSTRUCTION
Tumor
• Benign or malignant retroperitoneal mass may cause
ureteral obstruction; use CT to evaluate
• Primary transitional cell carcinoma (TCe) near UP]
may cause obstruction
Extrinsic indentation
• E.g., crossing bands, crossing vessels
• "Linear band" sign
Retrocaval ureter
• Ureter courses behind and medial to inferior vena cava
• Obstruction at mid-ureter ~ dilatation proximally
3 from mid-ureter, not proximal
Renal pelvis blood clot
26
• E.g., anticoagulated patient, trauma
• May obstruct UP]
I PATHOLOGY
General Features
• General path comments: Obstruction caused by
spectrum of pathophysiological processes of varying
etiologies
• Genetics: Familial occurrences in some cases
• Etiology
o Congenital (most common)
• Intrinsic stenosis
• Adynamic segment
• Valves and folds
• Kinks or angulations
• Adhesions or bands
• Crossing vessels near UP]
• High "insertion" of ureter
o Acquired
• Scarring: Inflammation, surgery, trauma
• Vesicoureteral reflux
• Malignant neoplasm: Transitional cell carcinoma,
squamous cell carcinoma, metastasis
• Benign neoplasm: Polyp, mesodermal tumor
• Intraluminal lesion: Stone, clot, papilla, fungus
ball, cholesteatoma, bullet, miscellaneous
• Herniation of kidney: Through lumbodorsal fascia
defect, into Bochdalek hernia
• Epidemiology: Neonates: 40% of all significant
neonatal hydronephrosis (1/500 pregnancies)
• Associated abnormalities
o Cystic renal dysplasia, primary megaureter
o Lower or upper segment of duplex kidney
o Ectopic, malrotated, pelvic and horseshoe kidneys
o Complex congenital anomaly: VATER(vertebral,
anus, tracheoesophageal, renal and radial)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Neonates
• Asymptomatic, diagnosed by prenatal screening
• Palpable, sometimes visible abdominal mass
o Children and adults
• Intermittent abdominal or flank pain, nausea,
vomiting
• Hematuria, renovascular hypertension (rare)
• Lab data: Microhematuria, pyuria, urinary tract
infection
Demographics
• Age: Any age; less common in adults
• Gender
o Overall, M:F = 2:1
o In infants, M:F = 5:1
Natural History & Prognosis
• Complications: Failure to thrive, renal insufficiency,
urinary tract infection, urolithiasis, gastroduodenal
obstruction, traumatic or spontaneous kidney rupture
• Prognosis: Good, after treating unilateral obstruction
Treatment
• Indicated when patient has symptoms, stones,
infection or renal function is impaired or at risk
o Infants and children: Open pyeloplasty
o Adults: Endopyelotomy
• Follow-up: 3-6 months with diuresis renography
I DIAGNOSTIC CHECKLIST
Consider
• Use CT or MR to evaluate potential acquired etiologies
of UP] obstruction
Image Interpretation Pearls
• Chronic obstruction ~ progressive changes to renal
pelvis and calyces ~ distinctive urographic signs
I SELECTED REFERENCES
1. Khaira HS et al: Helical computed tomography for
identification of crossing vessels in ureteropelvic junction
obstruction-comparison with operative findings. Urology.
62(1):35-9, 2003
2. Mitsumori A et al: Evaluation of crossing vessels in patients
with ureteropelvic junction obstruction by means of
helical CT. Radiographics. 20(5):1383-93; discussion
1393-5, 2000
3. Rouviere 0 et al: Ureteropelvic junction obstruction: use of
helical CT for preoperative assessment--comparison with
intraarterial angiography. Radiology. 213(3):668-73, 1999
4. Farres MT et al: Helical CT and 3D reconstruction of
ureteropelvic junction obstruction: accuracy in detection
of crossing vessels. J Comput Assist Tomogr. 22(2):300-3,
1998
5. Siegel CL et al: Preoperative assessment of ureteropelvic
junction obstruction with endoluminal sonography and
helical CT. AJRAm J Roentgenol. 168(3):623-6, 1997
6. Quillin SP et al: Helical (spiral) CT angiography for
identification of crossing vessels at the ureteropelvic
junction. AJRAm J Roentgenol. 166(5):1125-30, 1996
7. Wolf JSJr et al: Imaging for ureteropelvic junction
obstruction in adults. J Endourol. 10(2):93-104, 1996
8. Bagley DH et al: Endoluminal sonography in evaluation of
the obstructed ureteropelvic junction. J Endourol.
8(4):287-92, 1994
9. Bush WH et al: Ureteropelvic junction obstruction:
treatment with percutaneous endopyelotomy. Radiology.
171(2):535-8,1989
 URETEROPELVIC JUNCTION OBSTRUCTION
I IMAGE GALLERY
Typical
(Left) Axial NECT shows
massive dilation of pelvis +
calices in patient with
congenital UP}obstruction.
(Right) Axial CECT shows
dilated renal pelvis,
caliectasis and parenchymal
atrophy of the right kidney.

3
27

Typical
(Left) Axial CECT shows left
side pelvocaliectasis due to
congenital UP}obstruction.
Cas due to ureteral stent
(arrow). (Right)
Volume-rendered 3D CT
image shows pigtail ureteral
stent marking the UP}plus
the adjacent arteries.

Typical
(Left) Retrograde pyelogram
shows severe
pelvocaliectasis. (Right) Axial
NECT shows perirenal
urinoma due to rupture of a
chronically dilated left renal
pelvis.
 PYELONEPHRITIS
~, -
,.

~''-:' ~." .•. ' 3'":- ~: ...• \

, 0'"

-
.-

-
"" .;
--r - --_ ...

3
28

Axial CECT shows a swollen right kidney with a striated

c-a y
Axial CECT shows subtle heterogeneity of right kidney
nephrogram. + thick enhancing uroepithelium indicating pyelitis.

o Acute pyelonephritis
ITERMINOLOGY • Global or focal enlargement
Definitions • Impaired excretion: Delayed appearance, I
• Infection of renal pelvis, tubules & interstitium (not density, I nephrogram
glomerulus) • Striated nephrogram or lucent areas (I filling);
streaking & blushing
• Calyceal compression, pelvicaliceal or ureteral
I IMAGING FINDINGS dilatation, ± calculi
o Chronic pyelonephritis
General Features • Contracted small sized kidney, I & delayed
• Best diagnostic clue: Wedge-shaped + striated areas of excretion, dilated ureter
I enhancement & renal swelling on CECT (acute) • Focal or diffuse calyceal clubbing or blunting +
• Location: Usually multifocal cortical scarring
• Morphology • Contralateral diffuse or focal compensatory
o Acute pyelonephritis: Enlarged kidney hypertrophy
o Chronic pyelonephritis: Scarred contracted kidney
CT Findings
• Other general features
• Acute pyelonephritis
o Tubulointerstitial disease of kidney (upper UTI)
o Renal enlargement, focal swelling, sinus obliteration
o Route of infection
o Thickening of Gerota fascia + perinephric stranding
• Ascending infection (> common); hematogenous
o ± Areas of 1 HU (hemorrhagic bacterial nephritis)
o Classification based on clinical onset & pathology
o Nephrographic phase: "Patchy" nephrogram
• Acute pyelonephritis
• Cortical wedge-shaped areas of decreased density
• Chronic pyelonephritis
(hypoperfusion, edema, hypo concentration)
• Emphysematous pyelonephritis
• Striated nephrogram
• Xanthogranulomatous pyelonephritis
• Loss of normal corticomedullary differentiation
Radiographic Findings • Focal severe pyelonephritis: Mimics renal
• IVP neoplasm
o Excretory phase

DDx: Striated or Wedge Defects on CT Nephrogram

Renal Infarct Renal Lymphoma Renal Trauma Polyarteritis Nodosa

 PYELONEPHRITIS
Key Facts
Terminology
• Infection of renal pelvis, tubules & interstitium (not
glomerulus)
Imaging Findings
• Best diagnostic clue: Wedge-shaped + striated areas of
I enhancement & renal swelling on CECT (acute)
• Characteristic appearance: Small kidney with cortical
scarring over dilated calices (chronic)
• Nonfunctional kidney or part of kidney with
obstructing calculi (XGPN)
• Necrosis + gas replacement of renal parenchyma with
little or no pus (emphysematous type)
Top Differential Diagnoses
• Renal infarction
• Renal trauma
• Streaky linear bands: Alternating 1 + I attenuation
• Due to I concentration of contrast in tubules from
ischemia + tubular obstruction by inflammatory
cells + debris
• Calyceal effacement, dilated renal pelvis & ureter
• Thickening of walls of renal pelvis, calyces} ureter
o Delayed phase (3-4 hrs)
• 1 Enhancement in previously low density
wedge-shaped zones
• Due to eventual filling of tubules that are partially
obstructed by surrounding interstitial
inflammatory edema
• Chronic pyelonephritis
o Deep cortical scarring: Focal, segmental, diffusei
unilateral or bilateral
o Atrophy: Focal (> in upper pole) or diffuse
o Unilateral with compensatory hypertrophy of
contralateral kidney
o Characteristic appearance: Small kidney with
cortical scarring over dilated calices (chronic)
o Loss of corticomedullary differentiation
• Xanthogranulomatous pyelonephritis (XGPN)
o Nonfunctional kidney or part of kidney with
obstructing calculi (XGPN)
o Low attenuation collections: Foot print of a bear
paw (markedly dilated collecting system filled with
pus, xanthoma cells + mildly dilated pelvis)
o Lack of contrast excretion + bright rim
enhancement (due to capillary proliferation in
granulation tissue)
o Thickened Gerota fascia, perinephric soft tissue
stranding, abscess, extension into abdominal wall
• Emphysematous pyelonephritis
o Necrosis + gas replacement of renal parenchyma
with little or no pus (emphysematous type)
• Occurs almost exclusively in diabetics
• Due to infarction & infection with gas forming
organisms
• Usually requires urgent nephrectomy
Ultrasonographic Findings
• Real Time
o Acute pyelonephritis
• Vasculitis
Pathology
• Gram negative: E. coli, proteus, klebsiella,
Enterobacter (from fecal flora)
• Vesicoureteral reflux (VUR)i obstructive uropathy
• Risk factor ~ ascending infection (most common)
• 1 Incidence: Women under age 40, men above 65
• Associated abnormalities: BPHi VURi UT obstruction
Diagnostic Checklist
• Distinction of pyelonephritis from vasculitis or renal
infarction often requires clinical correlation
• Wedge-shaped parenchymal defects with renal
3
swelling, usually acute pyelonephritis 29
• Defects with cortical scarring usually chronic
pyelonephritis, vasculitis or infarction
• Normal or swollen kidney with I echogenicityi
loss of sinus echoes
• Wedge-shaped hypo-/isoechoic zoneSi
hyperechoic (hemorrhage)
• Blurred corticomedullary junctionsi ± anechoic
areas (abscesses)
Nuclear Medicine Findings
• Tc99m-DMSA or glucoheptonate cortical imaging
o Decreased uptake in foci of inflammation
o Used in follow-up of renal scarring
o Highly sensitive in diagnosing acute pyelonephritis
o Dimercaptosuccinic acid (DMSA) renal SPECT
• > Sensitive than planar scintigraphy in children
• Indium-Ill labeled leukocytes
o Normally do not accumulate in kidneys
o More specific than gallium (detecting inflammation)
o Increased uptake
• Acute focal or diffuse pyelonephritisi renal abscess
Imaging Recommendations
• Helical NE + CECT
.• Indium-Ill labeled leukocytes
o For early detection of renal & perinephric infection
o When renal ultrasonography is normal in a
clinically positive patient
I DIFFERENTIAL DIAGNOSIS
Renal infarction
• Focal segmental infarction
o Sharply demarcated, nonenhancing wedge-shaped
• Global infarction
o Total absence of renal enhancement, no excretion
o ± Medullary striations: "Spoke wheel" enhancement
• Due to collateral circulation
• Acute infarction
o Normal or large kidney with smooth contour
o Absent or I nephrogram + cortical enhancement
o "Cortical rim" sign on CECT (6-8 hrs after infarction)
• Preserved capsular & subcapsular enhancement
 PYELONEPHRITIS
lymphoma/metastases
• Multifocal tumor may simulate multifocal
pyelonephritis
• Unifocal tumor may simulate focal severe
pyelonephritis
• Clinical presentation usually characteristic
Renal trauma
• Best imaging clue
o Irregular linear or segmental nonenhancing tissue &
subcapsular or perinephric hematoma
• Subcapsular hematoma
o Round or elliptic fluid collection (40-70 HU clot)
3 • Lacerations: Irregular or linear hypodense areas
• Segmental infarction: Nonenhancing wedge-shaped
30 • "Shattered kidney"
o Renal artery avulsion
• Global infarction + perinephric hematoma
o Renal artery thrombosis
• Global infarction + no perinephric hematoma
Vasculitis
• E.g., polyarteritis nodosa; SLE;scleroderma, drug abuse
• Wedge-shaped or striated nephrogram
• Key differential
o Vasculitis: Capsular retraction over parenchymal
lesions
o Acute pyelonephritis: Capsular bulge over
parenchymal lesions
• Microaneurysms of small vessels are commonly seen
!PATHOLOGY
General Features
• Etiology
o Gram negative: E. coli, proteus, klebsiella,
Enterobacter (from fecal flora)
o Predisposing or increased risk factors
• Vesicoureteral reflux (VUR); obstructive uropathy
• Pregnancy, benign prostatic hypertrophy (BPH)
• Urethral instrumentation
• Diabetes mellitus & other renal pathology
o Pathogenesis
• Risk factor ~ ascending infection (most common)
• Hematogenous infection (less common)
• Epidemiology
o Incidence of urinary tract infection (UTI)
• t Incidence: Women under age 40, men above 6S
• Associated abnormalities: BPH; VUR; UT obstruction
Gross Pathologic & Surgical Features
• Acute pyelonephritis
o "Polar abscesses": Microabscesses on renal surface
• Lower & upper poles are most common
o Narrowed calyces, enlarged kidney
• Chronic pyelonephritis
o Blunted calyces + scarred shrunken kidney
Microscopic Features
• Acute pyelonephritis
o Interstitial or tubular necrosis
o Mononuclear cell infiltrate + fibrosis
• Chronic pyelonephritis
o Chronic inflammation, atrophy, interstitial fibrosis
• Xanthogranulomatous pyelonephritis
o Foamy, lipid-laden histiocytes; pus cells & necrosis
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Acute pyelonephritis
• Fever, malaise, dysuria, flank pain & tenderness
• Lab data
o t ESR; t WBC; t proteinuria
o Positive urine culture for bacilli; impaired RFTs
• Diagnosis: Clinical findings, imaging & biopsy
Demographics
• Age: Common in adults (also seen in children)
• Gender: Females under age 40 & males above age 6S
Natural History & Prognosis
• Complications
o Renal abscess, perinephric abscess, pyonephrosis
o Renal papillary necrosis (RPN)
o Renal atrophy (focal or global), renal failure
• Prognosis
o Acute pyelonephritis: Good
o Chronic, XGPN, emphysematous types: Poor
Treatment
• Acute: Antibiotic therapy
• Chronic: Treat reflux & obstruction; nephrectomy
I DIAGNOSTIC CHECKLIST
Consider
• Distinction of pyelonephritis from vasculitis or renal
infarction often requires clinical correlation
Image Interpretation Pearls
• Wedge-shaped parenchymal defects with renal
swelling, usually acute pyelonephritis
• Defects with cortical scarring usually chronic
pyelonephritis, vasculitis or infarction
I SELECTED REFERENCES
1. Kawashima A et al: Radiologic evaluation of patients with
renal infections. Infect Dis Clin North Am. 17(2):433-56,
2003
2. Kawashima A et al: CT of renal inflammatory disease.
Radiographies. 17(4):851-66; discussion 867-8, 1997
3. Saunders HS et al: The CT nephrogram: implications for
evaluation of urinary tract disease. Radiographies.
15(5):1069-85; discussion 1086-8, 1995
4. Talner LB et al: Acute pyelonephritis: Can we agree on
terminology? Radiology 192: 297-305, 1994
5. Soulen MC et al: Bacterial renal infection: Role of CT.
Radiology 171: 703-707, 1989
6. Morehouse HT et al: Imaging in inflammatory disease of
the kidney. AJRAm J Roentgenol. 143(1):135-41, 1984
 PYELONEPHRITIS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
focal severe pyelonephritis
with mass-like swelling and a
striated nephrogram. (Right)
Axial CECT shows severe
focal pyelonephritis with
decreased enhancement and
focal swelling of right kidney.

3
31

Typical
(Left) Axial CECT shows
result of chronic
pyelonephritis with cortical
loss over a dilated calyx in
the left kidney (arrow).
(Right) Axial CECT shows
effects of chronic
pyelonephritis with global
atrophy of left kidney and
proliferation of perirenal fat.

(Left) Axial CECT shows

heterogeneous
parenchymograms
bilaterally. more subtle due
to corticomedullary phase of
scan. (Right) Axial CECT
shows wedge-shaped and
striated nephrogram, plus
inflamed uroepithelium and
perirenal infiltration.
 RENAL ABSCESS

3
32

Axial CECT shows an encapsulated nonenhancing renal Sagittal sonogram shows spherical mass with low level
mass with an attenuation of 20 HU; abscess. echogenicity and acoustic enhancement.

ITERMINOlOGY CT Findings
Definitions • NECT
o Single (more common) or multiple; unilateral or
• Localized collection of pus caused by suppurative bilateral
necrosis in kidney
o Round, well-marginated, low-attenuation masses
o ± Gas within collection
• CECT
I IMAGING FINDINGS o Enlarged kidney with focal areas of hypoattenuation
General Features (acute)
o "Rim or ring" sign: Enhancement of abscess wall
• Best diagnostic clue: Spherical non enhancing renal
mass with perinephric stranding on CECT (subacute or chronic)
o No central enhancement of lesion; enhancement of
• Other general features
normal renal tissue
o Progress to perinephric abscess: Extension of renal
abscess through capsule o Obliteration of renal sinus or calyceal effacement
o Thickened walls and mild dilatation of renal pelvis
Radiographic Findings and ureter
• IVP o Perinephric reaction or extension
o Impaired excretion • Altered renal contour, indistinct renal outline or
• Delayed appearance time, decreased contrast renal displacement
density or decreased nephrogram • Edema or obliteration of perinephric fat
• ± Absence of nephrogram and calyceal • Thickened Gerota fascia and perinephric septa
opacification
MR Findings
o Heterogeneous nephrogram
o Single or multiple, well-defined, round or irregular • Tl WI: Hypointense mass
lucent mass • T2WI: Hyperintense mass and increased signal
surrounding the mass (perilesional edema)
o Calyceal or pelvic effacement
o ± Calyceal, pelvic or ureteral dilatation • Tl C+: Shows rim-enhancement (lesion < 1 cm
enhances homogeneously)

DDx: Thick-walled Cystic or Necrotic lesion

Cystic RCC Lymphoma Multiple Myeloma Hemorrhagic Cyst

 RENAL ABSCESS

Key Facts
Terminology Pathology
• Localized collection of pus caused by suppurative • Accounts for 2% of all renal masses
necrosis in kidney • Sequelae of acute renal infections
• Urinary tract infection ~ ascends to kidney ~ acute
Imaging Findings pyelonephritis or acute focal bacterial nephritis ~
• Best diagnostic clue: Spherical nonenhancing renal liquefaction ~ sequestration ~ renal abscess
mass with perinephric stranding on CECT
• ± Gas within collection Clinical Issues
• "Rim or ring" sign: Enhancement of abscess wall • Fever, flank or abdominal pain, chills and dysuria
(subacute or chronic) • Antibiotic therapy
• No central enhancement of lesion; enhancement of
normal renal tissue Diagnostic Checklist
• "Rim" sign; Absence of lesion enhancement; 3
Top Differential Diagnoses perinephric stranding
• Renal carcinoma (RCC) 33
• Metastases and lymphoma
• Infected or hemorrhagic cyst

Ultrasonographic Findings Metastases and lymphoma

• Real Time
o Anechoic or hypoechoic to echogenic fluid
collection that blends with the normal echogenic fat
within Gerota fascia
o Mass within or displacing kidney
o Round, thickened, or smooth-walled complex mass
o Low level internal echoes move with change of
position (internal debris)
o "Comet sign": Internal echogenic foci (gas within
abscess)
• Associated posterior "dirty" shadowing
o ± Internal septations or loculations
Angiographic Findings
• Conventional: Peripheral distribution and fine
neovascular pattern
Nuclear Medicine Findings
• WBC Scan
o 1 Uptake of indium-Ill labeled leukocytes noted
within renal ± perinephric abscess
o Possible false negative leukocyte scans: Prior
antibiotic therapy, walled-off abscesses or poorly
developed inflammatory responses
o More sensitive or specific in early detection of renal
± perinephric infection
Imaging Recommendations
• NECT and CECT
o To distinguish abscess from tumor
I DIFFERENTIAL DIAGNOSIS
Renal carcinoma (RCC)
• CT: Enhancement of solitary mass
• Angiography: Hypervascularity
• Symptoms are rare, usually asymptomatic
• 25-40% diagnosed by incidental findings on CT or US
• 25-30% present with metastases (e.g., lung,
mediastinum, bone, liver)
• Clinical history and urinalysis can differentiate
• Metastases (e.g., lung cancer, breast cancer,
gastrointestinal cancer, malignant melanoma)
o Almost all develop via hematogenous spread
o Lung, breast and colon carcinoma can occasionally
be large and solitary; difficult to differentiate from
renal carcinoma
oCT: Multifocal, small, enhancing (5-30 HU) nodules;
widespread nonrenal metastases
o Angiography: Hypovascular pattern
o Asymptomatic (most common) or flank pain and
hematuria
o CT or US guided biopsy for pathologic confirmation
• Lymphoma
oCT: Multiple distinct masses (45%); direct invasion
from enlarged retroperitoneal nodes (25%); solitary
mass (15%); diffuse infiltration (10%);
predominantly perinephric involvement (5%)
o Asymptomatic (most common), fever, weight loss,
flank pain, hematuria and renal failure
Infected or hemorrhagic cyst
• May be indistinguishable on imaging; solitary,
non enhancing lesion
• CT: Absence of perinephric stranding, "rim" sign,
shaggy wall, hyperintense mass
[PATHOLOGY
General Features
• General path comments
o Accounts for 2% of all renal masses
o Sequelae of acute renal infections
• Acute pyelonephritis or focal bacterial nephritis
• Usually seen 1 to 2 weeks after infection
• Etiology
o Ascending urinary tract infections (80%)
• Calculi, obstruction, renal anomalies and urinary
reflux (diabetes or pregnancy)
• Iatrogenic intervention (catheterization)
 RENAL ABSCESS
• Gram negative organisms (E. coli, Proteus species
or Klebsiella species)
• Abscesses likely form at corticomedullary junction
o Hematogenous spread (20%)
• LV. drug users and skin infection
• Hematogenous seeding from other infected sites
(e.g., valvular heart disease, prosthesis)
• Iatrogenic intervention (cyst aspiration,
embolization of kidney vessels)
• Gram positive and negative organisms
(Staphylococcus aureus, Streptococcus species or
species within Enterobacteriaceae family)
• Abscesses likely form at renal cortex
o Risk factors: Diabetes Mellitus, long term
3 hemodialysis and intravenous drug users
o Pathogenesis
34
• Urinary tract infection ~ ascends to kidney ~
acute pyelonephritis or acute focal bacterial
nephritis ~ liquefaction ~ sequestration ~ renal
abscess
• Epidemiology: Incidence: Renal abscess (0.2%);
perinephric abscess (0.02%)
• Associated abnormalities: 20-60% of patients with
renal or perinephric abscess have urolithiasis
Gross Pathologic & Surgical Features
• Well-defined, round, thickened or smooth-walled
mass
Microscopic Features
• Infected and necrotic tissue; ± gas
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Fever, flank or abdominal pain, chills and dysuria
o Symptoms are longer than 2 weeks
o Costovertebral angle tenderness and palpable flank
mass
• Lab data
o Urinalysis: Elevated white blood cells (WBe) (75%),
positive bacterial culture (33%)
o Blood tests: Elevated erythrocyte sedimentation rate
(ESR),positive bacterial culture (50%)
Demographics
• Age: All ages
• Gender: M = F
Natural History & Prognosis
• Complications
o Rupture ~ into perinephric space (perinephric
abscess) ~ beyond Gerota fascia (paranephric
abscess) ~ psoas or transversalis muscles ~ anterior
peritoneal cavity ~ subdiaphragmatic or pelvic
abscess
o Rupture ~ renal collecting system ~ pyonephrosis
o Compression or obstruction ~ hydronephrosis ~
renal atrophy
o Necrosis and cavitation
• Prognosis
o Good, in early diagnosis and treatment
o Poor, in delayed diagnosis and treatment
Treatment
• Antibiotic therapy
o If causative organisms are known, use specific
antibiotics
o If unknown, treat empirically with broad-spectrum
antibiotics (ampicillin or vancomycin with
aminoglycoside or third-generation cephalosporin)
• If abscess not resolved within 48 hours after treatment
with antibiotics, do percutaneous aspiration &
drainage under CT or US
o Well-defined mass on CT or fluid-filled mass on US
indicate a "ripe" abscess for drainage
• If abscess still not resolved, open surgical drainage or
nephrectomy
• Follow-up
o Imaging to confirm resolution of abscess
o Evaluate for underlying urinary tract abnormalities
I DIAGNOSTIC CHECKLIST
Consider
• Clinical history and urinalysis to diagnose and
differentiate from malignancy
Image Interpretation Pearls
• "Rim" sign; Absence of lesion enhancement;
perinephric stranding
I SELECTED REFERENCES
1. Dalla Palma L et al: Medical treatment of renal and
perirenal abscesses: CT evaluation. Clin Radiol.
54(12):792-7, 1999
2. Yen DR et al: Renal abscess: early diagnosis and treatment.
AmJ Emerg Med. 17(2):192-7, 1999
3. Kawashima A et al: CT of renal inflammatory disease.
Radiographies. 17: 851-866, 1997
4. Davidson AJ et al: Radiologic assessment of renal masses:
Implications for patient care. Radiology 202: 297-305, 1997
5. Brown ED et al: Renal abscesses: appearance on
gadolinium-enhanced magnetic resonance images. Abdom
Imaging. 21(2):172-6, 1996
6. Siegel JF et al: Minimally invasive treatment of renal
abscess. J Urol. 155(1):52-5, 1996
7. Rinder MR: Renal abscess: an illustrative case and review of
the literature. Md Med J. 45(10):839-43, 1996
8. Talner LB et al: Acute pyelonephritis: can we agree on
terminology? Radiology 192: 297-305, 1994
9. Goldman SM et al: Upper urinary tract infection: the
current role of CT, ultrasound, and MRI. Semin Ultrasound
CT MR. 12(4):335-60, 1991
10. Soulen MC et al: Sequelae of acute renal infections: CT
evaluation. Radiology. 173(2):423-6, 1989
11. Soulen MC et al: Bacterial renal infection: role of CT.
Radiology. 171(3):703-7, 1989
12. Edelstein R et al: Perinephric abscess. Modern diagnosis
and treatment in 47 cases. Medicine (Baltimore).
67(2):118-31, 1988
13. Jeffrey RB et al: CT and ultrasonography of acute renal
abnormalities. Radiol Clin North Am. 21(3):515-25, 1983
14. Roddick W et al: CT and sonography of severe renal and
perirenal infections. AJRAmJ Roentgenol. 140(3):517-20,
1983
15. Thornbury JR: Acute renal infections. Urol Radiol.
12(4):209-13, 1991
 RENAL ABSCESS

I IMAGE GAllERY
Typical
(Left) Axial CECTshows
heterogeneous mass (arrow)
simulating tumor; early
abscess + pyelonephritis.
(Right) Axial CECT in a
patient with pyelonephritis +
early abscess (left kidney)
shows striated and
wedge-shaped defects in
right kidney.
3
35

Typical
(Left) Axial CECT shows low
density mass in right kidney
plus perirenal infiltration due
to abscess. (Right) Axial
CECT in a young woman
(one week postpartum)
shows large low density,
nonenhancing renal abscess.

Typical
(Left) Axial CECT shows
renal and perirenal abscess.
(Right) Axial NECT shows
placement of a pigtail
catheter for abscess
drainage, obviating surgery.
 XANTHOGRANULOMATOUS PYELONEPHRITIS

3
36

Axial CECT shows large calculus in renal pelvis (arrow), Axial CECT shows proliferated fibrofatty tissue in
atrophic kidney (open arrow), and proliferation of perirenal space with infection and inflammation
perirenal fibrofatty tissue extending into back extending into back musculature.
musculature.

• Other general features

ITERMINOlOGY o 3 stages of XGP
Abbreviations and Synonyms • Stage 1: Lesion confined to kidney
• Xanthogranulomatous pyelonephritis (XGP) or • Stage 2: Lesion extends to perirenal space
(XGPN) • Stage 3: Lesion spreads to pararenal spaces ±
abdominal wall
Definitions
Radiographic Findings
• Chronic infection of kidney and surrounding tissues
characterized by destruction and replacement of renal • IVP
parenchyma by lipid-laden macrophages o Diffusely or focally absent nephrogram
o Contracted pelvis; dilated calices
o Centrally obstructing calculusi staghorn (75%)
I IMAGING FINDINGS • Retrograde pyelography
o Complete obstruction at ureteropelvic junctioni
General Features infundibulum; proximal ureter
• Best diagnostic clue: Obstructing calculus with o Contracted renal pelvis; dilated deformed calices
atrophic or nonfunctioning kidney and perirenal with nodular filling defects
fibrofatty proliferation o Irregular parenchymal masses with cavitation
• Location CT Findings
o Unilateral (most cases) > bilateral
• Multiple, focal low attenuating (-10 to +30 HU) masses
o 2 forms of XGP
scattered throughout involved portions of kidneYi
• Diffuse (83-90%): Involves entire kidney
represent dilated, debris-filled calyces & xanthoma
• Segmental or focal (10-17%): Tumefactive due to
collections
obstructed single infundibulumi one moiety of
• Bright enhancement of rims of xanthoma collections
duplex system
due to inflammatory hypervascularity; no
• Size: 2.5-5.8 cm; mean is 3.8 cm enhancement of collections
• Morphology: Well-circumscribed mass with global or
• Poor or no excretion of contrast into collecting system
focal renal enlargement
(nonfunctioning kidney)

DDx: Decreased Function and Mass

Renal Cell Carcinoma TCC Lymphoma Chronic Pyelonephritis

 XANTHOGRANULOMATOUS PYELONEPHRITIS

Key Facts
Terminology Pathology
• Chronic infection of kidney and surrounding tissues • Accumulation of lipid-laden "foamy" macrophages
characterized by destruction and replacement of renal (xanthoma cells) and granulomatous infiltrate
parenchyma by lipid-laden macrophages
Clinical Issues
Imaging Findings • Nephrectomy usually required
• Best .diagnostic clue: Obstructing calculus with
atrophic or nonfunctioning kidney and perirenal Diagnostic Checklist
fibrofatty proliferation • Some variations may occur (Le., small kidney, lack of
• Contracted pelvis; dilated calices calculi); difficult to distinguish XGP from other
• Centrally obstructing calculus; staghorn (75%) infections or neoplasms
• Histologic diagnosis must be made; cannot base
Top Differential Diagnoses solely on radiographic imaging studies 3
• Renal cell carcinoma • Poor or no excretion of contrast from kidney;
• Transitional cell carcinoma staghorn calculi 37
• Renal metastases and lymphoma
• Renal abscess

• Renal sinus fat obliterated (inflammation)

• Perinephric extension (14%): Extension of
I DIFFERENTIAL DIAGNOSIS
inflammation into perirenal space; pararenal space, Renal cell carcinoma
ipsilateral psoas muscle, colon, spleen, diaphragm, • Solitary, soft tissue density mass (30-50 HU range)
posterior abdominal wall and skin with with central necrosis
• Large central calculus, often staghorn • Hypervascular mass at renal cortex
• Gas may rarely be seen
Transitional cell carcinoma
MR Findings
• Renal infiltration ~ calyceal invasion, renal
• Thin rim of renal parenchyma enlargement and poorly defined margins without
• Loss of corticomedullary differentiation change in shape
• Dilated collecting system and surrounding renal • Renal pelvic filling defect, irregular narrowing of
parenchyma are of intermediate to high signal collecting system
intensity on both T1 & T2WI (1 fat content in
• Encasing pelvicaliceal system ~ hypovascular tumor
macrophages)
• Renal calculi: Negative defects within dilated Renal metastases and lymphoma
collecting system on T2WI • Metastases (e.g., lung cancer, breast cancer, colon
cancer, malignant melanoma)
Ultrasonographic Findings
o Usually hypovascular with infiltrative growth
• Real Time .
• Lymphoma
o Multiple anechoic or hypoechoic masses replacing
o Usually multiple or bilateral with infiltrative growth
normal corticomedullary differentiation and
o Hypoechoic, hypovascular, solitary, intrarenal mass
contracted pelvis
± adenopathy
o Peripelvic fibrosis may obscure acoustic shadowing
from central staghorn calculus Renal abscess
o Parenchymal thinning and hydronephrosis • Solitary or multiple, round, well-marginated, low
o Sonographic signs of chronic obstructive uropathy attenuation mass
caused by stones; echoes in the dilated collecting • Enhancement of walls without central enhancement
system • Perinephric stranding
o Perinephric fluid collection • ± Presence of gas
Angiographic Findings Hyd roneph rosis/pyoneph rosis
• Conventional • Pus-filled collecting system may simulate XGP
o Stretching of segmental/interlobar arteries around
large avascular masses
o Hypervascularity/blush around periphery of masses !PATHOLOGY
in late arterial phase (granulation tissue)
o Venous encasement with occlusion General Features
• General path comments
Imaging Recommendations o Usually found in setting of chronic obstruction (e.g.,
• Best imaging tool: CT calculus, stricture, carcinoma)
o Often renal pelvis less dilated than expected for high
grade chronic obstruction
• Etiology
 XANTHOGRANULOMATOUS PYELONEPHRITIS
o Escherichia coli • Nephrectomy usually required
o Proteus mirabilis o Radical nephrectomy in complete XGP
o Staphylococcus aureus o Partial nephrectomy in focal XGP
o Klebsiella species
o Pseudomonas species
o Enterobacter species I DIAGNOSTIC CHECKLIST
o Risk factors
• Recurrent or chronic urinary tract infections Consider
• Immunocompromised patients: Diabetes mellitus • Some variations may occur (Le., small kidney, lack of
• Abnormal lipid metabolism calculi)i difficult to distinguish XGP from other
o Pathogenesis infections or neoplasms
• Chronic renal obstruction and infection with • Histologic diagnosis must be madei cannot base solely
failure of local host immunity on radiographic imaging studies
• Epidemiology: Incidence: 1% all renal infections
3 • Associated abnormalities: Urolithiasis
Image Interpretation Pearls
• Poor or no excretion of contrast from kidney; staghorn
38 Gross Pathologic & Surgical Features calculi
• Diffuse form
o Renal enlargement with indurated or thickened
perinephric fat I SELECTED REFERENCES
o Dilated renal pelvis with staghorn calculus 1. Kim J: Ultrasonographic features of focal
o Replacement of corticomedullary junction with soft xanthogranulomatous pyelonephritis. J Ultrasound Med.
yellow nodulesi calices filled with pus and debris 23(3):409-16,2004
• Focal form 2. Cakmakci H et al: Pediatric focal xanthogranulomatous
o Yellowish white, solid or semisolid renal mass pyelonephritis: dynamic contrast-enhanced MRI findings.
Clin Imaging. 26(3):183-6, 2002
Microscopic Features 3. Tiu CM et al: Sonographic features of
• Accumulation of lipid-laden "foamy" macrophages xanthogranulomatous pyelonephritis. J Clin Ultrasound.
29(5):279-85, 2001
(xanthoma cells) and granulomatous infiltrate
4. Kim JC: US and CT findings of xanthogranulomatous
• Foam cells contain neutral fat and cholesterol (ester pyelonephritis. Clin Imaging. 25(2):118-21, 2001
granules)i positive for periodic acid-Schiff (PAS)stain 5. Verswijvel G et al: Xanthogranulomatous pyelonephritis:
• Diffuse infiltration by plasma cells and histiocytes MRI findings in the diffuse and the focal type. Eur Radiol.
10(4):586-9,2000
6. Fan CM et al: Xanthogranulomatous pyelonephritis. AJR
I CLINICAL ISSUES 7.
AmJ Roentgenol. 165(4):1008, 1995
Eastham J et al: Xanthogranulomatous pyelonephritis:
Presentation clinical findings and surgical considerations. Urology.
43(3):295-9, 1994
• Most common signs/symptoms Rabushka LS et al: Pictorial review: computed tomography
8.
o Dull or persistent flank pain, fever of renal inflammatory disease. Urology. 44(4):473-80, 1994
o Palpable mass, weight loss 9. Chuang CK et al: Xanthogranulomatous pyelonephritis:
• Lab data experience in 36 cases.J Urol. 147(2):333-6, 1992
o Urinalysis: Microscopic hematuria, proteinuria, 10. Hayes WS et al: From the Archives of the AFIP.
pyuria Xanthogranulomatous pyelonephritis. Radiographies.
o Urine culture: Specific bacterial species 11(3):485-98, 1991
o Liver function tests: Elevated 11. Mulopulos GP et al: MR imaging of xanthogranulomatous
pyelonephritis. J Comput Assist Tomogr. 10(1):154-6, 1986
o Erythrocyte sedimentation rate: Elevated Goldman SM et al: CT of xanthogranulomatous
12.
Demographics pyelonephritis: radiologic-pathologic correlation. AJR Am J
Roentgenol. 142(5):963-9, 1984
• Age 13. Bissada NK et al: Preoperative diagnosis of
o Any age: 45-65 years of age most common xanthogranulomatous pyelonephritis. Urology.
o Very rare in childhoodi focal form is more common 7(2):228-30, 1976
in children 14. Cha EM et al: Xanthogranulomatous pyelonephritis.
• Gender: M:F = 1:3-4 Angiographic evaluation. Urology. 3(2):159-62, 1974
15. Vinik M et al: Xanthogranulomatous pyelonephritis:
Natural History & Prognosis angiographic considerations. Radiology. 92(3):537-40, 1969
• Symptomatic for 6 months prior to diagnosis (40% of
cases)
• Complications: Hepatic dysfunction (reversible),
extrarenal extension, fistulas (e.g., pyelocutaneous,
ureterocutaneous), hemorrhage
• Prognosis: Good, mortality is rare, morbidity can be
substantial
Treatment
• Antibiotics: Treatment prior to surgical intervention
 XANTHOGRANULOMATOUS PYELONEPHRITIS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
enlarged right kidney with
minimal function, large
calculus and perirenal
fibrofatty proliferation.
(Right) Axial CECT shows
enlarged right kidney with
infiltrative "mass" in dorsal
segment and extensive
perirenal inflammation.

3
39

Typical
(Left) Renal scintigram
71 n ,.
" '" (posterior view) shows
GlD CBD ®D @D I}lD minimal function of right
kidney. (Right) Tc MOP

-_-
renogram shows minimal
perfusion or function of right
Re~<>9rom
kidney due to XCP

.........

13ODOO ....... ....

:>
!- ....
.!'- .. .......

I
•• 54

Typical
(Left) Axial CECT shows
staghorn calculus and
nonfunctional left kidney.
Extensive retroperitoneal and
body wall
inflamma tion /infection
(arrows). (Right) Axial CECT
shows calculus lying outside
kidney in pocket of chronic
perirenal inflammation due
to XCP
 EMPHYSEMATOUS PYELONEPHRITIS

3
40

Axial NECT shows gas replacement of infarcted, Axial NECT shows almost complete replacement of the
infected renal parenchyma. right kidney by gas, segmental gas in the left kidney, and
gas dissecting through retroperitoneum. Fatal EPN in
diabetic.

• ± Crescent of subcapsular or perinephric gas

ITERMINOLOGY o Type II (66%)
Abbreviations and Synonyms • Renal or perirenal fluid abscesses with bubbly gas
• Emphysematous pyelonephritis (EPN) pattern ± gas within renal pelvis
• Mottled j attenuation areas extend radially along
Definitions pyramids
• Life-threatening, fulminant, necrotizing upper urinary • Intraparenchymal, intracaliceal and intrapelvic gas
tract infection (UTI) associated with gas within kidney • Gas often extends into subcapsular, perinephric,
pararenal, contralateral retroperitoneal spaces
• ± Gas extends into renal veins and inferior vena cava
I IMAGING FINDINGS MR Findings
General Features • T1WI, T2WI: Void of signal
• Best diagnostic clue: Gas in renal parenchyma on CT Ultrasonographic Findings
• Location
o Unilateral> bilateral (5-7% of cases) • Real Time
o Highly echogenic areas within renal sinus and
o Left (52%) > right (43%)
parenchyma with "dirty" shadowing
Radiographic Findings o Ring-down artifacts: Air bubbles trapped in fluid
• Radiography: Gas in parenchyma ± paranephric space o Gas in perinephric spacei may obscure kidney
• IVP (generally contraindicated) Imaging Recommendations
o Renal enlargement with delayed or absent excretion
• Best imaging tool: CT
o Renal edema with destruction of renal parenchyma

CT Findings
• 2 types of EPN I DIFFERENTIAL DIAGNOSIS
o Type I (33%) (true EPN)
Emphysematous pyelitis
• Parenchymal destruction without fluidi streaky or
mottled gas radiating from medulla to cortex • Gas in renal pelvis & calices, not parenchyma

DDx: Gas in or Around Kidney

Perforated Ulcer Nephrostomy Nephrostomy Acute Infarction

 EMPHYSEMATOUS PYELONEPHRITIS

Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Gas in renal parenchyma on CT • Emphysematous pyelitis
• Parenchymal destruction without fluid; streaky or • Perforated duodenal ulcer
mottled gas radiating from medulla to cortex • Iatrogenic
• Renal or perirenal fluid abscesses with bubbly gas
pattern ± gas within renal pelvis Diagnostic Checklist
• Best imaging tool: CT • Exclude EPN in diabetics with pyelonephritis
• Type I (true EPN) is an medical and surgical
emergency

• 50% diabetics; less grave prognosis than EPN • Prognosis: Poor

Perforated duodenal ulcer
• Occasionally, gas "outlines" the kidney
Iatrogenic
• E.g., retrograde pyelography, nephrostomy
• Chemoembolization or ablation of renal tumor with
sudden infarction of tissue
I PATHOLOGY
General Features
• Etiology
o Single or mixed organism(s) infection
• E. coli (68%), Klebsiella pneumoniae (9%)
• Proteus mirabilis, Pseudomonas, Enterobacter,
Candida, Clostridia species
o Risk factor
• Recurrent or chronic UTIs
• Immunocompromised: Diabetes mellitus (87-97%)
• Ureteral obstruction (20-40%): Calculi, stenosis
• Renal failure: Polycystic kidney, end-stage
o Pathogenesis
• Pyelonephritis ~ ischemia and low oxygen
tension ~ facultative anaerobes proliferation in an
anaerobic environment ~ C02 production
• Epidemiology: Very rare
Gross Pathologic & Surgical Features
• Suppurative necrotizing infection of renal parenchyma
and perirenal tissue with multiple cortical abscesses
o Mortality
3
• 66% with type I, 18% with type II 41
• 60-75% with antibiotic therapy
• 21-29% after antibiotic therapy and nephrectomy
• 80% if extends into perinephric space
Treatment
• Antibiotic therapy; nephrectomy for type I
• CT-guided drainage procedures: Safe, quick and
life-saving with 70% success rate, only for type II
I DIAGNOSTIC CHECKLIST
Consider
• Exclude EPN in diabetics with pyelonephritis
Image Interpretation Pearls
• Type I (true EPN) is an medical and surgical emergency
I SELECTED REFERENCES
1. Grayson DE et al: Emphysematous infections of the
abdomen and pelvis: a pictorial review. Radiographics.
22(3):543-61, 2002
2. Wan YL et al: Acute gas-producing bacterial renal
infection: correlation between imaging findings and
clinical outcome. Radiology. 198(2):433-8, 1996
3. Joseph RC et al: Genitourinary tract gas: imaging
evaluation. Radiographics. 16(2):295-308, 1996
4. Rodriguez-de- Velasquez A et al: Imaging the effects of
diabetes on the genitourinary system. Radiographics.
15(5):1051-68, 1995

I CLINICAL ISSUES
jlMAGE GALLERY
Prese nta ti 0 n
• Most common signs/symptoms
o Fever, chills, flank pain, lethargy, confusion
o Nausea, vomiting, dyspnea, crepitant mass
• Lab data
o Hyperglycemia, acidosis, electrolyte imbalance,
thrombocytopenia
o Blood, urine, aspiration culture: Positive
Demographics
• Age: 19-81 years of age; mean 54 years of age
• Gender: M:F = 1:2-6
Natural History & Prognosis (Left) Radiograph shows gas in and around right kidney (arrows).
• Complications: Sepsis (Right) Axial NEeT shows gas in and around a transplanted kidney in
the iliac fossa, due to emphysematous pyelonephritis.
 HIV NEPHROPATHY

3
42

Sonography of HIV nephropathy. Transverse scan Sagittal sonogram of HIV nephropathy demonstrates
demonstrates markedly echogenic renal parenchyma enlarged kidney with loss of cortico-medullary interface
(arrow). (arrow).

o Abnormally increased echogenicity (89%), globular

ITERMINOlOGY renal morphology (53%), decreased
Abbreviations and Synonyms corticomedullary definition (38%)
o Heterogeneous parenchyma (43%)} renal
• HIV-associated nephropathy (HIVAN), focal segmental
enlargement (20%)} pelvocalyceal thickening
glomerulosclerosis (FSGS)
o No hydronephrosis
Definitions
Imaging Recommendations
• HIV-l infection associated with focal segmental
glomerulosclerosis • Best imaging tool: US

I IMAGING FINDINGS I DIFFERENTIAL DIAGNOSIS

General Features Medical renal disease
• Acute interstitial nephritis results in enlarged
• Best diagnostic clue: Enlarged echogenic kidneys on
echo genic kidneys
sonography
• Renal US in glomerulonephritis is variable, may
• Size: Kidneys> 11 cm in length
demonstrate normal size & echogeneity
• Morphology: Kidneys may be globular (53%)
• Kidneys> 11 cm; increased echogenicity; prominent
CT Findings renal pyramids
• NECT: Hyperattenuation of medulla (14%)
Acute tubular necrosis (ATN)
• CECT: Striated nephrogram
• Most common cause of reversible acute renal failure'
MR Findings hypotension most common cause '
• T2WI: Loss of corticomedullary differentiation • Other etiologies include drugs (e.g., contrast reaction),
heavy metals or solvent exposure
Ultrasonographic Findings • US of hypotension-related ATN is often normal
• Real Time • US of ATN caused by drugs, heavy metals or solvent
exposure results in enlarged echogenic kidneys

DDx: Renal lesions Mimicking HIVAN

Medical Renal Disease ATN Renal PC Obstructive Uropathy

 HIV NEPHROPATHY

Key Facts
Terminology Top Differential Diagnoses
• HIV-l infection associated with focal segmental • Medical renal disease
glomerulosclerosis • Acute tubular necrosis (ATN)
• Renal Pneumocystis carinii (PC)
Imaging Findings
• Best diagnostic clue: Enlarged echogenic kidneys on Clinical Issues
sonography • Clinical profile: African-American, HIV-positive
patient with renal failure

• Elevated resistive index (RI > 0.7)

• Kidneys after contrast reaction demonstrate delayed Natural History & Prognosis
3
cortical transit & excretion • 2 yr survival 36% 43
Renal Pneumocystis carinii (PC) Treatment
• Punctate calcifications of renal cortex in AIDS patients; • Triple drug anti-retroviral therapy reduces incidence,
associated calcifications in liver, spleen, lymph nodes stabilizes patient
• Dialysis for end-stage renal disease
Acute obstructive uropathy
• Bilateral hydronephrosis
• Non-dilated obstructive uropathy may lead to I DIAGNOSTIC CHECKLIST
false-negative US; causes: Prostate and bladder cancers,
retroperitoneal fibrosis Consider
• Acute medical renal disease
Image Interpretation Pearls
I PATHOLOGY • Enlarged echogenic kidneys
General Features
• General path comments
o Focal and segmental glomerulosclerosis; interstitial I SELECTED REFERENCES
infiltrates 1. Kopp JB et al: HIV-associated nephropathy in African
o Degenerative changes within tubules; interstitial Americans. Kidney lnt Suppl. (83):S43-9, 2003
tubular microcystic ectasia with protein casts 2. Herman ES et al: HIV-associated nephropathy:
• Etiology: HIV-l infection Epidemiology, pathogenesis, and treatment. Semin
Nephrol. 23(2):200-8, 2003
• Epidemiology
3. Rao TK: Human immunodeficiency virus infection in
o HIV infected individuals
end-stage renal disease patients. Semin Dial. 16(3):233-44,
o Substantially higher incidence in African-Americans 2003
- 90% of reported cases
Microscopic Features
• Focal segmental glomerulosclerosis with collapse of I IMAGE GALLERY
glomerular tuft associated with dilated tubules with
micro cysts

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Azotemia, hypoalbuminemia, proteinuria
o Other signs/symptoms: Normotensive
• Clinical profile: African-American, HIV-positive
patient with renal failure
Demographics (Left) Sonography of HIVAN. Longitudinal scan shows echogenic
• Age: 20-64 yrs enlarged kidney with minimal pelvo-calyceal dilation (arrow)
• Gender: M > F (Courtesy Philip W Ralls, MO). (Right) Sonography of HIVAN shows
• Ethnicity: African-American: HIVAN is third leading striking increase in renal echogenicity and poor definition of renal
pyramids (arrow) (Courtesy Philip W Ralls, MO).
cause of renal failure in African-Americans age 20-64
yrs
 GLOMERULONEPHRITIS

3
44

Coronal oblique US shows normal appearing kidney in Coronal oblique US shows markedly echogenic kidney
patient with biopsy-proven acute glomerulonephritis. in patient with acute renal failure due to
glomerulosclerosis (Courtesy H. Harvin, MOJ.

o Chronic GN
ITERMINOlOGY • Increased echogenicity
Abbreviations and Synonyms • Small, smooth kidneys
• Glomerulonephritis (GN) • Proliferation of sinus fat
• Color Doppler: May see renal vein thrombosis
Definitions
Imaging Recommendations
• Inflammation and proliferation of glomerular tissue
• Best imaging tool: US to rule out hydronephrosis
• Protocol advice: US useful to document signs of
IIMAGING FINDINGS chronic vs. acute renal failure (ARF)

General Features
• Best diagnostic clue: Nonspecific imaging findings I DIFFERENTIAL DIAGNOSIS
• Size Bilateral smooth renal enlargement
o Enlarged kidneys: Acute GN
o Small kidneys: Chronic GN • Amyloidosis, multiple myeloma
• Morphology: Smooth renal contour • Acute tubular necrosis (ATN)
• Acute interstitial nephritis (AIN)
CT Findings
Bilateral small kidneys with smooth contour
• NECT
o Acute GN: Normal or bilateral renal enlargement • Arteriosclerosis, nephrosclerosis
o Chronic GN: May have cortical calcification • Renal embolic disease

Ultrasonographic Findings
• Real Time I PATHOLOGY
o Acute GN
• Normal to increased renal echogenicity General Features
• Bilateral normal or enlarged kidneys • General path comments: GN is one of several intrinsic
• No hydronephrosis to explain renal failure causes of acute renal failure

DDx: Acute Renal Failure

Hydronephrosis ATN AIN

 GLOMERULONEPHRITIS

Key Facts
Imaging Findings
• Best diagnostic clue: Non-specific imaging findings
• Chronic GN: May have cortical calcification
• Normal to increased renal echogenicity
• No hydronephrosis to explain renal failure
• Proliferation of sinus fat
• Best imaging tool: US to r/o hydronephrosis
Clinical Issues
• Post-streptococcal: Follows pharyngitis or impetigo
• May progress to end-stage renal disease
Diagnostic Checklist
• Look for associated renal vein thrombosis
• Try to distinguish acute from chronic and
pre/post-renal causes of renal failure from intrinsic
causes

• Etiology o Chronic GN: Develops over months-years 3

o Primary GN: Confined to kidney • Clinical profile
• Systemic features secondary to renal dysfunction o Post-streptococcal: Follows pharyngitis or impetigo 45
• Most are immune-mediated o Secondary: Autoimmune diseases, other entities
• Common: Post-streptococcal
o Secondary: Part of multisystem disorder; many Natural History & Prognosis
immune-mediated • May progress to end-stage renal disease
• Epidemiology Treatment
o Causes of acute renal failure
• Options, risks, complications
• Pre-renal (renal hypoperfusion): 55%
o Immunosuppressive therapy
• Intrinsic renal disease"" 40%
o Treatment of underlying systemic disorder
• Post-renal (obstruction) "" 5%
• Associated abnormalities: Renal vein thrombosis
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
• Kidneys grossly normal or enlarged
Consider
• May see renal pallor or petechial hemorrhages
• Look for associated renal vein thrombosis
Microscopic Features • Try to distinguish acute from chronic and
• Hypercellularity pre/post-renal causes of renal failure from intrinsic
• Glomerular basement membrane (GBM) thickening causes
• Hyalinization: Deposition of amorphous
proteinaceous material
• Sclerosis: Obliteration of glomerular tuft I SELECTED REFERENCES
1. Braunwald E et al: Harrison's principles of internal
Staging, Grading or Classification Criteria
medicine. 15th ed. New York, McGraw-Hill. 1541-98,2001
• Morphologic criteria 2. Davidson AJ et al: Radiology of the kidney and
o Proliferative: Increased glomerular cell number genitourinary tract. 3rd ed. Philadelphia, W.B. Saunders,
o Crescentic: Half-moon collection of cells in Bowman 143-83, 1999
space
o Membranous: Expansion of GBM with immune
deposits I IMAGE GAllERY
• Extent within kidney
o Focal: Involves < 50% of glomeruli
o Diffuse: Involves majority of glomeruli
• Glomerular involvement
o Global: Involves whole of glomerular tuft
o Segmental: Involves part of glomerular tuft

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Major presentations of glomerular disease
• Acute nephritic or nephrotic syndrome
• Asymptomatic urinalysis abnormalities
o Acute GN: Develops over days
• Proteinuria, hematuria, red cell casts
• Hypertension, edema, oliguria
o Rapidly progressive GN: Weeks ~ months
(Left) Axial NECT shows punctate parenchymal calcification in
dialysis patient with chronic glomerulonephritis and secondary
hyperparathyroidism. (Right) Micropathology shows diffusely
enlarged glomerulus with increased cellularity and thickening of the
glomerular capillary walls in Hepatitis C virus-associated MPCN
(Courtesy T. Morgan, MOJ.
 RENAL PAPILLARY NECROSIS

3
46

Urogram shows club-shaped calices bilaterally due to Nephrotomogram shows streak of contrast extending
papillary necrosis attributed to chronic analgesic abuse. from fornix parallel to papilla (arrow).

o !(Rarely t) density of contrast in nephrogram

ITERMINOLOGY
Definitions
• Necrosis of renal papilla within medulla secondary to
interstitial nephritis or ischemia
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Triangular or bulbous cavitation
adjacent to calyx on IVP or retrograde pyelogram
• Location
o Bilateral: Analgesics & diabetes
o Unilateral: Obstructioni infectioni venous thrombus
• Morphology: Papillary swelling, tract formation,
cavitation & sloughing
Radiographic Findings
• IVP
o Subtle streak of contrast extending from fornix
parallel to long axis of papilla
o Triangular or bulbous cavitation of papilla
o Widened fornix (necrotic shrinkage of papilla)
o Calyx: Club-shaped or saccular (sloughed papilla)
o Sloughed papilla: Triangular filling defect in calyx,
pelvis, ureter or ring-shaped peripheral calcification
o "Ring shadow": Outlining detached papilla
o ± Displaced collecting system (due to edema)
o ± Calcification or ossification of necrotic papillae
• Retrograde pyelography: Rapid filling of cystic cavities
CT Findings
• Normal, enlarged or small contracted kidneys
• Ring shaped medullary calcification
• Hematoma, lobar infarct, scarring (sickle cell)
• Contrast filled clefts in renal parenchyma
• Filling defects: Renal pelvis/ureter (sloughed papillae)
• ± Hydronephrotic changes (due to obstruction)
• ± Renal vein thrombosis
• ± Heterogeneous parenchymal enhancement due to
pyelonephritis
Ultrasonographic Findings
• Real Time
o Early stage
• Necrotic renal papillae: Seen as echo genic foci
• Sonolucent rims: Fluid around necrotic papillae
o Advanced stage
• Single or multiple cystic cavities in medullary
pyramids continuous with calices ± calcification
Imaging Recommendations
• IVP or RGPi helical NE + CECT
o Nonionic contrast agents recommended due to t
incidence of contrast associated nephropathy

DDx: Blunted or Dilated Calices

Hydronephrosis Hydronephrosis Medullary Sponge Medullary Sponge

 RENAL PAPILLARY NECROSIS

Key Facts
Terminology Top Differential Diagnoses
• Necrosis of renal papilla within medulla secondary to • Hydronephrosis
interstitial nephritis or ischemia • Medullary sponge kidney
Imaging Findings Pathology
• Best diagnostic clue: Triangular or bulbous cavitation • Analgesic nephropathy} DM, S-hemoglobinopathy
adjacent to calyx on IVP or retrograde pyelogram
• Calyx: Club-shaped or saccular (sloughed papilla) Diagnostic Checklist
• "Ring shadow": Outlining detached papilla • Correlate imaging with history of analgesics, DM, SC

I DIFFERENTIAL DIAGNOSIS Natural History & Prognosis

Hydronephrosis
• Dilated pelvicaliceal system & ureter; I renal function
Medullary sponge kidney
• Multiple small cystic cavities or tubular ectasia;
medullary nephrocalcinosis
• Excretory urography
o Linear densities ("brush" appearance) in pyramids
Congenital megacalices
• Large & wide calyceal system; normal renal function
I PATHOLOGY
General Features
• General path comments
o Three types of parenchymal involvement in RPN
• Medullary type: Central cavitation of papillae
extending from fornix
• Papillary type: Necrosis & cavitation at periphery
of papillae ~ sloughing of papillae
• Necrosis in situ of papillae ± Ca++ or ossification
o Ca++ within necrotic papillae: Analgesics (common)
• Etiology
o Analgesic nephropathy} DM, S-hemoglobinopathy
o UTI & obstruction, TB}kidney transplant, alcoholics
3
• Complications: Obstruction, infection, renal failure, 47
transitional cell carcinoma
• Prognosis: Early stage (good); advanced stage (poor)
Treatment
• Early stage: Symptomatic treatment
• Advanced stage (obstruction} infection, failure)
o Ureteral stent, surgical treatment
I DIAGNOSTIC CHECKLIST
Consider
• Correlate imaging with history of analgesics, DM, SC
Image Interpretation Pearls
• Club-shaped calyx, triangular cavitation of papillae or
filling defect of sloughed papillae with ring shadow
I SELECTED REFERENCES
1. Hoffman]C et al: Demonstration of renal papillary
necrosis by sonography. Radiology 145: 785-787, 1982
2. McCall IW et al: Urographic findings in homozygous sickle
cell disease. Radiology 126: 99-104, 1978
3. Poynter ]D et al: Necrosis in situ: A form of renal papillary
necrosis seen in analgesic nephropathy. Radiology 111:
69-76, 1974

Gross Pathologic & Surgical Features

• Mild cases: Normal or enlarged kidneys I IMAGE GALLERY
• Severe cases: Small, smooth or scarred
Microscopic Features
• Analgesics: RPN, centrilobar cortex atrophy, septal
cortex hypertrophy
• Sickle disease: Vessel occlusion, lobar infarcts} tubular
obliteration, fibrosis

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Flank pain, dysuria, fever, ureteral colic, HTN
o Pyuria, hematuria, acute oliguric renal failure (Left) Nephrotomogram shows sloughed papilla (arrow) as a filling
defect in calyx; clubbed calices. (Right) Axial CECT shows
• Lab: ± i WBC or I RBC; proteinuria; impaired RFT
ring-shaped medullary calcification (arrow) on the surface of a
Demographics necrotic papilla.
• Age: Middle-aged women (analgesic nephropathy)
 UROLITHIASIS

3
48

Urogram shows mild right hydronephrosis with Axial NEeT through UV junction shows 2 mm calculus
complete opacification of ureter to UV junction; no (arrow) responsible for obstruction and pain.
stone seen.

!TERMINOLOGY • Uric acid stones (5-10%)

• Cystine stones (1-3%)
Abbreviations and Synonyms • Matrix stones (rare): Mucoproteins
• Calculous disease; nephrolithiasis; kidney, renal or • Xanthine stones (extremely rare)
urinary stones • Milk of calcium: Calcium carbonate + calcium
phosphate (carbonate apatite)
Definitions • Protease inhibitor stones: Indinavir-induced
• Concretions within the urinary system
Radiographic Findings
• Radiography
I IMAGING FINDINGS o Pre-CT belief
• Radiography detects 90% of calcium stones, some
General Features struvite and "misses" uric acid stones
• Best diagnostic clue: Small dense focus at o Based on CT correlation: Radiography "misses"
ureterovesical junction (UVJ) with proximal majority of calculi
hydronephrosis and perinephric stranding • Due to small size, insufficient radiopacity,
• Location overlying bones, bowel, etc.
o Upper urinary tract (UT): Calyceal, renal pelvis or o Calcium oxalate or phosphate stones
ureteropelvic junction (UP]) • Usually very opaque, visible if large
o Ureteral calculi: Ureter or UV] • Can be smooth, irregular or jagged
o Lower UT: Bladder, urethral, prostatic o Struvite and cystine stones
• Other general features • Staghorn calculi: Shape may conform to
o Types of stones pelvocaliceal system
• Calcium stones (75-80%): Calcium oxalate and/or • Usually opaque, detectible if large
calcium phosphate o Uric acid and xanthine stones
• Struvite stones (15-20%): Magnesium ammonium • Usually small, smooth, disc-shaped
phosphate (struvite), magnesium ammonium • Rarely opaque or detectible (when mixed with
phosphate + calcium phosphate (triple phosphate) calcium salts)
o Milk of calcium

DDx: High Density Focus in Kidney or Ureter

Arterial Calcification Phleboliths Phleboliths Primary Megaureter

 UROLITHIASIS
Key Facts
Terminology
• Calculous disease; nephrolithiasis; kidney, renal or
urinary stones
Imaging Findings
• Best diagnostic clue: Small dense focus at
ureterovesical junction (UV]) with proximal
hydronephrosis and perinephric stranding
• Based on CT correlation: Radiography "misses"
majority of calculi
• Radiopacity (most to least): Calcium oxalate and/or
phosphate> cystine> struvite > uric acid
Top Differential Diagnoses
• Renal artery calcification
• Nephrocalcinosis
• Phlebolith
• Liquid suspension of calcium salts that are
"trapped" in a calyceal diverticulum or ureterocele
• Moderately opaque
o Protease inhibitor stones
• Nonopaque
• IVP
o Lucent (uric acid, cystine, matrix): Filling defects
o Opaque (calcium, milk of calcium): Obscured by
contrast-opacified urine
o ± PC diverticula, UP] obstruction, tubular ectasia
(medullary sponge kidney), urinary diversion
o Ureteral calculi: Nephrographic phase
• Enlarged kidney
• Delayed ("obstructive", peak at 6 hrs.); prolonged
• Dense; striated; absent ("negative")
o Ureteral calculi: Pyelographic phase
• Delayed opacification (;;0: 24 hrs)
• Hydronephrosis; stone in ureter
• t Contrast density in collecting system
• Contrast extravasation; ± forniceal rupture
• Asymmetry of ureteral caliber to obstructed level
• "Standing column" of contrast to obstructed level
• Interureteric ridge or edema (pseudoureterocele)
o Ureteral calculi: Late phase
• Vicarious excretion of contrast (to gallbladder)
CT Findings
• NECT
o Stones are uniformly dense except matrix &
indinavir stones
o Radiopacity (most to least): Calcium oxalate and/or
phosphate> cystine> struvite > uric acid
o Calcium stones: 400-600 HU
o Uric acid & cystine stones: 100-300 HU
o Matrix stones
• Soft tissue attenuation (pure)
• Laminated peripheral calcification, diffuse 1
density or round faintly opaque nodules with
densely calcified center (when mixed with calcium
salts)
o Milk of calcium: Layered opaque suspension; stone
movement
Pathology
• Majority are mixed composition; > 50% contain
calcium salts
Clinical Issues
• Acute colicky flank pain radiating to groin (60-95%
with these symptoms have stones)
• Age: 1 in 8 have stones by 70 years of age
• Spontaneous passage through ureter: 80% « 4 mm),
50% (4-6 mm), 20% (> 8 mm)
Diagnostic Checklist
• Avoid repeat CT in young patients with known or 3
clinically evident stones (excessive radiation)
• Small dense focus in course of ureter with perinephric 49
stranding ± hydronephrosis
o Indinavir stones: Not or faintly opaque; deduced
from secondary findings (obstruction)
o Ureteral calculi: Visualize stone and secondary signs
• "Soft tissue rim" sign: Ureteral wall edema at stone
• Pseudoureterocele: UV] edema around calculus
• Hydronephrosis; hydroureter; perinephric or
periureteral stranding
• CECT: Lucent (matrix and indinavir stones): Filling
defects
MR Findings
• No signal (no mobile protons); large: Signal voids
• Ureteral calculi: Abrupt change in ureteral caliber
indicates obstruction level; secondary signs
Ultrasonographic Findings
• Real Time
o Visualize calculi as bright echo genic foci with
acoustical shadowing
• Not dependent on chemical consistency
o Best visualized in kidney and at UV]; poor
visualization of ureteral stones
• Color Doppler: Ureteral jet: Visualization of "jet" of
urine from UV] into bladder excludes obstructing
distal stone
I DIFFERENTIAL DIAGNOSIS
Renal artery calcification
• Radiography: Curvilinear parallel lines of calcification
in extrarenal, intrarenal or pelvic arteries
• US: Echogenic foci within renal sinus with acoustical
shadowing; indistinguishable from < 1 cm calculi
• US: Curvilinear appearance; distinguish> 1 cm calculi
• Differentiate by correlation with radiography
Nephrocalcinosis
• Calcification within parenchyma: Cortex & medulla
(most common); can erode & evolve into urolithiasis
• CT & US: Indistinguishable except by location
Phlebolith
• Stone within vein (pelvic, gonadal)
 UROLITHIASIS
• Radiography: Multiple, round, central lucency
• CT: :0; 4 mm calcification without "tissue rim" sign
• CT: "Comet" or "tail" sign: Adjacent eccentric, tapering
soft tissue mass (noncalcified portion of pelvic vein) &
a central lucency within calcification
• Distinguishing ovarian vein phlebolith from ureteral
calculus is difficult
o Follow course of vessel and look for signs of ureteral
obstruction
Primary megaureter
• Rare congenital anomaly causes dilated ureters
3 I PATHOLOGY
50 General Features
• General path comments: Majority are mixed
composition; > 50% contain calcium salts
• Etiology
o Calcium stones
• Idiopathic (85%): Idiopathic hypercalciuria
• Acquired (15%): Hyperparathyroidism, sarcoidosis,
renal tubular acidosis, hyperoxaluria, steroids,
Cushing syndrome, immobilization, 1 vitamin D
o Struvite stones: Urinary tract infections (UTI)
(Proteus, Klebsiella, Pseudomonas; urea-splitting)
o Uric acid stones: Hyperuricosuria (25% with gout),
ileostomy, chemotherapy, acidic & concentrated
urine, adenine phosphoribosyltransferase deficiency
o Cystine stones: Cystinuria (autosomal recessive)
o Matrix stones: Chronic UTI, urine stasis, obstruction
o Xanthine stones: Xanthine oxidase deficiency
o Milk of calcium: PC diverticula, ureteroceles
o Risk factors
• Environment: Warm climates, summer
• Medications: Acetazolamide, indinavir
• Anatomical abnormalities: UP] obstruction
(horseshoe or ectopic kidney), PC diverticula,
tubular ectasia, urinary diversion
o· Pathogenesis
• Supersaturated solution ~ crystal formation in
urine (excessive excretion & precipitation theory)
• Lack of substances that inhibit crystal deposition,
stone formation & growth (inhibitor theory)
• Presence of specific macromolecules that are
essential for stone formation (matrix theory)
• Epidemiology
o Prevalence: 2-3%; 40-60 years of age (in Caucasians)
o Incidence: 1-2 per 1,000; peak at 20-40 years of age
Gross Pathologic & Surgical Features
• Matrix stones: Gelatinous or soft putty texture; tan to
red-brown
Microscopic Features
• Crystals dependent on type of stones
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Upper UT: Asymptomatic, flank pain, fever
o Ureteral calculi
• Acute colicky flank pain radiating to groin
(60-95% with these symptoms have stones)
o Lower UT: Asymptomatic, dysuria, dull/sharp pain
radiating to penis, buttocks, perineum or scrotum
• Lab data
o Urinalysis: Hematuria, crystals ± bacteruria or pyuria
• Diagnosis: Ureteral calculi by CT
o :0; 4 mm with "tissue rim" sign (91% sensitive)
o Hydroureteronephrosis & perinephric stranding
(97% positive & 93% negative predictor value)
Demographics
• Age: 1 in 8 have stones by 70 years of age
• Gender
o M:F = 3:1
o Struvite & matrix stone: M:F = 1:2-3
Natural History & Prognosis
• Spontaneous passage through ureter: 80% « 4 mm),
50% (4-6 mm), 20% (> 8 mm)
• Complications: Obstruction, infection, abscess and
renal insufficiency
• Prognosis: Recurrence without treatment: 10% at 1
year, 35% at 5 years, 50% at 10 years
Treatment
• 1 Hydration (2L urine/day), restrict diet (protein,
sodium, calcium) & drugs (thiazides or allopurinol)
• Extracorporeal shock wave lithotripsy (ESWL),
percutaneous nephrostolithotomy, endoscopic
retrieval or suprapubic cystolithotomy
• Follow-up recurrence only: 4-6 weeks after treatment,
24-hour urine (volume, calcium, phosphorus, uric
acid, creatine, oxalate, citrate, cystine screen)
I DIAGNOSTIC CHECKLIST
Consider
• Avoid repeat CT in young patients with known or
clinically evident stones (excessive radiation)
o Consider US, especially in young women
Image Interpretation Pearls
• Small dense focus in course of ureter with perinephric
stranding ± hydronephrosis
I SELECTED REFERENCES
1. Sheafor DH et al: Nonenhanced helical CT and US in the
emergency evaluation of patients with renal colic:
Prospective comparison. Radiology. 217: 792-7,2000
2. Smith RC et al: Diagnosis of acute flank pain: Value of
unenhanced helical CT. AJR. 166: 97-101, 1996
3. Katz DS et al: Un enhanced helical CT of ureteral stones:
Incidence of associated urinary tract findings. AjR. 166:
1319-22, 1996
4. Sommer FG et al: Detection of ureteral calculi in patients
with suspected renal colic: value of reformatted
noncontrast helical CT. AJRAm J Roentgenol.
165(3):509-13, 1995
5. Jeffrey RB et al: CT and ultrasonography of acute renal
abnormalities. Radiol Clin North Am. 21(3):515-25, 1983
 UROLITHIASIS
I IMAGE GALLERY

Typical
(Left) Axial NECT shows mild
left hydronephrosis and
perirenal stranding. (Right)
Axial NECT shows small
stone (arrow) at
ureterovesical junction.

