Extrahepatic portosystemic shunt

Case contributed by James Mirow


Background cirrhosis. US showed dilated bile duct ?cause

Patient Data

Age: 65 years
Gender: Female

Two calcified splenic artery aneurysms (max diameter 16mm) and splenomegaly.

A large extrahepatic portosystemic shunt is present, originating from the left distal external iliac vein, weaving tortuously up the anterior abdomen where it communicates with a recanalized umbilical vein before terminating at the left branch of the portal vein (see CPR reformat).

The portal vein max diameter measures 21mm, with no evidence of a venous thrombus.

Irregular liver margins indicate cirrhosis.

Small pericardial and bilateral pleural effusions.

Two splenic artery aneurysms (open red arrows). 

Case Discussion

Extrahepatic portosystemic shunts are a rare venous abnormality that can be congenital or acquired.

Those of congenital origin are classified by the Abernethy Classification System, either type 1 or 2. This kind is associated with other issues such as an increase in liver tumors (FNH, HCC etc.)

Shunts that are acquired are the result of chronic portal venous hypertension that results in small portosystemic anastomoses to dilate. Lee et al mention that there are over 20 variations of acquired shunts described in the literature, with varices occurring in a number of areas including esophagus, gastric, paraumbilical, coronary and azygos veins.

It is important that venous anomalies like these are assessed and described as issues (some life-threatening) can be associated, including pyelothrombosis, or portal vein thrombus, pylephlebitis, gas within the venous system and venous calcifications.

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