AN

UNCOMMON CAUSE OF PORTAL

HYPERTENSION
Resident(s): Bryan I. Hartley, MD
Attending(s): Leann S. Stokes, MD
Program/Dept(s): Vanderbilt University Medical Center

CHIEF COMPLAINT & HPI

Chief Complaint
My stomach hurts.

History of Present Illness

A 55-year-old man presented with complaints of abdominal swelling, discomfort and
associated shortness of breath.

RELEVANT HISTORY
Past Medical History

Gastroesophageal reflux
Denies history of liver disease, liver biopsy or trauma, retrograde or transhepatic
cholangiography or hepatobiliary operation

Past Surgical History

Splenectomy

Medications

Aspirin 81 mg and Esomeprazole

Allergies
NKDA

DIAGNOSTIC WORKUP CT ANGIOGRAM

A

Figure A: There was marked hypertrophy of the celiac, common

hepatic, proper hepatic and right hepatic arteries. The right
hepatic artery branch directly communicates with a branch of the
right portal vein. Note atrophy of the right hepatic lobe.
Figure B: Reformatted image from CT angiogram shows
opacification of the portal vein (arrows) on arterial phase imaging.

DIAGNOSIS
Congenital high flow arteriovenous fistula between a peripheral branch of
the right hepatic artery and a subcapsular branch of the right portal vein.

QUESTION
True or false: Most congenital arterioportal fistulas are commonly diagnosed in
adulthood.

A. True
B. False

CORRECT!
True or false: Most congenital arterioportal fistulas are commonly diagnosed in
adulthood.

A. True
B. False

CONTINUE WITH CASE

SORRY, THATS INCORRECT.

True or false: Most congenital arterioportal fistulas are commonly diagnosed in
adulthood.

A. True
B. False

CONTINUE WITH CASE

INTERVENTION

A 5-F Cobra II catheter (Angiodynamics, Latham NY) was used to select the
hypertrophied right hepatic artery.

INTERVENTION

The Cobra II catheter was exchanged over a wire for a 5-F

vertebral catheter (Angiodynamics, Latham, NY).
A 10 mm x 14 cm Nester coil (Cook Medical, Bloomington,
Indiana) was deployed proximal to the tapered portion of
the distal hepatic arterial branch.
The coil (circle) crossed the fistula and embolized into a
right portal vein branch. Subsequent injections
demonstrated no disruption of flow in the main or left
portal systems.
A decision was made to proceed with Amplatzer II plug
(St. Jude Medical, St. Paul, MN) placement.
The vertebral catheter was replaced with a 6-F MDC
guiding catheter (Boston Scientific, Natick, MA).
A 12 mm Amplatzer II plug (arrow) was deployed in the
right hepatic arterial branch through the guiding catheter.
Final injection of contrast demonstrated occlusion of the
AV fistula.

INTERVENTION

48 hours after embolization

Repeat CT angiogram shows occlusion of the AV

fistula

QUESTION
The arrows point to which of the
following structures?

A.
B.
C.
D.
E.

Splenic vein
Superior mesenteric artery
Celiac artery
Portal vein
Superior mesenteric vein

CORRECT!
The arrows point to which of the
following structures?

A.
B.
C.
D.
E.

Splenic vein
Superior mesenteric artery
Celiac artery
Portal vein
Superior mesenteric vein

CONTINUE WITH CASE

SORRY, THATS
INCORRECT.
The arrows point to which of the
following structures?

A.
B.
C.
D.
E.

Splenic vein
Superior mesenteric artery
Celiac artery
Portal vein
Superior mesenteric vein

CONTINUE WITH CASE

SUMMARY & TEACHING POINTS

Congenital arterioportal fistulas are rare entities and uncommon causes of portal hypertension.
Treatment goals include relieving the sequelae of portal hypertension.
Endovascular options for occlusion include stainless steel coils, detachable coils, or Amplatzer occlusion
devices.
Factors to consider: diameter of feeding vessel, length of the vessel that can be occluded without
disruption of flow to normal parenchymal branches, and the type of delivery system that can be
successfully advanced to the arteriovenous communication.
Cross sectional imaging findings that support the diagnosis of a high flow arterioportal fistula in this
patient include: direct communication between right hepatic artery branch and right portal vein,
hypertrophy of the celiac, common hepatic, proper hepatic and right hepatic arteries, and relative
atrophy of the right lobe of the liver.
The benefits to using an Amplatzer plug for occlusion of an AV fistula: correct size can be determined
prior to deployment, less risk of distal embolization, decreased time and radiation exposure required
for complete embolization compared with coils.

REFERENCES