3
51

Typical
(Left) Radiograph shows
large staghorn calculus.
(Right) Radiograph shows
multiple stone fragments
filling distal ureter (arrow)
following lithotripsy.

(Left) Axial NECT shows

proximal ureteral stone
(arrow) with a "rim" of 50ft
tissue representing the
ureteral wall. (Right)
Excretory urogram shows
persistent right nephrogram
and delayed excretion due to
proximal ureteral stone
(arrow).
 NEPHROCALCINOSIS

3
52

Drawing shows calcificauon in renal pyramids, Anteroposterior radiography shows dense calcificauon
representing nephrocalcinosis. within the renal pyramids in a patient with sarcoid and
medullary nephrocalcinosis.

ITERMINOLOGY Radiographic Findings

• Radiography
Abbreviations and Synonyms o Fine stippled calcification in renal pyramids
• Medullary nephrocalcinosis, cortical nephrocalcinosis o Coarse, confluent calcification
Definitions o Punctate or "tramline" cortical calcification
• Radiologically detectable diffuse calcium deposition • IVP
o Medullary: Calcification in renal pyramids on
within the renal substance
preliminary films
o May see linear striations and/or cystic spaces in
papillae in patients with underlying medullary
I IMAGING FINDINGS sponge kidney
General Features CT Findings
• Best diagnostic clue: Calcification within renal
• NECT
parenchyma o Stippled or confluent calcifications in renal
• Location parencyma
o Renal parenchyma o May see ringlike pattern due to relatively increased
• Medullary nephrocalcinosis: 95% calcification at corti comedullary junction
• Cortical nephrocalcinosis: 5%
• Both cortical and medullary: Rare Ultrasonographic Findings
• Size: Kidneys often normal size and contour • Real Time
• Morphology o Medullary: Echogenic renal pyramids
o Variable patterns of calcification • May have ring-like pattern with increased
• Scattered punctate calcifications in renal medullae echogenicity along periphery of pyramid
• Dense, confluent medullary calcification: o Acoustic shadowing may be absent
Common in renal tubular acidosis
• "Tram line" calcification or punctate calcifications Imaging Recommendations
in renal cortex • Best imaging tool: Noncontrast CT

DDx: Mimics of Nephrocalcinosis

Hemorrhagic Cysts Renal TB Renal Failure Pneumocystis

 NEPHROCALCINOSIS
Key Facts
Terminology
• Radiologically detectable diffuse calcium deposition
within the renal substance
Imaging Findings
• Medullary nephrocalcinosis: 95%
• Cortical nephrocalcinosis: 5%
• Fine stippled calcification in renal pyramids
• Coarse, confluent calcification
• May see ringlike pattern due to relatively increased
calcification at corticomedullary junction
• Medullary: Echogenic renal pyramids
• Best imaging tool: Noncontrast CT
Top Differential Diagnoses
• Papillary necrosis
• Renal tuberculosis
• Protocol advice: Detection of nephrocalcinosis on
plain films is improved by low kV technique
I DIFFERENTIAL DIAGNOSIS
Technical
• NECT: Residual contrast material
Papillary necrosis
• Common in analgesic nephropathy
• Curvilinear, ring-shaped, or triangular calcification in
sloughed papillae
• Calcifications arranged in arc conforming to positions
of papillary tips
• May be indistinguishable from nephrocalcinosis
Renal tuberculosis
• Focal amorphous calcification within kidney at sites of
caseating infection
• Diffuse: Dense calcification throughout
auto nephrectomized kidney ("putty kidney")
Extrapulmonary pneumocystis carinii or MAl
infection in AI DS
• May have both cortical and medullary punctate
calcification
[PATHOLOGY
General Features
• General path comments
o Calcium stones grow on papillae
• Most break loose and enter collecting system -
urolithiasis
• If calcium stones remain in place - medullary
nephrocalcinosis
• Genetics
o Type I renal tubular acidosis (RTA):Familial form
• Autosomal dominant inheritance pattern most
common
• May be due to defect in chloride-bicarbonate
exchange gene AE1
Pathology
• Medullary: Renal tubular acidosis type 1 (distal)
• Medullary: Hyperparathyroidism
• Medullary sponge kidney (MSK): Cystic or fusiform
dilation of collecting ducts in renal pyramids
• Acute cortical necrosis secondary to shock, placental
abruption, nephrotoxins
• Cortical: Chronic glomerulonephritis
• Associated abnormalities: Urolithiasis when calculi
formed in renal medulla erode into collecting system
Diagnostic Checklist
• Massive, dense medullary nephrocalcinosis usually 3
due to RTAtype I
• Unilateral or segmental medullary nephrocalcinosis
53
- medullary sponge kidney
o Hyperoxaluria: Familial form
• Autosomal recessive
• Etiology
o Medullary nephrocalcinosis
• 40%: Hyperparathyroidism
• 20%: Renal tubular acidosis type I
• 20%: Medullary sponge kidney
o Cortical nephrocalcinosis
• Chronic glomerulonephritis
• Renal cortical necrosis
• Transplant kidney: Chronic rejection
o Three primary mechanisms for calcium deposition
• Metastatic: Metabolic abnormality leads to
calcium deposition in the medullae of
morphologically normal kidneys
• Urinary stasis: Calcium salts precipitate in dilated
collecting ducts containing static urine
• Dystrophic: Calcium deposition in damaged renal
tissue
o Entities causing metastatic calcification
• Medullary: Renal tubular acidosis type 1 (distal)
• Medullary: Hyperparathyroidism
• Medullary: Hypercalcuria
• Medullary: Hyperoxaluria
o Nephrocalcinosis due to urinary stasis
• Medullary sponge kidney (MSK): Cystic or
fusiform dilation of collecting ducts in renal
pyramids
o Nephrocalcinosis due to dystrophic calcification
• Acute cortical necrosis secondary to shock,
placental abruption, nephrotoxins
• Cortical: Chronic glomerulonephritis
• Epidemiology
o Incidence 0.1-6%
o Medullary sponge kidney: Seen in 0.5% of excretory
urograms
• Associated abnormalities: Urolithiasis when calculi
formed in renal medulla erode into collecting system
Gross Pathologic & Surgical Features
• Depends on underlying etiology of nephrocalcinosis
 NEPHROCALCINOSIS
• Unilateral or segmental medullary nephrocalcinosis ~
Microscopic Features medullary sponge kidney
• Calcium deposition in the interstitium, tubule
epithelial cells, along basement membranes
• Calcium deposition within lumina of tubules I SELECTED REFERENCES
1. Hoppe B et al: Diagnostic and therapeutic approaches in
patients with secondary hyperoxaluria. Front Biosci.
I CLINICAL ISSUES 8:e437-43,2003
2. Peacock M: Primary hyperparathyroidism and the kidney:
Presentation biochemical and clinical spectrum. J Bone Miner Res. 17
• Most common signs/symptoms SuppI2:N87-94,2002
o Most often asymptomatic 3. Gobel U et al: The protean face of renal sarcoidosis. J Am
o Other signs/symptoms Soc Nephrol. 12(3):616-23, 2001
• Flank pain, hematuria if associated with 4. Unwin RJ et al: The renal tubular acidoses. J R Soc Med.
3 urolithiasis
5.
94(5):221-5, 2001
Sayer JA et al: Diagnosis and clinical biochemistry of
• Clinical profile: Cortical nephrocalcinosis
inherited tubulopathies. Ann Clin Biochem. 38(Pt
54 o Acute cortical necrosis 5):459-70, 2001
• Nephrotoxic drugs (ethylene glycol, 6. Boonen S et al: Primary hyperparathyroidism:
methoxyflurane anesthesia, amphotericin B) pathophysiology, diagnosis and indications for surgery.
• Acute vascular insult (shock, placental abruption) Acta Otorhinolaryngol Belg. 55(2):119-27, 2001
o Chronic glomerulonephritis 7. Sharma OP: Hypercalcemia in granulomatous disorders: a
o Alport syndrome: Hereditary nephritis and nerve clinical review. Curr Opin Pulm Med. 6(5):442-7, 2000
deafness 8. Audran M et al: Hypercalciuria. Joint Bone Spine.
67(6):509-15,2000
• Clinical profile: Medullary nephrocalcinosis
9. Davidson AJ et al: Radiology of the kidney and
o Skeletal deossification genitourinary tract. 3rd ed. Philadelphia, W.B. Saunders,
• Primary and secondary hyperparathyroidism 213-216, 1999
• Bony metastases 10. Chen MY et al: Abnormal calcification on plain
• Prolonged immobilization radiographs of the abdomen. Crit Rev Diagn Imaging.
o Increased intestinal absorption of calcium 40(2-3):63-202, 1999
• Sarcoidosis 11. Dyer RB et al: Abnormal calcifications in the urinary tract.
• Milk-alkali syndrome Radiographies. 18(6):1405-24, 1998
12. Miller FH et al: Renal manifestations of AIDS.
o Medullary sponge kidney
Radiographics. 13(3):587-96, 1993
o Hyperoxaluria 13. Cockerell CJ et al: Widespread cutaneous and systemic
• Hereditary type calcification (calciphylaxis) in patients with the acquired
• Acquired: Secondary to small bowel disease or immunodeficiency syndrome and renal disease. J Am Acad
bariatric surgery Dermatol. 26(4):559-62, 1992
o Renal tubular acidosis (RTA)type 1 (distal RTA) 14. Dunnick NR et al: Textbook of uroradiology. Baltimore,
• May be primary or secondary to other systemic Williams and Wilkins. 189-190, 1991
disease (Sjogren, lupus, others) 15. Amis ES et al: Essentials of uroradiology. Boston, Little,
Brown and company. 213-216, 1991
• Distal tubule unable to secrete hydrogen ions
16. Falkoff GE et al: Partial, combined cortical and medullary
• Metabolic acidosis with urinary pH> 5.5 nephrocalcinosis: US and CT patterns in AIDS-associated
• Type II (proximal) RTAnever causes MAl infection. Radiology. 162(2):343-4, 1987
nephrocalcinosis 17. Hoffbrand BI et al: Nodular cortical nephrocalcinosis: a
benign and hitherto undescribed form of renal
Demographics calcification. Nephron. 46(4):370-2, 1987
• Age: Any 18. Martijn A et al: Radiologie findings in primary
• Gender: M > F hyperoxaluria. Skeletal Radiol. 8(1):21-4, 1982

Natural History & Prognosis

• Depends on underlying cause of nephrocalcinosis
Treatment
• Options, risks, complications: Medullary
nephrocalcinosis often complicated by urolithiasis

I DIAGNOSTIC CHECKLIST
Consider
• Focal areas of dystrophic calcification in masses or
infection are not considered nephrocalcinosis
Image Interpretation Pearls
• Massive, dense medullary nephrocalcinosis usually due
to RTAtype I
 NEPHROCALCI.NOSIS

I IMAGE GALLERY

Typical
(Left) Axial NECT shows
densely calcified renal
pyramids in a patient with
sarcoid and medullary
nephrocalcinosis. Note
ring-like pattern (arrow) in
left kidney at
cortico-medullary junction.
(Right) Axial NECT shows
thin rim of calcification in
renal cortices bilaterally,
greater on the left than right 3
in a vascular surgery patient
with renal cortical necrosis. 55

Typical
(Left) Sagittal US shows
echogenic renal pyramids
(arrows) in patient with
medullary nephrocalcinosis.
(Right) Anteroposterior
radiography shows densely
calcified pyramids in patient
with renal tubular acidosis.

Variant
(Left) Coronal NECT shows
unilateral nephrocalcinosis in
a patient with medullary
sponge kidney. (Right) Axial
NECT shows coarse
calcification in renal cortical
regions in patient with
chronic glomerulonephritis.
Exophytic cyst in left kidney
(arrow) is secondary to
chronic dialysis.
 ACQUIRED CYSTIC DISEASE OF UREMIA

3
56

Axial NECT shows small multicystic kidneys. Axial CECT shows innumerable cysts in bilaterally
enlarged kidneys in a patient who has been on dialysis
for many years.

• After 5-10 years: More than 90%

ITERMINOlOGY o Prevalence, size & number of cysts correlate with
Abbreviations and Synonyms duration of dialysis, but not with renal disease
o ACKD has similar prevalence in patients treated
• Acquired cystic kidney disease (ACKD) of uremia
with hemodialysis & peritoneal dialysis
Definitions o Several types of renal neoplasms occur in ESRD
• Development of multiple bilateral renal cysts with • Adenomas or carcinomas (extensive papillary
end-stage renal disease (ESRD) on long term dialysis epithelial hyperplasia in renal cysts of ACKD)
• Histologically 50% nonpapillary & 50% papillary
o Approximately 7% of ACKD patients develop renal
IIMAGING FINDINGS cell carcinoma of low grade malignancy
o 86% of RCCs occurring in patients undergoing
General Features dialysis affect those with ACKD
• Best diagnostic clue: Small kidneys with multiple small
cysts bilaterally CT Findings
• Location: Renal cortex (predominantly) & medulla • Bilateral small renal cysts in small to normal kidneys
• ± Hemorrhage within cysts may be seen
• Size
o Cysts: Usually smaller than 0.5 cm • Larger cysts may occur
o Kidneys: Small to normal size, may enlarge as cysts o When present, mimics polycystic kidney disease
expand • Calcification of cyst walls may occur
• Morphology: Multiple small bilateral renal cysts in • Focal hypervascular areas suggest associated renal cell
shrunken kidneys carcinoma
o Small tumors are the rule
• Other general features
o ACKD develops in patients with ESRD undergoing MR Findings
long term dialysis • Bilateral multiple small cysts in small-normal kidneys
o Prevalence of ACKD in dialysis patients
o T1WI: Hypointense
• After 1-3 years: 10-20%
o T2WI: Hyperintense
• After 3-5 years: 40-60%
o T1 C+: Nonenhancing

DDx: Multiple Renal "Cysts"

Renal Cysts + RCC Tuberous Sclerosis AD Polycystic von Hippel Lindau

 ACQUIRED CYSTIC DISEASE OF UREMIA
Key Facts
Terminology
• Acquired cystic kidney disease (ACKD) of uremia
• Development of multiple bilateral renal cysts with
end-stage renal disease (ESRD)on long term dialysis
Imaging Findings
• Best diagnostic clue: Small kidneys with multiple
small cysts bilaterally
• Location: Renal cortex (predominantly) & medulla
• Cysts: Usually smaller than 0.5 cm
• ± Hemorrhage within cysts may be seen
• Calcification of cyst walls may occur
• Focal hypervascular areas suggest associated renal cell
carcinoma
• RCC distinguished from cysts by their lower signal
intensity on T2WI images
• Hemorrhagic cysts
o Tl & T2WI: Variable signal (age of hemorrhage)
o Tl C+: No enhancement
• Renal cell carcinoma (RCC)
o RCC distinguished from cysts by their lower signal
intensity on T2WI images
o Tl C+: Significant enhancement
• Enhancement (Tl C+) is helpful in distinguishing
hemorrhagic cysts from small renal cell carcinomas
Ultrasonographic Findings
• Real Time
o Small to normal size kidneys with 1 echogenicity
o Corticomedullary differentiation is lost
o Cysts are seen as sonolucent lesions
• 3-5 cysts in patients with ESRDis diagnostic of
ACKD
o Hemorrhagic cyst
• Anechoic with a smooth wall
• Prominent posterior acoustic enhancement
• Internal echoes may be seen due to prior
hemorrhage
• Differentiation from neoplasm often not possible
o RCC
• Solid or with mural nodules
Imaging Recommendations
• Ultrasound establishes initial diagnosis
• Enhanced CT or MR used to look for RCC
• Early phase enhanced CT best for detecting RCC
o In this setting minimal renal enhancement seen
I DIFFERENTIAL DIAGNOSIS
Multiple simple renal cysts
• Patients with normal renal function
• No history of dialysis present
• Kidneys are normal size
Tuberous sclerosis (TS)
• Multiple bilateral renal cysts
o Cysts have typical histological features in TS
• Often associated with renal angiomyolipomas
Top Differential Diagnoses
• Multiple simple renal cysts
• Tuberous sclerosis (TS)
• Adult polycystic kidney disease (ADPKD)
• Medullary cystic disease
• von Hippel-Lindau disease
Diagnostic Checklist
• Check history & rule out hereditary cystic diseases
• CT or US screening of asymptomatic patients for RCC
& ACKD at the end of 3rd year of dialysis
• Annual screening of native kidneys for RCC in
transplant recipients 3
• Bilateral small cysts within small to normal kidneys
• Differentiate hemorrhagic cyst from a small RCC
57
• Enhancement: Hemorrhagic cyst (no) ; RCC (yes)
o Solid lesion, usually with fat
• Brain: Paraventricular calcifications
Adult polycystic kidney disease (ADPKD)
• Autosomal dominant polycystic kidney disease
• Cysts & kidneys are large in size
• Liver cysts may also be present
Medullary cystic disease
• Rare disease associated with progressive salt wasting
nephropathy with renal insufficiency
• Cysts are predominantly in medulla
• Kidneys are almost invariably small in size
• Clinically, progressive renal failure in young patients
• Imaging
o Renal cysts may be too small to be seen
o Visible cysts occur only in renal medulla
• Cysts in ACKD involve both cortex & medulla
von Hippel-Lindau disease
• Autosomal dominant multi systemic disorder
• Multiple renal cysts & cysts in other organs (e.g.,
pancreas)
• Kidneys are normal in size & functioning
• Hemangioblastomas: Retinal, cerebellar, spinal,
medulla
• Bilateral renal cell carcinomas; pheochromocytomas
I PATHOLOGY
General Features
• Etiology
o Seen in patients with all etiologies of ESRD,
particularly in patients who are dialysis dependent
o Three most common causes of ESRDin USA
• Nephrosclerosis
• Diabetic nephropathy
• Chronic glomerulonephritis
o Incidence, number & size of cysts increase in
proportion to duration of dialysis
o Pathophysiology: Theories of ACKD
• Secondary to obstruction of tubules by fibrosis or
oxalate crystals
 ACQUIRED CYSTIC DISEASE OF UREMIA
• Altered compliance of tubular basement
membrane
• Toxicity from circulating metabolites
(endogenous/exogenous toxins, growth factors)
• Vascular insufficiency
o Pathogenesis of cyst formation
• Most cysts derive from proximal renal tubules
• Begins with proliferation of epithelial cells
• Marked 1 in surface area of affected tubule wall
• Originates as mural diverticula of nephrons
• Early stage, cyst retains its connection with tubule
& glomerular filtrate enters the enlarging cyst
• Later stage, cyst loses its connection with parent
3 tubule & becomes an isolated fluid-filled sac
• Epidemiology
o More than 90% of cases after 5-10 years of dialysis
58
o Up to 7% of ACKD patients develop RCC
• Associated abnormalities
o Renal cell carcinoma in 7% of ACKD
o Small papillary, tubular or solid clear cell adenomas
Gross Pathologic & Surgical Features
• Contracted small size kidneys + multiple small cysts
o Cysts: Renal cortex more than medulla
• ± Associated renal tumors: Renal cell carcinoma
Microscopic Features
• Typical Cysts: Lined with a single layer of flattened or
cuboidal epithelial cells
• Atypical cysts: Multilayered epithelial lining
• Papillary projections from cyst walls often seen
• Papillary lining cells may exhibit nuclear atypia
• Cyst content: Usually clear fluid; few hemorrhagic
• Crystal deposition: Prominent feature in cyst walls,
cyst lumina & renal interstitium
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Most patients with ACKD are asymptomatic
o Symptomatic, if they develop complications
• Flank pain, fever, hematuria
• Oliguria or anuria
• Clinical profile: Patient with history of chronic renal
failure undergoing long term dialysis
• Lab data: 1 Blood urea nitrogen & creatinine; I GFR
• Diagnosis: Bilateral, with at least 4-5 cysts per kidney
in ESRD patient on long term dialysis
Demographics
• Age: Any age group, but more common in older age
• Gender: M > F
• Ethnicity: African Americans> Caucasians
Natural History & Prognosis
• Complications
o Intra- and pericystic bleeding
o Microhematuria, cyst infection, renal calculi
o Rupture with retroperitoneal hemorrhage
o 1 Risk of renal neoplasms particularly RCC
• 7 times more common in men than in women
• Prognosis
o In absence of RCC, prognosis relates to course of
their renal failure
o Mortality rate in dialysis-treated population with
ESRD in US is high relative to general population
• Average life expectancy in 30-49 yrs: 5-10 yrs
• Average life expectancy in> 50 yrs: 1.5-6 yrs
Treatment
• If renal cell carcinoma detected, nephrectomy is done
using laparoscopic flank approach
• Renal transplantation
I DIAGNOSTIC CHECKLIST
Consider
• Check history & rule out hereditary cystic diseases
• CT or US screening of asymptomatic patients for RCC
& ACKD at the end of 3rd year of dialysis
• Annual screening of native kidneys for RCC in
transplant recipients
Image Interpretation Pearls
• Bilateral small cysts within small to normal kidneys
• Differentiate hemorrhagic cyst from a small RCC
• Enhancement: Hemorrhagic cyst (no); RCC (yes)
I SELECTED REFERENCES
1. Takebayashi S et al: Using helical CT to evaluate renal cell
carcinoma in patients undergoing hemodialysis: value of
early enhanced images. A]R Am] Roentgenol.
172(2):429-33,1999
2. Levine E: Acquired cystic kidney disease. Radiol Clin North
Am. 34(5):947-64, 1996
3. Levine LA et al: Acquired cystic disease and renal
adenocarcinoma following renal transplantation. ] Urol.
151(1):129-32, 1994
4. Levine E et al: Natural history of acquired renal cystic
disease in dialysis patients: a prospective longitudinal CT
study. A]R Am] Roentgenol. 156(3):501-6, 1991
5. Ishikawa I et al: Ten-year prospective study on the
development of renal cell carcinoma in dialysis patients.
Am] Kidney Dis. 16(5):452-8, 1990
6. Matson MA et al: Acquired cystic kidney disease:
occurrence, prevalence, and renal cancers. Medicine
(Baltimore). 69(4):217-26, 1990
7. Miller LR et al: Acquired renal cystic disease in end-stage
renal disease: an autopsy study of 155 cases. Am] Nephrol.
9(4):322-8, 1989
8. Taylor A] et al: Renal imaging in long-term dialysis
patients: a comparison of CT and sonography. A]RAm]
Roentgenol. 153(4):765-7, 1989
9. Soffer 0 et al: CT findings in complications of acquired
renal cystic disease.] Comput Assist Tomogr. 11(5):905-8,
1987
10. Ishikawa I: Uremic acquired cystic disease of kidney.
Urology. 26(2):101-8, 1985
11. Levine E et al: CT of acquired cystic kidney disease and
renal tumors in long-term dialysis patients. A]R Am]
Roentgenol. 142(1):125-31, 1984
 ACQUIRED CYSTIC DISEASE OF UREMIA
[IMAGE GALLERY

Typical
(Left) Axial CECT shows a
small, perfused but
nonfunctional kidney with
innumerable cysts. (Right)
Axial CECT shows bilateral
small, multicystic kidneys
and ascites due to peritoneal
dialysis-induced peritonitis.

3
59

(Left) Axial NECT shows high

density, representing
spontaneous hemorrhage
within one of many renal
cysts due to ACKD. (Right)
Axial NECT shows bilateral
ACKD with spontaneous
retroperitoneal hemorrhage
from right kidney.

Typical
(Left) Axial CECT shows an
enhancing mass (arrow)
representing renal cell
carcinoma in a patient with
ACKD. (Right) Axial CECT
shows two enhancing masses
(arrows), renal cell
carcinomas in a patient with
ACKD.
 MEDULLARY SPONGE KIDNEY

3
60

Radiograph shows multiple calcifications in both Urogram shows dilated collecting ducts within papillae
kidneys. bilaterally and a "bouquet of flowers" appearance
caused by ectatic ducts and calculi.

o Moderate ductal ectasia

ITERMINOlOGY • More prominent linear radiodense striae
Abbreviations and Synonyms • Clusters of small rounded opacities in the papillae
• Medullary sponge kidney (MSK);renal tubular ectasia; (cystic dilatation of the collecting ducts)
cystic disease of the renal pyramids; precalyceal • ± Enlarged papillae and splayed calyceal cups
canalicular ectasia; cystic dilatation of the renal • "Bouquet of flowers" appearance: Ectatic collecting
tubules ducts filled with calcification
• Medullary calcifications: Large & dense
Definitions o Advanced ductal ectasia
• Multiple cystic dilations in collecting ducts of medulla • Gross deformity of the papillae with beaded or
striated cavities
• Distortion of calyces: Broad, shallow or widely
IIMAGING FINDINGS cupped
• Numerous and large calcifications
General Features o ± Nephrolithiasis
• Best diagnostic clue: "Paint brush" appearance of o Obstruction, calyceal distortion or destruction,
dilated opacified pyramid tubules on IVP urinary tract infection due to calculi complications
• Early films: No visualization of affected kidney
Radiographic Findings • Delayed films: Hydronephrosis or contrast-filled
• Radiography: ± Medullary nephrocalcinosis areas of destruction
(calcifications within medulla) or urolithiasis o Evaluation of adjacent calyx for tumor (e.g.,
• IVP transitional cell carcinoma) or for obstructing calculi
o Bilateral (75%); unilateral (25%); can be in single o Complications j visualization (j renal function)
pyramid or segment • Retrograde Pyelography
o Mild ductal ectasia o No filling or poor filling of dilated collecting ducts;
• "Paint brush" appearance: Discrete linear densities in contrast injection that is not forceful
3-4 in ~ 1 papillae (benign tubular ectasia) o Tips of papillary ducts are not prone to reflux,
• Collecting ducts wider than 200-300 I.Imin regardless of disease severity; same as normal
diameter (normal)

DDx: Nephrocalcinosis+/- Pelvo-Calyceal Abnormality

Med. Nephrocalcinosis Renal Tuberculosis Oxalosis (NECT) Oxalosis (NECT)

 MEDULLARY SPONGE
Key Facts
Terminology
• Multiple cystic dilations in collecting ducts of
medulla
Imaging Findings
• Best diagnostic clue: "Paint brush" appearance of
dilated opacified pyramid tubules on IVP
• Bilateral (75%); unilateral (25%); can be in single
pyramid or segment
• "Paint brush" appearance: Discrete linear densities 3-4
in ~ 1 papillae (benign tubular ectasia)
• ± Medullary nephrocalcinosis and urolithiasis
Top Differential Diagnoses
• Hypercalcemia
• Acute cortical necrosis
• Renal papillary necrosis
o Differentiate cysts of medullary sponge kidney from
cysts of other medullary cavities (e.g., papillary
necrosis or tuberculosis)
• Nephrotomography
o Before contrast: Multiple areas of calcification
o After contrast: Rounded opacities in medulla
CT Findings
• NECT
o ± Medullary nephrocalcinosis and urolithiasis
o ± Hydronephrosis and hydroureter (obstruction)
• CECT
o "Paint" brush appearance: Retention of contrast
within dilated tubules in pyramids
o ± Medullary nephrocalcinosis and urolithiasis:
Localizes the calcifications
o Severe disease: ± Extracaliceal contrast accumulation
within papillae or abscess
MR Findings
• Insensitive in detecting calcium
• Decreased sensitivity in detecting tubular ectasia
Ultrasonographic Findings
• Real Time
o Nonspecific; can demonstrate medullary cavities
o ± Medullary nephrocalcinosis
• Discrete hyperechoic foci with acoustic shadowing
within the pyramids
• Increased echogenicity between interlobar
cortices, surrounding the tips and sides of the
pyramids (tiny calcifications)
Imaging Recommendations
• Best imaging tool
o IVP: Discrete linear papillary densities
oCT: Medullary nephrocalcinosis and dilated tubules
("paint brush")
KIDNEY
• Renal tubular acidosis
• Tuberculosis
• Oxalosis
• Papillary Blush
Pathology
• Predisposing to urolithiasis (urinary stasis)
Clinical Issues
• Asymptomatic
Diagnostic Checklist
• Tubular ectasia can be caused by a adjacent tumor;
need to evaluate
3
• Bilateral, "paint brush" appearance; medullary
61
calcifications
I DIFFERENTIAL DIAGNOSIS
Hypercalcemia
• Examples: Milk-alkali syndrome, sarcoidosis,
hyperparathyroidism
• t Calcium uptake by gut or t bone destruction
• Associated with nephrocalcinosis (calcification within
the parenchyma, including the medulla and cortex)
• Calcifications of medulla without ectatic ducts or cysts
• Calcifications more diffuse
Acute cortical necrosis
• Acute renal failure secondary to ischemic necrosis of
renal cortex (prolonged vascular spasm, microvascular
injury or intravascular coagulation)
• Radiography: Thin cortical shells & tram lines caused
by calcification
• CT: Absent opacification of the renal cortex
• Anuriawith poor prognosis
Renal papillary necrosis
• Destruction of the apex of the pyramid ~ irregular
cavitation & sinus formation between papilla & calyx
• Ring or triangular calcification of sloughed papilla
• Retrograde pyelography: Fill 1 or 2 irregular cavities
Renal tubular acidosis
• Failure to secrete hydrogen ion & form ammonium
ion ~ "fixed base" sodium, potassium & calcium
• 50% have medullary nephrocalcinosis
• Calcifications more diffuse
• Vomiting, poor growth, weakness & bone discomfort
• Metabolic acidosis with urinary anion gap
Tuberculosis
• Reactive or secondary tuberculosis of the kidney
• Unilateral ulceration of papilla in areas that are
initially small and irregular
• Tendency toward coalescence into large cavities,
stricture formation & calcification in caseous necrosis
Oxalosis
• Secondary to t urinary oxalate (> 40 mg/24 hrs)
• Three types
 MEDULLARY SPONGE KIDNEY
o Primary (hereditary, rare) ~ i hepatic production
o Acquired (i oxalate ingestion)
I CLINICAL ISSUES
o Absorptive or enteric (Le., malabsorption, Presentation
inflammatory bowel disease) ~ dehydration & i • Most common signs/symptoms
uptake by gut o Asymptomatic
• i Calcium oxalate calculi ~ medullary and cortical o Ureteral colic, fever, dysuria and flank pain
(5%) nephrocalcinosis and nephrolithiasis
• Lab data
Papillary blush o Renal function: ± Impaired acidification or
• Homogeneous with no individual tubules concentration of urine, absorptive and renal leak
• IVP: Cone-shaped density fades into cortex at base hypercalciuria and distal renal tubular acidosis
• Dose related, less prominent on delayed film, o Urinalysis: ± Hematuria, ± positive culture
accentuated by ureteral compression and no o Blood tests: ± Positive culture
nephrocalcinosis • Diagnosis: Incidental findings by urography
3 Demographics
• Gender
62 I PATHOLOGY o Unknown
General Features o Males < females in disease morbidity
• General path comments: No cysts in other organs Natural History & Prognosis
including liver (unlike autosomal dominant or • Number and size of renal calcifications i with time
autosomal recessive polycystic kidney disease • Previously uninvolved papillae develops new cavities
• Genetics: Sporadicj occasionally hereditary (Le., Caroli (less common)
disease, hemihypertrophy, Beckwith-Wiedemann • Complications: Infection, extensive calculus
syndrome, Ehlers-Danlos syndrome, autosomal formation, renal insufficiency and failure
recessive polycystic kidney disease) • Prognosis: Very good, patients have normal life span if
• Etiology without complications
o Unknown
o Pathogenesis: Unknownj developmental/progressive Treatment
degeneration of collecting tubules by i in age • Only treat complications, otherwise, no treatment
• Epidemiology o Infection: Antibiotics
o Prevalence estimated at 1 in 5,000-20,000 o I Stone formation: Thiazide & alkali therapy
o Incidence by urography: 0.5% o Stone removal: Extracorporeallithotripsy and
o Incidence in nephrolithiasis patients: 2.6-21 % percutaneous nephrolithotomy
• Associated abnormalities
o Predisposing to urolithiasis (urinary stasis)
o 33-50% hypercalcemia (Le., hyperparathyroidism) I DIAGNOSTIC CHECKLIST
Gross Pathologic & Surgical Features Consider
• Cross-section of kidney appears as a sponge • Tubular ectasia can be caused by a adjacent tumor;
• Multiple cystic cavities ranging from 1-7.5 mm in need to evaluate
diameter in one or more pyramids
• Dilatation of the terminal portion of the collecting Image Interpretation Pearls
tubules and papillary Bellini's ducts • Bilateral, "paint brush" appearancej medullary
• Cysts confined to papillary portion of the pyramids calcifications
• Cysts contain yellow-brown fluid and desquamated
cells or calcified material
• Cysts communicate proximally with collecting tubules I SELECTED REFERENCES
and distally with papillary ducts or the calyx 1. Levine E et al: Current concepts and controversies in
• Occasionally have inter- or non communication imaging of renal cystic diseases. Urol Clin North Am.
• Calculi within cysts and their walls; calcium oxalate ± 24(3):523-43, 1997
calcium phosphate 2. Ginalski JM et al: Medullary sponge kidney on axial
computed tomography: comparison with excretory
Microscopic Features urography. Eur J Radiol. 12(2):104-7, 1991
• Minimally ectatic collecting tubules 3. Ginalski JM et al: Does medullary sponge kidney cause
o Normal columnar epithelium in interpapillary & nephrolithiasis? AJRAm J Roentgenol. 155(2):299-302,
1990
high cuboidal epithelium in proximal
4. Boag GS et al: CT visualization of medullary sponge
intramedullary regions kidney. Urol Radiol. 9(4):220-1, 1988
o Normal appearing medullary interstitium 5. Parks JH et al: Calcium nephrolithiasis and medullary
• Large or prominent cysts sponge kidney in women. N Engl] Med. 306(18):1088-91,
o Cell lining: Transitional, columnar, or stratified 1982
(rare) squamous epithelium 6. Madewell JE et al: Radiologic-pathologic correlations in
o Interstitial fibrosis and inflammation cystic disease of the kidney. Radiol Clin North Am.
17(2):261-79, 1979
 MEDULLARY SPONGE KIDNEY
I IMAGE GALLERY
Typical
(Left) Axial NEeT shows
medullary nephrocalcinosis
and calculi. (Right) Axial
CECT shows paintbrush
appearance of dilated
tubules and
nephrocalcinosis.

3
63

(Left) Excretory urogram

shows classic "paintbrush"
appearance of tubular
ectasia bilaterally. (Right)
Oblique film from urogram
shows severe ductal ectasia
with beaded cavities
replacing the renal papillae
and wide calices. Note
obstructing calculus (arrow).

Typical
(Left) Radiograph shows
unilateral medullary sponge
kidney with multiple
medullary calculi. (Right)
Urogram shows unilateral
(left) medullary sponge
kidney with dilated tubules
containing innumerable
calculi.
 RENAL ARTERY STENOSIS

3
64

Angiography shows small left renal artery (and kidney). Angiogram shows beaded appearance of mid portion of
Right renal artery is concentrically stenotic just beyond renal artery due to fibromuscular dysplasia.
its origin from atherosclerosis.

• Majority of affected: Above 50 years old (M > F)

ITERMINOLOGY • Bilateral: In 30-40% of atherosclerotic RAScases
Abbreviations and Synonyms a Fibromuscular dysplasia (FMD)
• Renal artery stenosis (RAS) • Second most common cause of RAS
• Most common cause of RVH in children & young
Definitions adults
• Narrowing of renal arterial lumen • Account for one-third of cases of RAS
• Stenosis: Due to medial fibroplasia (> common)
• Location: Mid or distal main renal artery (R > L)
IIMAGING FINDINGS • Majority of affected: Young & middle-aged (F > M)
• Bilateral: In 2/3 cases of fibromuscular dysplasia
General Features a Classification of fibromuscular dysplasia: Based on
• Best diagnostic' clue: Focal or segmental luminal site of involvement & pathology within vessel wall
narrowing of renal artery • Medial fibroplasia (70-80%): Most common type
• Location • Medial or fibromuscular hyperplasia (2-3%)
a RAS(atherosclerosis) • Intimal hyperplasia (10-15%)
• Renal artery ostium or within proximal 2 cm • Perimedial or subadventitial fibroplasia (15%)
a RAS(fibromuscular dysplasia): Right> left
• Mid or distal main RA ± intrarenal arteries Ultrasonographic Findings
• Other general features • Color Doppler
a Most common cause of secondary hypertension a Direct signs: Criteria to diagnose RASor occlusion
a Renovascular hypertension (RVH) • 1 In peak systolic velocity 100-200 cm/sec (mild
• Accounts 1-4% of all patients with hypertension with < 50% stenosis)
a Atherosclerotic disease • 1 In peak systolic velocity> 200 cm/sec (severe
• Most common cause of renal artery stenosis (RAS) with 50-99% stenosis)
• Accounts for two-thirds of cases of RAS • Renal-to-aortic ratio of peak systolic velocity> 3.5
• Stenosis: Due to atherosclerotic plaque & Ca++ • Poststenotic turbulent flow + spectral broadening
• Location: Ostium or proximal 2 cm of renal artery ± flow reversal

DDx: Focal Narrowing of Renal Artery

Aortic Dissection Traumatic RA Dissect.

 RENAL ARTERY STENOSIS
Key Facts
Terminology
• Renal artery stenosis (RAS)
Imaging Findings
• 1 In peak systolic velocity 100-200 cm/sec (mild with
< 50% stenosis)
• 1 In peak systolic velocity> 200 cm/sec (severe with
50-99% stenosis)
• Renal-to-aortic ratio of peak systolic velocity> 3.5
• Poststenotic turbulent flow + spectral broadening ±
flow reversal
• Color imaging: Focal color aliasing (in systolic
turbulence phase)
• Focal/segmental; eccentric/concentric stenosis
• Truncated (ostial stenosis); ± Ca++; uni-/bilateral
• Stenosis; "string-of-beads" pattern; focal aneurysms
• Color imaging: Focal color aliasing (in systolic
turbulence phase)
o Indirect signs: Distal manifestations of proximal RAS
• Pulsus tardus-parvus: Dampened Doppler
waveform during systole
• Early systolic acceleration: < 20-30 cm/sec (best
predictor)
• Time of acceleration: > 0.05-0.08 seconds
• Resistive index: < 5% on affected side compared to
contralateral normal kidney
o Administration of captopril
• 1 Detection when percentage of RAS is > 50%
Angiographic Findings
• CTA & MRA following bolus contrast injection
• Angiography: Catheter injection + digital subtraction
angiography (DSA)
o Atherosclerotic lesions
• Focal/segmental; eccentric/concentric stenosis
• Location: Ostium or proximal 2 cm of renal artery
• Truncated (ostial stenosis); ± Ca++; uni-/bilateral
o Medial fibroplasia
• Stenosis; "string-of-beads" pattern; focal
aneurysms
• Location: Mid or distal long segment of main RA ±
intrarenal arteries
• Uni-/bilateral RAS
o Medial hyperplasia or fibromuscular hyperplasia
• Focal concentric narrowing of distal main RA +
intrarenal branches
o Secondary signs of RAS
• Post-stenotic dilatation ± collateral vessels
• Decreased or delayed or absent nephrogram
• Normal or atrophic kidney
Imaging Recommendations
• Digital subtraction angiography (DSA)
• MR angiography (MRA)
o Multiplanar, contrast-enhanced, 3D, maximum
intensity projection, fast gradient echo sequences
• Color Doppler US (3.5-MHz curvilinear probe)
• CT angiography (CTA)
o MIP; volume rendering techniques (complimentary)
Top Differential Diagnoses
• Arterial dissection
• Vasculitis
• Extrinsic compression
Pathology
• Congenital RAS
• Atherosclerosis (60-90%): > Common in elderly
• Fibromuscular dysplasia (10-30%): Young females
Diagnostic Checklist
• RAS in a patient with high or sudden 1 in blood
pressure + unexplained impairment of renal function 3
• Atherosclerotic RAS: Ostial or proximal 2 cm
narrowing of renal artery (RA) 65
• FMD-RAS: Mid or distal main RA narrowing ±
intrarenal arteries ("string-of-beads" appearance)
I DIFFERENTIAL DIAGNOSIS
Arterial dissection
• E.g., aortic dissection extending into renal artery
• Classification of aortic dissection based on DeBakey
system
o Type I: Extend from aortic root into descending
aorta
o Type II: Involve only ascending aorta
o Type III: Extend from distal to left subclavian artery
into abdomen
• Stanford classification of aortic dissection
o Type A: Involves ascending aorta ± distal extent
• Surgical treatment
o Type B: Distal to origin of left subclavian artery
• Medical treatment
• Aortic dissection + extension into renal artery (type III)
• Most frequently seen in elderly people (M > F)
• Pathogenesis: Aging ~ aortic media degeneration
• Predisposing conditions
o Atherosclerosis, hypertension, pregnancy
o Aortic stenosis, bicuspid aortic valve, coarctation,
trauma
o Marfan syndrome & other connective tissue
disorders
• Digital subtraction angiography (DSA) or CT
angiography
o Irregular caliber of aortic lumen
o False or occluded lumen + intimal flap
o Thickened aortic wall
o Narrowing or occlusion of renal artery due to a false
lumen of dissection
• May occlude or narrow renal artery at its origin
• May extend into renal artery producing more
distal narrowing
• MR imaging
o Spin echo sequences: Demonstrate aortic dissection
with intimal flap + extension up to renal arteries
Vasculitis
• E.g., polyarteritis nodosa; Takayasu arteritis
• Takayasu: Inflammation of medium-large arteries
• Most common in Asian women 15-45 y
 RENAL ARTERY STENOSIS
• Etiology: Unknown
• Pathology: Fibrous thickening of wall of aorta
narrowing orifices of major branches
• Common location: Orifices of aortic arch vessels &
pulmonary arteries
o Occasionally distal part of aorta & its branches
• Renal artery involvement is particularly common
• May also cause atypical coarctation of distal aorta with
narrowing of renal arteries
• Clinical presentation
o Aortic arch vessels
• Visual & neurological abnormalities
• Weak pulses in upper extremities
3 o Renal artery involved: Hypertension
Extrinsic compression
66 • Causes of extrinsic compression of renal artery
o Aortic aneurysm: Suprarenal & infrarenal
o Retroperitoneal tumors
o Retroperitoneal fibrosis
I PATHOLOGY
General Features
• Etiology
o Congenital RAS
• Due to intimal fibroplasia of distal two-third renal
artery + branches
o Atherosclerosis (60-90%): > Common in elderly
o Fibromuscular dysplasia (10-30%): Young females
o Other causes of RAS
• Thromboembolic; arterial dissection; infrarenal
aortic aneurysm; post radiation
• Buerger & Takayasu disease; polyarteritis nodosa
• Neurofibromatosis; retroperitoneal fibrosis
• Pheochromocytoma; diaphragmatic crus
• Epidemiology: Seen in adults < 5% of HTN patients
Gross Pathologic & Surgical Features
• RAS (atherosclerosis)
o Eccentric plaque in ostium or proximal RA
• RAS (fibromuscular dysplasia): Medial fibroplasia
o "String-of-beads" appearance in mid or distal RA
• Mild RAS: Normal size kidney
• Moderate-severe RAS: Atrophic kidney
• Post-stenotic dilatation of main RA ± collateral vessels
Microscopic Features
• RAS (atherosclerosis)
o White-yellow intimal plaque of vessel wall
o Mixture of fatty, fibrous & calcium components
• RAS (fibromuscular dysplasia)
o Focal muscular thinning of media + fibrous ridges
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Very high or sudden t BP in a child, young adult or
after age 50 years; epigastric or flank bruit
o t BP + unexplained impairment of renal function
o Symptoms of increased intracranial pressure
• Papilledema, headache, vomiting
• Lab data
o Positive captopril test
• Exaggerated t in plasma renin activity (PRA)
o ± Impaired renal function tests (based on stenosis)
Demographics
• Age
o RAS (atherosclerosis): Adult age group
o RAS (fibromuscular dysplasia): Young & middle-age
• Gender
o RAS (atherosclerosis): M = F
o RAS (fibromuscular dysplasia): M < F (1:3)
Natural History & Prognosis
• Complications
o Severe HTN; CVA; azotemia; occlusion
o Infarction; dissection; thrombosis
• Prognosis
o Transluminal angioplasty
• Success rate: Nonostial 80%; ostial lesions 25-30%
o Surgical revascularization
• Success rate: 80-90% for any lesion & location
• FMD: t Incidence of restenosis & improved GFR
Treatment
• Angiotensin converting enzyme (ACE) inhibitors
• Transluminal angioplasty
• Surgical revascularization (80-90% success rate)
I DIAGNOSTIC CHECKLIST
Consider
• Rule out other causes of RAS especially dissection
• RAS in a patient with high or sudden t in blood
pressure + unexplained impairment of renal function
Image Interpretation Pearls
• Atherosclerotic RAS: Ostial or proximal 2 cm
narrowing of renal artery (RA)
• FMD-RAS: Mid or distal main RA narrowing ±
intrarenal arteries ("string-of-beads" appearance)
I SELECTED REFERENCES
1. Sheth S et al: Multi-detector row CT of the kidneys and
urinary tract: techniques and applications in the diagnosis
of benign diseases. Radiographies. 24(2):e20, 2004
2. Urban BA et al: Three-dimensional volume-rendered CT
angiography of the renal arteries and veins: normal
anatomy, variants, and clinical applications.
Radiographies. 21(2):373-86; questionnaire 549-55, 2001
3. Kawashima A et al: CT evaluation of renovascular disease.
Radiographies. 20(5):1321-40, 2000
4. Johnson PT et al: Renal artery stenosis: CT
angiography--comparison of real-time volume-rendering
and maximum intensity projection algorithms. Radiology.
5.
211(2):337-43, 1999
Gilfeather M et al: Renal artery stenosis: evaluation with
conventional angiography versus gadolinium-enhanced
MR angiography. Radiology. 210(2):367-72, 1999
6. Rubin GD et al: Helical CT angiography of renal artery
stenosis. AJRAm J Roentgenol. 168(4):1109-11, 1997
 RENAL ARTERY STENOSIS

I IMAGE GALLERY

(Left) Axial CECT in a 41

year old woman with
worsening hypertension
shows global atrophy of right
kidney and a beaded renal
artery due to fibromuscular
dysplasia (FMD). (Right) CT
Angiography shows beaded
appearance of renal artery
due to fibromuscular
dysplasia. 3
67

Typical
(Left) Coronal T1WI MR
(nonenhanced) in 70 year
old man shows atrophic left
kidney (arrow). (Right) MR
Angiography shows
abdominal aortic aneurysm,
tiny left and stenotic right
renal artery.

Typical
(Left) Color Doppler
sonography with gate over
left renal artery shows high
velocity flow (3.7 m/sec)
indicating renal artery
stenosis. (Right) Color
Doppler sonography shows
color aliasing within the left
renal artery indicating
turbulent, rapid flow, signs of
renal artery stenosis.
 RENAL INFARCTION

3
68

Axial CECT shows nonenhancement of left kidney due Axial CECT shows global nonenhancement of right
to aortic dissection occluding renal artery. kidney due to embolic occlusion of artery. Atrial
fibrillation.

• Solitary or multiple, frequently associated with

ITERMINOlOGY other renal injuries
Definitions • Finally results in a renal scar
• Localized or global area of ischemic necrosis in kidney, o Global traumatic renal infarct (less common)
resulting most often from sudden occlusion of its • Categorized as grade III renal injury
arterial supply • Thrombosis, transection or avulsion of main RA
Radiographic Findings
• IVP
IIMAGING FINDINGS o Segmental or subsegmental infarction
General Features • Focal absent or decreased nephrogram
o Global infarction
• Best diagnostic clue: Nonenhancing wedge-shaped
• Complete absence of nephrogram + excretion
area + enhancing cortical rim
• Other general features CT Findings
o Major causes of renal infarction • CT findings may be variable based on etiology of
• Thrombosis, embolism, trauma infarction
o Classification based on onset o Embolic infarction: Multifocal & bilateral
• Acute, subacute & chronic renal infarction o Traumatic & thrombotic infarction: Segmental or
o Classification based on anatomy & vascular global & unilateral
distribution • Focal subsegmental infarction
• Focal: Segmental or subsegmental infarction o Small, sharply demarcated, wedge-shaped area of
(cortex ± medulla) decreased or poor contrast-enhancement
• Global infarction o Base of wedge pointed towards renal capsule & apex
o Focal traumatic segmental or subsegmental renal towards hilum
infarct (more common) • Focal segmental infarction
• Categorized as grade I & II renal injuries o Sharply demarcated, dorsal or ventral segmental
• Thrombosis or laceration of a segmental renal decreased enhancement area
arterial (RA)branch

DDx: Striated, Wedge or Global Nonenhancement

,--
, .. ' ..
"-

Pyelonephritis
'~f ••
Pyelonephritis Polyarteritis Nodosa Renal Laceration
 RENAL INFARCTION
Key Facts
Terminology
• Localized or global area of ischemic necrosis in
kidney, resulting most often from sudden occlusion
of its arterial supply
Imaging Findings
• Best diagnostic clue: Nonenhancing wedge-shaped
area + enhancing cortical rim
• Total absence of renal enhancement + no excretion +
no perinephric hematoma (renal artery thrombosis)
• Total absence of enhancement + large perinephric
hematoma (renal artery avulsion)
• "Cortical rim" sign: Reliable sign of subacute
infarction
• Small-sized kidney with smooth or irregular contour
(chronic infarction)
o A straight-line demarcation between normal
enhancing & abnormal non enhancing parenchyma
• Strongly suggestive of ischemia
• Global infarction
o Total absence of renal enhancement + no excretion
+ no perinephric hematoma (renal artery
thrombosis)
• Renal outline is preserved
o Total absence of enhancement + large perinephric
hematoma (renal artery avulsion)
o ± Medullary striations: "Spoke-wheel" pattern
enhancement (due to collateral circulation)
• Acute renal infarction
o Normal or large-sized kidney; smooth contour; ±
subcapsular fluid collection
o Absent or decreased nephrogram
o "Cortical rim" sign: Reliable sign of subacute
infarction
• Preserved capsular or subcapsular enhancement
• Usually seen 6-8 hrs after infarction
• Seen in 50% cases of infarction (due to intact
collateral circulation)
• Chronic renal infarction
o Small-sized kidney with smooth or irregular contour
(chronic infarction)
o Absent or diminished enhancement; no "cortical
rim" sign
MR Findings
• Tl WI: Low signal intensity lesion
• T2WI: Low signal intensity lesion
• Tl C+: Sharply demarcates nonenhanced infarcted
area from densely enhancing noninfarcted portion
Ultrasonographic Findings
• Real Time: Findings of renal infarction are nonspecific
• Color Doppler: May show focal or global absence of
blood flow in involved kidney
Angiographic Findings
• Conventional
o Diagnosis of renal infarction: Confirmed at selective
renal arteriography
Top Differential Diagnoses
• Pyelonephritis
• Vasculitis
• Renal trauma
Pathology
• Embolism: Cardiac (most common)
• Thrombosis
• Trauma
Diagnostic Checklist
• Correlate with history: Trauma, cardiac, aortic disease
• Pyelonephritis + acute infarction may have similar 3
appearance
• Straight line demarcation + "cortical rim" sign favor 69
infarction
• Perinephric stranding favors pyelonephritis
• Focal segmental infarction: Nonopacification of
focal cortical area
• Global infarction: Total absence of renal
enhancement
Nuclear Medicine Findings
• SPECT imaging with Tc99m DMSA
o Acute infarction: Photon deficient area
Imaging Recommendations
• Helical CECT; color Doppler sonography
• Selective renal angiography
I DIFFERENTIAL DIAGNOSIS
Pyelonephritis
• Acute pyelonephritis
o Cortical wedge-shaped or striated nephrogram
• May simulate focal segmental or sub segmental
infarction
• "Cortical rim" sign favors infarction
o Loss of normal corticomedullary differentiation
• Corticomedullary differentiation is intact in focal
infarction & is lost in global infarction
o Renal enlargement, focal swelling
o Thickening of Gerota fascia + perinephric stranding
o Calyceal effacement, dilated renal pelvis & ureter
which are not seen in acute infarction
• Chronic pyelonephritis
o Small kidney with cortical scarring over dilated
calices
o Unilateral with compensatory hypertrophy of
contralateral kidney
o May simulate chronic global renal infarction
Vasculitis
• eg; Polyarteritis nodosa; SLE;scleroderma; drug abuse
• Wedge-shaped or striated nephrogram (usually
bilateral & diffuse)
• Both vasculitis & infarction cause parenchymal
scarring + capsular retraction (may be same pathology)
• Microaneurysmal dilatation of small vessels
 RENAL INFARCTION
o Antithrombolytics, anticoagulants,
Renal trauma antihypertensives
• Best imaging clue • Surgery or angioplasty
o Irregular linear or segmental nonenhancing tissue & o Atherosclerotic renal artery stenosis
subcapsular or perinephric hematoma • Nephrectomy
• Lacerations: Irregular or linear hypodense areas o Irreversible traumatic global infarction
• Segmental infarction
o Nonenhancing wedge-shaped areas
• "Shattered kidney" I DIAGNOSTIC CHECKLIST
o Renal artery avulsion
• Global infarction + perinephric hematoma Consider
o Renal artery thrombosis • Correlate with history: Trauma, cardiac, aortic disease
• Global infarction + no perinephric hematoma
Image Interpretation Pearls
3 • Pyelonephritis + acute infarction may have similar
I PATHOLOGY appearance
70 • Straight line demarcation + "cortical rim" sign favor
General Features infarction
• Etiology • Perinephric stranding favors pyelonephritis
o Embolism: Cardiac (most common)
• Rheumatic, arrhythmias, MI, prosthetic valve, SBE
o Thrombosis I SELECTED REFERENCES
• Atherosclerosis, polyarteritis nodosa 1. Suzer 0 et al: CT features of renal infarction. Eur J Radiol.
• Aneurysm or dissection (aorta/renal artery) 44(1):59-64, 2002
• Sickle cell disease, thrombotic thrombocytopenic 2. Kawashima A et al: Imaging evaluation of posttraumatic
purpura, thromboangiitis obliterans renal injuries. Abdom Imaging. 27(2):199-213, 2002
o Trauma 3. Kawashima A et al: Imaging of renal trauma: a
• Blunt or penetrating comprehensive review. Radiographies. 21(3):557-74, 2001
• Surgery, interventional procedures 4. Kawashima A et al: CT evaluation of renovascular disease.
Radiographies. 20(5):1321-40, 2000
• Associated abnormalities: Cardiac abnormalities,
5. Carey HB et al: Bilateral renal infarction secondary to
hypercoagulable state, aortic aneurysm or dissection paradoxical embolism. AmJ Kidney Dis. 34(4):752-5, 1999
Gross Pathologic & Surgical Features 6. Kawashima A et al: CT of renal inflammatory disease.
Radiographies. 17(4):851-66; discussion 867-8, 1997
• Wedge shaped infarct (white or pale in color) 7. Dalla-Palma L et al: Delayed CT in acute renal infection.
• Renal artery thrombus or traumatic avulsion Semin Ultrasound CT MR. 18(2):122-8, 1997
• Large or small sized kidney + smooth or irregular 8. Nunez D Jr et al: Traumatic occlusion of the renal artery:
contour helical CT diagnosis. AJRAm J Roentgenol. 167(3):777-80,
1996
Microscopic Features 9. Kamel IR et al: Assessment of the cortical rim sign in
• Focal or global renal ischemic changes posttraumatic renal infarction. J Com put Assist Tomogr.
• Necrosis & scarring 20(5):803-6, 1996
10. Krinsky G: Unenhanced helical CT in patients with acute
flank pain and renal infarction: the need for contrast
material in selected cases. AJRAm J Roentgenol.
I CLINICAL ISSUES 167(1):282-3, 1996
11. Saunders HS et al: The CT nephragram: implications for
Presentation evaluation of urinary tract disease. Radiographies.
• Most common signs/symptoms 15(5):1069-85; discussion 1086-8, 1995
o Asymptomatic, flank pain, tenderness (traumatic), 12. Fanney DR et al: CT in the diagnosis of renal trauma.
hematuria Radiographies. 10(1):29-40, 1990
o Hypertension in chronic infarction 13. Bankoff MS et al: Computed tomography differentiation of
pyelonephritis and renal infarction. J Com put Tomogr.
Demographics 8(3):239-43, 1984
• Age: Any age group 14. Wong WS et al: Renal infarction: CT diagnosis and
• Gender: Equal in both males & females (M = F) correlation between CT findings and etiologies. Radiology.
150(1):201-5, 1984
Natural History & Prognosis 15. Haaga JR et al: CT appearance of renal infarct. J Com put
Assist Tomogr. 4(2):246-7, 1980
• Complications
16. Sant GR et al: Computed tomography in evaluation of
o Necrosis, infection, abscess formation blunt renal trauma. Potential for misdiagnosis of renal
• Prognosis infarction. Ural Int. 43(6):321-3, 1988
o Focal infarction: Good
o Global infarction: Poor
Treatment
• Medical
 RENAL INFARCTION

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
straight line demarcation
between normal ventral
kidney and infarcted dorsal
half. (Right) Axial CECT
shows global infarction of left
kidney due to emboli from
prosthetic mitral valve. Note
"cortical rim" sign (arrow).

3
71

Typical

~·-
..
~~
(Left) Axial CECT in a patient

.- with recent myocardial

infarct shows global
infarction of left kidney.
-.~ ~
~~

-.
I
(Right) Axial CECT in a
'-.' -" .$- patient with recent
- ~ .... myocardial infarction shows

g.•.
~~
' "
multiple splenic infarctions
••.•.•.• ": ....•• -. 1t
.. as well.
., •• - #.

' !.- /

" Y
Typical
(Left) Axial CECT shows
global atrophy and minimal
function of left kidney due to
chronic ischemia. (Right)
Axial CECT shows renal
cortical loss due to chronic
focal infarction .
 RENAL VEIN THROMBOSIS

,-

"-'ft' \ ~
'-
,.,'
- . 'f:! " -

~
' ' ' , ... ~! " ~:-,-', ,,:If ':1. t'l',',,'
3 ;} ...., ... ;":~ ts.
72 "'. , '''It: '~

Axial CECT shows thrombus as a filling defect (arrow) in Axial CECT shows renal vein thrombus extending into
the left renal vein, the inferior vena cava.

o Dense, prolonged nephrogram (partial)

ITERMINOLOGY o Little or no nephrographic opacification (complete)
Abbreviations and Synonyms o I Amount of opaque urine in renal calyces,
• Renal vein thrombosis (RVT) infundibula and pelvis
o Opacified veins in perinephric space
Definitions o Venous notching or indentation of ureter by
• Obstruction of renal vein by a thrombus tortuous collateral veins
o Rarely, alternating radiopaque and radiolucent
striations in renal cortex
I IMAGING FINDINGS CT Findings
General Features • CECT
• Best diagnostic clue: Mass in renal vein with renal o Low-attenuating filling defect within renal vein
enlargement and delayed renal function o I Nephrographic attenuation
• Location o Persistent parenchymal opacification
o Unilateral> bilateral (more common in children) o No corticomedullary differentiation
o Left renal vein> right renal vein o Delayed corticomedullary junction time and
• Size contrast excretion into renal calyces and pelvis
o Renal enlargement (75% of cases) o Renal vein attenuation and enlargement; easier seen
o Small shrunken kidney (rare) on left side
• Morphology: Mass in renal vein ± extension to inferior o Thickening of the Gerota fascia and perinephric
vena cava (rve) ± right atrium "whiskering" (edema or hemorrhage)
• Other general features o Opacified periureteral and perinephric ("cobwebs")
o Features may vary depending on type of obstruction collaterals
• Acute or chronic (more common) • CTA
• Partial or complete o Tortuous and dilated collateral veins close to ureters
o Venous collateralization
Radiographic Findings o Retrograde flow in dilated superficial epigastric veins
• rvp

DDx: Enlarged Kidney Delayed Function

Ureteral Calculus Renal Cell Cancer Transitional Cell Ca Pyelonephritis

 RENAL VEIN THROMBOSIS

Key Facts
Terminology • Pyelonephritis
• Obstruction of renal vein by a thrombus Pathology
Imaging Findings • Most commonly associated with nephrotic syndrome
• Best diagnostic clue: Mass in renal vein with renal in adults
enlargement and delayed renal function • Most commonly associated with dehydration and
• Renal enlargement (75% of cases) sepsis in children
• Morphology: Mass in renal vein ± extension to Clinical Issues
inferior vena cava (IVe) ± right atrium • Asymptomatic or thromboembolic disease
• Low-attenuating filling defect within renal vein • Age: Adults (more common) or < 2 years of age
• No corticomedullary differentiation • Anticoagulation therapy
• Venous collateralization
• Best imaging tool: US followed by CT or MR Diagnostic Checklist 3
• Adequate return of renal circulation may prevent
Top Differential Diagnoses renal function deterioration
73
• Ureteral obstruction • Filling defect within renal vein; venous collaterals
• Infiltrating tumor

• Protocol advice: CTA: Corticomedullary phase best;

MR Findings second helical acquisition performed 90-120 seconds
• Tl WI: No corticomedullary differentiation
• MRA: High contrast between flowing blood, vascular
walls and surrounding tissues I DIFFERENTIAL DIAGNOSIS
• TlWI, T2WI
o Filling defect in renal vein Ureteral obstruction
o Prolongation of renal cortex and medulla relaxation • Filling defect and irregular narrowing of ureter
times ~ low signal intensity • ! Contrast density in collecting system
o Low signal intensity band in outer part of medulla • Hydronephrosis; hydroureter; perinephric or
o Obliteration of renal sinus fat and compression of periureteral stranding
renal collecting systems
o 1 Attenuation of renal veins Infiltrating tumor
o Multiple perinephric collateral veins • E.g., transitional cell carcinoma, renal metastases and
o Gonadal vein dilatation lymphoma
• Poorly defined margins without change in shape
Ultrasonographic Findings • Heterogenous mass ± calcification
• Real Time • Enhancing mass with soft tissue attenuation
o Renal edema
o ! Echogenicity (acute), then 1 (after 10-14 days) Pyelonephritis
o ± Corticomedullary differentiation • Multiple necrotic masses with renal enlargement
o Thrombus or tumor in IVC (:$ 20%) • Differentiate by clinical history and urinalysis
o Intraluminal echoes with renal vein dilatation
proximal to occlusion; easier seen on left> right side
• Color Doppler I PATHOLOGY
o Renal artery and proximal branches
• Peaked, sudden! systolic-frequency shifts General Features
• Retrograde plateau-like shifts during diastole • General path comments
o Absent venous signal o Most commonly associated with nephrotic
o 1 Blood velocity and turbulence (partial) syndrome in adults
o No blood flow (complete) o Most commonly associated with dehydration and
o Anechoic or hypoechoic clot (acute) sepsis in children
o Echogenic thrombus (chronic) • Genetics: Inherited hypercoagulable states (e.g.,
antithrombin III, protein S, protein C deficiency)
Angiographic Findings • Etiology
• Conventional o Nephrotic syndrome
o Reflux into renal vein • Membranous glomerulonephritis
o Venous collateralization • Membranoproliferative glomerulonephritis
• Focal sclerosis
Nuclear Medicine Findings • Amyloidosis
• Delayed or absent renal perfusion • Lipoid nephrosis
Imaging Recommendations o Other primary renal disease
• Other glomerulonephritis
• Best imaging tool: US followed by CT or MR
• Pyelonephritis
 RENAL VEIN THROMBOSIS
• Vasculitis • Asymptomatic or thromboembolic disease
o Renal hypoperfusion by hypovolemia or vascular • Fever, edema
stasis • Lab data
• Dehydration o Acute: Urinalysis: Gross or microscopic hematuria
• Sepsis o Chronic: Urinalysis: Proteinuria
• Gastrointestinal fluid loss
• Hemorrhage
Demographics
• Congestive heart failure • Age: Adults (more common) or < 2 years of age
• Aortic insufficiency Natural History & Prognosis
• Constrictive pericarditis
• Effects of RVT depend on site of origin, time to
o Tumor extension
occlusion, collateral veins and extent of recanalization
• Renal cell carcinoma
• Complications: Pulmonary embolism (most common),
• Renal angiomyolipoma
widespread thrombosis, renal hemorrhage, atrophy
3 • Wilms tumor
• Transitional cell carcinoma
and failure
• Prognosis: Good, with frequent spontaneous recovery
• Metastasis (e.g., adrenal, gonadal carcinoma)
74
o Iatrogenic Treatment
• Drugs (e.g., oral contraceptive pills, estrogens) • Anticoagulation therapy
• Abdominal surgery o Intravenous heparin, then oral warfarin
• Renal transplant rejection • Thrombolytic therapy: Bilateral RVT,extension of RVT
o Other hypercoagulable states into IVC, massive clot, pulmonary emboli, severe flank
• Pregnancy pain or failed anticoagulation therapy
• Disseminated malignancy • Steroids or other immunosuppressive medications:
• Septic abortion Autoimmune disease
• Genetics • Suprarenal vena cava filter: Thrombus extending into
o Abdominal trauma IVC
o Thrombus extension • Surgical thrombectomy or nephrectomy: Failed
• Left ovarian vein thrombosis medical treatment, tumor thrombus
• Deep vein thrombosis (e.g., leg, pelvic)
• Retrograde IVC extension
o Other systemic disease I DIAGNOSTIC CHECKLIST
• Polyarteritis nodosa
• Diabetes mellitus (e.g., maternal diabetes, Consider
glomerulosclerosis) • Adequate return of renal circulation may prevent renal
• Sickle cell anemia function deterioration
• Systemic lupus erythematosus
o Mechanical compression Image Interpretation Pearls
• Pregnancy • Filling defect within renal vein; venous collaterals
• Retroperitoneal fibrosis
• Tumor (e.g., lymphoma, carcinoma of tail of
pancreas) I SELECTED REFERENCES
• Abscess 1. Urban BA et al: Three-dimensional volume-rendered CT
• Hematoma (e.g., adrenal) angiography of the renal arteries and veins: normal
• Urinoma anatomy, variants, and clinical applications.
• Lymphocele Radiographies. 21(2):373-86; questionnaire 549-55, 2001
• Aberrant arteries 2. Kawashima A et al: CT evaluation of renovascular disease.
Radiographies. 20(5):1321-40, 2000
• Arterial aneurysms
3. Tempany CM et al: MRI of the renal veins: assessment of
• Epidemiology nonneoplastic venous thrombosis. J Com put Assist
o Incidence Tomogr. 16(6):929-34, 1992
• Unknown in asymptomatic patients 4. Gatewood OM et al: Renal vein thrombosis in patients
• Nephrotic syndrome: 16-42% of patients with nephrotic syndrome: CT diagnosis. Radiology.
159(1):117-22, 1986
Gross Pathologic & Surgical Features 5. Glazer GM et al: Computed tomography of renal vein
• Renal fibrosis, hemorrhage, necrosis, calcification thrombosis. J Comput Assist Tomogr. 8(2):288-93, 1984
6. Jeffrey RB et al: CT and ultrasonography of acute renal
abnormalities. Radiol Clin North Am. 21(3):515-25, 1983
I CLINICAL ISSUES 7. Bradley WG Jr et al: Renal vein thrombosis: occurrence in
membranous glomerulonephropathy and lupus nephritis.
Presentation Radiology. 139(3):571-6, 1981
8. Chait A et al: Renal vein thrombosis. Radiology.
• Most common signs/symptoms 90(5):886-96, 1968
o Acute (more common in children)
• Flank pain, nausea, vomiting
• Palpable kidney, hypertension
o Chronic
 RENAL VEIN THROMBOSIS
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
renal cell carcinoma
replacing most of kidney and
invading renal vein and IVC
(arrows). (Right) Axial CECT
shows transitional cell
carcinoma infiltrating left
kidney and extending into
renal vein and IVC (arrows).

