Congenital extrahepatic portosystemic shunt

Case contributed by Dr Hernán Walter Brouver de Koning

Presentation

Abdominal discomfort. Referred to evaluate an abnormal vessel seen on US.

Patient Data

Age: 60 years
Gender: Male

Abnormal vascular structure connecting the proximal portal vein with the left renal vein. It runs through the minor omentum surrounding the gastric fundus. Hypoplastic distal portal vein. Intrahepatic portal system is developed. Liver with normal morphology and size, a small biliary cyst is seen in segment IVa. No signs of portal hypertension or ascites.

Case Discussion

Congenital extrahepatic portosystemic shunts (CEPS) are rare disorders in which the portomesenteric blood diverts to the systemic veins via congenital shunts.

In type II CEPS there is partial preservation of the portal circulation, frequently the portal vein is hypoplastic, and drains via a fistula to a systemic vein, as in this case the left renal vein.

Occasionally patients are asymptomatic and these can be an incidental finding. The patient must be monitored as they are at risk of developing hepatic encephalopathy.

It is important to diagnose portal hypertension, other congenital abnormalities, and liver lesions secondary to the shunt - the most frequently described is nodular regenerative hyperplasia.

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