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

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.

A B

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. Splenic vein B. Superior mesenteric artery C. Celiac artery D. Portal vein E. Superior mesenteric vein

CORRECT!

The arrows point to which of the following structures?

A. Splenic vein B. Superior mesenteric artery C. Celiac artery D. Portal vein E. Superior mesenteric vein

CONTINUE WITH CASE

SORRY, THATS INCORRECT.

The arrows point to which of the following structures?

A. Splenic vein B. Superior mesenteric artery C. Celiac artery D. Portal vein E. 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