3
75

Typical
(Left) Axial CECT (delayed
excretory phase) in a 79 year
old boy shows prolonged
nephrogram in left kidney,
hyperdense clot in renal vein
(arrow). (Right) Axial CECT
in a 79 year old boy shows
left persistent nephrogram,
decreased function, perirenal
standing. Acute RVT due to
glomerulonephritis.

(Left) Axial NEeT in a girl

with left flank pain and
chronic hematuria shows
compression of left renal
vein by superior mesenteric
artery; "nutcracker" (arrow).
(Right) Sagittal sonogram of
patient with two-week
history of flank pain and
hematuria. Kidney is swollen,
echogenic with loss of
corticomedullary
differentiation due to RVT.
 RENAL TRAUMA

3
76

Axial CECTshows deep laceration of right kidney with Renal angiogram shows active bleeding (arrow) that
perirenalblood and active hemorrhage (arrow). was controlled by coil embolization of the bleeding
vessel.

• No extension to collecting system or medulla

ITERMINOLOGY • Small sub segmental cortical infarct
Definitions o Grade II: 10% of all renal injuries
• Injury to the kidney • Major injury (major cortical laceration + extension
to medulla & collecting system)
• With or without urine extravasation or segmental
I IMAGING FINDINGS renal infarct
o Grade III: 5% of cases
General Features • Catastrophic injury (multiple renal lacerations &
• Best diagnostic clue: Renal parenchymal defect with vascular injury involving renal pedicle)
perirenal hemorrhage ± extravasation of blood/urine o Grade IV: Rare consequence
• Other general features • Ureteropelvic junction injury: Complete
o Seen in 8-10% of patients with blunt or penetrating transection or laceration
abdominal injuries
Radiographic Findings
o 80-90% of cases involve blunt rather than
penetrating injury • IVP
o Grade I: Normal
o Serious renal injuries usually associated with
o Grade II-IV
multi-organ involvement
• Delayed, absent excretion or extravasation
• Due to penetrating trauma: 80% of cases
• Due to blunt trauma: 75% of cases CT Findings
o 98% of isolated renal injuries are minor & require no • Grade I lesions
specific therapy o Intrarenal hematoma or contusion
o Radiologic classification of renal injuries • Ill-defined, round or ovoid lesion
• Grade I to IV • Parenchymal phase: ! Enhancement relative to
o Grade I: 75-85% of all renal injuries normal kidney
• Minor injury (contusion; intrarenal or subcapsular • Delayed phase: Hyperdense due to urine stasis +
hematoma) clot filled tubules
• Minor laceration + limited perinephric hematoma o Subcapsular hematoma

DDx: Parenchymal lesion & Perirenal Fluid

Bleeding AML Bleeding RCC Polyarteritis Nodosa Polyarteritis Nodosa

 RENAL TRAUMA
Key Facts
Imaging Findings
• Best diagnostic clue: Renal parenchymal defect with
perirenal hemorrhage ± extravasation of blood/urine
• Segmental renal infarct: Sharply demarcated,
wedge-shaped area of decreased enhancement
• Global infarction (nonenhancement) + no
perinephric hematoma (renal artery thrombosis)
• Global infarction (nonenhancement) + perinephric
hematoma (renal artery avulsion)
• Protocol advice: For any renal laceration evident on
CT, must obtain 8-10 min. delayed scans to evaluate
for urinary extravasation
Top Differential Diagnoses
• Renal tumor
• Vasculitis
• Round or elliptic fluid collection (40-70 HU
clotted blood)
o Minor lacerations: Small linear hypodense areas in
periphery
o Limited perinephric hematoma: Adjacent to
laceration
o Subsegmental cortical infarct
• Small, sharply demarcated, wedge-shaped
decreased attenuation area ~ scar
• Grade II lesions
o Major laceration through cortex extending to
medulla
• Long irregular or linear hypodense area
o When laceration extends into collecting system
• Nephrographic phase: Large, distracted renal
fracture (hypo dense)
• Excretory phase: Contrast extravasation into
perinephric space
• ± Antegrade filling of ureter
o Segmental renal infarct: Sharply demarcated,
wedge-shaped area of decreased enhancement
• Grade III lesions
o Multiple renal lacerations & vascular injury
• Nephrographic phase: Several irregular, linear or
band like interpolar hypodense areas ± areas of
active arterial contrast extravasation
o Subacute infarction
• "Cortical rim" sign: Preserved capsular or
subcapsular enhancement (reliable sign)
• Seen 6-8 hrs aft~r infarction
o "Shattered kidney"
• Segmental infarction: Nonenhancing
wedge-shaped area (devitalized upper or lower
renal pole branch)
• Global infarction (nonenhancement) + no
perinephric hematoma (renal artery thrombosis)
• Global infarction (nonenhancement) +
perinephric hematoma (renal artery avulsion)
• Grade IV lesions
o Ureteropelvic junction: Complete transection
(avulsion) or laceration
• Good excretion of contrast + medial perinephric
extravasation
Pathology
• Blunt, penetrating & deceleration injuries
• Other organ injuries in 75% of cases
Clinical Issues
• Flank pain, tenderness or ecchymosis
• Hematuria, anuria, uremia, shock
• Poor correlation between degree of hematuria &
severity of renal injury
Diagnostic Checklist
• Possibility of underlying renal tumor if bleeding
seems out of proportion to degree of trauma 3
• Arterial extravasation usually requires catheter
embolization to control bleeding 77
• Urinary extravasation often requires ureteral stent ±
catheter drainage of urinoma
• A circumferential urinoma may be seen around
affected kidney
Imaging Recommendations
• Protocol advice: For any renal laceration evident on
CT, must obtain 8-10 min. delayed scans to evaluate
for urinary extravasation
• Helical CECT: Gold standard imaging
• IVU: Limited urography (to evaluate hemodynamically
unstable patient)
o Take a plain film abdomen & administer 100-150 ml
of 60% contrast LV. obtain immediate "cone down"
nephrogram film + full film after 8 min.
o "One-shot IVU": Not to evaluate injured kidney but
to asses normal kidney
• Retrograde pyelography
o To assess ureteral & renal pelvic injuries
• US: To assess hemoperitoneum in a hemodynamically
unstable patient
I DIFFERENTIAL DIAGNOSIS
Renal tumor
• Spontaneous bleed or rupture may be seen in renal
tumors
• Perinephric fluid collection of blood density
• Look for underlying renal mass lesion
o Renal cell carcinoma (RCC)
o Angiomyolipoma
• Renal cell carcinoma
o Solid renal mass & is usually hypervascular
o Renal vein & IVC extension may be seen
o Hypervascular metastatic foci are often seen
• Angiomyolipoma (AML)
o Composed of vascular, smooth muscle & fat
components
o Renal mass with variable amounts of fat is
diagnostic
o May enhance significantly with contrast infusion
Vasculitis
• E.g., polyarteritis nodosa; SLE;scleroderma, drug abuse
 RENAL TRAUMA
• Renal imaging
o Wedge-shaped or striated nephrogram Treatment
o Capsular retraction over parenchymal lesions • Grade I & II: Conservative therapy
o Microaneurysms of small vessels are usually seen • Grade III & IV
o Active bleeding: Angioembolization
o Renal artery (RA) thrombosis: Anticoagulants; stent
!PATHOLOGY placement
o Active urinary extravasation
General Features • Consider ureteral stent & catheter drainage
• Etiology o Indications for surgery
o Motor vehicle accidents (MVA), falls, fights, assaults • Vascular (renal pedicle) injury
• Blunt, penetrating & deceleration injuries • Shattered kidney
o Adults: Kidneys protected by ribs, heavy • Expanding or pulsatile hematoma
musculature of back & flank • Shocked poly trauma patient
3 o Children: Kidneys relatively large, more mobile & o Severely damaged kidney: Surgical nephrectomy
more vulnerable to trauma
78 • Epidemiology
o Renal trauma incidence I DIAGNOSTIC CHECKLIST
• 8-10% of injuries in emergency department
• Associated abnormalities Consider
o Other organ injuries in 75% of cases • Possibility of underlying renal tumor if bleeding seems
• Liver, spleen, bowel, pancreas out of proportion to degree of trauma

Gross Pathologic & Surgical Features Image Interpretation Pearls

• Contusion, laceration, hematoma, infarction, vascular • Arterial extravasation usually requires catheter
or ureteropelvic injury embolization to control bleeding
• Urinary extravasation often requires ureteral stent ±
Microscopic Features catheter drainage of urinoma
• Contusion, laceration, ischemia of cortico-medullary
or collecting system
I SELECTED REFERENCES
1. Smith JK et al: Imaging of renal trauma. Radiol Clin North
I CLINICAL ISSUES Am. 41(5):1019-35, 2003
2. Kawashima A et al: Imaging evaluation of posttraumatic
Presentation renal injuries. Abdom Imaging. 27(2):199-213, 2002
• Most common signs/symptoms 3. YaoDC et al: Using contrast-enhanced helical CT to
o Flank pain, tenderness or ecchymosis visualize arterial extravasation after blunt abdominal
o Hematuria, anuria, uremia, shock trauma: incidence and organ distribution. AJRAmJ
o Poor correlation between degree of hematuria & Roentgenol. 178(1):17-20,2002
4. Harris AC et al: Ct findings in blunt renal trauma.
severity of renal injury Radiographies. 21 Spec No:S201-14, 2001
• 14% of major & 10% of minor injuries may not 5. Mizobata Y et al: Successful evaluation of pseudo aneurysm
have hematuria formation after blunt renal injury with dual-phase
• Clinical profile: Patient with history of MVA, flank contrast -enhanced helical CT.AJRAmJ Roentgenol.
pain, hematuria or anuria 177(1):136-8,2001
• Lab data 6. Kawashima A et al: Imaging of renal trauma: a
o Blood in urine (> 5 RBCs per high power field) comprehensive review. Radiographies. 21(3):557-74, 2001
• Diagnosis: Clinical & classic imaging features are 7. Morey AFet al: Single shot intraoperative excretory
urography for the immediate evaluation of renal trauma. J
diagnostic of renal trauma Urol. 161(4):1088-92, 1999
Demographics 8. Kawashima A et al: Ureteropelvic junction injuries
secondary to blunt abdominal trauma. Radiology.
• Age: Any age group (children> vulnerable than adults) 205(2):487-92,1997
• Gender: M = F 9. Mirvis SE:Trauma. Radiol Clin North Am. 34(6):1225-57,
1996
Natural History & Prognosis 10. Pollack HM et al: Imaging of renal trauma. Radiology.
• Complications 172(2):297-308, 1989
o Early: Urinoma, perinephric abscess, sepsis, AV 11. Federle MP et al: Penetrating renal trauma: CT evaluation. J
fistula, pseudoaneurysm Comput AssistTomogr. 11(6):1026-30, 1987
o Late: Hydronephrosis, HTN, calculus formation, 12. Lang EKet al: Renal trauma: radiological studies.
chronic pyelonephritis, renal failure & atrophy Comparison of urography, computed tomography,
angiography, and radionuclide studies. Radiology.
• Prognosis 154(1):1-6, 1985
o Grade I & II: Good 13. Federle MP et al: The role of computed tomography in
o Grade III & IV renal trauma. Radiology. 141(2):455-60, 1981
• Unilateral after treatment: Good
• Bilateral: Poor
 RENAL TRAUMA
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
deep laceration through the
kidney and a large perirenal
hemorrhage. (Right) Axial
NECT shows a deep
laceration through the
kidney and large perirenal
hemorrhage.

3
79

(Left) Axial CECT shows

nonenhancement of the
ventral half of the kidney but
minimal bleeding, suggesting
traumatic infarction due to
renal arterial branch
occlusion. (Right) Renal
arteriogram shows traumatic
dissection (arrow) that
resulted in segmental renal
infarction.

(Left) Axial NECT (delayed)

shows opacified urine
(arrow) without a
parenchymal renal injury,
suggesting ureteral injury.
(Right) Retrograde
pyelogram shows urinary
extravasation (arrow) due to
traumatic uretero-pelvic
laceration.
 RENAL CYST

3
80

Excretory urogram shows spherical absence of Axial CECT shows water density mass in upper pole
nephrogram and splaying of calices in upper pole with no visible wall; Bosniak I cyst.
(arrow).

o Uncomplicated cyst: Parenchymal deformity at

ITERMINOlOGY edges of cyst ("beak or claw" sign)
Definitions o Neoplastic wall: Rare calcification of mural nodule
• A benign, fluid-filled, nonneoplastic renal lesion CT Findings
• Bosniak classification for renal cysts is standard
o Class I: Benign cysts
I IMAGING FINDINGS • Well-defined, rounded, homogeneous, lucent «
General Features 20 HU, near water density) mass with a thin or
imperceptible, nonenhancing wall
• Best diagnostic clue
o Class II: Minimally complicated cysts; benign
o Water density, nonenhancing, spherical lesion with
• Minimally irregular, calcified, septated
no visible wall on CT
• Hyperdense cyst: Contents 60 to 90 HU, no
o Lucent mass with no visible wall and acoustic
enhancement, spherical, partially exophytic,
enhancement on US
usually .:0:; 3 cm diameter
• Size: Usually 2-5 cm diameter (up to 10 cm)
• Septa are thin « 1 mm), smooth, and attached to
• Other general features
wall without thickened elements or enhancement
o Simple renal cyst classification
o Class IIF: Require follow-up (3, 6, 12-month interval)
• Typical or uncomplicated
• Some hyperdense cysts, lesions with more calcium
• Complicated: Hemorrhagic, infected, ruptured,
in wall or slightly more complicated cysts
neoplasm from cystic wall .
o Class III: More complicated cysts; biopsy
• Atypical: Calcified, hyperdense, septated, multiple
(controversial) or excision
simple, localized cystic disease, milk of calcium
• Irregular and thickened septa ± enhancement
Radiographic Findings • Thickened and irregular calcification
• IVP • Irregular margination
o Large cyst ~ distorts contour & splays, elongates, • Uniform wall thickening
displaces, obstructs or totally obliterates calyces • Multilocular; small, non enhancing nodular mass
• Usually simulate cystic or necrotic renal cell
carcinoma or multilocular cystic nephroma

DDx: Hypodense Renal Mass

Papillary RCC Cystic RCC MC Nephroma Lymphoma

 RENAL CYST
Key Facts
Terminology
• A benign, fluid-filled, nonneoplastic renal lesion
Imaging Findings
• Water density, nonenhancing, spherical lesion with
no visible wall on CT
• Lucent mass with no visible wall and acoustic
enhancement on US
• Bosniak classification for renal cysts is standard
• Best imaging tool: CT (100% sensitivity); US (> 98%)
Top Differential Diagnoses
• Renal carcinoma
• Multilocular cystic nephroma (MCN)
• Renal abscess
• Hydronephrosis
• Metastases and lymphoma
o Class IV: Malignant lesions with large cystic or
necrotic components; require surgery
• Irregular wall thickening or enhancing mass
• Uncomplicated cyst
o Sharply marginated, round, smooth, homogeneous,
hypodense (-10 - +20 HU) mass
o Thin, imperceptible, nonenhancing wall
o Small « 1 cm): Cannot measure region of interest; if
less than blood density on NECT, probably cyst
• Infected cyst: Thick wall, septated, heterogeneous
enhancing fluid, debris- or gas-fluid level; ±
calcification (chronic)
• Hemorrhagic cyst
o NECT: Hyperdense & CECT: Hypodense (flip-flop
phenomenon), homogeneous (70-90 HU) (acute)
o No contrast-enhancement
o Heterogeneous (clot or debris), t wall thickness & j
attenuation ± calcification (chronic)
• Ruptured cyst: Retroperitoneal or perinephric
collection of fluid, blood (varied density) or pus
• Neoplastic wall: Focal thickening or enhancing nodule
• Calcified cyst: Calcification, usually in thickened wall
• Hyperdense cyst: Homogeneous, hyperdense,
non enhancing mass
• Septated cyst: ;:: 1; partial or complete thin septa
• Multiple simple cysts: Bilateral> unilateral
• Localized cystic disease
o 1 portion of unilateral kidney; ill-defined
multiloculated mass and/or cluster of cysts
o No capsule; small cysts not within main cluster
• Milk of calcium cyst: Dependent, fluid-calcium layer
MR Findings
• Uncomplicated cyst
o TIWI: Round/oval, homogenous, hypointense mass
o T2WI: Homogeneous, hyperintense mass with
imperceptible wall; smooth & distinct inner margin
o CEMR: No enhancement
• Infected cyst: Tl WI:.t Intensity, less homogenous
than uncomplicated cyst; j intensity than subacute
hemorrhage (similar to chronic); ± thickened wall
• Hemorrhagic cyst
o Variable signal intensity due to age of hemorrhage
Pathology
• Most common renal mass in adults (62%)
Clinical Issues
• Asymptomatic or palpable mass and flank pain
• Renal cyst aspiration: Used only to culture infected
cyst and diagnose indeterminate mass by cytology
• Age: 50% > 50 years of age; rare in < 30 years of age
• Bosniak class I, II and IIF: No treatment ± follow-up
• Bosniak class III and IV: Surgical excision
Diagnostic Checklist
• Image findings generally more reliable than "clinical 3
correlation"
• Image evaluation and classification of cystic masses is 81
key to management
o TIWI: Highest intensity in subacute « 72 hours)
o T2WI: High intensity (less than uncomplicated cyst);
fluid-debris level (intensity less in dependent); ±
heterogeneous mass and lobulation of contour
• Neoplastic wall: Focal mass or wall thickening; fluid
simulates uncomplicated or hemorrhagic cyst
• Hyperdense cyst: t Protein simulates hemorrhage
Ultrasonographic Findings
• Real Time
o Uncomplicated cyst: Spherical or ovoid; sharply
defined distant wall with smooth, distinct margins;
no internal echoes; acoustical enhancement
o Infected cyst: Thick wall with scattered internal
echoes ± debris-fluid level
o Hemorrhagic cyst: Internal echoes (clot); thick
calcified wall ± multiloculated (chronic)
o Neoplastic wall: Tumor nodule
o Hyperdense cyst: Few scattered internal echoes
o Localized cystic disease: 1 large multiloculated mass
o Milk of calcium cyst: Line of calcified debris
Angiographic Findings
• Conventional
o Vascular displacement; hypo- or avascular
o Infected cyst: Neovascularity in granulation tissue
o Neoplastic wall: Focal blush of nodule
Imaging Recommendations
• Best imaging tool: CT (100% sensitivity); US (> 98%)
• Protocol advice: CT: Noncontrast and nephrographic
phase (90 seconds after contrast)
o Same day, scanner, CT technique and collimation
I DIFFERENTIAL DIAGNOSIS
Renal carcinoma
• Angiography: Neovascularity (papillary renal cell
carcinoma may be "avascular")
• Features to distinguish from calcified cyst
oCT: Hyperintense (> water), discrete enhancing mass
• Features to distinguish from localized cystic disease
o Capsule between cluster of cysts and parenchyma
 RENAL CYST
o No cysts other than within main cluster of cysts
Multilocular cystic nephroma (MCN)
• Septated, encapsulated cystic mass
• Herniation into renal sinus
Renal abscess
• "Rim" sign; ± perinephric stranding
• Shaggy wall; hyperdense (> water)
Hydronephrosis
• No distinct mass; dense "obstructive" nephrogram with
enlarged kidney & dilated collecting structures
Metastases and lymphoma
3 • Metastases (e.g., melanoma & lung, breast & GI
cancer)
82 oCT: Multifocal, small, Isodense, enhancing (5-30
HU) nodules; widespread nonrenal metastases
• Lymphoma (non-Hodgkin> Hodgkin)
oCT: Multiple (45%); invasion from retroperitoneal
nodes (25%); solitary (15%)
I PATHOLOGY
General Features
• General path comments
o Most common renal mass in adults (62%)
o Hemorrhagic cyst: 6% all cysts; calcified cyst: 1-3%
• Etiology
o Uncomplicated cyst: Unknown, ischemia, tubular
obstruction by solid tumor (sentinel cyst) or
medullary interstitial fibrosis
o Infected cyst: Hematogenous spread, vesicoureteric
reflux, surgery or cyst puncture
o Hemorrhagic cyst: Unknown, trauma, bleeding
diathesis or varicosities in simple cyst
o Ruptured cyst: Spontaneous
o Calcified cyst: Hemorrhage, infection or ischemia
o Hyperdense cyst: Hemorrhage or tumor
o Septated cyst: 2 adjacent cysts or healing and
organization of hemorrhagic or infected cyst
o Localized cystic disease: No family history
Gross Pathologic & Surgical Features
• Uncomplicated cyst
o Unilocular; arise in cortex (superficial) and bulge
from renal surface; less common from medulla
o Clear or straw-colored fluid; up to several liters
o Smooth, yellow-white, thin and translucent wall
o Rarely calcified; no communication to renal pelvis
• Infected cyst: Markedly thickened wall ± calcification;
varying pus, fluid and calcified or noncalcified debris
• Hemorrhagic cyst: Rust-colored putty-like material
surrounded by thick fibrosis and plates of calcification
• Neoplastic wall: Discrete nodule at base of cyst
• Hyperdense cyst: Rusty-colored material, i protein
content, i viscosity, solidified colloid
• Localized cystic disease: Nonencapsulated, numerous
simple cysts in 1 portion of kidney
• Milk of calcium cyst: Small calcified debris (calcium
carbonate) in cystic fluid
Microscopic Features
• Uncomplicated cyst
o Cystic wall: Cuboidal or flattened, discontinuous
epithelium; Fibrosis & hyalinization; ± calcification
o 1-2 mm thickness of wall; rare thin septa divide
cysts ± communicate with each other
o Adjacent tissue: Compression and fibrosis
• Hemorrhagic cyst: Uni- or multilocular, thickened wall
• Neoplastic wall: Well differentiated clear/granular cell
• Septated cyst: Compressed normal parenchyma or
nonneoplastic connective tissue
• Localized cystic disease: Dilatation of ducts & tubules
(mm to cm); no neoplastic or dysgenetic stroma
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic or palpable mass and flank pain
o Infected cyst: Pain in flank, malaise and fever
o Hemorrhagic cyst: Abrupt and severe pain
o Ruptured cyst: Severe abdominal pain, hematuria
• Lab data
o Cystic fluid: Similar to plasma transudate
• Renal cyst aspiration: Used only to culture infected
cyst and diagnose indeterminate mass by cytology
Demographics
• Age: 50% > 50 years of age; rare in < 30 years of age
• Gender: M > F
Natural History & Prognosis
• Complications: Hydronephrosis, hemorrhage,
infection, cyst rupture or carcinoma
• Prognosis: Very good
Treatment
• Bosniak class I, II and IIF: No treatment ± follow-up
• Bosniak class III and IV: Surgical excision
• Follow-up: Changes size, configuration & internal
consistency; excision if changes suggest carcinoma
I DIAGNOSTIC CHECKLIST
Consider
• Image findings generally more reliable than "clinical
correlation"
Image Interpretation Pearls
• Image evaluation and classification of cystic masses is
key to management
I SELECTED REFERENCES
1. BosniakMA:Diagnosisand management of patients with
complicated cysticlesions of the kidney.AJR.169: 819,
1997
2. DavidsonAJet al: Radiologicassessmentof renal masses:
Implication for patient care. Radiology.202: 297, 1997
3. SiegelCLet al: CTof cysticrenal masses:Analysisof
diagnostic performance and interobservervariation. AJR.
169: 813, 1997
 RENAL CYST

I IMAGE GALLERY
Typical
(Left) Axial NECT and CECT
show a water density mass
with no enhancement and
no definable wall; Bosniak I
cyst. (Right) Sagittal
sonogram shows anechoic
mass in upper pole with
posterior acoustic
enhancement.

3
83

Typical
(Left) Axial CECT shows
multiple water density cystic
lesions, including calcified
thin septum (arrow); Bosniak
/I cyst. (Right) Axial CECT
shows multiple cortical and
parapelvic cysts. Thin septal
calcification indicates benign
Bosniak /I classification.

(Left) Axial CECT shows

heterogeneous
nonenhancing mass and
perirenal stranding.
Hemorrhagic cyst. (Right)
Axial CECT shows water
density mass with relatively
thick, calcified wall; was
resected and was a benign
cyst.
 RENAL ONCOCYTOMA

3
84

Coronal T7WI MR (enhanced) shows well-defined Surgical photograph shows resected oncocytoma with a
spherical renal mass (arrow) with a central scar (open central scar and thin septa within a homogeneous solid
arrow) and a pseudocapsule characteristic of an mass,
oncocytoma,

o Tumors composed of oncocytes occur in thyroid,

ITERMINOlOGY parathyroid, adrenal & salivary glands
Abbreviations and Synonyms o In less than 10% of cases, oncocytoma &
chromophobe renal cell carcinoma (RCC) may
• Synonyms
o Proximal tubular adenoma with oncocytic features coexist
o Benign oxyphilic adenoma Radiographic Findings
Definitions • Radiography
• Epithelial tumor composed of eosinophilic epithelial o Plain radiographic findings are nonspecific
cells, arising from intercalated cells of collecting ducts • Large, soft tissue mass in renal area with
displacement of fat planes
• Calcification is rare
I IMAGING FINDINGS • IVP: Large mass with a renal contour abnormality
compression of collecting system
&

General Features
CT Findings
• Best diagnostic clue: Solid renal cortical mass lesion
with central stellate scar • NECT
o Well-defined, homogeneous, solid density mass
• Location: Usually renal cortex
o Isodense or slightly hyperdense relative to kidney
• Size: Varies from 3 to 10 cm (average of 7 cm)
o Central hypoattenuating area suggestive of scar
• Morphology: Encapsulated, well-marginated, smooth
• Seen in 33-54% of cases
contour, solid mass
• Difficult to differentiate from central necrosis of
• Other general features
renal cell carcinoma
o 2nd Most common benign renal tumor after AML
o Calcification, necrosis & hemorrhage are rare
o Accounts for approximately 3-7% of all renal
o Occasionally multifocal & bilateral
neoplasms
o About 2-12% are multifocal & 4-14% are bilateral • CECT
o Nephrographic phase
o 50-90% of tumors are incidental on imaging
• Tumor appears less dense than renal parenchyma
o 17-21% of cases present with clinical manifestations

DDx: Expansile Renal Mass

'.
'..
;:;
-~
" 0.

~ -
Renal Cell Carcinoma Renal Cell Carcinoma Lymphoma Angiomyolipoma
 RENAL ONCOCYTOMA

Key Facts
Terminology Top Differential Diagnoses
• Proximal tubular adenoma with oncocytic features • Renal cell carcinoma (RCe)
• Benign oxyphilic adenoma • Renal angiomyolipoma (AML)
• Epithelial tumor composed of eosinophilic epithelial • Renal metastases & lymphoma
cells, arising from intercalated cells of collecting ducts
Pathology
Imaging Findings • Deep-brown or mahogany color in contrast to
• Best diagnostic clue: Solid renal cortical mass lesion yellowish-orange RCC
with central stellate scar • Fleshy central scar: Characteristic of oncocytoma
• Calcification, necrosis & hemorrhage are rare
• Tumor: Homogeneous enhancement Diagnostic Checklist
• Not possible to distinguish from RCC on imaging
• Central stellate scar: Nonenhancing
• "Spoke-wheel" pattern of tumor vascularity • May suggest diagnosis ~ nephron-sparing surgery 3
• Absence of bizarre neoplastic vessels • Pathologic diagnosis requires entire tumor due to
oncocytic features of RCC 85
• No AV shunting, vascular puddling, renal vein
invasion • Well-defined, solid cortical mass lesion with central
scar & "spoke-wheel" pattern of vessels on angiogram

• Central scar with radiating low-density areas (seen

in large tumors) Nuclear Medicine Findings
• ± Displacement of renal collecting system • FDG-PET
• Absence of malignant features: Invasion or o Less FDG uptake than RCCs
infiltration into perinephric fat, collecting system o Amount of uptake, usually isointense to renal tissue
or vessels o Occasionally have uptake in the range of RCC
• No regional lymphadenopathy & metastases • Tc99m dimercaptosuccinic acid (DMSA) scans
o Photopenic area displacing cortex & collecting
MR Findings system
• TlWI
o Well-defined, homogeneous mass Imaging Recommendations
o Iso-/hypointense relative to renal cortex • Helical NE + CECT; MR
o Internal radiating architecture may be seen
• T2WI
o Slightly hyperintense relative to renal cortex I DIFFERENTIAL DIAGNOSIS
o "Spoke-wheel" pattern
Renal cell carcinoma (RCC)
• Internal radiating low signal areas
• Most common primary renal malignant neoplasm
• Rarely may appear bright on T2WI
o Tumor necrosis: Common feature of malignant mass • NECT
o Solid mass lesion of density, 30-50 HU range
• Hypointense on Tl WI
o Necrotic, calcific, hemorrhagic, cystic components
• Hyperintense on T2WI
often seen in RCC, rare or absent in oncocytoma
• Tl C+
o Tumor: Homogeneous enhancement • Central necrosis of RCC may mimic central scar of
o Central stellate scar: Nonenhancing oncocytoma
o Malignant features of an invasive tumor are absent • CECT
o Highly enhancing mass with thick septa &
Ultrasonographic Findings nodularity
• Real Time o ± Invasion or infiltration into perinephric fat,
o Well-defined, homogeneous, hypo-/isoechoic mass collecting system
o Central scar: Usually echogenic o ± Tumor extension into renal vein & IVC
• Color Doppler: Tumor with central radiating vessels o ± Regional lymphadenopathy & hypervascular
metastases
Angiographic Findings • Bilateral RCC
• Conventional o von Hippel-Lindau disease, tuberous sclerosis
o "Spoke-wheel" pattern of tumor vascularity • Cut section: Yellowish-orange appearance
o Homogeneous dense tumoral contrast during • Histology: Granular eosinophilic cytoplasm with
capillary phase perinuclear clearing
o Sharp demarcation from kidney and surrounding • Immunohistochemical tests: Vimentin positivity
areas
o Peritumoral halo (lucent-rim sign) Renal angiomyolipoma (AML)
o In contrast to RCC • Tumor composed of abnormal blood vessels, smooth
• Absence of bizarre neoplastic vessels muscle & fatty components
• No AV shunting, vascular puddling, renal vein • NECT
invasion
 RENAL ONCOCYTOMA
o Well-defined, cortical heterogeneous tumor, • Lab-data
predominantly of fat density (-30 to -100 HU) o Immunohistochemical tests
• Renal mass with fat is almost diagnostic of AML • Oncocytoma: Cathepsin H positivity
o Calcification rarely seen (if present, suspect RCC) • Chromophobe RCC: Vim en tin positivity
o Hemorrhage may be seen in large tumors> 4 cm • Diagnosis
o When multiple AML seen, suspect tuberous sclerosis o Percutaneous needle biopsy unreliable
• CECT o Whole tumor resection & histopathology
o Varied enhancement based on amount of fat &
vascular components Demographics
• CTA: Aneurysmal renal vessels may be seen • Age
o Usually seen in older age group
• MR
o Tumor with increased fat content o Peak incidence occurring in sixth & seventh decades
• T1WI: Hyperintense • Gender: M:F = 2-3:1

3 • Fat-suppression sequences: Show signal "drop-out" Natural History & Prognosis

• Oncocytomas are benign tumors
Renal metastases & lymphoma
86 • Renal metastases • Complications (rare)
o Multiple renal masses of low density o Hematuria
o When seen as a solitary dense mass, may mimic o Obstructive nephropathy (extrinsic compression of
oncocytoma renal pelvis)
o Usually known primary tumor & metastases in other • Prognosis
sites strongly suggests renal metastases o Excellent after partial or total nephrectomy
• Renal lymphoma Treatment
o Primary very rare; secondary from generalized
• Surgical resection: Partial or total nephrectomy
spread (more common)
o Bilateral involvement seen in 40-60% of cases
o Non-Hodgkin lymphoma> common than Hodgkin
o Hypovascular; moderate enhancement with contrast
I DIAGNOSTIC CHECKLIST
Consider
• Not possible to distinguish from RCC on imaging
I PATHOLOGY • May suggest diagnosis ~ nephron-sparing surgery
General Features • Pathologic diagnosis requires entire tumor due to
oncocytic features of RCC
• Genetics
o Deletion of chromosome 1 & sex chromosomes Image Interpretation Pearls
o Balanced translocation involving llq13 • Well-defined, solid cortical mass lesion with central
• Etiology: Exact etiology unknown scar & "spoke-wheel" pattern of vessels on angiogram
• Epidemiology: Usually incidental on imaging or
autopsy
• Associated abnormalities: Oncocytoma & RCC may I SELECTED REFERENCES
coexist
1. Landau A et al: Adrenocortical oncocytoma: benign or
Gross Pathologic & Surgical Features malignant? ANZ] Surg. 74(5):390, 2004
2. Bandhu S et al: Spoke-wheel pattern in renal oncocytoma
• Large & spherical tumor with a pseudo capsule seen on double-phase helical CT. Australas Radiol.
• Cut section 47(3):298-301, 2003
o Deep-brown or mahogany color in contrast to 3. Katz DS et al: Renal oncocytomatosis. Am J Kidney Dis.
yellowish-orange RCC 27(4):579-82, 1996
o Fleshy central scar: Characteristic of oncocytoma 4. Licht MR: Renal adenoma and oncocytoma. Semin Urol
o Rarely necrosis, hemorrhage, calcification Oncol. 13(4):262-6, 1995
5. Davidson A] et al: Renal oncocytoma and carcinoma:
Microscopic Features failure of differentiation with CT. Radiology. 186(3):693-6,
• Composed of oncocytes 1993
o Large cells with granular eosinophilic cytoplasm 6. Remark RR et al: Magnetic resonance imaging of renal
oncocytoma. Urology. 31(2):176-9, 1988
o Cells arranged in sheets, tubulocystic or combined
7. Curry NS et al: Small renal neoplasms: diagnostic imaging,
o Nuclei appear smooth, round, with minimal atypia pathologic features, and clinical course. Radiology.
• Electron microscopy: Cells packed with mitochondria 158(1):113-7, 1986
8. Levine E: Small renal neoplasms: diagnostic imaging,
pathologic features, and clinical course. Radiology.
I CLINICAL ISSUES 159(3):817-8, 1986
9. Levine E et al: Computed tomography of renal
Presentation oncocytoma. A]R Am] Roentgenol. 141(4):741-6, 1983
• Most common signs/symptoms
o Mostly asymptomatic
o Occasionally flank pain, hematuria
o Hypertension or palpable mass on physical exam
 RENAL ONCOCYTOMA
I IMAGE GALLERY

(Left) Axial NECT shows

spherical mass that is slightly
hyperdense to the kidney.
(Right) Axial CECT shows
only slight enhancement of
the mass and a small central
scar.

3
87

(Left) Coronal TlWI MR

(nonenhanced) shows an
encapsulated spherical mass
that is slightly hyperintense
to kidney. (Right) Coronal
TlWI MR (enhanced) shows
slight enhancement of the
mass and a small central
scar.

Variant
(Left) Axial NECT shows an
exophytic spherical mass that
is moderately heterogeneous
with hyperdense focus.
(Right) Axial CECT shows
slight enhancement of the
mass, including the
hyperdense focus, indicating
that this does not represent
hemorrhage.
 RENAL ANGIOMYOLIPOMA

3
88

Graphic shows vascular renal mass with fatty and soft Axial CECT shows spherical renal mass with fat
tissue components. component and prominent vessels.

o Most common benign tumor of kidney

ITERMINOlOGY o 80% of cases are incidental findings on imaging
Abbreviations and Synonyms o Usually solitary & unilateral; occasionally multiple
& bilateral
• Angiomyolipoma (AML)
o 90% unilateral: Usually not associated with tuberous
• Synonyms: Renal hamartoma or choristoma
sclerosis
Definitions o 10% multiple & bilateral: Usually due to tuberous
• Benign renal tumor composed of abnormal blood sclerosis
vessels, smooth muscle & fatty components o 20% of renal AML cases have tuberous sclerosis
o 80% of tuberous sclerosis patients have renal AMLs
o Tuberous sclerosis (Bourneville disease)
IIMAGING FINDINGS • Facial angiofibroma, ungual fibroma
• Retinal hamartoma
General Features • Cortical tubers & subependymal nodules
• Best diagnostic clue: Intrarenal fatty mass • Multiple renal angiomyolipomas
• Location: Intrarenal (cortex) or exophytic in location o AML occurs in 15% of patients with
• Size lymphangiomyomatosis
o Varies in size o Lymphangiomyomatosis
• May range from few mm to 25 cm or more • Hamartomatous proliferation of smooth muscle
• Morphology along lymphatic system, most commonly in lung
o Lobulated mass lesion of varied size + number • Exclusively affects women
o Usually discrete, rarely diffuse parenchymal mass
o Tumor components
Radiographic Findings
• Abnormal blood vessels • Radiography
• Smooth muscle o Tomographic sections: Radiolucent mass
• Adipose tissue • When lesion contains large amount of fat
o One or two of the components may predominate • IVP
• Other general features o Small tumor: Well defined radiolucent defect
o Large tumor: Distortion of collecting system

DDx: Renal Mass

'~'::...
~ .I ' ~
...,-
..•

Renal Carcinoma RCC with Bleed Renal Lymphoma Liposarcoma

 RENAL ANGIOMYOLIPOMA
Key Facts
Terminology
• Benign renal tumor composed of abnormal blood
vessels, smooth muscle & fatty components
Imaging Findings
• Best diagnostic clue: Intrarenal fatty mass
• May range from few mm to 2S cm or more
• Well-marginated cortical heterogeneous tumor,
predominantly of fat density (-30 to -100 HU)
• When multiple AML seen, suspect tuberous sclerosis
• - S% have no detectable fat on CT, such AML cannot
be diagnosed by CT or other imaging modalities
• Hemorrhage may be seen in large tumors> 4 cm
• Calcification rarely seen; if present suspect RCC
• RCC may "de-d.ifferentiate", form bone + fat
• CTA: Aneurysmal renal vessels may be seen
o Multiple lucent defects when associated with
tuberous sclerosis
CT Findings
• NECT
o Well-marginated cortical heterogeneous tumor,
predominantly of fat density (-30 to -100 HU)
o Renal mass with fat is almost diagnostic of AML
• Variable amounts of fat present
o When multiple AML seen, suspect tuberous sclerosis
o - S% have no detectable fat on CT, such AML
cannot be diagnosed by CT or other imaging
modalities
o Hemorrhage may be seen in large tumors> 4 cm
o Calcification rarely seen; if present suspect RCC
• RCC may "de-differentiate", form bone + fat
• CECT
o Varied enhancement pattern based on amount of fat
& vascular components
o AML is not known to undergo malignant change,
but benign satellite deposits may be seen in regional
lymph nodes, liver & spleen
o Rarely extension of tumor into IVC seen
• CTA: Aneurysmal renal vessels may be seen
MR Findings
• Varied signal intensity due to vessels, muscle & fat
• Tumor with increased fat content
o T1WI: Hyperintense
o Fat suppression sequences: Signal loss
• T1 C+
o Tumor with increased fat content
• Show minimal enhancement
o Tumor with high vascular component
• Show significant enhancement
Ultrasonographic Findings
• Real Time
o Markedly hyperechoic mass relative to normal renal
tissue .
o May look similar to small renal cell carcinoma
which may also be hyperechoic
Top Differential Diagnoses
• Renal cell carcinoma (RCe)
• Renal metastases & lymphoma
• Renal oncocytoma
• Perirenal liposarcoma
Pathology
• Benign mesenchymal tumor of kidney
• 80% isolated (sporadic) AML
• 20% AML associated with tuberous sclerosis
Diagnostic Checklist
• Use CT pixel analysis to detect small foci of fat on 3
NECT
• Calcification or ossification within tumor highly 89
suggestive of renal cell carcinoma, not AML
Angiographic Findings
• Tumor with increased vascular component
o Highly vascular mass lesion
o Multisacculated pseudoaneurysms
o Absence of arteriovenous shunts
o "Sunburst" appearance of capillary nephrogram
o "Onion peel" appearance of peripheral vessels in
venous phase
Imaging Recommendations
• Best imaging approach is thin-section (:s: 3 mm) NECT
and CECT
I DIFFERENTIAL DIAGNOSIS
Renal cell carcinoma (RCC)
• Rarely reported to contain fat (engulfed renal sinus fat)
• If AML is fat deficient, may mimic renal cell carcinoma
• Calcification or ossification within tumor highly
suggestive of RCC (calcification very rare in AML)
Renal metastases & lymphoma
• Renal metastases
o Multiple renal masses of low density easily
differentiated from multiple fatty AML
o Usually known primary tumor & metastases in other
sites strongly suggests renal metastases
o Occasionally, present as a large solitary mass, but
devoid of fat
• Renal lymphoma
o Primary very rare; secondary from generalized
spread (more common)
o Bilateral involvement is seen in 40-60% of cases
o Tuberous sclerosis patients with bilateral AML can
simulate renal lymphoma if AML lesions lack fat
o Non-Hodgkin lymphoma> common than Hodgkin
o Hypovascular; moderate enhancement with contrast
Renal oncocytoma
• Rare benign renal tumor; rarely contains fat
• Proximal tubular adenoma with oncocytic features
• Well-defined mass of homogeneous density
 RENAL ANGIOMYOLIPOMA
Perirenal liposarcoma
• Large exophytic AML may simulate retroperitoneal
liposarcoma (both contain fat)
o Renal parenchymal defect & enlarged vessels favor
angiomyolipoma (AML)
o Smooth compression of kidney & extension beyond
perirenal space favor liposarcoma
• Very rarely liposarcoma originate within kidney
o Typically subcapsular in location, lenticular in shape
• Diagnosis: Require entire tumoral resection
Wilms tumor
• Pediatric renal tumor that may contain fat
3 • Age difference at presentation avoids confusion
90
I PATHOLOGY
General Features
• Etiology
o Benign mesenchymal tumor of kidney
o Hamartoma: Benign tumor consisting of tissues that
normally occur in organ of origin
o Choristoma: Benign tumor composed of tissues not
normally occurring within organ of origin
• Epidemiology
o 0.3-3% in autopsy series
o 80% isolated (sporadic) AML
o 20% AML associated with tuberous sclerosis
• Associated abnormalities: Tuberous sclerosisi
lymphangiomyomatosis
Gross Pathologic & Surgical Features
• Round, lobulated, yellow-to-gray color secondary to
fat content
Microscopic Features
• Variable amounts of angioid (vascular), myoid
(smooth muscle), & lipoid (fatty) components
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Often asymptomatic, incidental CT finding
o Hematuria, flank pain or palpable flank mass
o Acute abdomen (spontaneous hemorrhage, rupture)
o Occasionally, hypertension & chronic renal failure
o Detected while screening tuberous sclerosis patients
for renal cell carcinoma
o Tuberous sclerosis
• Mental retardation, epilepsy
• Sebaceous adenomas of face
• Diagnosis: Imaging & biopsy
Demographics
• Age: Usually beyond 40 years old age group
• Gender
o More common in females than males (M:F = 1:4)
o AML associated with tuberous sclerosis (M:F = 1:1)
Natural History & Prognosis
• Complications
o No malignant potential
o Hemorrhage & rupture
• Prognosis
o AML usually a slowly growing tumor
o Usually good: After partial or complete nephrectomy
o Poor: With hemorrhage, rupture, no treatment
Treatment
• Debate exists as to how to manage asymptomatic
patients with AML
• Tumor size less than 4 cm
o Conservative management with follow-up
• Tumor size more than 4 cm
o Partial nephrectomy often recommended
• Those patients presenting with spontaneous bleeding
treated with embolization initially
o Surgery postponed until patient stabilized
I DIAGNOSTIC CHECKLIST
Consider
• Use CT pixel analysis to detect small foci of fat on
NECT
Image Interpretation Pearls
• Well-marginated, discrete fatty renal mass
• Calcification or ossification within tumor highly
suggestive of renal cell carcinoma, not AML
I SELECTED REFERENCES
1. Kim JK et al: Angiomyolipoma with minimal fat:
differentiation from renal cell carcinoma at biphasic helical
CT. Radiology. 230(3):677-84, 2004
2. Israel GM et al: CT differentiation of large exophytic renal
angiomyolipomas and perirenal liposarcomas. AJRAm J
Roentgenol. 179(3):769-73,2002
3. Wilson SS et al: Angiomyolipoma with vena caval
extension. Urology. 60(4):695-6, 2002
4. Yamakado K et al: Renal angiomyolipoma: relationships
between tumor size, aneurysm formation, and rupture.
Radiology. 225(1):78-82, 2002
5. Lemaitre L et al: Imaging of angiomyolipomas. Semin
Ultrasound CT MR. 18(2):100-14, 1997
6. Katz DS et al: Massive renal angiomyolipoma in tuberous
sclerosis. Clin Imaging. 21(3):200-2, 1997
7. Siegel CL et al: Angiomyolipoma and renal cell carcinoma:
US differentiation. Radiology. 198(3):789-93, 1996
8. Curry NS: Small renal masses (lesions smaller than 3 cm):
imaging evaluation and management. AJRAm J
Roentgenol. 164(2):355-62, 1995
9. Curry NS et al: Intratumoral fat in a renal oncocytoma
mimicking angiomyolipoma. AJR Am J Roentgenol.
154(2):307-8, 1990
10. Bosniak MA et al: CT diagnosis of renal angiomyolipoma:
the importance of detecting small amounts of fat. AJRAm J
Roentgenol. 151(3):497-501, 1988
 RENAL ANGIOMYOLIPOMA

I IMAGE GALLERY

Typical
(Left) Axial NECT and CECT
show subtle foci of fat in
multiple AMLs in right
kidney. Simple cyst in left
kidney (arrows). (Right)
Sagittal sonogram shows
hyperechoic mass (arrow) in
upper pole right kidney.

3
91

Typical
(Left) Axial CECT shows
large heterogeneous
fat-containing mass in right
kidney. (Right) Axial CECT
shows spontaneous perirenal
bleed from left renal AML
(arrows).

Variant
(Left) Axial NECT shows
spontaneous bleed from right
renal mass (AML) without an
obvious fat component.
(Right) Axial CECT shows
bilateral enhancing renal
masses (arrows) in a patient
with tuberous sclerosis;
AMLs without obvious fat
component.
 MULTILOCULAR CYSTIC NEPHROMA

3
92

Graphic shows multilocular cystic mass that herniates Axial CECT shows a multiloculated cystic mass with
into the renal hilum. enhancing septa.

• Morphology: Solitary, unilateral, well-circumscribed

ITERMINOLOGY cystic mass with a thick fibrous capsule ± herniation
Abbreviations and Synonyms into renal pelvis
• Multilocular cystic nephroma (MLCN) • Other general features
o Usually solitary (rarely multiple) & unilateral
• Synonym(s)
o Multilocular cystic renal tumor; benign cystic o Usually symptomatic in adults & asymptomatic
nephroma; cyst adenoma palpable mass in children
o Cystic nephroblastoma; cystic partially o Tumor may grow slowly over years or rapidly within
differentiated nephroblastoma (CPDN) months
o Histological classification of multilocular cystic renal
Definitions tumors
• Rare nonhereditary benign renal neoplasm arising • Multilocular cystic nephroma
from metanephric blastema • Cystic partially differentiated nephroblastoma
o MLCN & CPDN indistinguishable by imaging alone
Radiographic Findings
I IMAGING FINDINGS
• Radiography
General Features o Plain x-ray abdomen
• Best diagnostic clue: Multilocular cystic mass • Soft tissue mass (particularly if large & displaces
herniating into renal hilum on CECT adjacent structures)
• Location • ± Curvilinear or amorphous calcification
o Typically unilateral (usually lower pole) • IVP
o Occasionally mass completely replaces kidney o Depending on size & location of mass
• Size • ± Obstructed collecting system/pyelocaliectasis
o Mass: Few cm to > 30 cm (average size 10 cm) • ± Tumor herniates/protrudes into renal pelvis
o Locules: Few millimeters to 2.5 cm (mimicking a primary tumor of collecting system)
o Tomogram of nephrographic phase show septations

DDx: Unilateral Multicystic Kidney

Renal Cell Carcinoma Renal Cell Carcinoma Renal Cell Carcinoma Segmental MCOK
 MULTILOCULAR CYSTIC NEPHROMA
Key Facts
Terminology
• Multilocular cystic nephroma (MLCN)
• Rare nonhereditary benign renal neoplasm arising
from metanephric blastema
Imaging Findings
• Best diagnostic clue: Multilocular cystic mass
herniating into renal hilum on CECT
• Typically unilateral (usually lower pole)
• CT density: Equal to water or higher than water
(proteinaceous fluid)
• Capsule: ± Enhancement
• Septa: Moderate enhancement (thin & linear)
• Cystic component: No enhancement
• No communication with collecting system
• Varied signal intensity (protein or blood product)
CT Findings
• NECT
o Large, well-defined, encapsulated, multiloculated
cystic mass
o CT density: Equal to water or higher than water
(proteinaceous fluid)
o Smalliocuies « 1 cm) & myxomatous material
within cysts + fibrous septa may appear as solid mass
o ± Curvilinear or amorphous calcification
• CECT
o Capsule: ± Enhancement
o Septa: Moderate enhancement (thin & linear)
o Cystic component: No enhancement
• No communication with collecting system
o ± Distortion of collecting system or herniation of
mass into renal hilum + obstructive changes
MR Findings
• TlWI
o Multiloculated hypointense mass (clear fluid)
o Varied signal intensity (protein or blood product)
• T2WI
o Multiloculated hyperintense mass (clear fluid)
o Varied signal intensity (protein or blood product)
o Capsule & septa: Hypointense (fibrous content)
• Tl C+
o Enhancement of thin septa
o Herniation of mass into renal pelvis well depicted
Ultrasonographic Findings
• Real Time
o Large, well-defined multiloculated cystic mass
o Innumerable anechoic cysts + hyperechoic septa
o Thick hyperechoic fibrous capsule
o ± Hyperechoic areas within cystic tumor mimicking
a solid component (due to numerous tiny cysts
causing acoustic interfaces)
Angiographic Findings
• Conventional: Hypovascular mass (rarely avascular or
hypervascular)
Top Differential Diagnoses
• Multilocular cystic renal cell carcinoma
• Cystic Wilms tumor
• Multicystic dysplastic kidney (MCDK)
• Cortical (simple) cysts
Clinical Issues
• Boys between 3 months & 2 years old (90% tumors)
• Females: < 5 yrs & peak in 5th & 6th decades
Diagnostic Checklist
• MLCN appears as a Bosniak class III or IV complex
cystic mass (surgical excision is treatment of choice) 3
• Difficult to distinguish from multilocular cystic RCC
• MLCN: Unilateral, solitary, multiloculated, cystic 93
mass of near water HU ± herniation into renal pelvis
Nuclear Medicine Findings
• Technetium-99m diethylenetriaminepentaacetic acid
o Photon defect corresponding to renal mass
o Occasionally faint uptake by septa noted
Imaging Recommendations
• Helical NE + CECTi MR + Tl C+i US
I DIFFERENTIAL DIAGNOSIS
Multilocular cystic renal cell carcinoma
• On imaging it mimics multilocular cystic nephroma
• MLCN may herniate into renal pelvis causing filling
defect, obstructive uropathy & hematuria which is not
seen in multilocular cystic RCC
• Occasionally focal areas of solid component may be
seen to differentiate from MLCN
• Usually seen in adult men more than women
o MLCN seen in boys & adult women
• Gross pathology: Simulates MLCN
• Diagnosis: Histology (clear cell type, I nuclear grade)
Cystic Wilms tumor
• Grows entirely by expansion of large cystic spaces
within stroma
• Septa: Numerous/thick where as thin/linear in MLCN
Multicystic dysplastic kidney (MCDK)
• Usually involves entire kidney
• Rarely focal or segmental
• Segmental MCDK: Mostly associated with ureteral
duplication
• Segmental MCDK + absence of ureteral duplication
may simulate multilocular cystic tumor
o Both are histologically distinctive
o Segmental MCDK have no capsule where as MLCN
have a capsule
Cortical (simple) cysts
• Solitary or multiplei unilateral or bilateral
• Most cysts are unilocular & arise in cortexi less
commonly from medulla
 MULTILOCULAR CYSTIC NEPHROMA
• Sharply marginated, round & smooth in contour
• Thin imperceptible wall & no septations
• Low density masses of near water HU (-10 to + 20)
• Unilateral, solitary, multiloculated cyst with thin septa
may simulate MLCN
I PATHOLOGY
General Features
• General path comments
o Embryology/anatomy
• Tumor arises from metanephric blastema
• Etiology
3 o Unknown
o Pathogenesis: Benign equivalents of
94 nephroblastoma, hamartoma
• Epidemiology: Rare tumor
• Associated abnormalities
o Sporadic association with other anomalies
o Coexist with angiomyolipoma; adrenal adenoma
Gross Pathologic & Surgical Features
• "Honeycombed" cystic areas of varied sizes
• Encapsulated by a thick fibrous capsule
• Noncommunicating locules separated by thin septa
o CPDN locules separated by thick or irregular septa
• Mostly intraparenchymal tumors
Microscopic Features
• Locules: Lined by flattened or cuboidal epithelium
• Septa contains fibrous tissue + tubular elements
• Between cysts, loose stroma with spindle-shaped
fibroblasts & cells resembling smooth muscle cells
• Tumor extended into renal pelvis may show foci of
necrosis & hemorrhage
• No capsular, lymphatic or vascular invasion
• No mitotic figures
• MLCN: Septa have no undifferentiated elements
• Cystic partially differentiated nephroblastoma (CPDN)
o Septa contain blastemal ± other embryonal elements
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Adults
• Abdominal or flank pain; ± palpable mass (adults)
• Hematuria; urinary tract infection (UTI); HTN
o Children
• Nonpainful; palpable abdominal or flank mass
• ± Hematuria & urinary tract infection (UTI)
• Lab data
o Urine analysis may show RBC ± WBC
• Diagnosis: Biopsy & histology
Demographics
• Age
o Biphasic age distribution
o Boys between 3 months & 2 years old (90% tumors)
o Females: < 5 yrs & peak in 5th & 6th decades
• Gender
o Biphasic sex distribution
o Predominantly boys (2/3rd) in childhood & women
(l/3rd) in adulthood
Natural History & Prognosis
• Complications
o Local recurrence; obstructive uropathy; infection
o Hemorrhagic when extended into renal pelvis
o Very rarely malignant transformation
• Prognosis
o Good: After nephrectomy
o Few cases: Local recurrence
Treatment
• Surgical excision: Nephrectomy
o Complete or partial with tumor-free margins
I DIAGNOSTIC CHECKLIST
Consider
• MLCN appears as a Bosniak class III or IV complex
cystic mass (surgical excision is treatment of choice)
• Difficult to distinguish from multilocular cystic RCC
Image Interpretation Pearls
• MLCN: Unilateral, solitary, multiloculated, cystic mass
of near water HU ± herniation into renal pelvis
I SELECTED REFERENCES
1. Hopkins JK et al: Best cases from the AFIP: cystic
nephroma. Radiographics. 24(2):589-93, 2004
2. Fujimoto K et al: Spontaneously ruptured multilocular
cystic nephroma. Int J Urol. 9(3): 183-6, 2002
3. Kural AR et al: Multilocular cystic nephroma: an unusual
localization. Urology. 52(5):897-9, 1998
4. Eble IN et al: Extensively cystic renal neoplasms: cystic
nephroma, cystic partially differentiated nephroblastoma,
multilocular cystic renal cell carcinoma, and cystic
hamartoma of renal pelvis. Semin Diagn Pathol.
15(1):2-20, 1998
5. Levine E et al: Current concepts and controversies in
imaging of renal cystic diseases. Urol Clin North Am.
24(3):523-43, 1997
6. Kettritz U et al: Multilocular cystic nephroma: MR imaging
appearance with current techniques, including gadolinium
enhancement. J Magn Reson Imaging. 6(1):145-8, 1996
7. Agrons GA et al: Multilocular cystic renal tumor in
children: radiologic-pathologic correlation. Radiographics.
15(3):653-69, 1995
8. Yamashita Y et al: Multilocular cystic renal cell carcinoma
presenting as a solid mass: radiologic evaluation. Abdom
Imaging. 20(2):164-8, 1995
9. Castillo OA et al: Multilocular cysts of kidney. A study of
29 patients and review of literature. Urology. 37(2):156-62,
1991
10. Joshi VV et al: Multilocular cyst of the kidney (cystic
nephroma) and cystic, partially differentiated
nephroblastoma. Terminology and criteria for diagnosis.
Cancer. 64(2):466-79, 1989
11. Madewell JE et al: Multilocular cystic nephroma: a
radiographic-pathologic correlation of 58 patients.
Radiology. 146(2):309-21, 1983
 MULTILOCULAR CYSTIC NEPHROMA
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
mu/tiloculated cystic mass
with calcification of many of
the septa. (Right) Axial CECT
shows mu/ticystic mass with
calcified septa, herniating
into the renal hilum.

3
95

(Left) Axial CECT shows

mu/tiloculated cystic mass
with enhancing septa,
herniating into the renal
hilum. (Right) Surgical
photograph of cross section
of excised kidney shows
mu/tiloculated cystic mass
extending into renal hilum.

Typical
(Left) Axial CECT shows
mu/tiloculated cystic mass
with enhancing septa.
(Right) Axial CECT shows
mu/tiloculated cystic mass
with enhancing septa.
 RENAL CELL CARCINOMA

3
96

Graphic shows heterogeneous, vascular, expansile mass Coronal T7WI MR (contrast enhanced) shows expansile
arising from renal cortex, invading renal vein and IVC. mass in upper pole of left kidney extending into renal
vein and IVC (arrows).

o Heterogenous mass (hemorrhage and necrosis); high

ITERMINOlOGY (acute hemorrhage) or low attenuation (chronic)
Abbreviations and Synonyms o Alteration of renal contour
• Renal cell carcinoma (RCe) o ± Calcifications (10% of cases); amorphous internal
• Hypernephroma, renal carcinoma (most common), curvilinear, (peripheral or central),
dense or diffuse calcification
Definitions o Small areas of fat attenuation (-80 to -120 HU) (rare)
• Malignant renal tumor arises from tubular epithelium o Combination of fat and calcification suggests RCC,
not renal angiomyolipoma
o Cystic RCC
I IMAGING FINDINGS • Uni- or multilocular cystic mass with a thick wall
• Calcification of septa or tumor capsule
General Features • CECT
• Best diagnostic clue: Hypervascular cortical renal mass o Enhancement (attenuation value t ~ 20 HU) of solid
• Location mass, usually less than normal renal tissue
o 2% of sporadic RCC are bilateral and 16-25% of o Small (~ 3 cm), hypervascular mass better seen on
sporadic RCC are multicentric in the same kidney nephrographic phase than arterial or
o Renal cortex (most common) corticomedullary phase
• Morphology: Usually solid mass; occasionally cystic o Heterogenous enhancement (hemorrhage and
• Other general features necrosis)
o 25-40% found incidentally on abdominal CT or US o Rounded, lobulated margin demarcating from
adjacent renal tissue
CT Findings o Lucent zone surrounding the mass (pseudocapsule)
• NECT o ± Infiltration of calyces or renal pelvis; may simulate
o Hyperdense, isodense or hypodense mass as transitional cell carcinoma
compared with normal renal tissue o Subcapsular hemorrhage, perinephric hemorrhage or
o High density rim may separate mass from adjacent both; hyperdense fluid collection (hematoma)
renal tissue (pseudocapsule) o Exophytic growth pattern

DDx: Renal Mass

Oncocytoma Angiomyolipoma Lymphoma Pyelonephritis

 RENAL CELL CARCINOMA
Key Facts
Imaging Findings
• Best diagnostic clue: Hypervascular cortical renal
mass
• Morphology: Usually solid mass; occasionally cystic
• Rounded, lobulated margin demarcating from
adjacent renal tissue
• Lucent zone surrounding the mass (pseudo capsule)
• Metastases (most to least common) in lung, liver,
bone, adrenal, opposite kidney and brain; often
hypervascular
• Mandatory: Nonenhanced and parenchymal phase (2'::
80 sec delay); optional corticomedullary (60 sec),
excretory (2-5 min delay)
Top Differential Diagnoses
• Renal oncocytoma
o Renal venous (23%) and inferior vena cava (lYC)
tumor extension (7%)
o Direct extension to adjacent muscles (Le.,
diaphragm, psoas, quadratus lumborum, erector
spinae) & viscera (Le., colon, liver, pancreas, spleen)
o Metastases in local lymph nodes (2':: 1 cm)
o Metastases (most to least common) in lung, liver,
bone, adrenal, opposite kidney and brain; often
hypervascular
o Cystic RCC: Enhancing, smooth or nodular septa
MR Findings
• lsointense (60%) on Tl & T2WI or hyperintense (40%)
on T2WI
• Hypointense band/rim on TlWI (25%) & T2WI (60%)
• T1 C+: Enhances, usually less than renal tissue
• Multiplanar ideal for renal venous & IVC extension
Ultrasonographic Findings
• Real Time
o Liver overlies right kidney, a good acoustic window
o Detect 85% of mass> 3 cm, :::;60% < 2 cm
o Hyperechoic (48%), isoechoic (42%) or hypoechoic
(10%) renal mass
o Poor acoustic transmission, lobulated contour
o Hypoechoic rim, irregular or poorly defined
interface with adjacent renal tissue
o Cystic RCC
• Multiple septations with septal thickening
nodules and/or calcification
• Mural thickening, nodules and calcification
• Diffuse, hypoechoic mass (hemorrhage and
necrosis) with fluid-debris levels
• Color Doppler
o Assess vascularity of renal mass; most prominent
color flow around the periphery of the mass
o High velocity signal from arteriovenous shunting
Imaging Recommendations
• Best imaging tool
o Multiphase CT: Diagnosis and staging
• Arterial phase: Early corticomedullary phase
• Corticomedullary phase: Enhancing renal cortex,
limited medullary
• Renal angiomyolipoma
• Transitional cell carcinoma
• Renal metastases and lymphoma
• Renal infection
• Hemorrhagic renal cyst
Clinical Issues
• Gross hematuria (60%), flank pain (40%), palpable
flank mass (30-40%) (classical triad < 10%)
• Age: 50-70 years of age
• Gender: M:F = 1.5-2:1
Diagnostic Checklist
• RCC frequently found "by accident" on CT
3
• Papillary type RCC is hypovascular, easily mistaken 97
for cyst on all imaging modalities
• Nephrographic phase: Full capillary level renal
enhancement
• Excretory phase: Contrast in collecting system
o MR: Staging is equal or better than CT
• Protocol advice
o Multiphase CT
• Mandatory: Nonenhanced and parenchymal
phase (2':: 80 sec delay); optional corticomedullary
(60 sec), excretory (2-5 min delay)
• 3D mapping with volume rendering ± maximum
intensity projection technique for pre-operative
staging
I DIFFERENTIAL DIAGNOSIS
Renal oncocytoma
• Sharply marginated, homogeneous mass with smooth,
rounded margins
• Stellate area of low attenuation representing scar; may
simulate hemorrhagic or necrotic RCC
• Imaging cannot differentiate; surgical resection needed
Renal angiomyolipoma
• Intratumoral, fat attenuating mass without
calcification; very rare in RCC
• Indistinguishable from RCC if no visible fat on CT
Transitional cell carcinoma
• Renal infiltration ~ kidney enlargement and poorly
defined margins without change in shape
• Renal pelvic filling defect, irregular narrowing of
collecting system
• Encasing pelvicaliceal system ~ hypovascular tumor
• Rarely simulates RCC
Renal metastases and lymphoma
• Metastases (e.g., lung cancer, breast cancer, colon
cancer, malignant melanoma)
o Usually hypovascular with infiltrative growth
o If hypervascular, may simulate RCC
• Lymphoma
o Usually multiple or bilateral with infiltrative growth
 RENAL CELL CARCINOMA
o Hypoechoic, hypovascular, solitary, intrarenal mass
± adenopathy may simulate RCC
o Biopsy or chemotherapy may be used for diagnosis
Renal infection
• E.g., focal pyelonephritis, renal abscess
• Necrotic mass with renal enlargement
• Differentiate by clinical history and urinalysis
Hemorrhagic renal cyst
• Avascular RCC appears similar; use multi phase CT
I PATHOLOGY
3 General Features
98 • Genetics: Mutation of von Hippel-Lindau (VHL) gene
(3p25); autosomal dominant
• Etiology
o Risk factors
• Genetics: VHL
• Environment: Smoking
• Chemical: Diethylstilbestrol, fluoroacetamide,
dimethylnitrosamine, lead, cadmium
• Epidemiology
o Accounts for 2% of all cancers
o Incidence: 30,000 per year in U.S.
o Mortality: 12,000 per year in U.S.
• Associated abnormalities: Hereditary RCC, VHL,
tuberous sclerosis, acquired cystic renal disease
Gross Pathologic & Surgical Features
• Completely solid to cystic mass with irregular
lobulated margins
• Heterogenous appearance with hemorrhage and
necrosis (cut section)
Microscopic Features
• Clear cell (70%), papillary (10-15%), granular cell
(7%), chromophobe cell (5%), sarcomatoid (1.5%),
collecting duct « 1%)
Staging, Grading or Classification Criteria
• Robson classification of RCC with TNM correlation
o Stage I (Tl or 2, NOMO):Tumor confined to kidney
o Stage II (T3a, NOMO):Tumor spread to perinephric
fat, but confined within renal fascia; possible
ipsilateral adrenal gland
o Stage IlIA (T3b-3c, NOMO):Tumor spread to renal
vein, IVC or both
o Stage IIIB (TI-3a, NI-3, MO): Tumor spread to local
lymph nodes
o Stage IIIC (T3b-3c, NI-3, MO): Tumor spread to local
vessels and lymph nodes
o Stage IVA (T4, any N, MO): Tumor spread to adjacent
organs (except ipsilateral adrenal gland)
o Stage IVB (any T & N, Ml): Distant metastases
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Gross hematuria (60%), flank pain (40%), palpable
flank mass (30-40%) (classical triad < 10%)
o Fever, anorexia, weight loss, malaise, nausea,
vomiting, constipation
o Systemic manifestations by paraneoplastic (e.g.,
erythropoietin secretion, renin secretion),
paraendocrine (e.g., hepatopathy) and serological
(e.g., amyloidosis) syndromes
o Distant metastases may cause symptoms of cough,
hemoptysIs, bone pain
Demographics
• Age: 50-70 years of age
• Gender: M:F = 1.5-2:1
Natural History & Prognosis
• Prognosis
o 5, 10 year survival rate
• Stage I: 67%, 56%
• Stage II: 51%, 28%
• Stage III: 33.5%, 20%
• Stage IV: 13.5%, 3%
o 5, 10 year survival rate in patients with multiple
distant metastases: 5-10%, < 5%
o Bilateral or multiple RCC have poorer survival rate;
solitary RCC or metastasis has better survival rate
o 20-30% with localized tumor who underwent
surgical resection relapse in 15-18 months
Treatment
• Radical nephrectomy is the standard treatment
• Partial nephrectomy is a common alternative
o Preoperatively, do CT angiography & 3D mapping
o Requires::; 5 cm tumor size, peripheral location,
exophytic extension and no invasion of vessels or
lymph nodes
• Chemotherapy and hormone therapy: Limited
• Immunotherapy: 15% complete/partial response
I DIAGNOSTIC CHECKLIST
Consider
• RCC frequently found "by accident" on CT
Image Interpretation Pearls
• Papillary type RCC is hypovascular, easily mistaken for
cyst on all imaging modalities
I SELECTED REFERENCES
1. Catalano C et al: High-resolution multidetector CT in the
preoperative evaluation of patients with renal cell
carcinoma. AJRAm J Roentgenol. 180(5):1271-7,2003
2. Sheth 5 et al: Current concepts in the diagnosis and
management of renal cell carcinoma: Role of multidetector
CT & three-dimensional CT. Radiographics. 21:5237-54,
2001
3. Herts BRet al: Triphasic helical CT of the kidneys:
Contribution of vascular phase scanning in patients before
urologic surgery. AJR. 173: 1273-77, 1999
4. Birnbaum BAet al: Multiphasic renal CT: comparison of
renal mass enhancement during the corticomedullary and
nephrographic phases. Radiology. 200(3):753-8, 1996
5. Motzer RJ et al: Renal-cell carcinoma. N Engl J Med.
335(12):865-75, 1996
 RENAL CELL CARCINOMA

I IMAGE GAllERY
Typical
(Left) Axial CECT shows an
expansile renal mass with
calcification and extensive
regional lymphadenopathy.
(Right) Selective angiogram
shows hypervascular lower
pole renal mass.

