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

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).

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.

REFERENCES