3
99

(Left) Axial CECT shows a

homogeneous renal mass
that enhanced only 20 HU
over the unenhanced scan;
papillary renal cell
carcinoma. (Right) Selective
angiography in a patient with
papillary RCC shows no
obvious vascularity to this
malignant mass (arrow).

Typical
(Left) Axial CECT shows a
spherical hypodense right
renal mass (R01=60 HU):
RCC. (Right) Sagittal
sonogram shows echogenic
mass in right kidney; RCC.
 RENAL MEDULLARY CARCINOMA

3
100

Axial CECT in a 32 year old African-American man with Arteriogram in a 32 year old man with sickle cell anemia
sickle cell trait shows an infiltrative tumor with the shows a hypovascular mass in the center of the kidney.
preservation of renal outline due to sickle cell disease.

ITERMINOlOGY Angiographic Findings

• Hypovascular mass
Definitions
• Highly aggressive, very rare tumor of renal medulla Imaging Recommendations
arises in calyceal transitional epithelium • Best imaging tool: CECT

I IMAGING FINDINGS I DIFFERENTIAL DIAGNOSIS

General Features Renal cell carcinoma
• Best diagnostic clue: Infiltrative, centrally located mass • Usually rounded or oval mass in cortical location and
with preserved renal shape in a sickle cell patient grow by expansion
• Size: 4-12 em; mean is 7 em • Collecting duct variant: Tumor location and pattern of
growth may simulate renal medullary carcinoma
CT Findings
• CECT Transitional cell carcinoma
o Central infiltrative tumor growth ~ caliectasis • Renal infiltration ~ kidney enlargement and poorly
without pelviectasis defined margins without change in shape
o Fistula between necrotic cavity & collecting system • Renal pelvic filling defect, irregular narrowing of
o Infiltration from renal medulla to renal cortex ~ collecting system
renal enlargement & preservation of reniform shape • Encasing pelvicaliceal system ~ hypovascular tumor
o Heterogeneous enhancement (necrosis)
o Infiltration into peripelvic or perinephric fat Renal angiomyolipoma
o Renal venous & inferior vena cava tumor extension • Intratumoral fat attenuating mass
o Direct extension to retroperitoneal structures and
Renal metastases and lymphoma
ipsilateral adrenal gland
• Metastases (e.g., lung cancer, breast cancer, colon
o Metastases in lung, liver, local & retroperitoneal
cancer, malignant melanoma)
lymph nodes
o Usually hypovascular with infiltrative growth

DDx: Renal Mass

Renal Cell Carcinoma Transitional Cell Ca. Renal Lymphoma Renal Metastases
 RENAL MEDULLARY CARCINOMA

Key Facts
Imaging Findings • Renal angiomyolipoma
• Best diagnostic clue: Infiltrative, centrally located • Renal metastases and lymphoma
mass with preserved renal shape in a sickle cell Clinical Issues
patient
• Hematuria, abdominal or flank pain
• Best imaging tool: CECT
• Prognosis: Very poor
Top Differential Diagnoses Diagnostic Checklist
• Renal cell carcinoma • Young, African-American patient with sickle cell trait
• Transitional cell carcinoma • Centrally located renal mass with Infiltrative pattern

• Lymphoma
Natural History & Prognosis
3
o Usually multiple or bilateral with infiltrative growth
• Prognosis: Very poor 101
Focal pyelonephritis o Mean survival is 15 weeks, rarely survive> 1 year
• Differentiate by clinical history and urinalysis
Treatment
• Nephrectomy for disease without metastases
I PATHOLOGY • Chemotherapy and radiation therapy: Limited

General Features
• Genetics: Hemoglobin (Hb) beta globin gene mutation I DIAGNOSTIC CHECKLIST
• Etiology: Genetics: Sickle cell trait or Hb SC disease
• Epidemiology: Very rare; - 50 cases reported in U.S. Consider
• Young, African-American patient with sickle cell trait
Gross Pathologic & Surgical Features
• Infiltration of renal cortex Image Interpretation Pearls
• Nephromegaly with preservation of renal shape • Centrally located renal mass with Infiltrative pattern
• Poorly defined lobulated mass in renal medulla
• Tan to gray with variable hemorrhage and necrosis
• Usually with edematous or myxoid appearance, I SELECTED REFERENCES
sometimes collagenous 1. Choyke PL et al: Hereditary renal cancers. Radiology.
226(1):33-46, 2003
Microscopic Features 2. Khan A et al: Renal medullary carcinoma: sonographic,
• Reticular pattern with the cell aggregates forming computed tomography, magnetic resonance and
spaces of varying size (most common) angiographic findings. Eur J Radiol. 35(1):1-7, 2000
• Microcystic pattern with micropapillae projecting into 3. Avery RA et al: Renal medullary carcinoma: clinical and
the cyst lumina (less common) therapeutic aspects of a newly described tumor. Cancer.
78(1):128-32, 1996
• Associated with areas of compact adenoid cystic
4. Davidson AJ et al: Renal medullary carcinoma associated
growth pattern and poorly differentiated areas as solid with sickle cell trait: radiologic findings. Radiology.
sheets of cells 195(1):83-5, 1995
• Cells show a dark cytoplasm, clear nuclei and
prominent nucleoli
• ± Cytoplasmic mucin droplets
• ± Cells display squamoid appearance
I IMAGE GALLERY
• Polymorphonuclear leukocytes within tumor
• Sickled erythrocytes in focal areas of tumor or adjacent
renal tissue
• Tumor invasions of lymphatics and veins

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Hematuria, abdominal or flank pain
o Fever, weight loss, palpable abdominal mass
Demographics (Left) Axial NECT in a 35 year old man with sickle cell trait shows a
heterogeneous, infiltrative mass with invasion of perirenal space.
• Age: 11-39 years of age, average is 22 years of age
Sclerotic bones due to sickle cell. (Right) Axial NECT in 35 year old
• Gender: M:F = 2:1 man with renal medullary carcinoma shows multiple lung and pleural
• Ethnicity: African-American patients metastases.
 TRANSITIONAL CELL CARCINOMA

3
102

Graphic shows multifocal Tee involving proximal ureter Retrograde pyelogram shows "oncoca/ices" in upper
& upper pole calices. Partial obstruction of pole, distended with tumor.
infundibulum results in upper pole hydronephrosis with
filling defects (" oncoca/ices").

• "Stipple sign": Contrast within interstices of tumor

ITERMINOLOGY
Abbreviations and Synonyms
• Transitional cell carcinoma (TCC)
IIMAGING FINDINGS
General Features
• Best diagnostic clue: Dilated lumen upstream and
downstream ureteral mass on retrograde pyelography
• Location
o Bladder (90%): Lateral wall, anterior superior surface
o Kidney (8%): Extrarenal part of renal pelvis more
common than infundibulocalyceal
o Ureter & proximal two thirds urethra (2%)
Radiographic Findings
• IVP
o Nonfunctioning parenchymal segments secondary
to collecting duct obstruction; global non function if
obstructed at ureteropelvic junction or ureter
o Single or multiple discrete filling defects; surface is
usually irregular, stippled, serrated or frond-like
• Can also be seen in retrograde pyelography
o Mass may be flat with minimal or no intraluminal
intrusion (nonpapillary); or have a pedicle
o Renal pelvis
• "Oncocalyx": Ballooned tumor-filled calyx
• "Phantom calyx": Unopacified calyx from
obstruction of calyceal infundibulum
o Ureter
• Normal or delayed excretion (partial obstruction)
• Hydronephrosis ± hydroureter
• Fixation of ureter with irregular narrowing of
lumen and non-tapering margins
o Bladder
• Irregular filling defect with broad base and
frondlike projections
• Retrograde pyelography
o Renal pelvis
• Pyelotumoral backflow: Contrast in interstices
• Opacification of phantom calyces; irregular
papillary or nodular mucosa
o Ureter
• Goblet or champagne glass sign: Cup-shaped
contrast collection distal to an intraluminal filling
defect of ureter (intraluminal growth of tumor)
• Ovoid filling defect from wall: Short stalk
• Smooth or frondlike surface; brush border or
stippled appearance
• Irregular decrease in luminal diameter over
variable lengths (varying obstruction)

DDx: Filling Defect or Infiltrative Mass

Renal Pelvic Clot Renal Cell Cancer Renal Tuberculosis

 TRANSITIONAL CELL CARCINOMA

Key Facts
Imaging Findings Top Differential Diagnoses
• Best diagnostic clue: Dilated lumen upstream and • Calculus
downstream ureteral mass on retrograde pyelography • Blood clot
• Bladder (90%): Lateral wall, anterior superior surface • Infection (e.g., tuberculosis)
• Kidney (8%): Extrarenal part of renal pelvis more • Papillary necrosis
common than infundibulocalyceal • Renal cell carcinoma (RCe)
• Ureter & proximal two thirds urethra (2%)
• Single or multiple discrete filling defects; surface is Clinical Issues
usually irregular, stippled, serrated or frond-like • Gross hematuria (70-80%), dull or colicky pain (50%)
• Goblet or champagne glass sign: Cup-shaped contrast • Age: > 60 years of age
collection distal to an intraluminal filling defect of • Gender: M:F = 2-3:1
• Follow-up: Lifetime increased risk of recurrent TCC;
ureter (intraluminal growth of tumor)
• Best imaging tool: Retrograde pyelography and/or CT require yearly surveillance 3
urography Diagnostic Checklist 103
• Synchronous or metachronous TCC
• Filling defects; "stipple" sign; goblet sign

o T1 C+: Mild enhancement (primary, perivesical,

CT Findings
• NECT: Isodense to slightly hyperdense (31-48 HU)
mass relative to urine
• CECT: Hypovascular infiltrative tumor with minimal
enhancement (43-82 HU); preserved renal shape
• Renal pelvis
o Sessile, flat or polypoid solid mass
o ± Calcifications (2% of renal pelvis TCe)
o Focal renal pelvic wall thickening
o Identify intracaliceal or anterior/posterior wall
tumor; obscured in IVP
o Compression or invasion of renal sinus fat and
parenchyma
o Crust-like rims: Contrast in curvilinear calyceal
spaces around periphery of the tumor
o Coarse striations or diminished nephrogram (partial
obstruction)
o Oncocalyx: Ballooned tumor-filled calyx
o. ± Hydronephrosis
o Variable enhancement: Based on growth pattern,
vascularity and obstruction
o ± Metastases
• Ureter
o Dilated, urine-filled collecting system
o Demonstrate level of obstruction
o Intraluminal soft tissue mass (30-60 HU)
o ± Extraureteral extension; ± enhancement
o Eccentric or circumferential wall thickening
• Bladder
o Focal wall thickening and mass protruding into
lumen; ± enhancement
MR Findings
• Renal pelvis & ureter
o T1WI: Same or slightly I versus normal parenchyma
o T2WI: Same or slightly t versus normal parenchyma
o T1 C+: I or t Enhancement (hypo-/hypervascularity)
• Bladder: MR is staging modality of choice
o T1WI: TCC isointense to bladder muscle
o T2WI: Hyperintense than normal bladder wall; ±
perivesical invasion
nodal or bone invasion)
Imaging Recommendations
• Best imaging tool: Retrograde pyelography and/or CT
urography
o CT urography
• Combines axial CECT with subsequent
radiography or multiplanar reconstruction
• Provides "IVP"-type image plus conventional CT
views
• Most informative, accurate single imaging
modality for renal or ureteral TCC
I DIFFERENTIAL DIAGNOSIS
Calculus
• IVP: Ureteral filling defects
• Retrograde pyelography: No goblet sign
• CT: Most calculi are markedly hyperdense (> 200 HU);
differentiate from tumors
• US: Echogenic foci with posterior shadowing
Blood clot
• IVP: "Stipple sign" simulates TCC
• CT: Denser than renal parenchyma (50-90 HU), no
enhancement; disappear with time
• US: Same echogenicity as tumors; mobile and
disappear with time
Infection (e.g., tuberculosis)
• IVP: Amputated calyx (TB)
• CT: Parenchymal mass ± calcifications (TB)
o Filling defect in pelvis; nonenhancing (fungus ball)
Papillary necrosis
• Destruction of the apex of the pyramid - irregular
cavitation & sinus formation between papilla & calyx
• Retrograde pyelography: Fill 1 or 2 irregular cavities
• CT: Ring or triangular calcification of sloughed papilla
 TRANSITIONAL CELL CARCINOMA
• T4a: Invades adjacent organs; T4b Invades pelvic or
Renal cell carcinoma (RCC) abdominal wall
• May also cause infiltrative mass, extend into renal • Nl-3: Pelvic nodes; N4: Above renal bifurcation
pelvis • M1 Distant metastases

I PATHOLOGY I CLINICAL ISSUES

General Features Presentation
• General path comments • Most common signs/symptoms: Gross hematuria
o Uroepithelial cancers (70-80%), dull or colicky pain (50%)
• TCC: 90% of renal pelvic & 97% of ureteral • Lab data
cancers o Urinalysis: Hematuria, ± pyuria and bacteriuria
• Squamous cell carcinoma: 5-10% of renal pelvic & o Urine cytology or ureteroscopy with biopsy: Positive
3 rare in ureteral cancers
Demographics
• Adenocarcinoma: Very rare in both
104 o Synchronous or metachronous (multicentricity) • Age: > 60 years of age
• Metachronous upper tract TCe: 11-13% • Gender: M:F = 2-3:1
• Metachronous ipsilateral upper tract TCC: 14-30%
• Synchronous bilateral renal pelvic TCC: 1-2%; Natural History & Prognosis
ureteral: 2-9% • Complications: Hydronephrosis, infections, fistulas
• Synchronous bladder TCC & renal pelvic TCC: • Renal pelvis & ureteral
24%; ureteral: 39% o :s T1: 77-80% 5 year survival rate
• Upper tract & bladder TCC at some time: 30-75% o T2: 44% 5 year survival rate
• Upper tract TCC in 10%, 26% & 34% at 5,5-10 & o 2c T3: 0-20% 5 year survival rate
> 10 years of follow-up of bladder TCC • Bladder: Overall 5 year survival rate is 30%
• Etiology Treatment
o Environment: Smoking (2c 3-folds), Balkan countries
• Renal pelvis and ureteral
o Occupation: Dyes, rubber, cable, plastics
o Total nephroureterectomy and bladder cuff excision
o Iatrogenic: Analgesic, cyclophosphamide, Thorotrast
o Metastases: Chemotherapy and/or radiation
o Infections: Chronic bacterial urinary tract infections,
Schistosoma haematobium (squamous cell • Bladder
o Superficial: Transurethral resection + Bacillus
carcinoma of bladder and ureter)
Calmette-Guerin (BCG) (j 40-45% recurrence rate)
• Epidemiology
o Deep: Partial/radical cystectomy (with "neobladder"
o Urothelial tumors: 3% of mortality in U.S.
or ileal conduit) or radiation ± chemotherapy
o TCC is 5% of all urothelial tumors
o Metastases: Surgery or radiation ± chemotherapy
• Most TCC are bladder cancers
• Follow-up: Lifetime increased risk of recurrent TCC;
o Ureteral: Incidence is % of renal pelvis
require yearly surveillance
o Bladder: Cancer mortality: Males: 4th; females: 10th
Gross Pathologic & Surgical Features
• 2c 85% papillary (low grade); ± infiltrative (high) I DIAGNOSTIC CHECKLIST
• 4 types of features
Consider
o Nonpapillary, noninfiltrative (carcinoma in situ):
Slight planar thickening ± hyperemia or hemorrhage • Synchronous or metachronous TCC
o Papillary, noninfiltrative: Long, cylindrical villi; few • Cystoscopy still necessary to diagnose bladder cancer
mm to several cm Image Interpretation Pearls
o Papillary infiltrative: Large, thick villi; broad base • Filling defects; "stipple" sign; goblet sign
o Nonpapillary, infiltrative: Thickening of renal pelvic
or ureteral walls
Microscopic Features I SELECTED REFERENCES
• Transitional epithelium: In renal pelvis, ureter, bladder 1. Akbar SA et al: Multidetector CT urography: techniques,
and proximal two thirds urethra clinical applications, and pitfalls. Semin Ultrasound CT
• Epithelial atypia or dysplasia; abnormal fibrovascular MR. 25(1):41-54, 2004
2. Caoili EM et al: Urinary tract abnormalities: initial
core of lamina propria
experience with multi-detector row CT urography.
Staging, Grading or Classification Criteria Radiology. 222(2):353-60, 2002
3. Wong- You-Cheong JJ et al: Transitional cell carcinoma of
• TO: No tumor; Tis: Carcinoma in situ; Ta: Tumor
the urinary tract: Radiologic-pathologic correlation.
confined to mucosa Radiographies 18: 123-42, 1998
• T1: Invades lamina propria 4. Urban BA et al: CT appearance of transitional cell
• T2a: Invades inner muscularis; T2b: Outer muscularis carcinoma of the renal pelvis: Part 2. advanced-stage
• T3a: Microscopic perivesical fat invasion; T3b: disease. AJR 169: 163-8, 1997
Macroscopic perivesical fat invasion
 TRANSITIONAL CELL CARCINOMA
I IMAGE GALLERY

(Left) Axial CECT shows

infiltrative hypovascular
tumor "replacing" renal
parenchyma. (Right) Axial
CECT shows infiltrative
hypovascular mass with
preserved renal outline;
enlarged node (arrow).

3
105

(Left) Axial CECT shows

hypovascular mass in renal
pelvis. (Right) Retrograde
pyelogram shows
amputation of upper pole
infundibulum and mass
effect on mid and lower pole
calices.

Typical
(Left) Axial CECT shows
infiltrative heterogeneous
hypovascular mass with
invasion of left renal vein
and nodes. (Right) Axial
CECT with coronal
reformation shows irregular
mass (arrows) with dilation
of ureteral lumen ("goblet"
sign).
 RENAL METASTASES AND LYMPHOMA

3
106

Axial CECT shows multiple homogeneous hypodense Axial CECT shows multiple masses in the kidneys and
masses in the kidneys and retroperitoneal nodes; liver; metastatic squamous cell cancer (from paranasal
non-Hodgkin lymphoma. sinuses).

o Usually located within renal parenchyma; rarely

ITERMINOLOGY
Definitions
• Metastases from primary cancer of other sites
• Lymphoma: Malignant tumor of B lymphocytes
I IMAGING FINDINGS
General Features
• Best diagnostic clue: Multiple masses in bilateral
kidneys
• Other general features
o Renal metastases
• Types of spread: Hematogenous, direct
• Hematogenous spread: Most common
o Renal lymphoma
• Primary: Rare, 3% of all cases of renal lymphoma
• Secondary: Hematogenous spread (90%) or direct
extension via retroperitoneal lymphatic channels
CT Findings
• Renal metastases
o Multiple, small, low density renal masses
o Mass occasionally is large and solitary; may simulate
renal cell carcinoma
o Usually bilateral renal involvement; rarely unilateral
o Wedge-shaped appearance occasionally
cause deformity of renal contour or capsule
o Iso- to hypodense (10-40 HU) masses on NECT
o Limited enhancement after contrast (10-20 HU),
except highly vascular tumors (Le., melanoma)
o Extension through renal capsule into perinephric
space
o Widespread extrarenal metastases usually present
• Renal lymphoma
o Renal masses are homogeneous with minimal
contrast-enhancement (10-20 HU)
o Multiple, bilateral renal masses (40-60%)
o Retroperitoneal adenopathy (25%), splenomegaly or
lymphadenopathy at other sites
o Solitary renal mass in one kidney (15%)
o Infiltration of renal parenchyma or sinus,
maintaining reniform shape; may simulate
transitional cell carcinoma
o Direct extension into renal hilum or pericapsular
region from retroperitoneal lymph nodes (25%)
o Diffuse parenchymal involvement (10%) of one or
both kidneys ~ diffuse enlargement
o Attenuated and poorly opacified collecting systems
with variable degree of I enhancement on CECT
o Renal vein & inferior vena cava tumor thrombus
o Extranodal involvement of gastrointestinal tract,
brain, liver and bone marrow (especially in AIDS)

DDx: Renal Masses

Renal Cell Carcinoma Transitional Cell Angiomyolipomas Renal Infection

 RENAL METASTASES AND LYMPHOMA
Key Facts
Imaging Findings • Transitional cell carcinoma
• Best diagnostic clue: Multiple masses in bilateral • Renal angiomyolipoma
kidneys • Renal infection
• Usually located within renal parenchyma; rarely Clinical Issues
cause deformity of renal contour or capsule
• Usually asymptomatic
• Limited enhancement after contrast (10-20 HU),
• Renal lymphoma: 57% have complete remission after
except highly vascular tumors (Le., melanoma) treatment
• Infiltration of renal parenchyma or sinus,
maintaining reniform shape; may simulate Diagnostic Checklist
transitional cell carcinoma • Check for history of primary cancer
• Best imaging tool: Helical CT • Overlapping radiographic features of renal
• Protocol advice: CT: Renal lymphoma:
Nephrographic phase imaging is ideal
metastases, lymphoma and primary carcinoma
• Suspected metastases or lymphoma is one of the rare
3
indications for percutaneous biopsy of a renal mass 107
Top Differential Diagnoses
• Renal cell carcinoma

MR Findings I DIFFERENTIAL DIAGNOSIS

• Renal lymphoma
o T1WI: Iso- to slightly hypointense Renal cell carcinoma
o T2WI: Hypointense • Solitary renal mass with central necrosis
o T2* GRE, T1 C+: Minimal enhancement • Mass is usually rounded or oval in cortical location
and grows by expansion
Ultrasonographic Findings • Hypervascular mass
• Real Time
o Renal lymphoma Transitional cell carcinoma
• Hypoechoic relative to renal parenchyma; may • Renal infiltration ~ kidney enlargement and poorly
simulate renal cyst defined margins without change in shape
• Limited posterior acoustic enhancement • Renal pelvic filling defect, irregular narrowing of
• Rarely shows a sonographic halo or hypoechoic collecting system
mass in perinephric regions • Encasing pelvicaliceal system ~ hypovascular tumor

Angiographic Findings Renal angiomyolipoma

• Conventional • Mass with foci of fat
o Renal metastases Renal infection
• Usually masses are hypovascular with infiltrative
• E.g., focal pyelonephritis, renal abscess
renal pattern with little or no vascularity
• Multiple necrotic masses with renal enlargement
• Hypervascularity (e.g., melanoma); may simulate
• Differentiate by clinical history and urinalysis
renal cell carcinoma
• Perinephric hemorrhage (e.g., melanoma)
o Renal lymphoma
• Marked attenuation of segmental and interlobar
I PATHOLOGY
arteries General Features
• Masses are usually hypovascular or avascular • General path comments
• Rarely, masses demonstrate neovascularity or o Renal metastases
hypervascularity with arterial venous shunting • In autopsy, 2-3 times more common than primary
• Multiple low density cortical defects (nephrogram renal neoplasms
phase) • Fifth most common site of metastases in the body
Nuclear Medicine Findings after lung, liver, bone and adrenals
• Bone Scan • Usually too small to be found by imaging due to
o Renal metastases brief survival of patient
o Renal lymphoma
• Increased activity (e.g., breast carcinoma, sarcoma)
• 6% of patients with lymphoma at presentation
Imaging Recommendations • 33% of patients dying from malignant lymphoma
• Best imaging tool: Helical CT • Renal involvement of non-Hodgkin lymphoma to
• Protocol advice: CT: Renal lymphoma: Nephrographic Hodgkin lymphoma ratio: 10:1
phase imaging is ideal • Etiology
o Renal metastases
• Bronchogenic carcinoma (19.8-23.3%)
 RENAL METASTASES AND LYMPHOMA
• Breast carcinoma (12.3%) • Nephrectomy if metastases and lymphoma is small
• Gastric carcinoma (11.1-15.1%) and isolated to one kidney or have other extenuating
• Malignant melanoma circumstances (Le., severe renal hemorrhage)
• Renal cell carcinoma of contralateral kidney
• Colonic carcinoma
• Cervical carcinoma [DIAGNOSTIC CHECKLIST
• Ovarian carcinoma
• Pancreatic carcinoma Consider
• Prostate carcinoma • Check for history of primary cancer
• Thyroid carcinoma • Patient with multiple renal masses and widespread
• Testicular carcinoma systemic metastases or a history of extrarenal primary
• Various sarcomas (Le., osteogenic sarcoma) cancer should suspect renal metastases
o Renal lymphoma
Image Interpretation Pearls
• Immunosuppression: Iatrogenic (Le., organ
3 transplantation) or acquired (Le., acquired
• Overlapping radiographic features of renal metastases,
lymphoma and primary carcinoma
immunodeficiency syndrome (AIDS),
108 • Suspected metastases or lymphoma is one of the rare
autoimmune diseases, Burkitt lymphoma)
indications for percutaneous biopsy of a renal mass
Microscopic Features
• Renal metastases: Varies based on primary cancer
• Renal lymphoma I SELECTED REFERENCES
o Lymphoepitheliallesions 1. Rendon RA et al: The natural history of small renal masses.
o Diffuse (more common) or nodular J Urol. 164(4):1143-7, 2000
o Non-Hodgkin lymphoma: Large cell lymphoma is 2. Urban BA et al: Renal lymphoma: CT patterns with
most common emphasis on helical CT. Radiographics. 20(1):197-212,
o AIDS: Small cell lymphoma is most common 2000
3. Sheeran SR et al: Renal lymphoma: spectrum of CT
findings and potential mimics. AJRAm J Roentgenol.
171(4):1067-72, 1998
I CLINICAL ISSUES 4. Smith PA et al: Spiral computed tomography evaluation of
the kidneys: state of the art. Urology. 51(1):3-11, 1998
Presentation 5. Ferrozzi F et al: Computed tomography of renal metastases.
• Most common signs/symptoms Semin Ultrasound CT MR. 18(2):115-21, 1997
o Renal metastases 6. Wyatt SH et al: Spiral CT of the kidneys: role in
• Usually asymptomatic characterization of renal disease. Part II: Neoplastic disease.
• Hematuria or flank pain; rarely as the initial Crit Rev Diagn Imaging. 36(1):39-72, 1995
symptoms of the primary tumor elsewhere 7. Honda H et al: CT analysis of metastatic neoplasms of the
kidney. Comparison with primary renal cell carcinoma.
o Renal lymphoma
Acta Radiol. 33(1):39-44, 1992
• Usually asymptomatic 8. Volpe JP et al: The radiologic evaluation of renal
• Hematuria, flank pain, palpable mass or renal metastases. Crit Rev Diagn Imaging. 30(3):219-46, 1990
insufficiency 9. Levine E et al: Small renal neoplasms: clinical, pathologic,
o Other signs/symptoms and imaging features. AJRAm J Roentgenol. 153(1):69-73,
• Fever, weight loss 1989
10. Pollack HM et al: Other malignant neoplasms of the renal
• Diagnosis
parenchyma. Semin Roentgenol. 22(4):260-74, 1987
o CT or US-guided percutaneous biopsy
11. Curry NS et al: Small renal neoplasms: diagnostic imaging,
Demographics pathologic features, and clinical course. Radiology.
158(1):113-7, 1986
• Age: Any age; middle-age to elderly more common Mitnick JS et al: Metastatic neoplasm to the kidney studied
12.
• Gender: Unknown by computed tomography and sonography. J Com put
Assist Tomogr. 9(1):43-9, 1985
Natural History & Prognosis 13. Nishitani H et al: Computed tomography of renal
• Complications metastases. J Comput Assist Tomogr. 8(4):727-30,1984
o Renal or perinephric hemorrhage, renal obstruction, 14. Heiken JP et al: Computed tomography of renal lymphoma
renovascular hypertension, acute renal failure with ultrasound correlation. J Comput Assist Tomogr.
• Prognosis 7(2):245-50, 1983
o Renal metastases: Very poor 15. Bhatt GM et al: CT diagnosis of renal metastases. J Com put
o Renal lymphoma: 57% have complete remission Assist Tomogr. 7(6):1032-4, 1983
16. Hartman DS et al: Renal lymphoma: radiologic-pathologic
after treatment
correlation of 21 cases. Radiology. 144(4):759-66, 1982
• 4 year survival rate: - 40% 17. Jafri SZ et al: CT of renal and perirenal non-Hodgkin
lymphoma. AJRAmJ Roentgenol. 138(6):1101-5, 1982
Treatment
18. Rubin BE: Computed tomography in the evaluation of
• Renal metastases: Systemic therapy or palliative renal lymphoma. J Comput Assist Tomogr. 3(6):759-64,
treatment only 1979
• Renal lymphoma: Chemotherapy ± radiation therapy
 RENAL METASTASES AND LYMPHOMA
I IMAGE GALLERY

Typical
(Left) Axial CECT shows a
lobulated renal mass in a
patient with AIDS; isolated
renal lymphoma. (Right)
Axial CECT shows multiple
small homogeneous
hypodense masses;
lymphoma.

3
109

Typical
(Left) Axial CECT in May
shows two spherical
hypodense renal masses due
to lymphoma. (Right) Axial
CECT in July shows no
residual mass, only scarring
at site of larger tumor;
following chemotherapy.

Typical
(Left) Axial CECT shows
renal and nodal masses due
to leukemia. (Right) Axial
NECT shows bilateral renal
and perirenal masses due to
multiple myeloma.
 SECTION 4: Ureter

Congenital
Ureteral Duplication 11I-4-2
Ectopic Ureter 11I-4-4
Ureterocele 11I-4-8

Infection
Ureteritis Cystica 11I-4-10
 URETERAL DUPLICATION

Excretory urogram shows "drooping lily" sign, with Pelvic film from urogram shows large filling defect in the
downward displacement of left lower pole calices by a bladder (arrow) representing a ureterocele from the
hydronephrotic, non functioning upper pole of a ectopic upper pole ureter that inserted into the prostatic
4 duplicated collecting system. urethra.

2
o "Drooping lily" sign: Hydronephrosis and I function
ITERMINOLOGY of obstructed upper pole ~ downward displacement
Abbreviations and Synonyms of lower pole calyces
• Double ureters o "Nubbin" sign: Scarring, atrophy and I function of
lower pole moiety; may simulate renal mass
Definitions o Fewer calyces & infundibula of lower pole collecting
• 2 ureters drain a duplex kidney and remain separate to system; shortened upper pole infundibulum
bladder or beyond o Single or diffuse calyceal clubbing, thin overlying
parenchyma ± scarring in lower pole
o ± Ureteropelvic junction obstruction of lower pole
I IMAGING FINDINGS • Voiding cystourethrogram
o ± Reflux, ureterocele, diverticulum of urethra
General Features
• Best diagnostic clue: Double collecting system CT Findings
ipsilaterally on IVP or CT urography • "Faceless kidney": No renal sinus or collecting system
• Other general features at junction of upper & lower pole of a duplex kidney
o 85% obey Weigert Meyer rule: Upper pole ureter • ± Obstruction in either pole of a duplex kidney
inserts medial & caudal to lower pole ureter
Ultrasonographic Findings
o 15% upper pole ureter inserts anywhere along
• 2 central echo complexes with intervening renal
ectopic pathway
parenchyma
o Most commonly, upper pole ureter is ectopic &
• Will show upper pole hydronephrosis
obstructed and lower pole ureter refluxes
o Kidney & ureter may be normal, except duplicated Nuclear Medicine Findings
o 20% of contralateral ureter is also duplicated • ± Reflux up one or both ureters
Radiographic Findings • Estimate degree of renal function
• IVP (or CT urography) Imaging Recommendations
o Duplex kidney with double ureters; 2 jets of contrast • Best imaging tool: IVP or CT urography
o Poor or no excretion by upper pole of duplex kidney

DDx: Variations or Mimics of Duplication

. ',!", ',..,,:~
" ,"~

• ~~
. t"

r&,
;- -:- t
I

\
-\ .I
,1"" Ii'
Phleboliths Ureterocele Ureterocele
 URETERAL DUPLICATION

Key Facts
Terminology Top Differential Diagnoses
• Double ureters • Bifid ureters
• 2 ureters drain a duplex kidney and remain separate • Gonadal vein phlebolith
to bladder or beyond • Ureterocele
Imaging Findings Diagnostic Checklist
• Best imaging tool: IVP or CT urography • Young females with recurrent urinary tract infections
• 2 distinct uretersi "drooping lily" sign

I DIFFERENTIAL DIAGNOSIS Natural History & Prognosis

• Complications: Urolithiasis, abscess, renal failure
Bifid ureters
• 2 ureters drain a duplex kidney, but join before Treatment
emptying into bladderi can be ectopic • Lower grades of reflux: Medical treatment
• Ureters may join at intravesical junction: V-shapedi • Higher grades of reflux, upper pole obstruction,
extravesical (most common) junction: Y junction
• IVP
ectopy, poor renal function: Surgical treatment 4
o Ureteroureteral reflux or yo-yo phenomenon: 3
Peristalsis down 1 limb of ureter forcing urine via I DIAGNOSTIC CHECKLIST
reflux up the other
o Asymmetric dilatation of 1 ureteral segment Consider
• Young females with recurrent urinary tract infections
Gonadal vein phlebolith
• May simulate stone in ureter (single or duplicated) Image Interpretation Pearls
• 2 distinct uretersi "drooping lily" sign
Ureterocele
• Can be isolated finding
I SELECTED REFERENCES
1.
I PATHOLOGY Zissin R et al: Renal duplication with associated
complications in adults: CT findings in 26 cases. Clin
General Features Radiol. 56(1):58-63, 2001
2. Fernbach SK et al: Ureteral duplication and its
• General path comments: Both ureters pass through complications. Radiographies. 17(1):109-27, 1997
bladder wall through a common tunnel 3. Share JC et al: The unsuspected double collecting system
• Etiology on imaging studies and at cystoscopy. AJRAm J
o Genetics: Autosomal dominant with low penetrance Roentgenol. 155(3):561-4, 1990
o Environment: Geographic areas ~ t prevalence
• Epidemiology: 1 per 500 persons
• Associated abnormalities I IMAGE GALLERY
o Solitary or dysplastic kidney, hypoplastic kidneys, all
types of fused kidneys or posterior urethral valves
o Complex congenital anomalies: VATER,VACTERL
(vertebral, anal, cardiovascular, tracheoesophageal,
renal and limb)

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, nocturnal incontinence
o Incontinence, only in females due to insertion of
upper pole ureteral orifice below bladder sphincter (Left) Axial CECT shows hydronephrotic upper pole collecting
o Intermittent or persistent urinary tract infections ± system. (Right) Axial CECT shows normal lower pole calices + ureter
acute pyelonephritis, frequently in females (arrow). Ureter draining upper pole (open arrow) is dilated.

Demographics
• Gender: M:F = 1:10
 ECTOPIC URETER

IVP shows "drooping lily" sign, with lower pole moiety Retrograde pyelogram shows opacification of dilated,
displaced inferiorly and laterally by obstructed upper tortuous ectopic ureter and severely hydronephrotic
pole moiety and dilated ectopic ureter (Courtesy FC upper pole moiety. Note displaced lower pole moiety
4 Sommer; MOJ. (Courtesy FC Sommer; MOJ.

4
• Urethra or vestibule most common insertion site
!TERMINOlOGY in female
Abbreviations and Synonyms o 5-17% of ectopic ureters are bilateral
• Ectopic ureter (EU), ureteral ectopia • Morphology
o Complete duplication: Ectopic ureter drains upper
Definitions moiety
• Ureter that does not terminate at bladder trigone o Orifice commonly stenotic, leading to obstruction of
o Ectopic insertion within bladder: Usually no upper pole moiety
significant pathology
Radiographic Findings
• Common usage: Ureter that terminates outside
bladder • Fluoroscopy
o Voiding cystourethrogram (VCUG): Reflux into
either moiety
I IMAGING FINDINGS o VCUG useful to locate insertion of ectopic ureter if
within urinary tract
General Features • Will not visualize ectopic insertion if outside
• Best diagnostic clue: 70-80% associated with complete urinary tract
ureteral duplication • IVP
• Location o Dilated upper pole collecting system
o Usually extravesicular insertion; males always above o Non-visualization of upper pole moiety with severe
external sphincter obstruction/dysplasia
o Males: Vas deferens 10%, seminal vesicle 28%, • Visualized lower pole moiety: Fewer calyces than
prostatic urethra 54%, ejaculatory duct 8% normal for whole kidney
• Prostatic urethra most common insertion site in • Lower pole displaced infero-Iaterally
male ("drooping-lily" sign)
o Females: Uterus or cervix 3%, vagina 27%, urethra o Ectopic insertion of single system ureter: Involved
32%, vestibule 38% kidney usually small, dysplastic and nonfuntional

DDx: Mimics of Ectopic Ureter

Sem. Vesicle Cyst Prostatic Cyst Cartner Duct Cyst

 ECTOPIC URETER
Key Facts
Terminology Pathology
• Common usage: Ureter that terminates outside • Hypoplasia or dysplasia of renal moiety drained by
bladder ectopic ureter
• Distance from trigone correlates with degree of
Imaging Findings ipsilateral renal dysplasia
• Best diagnostic clue: 70-80% associated with • More distal the ureter, the greater the dysplasia
complete ureteral duplication
• Prostatic urethra most common insertion site in male Clinical Issues
• Urethra or vestibule most common insertion site in • Females: Continual dribbling urinary incontinence
female (50%)
• Complete duplication: Ectopic ureter drains upper • Males: No incontinence because ectopic ureteral
moiety orifice always above external sphincter
• Orifice commonly stenotic, leading to obstruction of
upper pole moiety Diagnostic Checklist
• Lower pole displaced infero-Iaterally ("drooping-lily" • Weigert-Meyer law: Upper moiety ureter inserts
sign) inferior and medial to lower moiety ureter

I DIFFERENTIAL DIAGNOSIS
4
CT Findings
• CECT 5
o Hydronephrotic upper pole moiety with variable
Seminal vesicle cyst
function • Associated with renal agenesis
o Dilated, tortuous ureter to level of insertion Gartner duct cyst
o Males with single ectopic ureters: Non-functional
• Inclusion cyst in lateral wall of vagina, remnant of
kidney and dilated ipsilateral seminal vesicle mesonephric duct
MR Findings
• TlWI
o Tortuous low signal intensity ureter dilated to level I PATHOLOGY
of ectopic insertion
General Features
o Severe hydronephrosis of upper pole moiety
• General path comments
• T2WI
o Tortuous high signal intensity ectopic ureter dilated o Ectopic ureters opening to bladder neck or posterior
to level of insertion urethra may reflux
o High signal cystic dysplasia of ipsilateral upper pole o Ectopic ureters terminating outside urinary tract:
moiety Usually obstructed
• Tl C+: Variable degree of function in obstructed upper • Etiology
pole moiety o Congenital: Abnormal ureteral bud migration
• Failure of separation of ureteral bud from Wolffian
Ultrasonographic Findings duct results in caudal ectopia
• Real Time • Epidemiology
o Cystic area in medial upper pole of kidney = o Incidence: At least 1 in 1,900
non-functional obstructed upper moiety o True incidence uncertain since many cases
o Dilated ureter behing otherwise normal bladder asymptomatic
• Associated abnormalities
Nuclear Medicine Findings o Hypoplasia or dysplasia of renal moiety drained by
• Renal scintigraphy ectopic ureter
o Variable function of moiety drained by ectopic o Degree of ureteral ectopia correlates with degree of
ureter renal abnormality
Imaging Recommendations o Imperforate anus, trachea-esophageal fistula
• Best imaging tool Gross Pathologic & Surgical Features
o Ultrasound • Single system ectopic ureter: Absent ipsilateral
o CT may be useful to locate small poorly functioning hemitrigone
dysplastic kidney with single ectopic ureter • Distance from trigone correlates with degree of
o MR urography can display ectopic ureteral insertions ipsilateral renal dysplasia
even if outside urinary tract o More distal the ureter, the greater the dysplasia
• Protocol advice: Trace dilated ureter on US to its o Very distal insertions ~ usually very poor renal
terminus below bladder function
 ECTOPIC URETER
5. Staatz G et al: Magnetic resonance urography in children:
Microscopic Features evaluation of suspected ureteral ectopia in duplex systems.
• Muscularis of ectopic ureteral wall may have ] Urol. 166(6):2346-50, 2001
ultrastructural abnormalities 6. Patel PM et al: Ureteral triplication with ectopic upper pole
moiety. Urology. 58(2):279-80, 2001
7. Damry N et al: Ectopic vaginal insertion of a duplicated
ureter: demonstration by magnetic resonance imaging
I CLINICAL ISSUES (MRI).]BR-BTR.84(6):270, 2001
8. Lee SS et al: Giant hydronephrosis of a duplex system
Presentation
associated with ureteral ectopia: a cause of retrograde
• Most common signs/symptoms ejaculation. Arch Androl. 45(1):19-23, 2000
o Recurrent or chronic urinary tract infections (UTIs) 9. Engin G et al: MR urography findings of a duplicated
o Females: Continual dribbling urinary incontinence ectopic ureter in an adult man. Eur Radiol. 10(8):1253-6,
(50%) 2000
• Males: No incontinence because ectopic ureteral 10. Bourian M et al: Clinical quiz. Ectopic ureter. Pediatr
orifice always above external sphincter Nephrol. 14(8-9):879-81, 2000
11. Cabay]E et al: Ectopic ureter associated with renal
o Males: Chronic or recurrent epididymitis
dysplasia. ]BR-BTR.82(5):228-30, 1999
• Clinical profile 12. Komatsu K et al: Single ectopic vaginal ureter diagnosed by
o Girl with continuous dribbling urinary incontinence computed tomography. Urol Int. 63(2):147-50, 1999
o Prepubertal boy with epididymitis or UTI 13. De Vuyst D et al: Ectopic ureter.] Beige Radiol. 81(5):239,
1998
Demographics 14. Carrico C et al: Incontinence due to an infra sphincteric
4 • Age ectopic ureter: why the delay in diagnosis and what the
radiologist can do about it. Pediatr Radiol. 28(12):942-9,
o Age at diagnosis varies widely; some cases not
6 detected during life 1998
o Many cases diagnosed with prenatal ultrasound 15. Dunnick NR et al: Textbook of uroradiology. 2nd ed.
Baltimore, Williams and Wilkins, 29-33, 1997
• Gender 16. Plaire]C et al: Management of ectopic ureters: experience
o M:F = 1:6 with the upper tract approach.] Urol. 158(3 Pt 2):1245-7,
o Single system ectopic ureters more common in 1997
males 17. Yanagisawa N et al: Diagnostic magnetic
o Ectopic ureters in males usually drain single systems resonance-urography in an infant girl with an ectopic
o Females: 80% of ectopic ureters are duplicated ureter associated with a poorly functioning segment of a
systems duplicated collecting system. Int] Urol. 4(3):314-7, 1997
18. Amatulle P et al: Ureteral duplication anomaly with ectopic
Natural History & Prognosis intraprostatic insertion.] Ultrasound Med. 16(3):231-3,
• Most ectopic ureters drain single kidneys or upper pole 1997
19. el Ghoneimi A et al: Ectopic ureter with complete ureteric
moieties with minimal function
duplication: conservative surgical management. ] Pediatr
Treatment Surg. 31(4):467-72, 1996
20. Rothpearl A et al: MR urography: technique and
• Options, risks, complications application. Radiology. 194(1):125-30, 1995
o' Ectopic ureter with duplicated system: Surgical 21. Dudak SD et al: Transitional cell carcinoma in a duplicated
upper pole nephrectomy ectopic ureter. Urology. 46(2):251-3, 1995
o Single system: Nephrectomy if minimal function 22. Jelen Z: The value of ultrasonography as a screening
o If renal function preserved or dx made prenatally: procedure of the neonatal urinary tract: a survey of 1021
Ureteropyelostomy or common sheath ureteral infants. Int Urol Nephrol. 25(1):3-10, 1993
implantation 23. Herman TE et al: Radiographic manifestations of
congenital anomalies of the lower urinary tract. Radiol
Clin North Am. 29(2):365-82, 1991
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
• Weigert-Meyer law: Upper moiety ureter inserts
inferior and medial to lower moiety ureter

I SELECTED REFERENCES
L Wille S et al: Magnetic resonance urography in pediatric
urology. Scand] Urol Nephrol. 37(1):16-21, 2003
2. Berrocal T et al: Anomalies of the distal ureter, bladder, and
urethra in children: embryologic, radiologic, and
pathologic features. Radiographies. 22(5):1139-64, 2002
3. Riccabona M et al: Feasibility of MR urography in neonates
and infants with anomalies of the upper urinary tract. Eur
Radiol. 12(6):1442-50, 2002
4. Oge 0 et al: Nephron-sparing surgery in a duplex system
associated with a vaginal ectopic ureter. Pediatr Nephrol.
16(12):1135-6, 2001
 ECTOPIC URETER

I IMAGE GALLERY

(Left) Sagittal US shows

tortuous dilated ureter
(arrows) continuing beyond
the expected location for the
UV}. (Right) Sagittal US
shows cystic mass in vagina
(arrow), representing
insertion of dilated ectopic
ureter (Courtesy FC
Sommer, MO).

4
Variant 7
(Left) Coronal T2WI MR
shows duplicated left
collecting system with
hydronephrotic upper pole
and mild lower pole
dilatation (Courtesy FC
Sommer, MO). (Right)
Coronal T2WI MR slightly
more anterior to previous
image shows markedly
dilated, tortuous ectopic
ureter (Courtesy FC
Sommer, MO).

(Left) Voiding
cystouretrethrogram shows
ectopic left ureter draining
into urethra (Courtesy C.
Friedland, MO). (Right)
Sagittal US shows dilated
ectopic ureter (open arrows)
deep to kidney and cystic
dysplasia of upper pole
moiety (arrow) (Courtesy FC
Sommer, MO).
 URETEROCELE

Drawing shows simple ureterocele, with prolapsing, IV? shows bilateral simple ureteroceles, with cystic
dilated end of orthotopic ureter. dilatation of intramural portions of orthotopic ureters
and surrounding radiolucent halos (arrows) (Courtesy
4 M. Nino-Murcia, MD).

8 o Orthotopic ureterocele: Cobrahead deformity

ITERMINOLOGY • Dilated distal ureter projecting into lumen of
Abbreviations and Synonyms bladder with surrounding radiolucent halo
o Ectopic ureterocele: Smooth, radiolucent
• Simple = orthotopic = adult-type ureterocele
intravesicular mass near bladder base
Definitions • May evert during voiding and mimic diverticulum
• Cystic dilatation of distal ureter with prolapse into • Lumen opacification depends on function of
bladder lumen upper pole moiety
• Orthotopic ureterocele: Normal insertion at trigone • Drooping lily sign: Displacement of lower pole
o Orthotopic: Single ureter systems collecting system by obstructed upper pole moiety
• Ectopic ureterocele: Inserts below trigone CT Findings
o Ectopic: Duplicated systems in 80%
• CECT: Intravesicular mass at UVJ
MR Findings
I IMAGING FINDINGS • T2WI
o Intravesicular mass at UVJ
General Features • Ectopic: May see ectopic insertion into urethra,
• Best diagnostic clue: Orthotopic: "Cobra-head" or vagina, etc.
"spring onion" deformity of distal ureter with
surrounding radiolucent halo Ultrasonographic Findings
• Location • Real Time: Thin walled, cystic intravesical mass near
o Ectopic: 1/2 in bladder and 1h in posterior urethra; ipsilateral ureter
10% bilateral
• Males: Insertion always above external sphincter Imaging Recommendations
• Size: Up to several cm in diameter • Best imaging tool: Excretory urography
• Morphology: Smooth, round or ovoid • Protocol advice: Cystogram: Get early images of
bladder filling; overfilling may collapse low-pressure
Radiographic Findings ureterocele
• IVP/cystography

DDx: Ureterocele Mimics

Bladder Cancer Gartner Duct Cyst Bladder Cancer

 URETEROCELE
Key Facts
Terminology
• Cystic dilatation of distal ureter with prolapse into
bladder lumen
• Orthotopic ureterocele: Normal insertion at trigone
• Orthotopic: Single ureter systems
• Ectopic ureterocele: Inserts below trigone
• Ectopic: Duplicated systems in 80%
I DIFFERENTIAL DIAGNOSIS
Pseudoureteroceles
• Bladder or cervical cancer invading UVJ
• Radiation cystitis
• Distal UVJ stone or edema 2° to recent stone passage
Prolapsing ureterocele: Vaginal mass in girls
• Rhabdomyosarcoma: Grape-like clustered mass
• Gartner duct cyst: Cyst in vaginal wall
I PATHOLOGY
General Features
• Etiology: Congenital anomaly
• Epidemiology: US: 1 in 12,000 to 1 in 5,000
• Associated abnormalities: Single system ectopic
ureteroceles: Cardiac and genital anomalies
Gross Pathologic & Surgical Features
• Simple ureteroceles: Pin-point orifices but no
significant obstruction
• Ectopic ureteroceles: Often obstructed, with dysplasia
of upper pole kidney
Microscopic Features
• Wall: Covered by bladder mucosa and lined by
ureteral mucosa
Staging, Grading or Classification Criteria
• Orifice type: Stenotic, sphincteric, sphincterostenotic,
cecoureterocele
o Sphincteric: Orifice distal to bladder neck
o Cecoureterocele: Intravesical orifice; submucosal
extension to urethra
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Orthotopic: Usually asymptomatic; incidental
finding
o Ectopic ureteroceles: Urinary tract infection (UTI),
incontinence, vaginal mass
• Clinical profile: Ectopic: Infant or child with UTI or
sepsis
Demographics
• Age
Imaging Findings
• Best diagnostic clue: Orthotopic: "Cobra-head" or
"spring onion" deformity of distal ureter with
surrounding radiolucent halo
• Ectopic ureterocele: Smooth, radiolucent
intravesicular mass near bladder base
• May evert during voiding and mimic diverticulum
• Real Time: Thin walled, cystic intravesical mass near
ipsilateral ureter
o Ectopic: Median age 3 months at diagnosis
• Often diagnosed with prenatal ultrasound
• Gender: Ectopic ureterocele with duplicated system:
M:F = 1:4
• Ethnicity: Almost exclusively Caucasians
Natural History & Prognosis
• Severe obstruction: Primarily ectopic ureteroceles
o Dysplasia of obstructed upper pole moiety
4
9
Treatment
• Options, risks, complications: Obstructed ureteroceles
may cause stasis and stone formation
I DIAGNOSTIC CHECKLIST
Consider
• Check obliques for mucosal irregularity to rule out
tumor
Image Interpretation Pearls
• Long axis of ectopic ureterocele points to side of origin
I SELECTED REFERENCES
1. Walsh PC et al: Campbell's Urology. 8th ed. Philadelphia,
Saunders. 2007-52, 2002
2. Davidson AJ et al: Radiology of the kidney and
genitourinary tract. 3rd ed. Philadelphia, W.B. Saunders.
213-6, 1999
I IMAGE GALLERY
(Left) Axial CECT shows large left orthotopic ureterocele (arrow).
(Right) IVP shows complete left system duplication and ectopic
ureterocele at bladder base (arrow). Filling defects in dilated ureter
(open arrows) represent ureteritis cystica (Courtesy M. Nino-Murcia,
MO).
 URETERITIS CYSTICA

IV? shows tiny smooth, round filling defects (arrow) in IV? shows image of left ureter in the same patient, with
right ureter in patient with ureteritis cystica (Courtesy M. several tiny nodular filling defects (arrows) represendng
Nino-Murcia, MO). submucosal cysts of ureteritis cystica (Courtesy M.
4 Nino-Murcia, MO).

10
ITERMINOlOGY Radiographic Findings
• IVP
Abbreviations and Synonyms o Nodular radiolucent small filling defects in ureter
• Ureteritis cystica lumen
• Pyeloureteritis cystica if renal pelvis involved o In profile: Scalloping of ureteral margins
Definitions o May have ragged appearance of ureter wall from
confluent groups of tiny cysts
• Multiple small subepithelial cysts in ureter wall
• Retrograde pyelogram: Tiny radiolucent filling defects
MR Findings
I IMAGING FINDINGS • T2WI: Beading of ureter wall on MR urography
General Features Imaging Recommendations
• Best diagnostic clue: Multiple small mural-based filling • Best imaging tool: IVP or retrograde pyelography
defects in ureter lumen • Protocol advice: Oblique films helpful to show mural
• Location location and smooth surface of tiny cysts
o Proximal 1/3 of ureter
o May also occur in renal pelvis (pyelitis cystica) and
urinary bladder (cystitis cystica) I DIFFERENTIAL DIAGNOSIS
o May be unilateral or bilateral, symmetric or
asymmetric Air bubbles
• Size • Should move; do not elevate uroepithelium
o Cysts 2-3 mm in size; usually < 5 mm
o Occasionally may be as large as 2 cm Multifocal transitional cell carcinoma (TCCA)
• Morphology • Usually have more irregular surface
o Smooth, spherical radiolucent filling defects • Not as numerous as ureteritis cystica
o May have few cysts or dozens Metastatic melanoma
o Lesions may be isolated or clustered in groups
• Usually larger, fewer nodules

DDx: Ureteral Filling Defects

rr. ,
Melanoma
l Muttifocal TCCA Melanoma
 URETERITIS CYSTICA
Key Facts
Terminology Top Differential Diagnoses
• Multiple small subepithelial cysts in ureter wall • Air bubbles
• Multifocal transitional cell carcinoma (TCCA)
Imaging Findings
• Metastatic melanoma
• May also occur in renal pelvis (pyelitis cystica) and
urinary bladder (cystitis cystica) Pathology
• May be unilateral or bilateral, symmetric or • Relatively rare reactive hyperplastic lesion of
asymmetric uroepithelium
• Cysts 2-3 mm in size; usually < 5 mm • Not premalignant or neoplastic

• Clinical profile: Older patient with chronic UTI

Crossing vessels
• Usually not spherical; filling defects not as numerous Demographics
• Age: All ages, most common in ages 50-60
Blood clots, radiolucent stones
• Gender: M < F
• Fewer, larger, mobile
Natural History & Prognosis

I PATHOLOGY
• Usually inconsequential; should rule out UTI
• No risk of malignant degeneration
4
General Features Treatment 11

• General path comments • Options, risks, complications: Treat any concomitant

o Relatively rare reactive hyperplastic lesion of pathology such as stones or UTI
uroepithelium
o Not premalignant or neoplastic
• Etiology I DIAGNOSTIC CHECKLIST
o May be response to chronic urinary tract infection
(UTI) Image Interpretation Pearls
o May persist even after infection resolves; can occur • Ureteritis cystica does not cause obstruction
without UTI
o Theory of pathogenesis: Von Brunn cell nest
• Inflammatory stimulation ~ proliferation of I SELECTED REFERENCES
urothelium ~ "cell nest" shifting mucosa inward 1. Wang IK et al: Pyeloureteritis cystica: case report. Chang
• Cells become isolated within tunica propria ~ Gung MedJ. 24(1):62-7, 2001
metaplastic transformation into glandular 2. Menendez V et al: Cystic pyeloureteritis: review of 34 cases.
structures ~ fluid-filled cysts Radiologic aspects and differential diagnosis. Urology.
o Can be seen with bacterial infection and 50(1):31-7, 1997
schistosomiasis 3. Dunnick NR et al: Textbook of uroradiology. 2nd ed.
Baltimore, Williams and Wilkins, 381-2, 1997
• Ureteritis cystica calcinosa: Punctate calcifications 4. Noda S et al: Histopathological studies on the cystic
• Associated abnormalities: Calculi, urinary tract formation of the human urothelium. Kurume Med J.
infections (UTIs) 37(2):55-65, 1990
Gross Pathologic & Surgical Features
• Smooth, round, grayish subepithelial nodules
containing translucent or yellowish fluid I IMAGE GALLERY
• Cyst fluid may be thin or thick
• Fluid may be hemorrhagic
Microscopic Features
• Tiny cysts in ureter wall lined with epithelial cells

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Usually asymptomatic
o Other signs/symptoms
• Hematuria from UTI, cyst rupture or coexistent (Left) IV? shows duplicated collecting system with ureteritis cystica
stones (arrows) (Courtesy M. Nino-Murcia, MO). (Right) Retrograde
• UTI symptoms pyelogram shows mural-based nodular filling defects (arrows) in
distal ureter.
 SECTION 5: Bladder

Introduction and Overview

Bladder Anatomy and Imaging Issues 11I-5-2

Congenital
Urachal Remnant 11I-5-6

Infection
Cystitis 11I-5-8

Inflammation
Bladder Calculi 11I-5-10
Bladder Diverticulum 11I-5-12
Bladder Fistulas 11I-5-14
Neurogenic Bladder 11I-5-16

Trauma
Bladder Trauma 11I-5-18

Neoplasm
Bladder Carcinoma 11I-5-22
 BLADDER ANATOMY AND IMAGING ISSUES
Graphic shows staging system for bladder carcinoma,
based on depth of invasion of bladder wall. T3 tumor
involves the perivesicle fat.
I IMAGING ANATOMY
• Bladder and ureters are lined by transitional
epithelium
5 o Can undergo metaplasia to squamous epithelium in
response to chronic inflammation
2 • Bladder is predominately a pelvic, extraperitoneal
organ
o Dome is covered by peritoneum, prone to
intraperitoneal rupture when distended and
subjected to blunt trauma
o Extraperitoneal space between anterior bladder wall
and the symphysis pubis is the anterior perivesical
space (of Retzius)
I ANATOMY-BASED IMAGING ISSUES I
Key Concepts or Questions
• Ureteral duplication
o Can be complete (due to second ureteric bud from
mesonephric duct)
o Can be incomplete (branching of a single ureteric
bud)
o Weigert-Meyer rule
• Upper moiety ureter inserts inferior and medial to
the ureter draining lower moiety
• Upper pole ureter inserts below the trigone into
any Woltfian duct derivative (e.g., vagina,
prostate)
o Complications
• Vesicoureteral reflux (most common)
• Ureteral obstruction
• Enuresis (only in females; insertion always above
external sphincter in males)
• Obstruction: Upper pole ureter often obstructed
(ectopic ureterocele, etc.); often non-functional
upper renal pole at time of diagnosis
o Diagnosis
• "Drooping lily sign" on urography
Axial CECT shows multiple bladder diverticula, one of
which contains a solid mass; transitional cell carcinoma.
• Hydronephrosis and decreased function of upper
pole displace the lower pole calices
• Both ureters evident on CT
• Upper pole ureter often dilated and unopacified
•
on CECT
Retrocaval ureter
o Congenital anomaly of lVC
• Persistent sub cardinal vein "traps" ureter
o Appearance: Proximal ureter swings medially over
the pedicle of L3 or 4 vertebra, behind lVC, then
returns to normal position
o Causes variable degree of hydronephrosis
• Primary megaureter (megaloureter)
o "Achalasia" of ureter
o Congential dilation of lower ureter (occasionally,
entire collecting system)
o Due to faulty development of muscle layers of ureter
• (Not obstruction)
o Can be associated with other renal and ureteral
anomalies
• (Duplication, ectopia, ureterocele)
o May be asymptomatic or associated with pain,
infection
• Ureteropelvic junction obstruction
o Most common cause of neonatal hydronephrosis
o Commonly encountered in adults also
o Sharp transition from dilated pelvis to normal ureter
o May be congenital or acquired
• Hydronephrosis (obstructive uropathy)
o Dilation of collecting structures without functional
impairment
o Obstructive nephropathy = dilation + impairment
o Degree of dilation depends on
• Duration of obstruction
• Renal output, hydration
• Presence of spontaneous decompression
o Acute causes
• Ureteral calculus
• Passage of blood clot (e.g., tumor, trauma)
• Surgical mishap (e.g., ligation)
• Ureteral edema following instrumentation
 BLADDER ANATOMY AND IMAGING ISSUES

DIFFERENTIAL DIAGNOSIS
Hydronephrosis on Sonography Ureteropelvic junction obstruction (UPJ)
• Congenital
False negatives • Eosinophilic ureteritis
• Staghorn calculus filling lumen • Ischemia
• Very acute obstruction • Extrinsic
• Spontaneous decompression (e.g., forniceal rupture) • ~ Crossing vessels
• Dehydration • ~ Adventitial bands
False positives • ~ Renal mass
• Distended bladder • ~ Aortic aneurysm
• Overhydration Urinary bladder tumors (malignant)
• Prior obstruction • Transitional cell carcinoma (90%)
• Vesico-ureteral reflux • Squamous cell cancer (chronic inflammation)
• (Parapelvic cyst) • Adenocarcinoma (urachal)
• (Renal sinus vessels) • Rhabdomyosarcoma (childhood)
• (Prominent extrarenal pelvis) • Lymphoma

• Sulfonamide crystallization in urine • Neurogenic bladder

• Pregnancy
• Emphysematous pyelitis
o Gas is confined to collecting system (not renal
parenchyma)
o Usually in diabetes with coliform bacterial infection
o Better prognosis than emphysematous
pyelonephritis
• Pyeloureteritis cystica
o Hyperplastic proliferation of transitional epithelium
projecting into ureteral lumen
o Caused by chronic urinary infection +/or
inflammation
o More common in diabetes, women
o Affects bladder more than ureter
o Appearance: Multiple small (1-3 mm) lucent detects
in ureteral or bladder lumen
o Can simulate or lead to transitional cell carcinoma
• Ureteral calculi (urolithiasis)
o Affects 12% of population by age 70
• 2 to 3% will have an attack of renal colic
• Peak age: 20-30
o Most patients have a specific metabolic disorder
• Hypercalcemia (e.g., sarcoidosis, Cushing
syndrome, hyperparathyroidism)
• Excess dietary calcium or sodium
• Bowel disease
• Gout
• Oxalosis
o Patients with Crohn disease or other cause of ileal
resection have excess oxalate resorption
o Patients with urinary tract infection with
urea-splitting organisms can get "triple phosphate"
(struvite) stones
• Especially common in patients with neurogenic
bladder, indwelling catheter
• Can lead to staghorn calculi
o Diagnosis
• CT is the most accurate and simple
• No need to document every instance of renal colic
with CT (many patients are young, will have
many stones and will form more; danger of
excessive radiation)
o Denervation or neurological disorder of the bladder
o "Spastic bladder"
• Lesion of "upper motor neuron" (above conus)
o Atonic bladder
• "Lower motor neuron" lesion (below conus)
• Resembles a pine cone or Christmas tree on
cystography
5
o Etiology 3
• Congenital (e.g., myelomeningocele)
• Trauma
• Neoplasm (spinal or brain)
• Infection (polio)
• Inflammation (multiple sclerosis, syrinx)
• Systemic disorder (e.g., diabetes)
• Urachal Anomalies
o Urachus is the remnant of the allantois
o Should regress to a cord (median umbilical
ligament) in fetal stage
o Variations
• Patent urachus - fistula from bladder to umbilicus
(urine draining from umbilicus)
• Urachal sinus - patent only at umbilicus
• Urachal diverticulum - communicates only with
bladder dome
• Urachal cyst - closed at both ends; most common
in adults, symptomatic midline cystic mass
between umbilicus and bladder; symptoms due to
enlargement or infection; can undergo malignant
degeneration (usually adenocarcinoma)
• Schistosomiasis (Bilharziasis)
o S. hematobium affects the urinary system
o Parasitic infection transmitted through fresh water
snails
• Usually invades humans through feet in contact
with contaminated water
• Passes through lymphatics to vessels
o Endemic in Africa and Puerto Rico
o Appearance
• Bladder wall calcification
• Ureteral, seminal vesicle calcification
• Ureteral strictures
• Bladder calculi
 BLADDER ANATOMY AND IMAGING ISSUES

Urogram shows "drooping lily" sign; left renal calices Axial CECT shows hydronephrotic upper pole calices
displaced downward by non-functioning and ureter (arrow), normal lower pole and ureter;
hydronephrotic duplicated upper pole . ureteral duplication with obstructed upper ureter.

• Bladder diverticulum • Key findings: Imaging evidence of gas, stool,

o Usually associated with bladder outlet obstruction enteric contrast in bladder (best shown by CT; also
• E.g., prostate enlargement; neurogenic bladder shows associated findings of diverticulitis, tumor,
• Often multiple etc.)
5 • Can be source of urinary stasis, calculi, infection,
tumor
4 o Can be congenital I CUSTOM DIFFERENTIAL DIAGNOSISI
• Hutch diverticulum (above and lateral to ureteral
orifice) Focal hydronephrosis
o Can be simulated by surgical augmentation of • Congenital: Duplicated system with obstruction
bladder (with bowel) or by herniation of bladder o Usually upper pole
into inguinal hernia ("bladder ears", most common • Infection
in male infants) o Usually TB; stricture of infundibulum
• Bladder cancer o Infundibular calculus
o Transitional cell cancer = 85 to 90% o Tumor
o Epidemiology: M:F = 3:1; mean age 64 • Transitional cell carcinoma
o Risk factors
Radiolucent filling defects in renal pelvis
• Chemical exposure (e.g., aniline dye)
• Calculus
• Tobacco
o Many are nonopaque on radiography
• Cyclophosphamide therapy
o Only matrix and indinavir stones are nonopaque on
• Analgesic abuse
• Recurrent or chronic infection
CT
o Staging accuracy • Blood clot
• Clinical (50%) • Fungus ball or pus
• Tumor (TCC or RCC)
• CT (30 to 80%)
• Sloughed tissue (papillary necrosis)
• MR (70%)
o Cytoscopy essential for diagnosis, staging • Gas (e.g., iatrogenic; pyelitis cystica)
o T1: Limited to submucosa • Foreign body
o T2 : Into superficial muscle
o T3: Into deep muscle
o T3b: Into perivesicle fat I SELECTED REFERENCES
o T4: Into adjacent organs or distant metastases 1. Yu JS et al: Urachal remnant diseases: spectrum of CT and
• Bladder fistulas US findings. Radiographies. 21(2):451-61, 2001
o Can be caused by trauma, tumor, infection, surgery 2. Morgan DE et al: CT cystography: radiographic and clinical
predictors of bladder rupture. AJRAm J Roentgenol.
or instrumentation of bladder or any adjacent organ
174(1):89-95, 2000
o Colovesicle fistula is most common 3. Kim B et al: Bladder tumor staging: comparison of
• Sigmoid diverticulitis most common etiology; contrast-enhanced CT, T1- and T2-weighted MR imaging,
tumor second dynamic gadolinium-enhanced imaging, and late
• Symptoms: Dysuria, urinary infection, fecaluria gadolinium-enhanced imaging. Radiology. 193(1):239-45,
1994
 BLADDER ANATOMY AND IMAGING ISSUES
IMAGE GALLERY

(Left) Urogram shows

congenital primary
mega ureter, simulating
obstruction. (Right) CECT,
MIP coronal reformation
shows dilated renal pelvis,
ureteral stent, and accessory
renal artery (arrow) crossing
the UP}.

Typical
(Left) Axial NECT shows
median umbilical ligament
containing gas bubble
(arrow), with ascites on both
sides. (Right) Axial NECT
shows triangular shaped
5
bladder due to urachal
5
diverticulum.

Typical
(Left) Axial CECT shows
extensive pre-sacral soft
tissue following surgery and
radiation therapy for cervical
carcinoma. (Right) Axial
CECT shows gas and feces in
vagina (arrow), and bladder
(open arrow), due to fistulas
to rectum.
 URACHAL REMNANT

Drawing shows tubular channel extending from the Cystogram shows tubular structure (arrow) arising from
dome of bladder along the anterior abdominal wall in bladder dome and extending to umbilicus, representing
the midline toward the umbilicus, representing a urachal patent urachus (Courtesy G. Friedland, MD).
diverticulum.

ITERMINOLOGY • Size
o Urachus: 5-6 cm in length
Abbreviations and Synonyms o Urachal diverticulum: Small or large
• Vestigial, obliterated urachus = median umbilical • Morphology: Urachal cyst: May be single or multiple
5 ligament Radiographic Findings
Definitions • Radiography: Urachal Ca: Stippled, granular or
6
• Urachal abnormalities: Incomplete obliteration of curvilinear calcifications above bladder outline
embryonic connection between bladder dome and • Fluoroscopy
allantoic duct o Sinogram: Tract extending inferiorly from umbilicus
• Patent urachus: Entire urachal channel fails to close o Voiding cystourethrogram: Tubular channel arising
• Urachal cyst: Umbilical and bladder openings close; from bladder dome
channel in between remains open and fluid-filled CT Findings
• Urachal sinus: Dilatation of urachus at umbilical end;
• CECT
no communication with bladder o Urachal diverticulum: Midline cystic lesion at
• Urachal diverticulum: Dilatation of urachus at vesical anterosuperior aspect of bladder
end; no communication with umbilicus o Urachal cyst: Midline cyst above bladder dome
• Urachal carcinoma (Ca): Adenocarcinoma (90%) • May have rim calcification
arising from urachus • Low density contents due to mucin production
• No soft tissue component unless complicated
o Urachal carcinoma: Midline supravesical soft tissue
I IMAGING FINDINGS mass; calcification in 70%
General Features Ultrasonographic Findings
• Best diagnostic clue: Cyst or mass between bladder and • Real Time
umbilicus o Urachal cyst: Midline cyst above bladder
• Location: Between peritoneum and transversalis fascia o Complicated urachal cyst: Heterogeneous
(space of Retzius) echogenicity

DDx: Mimics of Urachal Carcinoma

Bladder Ca Gastric Ca Met Bladder Ca

 URACHAL REMNANT
Key Facts
Terminology • Urachal diverticulum: Dilatation of urachus at vesical
• Urachal abnormalities: Incomplete obliteration of end; no communication with umbilicus
embryonic connection between bladder dome and Imaging Findings
allantoic duct
• Urachal carcinoma: Midline supravesical soft tissue
• Patent urachus: Entire urachal channel fails to close mass; calcification in 70%
• Urachal cyst: Umbilical and bladder openings close;
channel in between remains open and fluid-filled Clinical Issues
• Urachal sinus: Dilatation of urachus at umbilical end; • Urachal carcinoma: 5 year survival :s; 15%
no communication with bladder

o Urachal carcinoma: 40-70

Imaging Recommendations
• Gender: M:F = 3:1
• Best imaging tool: CT
Natural History & Prognosis
• Urachal carcinoma: 5 year survival :s; 15%
I DIFFERENTIAL DIAGNOSIS • Poor prognosis related to late presentation

Cystic lesions near bladder/umbilicus Treatment

• Vitelline cyst, mesenteric cyst • Options, risks, complications
o Complications: Adenocarcinoma in urachal remnant
Urachal carcinoma: Differential diagnosis o Stone formation in urachal diverticulum
• Primary bladder carcinoma at dome
• Metastasis (met) to bladder from rectal, stomach,
ovarian or other adenoca I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
5
I PATHOLOGY • Look for center of mass; if outside bladder, consider 7
urachal CA with bladder invasion
General Features
• Etiology: Congenital anomaly
• Epidemiology I SELECTED REFERENCES
o Patent urachus: Rare; prevalence 0.25-15 per 10,000
1. Yu JS et al: Urachal remnant diseases: spectrum of CT and
o Urachal carcinoma: 0.2-0.3% of all bladder cancers; US findings. Radiographies. 21(2):451-61, 2001
20-40% of primary bladder adenocarcinomas 2. Davidson AJ et al: Radiology of the kidney and
• Associated abnormalities: Patent urachus: Congenital genitourinary tract. 3rd ed. Philadelphia, W.B. Saunders.
lower urinary tract obstruction: Posterior urethral 213-16, 1999
values, prune belly syndrome, urethral atresia 3. Dunnick NR et al: Textbook of uroradiology. 2nd ed.
Baltimore, Williams and Wilkins. 29-33, 1997
Gross Pathologic & Surgical Features
• Urachal carcinoma: Frequently contains calcium;
invades bladder dome I IMAGE GALLERY
Microscopic Features
• Urachal anomalies: Mucosal lining of transitional
epithelium
• Urachal carcinoma: 85-90% are adenocarcinomas
resulting from metaplasia of transitional epithelium

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Patent urachus: Urine leakage from umbilicus
o Complicated urachal cyst: Suprapubic pain, fever
(Left) Sagittal US shows tubular anechoic channel (arrows)
o Urachal carcinoma: Clinically silent until bladder
connecting umbilicus to bladder (open arrow) representing patent
dome invaded; then get hematuria and/or mucoid urachus. (Right) Axial CECT shows exophytic mass (arrow) with
discharge coarse internal calcification invading bladder dome, representing
urachal adenocarcinoma.
Demographics
• Age
o Patent urachus: Diagnosed at birth
 CYSTITIS

Axial CECT shows thick-walled, irregular bladder IVP shows "cobblestone" appearance of bladder
representing radiation cystitis in patient who underwent mucosa in patient with cystitis secondary to perforated
radiation therapy for rectal cancer. sigmoid diverticulitis (Courtesy M. Nino-Murcia, MOJ.

o Bladder wall thickening +/- hypo dense wall

ITERMINOlOGY o Emphysematous cystitis: Gas in bladder wall and/or
Definitions lumen
• Inflammation of the urinary bladder wall Ultrasonographic Findings
5 • Acute cystitis: Recent onset, short duration • Real Time: Hypoechoic edematous bladder wall
• Chronic cystitis: Longer duration (months-years)
8 o Variants: Cystitis cystica, cystitis glandularis
I DIFFERENTIAL DIAGNOSIS
I IMAGING FINDINGS Thickened bladder wall: Nondistention,
General Features trabeculation, infiltrating Ca
• Location: Usually involves entire bladder wall, but can • Indistinguishable by imaging alone
be focal
• Morphology: Chronic cystitis: Contracted, nodular
bladder wall I PATHOLOGY
Radiographic Findings General Features
• Radiography: TB, schistosomiasis: Bladder wall • General path comments: Schistosomiasis is most
calcification frequent cause of bladder wall calcification
• IVP • Etiology
o Bladder often normal in acute cystitis o Infectious cystitis
o Acute: Thickened, coarse mucosal folds with • Bacterial: Transurethral invasion of bladder by
cobblestone appearance perineal flora in sexually active women
o Acute: Bullous edema of bladder mucosa • Bacterial: Bladder outlet obstruction and urinary
o Chronic: Contracted, irregular thick-walled bladder stasis in men
• Schistosomiasis: Inflammatory response to ova
CT Findings deposited in bladder submucosa
• CECT

DDx: Bladder Wall Thickening

'.,
..f...~··
..,'E ti

.c -
•.•. ...•. , ~
,

i'. 1/ ;",

'1 'j
'I.

..
I

'. \;.:j ••
Trabeculation Non-Distention Melanoma
 CYSTITIS
Key Facts
Imaging Findings Top Differential Diagnoses
• Acute: Bullous edema of bladder mucosa • Thickened bladder wall: Nondistention,
• Chronic: Contracted, irregular thick-walled bladder trabeculation, infiltrating Ca
• Bladder wall thickening +/- hypodense wall
• Emphysematous cystitis: Gas in bladder wall and/or Pathology
lumen • Bacterial: Transurethral invasion of bladder by
perineal flora in sexually active women
• Bacterial: Bladder outlet obstruction and urinary
stasis in men

• Tuberculosis: Descending infection from kidney • Fluid accumulation ~ pseudocysts = cystitis

• Emphysematous: E. coli, Aerobacter aerogenes or cystica
Candida infection • Transformation into glands ~ cystitis glandularis
• Alkaline encrustation: Infection by urea-splitting o Squamous metaplasia: Transformation of
organism ~ alkaline urine + focal necrosis ~ urothelium into keratin producing squamous cells
dystrophic calcification • May see white patches (leukoplakia) on foci of
• Others: Viral, fungal (Candida) squamous metaplasia
o Noninfectious cystitis o Schistosomiasis: Bladder carcinoma, especially
• Mechanical: Local irritation from Foley catheter, squamous
stone, foreign body, etc
• Drug-related: Cyclophosphamide (hemorrhagic
cystitis) I DIAGNOSTIC CHECKLIST
• Radiation-induced
Consider
• Idiopathic: Interstitial cystitis = pancystitis causing
severe urgency and frequency • Nodular bladder wall thickening in cystitis may mimic 5
• Associated abnormalities bladder carcinoma
9
o Chronic cystitis: Decreased bladder capacity and
vesicoureteral reflux
Image Interpretation Pearls
o Recurrent bacterial infection: Malakoplakia • Schistosomiasis progresses proximally; TB progresses
• Associated with E. coli infection distally
• Granulomatous inflammatory process • Gas in bladder wall always secondary to infection
• Caused by deficient function of lysosomes in
macrophages
I SELECTED REFERENCES
Gross Pathologic & Surgical Features 1. Ravakhah K et al: Images in clinical medicine. Unusual
• Ulceration, petechiae, erythema of bladder mucosa cystitis. N Engl J Med. 350(22):2280, 2004
• Chronic: Fibrosis and contraction of bladder wall 2. Zagoria R: Genitourinary radiology, 2nd ed. Philadelphia,
Mosby Ine. 211-29, 2004
3. Thoumas 0 et al: Imaging characteristics of
I CLINICAL ISSUES alkaline-encrusted cystitis and pyelitis. AJR Am J
Roentgenol. 178(2):389-92,2002
Presentation
• Most common signs/symptoms
o Dysuria, frequency, urgency I IMAGE GALLERY

r .. ~ ·
o Other signs/symptoms
• Gross hematuria, pyuria, bacteriuria
Demographics
• Gender: Bacterial cystitis: M < F due to short urethra
Natural History & Prognosis

•
• Emphysematous cystitis: Responds well to antibiotics;
not life-threatening
Treatment
• Options, risks, complications: Acute: Usually responds
to antibiotics
• Complications of chronic cystitis
(Left) IVP shows small capacity bladder with nodular wall in patient
o Hyperplastic uroepithelial cell clusters (Brunn nests)
with cystitis cystica (Courtesy G. Friedland, MO). (Right)
form in bladder submucosa Radiography shows crescent of gas in suprapubic location
representing gas in the bladder wall in a patient with emphysematous
cystitis (Courtesy M. Nino-Murcia, MO).
 BLADDER CALCULI

Pelvic radiograph shows smooth, round, lamillated Axial CECT shows lobulated large stone in bladder in a
calcification overlying bladder typical of a bladder male patient with a history of prostatic enlargement.
calculus.

• IVP: Filling defect or radiopacity, depending on

ITERMINOlOGY relative density of stone versus contrast material
Abbreviations and Synonyms CT Findings
• Bladder stones, vesical calculi, cystolithiasis
5 Definitions
• NECT: All bladder calculi radiopaque on CT
MR Findings
10 • Concretions of mineral salts within bladder lumen • All pulse sequences: Signal void(s) in bladder
Ultrasonographic Findings
IIMAGING FINDINGS • Real Time: Mobile echogenic foci in bladder with
acoustic shadowing
General Features
• Best diagnostic clue: Smooth round or ovoid Imaging Recommendations
laminated calcification in bladder • Best imaging tool: CT
• Location • Protocol advice: Low kilovoltage (60-70 kV) best for
o Bladder lumen: Usually midline with patient supine detecting stones on plain film
• Eccentric if within bladder augmentation or
diverticulum
• Size: Variable I DIFFERENTIAL DIAGNOSIS
• Morphology
o Round, oval, spiculated, laminated, faceted Other bladder calcifications
• Stones with multiple spicules: "]ackstones" • Urachal carcinoma: Punctate or coarse calcification in
• Stones with lobulated margins: "Mulberry stones" mass at bladder dome
• Schistosomiasis: Calcification in bladder wall
Radiographic Findings
• Radiography Other pelvic calcifications
o Solitary or multiple calcifications overlying bladder • Prostate calcification, calcified fibroids
o Most are radiopaque but opacity variable

DDx: Calcified Pelvic Masses

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'~ ...
.'
;."C ..... ~~-i~
,~
~
'It>

.. t'

Calcified Fibroid
)

Dermoid
v Urachal Carcinoma
 BLADDER CALCULI

Key Facts
Imaging Findings
• Round, oval, spiculated, laminated, faceted
• Most are radiopaque but opacity variable
• NECT: All bladder calculi radiopaque on CT
Top Differential Diagnoses
• Other bladder calcifications
• Other pelvic calcifications
Pathology
• Stasis: Bladder outlet obstruction, neurogenic bladder,
bladder diverticula
• Infection, especially Proteus mirabilis
• Renal calculus passing into bladder
• Suture material, migrated IUDs or other gyn
applicances
• Bladder augmentation: Local metabolic derangement
• Nutritional deficiencies in third world children

Filling defect in bladder Natural History & Prognosis

• Blood clot, fungus ball, ureterocele, bladder neoplasm • Complication: Malignant bladder tumors in patients
with stones from indwelling Foley catheters

I PATHOLOGY Treatment
• Options, risks, complications
General Features o Prevention and eradication of underlying cause
• General path comments o Cystoscopic fragmentation and removal
o Most are mixture of calcium oxalate and calcium o Open cystolithotomy only for very large or very
phosphate hard stones
o Infection stones: Magnesium ammonium phosphate
("struvite")
• Etiology I DIAGNOSTIC CHECKLIST
o Stasis: Bladder outlet obstruction, neurogenic
Consider
5
bladder, bladder diverticula
o Infection, especially Proteus mirabilis • Obstruction accounts for 70% of adult bladder stones 11
o Foreign bodies: Nidus for crystal growth Image Interpretation Pearls
• Renal calculus passing into bladder
• Carcinoma resulting from chronic bladder irritation
• Suture material, migrated IUDs or other gyn
may co-exist with bladder stone
applicances
• Pubic hairs introduced by catheterization
o Bladder augmentation: Local metabolic
derangement
I SELECTED REFERENCES
o Nutritional deficiencies in third world children 1. Ord J et al: Bladder management and risk of bladder stone
• Epidemiology formation in spinal cord injured patients. JUral.
170(5):1734-7,2003
o 5% of all urinary tract calculi in Western world
2. Schwartz BF et al: The vesical calculus. Ural Clin North
o Incidence declining in West over past 100 years Am. 27(2):333-46, 2000
• Upper tract> lower tract stones in developed 3. Dyer RBet-al: Abnormal calcifications in the urinary tract.
countries Radiographies. 18(6):1405-24, 1998
o Idiopathic: Endemic in underdeveloped areas,
especially in children
I IMAGE GALLERY
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Most asymptomatic
o Other signs/symptoms
• Suprapubic pain, microhematuria
• Gross hematuria is rare
• Clinical profile
o Spinal cord injury patient with indwelling Foley
o Elderly male with bladder outlet obstruction
o Older woman with pelvic prolapse
o Bladder augmentation with ileal or colonic segments (Left) Axial oblique US shows large echogenic focus (arrow) with
posterior acoustic shadowing representing a large bladder calculus.
Demographics (Right) Axial T1WI MR image shows innumerable round foci of low
• Gender: M > F signal (arrows) in the dependent portion of the bladder representing
calculi in a man with marked prostatic enlargement.
 BLADDER DIVERTICULUM

Axial NEeT shows right sided bladder diverticulum Axial color Doppler US shows a large bladder
(arrow) protruding through the muscle of the bladder diverticulum posterior to the bladder (arrow). Note
wall. color jet traversingneck of diverticulum (open arrow)
and connection to bladder lumen.

ITERMINOlOGY CT Findings
Abbreviations and Synonyms • CECT
o Fluid attenuation outpouching from bladder
• Bladder diverticulum (tic)
5 Definitions
o Usually fills with contrast on delayed images
MR Findings
12 • Sac formed by herniation of bladder mucosa and • Tl WI: Low signal mass contiguous with bladder
submucosa through muscular wall
• T2WI
o High signal mass contiguous with bladder
o May see dephasing with motion of urine between tic
I IMAGING FINDINGS and bladder lumen
General Features Ultrasonographic Findings
• Best diagnostic clue: Perivesical cystic mass with • Real Time
connection to bladder lumen o Anechoic outpouching from bladder
• Location o Narrow or wide neck
o Near ureterovesical junction (UVJ) o May contain stones, debris, or tumor
o Bladder dome: Likely urachal if solitary • Color Doppler: Color jet connecting to bladder very
• Size: Small to very large; can exceed size of bladder useful to distinguish diverticulum from other
• Morphology: Single or multiple; smooth wall paravesical masses
Radiographic Findings Imaging Recommendations
• IVP • Best imaging tool: Ultrasound, cystogram
o Medial deviation of ipsilateral ureter • Protocol advice: Check emptying of diverticulum on
o Usually fills with contrast unless obstructed post-void studies
o Diverticulum may contain stones, debris, or tumor
• Cystogram: Oblique films may show configuration of
diverticulum neck

DDx: Mimics of Bladder Diverticula

Lymphocele Pelvic Abscess Ev. Ureterocele

 BLADDER DIVERTICULUM

Key Facts
Imaging Findings
• Best diagnostic clue: Perivesical cystic mass with
connection to bladder lumen
• Near ureterovesical junction (UV])
• Size: Small to very large; can exceed size of bladder
• Color Doppler: Color jet connecting to bladder very
useful to distinguish diverticulum from other
paravesical masses
Clinical Issues
• Narrow-neck diverticula often drain poorly ~ chronic
infection and stone formation
• Secondary inflammation predisposes to development
of carcinoma within diverticulum
Diagnostic Checklist
• Continuity with urethra distinguishes bladder from
diverticulum

I DIFFERENTIAL DIAGNOSIS Treatment

• Options, risks, complications
lymphocele
o Complications: Carcinoma, vesico-ureteral reflux,
• Adjacent to extraperitoneal vessels; doesn't fill with ureteral obstruction
contrast o Surgery may be indicated for persistent infection,
Everted (Ev) ureterocele stone formation, or ureteral obstruction
• Continuous with ureter
Cystic masses: Abscess, ovarian cystic lesions I DIAGNOSTIC CHECKLIST
• No communication with bladder
Consider
• Large diverticulum may be confused with bladder
I PATHOLOGY especially if bladder is contracted
Image Interpretation Pearls
5
General Features
• Continuity with urethra distinguishes bladder from 13
• Etiology
o Most common: Acquired diverticulum
• Secondary to bladder outlet obstruction: Prostatic
enlargement, neurogenic bladder
• Children: Posterior urethral valves, prune-belly I SELECTED REFERENCES
syndrome, Ehlers-Danlos 1. Maynor CH et al: Urinary bladder diverticula: sonographic
o Congenital diagnosis and interpretive pitfalls. J Ultrasound Med.
• Weakness in detrusor muscle anterolateral to 15(3):189-94, 1996
2. Bellah RD et al: Ureterocele eversion with vesicoureteral
ureteral orifice (Hutch diverticulum)
reflux in duplex kidneys: findings at voiding
• Epidemiology: Prevalence 1.7% in children cystourethrography. AJR Am J Roentgenol. 165(2):409-13,
Microscopic Features 1995
3. Dondalski M et al: Carcinoma arising in urinary bladder
• Uroepitheliallining diverticula: imaging findings in six patients. AJR Am J
Roentgenol. 161(4):817-20, 1993

I CLINICAL ISSUES
Presentation I IMAGE GALLERY
• Most common signs/symptoms: Usually asymptomatic
• Clinical profile: Older male with BPR; spinal cord
injury patient
Demographics
• Age: 6th and 7th decade
• Gender: M:F = 9:1
Natural History & Prognosis
• Wide-neck diverticula: Empty readily with the bladder
• Narrow-neck diverticula often drain poorly ~ chronic
infection and stone formation
• Secondary inflammation predisposes to development
of carcinoma within diverticulum (Left) Axial T2WI MR shows large posterior bladder diverticulum
• Tumors in diverticula have worse prognosis; poorly (arrows) connected to bladder via a broad neck. Signal loss in
formed wall leads to more rapid local spread connecting channel is due to motion of urine and resultant
dephasing. (Right) Axial CECT shows large bladder diverticulum
(white arrows). Fillingdefect along medial wall of diverticulum (black
arrow) is transitional cell carcinoma (Courtesy M. Nino-Murcia, MO).
 BLADDER FISTULAS

Lateral view from a cystogram performed via Sagittal CECT shows fistula from sigmoid colon (arrow)
suprapubic catheter shows filling of vagina posteriorly to bladder (open arrow) in paUent with diverticuliUs.
(arrow) in patient with vesicovaginal fistula following Note associated abscesses (curved arrows) (Courtesy
hysterectomy. H. Harvin, MO).

ITERMINOLOGY CT Findings
Abbreviations and Synonyms • CECT
o Enterovesical: Gas in bladder (90%), bladder and/or
• Enterovesical, colovesical, vesicovaginal,
5 vesicocutaneous, or vesicouterine fistula
adjacent bowel wall thickening (90%)
o Extravesical soft tissue mass (75% of cases)
Definitions o Enterovesical: Direct visualization of fistulous tract
14
• Abnormal communication between bladder and in < 50% of cases
adjacent viscus o Vesicovaginal: Contrast in vagina on delayed
imaging
MR Findings
IIMAGING FINDINGS • Tl C+
General Features o High signal enhancing fistula wall and low signal
intensity tract on early post-contrast images
• Best diagnostic clue: Gas within bladder lumen
o Delayed imaging may show high signal fluid in tract
Radiographic Findings o Fat-saturation improves contrast between enhancing
• Radiography: Gas in bladder tract and background
• IVP Imaging Recommendations
o Bladder wall irregularity +/- mass effect
• Best imaging tool: CT
o Fistula rarely seen
• Protocol advice
• Cystography
oCT-cystogram technique or rectal contrast may
o Enterovesical: Filling of tract with contrast material
improve ability to visualize tract
seen in < 50% of cases; may see only bladder wall
o Multiplanar reformations may be helpful to
irregularity
demonstrate course of fistula
o Enterovesical: Tract may be unidirectional
o Post-void or post-evacuation scans may improve
o Vesicovaginal: Contrast opacifies vagina posteriorly
fistula detection
• Post-void and lateral films can be helpful to
demonstrate vaginal contrast

DDx: Mimics of Bladder Fistulae

..
. ".
~
,..
'
' J' -" \.
,

Catheterization Emph. Cystitis Cystitis

 BLADDER FISTULAS

Key Facts
Imaging Findings Pathology
• Vesicovaginal: Contrast opacifies vagina posteriorly • Inflammatory: Diverticulitis, Crohn disease,
• Enterovesical: Gas in bladder (90%), bladder and/or appendicitis, other pelvic abscesses
adjacent bowel wall thickening (90%) • Inflammatory: Granulomatous infection of bladder
• Extravesical soft tissue mass (75% of cases) with TB or schistosomiasis
• Enterovesical: Direct visualization of fistulous tract in • Neoplastic: Colon, bladder, gynecologic cancers
< 50% of cases • Penetrating trauma and iatrogenic injury
• Best imaging tool: CT

o
Most common: Nonspecific cystitis symptoms
I DIFFERENTIAL DIAGNOSIS o
Fecaluria or pneumaturia present in 40-70% of cases
Gas in bladder lumen: Gas-forming o
Persistent urinary tract infections (UTIs)
o
Vesicovaginal fistula: Painless dribbling
infection; catheterization incontinence of urine through vagina
• Emphysematous (Emph.) cystitis: May have gas in • Clinical profile
both wall and lumen o Vesicovaginal fistula: Post gynecologic surgery
Thick walled bladder: Cystitis • Pre-operative irradiation predisposes to fisula
o Enterovesical fistula: Patient with Crohn disease
• Clinical diagnosis
o Colovesical fistula: Patient with diverticulitis or
colon CA
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST 5
• General path comments Consider
o Diverticulitis is most common cause of colovesical 15
• Bowel fistulae harder to detect than vaginal
fistula, followed by colon CA
o Crohn disease is most common cause of Image Interpretation Pearls
enterovesical fistula • Bladder wall irregularity may be sole clue to presence
• Etiology of fistula
o Inflammatory: Diverticulitis, Crohn disease,
appendicitis, other pelvic abscesses
o Inflammatory: Granulomatous infection of bladder I SELECTED REFERENCES
with TB or schistosomiasis
o Neoplastic: Colon, bladder, gynecologic cancers 1. Pickhardt P] et al: Acquired gastrointestinal fistulas:
classification, etiologies, and imaging evaluation.
o Gynecologic surgery: Pelvic hematoma ~ infection Radiology. 224(1):9-23, 2002
~ erosion into bladder/vaginal wall 2. Pollack HM et al: Clinical urography. 2nd ed. Philadelphia,
o Penetrating trauma and iatrogenic injury WB Saunders, 3001-6, 2000
o Radiation: Usually 12-18 months after therapy 3. Semelka RC et al: Pelvic fistulas: appearances on MR
o Congenital: Rare; associated with other GU images. Abdom Imaging. 22(1):91-5, 1997
malformations
• Epidemiology
o 0.5-1% frequency of bladder injury with total I IMAGE GALLERY
abdominal hysterectomy
• 75% of vesicovaginal fistulae occur following
hysterectomy for benign disease
o Obstetrical trauma most common cause of
vesicovaginal fistula in developing countries
Gross Pathologic & Surgical Features
• Rectosigmoid colon is bowel segment most frequently
involved
• Cystoscopy shows inflammatory changes but fistula
usually not seen

I CLINICAL ISSUES (Left) Sagittal CT-cystogram shows fistulous connection (arrow)

between bladder & uterus, with filling of vagina (open arrow). Patient
Presentation was post Cesarian section for placenta accreta. (Right) Axial CECT
• Most common signs/symptoms shows thickened loop of small bowel (arrow) adjacent to
thick-walled bladder in patient with Crohn disease & bladder fistula.
 NEUROGENIC
Cystogram shows "pine cone" bladder, elongated and
pointed bladder with pseudodiverticula (arrow).
ITERMINOlOGY
Definitions
• Lesion of nervous system causing bladder dysfunction
5 (voiding), detrusor-sphincter dyssynergia
16 I IMAGING FINDINGS
General Features
• Best diagnostic clue: Contracted or distended bladder
• Other general features
o Types of lesions: Suprapontinei suprasacrali
epiconali peripherali sensory
Radiographic Findings
• IVP, cystourethrography or cystography findings
o Suprapontine (above pontine center) lesions ~
detrusor hyperreflexia
• Rounding of the bladder shape & serration of
mucosa above the trigone (detrusor contractions)
• Prominent interureteric ridge apparent only
during voiding (detrusor contractions)
• ± Trabeculations & 1 wall thickness
o Suprasacral (above S2-4) lesions ~ detrusor
hyperreflexia with detrusor-sphincter dyssynergia
• Beak of contrast (slightly open posterior urethra)
• Contrast extends to external sphincter/urogenital
diaphragm (distended posterior urethra)
DDx: Dilated Bladder or Mimics
Prostate Carcinoma Ovarian Tumor
BLADDER
Axial NECT in a patient with neurogenic bladder shows
bilateral renal cortical atrophy and parenchymal
calcification due to chronic reflux.
• "Christmas tree" or "pine cone" shape (severe):
Elongated & pointed with pseudo diverticula
o Epiconal (in and around S2-4) lesions ~ weak
detrusor hyperreflexia (storing), detrusor areflexia
• Upper & lower motor neuron featuresi "pine cone"
o Peripheral (below S2-4) lesions ~ detrusor areflexia
• Large atonic bladder: No signs of detrusor
contraction during voiding and poor emptying
• Downward displacement of bladder base & vagina
~ functional infravesical obstruction (females)
o Sensory lesions ~ unable to sense bladder fullness
• Voiding is preserved (early)
• Rounding of bladder shape & serration of mucosa
• Large atonic & smooth bladder: Indistinguishable
from peripheral nerve lesions (chronic)
o Secondary bladder & upper tract abnormalities
• Trabeculation (Le., hypertrophy), dilated upper
tracts, pseudodiverticula, vesicoureteral reflux
• Indistinguishable between detrusor-sphincter
dyssynergia & functional infravesical obstruction
Ultrasonographic Findings
• Real Time: Small contracted/large atonic bladderi wall
thickeningi vesicoureteric reflux during voidingi
increased post-void residual urine
Imaging Recommendations
• IVP or cystourethrography: At rest and voiding
•.
,,... - -
Foreign Body Foreign Body
 NEUROGENIC BLADDER
Key Facts
Terminology • Bladder foreign body
• Lesion of nervous system causing bladder dysfunction Clinical Issues
Imaging Findings • Frequency, nocturia, urgency, retention,
• Best diagnostic clue: Contracted or distended bladder incontinence

Top Differential Diagnoses Diagnostic Checklist

• Bladder outlet obstruction • Multiple sites of injury ~ mixed features
• Pelvic cystic mass • ± Trabeculationsi "pine tree" appearance

o t Volume: Cystoplasty, muscular or fascial slings,

I DIFFERENTIAL DIAGNOSIS
Bladder outlet obstruction
• Examples: Urethral strictures, benign or malignant
enlargement of prostate, congenital urethral valves
• Trabeculation of detrusor and urine retention
• Detrusor hyperreflexia (early) ~ areflexia (chronic)
• Relieve obstruction ~ t function, but never normal
Pelvic cystic mass
• Example: Ovarian cyst/carcinoma, tuboovarian abscess
• No trabeculationi features do not change with voiding
parasympatholytic drugs, botulinum-a toxin
o t Voiding: Catheter, transurethral sphincterotomy
• Hyporeflexia
o Bladder training, catheter, bladder neck
resection/denervation, parasympathomimetic drugs
I DIAGNOSTIC CHECKLIST
Consider
• Multiple sites of injury ~ mixed features

Bladder foreign body Image Interpretation Pearls

• Can distort lumen, calcify, cause dysuria • ± Trabeculationsi "pine tree" appearance 5
17
I PATHOLOGY I SELECTED REFERENCES
1. Zawin JK et al: Neurogenic dysfunction of the bladder in
General Features infants and children: Recent advances and the role of
• Etiology radiology. Radiology. 182: 297, 1992
o Suprapontine: Stroke, arteriosclerosis or multiple 2. Ruutu M et al: The value of urethrocystography in the
sclerosis investigation of patients with spinal cord injury. Clinical
o Suprasacral: Trauma, tumor or multiple sclerosis Radiology. 35: 485-9, 1984
3. Shapeero G et al: Transrectal sonographic voiding
o Epiconal: Myelomeningocele, tumor or trauma
cystourethrography: Studies in neuromuscular dysfunction.
o Peripheral (pelvic ± pudendal nerve): Pelvic surgery, AJR. 141: 83-90, 1983
cauda equina
o Sensory: Diabetes mellitus, pernicious anemia or
tabes dorsalis
I IMAGE GAllERY
Gross Pathologic & Surgical Features
• Small (hyperreflexic)/large capacity (areflexic) bladder

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Frequency, nocturia, urgency, retention,
incontinence
o Hyper- or hyporeflexiai impaired or no sensation
Natural History & Prognosis
• Complications: Infection, hydronephrosis, urolithiasis, (Left) Axial CECT shows distended neurogenic bladder and anterior
sexual dysfunction, autonomic dysreflexia meningocele in a patient with spina bifida. (Right) Axial CECT in a
• Prognosis: Good in early stage (treatment)i poor in late patient with spina biFida shows a distended neurogenic bladder and
stage (renal damage ~ renal failure) anterior meningocele.

Treatment
• Hyperreflexia
 BLADDER TRAUMA

Cystogram shows displaced pelvic fractures and Axial CECT following cystogram shows extravasated
extraperitoneal bladder rupture (arrows). urine in extraperitoneal pelvic spaces and proximal right
thigh; type 48.

• Contrast in paracolic gutters, around bowel loops,

ITERMINOLOGY pouch of Douglas and intraperitoneal viscera
Definitions • ± Pelvic fractures
• Bladder injury due to blunt, penetrating or iatrogenic o Interstitial injury (type 3)
5 trauma
o
• Focal mural defect along wall of bladder
Extraperitoneal rupture (type 4)
18 • Simple (type 4A): Flame-shaped extravasation
I IMAGING FINDINGS around bladder
• Complex (type 4B): Extravasation extends beyond
General Features the pelvis
• Best diagnostic clue: Extravasation of contrast in • Extravasation best seen on post-drainage films
cystography o Combined rupture (type 5)
• Other general features • Features of intra- and extraperitoneal ruptures
o Classification of bladder injury after blunt trauma o Penetrating injury
• Type 1: Bladder contusion • Foreign bodies (Le., metallic fragments from a
• Type 2: Intraperitoneal rupture bullet) along course of penetration
• Type 3: Interstitial injury • Extravasation ± foreign body (Le., bullet) best seen
• Type 4A: Simple extraperitoneal rupture on post-drainage films
• Type 4B: Complex extraperitoneal rupture CT Findings
• Type 5: Combined injury
• CT cystography
Radiographic Findings o Bladder contusion (type 1)
• Cystography • Normal findings
o Bladder contusion (type 1) o Intraperitoneal rupture (type 2)
• Teardrop bladder: Extrinsic compression of • Contrast in paracolic gutters, around bowel loops,
bladder (symmetrical extraperitoneal hematoma) pouch of Douglas and intraperitoneal viscera
o Intraperitoneal rupture (type 2) o Interstitial injury (type 3)
• Intramural and submucosal extravasation of
contrast without transmural extension

DDx: Pelvic Trauma with Hematuria

Hemoperitoneum Active Bleeding Pelvic Bleeding Blood Clot in Bladder

 BLADDER TRAUMA
Key Facts
Terminology
• Bladder injury due to blunt, penetrating or iatrogenic
trauma
Imaging Findings
• Best diagnostic clue: Extravasation of contrast in
cystography ,
• Focal mural defect along wall of bladder
• Extravasation best seen on post-drainage films
• Best imaging tool: Cystography or CT cystography:
85-100% sensitivity
Top Differential Diagnoses
• Simple male urethral injury
• Hemoperitoneum
• Pelvic bleeding
o Extraperitoneal rupture (type 4)
• Perforation by bony spicules
• "Knuckle" of bladder: Trapped bladder by
displaced fracture of anterior pelvic arch
• Simple (type 4A): Extravasation is confined to
perivesical space
• Complex (type 4B): Extravasation extends beyond
perivesical space; thigh, scrotum, penis, perineum,
anterior abdominal wall, retroperitoneum
(paranephric or perinephric spaces) or hip joint
• "Molar tooth sign": Rounded cephalic contour
(due to vertical perivesicle components of
extraperitoneal fluid)
o Combined rupture (type 5)
• Features of intra- and extraperitoneal ruptures
Ultrasonographic Findings
• Real Time
o "Bladder within a bladder": Fluid around bladder
o Pseudomass appearance: Intravesical hematoma
o Fluid in abdomen and pelvis (Le., pouch of Douglas)
Imaging Recommendations
• Best imaging tool: Cystography or CT cystography:
85-100% sensitivity
• Protocol advice
o Cystography
• Exclude urethral injury in males prior to
cystography; if positive, use suprapubic
cystostomy tube
• Post-drainage film to check for extravasation
hidden by contrast
• CT cystograph: Instill diluted contrast (10 ml
contrast, 300 ml saline
I DIFFERENTIAL DIAGNOSIS
Simple male urethral injury
• Contrast extravasation into base of penis or thigh may
result form urethral or extra peritoneal bladder
• Blood in urethral meatus is indicative of urethral
injury; avoid "blind" catheterization to bladder
Pathology
• Propensity for bladder injury is related to degree of
bladder distention; more distended, more injury
• Blunt trauma: Seat belt or steering wheel injury
• 84-100% of bladder trauma have pelvic fractures
• 10-29% of bladder trauma have urethral injuries
Clinical Issues
• Gross hematuria (84-95%)
• Bladder trauma including penetrating injury: 12-22%
mortality
Diagnostic Checklist
• Cystography ± CT still the procedure of choice
• Bloody urethral discharge requires urethrogram prior
to catheterization
• CT after only IV contrast may miss bladder rupture
Hemoperitoneum
• Clotted blood/active extravasation of enhanced blood
may simulate extravesicle urine from bladder rupture
• Compare with density of blood and urine
• CT cystography usually diagnostic
Pelvic bleeding 5
• Bleeding from other pelvic trauma can appear similar
19
to contrast extravasation from bladder
• Synchronous occurrence with bladder trauma is likely;
complete pelvic assessment is needed
Blood clot in bladder
• Blood from renal or bladder injury may cause irregular
filling defect within bladder
I PATHOLOGY
General Features
• General path comments
o Propensity for bladder injury is related to degree of
bladder distention; more distended, more injury
o Location of extravasation depends on 'anatomy
• Anterosuperior rupture: Extravasation to
intraperitoneal, Retzius space or both
• Posterosuperior rupture: Extravasation to
intraperitoneal, extra peritoneal or both
• Intact superior fascia of urogenital diaphragm
prohibits extravasation from escaping the pelvis
• Intact inferior fascia of urogenital diaphragm
prohibits extravasation into perineum
o Bladder contusion (type 1)
• Incomplete or partial tear of bladder mucosa;
ecchymosis of a localized segment of bladder wall
• Most common minor bladder injuries
• Diagnosed by exclusion
o Intraperitoneal rupture (type 2)
• Direct blow to lower abdomen with a distended
bladder
• 1 Bladder pressure ~ horizontal tear along bladder
wall; at dome of bladder covered by peritoneum
• 25% of major bladder injuries
 BLADDER TRAUMA
• more common in < 20 years of age due to
intra-abdominal bladder
o Interstitial injury (type 3)
• Intramural or partial-thickness laceration with
intact serosa
• Incomplete perforation; seen on either intra- or
extraperitoneal portion of bladder
• Rare bladder injuries
o Extraperitoneal rupture (type 4)
• Classic mechanism: Anterolateral laceration at
base of bladder by bony spicules (anterior pelvic
arch fractures)
• Other mechanism: Stress applied to hypogastric
wing or puboprostatic ligaments ~ laceration;
direct blow to distended bladder
• 62% (most common) of major bladder injuries
o Combined rupture (type 5)
• Both intra- and extraperitoneal bladder ruptures
• 12% bladder ruptures, include both blunt and
penetrating trauma
o Penetrating injury
• Results in intraperitoneal, extraperitoneal or
combined bladder injury
• t Incidence of vascular injuries ~ high mortality
• Etiology
o Blunt trauma: Seat belt or steering wheel injury
o Penetrating Injury: Gunshot or knife wounds
5 o Iatrogenic injury: Pelvic, urological, obstetrical and
gynecological procedures
20 • Associated abnormalities
o 6-10% of pelvic fractures have bladder trauma
o 84-100% of bladder trauma have pelvic fractures
o 10-17% of urethral in juries have bladder trauma
o 10-29% of bladder trauma have urethral injuries
o Penetrating injury
• Gunshot wounds: 83% have bowel injuries
• Knife wounds: 33% have colon injuries
Gross Pathologic & Surgical Features
• Asymmetrical compression, congestion, laceration or
fill-thickness tear
Microscopic Features
• Mural defect; contusion or clot within bladder mucosa
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Gross hematuria (84-95%)
o Suprapubic pain and tenderness, anuria, fever
o Bladder contusion (type 1): Hematuria
o Intraperitoneal rupture (type 2)
• No bowel sounds; acute abdomen
• Lab data
o Urinalysis: Hematuria
o Intraperitoneal rupture (type 2)
• Chemistry: Hypernatremia, hyperkalemia, uremia
and acidosis (reabsorption of urine)
• Peritoneal lavage: Urinary ascitic fluid
• Catheterization: No urine
Natural History & Prognosis
• Complications: Fistulas, sinuses, abscess, sepsis,
bladder calculi, hematoma, hemorrhage or shock
• Minor injuries: Good, spontaneous healing within
10-14 days
• Major injuries: Poor, if not corrected surgically and
with complications
o Bladder trauma including penetrating injury:
12-22% mortality
o Penetrating injury: 12% mortality
Treatment
• Bladder contusion (type 1) and interstitial injury (type
3): Self limiting, no treatment
• Intraperitoneal rupture (type 2): Open surgical
correction via trans peritoneal approach;
postoperatively, use suprapubic and urethral catheters
• Extraperitoneal rupture (type 4): Antibiotics and
urethral catheter drainage (10 days); large blood clots
or bladder neck injuries require surgery
• Penetrating injury: Exploratory laparotomy with
surgical correction
• Follow-up: Cystography 10 days after conservative or
surgical treatment
I DIAGNOSTIC CHECKLIST
Consider
• Cystography ± CT still the procedure of choice
• Bloody urethral discharge requires urethrogram prior
to catheterization
Image Interpretation Pearls
• CT after only IV contrast may miss bladder rupture
• .CT or conventional cystogram
I SELECTED REFERENCES
1. Morgan DE et al: CT cystography: Radiographic and
clinical predictors of bladder rupture. AJR. 174: 89-95, 2000
2. Vaccaro JP et al: CT cystography in the evaluation of major
bladder trauma. RadioGraphics. 20:1373-81, 2000
3. Bigongiari LR et al: Trauma to the bladder and urethra.
American College of Radiology. ACR Appropriateness
Criteria. Radiology. 215 Suppl:733-40, 2000
4. Deck AJ et al: Computerized tomography cystography for
the diagnosis of traumatic bladder rupture. J Urol.
164(1):43-6, 2000
5. Peng MY et al: CT cystography versus conventional
cystography in evaluation of bladder injury. AJRAm J
Roentgenol. 173(5):1269-72, 1999
6. Kane NM et al: The value of CT in the detection of bladder
and posterior urethral injuries. AJRAm J Roentgenol.
153(6):1243-6, 1989
7. Corriere IN Jr et al: Mechanisms of injury, patterns of
extravasation and management of extra peritoneal bladder
rupture due to blunt trauma. J Urol. 139(1):43-4, 1988
8. Sandler CM et al: Bladder injury in blunt pelvic trauma.
Radiology. 158: 633-8, 1986
 BLADDER TRAUMA
I IMAGE GALLERY
Typical
(Left) Axial CECT following
cystogram shows opacified
urine in left paracolic gutter
and between bowel loops
(intraperitoneal). (Right)
Axial CECT following
cystogram shows
extraperitoneal extravasated
urine with "molar tooth"
appearance. Type 5
(combined) rupture.

(Left) Cystogram shows

extravasation from base of
bladder (arrow). (Right)
Axial CECT following
cystogram shows
extravasation of urine into
5
thigh and base of penis.
21

(Left) Retrograde
urethrogram shows
extravasation into scrotum
and pelvis from tear of base
of bladder; pelvic fractures.
(Right) Axial CECT prior to
cystogram shows
extraperitoneal blood.
Difficult to recognize
extravasated urine.
 BLADDER CARCINOMA

Graphic shows tumor arising from posterior wall of Axial CECT shows asymmetric thickening of bladder
bladder;invading through the rightseminal vesicle with wall (open arrow) due to carcinoma. Lymph node
hematogenous metastases to the right pubic ramus; metastases (arrows).
lymph nodes metastases (arrows).

ITERMINOlOGY
Definitions
• Malignant tumor growth within the bladder
5 • ± Enlarged (> 10 mm) metastatic lymph nodes;
22 IIMAGING FINDINGS
General Features
• Best diagnostic clue: Bladder wall invasion by
intraluminal soft tissue mass on CT or MRI
Radiographic Findings
• IVP
o Multifocal (2-3% of urothelial cancer)
o Punctate or speckled calcification on fronds of
villous, papillary tumors (en face view)
o Linear or curvilinear calcification on the surface of
sessile tumors
o Central calcification (necrosis)
o ± Lytic bony metastasis or sclerotic
o ± Urinary tract obstruction
o ± Deviation of bladder from pelvic or retroperitoneal
adenopathy
o ± Luminal protrusions or invaginations
o Nonspecific filling defects within bladder
• Cystography
o ± Bladder diverticulum (2-10% contain neoplasm)
CT Findings
• Sessile or pedunculated soft tissue mass projecting into
the lumen; similar density to bladder wall
extravesical tumor extension
• Fine punctate calcifications with tumor; may suggest
mucinous adenocarcinoma
• Ring pattern of calcification; may suggest
pheochromocytoma
• Inability to distinguish tumors from bladder wall
hypertrophy, local inflammation and fibrosis
• Unable to differentiate Ta-T3a, invasion of dome/base
of bladder or local organ (due to partial volume effect),
nonenlarged lymph nodes
• Urachal adenocarcinoma
o Midline abdominal mass ± calcification
o Solitary lobulated tumor arising from dome of
bladder on ventral surface
MR Findings
• TIWI
o Tumor has intermediate signal intensity, equal to
muscle layer of bladder wall
o Infiltration of perivesical fat (high signal intensity)
o Endoluminal tumor in urine filled bladder (low
signal intensity)
o Bone marrow metastases; similar signal intensity as
primary tumor

DDx: Bladder Wall Thickening with Hematuria

••'" -
fl,"I' . .t .
--
<

Lymphoma of Bladder Cola vesicle Fistula Blood Clot Hemorrhagic Cystitis

 BLADDER CARCINOMA
Key Facts
Terminology
• Malignant tumor growth within the bladder
Imaging Findings
• Best diagnostic clue: Bladder wall invasion by
intraluminal soft tissue mass on CT or MRI
• Punctate or speckled calcification on fronds of
villous, papillary tumors (en face view)
• Sessile or pedunculated soft tissue mass projecting
into the lumen; similar density to bladder wall
• ± Enlarged (> 10 mm) metastatic lymph nodes;
extravesical tumor extension
Top Differential Diagnoses
• Extrinsic tumor
• Extrinsic inflammation
• Blood clot
• T2WI
o Tumor has intermediate signal intensity, higher
than bladder wall or fibrosis, lower than urine
o Determine tumor infiltration of perivesical fat
(either low or high signal intensity)
o Invasion of prostate, rectum, uterus, vagina ~ 1
signal intensity
o Direct invasion of seminal vesicles (sagittal plane) ~
1 size, !signal intensity & obliteration of angle
between seminal vesicle & posterior bladder wall
o Confirm bone marrow metastases
• T1 C+
o Mild enhancement in primary, perivesical, nodal or
bone invasion
o Tumor shows early & 1 enhancement than bladder
wall or other benign tissues; assess infiltration
o Earlier enhancement than edema and granulation
tissue
• ± Enlarged (> 10 mm) metastatic lymph nodes
• Unable to differentiate stage T1 from stage T2, acute
edema or hyperemia from first week post-biopsy or
nonenlarged lymph nodes
• Urachal adenocarcinoma
o Varied appearance
o T2WI: Increased signal intensity
Ultrasonographic Findings
• Fixed echogenicities not casting an acoustical shadow
• Inability to distinguish tumors from chronic cystitis,
local bladder wall hypertrophy, blood clots, bladder
wall invasion, abnormal lymph nodes
• Transabdominal: Unsuitable images caused by obesity,
scars on wall and poor bladder distention
• Intravesical: Invasive and requires anesthesia
Imaging Recommendations
• Best imaging tool
o IVP: Screening upper urinary tract
o MRI: Staging bladder carcinoma
• Accuracy: 73-96%; 10-33% greater than CT
• Protocol advice: MRI: Multiplanar imaging including
sagittal plane, gadolinium contrast, fast dynamic
first-pass MRI with acquisition every 2 seconds
• Bladder inflammation
• Trabeculation
Clinical Issues
• Most common signs/symptoms: Painless hematuria
• 50-60 years of age
• Gender: M:F = 4:1
• 5 year survival rate: 82% in all stages combined
Diagnostic Checklist
• Detection by cystoscopy; diagnosis by transurethral
resection ± biopsy
• Staging is needed for treatment and prognosis
• Check kidneys, ureters for synchronous and
metachronous tumors
• MRI is superior in staging and used in patients with
high grade stage T1 or > stage T2
I DIFFERENTIAL DIAGNOSIS
Extrinsic tumor
• Rectal, ovarian, vaginal tumor or fibroids overlying
bladder; may simulate bladder carcinoma
Extrinsic inflammation
• Diverticulitis often inflames and thickens bladder wall;
5
may cause fistula 23
Blood clot
• IVP: "Stipple sign": Contrast trapped within interstices
of tumor
• CT: Disappear with time, no enhancement
• MR: ! Signal intensity, no infiltration
• US: Mobile mass, does not cast an acoustical shadow
Bladder inflammation
• Cystitis may cause mural thickening and hemorrhage
Trabeculation
• Secondary sign of outlet obstruction
• Associated with increased bladder wall thickness
• May vary inversely with amount of urine in bladder; 2
cm thick bladder wall and crinkled mucosa are normal
for an empty bladder
• Caused by tumor or benign prostate hypertrophy
I PATHOLOGY
General Features
• General path comments
o 95% of bladder neoplasms are malignant
o Types of epithelial bladder carcinoma
• Transitional cell carcinoma (90-95%)
• Squamous cell carcinoma (5%)
• Adenocarcinoma (2%): Urachal origin, secondary
to cystitis glandularis, secondary to extrophy
• Carcinosarcoma
• Other rare tumors: Carcinoid, rhabdoid, villous,
small cell
• Metastasis: Gastrointestinal tract, melanoma
o Types of nonepithelial bladder carcinoma
 BLADDER CARCINOMA
• Pheochromocytoma o Multifocal, high grade or infiltrating tumor ~ 1 risk
• Leiomyosarcoma of metastases
• Embryonal rhabdomyosarcoma (most common o 5 year survival rate: 82% in all stages combined
bladder neoplasm in children) • 94% in localized stages
• Lymphoma • 48% in regional stages
• Plasmacytoma • 6% in distant stages
• Genetics: Lack of GSTM1 (1.8-fold 1 risk in smokers)
• Etiology
Treatment
o Aromatic amines, nitrosamines, aldehydes (e.g., • < T2: Local endoscopic resection ± intravesical
acrolein) instillation or bacille Calmette-Guerin therapy
o Risk factors • T2 to T4a: Radical cystectomy or radiotherapy (cure)
• Environment: Smoking • > T4b: Chemotherapy or radiotherapy ± adjuvant
• Infection: Schistosomiasis, chronic cystitis surgery (palliative)
• Iatrogenic: Cyclophosphamide, radiation therapy • Follow-up
• Occupation: Chemical, dye (e.g., aniline dye), o < T2: Repeated cystoscopy every 3-6 months
rubber and textile industries o T2 to T4a: Clinical; MRI if indicated
• Epidemiology 0> T4b: MRI
o Mortality: 12,710 in U.S.
o Incidence: 60,240 in U.S.
o Fourth leading cause of cancer incidence in males I DIAGNOSTIC CHECKLIST
o Tenth leading cause of cancer incidence in females
Consider
Gross Pathologic & Surgical Features • Detection by cystoscopy; diagnosis by transurethral
• Superficial and are usually papillary (66%) resection ± biopsy
• Infiltrating in/beyond muscular layer of wall (33%) • Staging is needed for treatment and prognosis
• Check kidneys, ureters for synchronous and
Staging, Grading or Classification Criteria metachronous tumors
5 • TNM classification of bladder carcinoma
o TO: No tumor Image Interpretation Pearls
o Tis: Carcinoma in situ • MRI is superior in staging and used in patients with
24
o Ta: Papillary tumor confined to mucosa (epithelium) high grade stage T1 or > stage T2
o T1: Invasion of lamina propria (subepithelial
connective tissue)
o T2: Invasion of inner half of muscle (detrusor) I SELECTED REFERENCES
o T2b: Invasion of outer half of muscle 1. Barentsz JO et al: Staging urinary bladder cancer after
o T3a: Microscopic invasion of perivesical fat transurethral biopsy: value of fast dynamic
o T3b: Macroscopic invasion of perivesical fat contrast-enhanced MR imaging. Radiology. 201(1):185-93,
o T4a: Invasion of surrounding organs 1996
o T4b: Invasion of pelvic or abdominal wall 2. Kim B et al: Bladder tumor staging: comparison of
o Nl-3: Pelvic lymph node metastases contrast-enhanced CT, Tl- and T2-weighted MR imaging,
dynamic gadolinium-enhanced imaging, and late
o N4: Lymph node metastases above bifurcation
gadolinium-enhanced imaging. Radiology. 193(1):239-45,
o M1: Distant metastases 1994
3. Narumi Y et al: Bladder tumors: staging with
gadolinium-enhanced oblique MR imaging. Radiology.
I CLINICAL ISSUES 187(1):145-50, 1993
4. Tanimoto A et al: Bladder tumor staging: comparison of
Presentation conventional and gadolinium-enhanced dynamic MR
• Most common signs/symptoms: Painless hematuria imaging and CT. Radiology. 185(3):741-7, 1992
• Lab data 5. Rholl KSet al: Primary bladder carcinoma: evaluation with
MR imaging. Radiology. 163(1):117-21, 1987
o Positive urine dip stick
6. Amendola MA et al: Staging of bladder carcinoma:
o ± Micro- to normocytic anemia MRI-CT-surgical correlation. AJRAm J Roentgenol.
• Diagnosis: Transurethral resection ± biopsy 146(6):1179-83, 1986
7. Hillman BJ et al: Recognition of bladder tumors by
Demographics excretory urography. Radiology. 138(2):319-23, 1981
• Age 8. Morgan CL et al: Computed tomography in the evaluation,
o 50-60 years of age staging, and therapy of carcinoma of the bladder and
o Increasing incidence in patients < 30 years of age prostate. Radiology. 140(3):751-61, 1981
• Gender: M:F = 4:1
• Ethnicity: Caucasian-to-African-American ratio: 1.5:1
Natural History & Prognosis
• Complications
o Hydronephrosis, incontinence & urethral stricture
• Prognosis
 BLADDER CARCINOMA
I IMAGE GALLERY

Typical
(Left) Axial CECT shows
small hydronephrotic kidney
and liver metastasis due to
bladder carcinoma. (Right)
Axial CECT shows
hydronephrotic and
atrophied right kidney due to
ureteral obstruction by
bladder cancer.

Typical
(Left) Axial CECT shows
pelvic lymphadenopathy
(arrow) due to bladder
cancer. (Right) Axial CECT
shows multiple sclerotic
bone metastases.
5
25

Typical
(Left) Axial CECT shows
multiple bladder diverticula,
with a mass (TCC) in one of
these (arrow). (Right) Axial
CECT shows multiple
bladder diverticula, with a
tumor (TCC) in one of these
(arrow).
SECTION 6: Genital Trad (Male)

Introduction and Overview

Genital Tract Anatomy and Imaging Issues 11I-6-2

Congenital
Cryptorchidism 11I-6-6

Infection
Epididymitis 11I-6-8
Prostatitis 11I-6-10

Inflammation
Urethral Stricture 11I-6-12
Hydrocele 11I-6-14
Varicocele 11I-6-16
Testicular Torsion 11I-6-18
Benign Prostatic Hypertrophy 11I-6-20

Trauma
Scrotal Trauma 11I-6-22

Neoplasm
Gonadal Stromal Tumors 11I-6-24
Testicular Carcinoma 11I-6-26
Prostate Carcinoma 11I-6-30
 GENITAL TRACT ANATOMY AND IMAGING ISSUES
Sagittal graphic of male genital tract anatomy. Vas
• deferens (white arrows) passes from scrotum through
inguinal ring to unite with ejaculatory ducts. Note
verumontanum (black arrow).
I IMAGING ANATOMY
• Prostatic capsule = blending of prostatic fibromuscular
stroma with endopelvic fascia
o Absent anteriorly and at apex
• Prostate
o Ovoid gland; tapers as it extends from bladder base
to urogenital diaphragm
o Base of prostate: Abuts base of bladder
o Apex of prostate: Inferior to base of prostate; abuts
urogenital diaphragm
6 o Glandular prostate surrounded by smooth muscle
and connective tissue stroma
2 • Anterior fibromuscular stroma blends with
periurethral muscle fibers at bladder neck
o Peripheral zone
• Acinar glandular tissue
• Accounts for 70% of prostatic volume
• Site of origin of > 70% of prostate cancers
• Extends from prostate base to apex
o Central zone
• Deep to peripheral zone
• Primarily stromal cells
• Site of origin for 10% of prostate cancers
• Surrounds ejaculatory ducts from insertion at
prostatic base to verumontanum
o Transition zone
• Bilobed; surround prostatic urethra
• Site of hyperplastic nodules of BPH
• Site of origin for 20% of prostate cancers
• Represents 5% of gland volume in youth versus up
to 90% of volume in old age
o Periurethral zone
• Short ducts lining prostatic urethra
o Neurovascular bundle
• Prostatic arteries and veins; cavernosal nerves
• Common site of extracapsular extension of
prostate CA
• At 5 and 7 o'clock positions posterolateral to
prostate
o Periprostatic venous plexus
Voiding cystourethrogram shows normal male posterior
urethral anatomy. Note verumontanum (black arrow),
prostatic urethra (white arrows) and membranous
urethra (open arrows).
o Lymphatic drainage of prostate
• Obturator, external iliac, internal iliac nodes
• Seminal vesicles
o Extend laterally from base of prostate
o Outpouchings of vas deferens
o Each is a highly convoluted tubule that produces
protein in seminal fluid; contracts during
ejaculation .
• Ejaculatory ducts
o At junction of seminal vesicles and vas deferens
o Cross peripheral and central zones of prostate to end
at verumontanum in prostatic urethra
• Testis
o Fibrous capsule: Tunica albuginea
o Mediastinum: Invagination of tunica into testis; site
of spermatic cord insertion
o Divided into cone-shaped lobules that converge to
mediastinum
o Lobules contain branching seminiferous tubules that
coalesce into 12-20 efferent ductules at mediastinum
o Efferent ductules unite and continue as epididymis
• Vas deferens
o Distal continuation of tail of epididymis
o Ascends posteriorly in spermatic cord to deep
inguinal ring
o Crosses anterior to external iliac artery then curves
downward into pelvis
o Joins seminal vesicle at base of prostate to form
ejaculatory duct
• Spermatic cord
o Contains vas deferens, lymphatics, blood vessels and
nerves
• Scrotum
o Outpouching of abdominal cavity
o Concentric layers reflect layers of abdominal wall
• Skin~dartos~external spermatic fascia
(ESF)~cremaster muscle~Internal spermatic fascia
(ISF)~parietal tunica vaginalis~potential
space~visceral tunica vaginalis~tunica albuginea
• Male urethra
 GENITAL TRACT ANATOMY AND IMAGING ISSUES
DIFFERENTIAL DIAGNOSIS
Intratesticular mass Urethral stricture
• Germ cell tumors, stromal tumors • Infectious/inflammatory: Gonococcus,
• Leukemia/lymphoma nongonococcal urethritis, TB
• Metastases • Bulbar urethra
• Orchitis/abscess • Multiple and serial strictures
• Hematoma/contusion • Iatrogenic: Surgery, instrumentation
• Focal infarct • Membranous urethra or penile-scrotal junction
• Traumatic: Following complete transection
Extratesticular mass • Solitary, short segment
• Cysts: Spermatoceles, epididymal cyst, tunica
albuginea cyst Urethral filling defects
• Calcifications: Scrotal pearl, tunica plaques • Common: Stone, polyp, carcinoma
• Non-neoplastic masses: Hernia, varicocele, abscess, • Unusual: Condyloma acuminata, malacoplakia,
sperm granuloma urethritis cystica, metastases
• Tumors: Adenomatoid tumor, lipoma, sarcoma,
lymphoma, leiomyoma

o Posterior urethra: From bladder neck to urogenital
diaphragm
• Prostatic urethra: Traverses transitional zone of
prostate
• Verumontanum: Site of insertion of prostate
glands
• Membranous: At external urethral sphincter
o Anterior urethra: Extends through corpus
spongiosum of penis
• Bulbar urethra
• Penile urethra
o Cowper glands
• Glands surround membranous urethra
• Drain into proximal bulbar urethra
Anatomic Relationships
• Arteries
o In spermatic cord: Testicular artery (from aorta),
cremasteric artery (from inferior epigastric), artery to
vas deferens (from vesical artery)
• Testicular artery: Arises from aorta; supplies testis
and epididymis
• Cremasteric artery: Arises from inferior epigastric;
supplies cremaster muscle and other layers of
spermatic cord
• Artery to vas deferens: Arises from superior vesical
artery; supplies vas
• Cremasteric and vas deferens arteries anastamose
with testicular artery
• Veins
o From scrotum: Pampiniform plexus in spermatic
cord~ipsilateral gonadal vein
o Right gonadal vein~directly into IVC
o Left gonadal vein ~ left renal vein
• Nerves: Cremasteric nerve, genital branch of
genitofemoral nerve, testicular sympathetic plexus
• Lymphatics
o Ascend in spermatic cord with testicular vessels
o Drain to lateral aortic and preaortic nodes
Fascia
• Scrotal wall layers: Derived from layers of abdominal
wall
o Tunica vaginalis: Continuation of peritoneal
processus vaginalis
• Dartos: Outer layer of the scrotum
I ANATOMY-BASED IMAGING ISSUES I
Normal Measurements
• Testis: 3.5-4 em length, 2-3 em width
• Prostate: 4.8 em width x 2.8 em x 2.8 em
o Normal weight: 20 ± 6 grams
• Epididymis: Head normally 7-8 mm in diameter
• Seminal vesicles: Each 3 em length x 1.5 em width but
size and shape variable
• Ejaculatory duct: 2 cm in length
6
• Male urethra: 20 em in length 3
I EMBRYOLOGY
Embryologic Events
• Primitive sex cords form as coelomic epithelium of
gonadal ridge proliferates and penetrates mesenchyme
• Gonad differentiates into testis under influence of
testes determining factor
• Two pairs of genital ducts in early embryo:
Mesonephric and paramesonephric ducts
• Mesonephric (Wolffian) duct persists in male: Forms
vas deferens, epididymis and ejaculatory ducts
• Mullerian ducts regress in male
• Testis descends from posterior abdominal wall into
scrotal sac following ligamentous band known as
gubernaculum
• Outpouching of peritoneal cavity follows migrating
gubernaculum to form processus vaginalis
• Evagination of peritoneum through abdominal wall
produces inguinal canal; fascial layers of scrotum
reflect abdominal wall layers
• Regression of gubernaculum fixes testis to scrotal wall
Practical Implications
• Incomplete testicular fixation predisposes to torsion
• Embryonic remnants
 GENITAL TRACT ANATOMY AND IMAGING ISSUES

Normal testis, epididymis, and spermaUc cord. Note Schematic drawing of testicular lymphatic drainage.
lobular architechture of testis. Primary pathways are shown in yellow.

o Appendix epididymis: Wolffian duct remnant; from • Malignant: Sarcomas (rhabdomyosarcoma,

cranial end liposarcoma, leibmyosarcoma, others), lymphoma
o Appendix testis (Hydatid of Morgagni): Mullerian o Epididymal tumors: Rare
duct remnant • Adenomatoid tumor: Most common; represents
o Inferior end of Mullerian duct persists as prostatic 30% of extratesticular masses
utricle at distal end of verumontanum • Leiomyomas
• Metastatic prostate CA, lymphoma
• Papillary cystadenoma: 65% of patients with von
I CUSTOM DIFFERENTIAL DIAGNOSISI Hippel-Lindau

Intratesticular mass Urethral stricture

6 •
•
Germ cell tumor
Stromal tumor
• Infectious/inflammatory:
urethritis, TB
Gonococcus, nongonococcal

4 • Leukemia/lymphoma o Bulbar urethra

• Metastases o Multiple and serial strictures
• Orchitis/abscess • Iatrogenic: Surgery, instrumentation
• Hematoma/contusion o Membranous urethra or penile-scrotal junction
• Focal infarct • Traumatic: Following complete transection
o Solitary, short segment
Extratesticular masses
• Cysts: Spermatoceles, epididymal cyst, tunica Urethral filling defects
albuginea cyst • Stone
• Calcifictions • Polyp
o Epididymis: Prior infection or trauma; age-related • Carcinoma
o Scrotal pearl: Within layers of tunica vaginalis • Unusual: Condyloma acuminata, malacoplakia,
• Adherent or mobile urethritis cystica, metastases
• Detached and calcified testicular appendages
secondary to prior torsion; or post-inflammatory
o Calcified tunica plaques I SELECTED REFERENCES
• Varicocele 1. Zagoria R: Genitourinary radiology, 2nd ed. Philadelphia,
• Hernia Mosby Ine. 312-51, 2004
• Abscess 2. Pavlica P et al: Imaging of male urethra. Eur Radiol.
• Non-neoplastic masses 13(7):1583-96, 2003
o Sperm granulomas: Iso-hypoechoic avascular mass; 3. Ali M et al: CT signs of urethral injury. Radiographies.
may be painful 23(4):951-63; discussion 963-6,2003
4. Sudakoff GS et al: Scrotal ultrasonography with emphasis
o Fibrous pseudotumor on the extra testicular space: anatomy, embryology, and
• Tumors pathology. Ultrasound Q. 18(4):255-73,2002
o Spermatic cord tumors: Rare 5. Kubik-Huch RAet al: CT and MRI of the male genital tract:
• 70% benign: Lipomas, mixed, fibromas, radiologic-pathologic correlation. Eur Radiol. 9(1):16-28,
leiomyomas 1999
 GENITAL TRACT ANATOMY AND IMAGING ISSUES

I IMAGE GALLERY
Typical
(Left) Cross-sectional graphic
shows concentric layers
surrounding testis: scrotal
skin (beige), Dartos (red),
ESF (orange), cremaster m.
(yellow), ISF (green), t.
vaginalis (blue), and t.
albuginea (gray). (Right)
Axial US shows normal testis
with echogenic mediastinum
testis (arrow). Vesselsenter
the testis through the
mediastinum.

(Left) Evolution of efferent

duct system. Wolffjan ducts
(yellow) are precursors of
epididymis, vas deferens and
appendix epididymis (open
arrow). Appendix testis
(arrow) is Mullerian duct
(green) remnant. (Right)
Sagittal color Doppler US
shows appendix testis
(arrow) arising from upper
pole of testis.
6
5

Typical
(Left) Drawing shows
prostate zonal anatomy
relative to urethra and
ejaculatory ducts: Transition
zone (green), central zone
(red), peripheral zone
(transparent) and anterior
fibromuscular stroma
(yellow). (Right) Axial T2WI
MR shows U-shaped high
signal peripheral zone
surrounding lower signal
intensity central gland
(transitional and central
zones).
 CRYPTORCHIDISM

Axial CECT shows ovoid mass (arrow) anterior to left Sagittal US at level just above left inguinal crease shows
iliac vessels representing cryptorchid testis. hypoechoic ovoid mass representing cryptorchid testis.
Note mediastinum testis (arrow),

ITERMINOLOGY Ultrasonographic Findings

• Real Time
Abbreviations and Synonyms o Ovoid homogeneous well-circumscribed structure
• Cryptorchidism = cryptorchism = undescended testis smaller than normal descended testis
Definitions o Echogenic line of mediastinum testis
• Incomplete descent of testis into base of scrotum Angiographic Findings
• Gonadal venography: Visualization of pampiniform
plexus used to locate undescended testis
I IMAGING FINDINGS o Testicular agenesis: Absent plexus and blind-ending
vein
6 General Features
• Best diagnostic clue: Unilateral absence of spermatic Imaging Recommendations
6 cord • Best imaging tool
• Location o Ultrasound for inguinal testis
o Anywhere from kidney to inguinal canal • MRI and CT: 90-95% sensitive for intra-abdominal
o Inguinal canal most common (80%) testis
o Bilateral in 10% • Protocol advice: Oral contrast may be helpful to
• Size: Cryptorchid testis smaller than normal testis distinguish bowel from small undescended testis
• Morphology: Ovoid well-circumscribed mass
CT Findings I DIFFERENTIAL DIAGNOSIS
• CECT: Low attenuation mass above scrotum in path of
testicular descent Absent testis: Anorchia
MR Findings • Congenital or prior resection
• T1WI: Low signal intensity ovoid mass Inguinal masses
• T2WI: High signal intensity ovoid mass • Lymphadenopathy: Hypoechoic mass
• Hernia: Bowel signature; real-time motion

DDx: Inguinal Masses

-:-:-:::~·
.....
~.
a
. : •• ',.<f,..••••••• - ~
- -

...
-_.,;#

~ '\,;.:. ~-
;h_~
.. --

Inguinal Hernia Lymphadenopathy Varicocele

 CRYPTORCHIDISM
Key Facts
Terminology Clinical Issues
• Incomplete descent of testis into base of scrotum • Infertility: Impaired function of cryptorchid testis
• 1 Risk of malignant neoplasm in cryptorchid testis;
Imaging Findings also 1 risk in contralateral testis
• Best diagnostic clue: Unilateral absence of spermatic
cord Diagnostic Checklist
• Anywhere from kidney to inguinal canal • Identify mediastinum to distinguish cryptorchid
• Echogenic line of mediastinum testis testis from other inguinal masses on US

• Varicocele: Distended veins; 1 with Valsalva

.Natural History & Prognosis
• 1 Risk of malignant neoplasm in cryptorchid testis;
I PATHOLOGY also 1 risk in contralateral testis

General Features Treatment

• General path comments: Testis may be absent in 5% • Orchiopexy before age 2 to preserve fertility; surgical
• Etiology: Interruption of embryologic process of removal post-puberty
testicular descent from abdomen into scrotal sac • Complications: Torsion, infertility, malignant change,
• Epidemiology trauma
o 4% of newborns; 0.8% by age 3 months (get
spontaneous descent after birth)
o Incidence of testis cancer in men with undescended I DIAGNOSTIC CHECKLIST
testis is 1:1,000-1:2,500
Consider
• Associated abnormalities
• Absent spermatic cord on one side suggests
o Renal agenesis/ectopia, prune belly syndrome,
cryptorchidism
epispadias
o Wolffian duct anomalies: Seminal vesical cyst, Image Interpretation Pearls
ectopic ureter, etc. • Identify mediastinum to distinguish cryptorchid testis
Gross Pathologic & Surgical Features from other inguinal masses on US
• Pre-operative imaging usually not necessary; surgical 6
exploration required
• Most common tumors in cryptorchid testis:
I SELECTED REFERENCES 7
Seminoma, embryonal cell CA 1. Zagoria R]. Genitourinary Radiology 2nd ed. Philadelphia,
Mosby. 327-329, 2004
Microscopic Features 2. Shadbolt CL et al: Imaging of groin masses: inguinal
• Histologic changes in cryptorchid testis irreversible anatomy and pathologic conditions revisited.
after age 2.5 years Radiographies. 21 Spec No:S261-71, 2001
3. Nguyen HT et al: Cryptorchidism: strategies in detection.
Staging, Grading or Classification Criteria Eur Radiol. 9(2);336-43, 1999
• Types: Retractile, canalicular, abdominal, ectopic
o Retractile: Moves intermittantly between groin and
scrotal base; not truly undescended I IMAGE GALLERY
o Canalicular: Between internal and external inguinal
rings
o Abdominal: Most immediately proximal to internal
ring
o Ectopic: Remote from normal pathway of descent

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Absent testis in scrotal sac
o Infertility: Impaired function of cryptorchid testis
(Left) Axial CECT shows heterogeneous ovoid mass (arrow) adjacent
Demographics to bladder and iliac vessels. Mass proved to be a seminoma arising in
• Age: Usually diagnosed in newborns a cryptorchid testis (Courtesy H. Harvin, MO). (Right) Sagittal US
shows hypoechoic mass in inguinal area representing atrophic,
cryptorchid testis. Note echogenic mediastinum testis (arrow).
 EPIDIDYMITIS

Schematic depiction of acute epididymitis. Note swollen Sagittal color Doppler US demonstrates hyperemia of
and inflammed tail of the epididymis with adjacent enlarged epididymis (arrows), consistent with
normal testis. epididymitis.

!TERMINOLOGY Nuclear Medicine Findings

• Technetium 99m
Abbreviations and Synonyms o 90% accurate in differentiating torsion from
• Acute scrotum, orchitis, epididymo-orchitis epididymitis
Definitions o Increased flow within testicular vessels and vas
• Infectious inflammation of epididymis and/or testicle deferens on flow study
Imaging Recommendations
• Best imaging tool: Color Doppler US; high frequency
I IMAGING FINDINGS transducers optimal (:0: 10 mHz)
6 General Features • Protocol advice: Comparison with contralateral testicle
useful in subtle cases of increased vascularity
• Best diagnostic clue: Enlarged, hyperemic epididymis
8 and/or testicle on color Doppler US
• Location: Early epididymitis often involves tail of
epididymis
I DIFFERENTIAL DIAGNOSIS
• Size: Epididymis typically 2-3 times larger than normal Testicular torsion
• Morphology: Focal enlargement of tail or diffuse • Absent or diminished color Doppler flow, "twist" of
enlargement of entire epididymis spermatic cord in inguinal region
Ultrasonographic Findings • Epididymis may be enlarged but not hyperemic on
color Doppler US
• Color Doppler
o Enlarged epididymis Testicular tumor
• Diffusely or focally in tail of epididymis; increased • Focal mass on US with abnormal vessels on color
vascularity of epididymis, testicle; reactive Doppler
hydrocele with internal low level echoes
• Echotexture may be decreased or increased from
normal; Doppler flow dramatically increased
• Scrotal wall inflammation with skin thickening

DDx: Testicular lesions Mimicking Epididymitis

Testicular Torsion Testicular Tumor Segmental Infarct

 EPIDIDYMITIS

Key Facts
Terminology Top Differential Diagnoses
• Infectious inflammation of epididymis and/or testicle • Testicular torsion
• Testicular tumor
Imaging Findings • Segmental infarct
• Best diagnostic clue: Enlarged, hyperemic epididymis
and/or testicle on color Doppler US Clinical Issues
• Protocol advice: Comparison with contralateral • Prognosis excellent if treated early with antibiotics
testicle useful in subtle cases of increased vascularity • Recurrent cases can lead to fertility problems long
term

o Testicular ischemia if entrapment of testicular artery

Segmental infarct with chronic inflammation
• Acute pain, no history of trauma; focal hypoechoic • Recurrent cases can lead to fertility problems long
area avascular on color Doppler; increased incidence term
in patients with hypercoagulable states or advanced
atherosclerosis, such as diabetics Treatment
• Antibiotic therapy; follow-up scans to exclude abscess
if not improved
I PATHOLOGY • Work-up for GU anomalies in younger children and
recurrent cases
General Features
• General path comments: Infectious inflammatory
response, can lead to abscess if not treated (6%) I DIAGNOSTIC CHECKLIST
• Etiology
o Bacterial seeding occurs directly in cases with Consider
genitourinary (GU) anomalies, and presumably • Torsion if low flow to testicle
hematogenously in cases without demonstrable
anomaly Image Interpretation Pearls
o Sexually transmitted ascending GU tract infection: • Hyperemic and enlarged epididymis and/or testis
Chlamydia, E. coli, Staphylococcus aurea, mumps
virus
• Epidemiology: Most frequently seen in sexually active I SELECTED REFERENCES 6
young men; also seen in infants and boys 1. Kraus 5J et al: Genitourinary imaging in children. Pediatr
Clin North Am. 48:1381-424, 2001 9
Gross Pathologic & Surgical Features 2. Munden MM et al: Scrotal pathology in pediatrics with
• Only treated surgically if abscess forms despite sonographic imaging. CUff Probl Diagn Radiol.
antibiotic treatment 29(6):185-205,2000
3. Bukowski TP et al: Epididymitis in older boys:
Microscopic Features Dysfunctional voiding as an etiology. J Urol. 154:762-5,
• Inflammatory infiltrate of testis and epididymis 1995

Staging, Grading or Classification Criteria

• Isolated epididymitis; isolated orchitis (may be seen in I IMAGE GALLERY
boys with mumps); combined epididymitis & orchitis

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms: Acutely painful
scrotum; scrotal swelling, erythema; fever; dysuria
• Clinical profile: Positive urinalysis for WBC and
bacteria; may have elevated WBC
Demographics
• Age: Most commonly 15-35 yrs
• Gender: Male
(Left) Grayscale imaging of epididymitis. Sagittal US of testis
Natural History & Prognosis demonstrates diffuse hypoechoic enlargement of epididymis
(arrows). (Right) Sagittal US demonstrates reactive hydrocele with
• Prognosis excellent if treated early with antibiotics
multiple septations (arrow).
• Complications
o Abscess formation
 PROSTATITIS

Axial CECT shows rim-enhancing collection (arrow) in Axial CECT shows low attenuation lesion with
right lateral aspect of prostate gland representing rim-enhancement in central portion of right prostate
prostatic abscess in a patient with leukemia. lobe, consistent with abscess.

• Morphology: ABP: Diffusely enlarged, edematous

ITERMINOLOGY gland
Abbreviations and Synonyms Radiographic Findings
• Acute/chronic/nonbacterial bacterial prostatitis
• Fluoroscopy
(ABP/CBP/NBP)
o Urethrography: Compression of prostatic urethra
Definitions • Chronic: May see reflux into prostate gland
• Clinical syndrome of pelvic/perineal pain often caused • IVP: Normal, or edema of interureteric ridge
by inflammation of the prostate gland CT Findings
• Prostatodynia: Prostatitis symptoms without histologic
• CECT
evidence of prostatic inflammation
6 o Prostatodynia = chronic prostatitis-chronic pelvic
o Abscess: Rim-enhancing unilocular or multilocular
low attenuation mass in prostate
pain syndrome (CPPS)
10
• May extend through capsule into periprostatic
tissues, seminal vesicles, peritoneum
I IMAGING FINDINGS MR Findings
• T2WI
General Features o Low signal areas indistinguishable from prostate CA
• Best diagnostic clue o Abscess: Focal area of high signal intensity
o Acute: Swollen, warm and tender prostate on digital
rectal exam Ultrasonographic Findings
o Chronic bacterial: Greater than lO-fold bacterial • Real Time
growth in the expressed prostatic secretions o Transrectal ultrasound (TRUS)
• Location • Focal hypoechoic areas indistinguishable from
o Prostatitis: Peripheral gland prostate CA
o Abscess: Usually peripheral; can be central following • Hypoechoic rim surrounding gland
transurethral prostatectomy (TURP) • Hypoechoic periurethral region
o Discrete hypoechoic mass suggests abscess formation

DDx: Prostatic Pathology

Prostatic Cyst BPH Prostate Cancer

 PROSTATITIS
Key Facts
Terminology Pathology
• Clinical syndrome of pelvic/perineal pain often • Ascending colonization of urinary tract, or
caused by inflammation of the prostate gland post-biopsy
• Granulomatous: BCG therapy used for treatment of
Imaging Findings superficial bladder CA
• Abscess: Usually peripheral; can be central following
transurethral prostatectomy (TURP) Clinical Issues
• Focal hypoechoic areas indistinguishable from • Acute: Fever, chills, dysuria, frequency and pain
prostate CA • Chronic: Dysuria, urgency, pain

• Color Doppler: Increased flow in area of abscess

I CLINICAL ISSUES
Imaging Recommendations
Presentation
• Best imaging tool: CT if large abscess suspected
• Most common signs/symptoms
o Acute: Fever, chills, dysuria, frequency and pain
o Chronic: Dysuria, urgency, pain
I DIFFERENTIAL DIAGNOSIS
Demographics
Prostate carcinoma
• Age: CBP: Men aged 40-70 years
• Low signal intensity in peripheral zone on T2WI
Treatment
Congenital prostatic cysts
• Antibiotics; abscess drainage
• Usually in or near midline, or along course of
ejactulatory duct
Cystic degeneration of BPH I DIAGNOSTIC CHECKLIST
• Not "pure" water intensity
Consider
• Prostatitis is not an imaging diagnosis
I PATHOLOGY
General Features I SELECTED REFERENCES 6
• Etiology 1. Hua VN et al: Acute and chronic prostatitis. Med Clin
o Ascending colonization of urinary tract, or North Am. 88(2):483-94, 2004 11
post-biopsy 2. Zagoria R: Genitourinary radiology, 2nd ed. Philadelphia,
o Bacterial prostatitis: E. coli, pseudomonas, Mosby, rne. 211-229, 2004
enterococci most common 3. Pollack H et al: Clinical urography. 2nd ed. Philadelphia,
WB Saunders. 1058-1069,2000
o Chronic non-bacterial: Chlamydia, others
4. Nghiem HT et al: Cystic lesions of the prostate.
o Granulomatous: BCG therapy used for treatment of Radiographies. 10(4):635-50, 1990
superficial bladder CA
o Eosinophilic prostatitis, malacoplakia: Stages in
resolution of bacterial prostatitis
• Epidemiology: Worldwide prevalence: 2-10%
I IMAGE GALLERY
Microscopic Features
• Acute: Glands and ducts of the prostate contain
exudate of polymorphonuclear leukocytes
Staging, Grading or Classification Criteria
• NIH classification: Based on leukocyte (WBC) count
and culture of midstream urine specimen (VB2) and
expressed prostate secretions (EPS)
• NIH category I: + VB2 and EPS culture and WBCs;
bacteremia = ABP
• NIH category II: + VB2 and EPS culture and WBCs;
recurrent bacteruria = CBP
• NIH category IlIa: - VB2 culture and WBCs; + EPS (Left) Micropathology shows polymorphonuclear leukocytes in
WBCs = NBP = inflammatory CPPS glandular lumens (arrows) and infiltrating epithelium of glands in a
• NIB category I1Ib: - VB2 and EPS culture and WBCs = patient with acute prostatitis (Courtesy T Morgan, MO). (Right)
Micropathology shows necrotizing granuloma in lower right of
Prostatodynia = noninflammatory CPPS
prostate biopsy specimen from a patient with chronic prostatitis
• NIH category IV: +/- VB2 and EPS cultures, + VB2 and (Courtesy T Morgan, MO).
EPS WBC but without symptoms
 URETHRAL STRICTURE

Combined retrograde urethrogram and cystogram Post-operative cystogram following repair of 2 cm long
(suprapubic catheter) seems to demonstrate long stricture shows normal urethra.
urethral stricture one month following pelvic trauma.

ITERMINOLOGY o Gonorrhea: Located in bulbous portion of urethra or

immediately proximal to meatus; multifocal
Definitions o Reiter syndrome: Involves entire penile urethra
• A narrowing of urethra due to scar tissue or tumor • Voiding cystourethrography
o Evaluate anterior urethra proximal to severe stricture
Ultrasonographic Findings
I IMAGING FINDINGS • Sonourethrography
General Features o Used mainly in evaluating anterior strictures
o Greater accuracy in predicting stricture length
• Best diagnostic clue: Focal narrowing of urethra with
proximal dilation o Staging of complex or reoperative strictures: Calculi,
6 Radiographic Findings
urethral hair, false passage & stent encrustation
Imaging Recommendations
12 • Retrograde urethrography
o Focal, asymmetrical indentations • Best imaging tool
o Retrograde urethrogram and voiding urethrography
o Long segmental, tubular or beaded constrictions
o Marked narrowing to slight irregularity of urethral • Combined to evaluate proximal & distal stricture
wall closely proximal and/or distal to main stricture
o Proximal & distal to stricture is normal (acute)
o Dilatation proximal to stricture, not distal (chronic)
I DIFFERENTIAL DIAGNOSIS
o Normal cone shape of proximal bulb Urethral trauma
o Small amount of reflux into Cowper gland duct
• Stretching or narrowing; ± pelvic fractures, fragments
o Thick-walled bladder (obstruction)
• ± Extravasation from a partial or complete tear
o Iatrogenic
• Blunt trauma ~ partial or complete urethral tears ~
• Located in penoscrotal junction and membranous post-traumatic stricture
portion of urethra (most common)
• Other narrow sites (meatus and bladder neck) Urethral tumor
• Occasionally entire urethra; diffuse or multifocal • Long, irregular narrowing ± fistula

DDx: Mimics of Urethral Stricture

Trauma, Stretching Urethral Tear Urethral Tear Urethral Tear

 URETHRAL STRICTURE
Key Facts
Terminology • Retrograde urethrogram and voiding urethrography
• A narrowing of urethra due to scar tissue or tumor Top Differential Diagnoses
Imaging findings • Urethral trauma
• Best diagnostic clue: Focal narrowing of urethra with • Urethral tumor
proximal dilation Diagnostic Checklist
• Focal, asymmetrical indentations
• Clinical presentation (e.g., trauma, infection)
• Long segmental, tubular or beaded constrictions
• Evaluate proximal and distal to stricture
• Normal cone shape of proximal bulb

• Intraluminal filling defect (rare) o UroLume endourethral prosthesis

• Primary neoplasm (rare): Squamous cell carcinoma
from bulbar or bulbomembranous portions
• Secondary neoplasm, in or near urethra I DIAGNOSTIC CHECKLIST
o Transitional cell carcinoma, melanoma, sarcoma,
adenocarcinoma, Cowper gland adenocarcinoma Consider
o Metastases: Prostate, bladder, colon, rectum • Clinical presentation (e.g., trauma, infection)
Image Interpretation Pearls
I PATHOLOGY • Evaluate proximal and distal to stricture

General features
• Etiology I SELECTED REFERENCES
o Idiopathic 1. Gallentine ML et al: Imaging of the male urethra for
o Iatrogenic trauma (most common) stricture disease. Urol Clin North Am. 29(2):361-72, 2002
• Instrumentation: Cystoscopy 2. Morey AF et al: Sonographic staging of anterior urethral
• Urethral catheters: Straight, indwelling, rubber strictures. J Urol. 163(4):1070-5,2000
• Surgery: Transurethral resection of prostate, 3. Gupta S et al: Sonourethrography in the evaluation of
urethroplasty anterior urethral strictures: correlation with radiographic
urethrography. J Clin Ultrasound. 21(4):231-9, 1993
• Irradiation: Treat uterine cervical cancer 4. McCallum RW et al: The radiologic assessment of
o Chemical trauma: Injection of corrosive substances
o Infection: Gonorrhea, tuberculosis, syphilis
iatrogenic urethral injury. J Can Assoc Radiol. 36(2):122-6,
1985
6
o Inflammatory disease: Reiter syndrome, amyloidosis 5. Cass AS: Urethral injury in the multiple-injured patient. J
13
o Pathogenesis Trauma. 24(10):901-6, 1984
• Urethritis or periurethritis ~ fibrotic changes ~ 6. Singh M et al: The pathology of urethral stricture. J Urol.
stricture 115(6):673-6, 1976
7. Shaver WA et al: Changes in the male urethra produced by
instrumentation for transurethral resection of the prostate.
Radiology. 116(3):623-6, 1975
I CLINICAL ISSUES 8. Lapides J et al: Usefulness of retrograde urethrography in
\ diagnosing strictures of the anterior urethra. J Urol.
Presentation 100(6):747-50, 1968
• Most common signs/symptoms: Obstructive
symptoms: Diminished force of stream, j urine flow
rate, prolonged voiding, frequency, nocturia I IMAGE GAllERY
Demographics
• Age: Any age, but incidence 1 with age
• Gender: Male> female
Natural History & Prognosis
• Complications: Obstruction, abscess, fistula
• Prognosis: Very good
Treatment
• Use one or more procedures
o Dilatation with catheters, sounds or balloons
o Internal or external urethrotomy
o Excision of stricture with reanastomosis
o Marsupialization of urethra with urethroplasty (Left) Retrograde urethrogram shows stricture (arrow) of
o Pedicle flap repair membranous urethra. (Right) Retrograde urethrogram shows stricture
o Full-thickness skin graft urethroplasty of prostatic urethra following pelvic fracture.
 HYDROCELE

Schematic drawing of hydrocele. Note large serous fluid Transverse grayscale scan of large hydrocele. Note
collection anterior to testis in tunica vaginalis. anechoic collecUon anterior and lateral to normal tesUs
(arrow).

ITERMINOLOGY I DIFFERENTIAL DIAGNOSIS

Definitions Pyocele
• Congenital or acquired serous fluid contained within • Septated fluid with low-level echoes
layers of tunica vaginalis • Associated with epididymitis, intrascrotal abscess and
clinical signs of inflammation
Spermatocele
I IMAGING FINDINGS
• Retention cyst of tubules connecting rete testis to head
General Features of epididymis
• Best diagnostic clue: Scrotal fluid collection • Cystic mass in head of epididymis
6 surrounding testis on US except for "bare area" where • Large spermatoceles have low level echoes and
tunica vaginalis does not cover testis & is attached to septations
14 epididymis • Displacement of testis inferiorly
• Location: Tunica vaginalis, located anterolateral aspect Hematocele
of testis
• Complex echogenic fluid in tunica vaginalis on
• Morphology: Simple fluid collection
ultrasound
MR Findings • Associated with trauma, torsion and infarct
• Tl WI: Low signal fluid collection Scrotal Hernia
• T2WI: High signal consistent with serous fluid
• Bowel or echo genic omentum seen within scrotum
Ultrasonographic Findings due to indirect inguinal hernia
• Real Time: Crescentic anechoic fluid collection
surrounding testis
• Color Doppler: Avascular on color Doppler I PATHOLOGY
Imaging Recommendations General Features
• us is modality of choice • General path comments

DDx: Scrotal lesions Mimicking Hydrocele

Pyocele Spermatocele Hematocele

 HYDROCELE

Key Facts
Imaging Findings • Spermatocele
• Best diagnostic clue: Scrotal fluid collection • Hematocele
surrounding testis on US except for "bare area" where • Scrotal Hernia
tunica vaginalis does not cover testis & is attached to Clinical Issues
epididymis • Excellent with surgical repair
• US is modality of choice
Diagnostic Checklist
Top Differential Diagnoses • Anechoic fluid collection along anterolateral aspect of
• Pyocele testis

o Simple serous fluid collection within tunica

vaginalis
I DIAGNOSTIC CHECKLIST
o Chronic cases show thickened layers of tunica and Consider
septation • Spermatocele
• Etiology
o Embryology-anatomy Image Interpretation Pearls
• Congenital or communicating hydrocele due to • Anechoic fluid collection along anterolateral aspect of
failure of processus vaginalis to close testis
• Congenital occurrance in 6% of male term infants
• Secondary occurrence in adults due to
epididymitis or surgery for varicocele I SELECTED REFERENCES
• Epidemiology: 10% of testicular tumors have 1. Belman AB: Abdominoscrotal hydrocele in infancy: A
associated hydrocele review and presentation of the scrotal approach for
correction. J Urol. 165(1): 225-7, 2001
Gross Pathologic & Surgical Features 2. Kapur P et al: Pediatric hernias and hydroceles. Pediatr Clin
• Serous fluid collection North Am. 45(4): 773-9, 1998
3. Rathour DP et al: Sonography of abdomino-scrotal
Staging, Grading or Classification Criteria hydrocele. Australas Radiol. 38(4): 282-3, 1994
• Congenital: Due to incomplete closure of tunica 4. Fowler RC et al: Scrotal ultrasonography: a clinical
vaginalis evaluation. Br J Radiol. 60(715):649-54, 1987
• Acquired: Epididymitis, torsion, trauma; rarely tumor 5. Krone KD et al: Scrotal ultrasound. Radiol Clin North Am.
23(1):121-39, 1985 6
I CLINICAL ISSUES 15
I IMAGE GAllERY
Presentation
• Most common signs/symptoms
o Asymptomatic scrotal mass
o Other signs/symptoms
• Mass "transilluminates" when evaluated with light
source
• Clinical profile
o Painless scrotal mass
o Transilluminating fluid collection
o Chronic hydrocele with thick walls
• May not transilluminate
Demographics
• Age (Left) Sagittal grayscale sonogram of large hydrocele in pediatric
o All ages patient. Note fluid surrounding appendix testis (arrow). (Right)
• Pediatric patients invariably have congenital form Transverse sonogram of large hydrocele in pediatric patient. Note
of hydrocele with persistent communication with siimple fluid collection anterior to normal testis (arrow).
peritoneal cavity
• Gender: Male
Natural History & Prognosis
• Excellent with surgical repair
Treatment
• Surgical resection of hydrocele sac with oversewing of
edges
 VARICOCELE

Anatomic drawing of varicocele. On left side, note Sagittal color Doppler sonogram of left varicocele. Note
normal veins of pampiniform plexus (arrow). Right side multiple color-coded venous channels within
of image demonstrates dilated veins representing epididymis (arrow).
varicocele (open arrow).

ITERMINOlOGY Other Modality Findings

• Catheter venography via retrograde injection in
Definitions
testicular vein demonstrates dilated venous channels
• Dilatation of veins of pampiniform plexus> 2-3 mm
in diameter due to retrograde flow in internal Imaging Recommendations
spermatic vein • Best imaging tool: US with color Doppler
• Protocol advice: Resting and Valsalva color Doppler
images of epididymis
I IMAGING FINDINGS
General Features I DIFFERENTIAL DIAGNOSIS
6 • Best diagnostic clue
o Dilated serpiginous veins behind superior pole of Tubular ectasia/rete testis
16 testis on color Doppler US • Normal variant of dilated seminiferous tubules in
o Distention due to retrograde flow with Valsalva mediastinum of testis
• Location: Dilated veins in cremasteric plexus, vein of • No flow on color Doppler
vas deferens and internal spermatic vein • May be associated with spermatocele
• Size: Normally veins in pampiniform plexus are::; 2
mm; varicocele diagnosed when multiple veins are> Tumor
2-3 mm and increased in size with Valsalva • Focal intra testicular mass on grayscale images
• Morphology: Tortuous vascular channels representing • Abnormal pattern of vasculature on color Doppler
dilated veins Epididymitis
Ultrasonographic Findings • Enlarged hypoechoic epididymis with increased flow
• Real Time: Tubular serpiginous vessels posterior to on color Doppler
testis on grayscale
Testicular torsion
• Color Doppler: Prominent color flow within vessels
with Valsalva due to retrograde flow • Absent or decreased flow to testis on color Doppler
• Hypoechoic testis in late stage

DDx: Testicular lesions Mimicking Varicocele

Rete Testis Testicular Tumor Epididymitis Torsion

 VARICOCELE

Key Facts
Terminology Pathology
• Dilatation of veins of pampiniform plexus> 2-3 mm • Most frequent cause of male infertility
in diameter due to retrograde flow in internal
spermatic vein Clinical Issues
• Clinical profile: Majority (80%) are left-sided; bilateral
Imaging Findings in 15% of patients
• Best imaging tool: US with color Doppler
• Protocol advice: Resting and Valsalva color Doppler Diagnostic Checklist
images of epididymis • Left renal vein occlusion by tumor in elderly male

• Surgical ligation
I PATHOLOGY
General Features
• General path comments: Dilated veins within I DIAGNOSTIC CHECKLIST
pampiniform plexus Consider
• Etiology
• Left renal vein occlusion by tumor in elderly male
o Primary: Incompetent venous valve
o Secondary: Venous obstruction or invasion of left Image Interpretation Pearls
renal vein by renal mass, nodes or adrenal tumor • Valsalva essential for diagnosis of small varicoceles
• Epidemiology
o 10-15% of men in US have varicoceles
o Subclinical varicocele in 40-75% of infertile men I SELECTED REFERENCES
o Most frequent cause of male infertility
1. Forti G et al: Varicocele and infertility. J Endocrinol Invest.
• Associated abnormalities
26(6):564-9, 2003
o Low sperm count 2. Evers JL et al: Assessment of efficacy of varicocele repair for
o Secondary (reactive) varicoceles caused by male subfertility: a systematic review. Lancet.
retroperitoneal mass obstructing testicular veins 361(9372):1849-52, 2003
3. Naughton CK et al: Pathophysiology of varicoceles in male
Gross Pathologic & Surgical Features infertility. Hum Reprod Update. 7(5): 473-81, 2001
• Dilated veins within pampiniform plexus 4. Munden MM et al: Scrotal pathology in pediatrics with

Staging, Grading or Classification Criteria

sonographic imaging. Curr Probl Diagn Radiol. 29(6):
185-205,2000
6
• Primary 5. Cornud F et al: Varicocele: Strategies in diagnosis and 17
o Idiopathic (incompetent valves) - 98% on left treatment. Eur Radiol. 9(3): 536-45, 1999
o Most common cause of correctable infertility
• Secondary
o Obstruction of left renal vein by mass or renal tumor I IMAGE GALLERY

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Infertility
o Vague scrotal discomfort or pressure, primarily
when standing
• Clinical profile: Majority (80%) are left-sided; bilateral
in 15% of patients
Demographics
• Age: > 15 yrs (Left) Grayscale sagittal sonogram of varicocele. Note hypoechoic
• Gender: Male dilated venous channels within epididymis (arrow). (Right) Sagittal
color Doppler sonogram of varicocele. Note numerous color-coded
Natural History & Prognosis veins along spermatic cord (arrow).
• Excellent prognosis in treated cases
• Results for increased fertility have been mixed, may
not be as effective as previously thought
Treatment
• Catheter embolization if symptomatic or causing low
sperm count
 TESTICULAR TORSION

Anatomic drawing of testicular torsion. Note twisted Transversecolor Doppler sonogram of testicular torsion.
cord (arrow) and enlarged epididymis (open arrow). Note normal flow to left testis (arrow) and absence of
flow to the ischemic right testis.

• Color Doppler: Absent or decreased flow; sensitivity

ITERMINOLOGY 80-90%; 10% of patients with early or partial torsion
Abbreviations and Synonyms have normal exam
• Torsion, late or "missed" torsion, acute scrotum Nuclear Medicine Findings
Definitions • Tc99m pertechnetate: Dynamic flow imaging at 2-5
• Spontaneous or traumatic twisting of testis & second intervals for 1 minute (vascular phase); 5
spermatic cord within scrotum, resulting in vascular minute intervals for tissue phase; sensitivity 80-90%
occlusion/infarction Imaging Recommendations
• Best imaging tool: US with high-frequency linear
6 I IMAGING FINDINGS transducer & color Doppler; power Doppler preferable
• Protocol advice: Power Doppler with comparison to
General Features contralateral normal testis
18
• Best diagnostic clue: Decreased or absent blood flow to
testicle on color Doppler US
• Location: Unilateral in 95% of patients
I DIFFERENTIAL DIAGNOSIS
Ultrasonographic Findings Epididymo-orchitis
• Real Time • Enlarged hypoechoic epididymis with increased flow
o May be entirely normal on color Doppler
o Enlarged testicle and epididymis, heterogeneous Testicular tumor
echotexture, most often decreased echogenicity; • Focal mass on US; abnormal flow within tumor
secondary hydrocele
o Intratesticular necrosis, hemorrhage or Testicular trauma
fragmentation if delayed diagnosis • Hematocele, irregular contours, heterogeneous
o "Spiral" twist of spermatic cord from inguinal canal parenchymal echogenicity
to testis

DDx: Testicular lesions Mimicking Torsion

Carcinoma Epididymo-Orchitis Testicular Trauma

 TESTICULAR TORSION

Key Facts
Imaging Findings • Testicular trauma
• Best diagnostic clue: Decreased or absent blood flow Clinical Issues
to testicle on color Doppler US • Clinical profile: Male child with acute scrotal pain
• Best imaging tool: US with high-frequency linear
transducer & color Doppler; power Doppler preferable Diagnostic Checklist
• Pitfall of normal color Doppler flow in early or partial
Top Differential Diagnoses
torsion; normal US doesn't exclude early torsion
• Epididymo-orchitis
• Testicular tumor

I PATHOLOGY Treatment
• Surgical exploration; de-torsion; bilateral orchidopexy
General Features
if viable testicle
• General path comments o Non-viable testicle usually removed; higher risk of
o Varying degrees of ischemic necrosis & fibrosis subsequent torsion on contralateral side
depending on duration of symptoms
o Embryology-anatomy: Deficient testicular fixation
related to tunica vaginalis & gubernaculum ("bell I DIAGNOSTIC CHECKLIST
clapper" deformity); testicle rotates within scrotum
and twists spermatic cord Consider
• Etiology: Most occur spontaneously; rarely occurs • Pitfall of normal color Doppler flow in early or partial
traumatically torsion; normal US doesn't exclude early torsion
• Epidemiology: Infant & adolescent boys most often
affected Image Interpretation Pearls
• Decreased or absent flow on power Doppler
Gross Pathologic & Surgical Features
• Purple, edematous, ischemic testicle, may rapidly
re-perfuse when manually de-torsed I SELECTED REFERENCES
Microscopic Features 1. Mernagh]R et al: Testicular torsion revisited. CUff Probl
Diagn Radiol. 33(2):60-73, 2004
• Hemorrhagic, interstitial edema; necrosis
2. Dogra VS et al: Sonography of the scrotum. Radiology. 6
Staging, Grading or Classification Criteria 227(1):18-36,2003
• Previously classified acute, subacute, or delayed based 3. Kravchick S et al: Color Doppler sonography: its real role in 19
the evaluation of children with highly suspected testicular
on duration of symptoms
torsion. Em Radiol. 11(6):1000-5, 2001

I CLINICAL ISSUES I IMAG E GALLERY

Presentation
• Most common signs/symptoms
o Acute scrotal/inguinal pain; swollen, erythematous
hemiscrotum without recognized trauma
o Other signs/symptoms: Low grade torsion may be
tolerated for long periods
• Clinical profile: Male child with acute scrotal pain
Demographics
• Age: < 15 yrs
• Gender: Male
Natural History & Prognosis
• Surgical emergency: Testicular infarction if not treated (Left) Color Doppler sonogram of false-negative study for torsion.
promptly Note preserved flow to the testis on longitudinal scan. At surgery,
• Testicular viability depends on degree of torsion (> testicle was twisted 780 and was only minimally ischemic. (Right)
0

540 worse), duration of symptoms & rapid surgical

0
Twisted spermatic cord on sagittal grayscale US image in testicular
intervention torsion. Note "mass" (arrow) near upper pole of testis representing
• Unilateral testicular loss typically does not lead to twisted spermatic cord and thickened epididymis due to torsion.
infertility problems
 BENIGN PROSTATIC HYPERTROPHY

BPH on IVP.Note extrinsic impression (open arrow) on BPH on axial CECT. Note medial lobe hypertrophy of
base of bladder with "fish hooking" of left ureter prostate pressing on base of bladder (arrows).
(arrow).

ITERMINOlOGY CT Findings
• NECT: Enlarged prostate; calcifications when prostate
Abbreviations and Synonyms
enlarged
• Benign prostatic hypertrophy (BPH), nodular
• CECT: Enlarged prostate with extrinsic impression on
hyperplasia
base of bladder
Definitions MR Findings
• Enlargement of prostate from benign hyperplasic
• T1WI: Enlarged prostate
nodule (fibromyoadenomatous nodule)
• T2WI: Low or heterogeneous signal nodular adenoma
involving transition or periurethral zone; cannot
6 I IMAGING FINDINGS
distinguish BPH from carcinoma
• T1 C+
20 General Features o Data mixed on whether dynamic contrast
enhancement may aid in differentiating BPH from
• Best diagnostic clue: Enlarged prostate on CT, US or
carcinoma
MRI with nodular hypertrophy in transitional or
o Carcinoma in general has more rapid uptake of
periurethral zone
gadolinium on dynamic MRI
• Location: Transition zone and periurethral zone
proximal to verumontaneum; "lateral lobe" = 82%, Ultrasonographic Findings
median lobe = 12% • Real Time
• Size: Variable; may be up to 10-12 cm o 80% demonstrate hypo echoic nodular enlargement
• Morphology: Rounded or lobulated soft tissue of transition and periurethral zones; 10-20% of
hypertrophy; nodules typically 60-100 gm hyperplastic nodules are isoechoic or echogenic
Radiographic Findings o Large residual urine volume post-voiding
• IVP: Extrinsic impression on base of bladder with "J Imaging Recommendations
hooking" or "fish hooking" of distal ureters • Best imaging tool: MRI, endorectal US
• Protocol advice: MRI: T2 axial and coronal images

DDx: Prostatic lesions Mimicking BPH

~~

,.-.;'-"'~
1I'
::~
~
~- - \ .

- - .

Prostate Carcinoma Bladder Carcinoma Prostate Abscess

 BENIGN PROSTATIC HYPERTROPHY

Key Facts
Terminology Top Differential Diagnoses
• Enlargement of prostate from benign hyperplasic • Prostate carcinoma
nodule (fibromyoadenomatous nodule) • Prostatic abscess
• Bladder carcinoma
Imaging Findings
• Best diagnostic clue: Enlarged prostate on CT, US or Pathology
MRI with nodular hypertrophy in transitional or • Epidemiology: 70% of men have BPH by age 70; 80%
periurethral zone of men have BPH by age 80

I DIFFERENTIAL DIAGNOSIS Natural History & Prognosis

• May progress to BOO, hydronephrosis if severe
Prostate carcinoma
• May lead to urinary infection, gross hematuria
• Typically involves peripheral zone (70%); hypoechoic
on endorectal US with increased color Doppler flow; Treatment
low signal mass on T2 MRI • Options, risks, complications: Open surgery for gland
> 80 gm; transurethral resection for smaller glands;
Prostatic abscess
medical therapy with alpha-adrengeric blockers,
• Low attenuation fluid collection bulging capsule finasteride for mild symptoms
Bladder carcinoma
• Posterior epithelial mass may simulate median lobe as
polypoid mass I DIAGNOSTIC CHECKLIST
Consider
• Prostate carcinoma
I PATHOLOGY
Image Interpretation Pearls
General Features
• Median lobe hypertrophy simulating bladder mass
• General path comments: Firm hypertrophied tissue
• Etiology: Stromal hyperplasia stimulated by normal
action of dehydrotestosterone and growth factors
(fibroblast growth factor, insulin-like growth factor)
I SELECTED REFERENCES 6
• Epidemiology: 70% of men have BPH by age 70; 80% 1. Tubaro A et al: Investigation of benign prostatic
hyperplasia. CUff Opin Urol. 13(1):17-22,2003 21
of men have BPH by age 80
2. Grossfeld GO et al: Benign prostatic hyperplasia: clinical
• Associated abnormalities: Bladder wall hypertrophy overview and value of diagnostic imaging. Radiol Clin
with trabeculation and diverticula North Am. 38(1):31-47, 2000
3. Aarnink RG et al: Aspects of imaging in the assessment and
Gross Pathologic & Surgical Features
follow up of benign prostatic hyperplasia. CUff Opin Urol.
• Enlarged, firm gland at prostatectomy 9(1):21-9, 1999
Microscopic Features
• Hyperplastic nodules due to glandular proliferatIon
and/or fibrous or muscular proliferation of stroma I IMAGE GALLERY

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Symptoms of retention and distended bladder;
nocturnal dribbling, poor urethral stream
o Other signs/symptoms: Symptom severity doesn't
correlate strongly with size of gland on imaging
• Clinical profile: Acute retention with bladder outlet
obstruction (BOO); enlarged prostate on rectal exam;
may have elevated prostate-specific antigen
(Left) Pre-void transverse sonogram of BPH. Note enlarged
Demographics hypoechoic prostate gland (arrows). (Right) Sagittal post-void
• Gender: Male sonogram demonstrates no significant change in bladder volume,
consistent with bladder outlet obstruction from BPH.
 SCROTAL TRAUMA

Grayscale sagittal sonogram of testicular fracture. Note Sagittal power Doppler sonogram of testicular rupture
hypoechoic fracture traversing testis (arrows). with intraparenchymal hematoma. Note rounded
hypoechoic hematoma (arrows) that is avascular.

o Abnormal echogenicity of testicular parenchyma

ITERMINOlOGY o Epididymal enlargement
Abbreviations and Synonyms o Discrete linear or irregular fracture plane within
• Testicular rupture, fracture of testis testis (17%)
o Focal intraparenchymal testicular hematoma
Definitions • Color Doppler
• Laceration of tunica albugines, extrusion of testicular o Distorted intraparenchymal vascularity with
tissue into scrotal sac interruption of vessels in area of injury
o Avascular intraparenchymal hematoma
o Post-traumatic epididymitis may demonstrate
I IMAGING FINDINGS enlarged epididymis with increased flow
6 General Features Imaging Recommendations
22 • Best diagnostic clue: Heterogeneous parenchymal • Best imaging tool: High-resolution US (:2: 7.5 mHz)
echogenicity of testis on sonography
• Morphology: Irregularity of testicular contour
I DIFFERENTIAL DIAGNOSIS
Radiographic Findings
• Radiography: May have associated pelvic fractures Testicular tumor
• Mild to moderate pain with palpable testicular mass
MR Findings but no history of trauma
• Tl WI: High signal acute hematoma • Focal grayscale abnormality with abnormal interval
• T2WI: High signal hematoma vascularity, vascular displacement on color Doppler
• T1 C+: Areas of injury are avascular post-contrast
Epididymo-orchitis
Ultrasonographic Findings • Acute or chronic pain without history of trauma
• Real Time • Enlarged hypo echoic epididymis with increased
o Hematocele with echo genic fluid, linear stranding in vascularity to epididymis and testis on color Doppler
tunica vaginalis

DDx: Testicular lesions Mimicking Scrotal Trauma

I

Testicular Tumor Epididymo-orchitis Testicular Torsion Segmental Infarct

 SCROTAL TRAUMA
Key Facts
Imaging Findings • Unless repaired within 72 hours, salvage rate only
• Best diagnostic clue: Heterogeneous parenchymal 45%
echogenicity of testis on sonography Diagnostic Checklist
• Best imaging tool: High resolution US ("2: 7.5 mHz) • Consider isolated hematocele without rupture
Clinical Issues • Irregularity of testicular contour; heterogeneous
• Most common signs/symptoms: Acute scrotal testicular parenchyma
hematoma following blunt trauma

Testicular torsion I DIAGNOSTIC CHECKLIST

• Decreased vascularity compared to normal testis
Consider
Segmental infarct • Consider isolated hematocele without rupture
• Acute pain, no history of trauma
• Focal hypoechoic area avascular on color Doppler
Image Interpretation Pearls
• Increased incidence in patients with hypercoagulable • Irregularity of testicular contour; heterogeneous
states or advanced atherosclerosis, such as diabetics testicular parenchyma

I PATHOLOGY I SELECTED REFERENCES

1. Micallef M et al: Ultrasound features of blunt testicular
General Features injury. Injury. 32(1):23-6, 2001
• General path comments 2. Wessells H et al: Testicular trauma. Urology. 47(5):750,
o Capsular disruption; hematocele 1996
o Necrotic testicular parenchyma extrudes into tunica 3. Simmons MZ: Re: Accuracy of ultrasound diagnosis after
blunt testicular trauma. J Urol. 152(3):968-9, 1994
vaginalis
4. Patil MG et al: The value of ultrasound in the evaluation of
• Etiology: Blunt trauma impales scrotal contents to patients with blunt scrotal trauma. Injury. 25(3):177-8,
symphysis pubis or pubic rami 1994
• Associated abnormalities: Pelvic fracture 5. Altarac S: Management of 53 cases of testicular trauma. Eur
Microscopic Features
Urol. 25(2):119-23, 1994 6
• Intraparenchymal hemorrhage and necrotic lacerated 23
tissue
I IMAGE GALLERY
Staging, Grading or Classification Criteria
• Extratesticular: Injury to scrotal wall, tunica vaginalis
or tunical & testicular parenchyma
• Intratesticular: Injury to tunical and testicular
parenchyma

ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Acute scrotal
hematoma following blunt trauma
Demographics (Left) Testicular fracture with large hematoma. Transverse grayscale
• Age: < 50 yrs image of scrotum demonstrates large complex fluid collection with
• Gender: Male septations representing hematocele (arrow). (Right) Testicular
fracture. Sagittal grayscale sonogram demonstrates large hypoechoic
Natural History & Prognosis area representing intraparenchymal hematoma (arrow) with adjacent
• Unless repaired within 72 hours, salvage rate only 45% hematocele (open arrow).

Treatment
• Options, risks, complications: Surgery mandatory
 GONADAL STROMAL TUMORS

Sagittal US shows small, round, well circumscribed Axial color Doppler US in a middle-aged man shows a
hypoechoic mass at upper pole of testis representing hypoechoic mass with internal color flow, which proved
Leydig cell tumor (Courtesy B.F. King, MDJ. to be Leydig cell tumor at pathology

o High signal intensity foci secondary to central scars

ITERMINOlOGY
Ultrasonographic Findings
Abbreviations and Synonyms
• Real Time
• Gonadal stromal tumors (GSTs) = nongerm cell tumors o Indistinguishable from germ cell tumors
= interstitial cell tumors = sex cord tumors o LCT: Small solid hypoechoic intra testicular mass
Definitions • Larger tumors: Hemorrhage or necrosis
o SCT: Solid and cystic components, +/~calcification
• Neoplasms arising from non-germ cell elements
• Leydig cell tumors (LCT): Arise from interstitial cells • Color Doppler: Internal/perinodular flow
• Sertoli cell tumors (SCT): Arise from sustentacular cells Imaging Recommendations
lining seminiferous tubules
6 • Granulosa cell tumor: Rare benign tumor
• Best imaging tool: Ultrasound
• Gonadoblastoma: Contains both stromal and germ
24 cell elements I DIFFERENTIAL DIAGNOSIS
Testicular germ cell tumors
I IMAGING FINDINGS • Indistinguishable from stromal tumors on US
General Features Testicular metastases, lymphoma, leukemia
• Location: Bilateral in 3% • Often multiple; otherwise indistinguishable
• Size
o Usually < 3 cm Focal orchitis
o Malignant tumors: Usually> 5 cm • Symptomatic with pain, tenderness
• Morphology: Well-circumscribed, round-lobulated Intratesticular hematoma
MR Findings • No internal color flow
• T2WI
o Low signal intratesticular mass +/- high signal
fibrous capsule rim

DDx: Intratesticular Masses

Germ Cefl Tumor Lymphoma Hematoma

 GONADAL STROMAL TUMORS

Key Facts
Terminology Pathology
• Leydig cell tumors (LCT): Arise from interstitial cells • Represent 4-5% of all testis tumors; 10-30% occur in
• Sertoli cell tumors (SCT): Arise from sustentacular childhood
cells lining seminiferous tubules • Leydig cell tumors: 90% benign
• Sertoli cell tumors (SCT): 85-90% benign
Imaging Findings
• Indistinguishable from germ cell tumors Clinical Issues
• Precocious virilization in children
• Gynecomastia, impotence, I libido in adults

I PATHOLOGY Demographics
General Features • Age
o LCT: Age 30-60; 25% occur before puberty
• General path comments
o Malignant Leydig cell tumors only occur in adults
o Represent 4-5% of all testis tumors; 10-30% occur in
o SCT: All age groups; 1/3 < age 12
childhood
• Leydig cell tumors: 3% of all testis tumors; most Natural History & Prognosis
common stromal tumor • Malignant tumors metastasize in same pattern as
• Sertoli cell tumors: 1% of all testis tumors testicular germ cell tumors
o Leydig cell tumors: 90% benign
• May produce testosterone Treatment
• Malignant potential difficult to assess • Orchiectomy or testis-sparing surgery
histologically
• Best criterion for malignancy: Presence of
metastases I DIAGNOSTIC CHECKLIST
o Sertoli cell tumors (SCT): 85-90% benign
• May produce estrogen or Mullerian inhibiting Image Interpretation Pearls
factor • Consider stromal tumor in ddx of patient with
• Epidemiology: Represent 10-13% of testicular endocrinopathy and intratesticular mass
neoplasms in pediatrics
• Associated abnormalities
o Leydig cell tumor: Kleinfelter syndrome I SELECTED REFERENCES 6
o SCT: Large cell calcifying subtype associated with 1. Fernandez GC et al: MRI in the diagnosis of testicular 25
Peutz-]egher syndrome and Carney syndrome Leydig cell tumour. Br] Radiol. 77(918):521-4,2004
2. Dogra VS et al: Sonography of the scrotum. Radiology.
Gross Pathologic & Surgical Features 227(1):18-36, 2003
• Well-circumscribed, solid, lobulated intratesticular 3. Woodward P] et al: From the archives of the AFIP: tumors
mass and tumorlike lesions of the testis: radiologic-pathologic
correlation. Radiographies. 22(1):189-216, 2002
Microscopic Features
• Tumors may have single or multiple cell types
• LCT: Large cells with central round nuclei, small jlMAGE GALLERY
nucleoli and eosinophilic cytoplasm
o Crystals of Reinke in 30-40% = rod-shaped,
intracytoplasmic inclusion bodies
• SCT: Sheets of uniform cells with areas of tubule
formation

I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Painless testicular enlargement
o 30% of patients have endocrinopathy secondary to
testosterone or estrogen production by tumor (Left) Pathology shows sheet-like nested pattern of Leydig cell tumor.
• Precocious virilization in children Cells are polygonal with eosinophilic cytoplasm and round nuclei.
• Gynecomastia; impotence, I libido in adults Crystals of Reinke are absent in this case (Courtesy T. Morgan, MDJ.
• Clinical profile: Malignant Leydig cell tumors: Elderly (Right) Sagittal color Doppler US shows prominent vessel and
men internai'color flow in a small, round, hypoechoic intratesticular mass.
Pathology confirmed Leydig cell tumor (Courtesy B.F. King, MDJ.
 TESTICULAR CARCINOMA

Schematic drawing of testicular carcinoma. Note Sagittal grayscale sonogram of pure seminoma. Note
heterogenous, solid mass arising from testicular uniform hypoechoic solid mass (arrows).
parenchyma and distending tunica albuginea.

ITERMINOlOGY MR Findings
• T2WI: Moderate high signal intensity
Abbreviations and Synonyms lymphadenopathy in retroperitoneum
• Germ cell tumor of testis
Ultrasonographic Findings
Definitions
• Real Time
• Malignant germ cell tumor of testis o Seminoma
• Hypoechoic, well-defined solid mass without
cystic areas, calcification or invasion
I IMAGING FINDINGS o Embryonal cell carcinoma
• Heterogeneous predominantly solid mass of
6 General Features
• Best diagnostic clue: Discrete hypoechoic or mixed
mixed echogenicity
• Poorly marginated
26 echo genic mass on us
• 1/3 have cystic areas
CT Findings • Invasion of tunica may distort contour of testis
o Teratoma/teratocarcinoma
• CECT
o Low attenuation nodes • Heterogenous with mixed echogenicity
o Even nodes < 1 em suspicious if located in typical • Cystic areas and calcification (cartilage, immature
drainage areas; left renal hilus and retrocaval bone, scarring or fibrosis)
location on right o Choriocarcinoma
o Residual low attenuation masses common in bulky • Heterogeneous with mixed echogenicity
disease • Cystic areas with calcification common
o Useful for staging retroperitoneal, nodal and • Color Doppler
pulmonary metastases o Variable vascularity; disorganized flow is typical
o Helpful to identify retroperitoneal recurrence and/or o Cystic areas are avascular
"growing teratoma" syndrome . Nuclear Medicine Findings
• PET

DDx: Testicular lesions Mimicking Carcinoma

Epidermoid Cyst Lymphoma Testicular Hematoma Segmental Infarct

 TESTICULAR CARCINOMA

Key Facts
Terminology Pathology
• Malignant germ cell tumor of testis • Etiology-pathogenesis: Associated with
cryptorchidism, previous contralateral cancer;
Imaging Findings possible association with mumps orchitis,
• Best imaging tool: US to identify and characterize microlithiasis and family history of tumor
scrotal mass; CT or MR for initial staging; PET to • Most common cancer in men aged 15-34
evaluate post-treatment residual masses
• Protocol advice: High frequency (~ 10 mHz) linear Clinical Issues
array transducer • Clinical profile: Young male with palpable testicular
mass, elevated tumor markers such as beta-HCG,
Top Differential Diagnoses alfa-feto- protein
• Epidermoid cyst
• Lymphoma Diagnostic Checklist
• Subacute hematoma • Consider testicular lymphoma if bilateral lesions
• Segmental Infarct identified, particularly if patient is > 50 yrs
• Focal orchitis • Discrete mass on grayscale with abnormal intrinsic
vessels on color Doppler

o Helpful to reduce false-negative CT • Reactive hydrocele with low level echoes

• May aid in differentiating residual tumor from • Scrotal wall thickening
scar in treated patients • Enlarged epididymis
Imaging Recommendations
• Best imaging tool: US to identify and characterize I PATHOLOGY
scrotal mass; CT or MR for initial staging; PET to
evaluate post-treatment residual masses General Features
• Protocol advice: High frequency (~ 10 mHz) linear • General path comments
array transducer o 95% of testicular tumors are malignant germ cell
tumors
o Single histologic subtype in 65% of tumors
I DIFFERENTIAL DIAGNOSIS (seminoma is the most common)
Epidermoid cyst
o Multiple subtypes in 35%
• Teratoma and embryonal cell (teratocarcinoma)
6
• Cystic cavity lined by stratified squamous epithelium • Seminoma and embryonal cell
• "Onion skin" appearance on US due to alternating 27
• Seminoma and teratoma
layers of keratin and desquamated squamous cells o 40-50% are seminoma
• May have peripheral calcified rim o 25% have embryonal subtype (often mixed with
• No enhancement on MRI other subtypes)
lymphoma o 5-10% are teratomas
• Older age group o Etiology-pathogenesis: Associated with
• Most common tumor of testes in men> 60 years cryptorchidism, previous contralateral cancer;
• 50% of cases bilateral possible association with mumps orchitis,
• Often multiple lesions microlithiasis and family history of tumor
• Hypoechoic and hypervascular on color Doppler • Genetics: Family history increases risk
• Epidemiology
Subacute hematoma o Most common cancer in men aged 15-34
• History of trauma o 1% of all cancers in men
• Associated hematocele o Seminomas most common in men 35-39 years old
• Hypoechoic on US o Seminomas rare before 10 years and after 60 years
o Lymphoma most common tumor over 50 years
Segmental Infarct • Associated abnormalities: Gynecomastia,
• Acute pain most common symptom pre-pubescent virilization
• Focal avascular area on color Doppler
• Infarct is typically hypo echoic Gross Pathologic & Surgical Features
• No palpable mass • Solid or solid/cystic intratesticular mass
• 10-15% have epididymis or spermatic cord
Focal orchitis involvement
• Irregular hypoechoic area on US • Bilateral in 2-3% of cases
• Increased vascularity on color Doppler without
displacement of vessels
• Signs of systemic infection
 TESTICULAR CARCINOMA
molecular determinants of oncogenesis and treatment
Microscopic Features success. Eur] Cancer. 39(2):147-56, 2003
• Malignant germ cell tumors with numerous histologic 5. Deshpande MS et al: Metastatic adenocarcinoma in testis
subtypes presenting as a testicular mass--a case report and review of
literature. Can] Urol. 10(1):1770-1,2003
Staging, Grading or Classification Criteria 6. Hussain A et al: The unsuspected non palpable testicular
• Stage I (A): Tumor confined to testis mass detected by ultrasound: a management problem. Can
• Stage II (B): Tumor metastatic to nodes below ] Urol. 10(1):1764-6,2003
7. Bach AM et al: Is there an increased incidence of
diaphragm
contralateral testicular cancer in patients with
• Stage IIA (B1): Retroperitoneal node enlargement <2 intratesticular microlithiasis? A]R Am] Roentgenol.
cm (5 cm3) 180(2):497 -500, 2003
• Stage lIB (B2): Retroperitoneal node enlargement <2 8. Hellerstedt BA et al: Germ cell tumors: review of selected
cm x < 5 cm (10 cm3) studies from 2002. Curr Opin Oncol. 15(3):234-8, 2003
• Stage IIC (B3): Retroperitoneal node enlargement> 5 9. Rajpert-De Meyts E et al: Testicular neoplasia in childhood
cm and adolescence. Endocr Dev. 5:110-23, 2003
• Stage III (C): Tumor metastatic to lymph nodes above 10. Huyghe E et al: Increasing incidence of testicular cancer
worldwide: a review.] Urol. 170(1):5-11,2003
diaphragm
11. Hain SF et al: Positron emission tomography for urological
• Stage IlIA (C1): Metastases confined to lymphatic tumours. B]U Int. 92(2):159-64, 2003
• Stage I1IB or IV: Extranodal metastases 12. Patel MI et al: Management of recurrence and follow-up
strategies for patients with seminoma and selected
high-risk groups. Urol Clin North Am. 30(4):803-17, 2003
IClINICAllSSUES 13. Jewett MA et al: Management of recurrence and follow-up
strategies for patients with nonseminoma testis cancer.
Presentation Urol Clin North Am. 30(4):819-30, 2003
14. Scholz M et al: Stage I testicular cancer. Curr Opin Urol.
• Most common signs/symptoms
13(6):473-6,2003
o Palpable mass in testis
15. Vergouwe Y et al: Predictors of occult metastasis in clinical
o Dull pain (27%) stage I nonseminoma: a systematic review. ] Clin Oncol.
o Acute pain (10%) 21(22):4092-9,2003
• Clinical profile: Young male with palpable testicular 16. Jones RH et al: Part II: testicular cancer--management of
mass, elevated tumor markers such as beta-HCG, advanced disease. Lancet Oncol. 4(12):738-47, 2003
alfa- feto- protein 17. Jones RH et al: Part I: testicular cancer--management of
early disease. Lancet Oncol. 4(12):730-7, 2003
Demographics 18. Woodward P] et al: From the archives of the AFIP: tumors
• Age: 15-40 yrs and tumorlike lesions of the testis: radiologic-pathologic
correlation. Radiographies. 22(1):189-216, 2002
• Gender: Male
6 • Ethnicity: Increased incidence in Caucasian and
19. Albers P et al: Positron emission tomography in the clinical
staging of patients with Stage I and II testicular germ cell
Jewish males tumors. Urology. 53(4):808-11, 1999
28 20. Lawton A] et al: Staging and prognostic factors in testicular
Natural History & Prognosis
cancer. Semin Surg Oncol. 17(4):223-9, 1999
• 95% 5-year survival rate overall 21. Foster RS et al: Testicular cancer: what's new in staging,
prognosis, and therapy. Oncology (Huntingt).
Treatment 13(12):1689-94; discussion 1697-700, 1703, 1999
• Radical orchiectomy; retroperitoneal node dissection 22. Bender H et al: Possible role of FDG-PETin the evaluation
for non-seminomatous tumor of urologic malignancies. Anticancer Res. 17(3B):1655-60,
• XRT or chemotherapy for metastatic disease 1997
23. Leibovitch L et al. Improved accuracy of computerized
tomography based clinical staging in low stage
I DIAGNOSTIC CHECKLIST nonseminomatous germ cell cancer using size criteria of
retroperitoneal lymph nodes.] Urol. 154(4):1759-63, 1995
24. Newhouse ]H: Clinical use of urinary tract magnetic
Consider
resonance imaging. Radiol Clin North Am. 29(3):455-74,
• Consider testicular lymphoma if bilateral lesions 1991
identified, particularly if patient is > 50 yrs 25. Heiken]P et al: Testicular tumors: oncologic imaging and
diagnosis. Int] Radiat Oncol BioI Phys. 10(2):275-87, 1984
Image Interpretation Pearls
• Discrete mass on grayscale with abnormal intrinsic
vessels on color Doppler

I SELECTED REFERENCES
1. Boyle P: Testicular cancer: the challenge for cancer control.
Lancet Oneal. 5(1):56-61, 2004
2. Owens E] et al: Imaging of testicular tumours. Hosp Med.
65(1):18-21, 2004
3. MacVicar GR et al: Testicular cancer. Curr Opin Oncol.
16(3):253-6, 2004
4. Jones RH et al: New directions in testicular cancer:
 TESTICULAR CARCINOMA

I IMAGE GALLERY
Typical
(Left) Sagittal grayscale
sonogram of testicular
seminoma. Note
well-defined hypoechoic
mass (arrows). (Right)
Sagittal color Doppler
sonogram of testicular
seminoma. Note internal
flow within mass and
disorganized pattern of
vascularity (arrows).

Typical
(Left) Embryonal cell
carcinoma of the testis on
sagittal sonogram. Note
ill-defined hypoechoic mass
(arrows). (Right) Sagittal
color Doppler sonogram
demonstrates increased flow
within mass (arrow).

6
29

Typical
(Left) Choriocarcinoma on
sagittal color Doppler
sonogram. Note hypoechoic
mass (arrows) with internal
flow. (Right) Mixed-cell type
testicular cancer on sagittal
sonogram. Note cystic
component (arrow) to mixed
seminoma and embryonal
cell carcinoma.
 PROSTATE CARCINOMA

Axial CECT shows enlarged prostate with extensive Axial CECT shows extensive pelvic lymphadenopathy
malignant lymphadenopathy. from prostate cancer.

o Most important factor affecting prognosis & choice

ITERMINOlOGY of treatment is presence or absence of extracapsular
Definitions extension (ECE)
• Malignancy of prostate gland o Role of diagnostic imaging in prostate cancer
remains unclear
o Imaging accuracy for local staging suboptimal
[IMAGING FINDINGS • Better for advanced disease & metastases
o 3D MR spectroscopy (1 choline & ! citrate levels) +
General Features endorectal MR imaging 1 accuracy in detecting &
• Best diagnostic clue: !Signal in a normally high signal staging of local + ECE of prostate cancer
peripheral zone on T2WI o Histopathological types of prostate carcinoma
6 • Location • Adults: 95% adenocarcinoma
o Peripheral zone (70%): Posterior region (> common) • Children: Rhabdomyosarcoma
30 o Transition zone (20%), central (10%) o Spread
o Typical bony metastases (pelvis & lower vertebrae) • Hematogenous: Prostatic venous plexus drains
• Other general features into internal iliac veins & communicates with
o Most common noncutaneous cancer in American vertebral venous plexus (osteoblastic metastases)
men • Lymphatic: Drains mostly to internal iliac nodes,
o 2nd most common cause of male cancer deaths in then to retroperitoneal nodes
US (after lung cancer) CT Findings
o Lifetime risk of developing prostate cancer is 18-19%
• Not accurate in detection of cancer within prostate
& 1 with age
• Signs of extracapsular extension (ECE)
o Diagnosis of prostate cancer is suggested on basis of o Obliteration of periprostatic fat plane
• Abnormal digital exam, transrectal US, MR
o Abnormal enhancement of contiguous
findings neurovascular bundle
• Confirmed at biopsy o Urinary bladder, rectal invasion
• 1 Prostate specific antigen (PSA)level
o Lymphadenopathy

DDx: Prostate Mass or Pelvic Malignancy

BPH BPH Rectal Cancer Rectal Cancer

 PROSTATE CARCINOMA
Key Facts
Imaging Findings
• Best diagnostic clue: 1 Signal in a normally high
signal peripheral zone on T2WI
• Peripheral zone (70%): Posterior region (> common)
• Imaging accuracy for local staging suboptimal
• Signs of extracapsular extension (ECE)
• Obliteration of periprostatic fat plane
• Obliteration of rectoprostatic angle & neurovascular
bundle
• Osteoblastic bone metastases
• Hypoechoic (60-70%) lesion in peripheral zone,
abutting the adjacent normal tissue
Top Differential Diagnoses
• Benign prostatic hypertrophy (BPH)
• Rectal carcinoma
Pathology
• Advancing age, hormonal influence, environmental
& genetic factors playa role in development
• 1 in 11 males will develop prostate cancer
• More common in Western world & rare in Asians
• Approximately 300,000 new cases detected annually
• 95% of tumors are adenocarcinoma
Diagnostic· Checklist
• Prostate cancer until proven otherwise in an elderly
man with osteoblastic metastases on a plain x-ray
• Probable cancer in a man with abnormally t PSA
levels + enlarged prostate on digital rectal exam
• Annual screening: PSA levels + digital rectal exam
• Area of abnormal low signal in a normal high signal
peripheral zone on T2WI ± ECE & bony metastases

MR Findings I DIFFERENTIAL DIAGNOSIS

• Prostate cancer is best seen on T2WI
.0 Abnormal low signal in a normally high signal
Benign prostatic hypertrophy (BPH)
peripheral zone • MR: T2WI
.0 Normal prostate: t Peripheral zone signal & 1
.0 Extracapsular extension (ECE)

• Obliteration of rectoprostatic angle & transition zone signal

.0 BPH: Enlarged gland nodular heterogeneous signal
neurovascular bundle
• Urinary bladder, rectal invasion well depicted intensity
.0 Osteoblastic bone metastases
• Glandular hyperplasia: t Signal intensity
• Low signal intensity on both Tl WI & T2WI • Fibrous & muscular hyperplasia: Intermediate-low
• Tl WI: Normal t signal in fatty marrow is replaced signal intensity
by low-intermediate signal • Compressed peripheral zone into a thin rind by
enlarged gland
• Tl C+
.0 Tumor foci & ECE are well depicted • Fibrous capsule: Low signal intensity 6
Ultrasonographic Findings Rectal carcinoma 31
• Transrectal ultrasonography (TRUS) • Barium enema: Advanced cancer (apple-core lesion)
.0 Circumferential narrowing of bowel
.0 Hypoechoic (60-70%) lesion in peripheral zone,

abutting the adjacent normal tissue .0 Shelf-like overhanging borders (mucosal destruction)
.0 Hyperechoic (1-5%) • Imaging
.0 Isoechoic (30-40% of lesions) .0 Mass with circumferential wall thickening
.0 Most peripheral isoechoic lesions close to capsule .0 Extracolonic tumor extension

• Asymmetrical contour + bulging along lateral
aspect of prostate
.0 ± ECE: Lesion extending beyond prostatic margin
into periprostatic fat
.0 Size versus rate of detection
• Loss of fat planes between rectum & adjacent
structures
• MR shows obliteration of rectoprostatic angle &
neurovascular bundle with invasion of prostate
.0 Metastases to lung, bone, liver & lymph nodes

• < 5 mm (36%); 6-10 mm (53%)

• 11-15 mm (53%); 16-20 mm (84%)
• 21-25 mm (75%); > 26 mm (92%) I PATHOLOGY
.0 TRUS in local staging: Most widely used but still

remains controversial
General Features
• General path comments
, Nuclear Medicine Findings .0 Embryology/anatomy

• PET • Base (cranially); apex (caudally)

.0 Increased uptake of FDG • Zones (peripheral, central & transition)
• Early detection of metastatic foci • Genetics
• Tc99m methylene diphosphonate (MDP) bone scan .0 Familial in 10% of caucasian American men

.0 Detects osteoblastic metastases by t uptake .0 Cancer susceptibility gene mapped to lq24-25

• Etiology
Imaging Recommendations .0 Unknown
• Transrectal US; MRI (T2WI multiplanar images) .0 Advancing age, hormonal influence, environmental
• Tc MDP scan for bone metastases & genetic factors playa role in development
 PROSTATE CARCINOMA
• Epidemiology
o Incidence
• 1 in 11 males will develop prostate cancer
• Mortality rate: 13%
o More common in Western world & rare in Asians
o Approximately 300,000 new cases detected annually
Gross Pathologic & Surgical Features
• Growth: Usually more common in peripheral zone
o Localized, diffuse or extracapsular extension
o Firm or "gritty" as a result of fibrosis
Microscopic Features
• 95% of tumors are adenocarcinoma
• Well-differentiated glandular pattern
o Uniform epithelium, oval nuclei
o Pale cytoplasm & rare mitotic figures
• Poorly differentiated glandular pattern
o Sheets of tumor cells, mitoses, cellular atypia
Staging, Grading or Classification Criteria
• Jewett-Whitmore & TNM staging
o A & Tl: Clinically localized (tumor not palpable on
digital rectal exam)
o Al & Tla: Focal tumor or low grade
o A2 & Tlb: Diffuse tumor or high grade
o B & T2: Clinically localized (tumor palpable)
o Bl T2a: Tumor involves < 1fzlobe; B2 & T2b - tumor
involves> Vzlobe
o C & T3: Locally invasive beyond prostatic capsule
(tumor palpable)
o Cl & T3a: Unilateral extracapsular extension
o Cl & T3b: Bilateral extracapsular extension
o Cl & T3c: Seminal vesicle invasion
o C2 & T4: Invades adjacent tissues
6 • E.g, bladder, rectum, levator ani
o D & N/M: Lymph node & distant metastases (bones,
32 lung, liver & brain)
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic, or bone pain
o Urination: Hesitancy, urgency, increased frequency
• Lab data
o Increased prostate specific antigen (PSA) level
• Normal levels: 0-4.0 ng/mL
• Values 4-10 ng/ml also seen in BPH
o Ratio of free PSA - bound to (){1-antichymotrypsin
distinguishes prostate cancer from BPH
• Percent of free PSA is lower in cancer compared to
BPH
o 1 Prostatic acid phosphatase levels (non-specific)
• Seen only with metastases; prostatic manipulation
• Diagnosis: Imaging findings & transperineal biopsy
Demographics
• Age: Adults above 40 years (risk 1 with age)
• Ethnicity: African-Americans> Caucasians
Natural History & Prognosis
• Complications
o Bladder outlet & rarely rectal obstruction
o Obstructive uropathy, uremia, pathological fractures
• Prognosis
o After radical prostatectomy (for local cancer)
• Life expectancy more than 15 years
o Radiation & chemotherapy without surgery
• Life expectancy less than 5 years
Treatment
• Radical resection (for cancer confined to capsule)
• Radiation therapy (for cancer confined to capsule +
outside capsule & no spread)
• Hormonal therapy for spread
o Diethylstilbestrol & leuprolide; surgical orchiectomy
• Chemotherapy & cryosurgery
I DIAGNOSTIC CHECKLIST
Consider
• Prostate cancer until proven otherwise 'in an elderly
man with osteoblastic metastases on a plain x-ray
• Probable cancer in a man with abnormally 1 PSA levels
+ enlarged prostate on digital rectal exam
• Annual screening: PSA levels + digital rectal exam
Image Interpretation Pearls
• Area of abnormal low signal in a normal high signal
peripheral zone on T2WI ± ECE & bony metastases
I SELECTED REFERENCES
1. Dhingsa R et al: Prostate cancer localization with
endorectal MR imaging and MR spectroscopic imaging:
effect of clinical data on reader accuracy. Radiology.
230(1):215-20, 2004
2. Beyersdorff D et al: Patients with a history of elevated
prostate-specific antigen levels and negative transrectal
US-guided quadrant or sextant biopsy results: value of MR
imaging. Radiology. 224(3):701-6, 2002
3. Kurhanewicz J et al: The prostate: MR imaging and
spectroscopy. Present and future. Radiol Clin North Am.
38(1):115-38, viii-ix, 2000
4. Yu KK et al: Prostate cancer: prediction of extracapsular
extension with endorectal MR imaging and
three-dimensional proton MR spectroscopic imaging.
Radiology. 213(2):481-8, 1999
5. Scheidler J et al: Prostate cancer: localization with
three-dimensional proton MR spectroscopic
imaging--clinicopathologic study. Radiology.
213(2):473-80, 1999
6. Presti JC Jr et al: Local staging of prostatic carcinoma:
comparison of transrectal sonography and endorectal MR
imaging. AJR Am J Roentgenol. 166(1):103-8, 1996
7. Harris RD et al: Staging of prostate cancer with endorectal
MR imaging: Lessons from a learning curve. Radiographics.
15: 813-829, 1995
8. Ellis JH et al: MR imaging and sonography of early
prostatic cancer: pathologic and imaging features that
influence identification and diagnosis. AJR Am J
Roentgenol. 162(4):865-72, 1994
9. Mirowitz SA et al: Evaluation of the prostate and prostatic
carcinoma with gadolinium-enhanced endorectal coil MR
imaging. Radiology. 186(1):153-7, 1993
10. Hricak H et al: Prostatic carcinoma: staging by clinical
assessment, CT, and MR imaging. Radiology. 162(2):331-6,
1987
 PROSTATE CARCINOMA

I IMAGE GALLERY

(Left) Axial CECT shows

retroperitoneal
lymphadenopathy (arrow)
and sclerotic vertebral
metastasis (open arrow).
(Right) Radionuclide bone
scan shows multiple bone
metastases, especially in the
spine and pelvis.

Typical
(Left) Axial T2WI MR shows
enlarged prostate (open
arrow) with loss of normal
high signal peripheral zone
at site of carcinoma (arrow).
(Right) Coronal T2WI MR
shows enlarged prostate
(open arrow) and loss of
normal high signal in
peripheral zone (arrow) at
site of carcinoma.

6
33

(Left) Barium enema shows

encasement and narrowing
of rectum by large prostate
carcinoma. (Right) Sagittal
sonogram shows encasement
and partial obstruction of
distal right ureter by prostate
cancer.
 INDEX

A adrenal insufficiency vs., III:2-16i

Abdominal abscess, 1:1-6 to 1:1-8, 1:1-9i
differential diagnosis, 1:1-6i, 1:1-7
ventral hernia vs., 1:1-31
Abdominal wall disorders. See Peritoneum, mesentery,
and abdominal wall
Abdominal wall hernia, traumatic, 1:1-44i, 1:1-44 to 1:1-45
Abscess
abdominal, 1:1-6 to 1:1-8, 1:1-9i
differential diagnosis, I:1-6i, 1:1-7
ventral hernia vs., 1:1-31
of appendix. See Appendicitis
gastric diverticulum vs., 1:3-7
of liver. See Hepatic abscess
pelvic
bladder diverticulum vs., III:5-12i, II1:5-13
retroperitoneallymphocele vs., III:1-32i, II1:1-33
prostatic, III:6-20i, II1:6-21
pyogenic. See Pyogenic abscess
renal. See Renal abscess
retroperitoneal, III:1-17
splenic. See Splenic infection and abscess
Acanthosis, glycogenic, I:2-8i, 1:2-9
Accessory spleen, 1:6-10 to 1:6-11
differential diagnosis, 1:6-10i, 1:6-11
polysplenia vs., 1:6-6i, 1:6-8
Achalasia
cricopharyngeal, 1:2-32i, 1:2-32 to 1:2-33
esophageal, 1:2-34 to 1:2-36, 1:2-37i
differential diagnosis, 1:2-34i, 1:2-35 to 1:2-36
scleroderma vs., 1:2-42i, 1:2-43
Acquired cystic disease of uremia, II1:3-56 to II1:3-58,
III:3-59i
autosomal dominant polycystic kidney disease vs.,
III:3-16i, II1:3-17
differential diagnosis, III:3-56i, II1:3-57
yon Hippel Lindau disease vs., III:3-20i, II1:3-22
Addison disease, III:2-16i, II1:2-16 to II1:2-17
Adenocarcinoma, pancreatic. See Pancreatic ductal
carcinoma
Adenoma
adrenal. See Adrenal adenoma
of ampulla, II:2-54i, 11:2-55
hemorrhagic
HELLPsyndrome vs., II:l-72i, 11:1-73
hepatic trauma vs., II:I-90i, 11:1-91
hepatic. See Hepatic adenoma
of small bowel, 1:4-73
villous. See Villous adenoma
Adenomyomatosis, II:2-46i, 11:2-47
Adrenal adenoma, II1:2-20 to II1:2-22, III:2-23i
carcinoma vs., III:2-30i, II1:2-31
collision tumor vs., III:2-38i, II1:2-39
cyst vs., III:2-18i
differential diagnosis, III:2-20i, II1:2-21 to II1:2-22
hemorrhage in, III:2-8i, II1:2-9
hyperplasia vs., III:2-12i, II1:2-13
metastasis vs., III:2-34i, II1:2-36
myelolipoma vs., III:2-24i
pheochromocytoma vs., III:2-26i, II1:2-27
tuberculosis vs., III:2-6i
Adrenal carcinoma, II1:2-30 to II1:2-32, III:2-33i
adenoma vs., III:2-20i, II1:2-22
collision tumor vs., III:2-38i, II1:2-39
hemorrhage in, II1:2-10
metastasis vs., III:2-34i, II1:2-36
myelolipoma vs., II1:2-25
pheochromocytoma vs., II1:2-27
Adrenal collision tumor, III:2-38i, II1:2-38 to II1:2-39
Adrenal cyst, III:2-18i, II1:2-18 to II1:2-19
Adrenal glands, II1:2-6 to II1:2-39
adenoma, II1:2-20 to II1:2-22, III:2-23i
adrenal collision tumor, II1:2-38 to II1:2-39
carcinoma, II1:2-30 to II1:2-32, III:2-33i
cyst, II1:2-18 to II1:2-19
hemorrhage, II1:2-8 to II1:2-10, III:2-11i
hyperplasia, II1:2-12 to II1:2-14, III:2-15i
infections
adrenal insufficiency vs., II1:2-16
hemorrhage in, II1:2-1O
pheochromocytoma vs., II1:2-28
insufficiency, II1:2-16 to II1:2-17
mass vs. gastric diverticulum., I:3-6i
metastases and lymphoma, III:2-34 to III:2-36, III:2-37i
myelolipoma, II1:2-24 to II1:2-25
necrotic tumors, cyst vs., III:2-18i, II1:2-19
pheochromocytoma, II1:2-26 to II1:2-28, III:2-29i
tuberculosis and fungal infection, II1:2-6 to II1:2-7
Adrenal hemorrhage, II1:2-8 to II1:2-10, III:2-11i
adenoma vs., II1:2-22
adrenal carcinoma vs., II1:2-31
adrenal collision tumor vs., II1:2-39
adrenal insufficiency vs., II1:2-16
differential diagnosis, III:2-8i, II1:2-9 to II1:2-10
hyperplasia vs., III:2-12i, II1:2-14
metastasis vs., III:2-34i, II1:2-36
pheochromocytoma vs., III:2-26i, II1:2-28
tuberculosis vs., III:2-6i, II1:2-7
Adrenal hyperplasia, II1:2-12 to II1:2-14, III:2-15i
adenoma vs., II1:2-22
adrenal insufficiency vs., III:2-16i
differential diagnosis, III:2-12i, II1:2-13 to II1:2-14
 INDEX
hemorrhage in, III:2-Si, III:2-9
tuberculosis vs., III:2-6i, III:2-7
Adrenal insufficiency, III:2-16i, III:2-16 to III:2-17
Adrenal metastases and lymphoma, III:2-34 to III:2-36,
III:2-37i
adenoma vs., III:2-20i, III:2-21 to III:2-22
adrenal collision tumor vs., III:2-3Si, III:2-39
adrenal insufficiency vs., III:2-16i
carcinoma vs., III:2-30i, III:2-31
cyst vs., III:2-1Si, III:2-19
differential diagnosis, III:2-34i, III:2-36
hemorrhage in, III:2-Si, III:2-9
hyperplasia vs., III:2-12i, III:2-13
myelolipoma vs., III:2-24i, III:2-25
pheochromocytoma vs., III:2-26i, III:2-27 to III:2-28
tuberculosis vs., III:2-6i
Aerophagia, I:4-6Si, 1:4-70
AIDScholangiopathy
ascending cholangitis vs., II:2-14i, I1:2-15
differential diagnosis, II:2-22i, I1:2-23
primary sclerosing cholangitis vs., II:2-42i, I1:2-44
AIDS-related infections. See also HIV infections
malabsorption conditions vs., I:4-74i, 1:4-75
retroperitoneal metastasis vs., III:1-29
splenic metastasis vs., 1:6-32
splenomegaly vs., I:6-34i, 1:6-36
sprue-celiac disease vs., 1:4-19
Whipple disease vs., I:4-22i
Air bubbles, III:4-10
Allograft, renal, III:3-6i, III:3-8
Amebiasis, 1:4-12 to 1:4-13
Amiodarone, II:l-S4i, I1:1-85
Ampullary carcinoma, I1:2-54 to II:2-56, II:2-S7i
choledocholithiasis vs., II:2-26i, II:2-28
differential diagnosis, II:2-S4i, II:2-S5
Ampullary stenosis, II:2-22i, I1:2-23
Amyloidosis
glomerulonephritis vs., III:3-44
hepatic sarcoidosis vs., II:l-S6i, II:I-57
hepatitis vs.., II:1-16i, II:I-17
primary biliary cirrhosis vs., II:l-4Si, I1:1-49
Angiodysplasia, colonic, I:4-SOi, 1:4-52
Angiomyolipoma
hepatic, II:l-llSi, II:1-118 to II:l-119
renal, III:3-88 to III:3-90, III:3-9li
column of Bertin vs., III:3-14i, III:3-15
differential diagnosis, III:3-8Si, III:3-89 to III:3-90
medullary carcinoma vs., III:3-100
metastases and lymphoma vs., III:3-106i, III:3-107
oncocytoma vs., III:3-S4i, III:3-85 to III:3-86
renal cell carcinoma vs., III:3-96i, III:3-97
retroperitoneal sarcoma vs., III:l-20i, IIl:1-21
trauma vs., III:3-76i, III:3-77
Angiosarcoma
of liver, II:1-140i, II:1-140 to I1:1-142, II:1-143i
splenic, I:6-30i, 1:6-31, 1:6-36
Annular pancreas, II:3-S to II:3-10, II:3-11i
differential diagnosis, II:3-Si, I1:3-9 to II:3-10
duodenal carcinoma vs., 1:3-S4i, 1:3-55
pancreas divisum vs., II:3-12i, II:3-13
Anorchia, III:6-6
Aortic aneurysms, abdominal
renal artery stenosis vs., III:3-66
ruptured, III:l-lOi, III:l-11
Aortic dissection, III:3-64i, III:3-65
Aortitis, III:l-lOi, III:1-11
Aorta-enteric fistula, I:3-76i, 1:3-76 to 1:3-77
Appendagitis, epiploic, 1:5-32 to 1:5-34, I:S-3Si
differential diagnosis, I:S-32i, 1:5-33
diverticulitis vs., I:S-2Si, 1:5-29 to 1:5-30
omental infarct vs., I:1-22i
Appendiceal tumors
appendicitis vs., 1:5-23
mucocele vs., I:S-26i, 1:5-27
Appendicitis, 1:5-22 to 1:5-24, I:S-2Si
differential diagnosis, I:S-22i, 1:5-23
epiploic appendagitis vs., I:S-32i, 1:5-33
Meckel diverticulum vs., 1:4-Si, 1:4-9
mucocele vs., I:S-26i, 1:5-27
omental infarct vs., I:1-22i
typhlitis vs., I:S-14i, 1:5-15
Appendix, mucocele of, I:S-26i, 1:5-26 to 1:5-27
Arterioportal shunts
arteriovenous malformation vs., II:1-60i, II:I-61
nodular regenerative hyperplasia vs., II:l-S2i, II:I-53
Arteriovenous fistula, II:1-60i, II:I-61
Arteriovenous malformation
colonic, I:4-SOi, 1:4-52
hepatic, II:1-60i, II:I-60 to II:1-62, II:1-63i
Arteriovenous shunts, II:I-60i, II:1-62
Ascariasis
biliary, I1:2-20 to II:2-21
intestinal, 1:4-12 to 1:4-13
Ascites,1:1-18 to 1:1-20, 1:1-2li
abdominal abscess vS., 1:1-6i, 1:1-7
differential diagnosis, I: 1-1Si, 1:1-20
malignant, I:l-lSi, 1:1-20
mesenteric cyst vs., I:l-S2i
mesothelioma vs., 1:1-59
peritonitis vs., 1:1-10i, 1:1-11
small bowel obstruction vs., I:4-6Si, 1:4-70
Asplenia, 1:6-6 to 1:6-8, I:6-9i
differential diagnosis, I:6-6i, 1:6-8
gastric diverticulum vs., 1:3-6i, 1:3-7
Atrophy, hepatic, acquired, II:1-6i, II:1-7
Autoimmune disease, I:4-34i, 1:4-35
B
Barrett esophagus, 1:2-20 to 1:2-22, I:2-23i
differential diagnosis, I:2-20i, 1:2-21 to 1:2-22
drug-induced esophagitis vs., I:2-2Si, 1:2-29
Benign prostatic hypertrophy, III:6-20 to III:6-21
differential diagnosis, III:6-20i, III:6-21
prostate carcinoma vs., III:6-30i, III:6-31
prostatitis vs., III:6-10i, III:6-11
Bezoar, gastric, 1:3-68 to 1:3-70, 1:3-71i
differential diagnosis, I:3-6Si, 1:3-69
following fundoplication, I:3-60i, 1:3-62
Bile ducts
cyst. See Hepatic cyst
injury. See Biliary trauma
 INDEX
intraductal papillary mucinous tumor, II:2-S8i, 1I:2- urachal remnant vs., III:S-6t, III:5-7
58 to 1I:2-59 ureterocele vs., III:4-8i, III:4-9
obstruction, 1I:1-45, 1I:2-11 Bochdalek hernia, I:1-48i, 1:1-49 to 1:1-50
Biliary calculi. See Choledocholithiasis Boerhaave syndrome, 1:2-74 to 1:2-76, I:2-77i
Biliary cirrhosis, primary, 1I:1-48 to 1I:1-50, II:1-Sli differential diagnosis, I:2-74i, 1:2-75
differential diagnosis, II:1-48i, 1I:1-49 esophageal perforation vs., I:2-70i, 1:2-71
sarcoidosis vs., II:1-S6i, 1I:1-57 pulsion diverticulum vs., I:2-62i, 1:2-64
Biliary hamartoma, 1I:1-114 to 1I:1-116, II:1-117i Bowel necrosis, I:4-34i, 1:4-35
autosomal dominant polycystic liver disease vs., Brunner glands
II:1-12i, 1I:1-13 duodenal polyps vs., 1:3-27, I:3-36i
Caroli disease vs., II:2-6i, 1I:2-7 hyperplasia vs. hamartomatous polyposis, I:4-S6i
congenital hepatic fibrosis vs., II:1-8i, 1I:1-9 1:4-57 '
differential diagnosis, II:1-114i, 1I:1-115 Budd-Chiari syndrome, II:1-64 to 1I:1-66, II:1-67i
hepatic candidiasis vs., II:1-20i, 1I:1-21 arteriovenous malformation vs., II:1-60i, II:1-62
Biliary sludge, II:2-40i, 1I:2-41 differential diagnosis, II:1-64i, 1I:1-65
Biliary system, 1I:2-6 to 1I:2-61 liver cirrhosis vs., II:l-4Oi, 1I:1-41
AIDScholangiopathy, 1I:2-22 to 1I:2-24, II:2-2Si passive hepatic congestion vs., II:1-68i, 1I:1-69
ampullary carcinoma, 1I:2-54 to 1I:2-56, II:2-S7i
ascending cholangitis, 1I:2-14 to 1I:2-15
Caroli disease, 1I:2-6 to 1I:2-8, II:2-9i
chemotherapy cholangitis, 1I:2-60 to 1I:2-61
c
Candida infections
cholangiocarcinoma, 1I:2-50 to 1I:2-52, II:2-S3i
esophagitis, 1:2-8 to 1:2-10, I:2-11i
cholecystitis, 1I:2-34 to 1I:2-36, II:2-37i
Barrett esophagus vs., 1:2-21
choledochal cyst, 1I:2-10 to 1I:2-12, II:2-13i
caustic esophagitis vs., 1:2-24i, 1:2-25
choledocholithiasis, 1I:2-26 to 1I:2-28, II:2-29i
differential diagnosis, I:2-8i, 1:2-9
gallbladder carcinoma, 1I:2-46 to 1I:2-48, II:2-49i
drug-induced esophagitis vs., 1:2-28i
hyperplastic cholecystoses, 1I:2-32 to 1I:2-33
foreign body vs., I:2-68i, 1:2-69
intraductal papillary mucinous tumor of biliary
intramural pseudodiverticulosis vs., 1:2-66i, 1:2-67
ducts, 1I:2-58 to 1I:2-59
radiation esophagitis vs., 1:2-30i, 1:2-31
milk of calcium bile, 1I:2-40 to 1I:2-41
reflux esophagitis vs., I:2-16i, 1:2-17
Mirizzi syndrome, 1I:2-30 to 1I:2-31
technical artifacts vs., 1:2-9
pancreato-biliary parasites, 1I:2-20 to 1I:2-21
viral esophagitis vs., I:2-12i, 1:2-13
porcelain gallbladder, 1I:2-38 to 1I:2-39
hepatic candidiasis, II:l-20i, II:1-20 to II:1-22, II:1-23i
primary sclerosing cholangitis, 1I:2-42 to 1I:2-44,
splenic metastasis vs., 1:6-32
II:2-4Si
splenomegaly vs., I:6-34i, 1:6-35 to 1:6-36
recurrent pyogenic cholangitis, 1I:2-16 to 1I:2-18
Carcinoid syndrome, 1:4-24i, 1:4-25
II:2-19i '
Carcinoid tumor, 1:4-60 to 1:4-62, I:4-63i
Biliary trauma, II:1-94i, II:1-94 to 1I:1-96, II:1-97i
desmoid tumorvs., 1:1-S4i, 1:1-55
Biloma, I:1-6i, 1:1-7
differential diagnosis, I:4-60i, 1:4-61 to 1:4-62
Bladder, III:5-6 to III:5-25
gastric polyps vs., I:3-32i
blood clots
ischemic enteritis vs., I:4-38i, 1:4-39
bladder carcinoma vs., III:S-22i, III:5-23
sclerosing mesenteritis vs., 1:1-14i, 1:1-15
trauma and, III:S-18i, III:S-19
small bowel carcinoma vs., 1:4-59
calculi, III:5-10 to III:5-11
Carc~noma. Se~ specific sites and histologic types
carcinoma, III:5-22 to III:S-24, III:S-2Si
CarcInOmatosIs
cystitis, III:5-8 to III:5-9
mesenteric, 1:1-16
diverticulum, III:5-12 to III:5-13
peritoneal
differential diagnosis, III:S-12i, III:5-13
mesothelioma vs., 1:1-58i, 1:1-59
retroperitoneallymphocele vs., III:1-32i
peritonitis vs., I:1-10i, 1:1-11
fistulas, III:5-14 to III:5-15
pseudomyxoma peritonei vs., I:1-66i, 1:1-67
lymphoma, III:S-22i, III:5-23
Caroli disease, 1I:2-6 to 1I:2-8, II:2-9i
neurogenic bladder, III:5-16 to III:5-17
autosomal dominant polycystic liver disease vs.,
outlet obstruction, III:S-16i, III:5-17
II:1-12i, 1I:1-13
thickened wall
biliary hamartoma vs., II:1-114i, 1I:1-115
bladder carcinoma vs., III:5-23
choledochal cyst vs., II:2-10i, 1I:2-11
cystitis vs., III:S-8i
congenital hepatic fibrosis vs., II:1-8i, 1I:1-9
trauma, III:S-18i, III:5-18 to III:5-20, III:5-2li
differential diagnosis, II:2-6i, 1I:2-7
urachal remnant, II1:5-6 to III:5-7
hepatic candidiasis vs., II:1-20i, 1I:1-21
Bladder carcinoma, III:5-22 to III:5-24, III:5-25i
Castleman disease, III:1-25
benign prostatic hypertrophy vs., III:6-20i, III:6-21
Cathartic colon, I:S-16i, 1:5-18
calculi vs., 1II:3-11, III:5-10i
Catheterization, bladder fistula vs., III:5-14i, III:5-15
differential diagnosis, III:5-22i, III:5-23
 INDEX
Caustic esophagitis, 1:2-24 to 1:2-26, 1:2-27i
Barrett esophagus vs., 1:2-21
carcinoma vs., 1:2-83
differential diagnosis, I:2-24i, 1:2-25
radiation esophagitis vs., 1:2-30i, 1:2-31
reflux esophagitis vs., 1:2-17
viral esophagitis vs., I:2-12i, 1:2-13
Cavernous hemangioma, 11:1-102 to 11:1-104, 1I:1-105i
differential diagnosis, 1I:1-102i, 11:1-104
fibrolamellar hepatocellular carcinoma vs., 11:1-
124i,II:1-125
focal confluent fibrosis vs., 11:1-45
focal nodular hyperplasia vs., 1I:1-106i, 11:1-107
peliosis hepatis vs., 1I:1-80i, 11:1-81
Cecal carcinoma
appendicitis vs., 1:5-23
mucocele of appendix vs., I:S-26i, 1:5-27
typhlitis vs., I:S-14i, 1:5-15
Cecal volvulus, 1:5-66 to 1:5-67
differential diagnosis, I:S-66i, 1:5-67
sigmoid volvulus vs., I:S-62i, 1:5-63
Celiac sprue. See Sprue-celiac disease
Cervical cancer, I:S-48i, 1:5-49
Chemoembolization
of liver, 1I:1-6i, 11:1-7
porcelain gallbladder vs., 1I:2-38i
Cholangiocarcinoma
AIDScholangiopathy vs., 1I:2-22i, 11:2-23
of bile ducts, 1I:2-SOi, 11:2-50 to 11:2-52, 1I:2-S3i
cavernous hemangioma vs., 1I:1-102i, 11:1-104
choledocholithiasis vs., 1I:2-26i, 11:2-28
epithelioid hemangioendothelioma vs., 1I:1-132i,
11:1-133
fibrolamellar hepatocellular carcinoma vs., 11:1-
124i,II:1-126
focal confluent fibrosis vs., 1I:1-44i, 11:1-45
hepatic metastases and lymphoma vs., 1I:1-144i,
11:1-146
hepatocellular carcinoma vs., 1I:1-120i, 11:1-122
intraductal papillary mucinous tumor vs., 1I:2-S8i
Mirizzi syndrome vs., 1I:2-30i, 11:2-31
pancreato-biliary parasites vs., 1I:2-20i, 11:2-21
peripheral, 1I:1-128i, 11:1-128 to 11:1-130, 1I:1-13li
recurrent pyogenic cholangitis vs., I1:2-16i, II:2-17
sclerosing, 1I:2-42i, 11:2-44
steatosis vs., 1I:1-36i
Cholangiopathy, AIDS-related. See AIDScholangiopathy
Cholangitis
ascending, 11:2-14 to 11:2-15
Caroli disease vs., 11:2-7
chemotherapy cholangitis vs., 1I:2-60i, 11:2-61
differential diagnosis, 1I:2-14i, 11:2-15
primary sclerosing cholangitis vs., 1I:2-42i, 11:2-44
bacterial
intraductal papillary mucinous tumor vs., 11:2-
S8i, 11:2-59
pancreato-biliary parasites vs., 1I:2-20i, 11:2-21
recurrent pyogenic cholangitis vs., 1I:2-16i, 11:2-17
chemotherapy-related, 11:2-60 to 11:2-61
ascending cholangitis vs., 1I:2-14i, 11:2-15
differential diagnosis, 1I:2-60i, 11:2-61
primary sclerosing cholangitis vs., 11:2-44
primary sclerosing, 11:2-42 to 11:2-44, 1I:2-4Si
AIDScholangiopathy vs., 1I:2-22i, 11:2-23
ascending cholangitis vs., 1I:2-14i, 11:2-15
Budd-Chiari syndrome vs., 1I:1-64i, 11:1-65
Caroli disease vs., 1I:2-6i, 11:2-7
chemotherapy cholangitis vs., 1I:2-6Oi
cholangiocarcinoma vs., 1I:2-SOi, 11:2-52
choledocholithiasis vs., 11:2-28
congenital hepatic fibrosis vs., 1I:1-8i, 11:1-9
differential diagnosis, 1I:2-42i, 11:2-44
focal confluent fibrosis vs., 1I:1-44i, 11:1-45
recurrent pyogenic cholangitis vs., 1I:2-16i, 11:2-17
recurrent pyogenic, 11:2-16 to 11:2-18, 1I:2-19i
ascending cholangitis vs., 1I:2-14i, 11:2-15
Caroli disease vs., 1I:2-6i, 11:2-7
choledochal cyst vs., 1I:2-10i, 11:2-11
differential diagnosis, 1I:2-16i, 11:2-17
intraductal papillary mucinous tumor vs., 11:2-
S8i, 11:2-59
pancreato-biliary parasites vs., 1I:2-20i, 11:2-21
Cholecystitis, 11:2-34 to 11:2-36, 1I:2-37i
AIDS cholangiopathy vs., 1I:2-22i, 11:2-23
differential diagnosis, 1I:2-34i, 11:2-35
following gastric bypass, I:3-64i, 1:3-66
gallbladder carcinoma vs., 1I:2-46i, 11:2-47
hyperplastic cholecystoses vs., 1I:2-32i, 11:2-33
porcelain gallbladder vs., 1I:2-38i
Cholecystoses, hyperplastic, 1I:2-32i, 11:2-32 to 11:2-33
Choledochal cyst, 1I:2-lOi, 11:2-10 to 11:2-12, 1I:2-13i
Choledocholithiasis, 11:2-26 to 11:2-28, 1I:2-29i
cholangiocarcinoma vs., 1I:2-S0i, 11:2-52
differential diagnosis, 1I:2-26i, 11:2-28
pancreato-biliary parasites vs., 1I:2-20i, 11:2-21
Chronic obstructive pulmonary disease (COPD), 1:4-35
Cirrhosis, 11:1-40 to 11:1-42, 1I:1-43i
Budd-Chiari syndrome vs., 1I:1-64i, 11:1-65
differential diagnosis, 1I:1-40i, 11:1-41 to 11:1-42
inferior vena cava anomalies vs., III:I-6i, II1:1-7
multiple arterioportal shunts vs. nodular
regenerative hyperplasia, 1I:1-S2i, 11:1-53
passive hepatic congestion vs., 1I:1-68i, 11:1-69
primary biliary cirrhosis vs., 1I:1-48i, 11:1-49
retroperitoneal metastasis vs., III:1-29
sclerosing mesenteritis vs., I:I-14i, 1:1-15 to 1:1-16
Clonorchiasis, biliary, 11:2-20 to 11:2-21
Coagulopathy
HELLP syndrome vs., 1I:1-72i, 11:1-73
hepatic trauma vs., 1I:1-90i, 11:1-91
intestinal trauma and, 1:4-46 to 1:4-47
Colitis
granulomatous. See Crohn disease
infectious, 1:5-6 to 1:5-8, I:S-9i
colon carcinoma vs., I:S-44i, 1:5-45
differential diagnosis, I:S-6i, 1:5-7
pseudomembranous colitis vs., I:S-10i, 1:5-11
rectal carcinoma vs., 1:5-49
ischemic, 1:5-36 to 1:5-38, I:S-39i
colon carcinoma vs., I:S-44i, 1:5-45
differential diagnosis, I:S-36i, 1:5-37 to 1:5-38
diverticulitis vs., 1:5-29 to 1:5-30
infectious colitis vs., I:S-6i, 1:5-7
pseudomembranous colitis vs., I:S-10i, 1:5-11
 INDEX
ulcerative colitis vs., I:5-16i, 1:5-17
neutropenic. See Typhlitis
pseudomembranous, 1:5-10 to 1:5-12, I:5-13i
differential diagnosis, I:5-10i, 1:5-11 to 1:5-12
diverticulitis vs., 1:5-28i, 1:5-30
epiploic appendagitis vs., I:5-32i, 1:5-33
infectious colitis vs., I:5-6i, 1:5-7
ischemic colitis vs., I:5-36i, 1:5-37 to 1:5-38
parasitic infections vs., I:4-12i, 1:4-13
typhlitis vs., I:5-14i, 1:5-15
ulcerative colitis vs., I:5-16i, 1:5-17
radiation-induced, I:5-28i, 1:5-29
ulcerative, 1:5-16 to 1:5-18, I:5-19i
colon carcinoma vs., 1:5-45
Crohn disease vs., I:4-26i, 1:4-27
differential diagnosis, I:5-16i, 1:5-17 to 1:5-18
epiploic appendagitis vs., 1:5-32i, 1:5-33
familial polyposis vs., I:5-56i, 1:5-58
Gardner syndrome vs., I:5-60i, 1:5-61
infectious colitis vs., I:5-6i, 1:5-7
ischemic colitis vs., I:5-36i, 1:5-38
parasitic infections vs., I:4-12i, 1:4-13
pelvic lipomatosis vs., III:l-14i, I1I:1-15
pseudomembranous colitis vs., I:5-10i, 1:5-11
Colon, 1:5-6 to 1:5-67
appendicitis, 1:5-22 to 1:5-24, I:5-25i
carcinoma, 1:5-44 to 1:5-46, 1:5-47i
cathartic, I:5-16i, 1:5-18
cecal volvulus, 1:5-66 to 1:5-67
diverticulitis, 1:5-28 to 1:5-30, I:5-3li
epiploic appendagitis, 1:5-32 to 1:5-34, I:5-35i
familial polyposis, 1:5-56 to 1:5-58, 1:5-59i
Gardner syndrome, 1:5-60 to 1:5-61
infectious colitis, 1:5-6 to 1:5-8, I:5-9i
intramural mass, villous adenoma vs., 1:5-54
ischemic colitis, 1:5-36 to 1:5-38, I:5-39i
metastasis and lymphoma
familial polyposis vs., I:5-56i, 1:5-57
Gardner syndrome vs., 1:5-61
mucocele of appendix, 1:5-26 to 1:5-27
obstruction
cecal volvulus vs., 1:5-66
sigmoid volvulus vs., 1:5-63
toxic megacolon vs., 1:5-21
polyps, 1:5-40 to 1:5-42, 1:5-43i
pseudomembranous colitis, 1:5-10 to 1:5-12, I:5-13i
rectal carcinoma, 1:5-48 to 1:5-50, I:5-Sli
sigmoid volvulus, 1:5-62 to 1:5-64, I:S-6Si
toxic megacolon, 1:5-20 to 1:5-21
typhlitis, 1:5-14 to 1:5-15
ulcerative colitis, 1:5-16 to 1:5-18, I:5-19i
villous adenoma, 1:5-52 to 1:5-54, I:5-55i
Colon carcinoma, 1:5-44 to 1:5-46, I:5-47i
cecal volvulus vs., I:5-66i, 1:5-67
differential diagnosis, I:5-44i, 1:5-45
diverticulitis vs., I:5-28i, 1:5-29
duodenal carcinoma vs., I:3-54i, 1:3-55
ischemic colitis vs., I:5-36i, 1:5-38
polyps vs., 1:5-41
radiation enteritis vs., I:4-82i, 1:4-83
sigmoid volvulus vs., I:5-62i, 1:5-63
toxic megacolon vs., I:5-2Oi
villous adenoma vs., I:5-52i, 1:5-53
Colovesicle fistula, III:5-22i, I1I:5-23
Column of Bertin, II1:3-14i, I1I:3-14 to I1I:3-15
Congenital foramen of Bochdalek, I:1-48i, 1:1-49 to 1:1-50
Congenital foramen of Morgagni, I:1-49
Contrast fluids, II:2-40i, 11:2-41
COPD (chronic obstructive pulmonary disease), 1:4-35
Corrosive esophagitis. See Caustic esophagitis
Cortical necrosis, acute, I1I:3-61
Cricopharyngeal achalasia, I:2-6i, 1:2-7
Crohn disease, 1:4-26 to 1:4-28, I:4-29i
appendicitis vs., 1:5-23
differential diagnosis, 1:4-26i, 1:4-27 to 1:4-28
duodenal carcinoma vs., 1:3-55
duodenal ulcer vs., I:3-18i, 1:3-19
familial polyposis vs., I:5-56i, 1:5-58
Gardner syndrome vs., I:5-60i, 1:5-61
gastric carcinoma vs., 1:3-47
infectious colitis vs., I:5-6i, 1:5-7
ischemic colitis vs., 1:5-38
ischemic enteritis vs., I:4-38i, 1:4-39
malabsorption conditions vs., I:4-74i, 1:4-75
Meckel diverticulum vs., 1:4-8i, 1:4-10
Menetrier disease vs., 1:3-26i, 1:3-27
metastases and lymphoma vs., I:4-64i, 1:4-66
parasitic infections vs., I:4-12i
pseudomembranous colitis vs., 1:5-11
radiation enteritis vs., I:4-82i, 1:4-83
reflux esophagitis vs., I:2-16i, 1:2-18
small bowel carcinoma vs., I:4-58i, 1:4-59
sprue-celiac disease vs., I:4-18i, 1:4-19
ulcerative colitis vs., I:5-16i, 1:5-17
vasculitis vs., I:4-42i, 1:4-43
viral esophagitis vs., 1:2-13
Zollinger-Ellison syndrome vs., 1:3-24
Crossing vessels, I1I:4-11
Cryptorchidism, II1:6-6i, I1I:6-6 to I1I:6-7
Cryptosporidiosis
Crohn disease vs., 1:4-27
malabsorption conditions vs., I:4-74i, 1:4-75 to 1:4-76
sprue-celiac disease vs., 1:4-19
Whipple disease vs., I:4-22i
Cystadenocarcinoma, biliary, 11:1-136 to 11:1-138,
II:I-139i
differential diagnosis, II:l-136i, 11:1-137
hepatic amebic abscess., 11:1-29
hepatic cyst vs., II:1-98i, 11:1-99
hepatic hydatid cyst vs., II:1-32i, 11:1-33
pyogenic abscess vs., II:1-24i, 11:1-26
Cystadenoma, serous, 11:3-36 to 11:3-38, II:3-39i
differential diagnosis, II:3-36i, 11:3-37 to 11:3-38
intraductal papillary mucinous tumor vs., 11:3-47
islet cell tumors vs., 11:3-55
mucinous cystic pancreatic tumor vs., II:3-42i, 11:3-43
pancreatic cyst vs., II:3-40i
pseudocyst vs., 1I:3-24i, 11:3-25
solid and papillary neoplasms vs., II:3-S8i, 11:3-59
Cystic duct stones, II:2-3Oi
Cystic fibrosis, 11:3-18 to 11:3-19
differential diagnosis, II:3-18i, 11:3-19
malabsorption conditions vs., I:4-74i, 1:4-76
small bowel obstruction vs., I:4-68i, 1:4-70
 INDEX
sprue-celiac disease vs., 1:4-20
Cystitis, III:5-8 to III:5-9
bladder carcinoma vs., III:5-22i, III:5-23
bladder fistula vs., I1I:5-14i, III:5-15
differential diagnosis, III:S-8i
Cysts. See specific sites and histologic types
Cytomegalovirus infections
Crohn disease vs., 1:4-27
metastases and lymphoma vs., 1:4-66
reflux esophagitis vs., 1:2-17
sprue-celiac disease vs., 1:4-19
D Duodenum
Dermoid tumor, III:3-11, III:S-I0i
Desmoid tumor, 1:1-54 to 1:1-56, I:l-S7i
carcinoid tumor vs., I:4-60i, 1:4-62
differential diagnosis, I:l-S4i, 1:1-55 to 1:1-56
sclerosing mesenteritis vs., 1:1-14i, 1:1-16
Diaphragmatic rupture, traumatic, I:I-48i, 1:1-48 to 1:1-
50, I:l-Sli .
Diffuse esophageal spasm (DES). See Esophageal motility
disturbances
Diverticulitis, 1:5-28 to 1:5-30, I:S-3li
cecal
appendicitis vs., I:S-22i, 1:5-23
Meckel diverticulum vs., I:4-8i, 1:4-10
typhlitis vs., I:S-14i, 1:5-15
colon carcinoma vs., I:S-44i, 1:5-45
differential diagnosis, I:S-28i, 1:5-29 to 1:5-30
epiploic appendagitis vs., I:S-32i, 1:5-33
hepatic flexure, II:2-34i, 11:2-35
ischemic colitis vs., I:S-36i, 1:5-37
polyps vs., I:S-40i, 1:5-41
ulcerative colitis vs., I:S-16i, 1:5-18
Diverticulosis, 1:4-52
Diverticulum
bladder, III:5-12 to III:5-13
differential diagnosis, I1I:S-12i, III:5-13
retroperitoneallymphocele vs., III:I-32i
colon, I:S-40i, 1:5-41
duodenal, 1:3-8 to 1:3-9
differential diagnosis, I:3-8i, 1:3-9
duodenal ulcer vs., I:3-18i, 1:3-19
epiphrenic. See Pulsion diverticulum
esophageal,I:2-71
gastric. See Gastric diverticulum
Killian-Jamieson, I:2-S6i, 1:2-57
Meckel, 1:4-8 to 1:4-10, I:4-1li
differential diagnosis, I:4-8i, 1:4-9 to 1:4-10
intussusception vs., I:4-78i, 1:4-79
traction, 1:2-60 to 1:2-61
differential diagnosis, I:2-60i, 1:2-60 to 1:2-61
pulsion diverticulum vs., I:2-62i, 1:2-63
Zenker, I:2-S6i, 1:2-56 to 1:2-58, 1:2-S9i
Drug abuse. See Substance abuse
Duodenal carcinoma, 1:3-54 to 1:3-56, I:3-S7i
ampullary carcinoma vs., II:2-S4i, 11:2-55
annular pancreas vs., II:3-8i, 11:3-9
differential diagnosis, I:3-S4i, 1:3-55
metastases and lymphoma vs., I:3-58i, 1:3-59
ulcer vs., I:3-18i, 1:3-19
Duodenal ulcer, 1:3-18 to 1:3-20, I:3-2li
differential diagnosis, I:3-18i, 1:3-19 to 1:3-20
duodenal carcinoma vs., I:3-S4i, 1:3-55
gastrointestinal bleeding vs., I:4-S0i, 1:4-51
intramural benign gastric tumors vs., 1:3-39
perforated
acute pancreatitis vs., II:3-20i, 11:3-21
duodenal diverticulum vs., 1:3-9
duodenal hematoma vs., I:3-30i, 1:3-31
emphysematous pyelonephritis vs., III:3-40i,
III:3-41
retroperitoneal hemorrhage in, III:I-16i, III:I-17
Duodenitis, I:3-18i, 1:3-19
duplication cyst, 1:3-55
hematoma, 1:3-30 to 1:3-31
annular pancreas vs., II:3-8i, 11:3-10
differential diagnosis, I:3-30i, 1:3-31
duodenal carcinoma vs., 1:3-55
duodenal ulcer vs., 1:3-20
traumatic pancreatitis vs., II:3-32i, 11:3-33
laceration, 1:3-30 to 1:3-31
metastases and lymphoma, 1:3-58 to 1:3-59
polyps, 1:3-36 to 1:3-27
rupture, II:3-32i, 11:3-33
stricture, 1:3-19
Dysgammaglobulinemia, I:4-22i, 1:4-23
E
Ebstein-Barr virus esophagitis. See Viral esophagitis
Echinococcal cyst, 1:6-23
Ectopic kidney, III:3-6 to III:3-8, I1I:3-9i
differential diagnosis, III:3-6i, III:3-8
horseshoe kidney vs., III:3-10i, III:3-11
Edema
anastomotic, 1:3-64i, 1:3-66
mesenteric, 1:1-15
postoperative, I:3-60i, 1:3-61
Endometrial implant, 1:4-79
Endometrioma,III:I-32i
Endometriosis, I:S-44i, 1:5-45
Enteric cyst, 1:1-52
Enteric duplication cyst, 1:1-52
Enteritis
ischemic, 1:4-38 to 1:4-40, I:4-4li
differential diagnosis, I:4-38i, 1:4-39
intestinal trauma vs., I:4-46i, 1:4-48
pneumatosis vs., I:4-34i, 1:4-35
radiation enteritis vs., I:4-82i, 1:4-83
vasculitis vs., I:4-42i, 1:4-43
radiation-induced, 1:4-82 to 1:4-84, I:4-8Si
Crohn disease vs., 1:4-28
differential diagnosis, I:4-82i, 1:4-83
Enterocolitis
necrotizing, I:4-34i, 1:4-35
neutropenic. See Typhlitis
Epidermoid cyst, III:6-26i, III:6-27
Epididymitis, III:6-8 to III:6-9
differential diagnosis, III:6-8i, III:6-9
varicocele vs., III:6-16i
Epididymo-orchi tis
 INDEX
scrotal trauma vs., III:6-22i
testicular torsion vs., III:6-18i
Epiphrenic diverticulum. See Pulsion diverticulum
Esophageal carcinoma, 1:2-82 to 1:2-84, 1:2-8Si. See also
Esophageal tumors
Barrett esophagus vs., 1:2-21
caustic esophagitis vs., I:2-24i, 1:2-25
differential diagnosis, I:2-82i, 1:2-83 to 1:2-84
drug-induced esophagitis vs., 1:2-29
esophageal achalasia vs., 1:2-34i, 1:2-36
esophageal motility disturbances vs., 1:2-38i, 1:2-39
esophageal varices vs., 1:2-46i, 1:2-47
fibrovascular polyp vs., 1:2-80i, 1:2-81
intramural benign tumors vs., 1:2-78i, 1:2-79
metastasis vs., 1:2-86i, 1:2-87
scleroderma vs., 1:2-42i, 1:2-43
Esophageal motility disturbances, 1:2-38 to 1:2-40, 1:2-4li
differential diagnosis, 1:2-38i, 1:2-39
esophageal achalasia vs., 1:2-36
Esophageal perforation, 1:2-70 to 1:2-72, 1:2-73i
differential diagnosis, 1:2-70i, 1:2-71
pulsion diverticulum vs., 1:2-62i, 1:2-64
traction diverticulum vs., 1:2-60i, 1:2-61
Esophageal tumors. See also Esophageal carcinoma
Barrett esophagus vs., 1:2-2Oi
cricopharyngeal achalasia vs., 1:2-32i
esophageal webs vs., 1:2-6i
foreign body vs., 1:2-68i, 1:2-69
intramural benign tumors, 1:2-78 to 1:2-79
differential diagnosis, 1:2-78i, 1:2-78 to 1:2-79
fibrovascular polyp vs., 1:2-80i, 1:2-81
metastasis vs., 1:2-86i, 1:2-87
intramural primary, 1:2-84
metastasis, 1:2-86i, 1:2-86 to 1:2-87
recurrence, 1:2-31
Schatzki ring vs., 1:2-S0i, 1:2-51
Esophageal ulcer
esophageal perforation vs., 1:2-71
traction diverticulum vs., 1:2-60i, 1:2-61
Esophageal varices, 1:2-46 to 1:2-48, 1:2-49i
Candida esophagitis vs., 1:2-9
differential diagnosis, 1:2-46i, 1:2-47
gastrointestinal bleeding vs., 1:4-S0i, 1:4-51
metastasis vs., 1:2-86i, 1:2-87
Esophageal webs
cricopharyngeal achalasia vs., I:2-32i
differential diagnosis, 1:2-6i, 1:2-6 to 1:2-7
Schatzki ring vs., 1:2-SOi, 1:2-51
Zenker diverticulum vs., 1:2-S6i, 1:2-57
Esophagectomy, postoperative changes
esophageal perforation vs., 1:2-71
hiatal hernia vs., 1:2-S2i, 1:2-53
Esophagitis
from Candida. See Candida infections, esophagitis
caustic. See Caustic esophagitis
drug-induced, 1:2-28 to 1:2-29
Barrett esophagus vs., 1:2-20i, 1:2-21 to 1:2-22
Candida esophagitis vs., 1:2-9
differential diagnosis, 1:2-28i, 1:2-29
radiation esophagitis vs., 1:2-30i, 1:2-31
viral esophagitis vs., 1:2-12i, 1:2-13
esophageal varices vs., 1:2-46i, 1:2-47
foreign body vs., 1:2-68i, 1:2-69
infectious
Barrett esophagus vs., 1:2-21
carcinoma vs., 1:2-83
caustic esophagitis vs., 1:2-25
drug-induced esophagitis vs., 1:2-29
radiation esophagitis vs., 1:2-31
viral esophagus vs., 1:2-13
intramural pseudodiverticulosis vs., 1:2-66i, 1:2-67
radiation-induced. See Radiation esophagitis
reflux. See Reflux esophagitis
viral. See Viral esophagitis
with stricture. See also Peptic stricture
esophageal achalasia vs., 1:2-34i, 1:2-35
esophageal motility disturbances vs., 1:2-38i, 1:2-39
esophageal webs vs., 1:2-6i
Esophagus, 1:2-6 to 1:2-87
achalasia
cricopharyngeal, 1:2-32 to 1:2-33
primary motility disorder, 1:2-34 to 1:2-36, 1:2-37i
Barrett esophagus, 1:2-20 to 1:2-22, 1:2-23i
Boerhaave syndrome, 1:2-74 to 1:2-76, 1:2-77i
carcinoma, 1:2-82 to 1:2-84, 1:2-8Si
esophageal webs, 1:2-6 to 1:2-7
esophagitis
Candida, 1:2-8 to 1:2-10, 1:2-11i
caustic, 1:2-24 to 1:2-26, 1:2-27i
drug-induced, 1:2-28 to 1:2-29
radiation-induced, 1:2-30 to 1:2-31
reflux, 1:2-16 to 1:2-18, 1:2-19i
viral, 1:2-12 to 1:2-14, 1:2-1Si
extrinsic encasement, 1:2-84
feline, 1:2-16i, 1:2-17
fibrovascular polyp, 1:2-80 to 1:2-81
foreign body, 1:2-68 to 1:2-69
hiatal hernia, 1:2-52 to 1:2-54, 1:2-SSi
intramural benign tumors, 1:2-78 to 1:2-79
metastases and lymphoma, 1:2-86 to 1:2-87
motility disturbances, 1:2-38 to 1:2-40, 1:2-4li
perforation, 1:2-70 to 1:2-72, 1:2-73i
pseudodiverticulosis, intramural, 1:2-66 to 1:2-67
pulsion diverticulum, 1:2-62 to 1:2-64, 1:2-6Si
Schatzki ring, 1:2-50 to 1:2-51
scleroderma, 1:2-42 to 1:2-44, 1:2-4Si
traction diverticulum, 1:2-60 to 1:2-61
varices, 1:2-46 to 1:2-48, 1:2-49i
Zenker diverticulum, 1:2-56 to 1:2-58, I:2-S9i
Eventration, diaphragmatic, I:1-48i, 1:1-49
F
Familial adenomatous polyposis syndrome, 1:5-56 to 1:5-
58,I:S-S9i
differential diagnosis, I:S-S6i, 1:5-57 to 1:5-58
hamartomatous polyposis vs., 1:4-57
Fatty liver. See Steatosis
Feces (fecal mass)
colonic polyps vs., I:S-40i, 1:5-41
familial polyposis vs., I:S-S6i, 1:5-57 to 1:5-58
Gardner syndrome vs., I:S-6Oi
villous adenoma vs., I:S-S2i, 1:5-53
Feline esophagus, 1:2-16i, 1:2-17
 INDEX
Femoral hernia, 1:1-28 to 1:1-29
differential diagnosis, I: 1-28i, I:1-29
inguinal hernia vs., I:I-24i, 1:1-25
obturator hernia vs., I:I-34i
Fetal lobulation, renal, III:3-14i, III:3-15
Fibroids, calcified, III:5-1Oi
Fibrolamellar carcinoma of liver. See Hepatocellular
carcinoma (HCC), fibrolamellar
Fibrovascular polyp, 1:2-80 to 1:2-81
differential diagnosis, I:2-80i, 1:2-81
esophageal carcinoma vs., 1:2-84
Focal confluent fibrosis, hepatic, II:1-44 to II:1-46, 1I:1-47i
differential diagnosis, 1I:1-44i, 11:1-45 to 11:1-46
epithelioid hemangioendothelioma vs., 1I:1-132i,
11:1-133
Focal nodular hyperplasia (FNH), 11:1-106 to 11:1-108,
II:I-I09i
angiosarcoma vs., 11:1-141
differential diagnosis, II:I-106i, 11:1-107
fibrolamellar hepatocellular carcinoma vs., 11:1-
124i,II:1-125
hepatic adenoma vs., 1I:1-110i, 11:1-111
hepatocellular carcinoma vs., II:1-122
peliosis hepatis vs., II:I-80i, 11:1-81
Food
impactation vs. intramural benign esophageal tumor,
I:2-78i, 1:2-79
postprandial, vs. bezoar, I:3-68i, 1:3-69
retention vs. gastric polyps, 1:3-33
Foreign bodies
esophageal, 1:2-68 to 1:2-69
carcinoma vs., I:2-82i, 1:2-84
differential diagnosis, I:2-68i, 1:2-69
neurogenic bladder vs., III:S-16i, III:5-17
Freezing, gastric, I:3-28i, 1:3-29
Fundoplication
complications, I:3-60i, 1:3-60 to 1:3-62, I:3-63i
postoperative changes
esophageal motility disturbances vs., I:2-38i, 1:2-39
normal appearance, I:3-6Oi
Fungal abscess, 1:6-23
Fungal infections
adrenal glands, III:2-6i, III:2-6 to III:2-7
splenic metastasis vs., 1:6-32
splenomegaly vs., 1:6-36
G bezoar vs., I:3-68i, 1:3-69
Gallbladder
milk of calcium bile vs. hemorrhage, 1I:2-4Oi, 11:2-41
polyps
gallbladder carcinoma vs., II:2-46i, 11:2-47
hyperplastic cholecystoses vs., II:2-32i, 11:2-33
porcelain, 1I:2-38i, 11:2-38 to 11:2-39
Gallbladder carcinoma, 11:2-46 to 11:2-48, 1I:2-49i
differential diagnosis, 1I:2-46i, 11:2-47
duodenal ulcer vs., 1:3-19
hyperplastic cholecystoses vs., II:2-32i, 11:2-33
milk of calcium bile vs., II:2-40i, 11:2-41
Gallstones
dropped, I:4-72i, 1:4-73
ileus from, 1:4-72 to 1:4-73
porcelain gallbladder vs., II:2-38i
Ganglioneuroma
adrenal carcinoma vs., III:2-32
extra-adrenal. See Pheochromocytoma
Gardner syndrome, 1:5-60 to 1:5-61
differential diagnosis, I:S-60i, 1:5-61
hamartomatous polyposis vs., I:4-56i, 1:4-57
Gartner duct cyst
ectopic ureter vs., III:4-4i, III:4-5
ureterocele vs., III:4-8i, III:4-9
Gastric bypass complications, I:3-64i, 1:3-64 to 1:3-66,
I:3-67i
Gastric carcinoma, 1:3-46 to 1:3-48, I:3-49i
bezoar vs., I:3-68i, 1:3-69
caustic gastroduodenal injury vs., I:3-28i, 1:3-29
differential diagnosis, I:3-46i, 1:3-47 to 1:3-48
ectopic pancreatic tissue vs., II:3-16i, 11:3-17
esophageal achalasia vs., I:2-34i, 1:2-35
gastric polyps vs., I:3-32i, 1:3-33
gastritis vs., I:3-10i, 1:3-11
intramural benign gastric tumors vs., 1:3-39
lymphoma and metastasis vs., I:3-SOi, 1:3-53
Menetrier disease vs., I:3-26i, 1:3-27
stromal tumor vs., I:3-42i, 1:3-43
Zollinger-Ellison syndrome vs., I:3-22i, 1:3-23
Gastric distension, I:3-64i, 1:3-66
Gastric diverticulum, 1:3-6 to 1:3-7
adrenal adenoma vs., III:2-20i, III:2-22
adrenal carcinoma vs., III:2-30i, III:2-31
adrenal cyst vs., III:2-18i
differential diagnosis, I:3-6i, 1:3-6 to 1:3-7
Gastric metastases and lymphoma, 1:3-50 to 1:3-52, 1:3-S3i
bezoar vs., I:3-68i, 1:3-69
caustic gastroduodenal injury vs., I:3-28i, 1:3-29
differential diagnosis, I:3-S0i, 1:3-53
ectopic pancreatic tissue vs., 1I:3-16i, 11:3-17
gastric carcinoma vs., I:3-46i, 1:3-48
gastric polyps vs., I:3-32i, 1:3-33
gastric ulcer vs., I:3-14i, 1:3-15, 1:3-15 to 1:3-16
gastritis vs., 1:3-10i, 1:3-12
intramural benign gastric tumors vs., 1:3-39
Menetrier disease vs., I:3-26i, 1:3-27
stromal tumor vs., I:3-42i, 1:3-43
Zollinger-Ellison syndrome vs., I:3-22i, 1:3-23
Gastric mucosa, ectopic, 1:3-27, I:3-36i
Gastric polyps, 1:3-32i, 1:3-32 to 1:3-34, I:3-3Si
Gastric stromal tumors (GIST), 1:3-42 to 1:3-44, I:3-4Si
differential diagnosis, I:3-42i, 1:3-43
ectopic pancreatic tissue vs., 1I:3-16i, 11:3-17
gastric carcinoma vs., I:3-46i, 1:3-48
gastric lymphoma and metastasis vs., I:3-50i, 1:3-53
gastric polyps vs., 1:3-33 to 1:3-34
solid and papillary pancreatic neoplasms vs., 11:3-
S8i, 11:3-59
Gastric tumors. See also Gastric carcinoma; Gastric
stromal tumors (GIST)
esophageal motility disturbances vs., I:2-38i
gastric ulcer vs., I:3-14i, 1:3-15
gastritis vs., I:3-10i, 1:3-12
intramural benign, I:3-38i, 1:3-38 to 1:3-40, 1:3-4li
splenomegaly vs., I:6-34i, 1:6-35
 INDEX
Gastric ulcer, 1:3-14 to 1:3-16, I:3-17i prostatitis, III:6-10 to III:6-11
benign. See Peptic ulcer scrotal trauma, III:6-22 to III:6-23
differential diagnosis, I:3-14i, 1:3-15 to 1:3-16 testicular carcinoma, III:6-26 to III:6-28, III:6-29i
ectopic pancreatic tissue vs., 1I:3-16i, 11:3-17 testicular torsion, III:6-18 to III:6-19
gastrointestinal bleeding vs., I:4-SOi, 1:4-51 urethral stricture, III:6-12 to III:6-13
intramural benign gastric tumors vs., 1:3-39 varicocele, III:6-16 to III:6-17
technical artifacts vs., 1:3-16 Germ cell tumors, testicular, III:6-24i
Gastric volvus, I:3-72i, 1:3-73 Giardiasis, 1:4-12 to 1:4-13
Gastritis, 1:3-10 to 1:3-12, I:3-13i malabsorption conditions vs., I:4-74i, 1:4-7S
differential diagnosis, I:3-10i, 1:3-11 to 1:3-12 metastases and lymphoma vs., 1:4-66
erosive opportunistic infections vs., 1:4-14i, 1:4-15
gastric carcinoma vs., 1:3-47 Whipple disease vs., I:4-22i
gastric lymphoma and metastasis vs., I:3-SOi, 1:3-53 GIST.See Gastric stromal tumors (GIST)
gastrointestinal bleeding vs., 1:4-51 Glomerulonephritis, III:3-44 to III:3-45
gastric carcinoma vs., I:3-46i, 1:3-47, 1:3-48 differential diagnosis, III:3-44i
gastric ulcer vs., 1:3-15 HIV nephropathy vs., III:3-42i
H. pylori Glycogen storage disease, 1I:1-84i, 11:1-85
Menetrier disease vs., I:3-26i, 1:3-27 Glycogenic acanthosis, I:2-8i, 1:2-9
Zollinger-Ellison syndrome vs., I:3-22i, 1:3-23 Gonadal stromal tumors, III:6-24i, III:6-24 to III:6-25
Gastroduodenal disorders, 1:3-6 to 1:3-77 Gonadal vein, III:1-7
aorto-enteric fistula, 1:3-76 to 1:3-77
caustic injury, 1:3-28 to 1:3-29
duodenal carcinoma, 1:3-54 to 1:3-56, I:3-S7i H
duodenal diverticulum, 1:3-8 to 1:3-9
Hamartoma
duodenal hematoma and laceration, 1:3-30 to 1:3-31
biliary. See Biliary hamartoma
duodenal metastases and lymphoma, 1:3-58 to 1:3-S9
renal. See Angiomyolipoma, renal
duodenal polyps, 1:3-36 to 1:3-27
splenomegaly vs., 1:6-36
duodenal ulcer, 1:3-18 to 1:3-20, I:3-2li
Heart disease, liver congestion in, 11:1-68 to 11:1-70,
fundoplication complications, 1:3-60 to 1:3-62, I:3-63i
1I:1-7li
gastric bezoar, 1:3-68 to 1:3-70, I:3-71i
Heller myotomy, I:2-70i, 1:2-71
gastric bypass complications, 1:3-64 to 1:3-66, I:3-67i
HELLPsyndrome, 11:1-72 to 11:1-74, 1I:1-7Si
gastric carcinoma, 1:3-46 to 1:3-48, 1:3-49i
differential diagnosis, 1I:1-72i, 11:1-73
gastric diverticulum, 1:3-6 to 1:3-7
hepatic trauma vs., 1I:1-90i, 11:1-91
gastric lymphoma and metastases, 1:3-50 to 1:3-52,
Hemangioendothelioma, epithelioid, II:1-132 to II:1-
I:3-S3i
134, 1I:1-13Si
gastric polyps, 1:3-32 to 1:3-34, I:3-3Si
differential diagnosis, 1I:1-132i, 11:1-133
gastric stromal tumor, 1:3-42 to 1:3-44, I:3-4Si
focal confluent fibrosis vs., 1I:1-44i, 11:1-46
gastric tumors, intramural benign, 1:3-38 to 1:3-40,
Hemangioma. See also Cavernous hemangioma
I:3-4li
angiosarcoma vs., 1I:1-140i, 11:1-141
gastric ulcer, 1:3-14 to 1:3-16, I:3-17i
hepatic
gastric volvus, 1:3-72 to 1:3-74, I:3-7Si
cystvs., 11:1-100
gastritis, 1:3-10 to 1:3-12, I:3-13i
epithelioid hemangioendothelioma vs., 11:1-
Menetrier disease, 1:3-26 to 1:3-27
132i,II:1-133
Zollinger-Ellison syndrome, 1:3-22 to 1:3-24, I:3-2Si
hepatocellular carcinoma vs., 1I:1-120i, 11:1-122
Gastroduodenitis, corrosive, 1:3-28 to 1:3-29
hepatic metastases and lymphoma vs., 1I:1-144i,
Gastroesophageal reflux disease (GERD). See also Reflux
11:1-146
esophagitis
splenic cyst vs., 1:6-24
fundoplication complications, 1:3-60 to 1:3-62, I:3-63i
splenic metastasis vs., 1:6-31
Gastrointestinal bleeding, 1:4-50 to 1:4-52, I:4-S3i
splenomegaly vs., 1:6-36
differential diagnosis, I:4-SOi, 1:4-51 to 1:4-52
Hematocele, III:6-14i
intestinal scleroderma vs., I:4-30i, 1:4-31
Hematoma
metastases and lymphoma vs., I:4-64i, 1:4-66
benign gastric tumors vs., 1:3-39
Gaucher disease
benign intestinal tumors vs., I:4-S4i, 1:4-55
hepatic sarcoidosis vs., 1I:1-S6i, 11:1-57
carcinoid tumor vs., 1:4-61
splenic infection and abscess vs., 1:6-12i, 1:6-13
desmoid tumor vs., I:I-54i, 1:1-56
Genital tract, III:6-6 to III:6-33
duodenal. See Duodenum, hematoma
benign prostatic hypertrophy, III:6-20 to III:6-21
femoral hernia vs., I:I-28i, 1:1-29
cryptorchidism, III:6-6 to III:6-7
inguinal hernia vs., I:I-24i, 1:1-25
epididymitis, III:6-8 to III:6-9
intestinal metastases and lymphoma vs., I:4-64i, 1:4-66
gonadal stromal tumors, III:6-24 to III:6-25
intestinal trauma and, I:4-46i
hydrocele, III:6-14 to III:6-15
rectus sheath
prostate carcinoma, III:6-30 to III:6-32, III:6-33i
spigelian hernia vs., I:I-32i, 1:1-33
 INDEX
traumatic abdominal wall hernia vs., 1:1-44i, 1:1-45
retroperitoneallymphocele vs., 11I:1-32i
splenic tumor vs., 1:6-26i, 1:6-28
testicular
carcinoma vs., 11I:6-26i, III:6-27
gonadal stromal tumors vs., 1I1:6-24i
ventral hernia vs., 1:1-31
Hemochromatosis, 1I:1-S4i, 11:1-84 to 11:1-86, 1I:1-S7i
Hemoperitoneum
ascites vs., l:l-lSi, 1:1-20
bladder trauma vs., 1I1:5-1Si, III:5-19
mesenteric trauma vs., 1:1-46i, 1:1-47
peritonitis vs., I:I-I0i, 1:1-11
Hemorrhage
adrenal. See Adrenal hemorrhage
bladder trauma and, 11I:5-1Si, III:5-19
gastrointestinal. See Gastrointestinal bleeding
spontaneous. See Coagulopathy
Hemorrhoids, 1:5-4Si
Hemosiderosis, 1I:1-S4i, 11:1-85
Henoch-Schonlein purpura
intestinal trauma vs., 1:4-46i, 1:4-48
metastases and lymphoma vs., 1:4-66
Hepatectomy, 1I:1-6i, 11:1-7
Hepatic abscess
amebic, 11:1-28 to 11:1-30, 1I:1-3li
differential diagnosis, II: 1-2Si, II:1-29
pyogenic abscess vs., 1I:1-24i, 11:1-26
cholecystitis vs., 1I:2-34i, 11:2-35
infarction vs., 1I:1-76i, 11:1-77
metastases and lymphoma vs., 1I:1-144i, 11:1-146
peliosis hepatis vs., 11:1-81
pyogenic, 11:1-24 to 11:1-26, 1I:1-27i
amebic abscess vs., 1I:1-2Si, 11:1-29
differential diagnosis, 1I:1-24i, 11:1-25 to 11:1-26
hydatid cyst vs., 1I:1-32i, 11:1-33
intraductal papillary mucinous tumor vs., 11:2-
5Si, 11:2-59
Hepatic adenoma, 11:1-110 to 11:1-112, 1I:1-113i
angiomyolipoma vs., 1I:1-11Si, 11:1-119
angiosarcoma vs., 1I:1-140i, 11:1-141
differential diagnosis, 1I:1-110i, 11:1-111 to 11:1-112
focal nodular hyperplasia vs., 1I:1-106i, 11:1-107
peliosis hepatis vs., 1I:1-SOi, 11:1-81
Hepatic artery thrombosis, 1I:1-94i, 11:1-95
Hepatic candidiasis, 1I:1-20i, 11:1-20 to 11:1-22, 1I:1-23i
Hepatic congestion, passive, 11:1-68 to 11:1-70, 1I:1-7li
differential diagnosis, 1I:1-6Si, 11:1-69
hepatitis vs., 1I:1-16i, 11:1-17
Hepatic cyst, 11:1-98 to 11:1-100, 1I:1-10li
autosomal dominant polycystic liver disease vs.,
1I:1-12i, 11:1-13
biliary hamartoma vs., 1I:1-114i, 11:1-115
cystadenocarcinoma vs., 1I:1-136i, 11:1-137
differential diagnosis, 1I:1-9Si, 11:1-99 to 11:1-100
metastases and lymphoma vs., 1I:1-144i, 11:1-146
Hepatic fibrosis, congenital, 1I:1-Si, 11:1-8 to 11:1-10,
1I:1-11i
Hepatic infarction, 11:1-76 to 11:1-78, 1I:1-79i
amebic abscess vs., 1I:1-2Si, 11:1-29
differential diagnosis, 1I:1-76i, 11:1-77
pyogenic abscess vs., 1I:1-24i, 11:1-26
radiation hepatitis vs., 1I:1-14Si, 11:1-149
Hepatic metastases and lymphoma, II:1-144 to II:1-146,
1I:1-147i
amebic abscess vs., 1I:1-2Si, 11:1-29
angiosarcoma vs., 1I:1-140i, 11:1-141
biliary hamartoma vs., 1I:1-114i, 11:1-115
candidiasis vs., 1I:1-20i, 11:1-21
cirrhosis vs., 1I:1-40i, 11:1-42
differential diagnosis, II: 1-144i, II:1-146
epithelioid hemangioendothelioma vs., 1I:1-132i,
11:1-133
focal confluent fibrosis vs., 1I:1-44i, 11:1-45
primary biliary cirrhosis vs., 1I:1-4Si, 11:1-49
primary sclerosing cholangitis vs., 1I:2-42i, 11:2-44
pyogenic abscess vs., 1I:1-24i, 11:1-25
sarcoidosis vs., 1I:1-56i, 11:1-57
trauma vs., 1I:1-90i, 11:1-91
Hepatic stones, 1I:2-16i, 11:2-17
Hepatic trauma, 11:1-90 to 11:1-92, 1I:1-93i
differential diagnosis, II: 1-90i, II:1-91
HELLP syndrome vs., 1I:1-72i, 11:1-73
mesenteric trauma vs., 1:1-46i, 1:1-47
Hepatitis, 11:1-16 to 11:1-18, 1I:1-1Si
differential diagnosis, 1I:1-16i, 11:1-17
passive hepatic congestion vs., 1I:1-6Si, 11:1-69
radiation-induced, 1I:1-14Si, 11:1-148 to 11:1-149
steatosis vs., 1I:1-36i, 11:1-37
Wilson disease vs., 1I:1-SSi, 11:1-89
Hepatocellular carcinoma, 11:1-120to 11:1-122,1I:1-123i
angiosarcoma vs., 1I:1-140i, 11:1-141
arteriovenous malformation vs., II: 1-60i, II:1-62
cholangiocarcinoma vs., 1I:1-12Si, 11:1-130
differential diagnosis, 1I:1-120i, 11:1-122
epithelioid hemangioendothelioma vs., 1I:1-132i,
11:1-133
fibrolamellar, 11:1-124 to 11:1-126, 1I:1-127i
cholangiocarcinoma vs., 1I:1-12Si, 11:1-130
differential diagnosis, 1I:1-124i, 11:1-125to 11:1-126
focal nodular hyperplasia vs., 11:1-107.
hepatic adenoma vs., 1I:1-110i, 11:1-111
focal nodular hyperplasia vs., 1I:1-106i, 11:1-107
HELLPsyndrome vs., 11:1-73
hepatic angiomyolipoma vs., 1I:1-11Si, 11:1-119
hepatic trauma vs., 1I:1-90i, 11:1-91
metastases and lymphoma vs., 11:1-146
nodular regenerative hyperplasia vs., 1I:1-52i, 11:1-53
Hepatolenticular degeneration, 1I:1-SSi, 11:1-88to 11:1-89
Hereditary hemorrhagic telangiectasia, 1I:1-60i, 11:1-60
to 11:1-62, 1I:1-63i
Hernia
Bochdalek, 1:1-4Si, 1:1-49 to 1:1-50
femoral, 1:1-28 to 1:1-29
differential diagnosis, 1:1-2Si, 1:1-29
inguinal hernia vs., 1:1-24i, 1:1-25
obturator hernia vs., 1:1-34i
hiatal, 1:2-52 to 1:2-54, 1:2-55i
differential diagnosis, 1:2-52i, 1:2-53
gastric volvus vs., 1:3-72i, 1:3-73
pulsion diverticulum vs., 1:2-62i, 1:2-63 to 1:2-64
inguinal. See Inguinal hernia
internal, 1:4-6i, 1:4-7
obturator, 1:1-34 to 1:1-35
 INDEX
paraduodenal, 1:1-36 to 1:1-38, I:I-39i
parastomal,I:I-30i
sciatic,I:I-34
scrotal, III:6-14
spigelian, 1:1-32 to 1:1-33
differential diagnosis, I:I-32i, 1:1-33
traumatic abdominal wall hernia vs., I:I-44i
ventral hernia vs., I:I-3Oi
trans mesenteric
paraduodenal hernia vs., I:I-36i, 1:1-37
postoperative, I:I-4Oi, 1:1-40 to 1:1-42, I:I-43i
umbilical
spigelian hernia vs., I:I-32i
ventral hernia vs., I:I-30i, 1:1-31
ventral, 1:1-30 to 1:1-31
differential diagnosis, I:I-30i, 1:1-31
spigelian hernia vs., I:I-32i
traumatic abdominal wall hernia vs., I:I-44i, 1:1-45
Herpes esophagitis
Candida esophagitis vs., I:2-8i, 1:2-9
drug-induced esophagitis vs., I:2-28i, 1:2-29
reflux esophagitis vs., I:2-16i, 1:2-17
Hiatal hernia, 1:2-52 to 1:2-54, I:2-55i
differential diagnosis, I:2-52i, 1:2-53
gastric volvus vs., I:3-72i, 1:3-73
pulsion diverticulum vs., I:2-62i, 1:2-63 to 1:2-64
Histoplasmosis, III:2-10
HIV infections. See a/so AIDS-related infections
esophagitis, 1:2-17
nephropathy, III:3-42i, III:3-42 to III:3-43
Hodgkin disease, II:I-144i, 11:1-144to 11:1-146,II:I-147i
Horseshoe kidney, III:3-10 to 111:3-12,III:3-13i
differential diagnosis, III:3-10i, III:3-11
renal ectopia vs., III:3-8
Human papilloma virus esophagitis. See Viral esophagitis
Hydatid cyst, 11:1-32to 11:1-34, II:I-35i
amebic abscess vs., II:I-28i, 11:1-29
cystadenocarcinoma vs., II:I-136i, 11:1-137
differential diagnosis, II:I-32i, 11:1-33 to 11:1-34
hepatic cyst vs., 11:1-100
pyogenic abscess vs., II:I-24i, 11:1-26
splenic cyst vs., I:6-22i, 1:6-23
Hydrocele, III:6-14i, III:6-14 to III:6-15
Hydronephrosis
glomerulonephritis vs., III:3-44i
renal cyst vs., III:3-81
renal papillary necrosis vs., III:3-46i, III:3-47
xanthogranulomatous pyelonephritis vs., III:3-37
Hypercalcemia, III:3-60i, III:3-61
Hyperparathyroidism, III:3-61
Hypersplenism. See Splenomegaly and hypersplenism
I pancreatic cyst vs., II:3-4Oi, 11:3-41
Iatrogenic injury
biliary, II:2-60i, 11:2-61
biliary trauma vs., II:I-94i, 11:1-95
Boerhaave syndrome vs., I:2-74i, 1:2-75
emphysematous pyelonephritis vs., III:3-4Oi, III:3-41
femoral hernia vs., I:I-28i, 1:1-29
inguinal hernia vs., I:I-24i, 1:1-25
porcelain gallbladder vs., II:2-38i
Ileocecal syndrome. See Typhlitis
Ileocolitis, I:5-22i, 1:5-23
Ileus
cecal volvulus vs., I:5-66i
gallstone, 1:4-72 to 1:4-73
postoperative, I:3-64i, 1:3-66
sigmoid volvulus vs., I:5-62i, 1:5-63
small bowel, 1:4-31
small bowel obstruction vs., 1:4-68i, 1:4-70
toxic megacolon vs., I:5-20i, 1:5-21
Immunoglobulin A (lgA) deficiency, 1:4-20
Immunologic disorders, I:4-18i, 1:4-20
Incisional hernia. See Ventral hernia
Inferior vena cava, duplications and anomalies, III:I-6i,
III:1-6 to III:1-7
Inguinal hernia, 1:1-24 to 1:1-26, 1:1-27i
cryptorchidism vs., III:6-6i
differential diagnosis, I:I-24i, 1:1-25 to 1:1-26
femoral hernia vs., I:I-28i
obturator hernia vs., I:I-34i
Interstitial nephritis, acute
glomerulonephritis vs., III:3-44i
HIV nephropathy vs., III:3-42i
Intestinal metastases and lymphoma, 1:4-64 to 1:4-66,
I:4-67i
carcinoid tumor vs., I:4-60i, 1:4-61
Crohn disease vs., 1:4-28
differential diagnosis, I:4-64i, 1:4-66
duodenal carcinoma vs., I:3-54i, 1:3-55
duodenal hematoma vs., I:3-30i, 1:3-31
duodenal ulcer vs., 1:3-20
familial polyposis vs., 1:5-57
Gardner syndrome vs., 1:5-61
hamartomatous polyposis vs., I:4-56i, 1:4-57
intramural benign intestinal tumors vs., I:4-54i
intussusception vs., I:4-78i, 1:4-79
opportunistic infections vs., I:4-14i, 1:4-15
radiation enteritis vs., 1:4-82i, 1:4-83
small bowel carcinoma vs., 1:4-58i, 1:4-59
Whipple disease vs., I:4-22i
Intestinal trauma, I:4-46i, 1:4-46 to 1:4-48, I:4-49i
Intestinal tumors
intramural benign, I:4-54i, 1:4-54 to 1:4-55
intussusception vs., I:4-78i, 1:4-79
radiation enteritis vs., 1:4-83
Intraductal papillary mucinous tumor
of bile ducts, II:2-58i, 11:2-58 to 11:2-59
of pancreas, 11:3-46 to 11:3-48, II:3-49i
chronic pancreatitis vs., II:3-28i, 11:3-29
differential diagnosis, II:3-46i, 11:3-47
duodenal diverticulum vs., I:3-8i, 1:3-9
mucinous cystic pancreatic tumor vs., II:3-42i,
11:3-43
pancreatic pseudocyst vs., II:3-24i, 11:3-25
serous cystadenoma vs., II:3-36i, 11:3-37
Intussusception, 1:4-78 to 1:4-80, I:4-8li
differential diagnosis, I:4-78i, 1:4-79
gallstone ileus vs., I:4-72i
Ischemia, intestinal. See a/so Colitis, ischemic; Enteritis,
ischemic
Crohn disease vs., 1:4-28
 INDEX
gallstone ileus vs., I:4-72i, 1:4-73
sprue-celiac disease vs., I:4-18i, 1:4-19
Islet cell tumors, II:3-54 to II:3-56, I1:3-S7i
differential diagnosis, I1:3-S4i, II:3-5S
metastasis vs., I1:3-60i, II:3-61
mucinous cystic pancreatic tumor vs., II:3-43
pancreatic ductal carcinoma vs., I1:3-SOi, II:3-51
pancreatic pseudocyst vs., II:3-25
serous cystadenoma vs., II:3-38
J gastric ulcer vs., I:3-14i, 1:3-15
Jejunal lymphoma, I:4-S8i
Juvenile polyposis, I:4-S6i, 1:4-56 to 1:4-57
K villous adenoma vs., 1:5-54
Kaposi's sarcoma
gastric ulcer vs., 1:3-15
retroperitoneal lymphoma vs., III:I-25
Kidney, III:3-6 to III:3-109
abscess, III:3-32 to III:3-34, III:3-3Si
acquired cystic disease of uremia, III:3-56 to III:3-58,
III:3-S9i
angiomyolipoma, III:3-88 to III:3-90, III:3-9li
autosomal dominant polycystic kidney disease, III:3-
16 to III:3-18, III:3-19i
carcinoma, III:3-96 to III:3-98, I1I:3-99i
medullary, III:3-100 to III:3-101
transitional cell, III:3-102 to III:3-104, III:3-lOSi
column of Bertin, III:3-14 to III:3-15
cyst, III:3-80 to III:3-82, III:3-83i
displacement
horseshoe kidney vs., I1I:3-10i, III:3-11
renal ectopia vs., I1I:3-6i, III:3-8
ectopic, III:3-6 to III:3-8, III:3-9i
extrinsic indentation, III:3-26
glomerulonephritis, III:3-44 to III:3-45
HIV nephropathy, III:3-42 to III:3-43
horseshoe kidney, III:3-10 to III:3-12, III:3-13i
infarction, III:3-68 to III:3-70, I1I:3-7li
medullary sponge kidney, III:3-60 to III:3-62, III:3-63i
metastases and lymphoma, III:3-106 to III:3-108,
II1:3-109i
multilocular cystic nephroma, III:3-92 to III:3-94,
II1:3-9Si
nephrocalcinosis, III:3-52 to III:3-54, I1I:3-SSi
oncocytoma, III:3-84 to III:3-86, III:3-87i
pyelonephritis, III:3-28 to III:3-30, II1:3-3li
emphysematous, III:3-40 to III:3-41
xanthogranulomatous, III:3-36 to III:3-38, III:3-39i
renal artery stenosis, III:3-64 to III:3-66, I1I:3-67i
renal papillary necrosis, III:3-46 to III:3-47
renal vein thrombosis, III:3-72 to III:3-74, III:3-7Si
trauma, III:3-76 to III:3-78, I1I:3-79i
ureteropelvic junction obstruction, III:3-24 to III:3-
26, III:3-27i
urolithiasis, III:3-48 to III:3-50, III:3-Sli
von Hippel Lindau disease, III:3-20 to III:3-22, III:3-23i
Kidney failure, III:3-S2i
Killian-Jamieson diverticulum, I:2-56i, 1:2-57
L
Laparoscopy port hernia
spigelian hernia vs., I:I-32i, 1:1-33
traumatic abdominal wall hernia vs., I:I-44i
Leiomyoma
carcinoma vs., I:2-82i, 1:2-84
colonic polyps vs., 1:5-41
fibrovascular polyp vs., I:2-80i, 1:2-81
gallstone ileus vs., 1:4-73
gastric polyps vs., 1:3-34
metastasis vs., I:2-86i, 1:2-87
villous adenoma vs., 1:5-54
Leiomyosarcoma
pseudomyxoma peritonei vs., I:I-66i
small bowel carcinoma vs., 1:4-58
Leukemia
gonadal stromal tumors vs., III:6-24
hepatic candidiasis vs., I1:1-20i, II:I-21
Lipodystrophy, intestinal. See Whipple disease
Lipoma
colonic polyps vs., 1:5-41, I:S-49i
gallstone ileus vs., 1:4-73
stromal tumor vs., I:3-42i, 1:3-43
villous adenoma vs., I:S-S2i, 1:5-54
Liposarcoma
angiomyolipoma vs., II:I-119, I1I:3-88i, III:3-90
myelolipoma vs., III:2-24i, III:2-25
Liver, II:1-6 to II:I-149
abscess
amebic, II:I-28 to II:1-30, I1:1-3li
pyogenic, II:1-24 to II:1-26, I1:1-27i
adenoma, II:1-110 to II:I-112, I1:1-113i
angiomyolipoma, II:I-118 to II:1-119
angiosarcoma, II:I-140 to II:I-142, I1:1-143i
arteriovenous malformation, II:1-60 to II:1-62, II:1-63i
autosomal dominant polycystic liver disease, II:I-12
to II:I-14, I1:1-1Si
biliary cirrhosis, primary, II:1-48 to II:1-50, I1:1-Sli
biliary cystadenocarcinoma, II:I-136 to II:1-138,
I1:1-139i
biliary hamartoma, II:1-114 to II:1-116, I1:1-117i
biliary trauma, II:I-94 to II:1-96, II:I-97i
Budd-Chiari syndrome, II:1-64 to II:I-66, I1:1-67i
candidiasis, II:1-20 to II:1-22, II: 1-23i
cavernous hemangioma, II:l-lO2 to II:l-lO4, II:l-lOSi
cholangiocarcinoma, peripheral, II:I-128 to II:1-130,
II: 1-13 li
congenital absence of hepatic segments, II:1-6 to II:I-7
epithelioid hemangioendothelioma, II:I-132 to II:l-
134,I1:1-135i
fibrolamellar hepatocellular carcinoma, II:1-124 to
II:I-126,I1:1-127i
fibrosis, congenital, II:l-lO to II:1-11
focal confluent fibrosis, II:I-44 to II:1-46, I1:1-47i
focal nodular hyperplasia, II:I-I06 to II:I-I08,
I1:1-109i
HELLPsyndrome, II:1-72 to II:I-74, I1:1-7Si
hemochromatosis, II:I-84 tolI:I-86, I1:1-87i
hepatic cyst, II:I-98 to II:I-I00, I1:1-10li
hepatic trauma, II:1-90 to II:1-92, I1:1-93i
 INDEX
hepatitis, II:1-16 to II:1-18, II:1-18i
hepatocellular carcinoma, II:1-120 to II:1-122,
II:1-123i
hydatid cyst, II:1-32 to II:1-34, II:1-35i
infarction, II:1-76 to II:1-78, II:1-79i
metastases and lymphoma, II:1-144 to II:1-146,
II:1-147i
nodular regenerative hyperplasia, II:1-52 to II:1-54,
II:l-SSi
passive hepatic congestion, II:1-68 to II:1-70, II:l-71i
peliosis hepatis, II:1-80 to II:1-82, II:1-83i
radiation hepatitis, II:1-148 to II:1-149
sarcoidosis, II:1-56 to II:1-58, II:l-S9i
steatosis, II:1-36 to II:1-38, II:1-39i
Wilson disease, II:1-88 to II:1-89
Lung cancer, I:2-82i, 1:2-84
Lupus erythematosus, systemic. See Systemic lupus
erythematosus
Lye burns. See Caustic esophagitis
Lymphadenopathy
cryptorchidism vs., III:6-6i
femoral hernia vs., I:1-28i
inferior vena cava anomalies vs., III:1-6i, III:1-7
inguinal hernia vs., I:1-24i, 1:1-26
pelvic lipomatosis vs., III:1-14i, III:1-15
Lymphangiectasia, I:4-24i, 1:4-25
Lymphangioleiomyomatosis, III:1-28i, III:1-29
Lymphangioma
mesenteric cyst vs., I:l-S2i
mucinous cystic pancreatic tumor vs., II:3-43
pancreatic cyst vs., II:3-40i, II:3-41
retroperitoneallymphocele vs., III:1-33
splenic cyst vs., 1:6-24
splenomegaly vs., 1:6-36
Lymphocele
abdominal abscess vs., I:1-6i, 1:1-7
bladder diverticulum vs., III:S-12i, III:5-13
retroperitoneal, III:1-32i, III:1-32 to III:1-33
Lymphogranuloma, I:S-48i, 1:5-49
Lymphoid follicles, I:4-S6i, 1:4-57
Lymphoid hyperplasia, I:S-60i, 1:5-61
Lymphoma. See also specific systems, metastases and
lymphoma
acute pancreatitis vs., II:3-20i, II:3-21
appendiceal, I:S-26i, 1:5-27
bladder, III:S-22i, III:5-23
desmoid tumor vs., I:1-54i, 1:1-55
esophageal, 1:2-86 to 1:2-87
gonadal stromal tumors vs., III:6-24i
hepatitis vs .., II:1-16i, II:1-17
mesothelioma vs., 1:1-59
non-Hodgkin. See Non-Hodgkin lymphoma (NHL)
pancreatic ductal carcinoma vs., II:3-50i, II:3-51
retroperitoneal, III:1-24 to III:1-26, III:1-27i
differential diagnosis, III:1-24i, III:1-25
fibrosis vs., III:l-lOi, III:1-11
metastasis vs., III:1-28i, III:1-29
small bowel carcinoma vs., I:4-58i, 1:4-59
steatosis vs., II:1-36i, II:1-37
testicular carcinoma vs., III:6-26i, III:6-27
Wilson disease vs., II:1-88i, II:1-89
Lymphomatosis, I:l-S8i, 1:1-59
M
Macroglobulinemia, Walden strom
sprue-celiac disease vs., I:4-18i, 1:4-20
Whipple disease vs., I:4-22i, 1:4-23
MAl infections. See Mycobacterium avium-intracellulare
infections
Malabsorption conditions, 1:4-74 to 1:4-76, I:4-77i
differential diagnosis, I:4-74i, 1:4-75 to 1:4-76
mastocytosis vs., I:4-24i, 1:4-25
Malignant melanoma. See Melanoma
Mallory-Weiss syndrome, 1:2-75
Malrotation, small intestine, I:4-6i, 1:4-6 to 1:4-7
Mastocytosis, I:4-24i, 1:4-24 to 1:4-25
Meckel diverticulum, 1:4-8 to 1:4-10, I:4-11i
differential diagnosis, I:4-8i, 1:4-9 to 1:4-10
intussusception vs., I:4-78i, 1:4-79
Mediastinum, normal structures, I:2-78i, 1:2-79
Medullary carcinoma, renal, III:3-100i, III:3-100 to
III:3-101
Medullary cystic disease
acquired cystic disease of uremia vs., III:3-5 7
autosomal dominant polycystic kidney disease vs.,
III:3-18
van Hippel Lindau disease vs., III:3-22
Medullary sponge kidney, III:3-60 to III:3-62, III:3-63i
differential diagnosis, III:3-60i, III:3-61 to III:3-62
renal papillary necrosis vs., III:3-46i, III:3-47
Megacalices, congenital, III:3-47
Megacolon, toxic, 1:5-20 to 1:5-21
cecal volvulus vs., 1:5-66
differential diagnosis, I:S-20i, 1:5-21
sigmoid volvulus vs., I:S-62i, 1:5-63
Megaureter, primary, III:3-48i, III:3-50
Melanoma
cystitis vs., III:S-8i
intramural benign intestinal tumors vs., I:4-54i
metastasis
adrenal adenoma vs., III:2-20i, III:2-21 to III:2-22
gastric polyps vs., I:3-32i, 1:3-33
gastric ulcer vs., I:3-14i, 1:3-15
intussusception vs., I:4-78i, 1:4-79
small bowel carcinoma vs., I:4-S8i, 1:4-59
splenic cyst vs., I:6-22i, 1:6-24
splenomegaly vs., I:6-34i, 1:6-35 to 1:6-36
uteritis cystica vs., III:4-10i, III:4-11
Menetrier disease, 1:3-26 to 1:3-27
differential diagnosis, I:3-26i, 1:3-27
gastric carcinoma vs., 1:3-48
Mesenchymal tumor, II:2-55
Mesenteric disorders
adenitis
appendicitis vs., I:5-22i, 1:5-23
Crohn disease vs., I:4-26i, 1:4-28
Meckel diverticulum vs., I:4-8i, 1:4-10
cyst, 1:1-52 to 1:1-53
differential diagnosis, I:l-S2i, 1:1-52 to 1:1-53
urachal remnant vs., III:5-7
edema, 1:1-15
ischemia. See Enteritis, ischemic
trauma, I:1-46i, 1:1-46 to 1:1-47
Mesenteritis
 INDEX
fibrosing
carcinoid tumor vs., I:4-60i, 1:4-62
desmoid tumor vs., I:l-S4i, 1:1-56
ischemic enteritis vs., I:4-38i, 1:4-39
omental infarct vs., I:1-22i, 1:1-23
retractile, mesothelioma vs., 1:1-59
sclerosing, I:1-14i, 1:1-14 to 1:1-16, I:1-17i
Mesothelioma, 1:1-58 to 1:1-60, I:1-6li
desmoid tumor vs., 1:1-55 to 1:1-56
differential diagnosis, I: 1-S8i, I:1-59
mesenteric cyst vs., 1:1-52
peritoneal metastases vs., I:1-62i, 1:1-63
Metastasis. See also specific systems, metastases and
lymphoma
chemotherapy cholangitis vs., II:2-60i, 11:2-61
cholangiocarcinoma vs., II:1-128i, 11:1-130
cystic
autosomal dominant polycystic liver disease vs.,
II:1-12i, 11:1-13
cystadenocarcinoma vs., II:1-136i, 11:1-137
hepatic cyst vs., II:1-98i, 11:1-99
hepatic hydatid cyst vs., II:1-32i, II:1-33
peritoneal, ascites in, I: 1-18i, I:1-20
desmoid tumor vs., I:l-S4i, 1:1-55
esophageal, 1:2-86 to 1:2-87
gallbladder carcinoma vs., II:2-46i, 11:2-47
hypervascular
cavernous hemangioma vs., II:I-I02i, 11:1-104
focal nodular hyperplasia vs., II:1-106i, 11:1-107
hepatic adenoma vs., II:l-llOi, 11:1-112
hepatocellular carcinoma vs., II:1-120i, 11:1-122
peliosis hepatis vs., II:1-80i, 11:1-81
islet cell tumors vs., II:3-S4i, 1I:3-S5
peritoneal, I:1-62i, 1:1-62 to 1:1-64, I:1-65i
retroperitoneal, III:1-28 to III:1-30, III:1-3li
differential diagnosis, III: 1-28i, III:1-29
fibrosis vs., III:I-IOi, III:l-11
hemorrhage in, III:1-16i, III:I-17
testicular
gonadal stromal tumors vs., III:6-24i
retroperitoneal lymphoma vs., III:1-24i, III:1-25
urachal remnant vs., III:S-6i, III:5-7
Milk-alkali syndrome, III:3-61
Milk of calcium bile, II:2-40i, 11:2-40 to 11:2-41
Mirizzi syndrome, II:2-30i, 11:2-30 to 11:2-31
Moniliasis. See Candida infections, esophagitis
Mucinous cystic pancreatic tumor, 11:3-42 to 11:3-44,
II:3-4Si
differential diagnosis, II:3-42i, 11:3-43
duodenal diverticulum vs., I:3-8i
intraductal papillary mucinous tumor vs., II:3-46i,
11:3-47
islet cell tumors vs., II:3-S4i, 11:3-55
pancreatic metastasis vs., II:3-60i, 11:3-61
pancreatic pseudocyst vs., II:3-24i, 11:3-25
serous cystadenoma vs., II:3-36i, 11:3-37
solid and papillary neoplasms vs., II:3-S8i
Mucinous tumors. See Intraductal papillary mucinous
tumor; Mucinous cystic pancreatic tumor
Mucocele, I:S-26i, 1:5-26 to 1:5-27
Multicystic dysplastic kidney, III:3-92i, III:3-93
Multilocular cystic nephroma, III:3-92 to III:3-94, III:3-9Si
differential diagnosis, III:3-92i, III:3-93
renal cyst vs., III:3-80i, III:3-82
Multiple myeloma
glomerulonephritis vs., III:3-44
renal abscess vs., III:3-32i, III:3-33
Muscular rings, esophageal, 1:2-S0i, 1:2-51
Mycobacterium avium-intracellulare infections
Crohn disease vs., 1:4-27
HIV nephropathy vs., III:3-43
metastases and lymphoma vs., 1:4-66
splenomegaly vs., I:6-34i, 1:6-36
Whipple disease vs., 1:4-22
Myelolipoma, adrenal, III:2-24 to III:2-25
adenoma vs., III:2-22
carcinoma vs., III:2-30i, III:2-31
collision tumor vs., III:2-38i, III:2-39
cyst vs., III:2-18i
differential diagnosis, III:2-24i, III:2-25
metastasis vs., III:2-36
pheochromocytoma vs., III:2-26i, III:2-28
retroperitoneal sarcoma vs., III:1-20i, III:I-21
tuberculosis vs., III:1-21, III:2-7
Myeloma, multiple
glomerulonephritis vs., III:3-44
renal abscess vs., III:3-32i, III:3-33
Myeloproliferative disorders
hepatitis VSoo, II:1-17
primary biliary cirrhosis vs., 11:1-49
N
Nasogastric intubation
caustic esophagitis vs., I:2-24i, 1:2-25
radiation esophagitis vs., 1:2-31
Necrotizing enteropathy. See Typhlitis
Nephrocalcinosis, III:3-52 to III:3-54, III:3-SSi
differential diagnosis, III:3-S2i, 1II:3-53
medullary sponge kidney vs., III:3-60i, III:3-61
urolithiasis vs., III:3-49
Nephrolithiasis. See Urolithiasis
Nephroma, multilocular cystic. See Multilocular cystic
nephroma
Nephrostomy, III:3-40i, III:3-41
Neurogenic bladder, III:S-16i, III:5-16 to III:5-17
Neurogenic mass, III:1-24i, III:I-25
Neuromuscular disorders, 1:2-36
Neutropenic enterocolitis. See Typhlitis
Nodular regenerative hyperplasia, 11:1-52 to 11:1-54,
II:l-SSi
differential diagnosis, II:l-S2i, 11:1-53
hepatocellular carcinoma vs., II:1-120i, 11:1-122
Non-Hodgkin lymphoma (NHL), 11:1-144 to 11:1-146,
II:1-147i
carcinoid tumor vs., I:4-60i, 1:4-61
Crohn disease vs., 1:4-28
desmoid tumor vs., I:1-55
differential diagnosis, II:1-144i, 11:1-146
intussusception vs., I:4-78i, 1:4-79
radiation enteritis vs., I:4-82i, 1:4-83
sclerosing mesenteritis vs., I:1-14i, 1:1-15
small bowel carcinoma vs., I:4-S8i
Whipple disease vs., I:4-22i
 INDEX
o pancreatitis
Obstructive uropathy, acute, III:3-42i, III:3-43
Obturator hernia, I:1-34i, 1:1-34 to 1:1-35
Ogilvie syndrome
cecal volvulus vs., I:S-66i, 1:5-67
sigmoid volvulus vs., I:S-62i, 1:5-63
Omental infarct, 1:1-22i, 1:1-22 to 1:1-23
Oncocytoma, renal, III:3-84 to III:3-86, 1I1:3-S7i
angiomyolipoma vs., III:3-89
column of Bertin vs., III:3-15
differential diagnosis, III:3-S4i, III:3-85 to III:3-86
renal cell carcinoma vs., 11I:3-96i, III:3-97
Opportunistic infections
AIDS-related,I:4-22i
hepatic sarcoidosis vs., 1I:1-S6i, II:1-57
intestinal, 1:4-14 to 1:4-16, I:4-17i
differential diagnosis, I:4-14i, 1:4-15
metastases and lymphoma vs., 1:4-66
malabsorption conditions vs., I:4-74i, 1:4-75 to 1:4-76
primary biliary cirrhosis vs., II:1-49
sprue-celiac disease vs., I:4-18i, 1:4-19
Orchitis
gonadal stromal tumors vs., III:6-24
testicular carcinoma vs., III:6-27
Osler-Weber Rendu disease, 1I:1-60i, II:1-60 to II:1-62,
1I:1-63i
Osteophytes, cervical, I:2-32i
Ovarian cancer
ascites in, I:1-1Si, 1:1-20
colon carcinoma vs., 1:5-45
neurogenic bladder vs., III:S-16i, III:5-17
solid and papillary pancreatic neoplasms vs., 11:3-
S8i, II:3-59
Ovarian cysts
bladder diverticulum vs., III:5-13
mucocele of appendix vs., 1:5-27
Oxalosis, III:3-60i, III:3-61 to III:3-62
p solid and papillary pancreatic neoplasms vs., 11:3-
Pancreas, II:3-6 to II:3-63
annular, II:3-8 to II:3-10, 1I:3-11i
cystic fibrosis, II:3-18 to II:3-19
cysts, II:3-40 to II:3-41
dorsal, agenesis of, II:3-6 to II:3-7
differential diagnosis, 1I:3-6i, Il:3-7
pancreas divisum vs., 1I:3-12i, Il:3-13
ductal carcinoma, Il:3-50 to II:3-52, 1I:3-53i
ectopic tissue, II:3-16 to II:3-17
differential diagnosis, 1I:3-16i, II:3-17
gastric polyps vs., I:3-32i, 1:3-34
intramural benign gastric tumors vs., 1:3-39
fatty lobulation, normal, 1I:3-1Si, II:3-19
intraductal papillary mucinous tumor, II:3-46 to II:3-
48, 1I:3-49i
islet cell tumors, II:3-54 to II:3-56, 1I:3-S7i
mass vs. gastric diverticulum, 1:3-7
metastases and lymphoma, II:3-60 to Il:3-62, 1I:3-63i
mucinous cystic pancreatic tumor, II:3-42 to II:3-44,
1I:3-4Si
pancreas divisum, Il:3-12 to II:3-14, 1I:3-1Si
acute, II:3-20 to II:3-22, 1I:3-23i
chronic, II:3-28 to II:3-30, 1I:3-3li
traumatic, II:3-32 to II:3-34, 1I:3-3Si
pseudocyst, II:3-24 to II:3-26, 1I:3-27i
serous cystadenoma, II:3-36 to II:3-38, 1I:3-39i
"shock" pancreas
acute pancreatitis vs., 1I:3-20i, II:3-21
traumatic pancreatitis vs., 1I:3-32i, II:3-33
solid and papillary neoplasms, II:3-58 to II:3-59
Pancreas divisum, II:3-12 to II:3-14, 1I:3-1Si
agenesis of dorsal pancreas vs., 1I:3-6i, II:3-7
differential diagnosis, 1I:3-12i, 11:3-13
Pancreatic cysts, II:3-40 to II:3-41
differential diagnosis, 1I:3-40i, II:3-41
mucinous cystic pancreatic tumor vs., 1I:3-42i, II:3-43
pancreatic pseudocyst vs., 1I:3-24i, II:3-25
serous cystadenoma vs., 1I:3-36i, II:3-38
Pancreatic ductal carcinoma, II:3-50 to II:3-52, 1I:3-S3i
acute pancreatitis vs., 1I:3-20i, II:3-21
agenesis of dorsal pancreas vs., 1I:3-6i
ampullary carcinoma vs., 1I:2-S4i, II:2-55
annular pancreas vs., 1I:3-Si, II:3-9
cholangiocarcinoma vs., 1I:2-S0i, II:2-51
choledochal cyst vs., 1I:2-10i, II:2-11
choledocholithiasis vs., 1I:2-26i, II:2-28
chronic pancreatitis vs., 1I:3-2Si, II:3-29
differential diagnosis, 1I:3-S0i, 11:3-51
duodenal ulcer vs., 1:3-19
intraductal papillary mucinous tumor vs., 1I:3-46i,
II:3-47
islet cell tumors vs., 1I:3-S4i, II:3-55
mucinous cystic pancreatic tumor vs., II:3-43
pancreas divisum vs., 1I:3-12i, Il:3-13
serous cystadenoma vs., 1I:3-36i, II:3-37
Pancreatic metastases and lymphoma, II:3-60 to Il:3-62,
1I:3-63i
acute pancreatitis vs., II:3-21
differential diagnosis, 1I:3-60i, II:3-61
pancreatic ductal carcinoma vs., 1I:3-SOi, II:3-51
SSi, II:3-59
Pancreatic neoplasms
carcinoma. See Pancreatic ductal carcinoma
solid and papillary, 1I:3-SSi, II:3-58 to II:3-59
Pancreatitis
acute, II:3-20 to II:3-22, 1I:3-23i
cholecystitis vs., 1I:2-34i, II:2-35
differential diagnosis, 1I:3-20i, II:3-21
chronic, II:3-28 to II:3-30, 1I:3-3li
agenesis of dorsal pancreas vs., 1I:3-6i, II:3-7
cholangiocarcinoma vs., 1I:2-S0i, II:2-5 1
choledocholithiasis vs., 1I:2-26i, II:2-28
cystic fibrosis vs., 1I:3-18i, II:3-19
differential diagnosis, 1I:3-2Si, II:3-29
intraductal papillary mucinous tumor vs., 11:3-
46i, II:3-47
pancreas divisum vs., 1I:3-12i, II:3-13
pancreatic ductal carcinoma vs., 1I:3-S0i, II:3-51
gastric carcinoma vs., 1:3-48
gastric lymphoma and metastasis vs., I:3-SOi, 1:3-53
gastritis vs., I:3-10i, 1:3-12
 INDEX
malabsorption conditions vs., I:4-74i, 1:4-76
Menetrier disease vs., I:3-26i, 1:3-27
omental infarct vs., I:I-22i
traumatic, 11:3-32 to 11:3-34, II:3-3Si
differential diagnosis, II:3-32i, 11:3-33
mesenteric trauma vs., I:I-46i, 1:1-47
Zollinger-Ellison syndrome vs., 1:3-24
Pancreato-biliary parasites, II:2-20i, 11:2-20 to 11:2-21
Pannus, I:I-30i, 1:1-31
Papillary blush, III:3-62
Papillary serous carcinoma, I:I-62i, 1:1-63
Papillary stenosis, II:2-26i, 11:2-28
Paraduodenal hernia, I:I-36i, 1:1-36 to 1:1-38, I:I-39i
Paraganglioma. See Pheochromocytoma
Parasitic infections
intestinal, I:4-I2i, 1:4-12 to 1:4-13
pancreato-biliary, II:2-20i, 11:2-20 to 11:2-21
Parastomal hernia, I:I-3Oi
Peliosis, splenic, 1:6-23
Peliosis hepatis, II:I-80i, 11:1-80 to 11:1-82, II:I-83i
Pelvic cysts, III:5-17
Pelvic inflammatory disease (PID), I:S-22i, 1:5-23
Pelvic lipomatosis,III:I-14i, III:1-14 to III:I-15
Pelvis, narrow, III:l-14i, III:I-15
Peptic stricture
esophageal achalasia vs., I:2-34i, 1:2-35
esophageal carcinoma vs., I:2-82i
esophageal motility disturbances vs., I:2-38i, 1:2-39
esophageal webs vs., I:2-6i
Schatzki ring vs., I:2-SOi
scleroderma vs., I:2-42i, 1:2-43
Peptic ulcer. See also Gastric ulcer
annular pancreas vs., II:3-8i, 11:3-9
cholecystitis vs., II:2-34i, 11:2-35
duodenal carcinoma vs., 1:3-55
gastric carcinoma vs., I:3-46i, 1:3-47
gastrointestinal bleeding vs., I:4-S0i, 1:4-51
Perfusion artifacts
splenic metastasis vs., I:6-30i, 1:6-32
splenic tumors vs., I:6-26i, 1:6-28
Periaortitis, I:3-76i
Perineal hernia, 1:1-34
·Peritoneal metastases, I:I-62i, 1:1-62 to 1:1-64, I:I-6Si
Peritoneum, mesentery, and abdominal wall, I:1-6 to I:1-69
abdominal abscess, 1:1-6 to 1:1-8, I:I-9i
ascites, 1:1-18 to 1:1-20, I:I-2li
desmoid, 1:1-54 to 1:1-56, I:I-S7i
femoral hernia, I:1-28 to I:1-29
inguinal hernia, 1:1-24 to 1:1-26, I:I-27i
mesenteric cyst, 1:1-52 to 1:1-53
mesenteric trauma, 1:1-46 to 1:1-47
mesothelioma, 1:1-58 to 1:1-60, I:I-6li
obturator hernia, I:1-34 to I:1-35
omental infarct, 1:1-22 to 1:1-23
paraduodenal hernia, 1:1-36 to 1:1-38, I:I-39i
peritoneal metastases, 1:1-62 to 1:1-64, I:I-6Si
peritonitis, 1:1-10 to 1:1-12, I:I-13i
pseudomyxoma peritonei, 1:1-66 to 1:1-68, I:I-69i
sclerosing mesenteritis, 1:1-14 to 1:1-16, I:I-17i
spigelian hernia, I:1-32 to I:1-33
transmesenteric postoperative hernia, 1:1-40 to 1:1-
42,I:I-43i
traumatic abdominal wall hernia, 1:1-44 to 1:1-45
traumatic diaphragmatic rupture, I:1-48 to I:I-SO,
I:l-Sli
ventral hernia, 1:1-30 to 1:1-31
Peritonitis, 1:1-10 to 1:1-12, I:I-13i
differential diagnosis, I:l-lOi, I:1-11
pseudomyxoma peritonei vs., I:I-66i, 1:1-67
tubercular
opportunistic infections vs., 1:4-I4i, 1:4-15
peritoneal metastases vs., I:I-62i, 1:1-63
pseudomyxoma peritonei vs., 1:1-66i, 1:1-67
Peutz-]eghers syndrome, I:4-S6i, 1:4-56 to 1:4-57
Pheochromocytoma, III:2-26 to III:2-28, 1II:2-29i
adrenal adenoma vs., III:2-20i, III:2-22
adrenal carcinoma vs., III:2-31
adrenal hyperplasia vs., III:2-14
adrenal metastasis vs., III:2-34i, III:2-36
adrenal myelolipoma vs., III:2-25
adrenal tuberculosis vs., III:2-6
differential diagnosis, 1II:2-26i, III:2-27 to III:2-28
Phleboliths
ureteral duplication vs., III:4-2i, III:4-3
urolithiasis vs., III:3-48i, III:3-49 to III:3-50
Phrenic ampulla, I:2-S2i, 1:2-53
PID (pelvic inflammatory disease), I:S-22i, 1:5-23
Pleural effusion, 1:1-49
Plication defect, 1:3-62
Pneumatosis
drug-induced, I:4-34i, 1:4-35
familial polyposis vs., I:S-S6i, 1:5-57
intestinal, I:4-34i, 1:4-34 to 1:4-36, I:4-37i
postoperative, 1:4-35
Pneumocystis carinii
HlV nephropathy vs., 1II:3-42i, III:3-43
nephrocalcinosis vs., III:3-S2i, III:3-53
splenic metastasis vs., 1:6-32
Polyarteritis nodosa
intestinal trauma vs., I:4-46i, 1:4-48
pyelonephritis vs., III:3-28i, III:3-30
renal artery stenosis vs., III:3-64i, III:3-65 to III:3-66
renal infarction vs., III:3-68i, III:3-69
renal trauma vs., III:3-76i, III:3-77
Polycystic kidney disease, autosomal dominant, III:3-16
to III:3-18, 1II:3-19i
acquired cystic disease of uremia vs., III:3-S6i, III:3-57
differential diagnosis, III:3-16i, I1I:3-17 to III:3-18
von Hippel Lindau disease vs., III:3-20i, I1I:3-22
Polycystic liver disease, autosomal dominant, 11:1-12 to
1I:1-14,II:I-ISi
biliary hamartoma vs., II:I-114i, 11:1-115
Caroli disease vs., II:2-6i, 11:2-7
congenital hepatic fibrosis vs., I1:I-8i, 11:1-9
differential diagnosis, I1:1-I2i, 11:1-13
Polyposis
familial, 1:5-56 to 1:5-58, I:S-S9i
differential diagnosis, I:S-S6i, 1:5-57 to 1:5-58
hamartomatous polyposis vs., 1:4-57
hamartomatous, I:4-S6i, 1:4-56 to 1:4-57
Polyps. See also Pseudopolyps
adenomatous, II:2-32i, 11:2-33
colonic, I:S-4Oi, 1:5-40 to 1:5-42, I:S-43i
duodenum, 1:3-36 to 1:3-27
 INDEX
fibrovascular, 1:2-80i, 1:2-80 to 1:2-81,1:2-84
gastric, 1:3-32i, 1:3-32 to 1:3-34, 1:3-35i
Polysplenia, 1:6-6 to 1:6-8, 1:6-9i
accessory spleen vs., 1:6-10i, 1:6-11
differential diagnosis, 1:6-6i, 1:6-8
Porcelain gallbladder, 1I:2-38i, 11:2-38 to 11:2-39
Porta hepatis tumor
cholangiocarcinoma vs., 11:2-52
Mirizzi syndrome vs., 1I:2-30i
Postoperative changes
aorto-enteric fistula, 1:3-72i
gastric volvus vs., 1:3-72i, 1:3-73
pelvic lipomatosis vs., III:1-15
Postoperative complications
aorto-enteric fistula vs., 1:3-72i
Boerhaave syndrome vs., 1:2-74i, 1:2-75
fundoplication, 1:3-60 to 1:3-62, 1:3-63i
gastric bypass surgery, 1:3-64 to 1:3-66, 1:3-67i
intestinal pneumatosis, 1:4-35
Pregnancy complications, acute fatty liver, 11:1-73
Proctitis, 11I:1-14i
Progressive systemic sclerosis, 1:4-30i, 1:4-30 to 1:4-32,
1:4-33i
Prostate
abscess, 11I:6-20i, III:6-21
benign hypertrophy. See Benign prostatic
hypertrophy
calcification, III:5-10
cysts
ectopic ureter vs., 11I:4-4i
prostatitis vs., 1I1:6-lOi, III:6-11
Prostate carcinoma, III:6-30 to III:6-32, 1I1:6-33i
benign prostatic hypertrophy vs., 11I:6-20i, III:6-21
differential diagnosis, II1:6-30i, III:6-31
neurogenic bladder vs., 11I:5-16i, III:5-17
prostatitis vs., II1:6-10i, III:6-11
Prostatitis, II1:6-10i, III:6-10 to III:6-11
Pseudocysts
mesenteric cyst vs., 1:1-53
pancreatic, 11:3-24to 11:3-26, 1I:3-27i
abdominal abscess vs., 1:1-6i, 1:1-7
choledochal cyst vs., 1I:2-10i, 11:2-11
desmoid tumorvs., 1:1-55
differential diagnosis, 1I:3-24i, 11:3-25
duodenal diverticulum vs., 1:3-8i
intraductal papillary mucinous tumor vs., 11:3-
46i,II:3-47
intramural benign gastric tumors vs., 1:3-38i, 1:3-39
mesenteric cyst vs., 1:1-52i, 1:1-53
mucinous cystic pancreatic tumor vs., 1I:3-42i,
11:3-43
pancreatic cyst vs., 1I:3-40i
solid and papillary pancreatic neoplasms vs., 11:3-59
serous cystadenoma vs., II:3-36i, 11:3-37
splenic cyst vs., 1:6-22i, 1:6-24
Pseudodiverticulosis, intramural, 1:2-66i, 1:2-66 to 1:2-67
Pseudomyxoma peritonei, 1:1-66 to 1:1-68, 1:1-69i
ascites in, 1:1-18i, 1:1-20
differential diagnosis, 1:1-66i, 1:1-67
mesenteric cyst vs., 1:1-52i
mesothelioma vs., 1:1-58i, 1:1-59
peritoneal metastases vs., 1:1-62i, 1:1-63
peritonitis vs., 1:1-10i, 1:1-11
Pseudoobstruction, intestinal, 1:4-72i, 1:4-73
Pseudopneumatosis, 1:4-34i, 1:4-35
Pseudopolyps
duodenal, 1:3-27
familial polyposis vs., 1:5-56i, 1:5-58
Gardner syndrome vs., 1:5-60i, 1:5-61
Pseudoureterocele, 1I1:4-8i, III:4-9
Pulmonary diseases, 1:4-35
Pulmonary embolism, 1:3-66
Pulsion diverticulum, 1:2-62 to 1:2-64, 1:2-65i
Boerhaave syndrome vs., 1:2-74i, 1:2-75
differential diagnosis, 1:2-62i, 1:2-63 to 1:2-64
esophageal perforation vs., 1:2-70i, 1:2-71
gastric volvus vs., 1:3-72i, 1:3-73
hiatal hernia vs., 1:2-52i, 1:2-53
traction diverticulum vs., 1:2-6Oi
Zenker diverticulum vs., 1:2-56i
Pyelitis, emphysematous, III:3-40
Pyelonephritis, III:3-28 to III:3-30, II1:3-3li
differential diagnosis, II1:3-28i, III:3-29 to III:3-30
emphysematous, II1:3-40i, III:3-40 to III:3-41
renal cell carcinoma vs., 1I1:3-96i, III:3-98
renal infarction vs., 1I1:3-68i, III:3-69
renal medullary carcinoma vs., III:3-101
renal metastases and lymphoma vs., III:3-107
renal vein thrombosis vs., 1I1:3-72i, III:3-73
xanthogranulomatous, 1I1:3-36i, III:3-36 to III:3-38,
II1:3-39i
Pyocele, testicular, 1I1:6-14i
Pyogenic abscess
cystadenocarcinoma vs., 1I:1-136i, 11:1-137
hepatic. See Hepatic access, pyogenic
hepatic cyst vs., 1I:1-98i, 11:1-100
hepatic metastases and lymphoma vs., 1I:1-144i,
11:1-146
splenic cyst vs., 1:6-23
splenic metastasis vs., 1:6-30i, 1:6-31
splenomegaly vs., 1:6-36
R
Radiation esophagitis, 1:2-30 to 1:2-31
Barrett esophagus vs., 1:2-21
caustic esophagitis vs., 1:2-25
differential diagnosis, 1:2-30i, 1:2-31
viral esophagitis vs., 1:2-13
Rectum
carcinoma, 1:5-48 to 1:5-50, I:S-Sli
differential diagnosis, I:S-48i, 1:5-49
prostate carcinoma vs., 11I:6-30i, III:6-31
villous adenoma vs., I:S-52i, 1:5-53 to 1:5-54
trauma, 1:5-49
ulcer, I:S-48i, 1:5-49
Rectus sheath hematoma
spigelian hernia vs., 1:1-32i, 1:1-33
traumatic abdominal wall hernia vs., I:1-44i, 1:1-45
Reflux esophagitis, 1:2-16 to 1:2-18, 1:2-19i
Barrett esophagus vs., 1:2-20i, 1:2-21
Candida esophagitis vs., 1:2-8i, 1:2-9
carcinoma vs., 1:2-83
caustic esophagitis vs., 1:2-24i, 1:2-25
 INDEX
differential diagnosis, I:2-I6i, 1:2-17 to 1:2-18
drug-induced esophagitis vs., I:2-2Si, 1:2-29
esophageal varices vs., I:2-46i
intramural pseudodiverticulosis vs., I:2-66i, 1:2-67
radiation esophagitis vs., I:2-30i, 1:2-31
viral esophagitis vs., I:2-I2i, 1:2-13
Reflux into stomach, I:3-64i, 1:3-66
Reflux ulcer. See Reflux esophagitis
Renal abscess, II1:3-32 to II1:3-34, III:3-35i
cyst vs., II1:3-81
differential diagnosis, III:3-32i, II1:3-33
metastases and lymphoma vs., II1:3-107
renal cell carcinoma vs., II1:3-98
xanthogranulomatous pyelonephritis vs., II1:3-37
Renal artery
calcification
transitional cell carcinoma vs., II1:3-102i, III:3-103
urolithiasis vs., III:3-4Si, II1:3-49
dissection, traumatic, III:3-64i, II1:3-65
stenosis, II1:3-64 to II1:3-66, III:3-67i
differential diagnosis, II1:3-64i, III:3-65 to III:3-66
transitional cell carcinoma vs., III:3-102i, III:3-103
Renal cell carcinoma, II1:3-96 to II1:3-98, III:3-99i. See
also Angiomyolipoma, renal; Oncocytoma
abscess vs., III:3-32i, II1:3-33
acquired cystic disease of uremia vs., III:3-56i
adrenal carcinoma vs., II1:2-31
angiomyolipoma vs., III:3-SSi, II1:3-89
column of Bertin vs., III:3-I4i, II1:3-15
cyst vs., III:3-SOi, II1:3-81
differential diagnosis, III:3-96i, II1:3-97 to II1:3-98
metastases and lymphoma vs., III:3-106i, II1:3-107
multilocular cystic nephroma vs., III:3-92i, II1:3-93
oncocytoma vs., III:3-84i, II1:3-85
renal medullary carcinoma vs., III:3-10Oi
renal vein thrombosis vs., III:3-72i, II1:3-73
transitional cell carcinoma vs., III:3-102i, II1:3-104
trauma vs., III:3-76i, II1:3-77
xanthogranulomatous pyelonephritis vs., III:3-36i,
II1:3-37
Renal cyst, II1:3-80 to II1:3-82, III:3-83i
abscess vs., III:3-32i, II1:3-33
acquired cystic disease of uremia vs., II1:3-56i, III:3-57
autosomal dominant polycystic kidney disease vs.,
III:3-17
differential diagnosis, III:3-80i, II1:3-81 to II1:3-82
multilocular cystic nephroma vs., II1:3-93
nephrocalcinosis vs., III:3-52i, II1:3-53
renal cell carcinoma vs., II1:3-98
yon Hippel Lindau disease vs., III:3-20i, II1:3-22
Renal ectopia, II1:3-6 to II1:3-8, III:3-9i
differential diagnosis, III:3-6i, III:3-8
horseshoe kidney vs., III:3-10i, II1:3-11
Renal failure, III:3-52i
Renal infarction, II1:3-68 to II1:3-70, III:3-7li
differential diagnosis, III:3-68i, II1:3-69 to II1:3-70
emphysematous pyelonephritis vs., III:3-40i, III:3-41
pyelonephritis vs., III:3-28i, II1:3-29
Renal medullary carcinoma, III:3-100i, II1:3-100 to
III:3-101
Renal metastases and lymphoma, II1:3-106 to III:3-108,
III:3-109i
abscess vs., III:3-32i, II1:3-33
angiomyolipoma vs., III:3-88i, II1:3-89
column of Bertin vs., II1:3-15
cyst vs., III:3-SOi, III:3-81, II1:3-82
differential diagnosis, III:3-106i, II1:3-107
oncocytoma vs., III:3-84i, II1:3-86
pyelonephritis vs., III:3-28i, III:3-30
renal artery stenosis vs., III:3-64i
renal cell carcinoma vs., III:3-96i, II1:3-97, II1:3-97
to II1:3-98
renal medullary carcinoma vs., III:3-100i, II1:3-101
xanthogranulomatous pyelonephritis vs., III:3-36i,
II1:3-37
Renal papillary necrosis, II1:3-46 to II1:3-47
differential diagnosis, III:3-46i, II1:3-47
medullary sponge kidney vs., II1:3-61
nephrocalcinosis vS., II1:3-53
transitional cell carcinoma vs., II1:3-104
Renal pelvis, blood clot, III:3-24i, II1:3-26
Renal scarring, II1:3-15
Renal trauma, II1:3-76 to II1:3-78, III:3-79i
differential diagnosis, III:3-76i, II1:3-77
pyelonephritis vs., III:3-28i, II1:3-30
renal infarction vs., III:3-6Si, II1:3-70
Renal tubular acidosis, II1:3-61
Renal tubular necrosis, acute, III:3-42i
Renal vein thrombosis, III:3-72i, II1:3-72 to II1:3-74,
III:3-75i
Rete testis, III:6-I6i
Retroperitoneal fibrosis, II1:1-10 to 111:1-12,III:I-13i
aorto-enteric fistula vs., I:3-76i
differential. diagnosis, III:I-IOi, 111:1-11to II1:1-12
hemorrhage vs., III:I-16i
lymphoma vs., III:1-25
metastasis vs., III:1-29
renal artery stenosis vs., II1:3-66
Retroperitoneal hemorrhage, III:I-16 to III:1-18, II1:I-I9i
differential diagnosis, III:I-16i, II1:1-17
fibrosis vs., III:I-IOi, II1:1-12
metastasis vs., III:I-28i, II1:1-29
retroperitoneal sarcoma vs., III: I-20i, III:1-22
Retroperitoneal mass
renal artery stenosis vs., II1:3-66
ureteropelvic junction obstruction vs., II1:3-26
Retroperitoneum, III:1-6 to III:1-33
asymmetric muscles vs. hemorrhage, II1:1-17
fibrosis, III:1-10 to III:1-12, III:I-13i
hemorrhage, II1:1-16 to III:1-18, III:I-19i
inferior vena cava, duplications and anomalies, II1:1-
6 to III:1-8, I1I:1-9i
lymphocele, III:1-32 to III:1-33
lymphoma, III:1-24 to III:1-26, I1I:1-27i
differential diagnosis, III:I-24i, II1:1-25
fibrosis vs., III:I-lOi, II1:1-11
metastasis vs., III:I-28i, III:I-29
metastases, III:1-28 to III:1-30, III:I-3li
pelvic lipomatosis, 111:1-14to II1:1-15
primary tumors, III:1-29
sarcoma, III:1-20 to 111:1-22,III:I-23i
Rhabdomyosarcoma, III:4-Si, II1:4-9
 INDEX
s volvulus, 1:5-62 to 1:5-64, 1:5-65i
Sarcoidosis
hepatic, 11:1-56to 11:1-58, II:I-59i
differential diagnosis, II: 1-56i, II:1-5 7
liver cirrhosis vs., II:I-40i, 11:1-42
primary biliary cirrhosis vs., 11:1-49
medullary sponge kidney vs., III:3-61
retroperitoneal metastasis vs., III:1-29
splenic
infection and abscess vs., 1:6-12i, 1:6-13
metastasis vs., 1:6-30i, 1:6-31
tumor vs., 1:6-26i, 1:6-28
splenomegaly vs., 1:6-36
Sarcoma. See also Angiosarcoma; Leiomyosarcoma;
Liposarcoma
gastric stromal tumor vs., 1:3-42i, 1:3-43
Kaposi's
gastric ulcer vs., 1:3-15
retroperitoneal lymphoma vs., III:I-25
retroperitoneal, III:1-20 to III:1-22, 1II:1-23i
differential diagnosis, III:I-20i, III:I-21 to III:1-22
hemorrhage in, 1II:1-16i, III:I-17
lymphoma vs., 1II:1-24i, III:I-25
Sarcomatosis, peritoneal, 1:1-67
Schatzki ring, 1:2-50 to 1:2-51
differential diagnosis, 1:2-50i, 1:2-50 to 1:2-51
esophageal webs vs., 1:2-6i
Schwachman-Diamond syndrome, II:3-18i, 11:3-19
Sciatic hernia, 1:1-34
Scleroderma
esophageal, 1:2-42 to 1:2-44, 1:2-45i
Barrett esophagus vs., 1:2-20i, 1:2-22
differential diagnosis, 1:2-42i, 1:2-43
esophageal achalasia vs., 1:2-34i, 1:2-35
iatrogenic, 1:2-43
reflux esophagitis vs., 1:2-17 to 1:2-18
intestinal, 1:4-30i, 1:4-30 to 1:4-32, 1:4-33i
renal, III:3-30, III:3-77
Sclerosis, progressive systemic. See Scleroderma,
intestinal
Scrotum
hernia, III:6-14
trauma, III:6-22 to III:6-23
differential diagnosis, III:6-22i, III:6-23
testicular torsion vs., III:6-18i
Seminal vesicle cyst, III:4-4i, III:4-5
Seminiferous tubular ectasia, III:6-16
Seroma, 1:3-38i, 1:3-39
Shock bowel
intestinal trauma and, 1:4-46i, 1:4-47
ischemic enteritis vs., 1:4-38i, 1:4-39
mesenteric trauma vs., 1:1-46i, 1:1-47
vasculitis vs., 1:4-42i, 1:4-43
Shock pancreas
acute pancreatitis vs., II:3-20i, 11:3-21
traumatic pancreatitis vs., II:3-32i, 11:3-33
Sickle cell disease
hepatitis vs .., II:1-16i, 11:1-17
primary biliary cirrhosis vs., II:1-48i, 11:1-49
Sigmoid colon
cancer vs. diverticulitis, 1:5-28i, 1:5-29 to 1:5-30
cecal volvulus vs., 1:5-66i
differential diagnosis, 1:5-62i, 1:5-63
Small bowel carcinoma, 1:4-58 to 1:4-59
carcinoid tumor vs., 1:4-61
differential diagnosis, 1:4-58i, 1:4-59
intramural benign intestinal tumors vs., 1:4-54i, 1:4-55
Small bowel obstruction, 1:4-68 to 1:4-70, 1:4-71i
differential diagnosis, 1:4-68i, 1:4-70
para duodenal hernia vs., 1:1-36i, 1:1-37
transmesenteric post-operative hernia vs., 1:1-40i,
1:1-41
vasculitis vs., 1:4-42i, 1:4-43
Small intestine, 1:4-6 to 1:4-85
carcinoid tumor, 1:4-60 to 1:4-62, 1:4-63i
carcinoma, 1:4-58 to 1:4-59
Crohn disease, 1:4-26 to 1:4-28, 1:4-29i
gallstone ileus, 1:4-72 to 1:4-73
gastrointestinal bleeding, 1:4-50 to 1:4-52, 1:4-53i
hamartomatous polyposis, 1:4-56 to 1:4-57
intramural benign intestinal tumors, 1:4-54 to 1:4-55
intussusception, 1:4-78 to 1:4-80, 1:4-8li
ischemic enteritis, 1:4-38 to 1:4-40, 1:4-4li
malabsorption conditions, 1:4-74 to 1:4-76, 1:4-77i
malrotation, 1:4-6 to 1:4-7
mastocytosis, 1:4-24 to 1:4-25
Meckel diverticulum, 1:4-8 to 1:4-10, 1:4-11i
metastases and lymphoma, 1:4-64 to 1:4-66, 1:4-67i
obstruction, 1:4-68 to 1:4-70, 1:4-71i
opportunistic infections, 1:4-14 to 1:4-16, 1:4-17i
parasitic disease, 1:4-12 to 1:4-13
pneumatosis, 1:4-34 to 1:4-36, 1:4-37i
radiation enteritis, 1:4-82 to 1:4-84, 1:4-85i
scleroderma, 1:4-30 to 1:4-32, 1:4-33i
sprue-celiac disease, 1:4-18 to 1:4-20, 1:4-2li
trauma, 1:4-46 to 1:4-48, 1:4-49i
vasculitis, 1:4-42 to 1:4-44, 1:4-45i
Whipple disease, 1:4-22 to 1:4-23
Solid and papillary neoplasms, 11:3-58 to 11:3-59
Spermatocele, III:6-14i
Spigelian hernia, 1:1-32 to 1:1-33
differential diagnosis, 1:1-32i, 1:1-33
traumatic abdominal wall hernia vs., 1:1-44i
ventral hernia vs., 1:1-3Oi
Spleen, 1:6-6 to 1:6-37
accessory spleen, 1:6-10 to 1:6-11
asplenia and polysplenia, 1:6-6 to 1:6-8, 1:6-9i
cyst, 1:6-22 to 1:6-24, 1:6-2Si
infarction, 1:6-16 to 1:6-18, 1:6-19i
infection and abscess, 1:6-12 to 1:6-14, 1:6-15i
metastases and lymphoma, 1:6-30 to 1:6-32, 1:6-33i
splenomegaly and hypersplenism, 1:6-34 to 1:6-36,
1:6-37i
trauma, 1:6-20 to 1:6-21
tumors, 1:6-26 to 1:6-28, 1:6-29i
Splenectomy, 1:6-8
Splenic cyst, 1:6-22 to 1:6-24, 1:6-2Si
differential diagnosis, 1:6-22i, 1:6-23 to 1:6-24
infarction vs., 1:6-16i, 1:6-17
splenic infarction vs., 1:6-16i, 1:6-17
trauma vs., 1:6-2Oi
tumors vs., 1:6-26i, 1:6-28
 INDEX
Splenic infarction, 1:6-16 to 1:6-18, I:6-19i
differential diagnosis, I:6-16i, 1:6-17
infection and abscess vs., I:6-12i, 1:6-13
metastasis vs., I:6-30i, 1:6-31
trauma vs., I:6-2Oi
Splenic infection and abscess, 1:6-12 to 1:6-14, I:6-15i
cyst vs., 1:6-23 .
differential diagnosis, .I:6-12i, 1:6-13
splenic infarction vs., I:6-16i, 1:6-17
splenomegaly vs., 1:6-36
trauma vs., I:6-20i
tumors vs., 1:6-28
Splenic metastases and lymphoma, 1:6-30 to 1:6-32, I:6-33i
accessory spleen vs., I:6-10i, 1:6-11
cyst vs., I:6-22i, 1:6-24
differential diagnosis, 1:6-30i, 1:6-31 to 1:6-32
splenic cyst vs., I:6-22i, 1:6-24
splenic infarction vs., I:6-16i, 1:6-17
splenomegaly vs., I:6-34i, 1:6-35 to 1:6-36
trauma vs., I:6-20i, 1:6-21
Splenic trauma, 1:6-20 to 1:6-21
differential diagnosis, 1:6-20i, 1:6-21
fundoplication and, I:3-60i, 1:3-62
infection and abscess vs., I:6-12i, 1:6-13
laceration vs. infarction, I:6-16i, 1:6-17
Splenic tumors, 1:6-26 to 1:6-28, I:6-29i
differential diagnosis, I:6-26i, 1:6-28
gastric diverticulum vs., I:3-6i, 1:3-7
infection and abscess vs., I:6-12i, 1:6-13
metastasis vs., I:6-30i, 1:6-31
splenic infarction vs., I:6-16i, 1:6-17
splenomegaly vs., 1:6-36
Splenomegaly and hypersplenism, 1:6-34 to 1:6-36, I:6-37i
differential diagnosis, I:6-34i, 1:6-35 to 1:6-36
splenic metastasis vs., 1:6-32
Splenosis
accessory spleen vs., I:6-1Oi
gastric diverticulum vs., I:3-6i, 1:3-7
intramural benign gastric tumors vs., I:3-38i, 1:3-39
polysplenia vs., I:6-6i, 1:6-8
Sprue-celiac disease, 1:4-18 to 1:4-20, I:4-2li
differential diagnosis, I:4-18i, 1:4-19 to 1:4-20
malabsorption conditions vs., I:4-74i, 1:4-75
mastocytosis vs., I:4-24i, 1:4-25
scleroderma vs., I:4-30i, 1:4-31
Squamous papillomatosis. See Viral esophagitis
Steatohepatitis, 1I:1-36i, 11:1-37
Steatosis, 11:1-36to 11:1-38, 1I:1-39i
differential diagnosis, 1I:1-36i, 11:1-37
hepatic angiomyolipoma vs., 1I:1-118i, 11:1-119
hepatic infarction vs., 1I:1-76i, 11:1-77
hepatitis vs., 1I:1-16i, 11:1-17
metastases and lymphoma vs., 11:1-146
passive hepatic congestion vs., 1I:1-68i, 11:1-69
ill pregnancy, HELLPsyndrome vs., 11:1-73
radiation hepatitis vs., 1I:1-148i, 11:1-149
Wilson disease vs., 1I:1-88i, 11:1-89
Stipple sign, III:5-22i, III:5-23
Stomach. See 'Gastric' terms
Substance abuse, renal trauma vs., 111:3-77
Superficial spreading carcinoma, I:2-8i, 1:2-9
Superior mesenteric root syndrome, I:4-30i, 1:4-31
Systemic lupus erythematosus
intestinal trauma vs., 1:4-48
metastases and lymphoma vs., 1:4-66
pyelonephritis vs., III:3-30
renal trauma vs.,III:3-77
T
Takayasu arteritis, III:3-65 to III:3-66
Telangiectasia, hereditary hemorrhagic, 1I:1-60i, 11:1-60
to 11:1-62, 1I:1-63i
Teratoma
hepatic angiomyolipoma vs., II: 1-118i, 11:1-119
mesenteric cyst vs., 1:1-52i, 1:1-53
retroperitoneal lymphoma vs., III:I-25
Testicular carcinoma, III:6-26 to III:6-28, III:6-29i
differential diagnosis, 1I1:6-26i, III:6-27
epididymitis vs., III:6-8i
scrotal trauma vs., III:6-22i
testicular torsion vs., 11I:6-18i
varicocele vs., III:6-16i
Testicular infarct, segmental
epididymitis vs., III:6-8i, III:6-9
scrotal trauma vs., 1I1:6-22i, III:6-23
testicular carcinoma vs., 1I1:6-26i, III:6-27
Testicular torsion, III:6-18 to III:6-19
differential diagnosis, III:6-18i
epididymitis vs., III:6-8i
scrotal trauma vs., 1I1:6-22i, III:6-23
varicocele vs., III:6-16i
Testicular trauma. See Scrotum, trauma
Thermal injuries, gastric, I:3-28i, 1:3-29
Toxic megacolon. See Megacolon, toxic
Trabeculation
bladder carcinoma vs., III:5-23
cystitis vs., III:5-8i
Traction diverticulum, 1:2-60 to 1:2-61
differential diagnosis, I:2-60i, 1:2-60 to 1:2-61
pulsion diverticulum vs., I:2-62i, 1:2-63
Transitional cell carcinoma, III:3-102 to III:3-104
III:3-105i '
differential diagnosis, III:3-102i, I1I:3-103 to I1I:3-104
renal cell carcinoma vs., III:3-96i, III:3-97
renal medullary carcinoma vs., III:3-10Oi
renal metastases and lymphoma vs., III:3-106i
III:3-107 '
renal vein thrombosis vs., III:3-72i, III:3-73
ureteropelvic junction obstruction vs., I11:3-24i
III:3-26 '
uteritis cystica vs., III:4-1Oi
xanthogranulomatous pyelonephritis vs. I1I:3-36i
III:3-37 "
Transmesenteric hernia
paraduodenal hernia vs., I:1-36i, 1:1-37
postoperative, I:I-40i, 1:1-40 to 1:1-42, I:1-43i
Transplant, renal, ectopia vs., 1I1:3-6i, III:3-8
Tuberculosis
adrenal, III:2-6 to III:2-7
differential diagnosis, III:2-6i, III:2-7
hemorrhage in, III:2-10
colon carcinoma vs., I:5-44i, 1:5-45
Crohn disease vs., I:4-26i, 1:4-27
 INDEX
duodenal carcinoma vs., 1:3-55
mesothelioma vs., l:l-S8i, 1:1-59
peritonitis in
opportunistic infections vs., 1:4-14i, 1:4-15
peritoneal metastases vs., 1:1-62i, 1:1-63
pseudomyxoma peritonei vs., 1:1-66i, 1:1-67
renal
medullary sponge kidney vs., III:3-60i, III:3-61
nephrocalcinosis vs., 11I:3-S2i, III:3-53
transitional cell carcinoma vs., 1I1:3-102i, III:3-103
retroperitoneal
lymphoma vs., III:1-25
metastasis vs., 1I1:1-28i, III:I-29
splenic metastasis vs., 1:6-32
splenomegaly vs., 1:6-36
sprue-celiac disease vs., 1:4-19
Tuberous sclerosis
acquired cystic disease of uremia vs., 11I:3-S6i, III:3-57
autosomal dominant polycystic kidney disease vs.,
III:3-16i, III:3-18
Tubular necrosis, acute
glomerulonephritis vs., III:3-44i
HIV nephropathy vs., III:3-42i
Typhlitis, 1:5-14 to 1:5-15
differential diagnosis, 1:5-14i, I:S-14i, 1:5-15
pseudomembranous colitis vs., 1:5-12
ulcerative colitis vs., I:S-16i, 1:5-18
u Villous adenoma, 1:5-52 to 1:5-54, 1:5-55i
Ulcer. See specific site or type
Umbilical hernia
spigelian hernia vs., 1:1-32i
ventral hernia vs., 1:1-30i, 1:1-31
Urachal remnant, III:S-6i, III:6 to III:5-7
Uremia, acquired cystic disease of. See Acquired cystic
disease of uremia
Ureter, III:4-2 to III:4-11
bifid, III:4-2i, III:4-3
duplication, III:4-2 to III:4-3
ectopic, III:4-4 to III:4-6, III:4-7i
obstruction vs. renal vein thrombosis, 1I1:3-72i,
111:3-73
retrocaval, III:3-26
ureterocele, III:4-8 to III:4-9
uteritis cystica, III:4-10 to III:4-11
Ureteropelvic junction obstruction, III:3-24i, III:3-24 to
III:3-26, III:3-27i
Urethra
stricture, 1I1:6-12i, III:6-12 to III:6-13
trauma
bladder trauma vs., III:5-19
stricture vs., 1I1:6-12i
tumor, 11I:6-12i, III:6-13
Urinoma, III:I-32i, II1:1-33
Urolithiasis, III:3-48 to III:3-50, 11I:3-Sli
differential diagnosis, III:3-48i, III:3-49 to III:3-50
ureteropelvic junction obstruction vs., 11I:3-24i,
III:3-25
Uropathy, obstructive, 1I1:3-42i, III:3-43
Uteritis cystica, III:4-10i, III:4-l0 to III:4-11
Utricle, III:4-8 to III:4-9
bladder diverticulum vS., III:S-12i, III:5-13
differential diagnosis, III:4-8i, III:4-9
ureteral duplication vs., 1I1:4-2i, III:4-3
V
Vagotomy, post-surgical changes
esophageal achalasia vs., 1:2-34i, 1:2-36
scleroderma vs., 1:2-42i, 1:2-43
Varicella-zoster virus esophagitis. See Viral esophagitis
Varicocele, III:6-16 to III:6-17
cryptorchidism vs., III:6-6i
differential diagnosis, III:6-16i
Vascular infarction, 1:6-23
Vasculitis
renal
infarction vs., 1I1:3-68i, III:3-69
pyelonephritis vs., III:3-28i, III:3-30
trauma vs., 1I1:3-76i, III:3-77
small intestine, 1:4-42 to 1:4-44, 1:4-4Si
differential diagnosis, 1:4-42i, 1:4-43
intestinal trauma and, 1:4-46i, 1:4-48
metastases and lymphoma vs., 1:4-64i, 1:4-66
Vena cava, inferior, duplications and anomalies, 111:1-
6i, III:1-6 to III:1-7
Ventral hernia, 1:1-30 to 1:1-31
differential diagnosis, 1:1-30i, 1:1-31
spigelian hernia vs., 1:1-32i
traumatic abdominal wall hernia vs., 1:1-44i, 1:1-45
differential diagnosis, 1:5-52i, 1:5-53 to 1:5-54
duodenal hematoma vs., 1:3-30i, 1:3-31
duodenal metastases and lymphoma vs., 1:3-58i
rectal carcinoma vS., 1:5-49
Viral esophagitis, 1:2-12 to 1:2-14, 1:2-15i
Barrett esophagus vs., 1:2-21
Candida esophagitis vs., 1:2-8i, 1:2-9
caustic esophagitis vs., 1:2-25
cytomegalovirus vs. reflux esophagitis, 1:2-17
differential diagnosis, 1:2-12i, 1:2-13
herpes esophagitis
Candida esophagitis vs., 1:2-8i, 1:2-9
drug-induced esophagitis vs., 1:2-28i, 1:2-29
reflux esophagitis vs., 1:2-16i, 1:2-17
HIV esophagitis, 1:2-17
technical artifacts vs., 1:2-13
Visceral mass, 1:6-11
Vitelline cyst, II1:5-7
Volvulus
cecal, 1:5-66 to 1:5-67
sigmoid, 1:5-62 to 1:5-64, 1:5-65i
differential diagnosis, 1:5-62i, 1:5-63
toxic megacolon vs., 1:5-20i, 1:5-21
small bowel malrotation vs., 1:4-6i, 1:4-7
trans mesenteric post-operative hernia vs., 1:1-40i,
1:1-41
von Hippel Lindau disease, III:3-20 to III:3-22, I11:3-23i
acquired cystic disease of uremia vs., 1I1:3-56i, III:3-57
autosomal dominant polycystic kidney disease vs.,
II1:3-16i, III:3-18
differential diagnosis, III:3-20i, III:3-22
pancreatic pseudocyst vs., 1I:3-24i, 11:3-25
 INDEX
Von Meyenburg complexes. See Biliary hamartoma

w
Waldenstrom macroglobulinemia
sprue-celiac disease vs., I:4-18i, 1:4-20
Whipple disease vs., I:4-22i, 1:4-23
Whipple disease, 1:4-22 to 1:4-23
differential diagnosis, 1:4-22i, 1:4-23
malabsorption conditions vs., I:4-74i, 1:4-75
metastases and lymphoma vs., I:4-64i, 1:4-66
opportunistic infections vs., 1:4-14i, 1:4-15
sprue-celiac disease vs., I:4-18i, 1:4-19
Wilms tumor
multilocular cystic nephroma vs., III:3-93
renal angiomyolipoma vs., III:3-89
Wilson disease, 1I:1-88i, 11:1-88 to 11:1-89

y
Yersinia infections, I:4-26i, 1:4-27

z
Zenker diverticulum, I:2-56i, 1:2-56 to 1:2-58, I:2-59i
Zollinger-Ellison syndrome, 1:3-22 to 1:3-24, I:3-25i
differential diagnosis, I:3-22i, 1:3-23 to 1:3-24
duodenal carcinoma vs., h3-55
gastritis vs., I:3-10i, 1:3-11
malabsorption conditions vs., I:4-74i, 1:4-76
mastocytosis vs., I:4-24i, 1:4-25