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Congenital extrahepatic portosystemic shunt

Case contributed by Ameen Rageh
Diagnosis probable


Hepatic encephalopathy.

Patient Data

Age: 55
Gender: Male

Hypoplastic portal vein. A large tortuous vein is seen communicating between the splenic vein and left renal vein consistent with a splenorenal shunt. 

Abdominal subcutaneous edema with dilated second left lumbar and subcutaneous veins. 

No finding suggesting chronic liver cirrhosis. Spleen size is normal. No ascites.


In the absence of signs of chronic liver disease/portal hypertension, the findings are suggestive of a congenital extrahepatic portosystemic shunt (type 2).

Case Discussion

Congenital portosystemic shunts are a rare condition, classified as:

  • intrahepatic portosystemic shunt. where communication occurs after the division of portal vein, to hepatic veins or IVC
  • extrahepatic portosystemic shunt (CEPS) communication occurs before the division of portal vein between the portomesenteric vasculature, and a systemic vein

CEPS further classified to:

  • type 1 (end-to-side shut) where the portal vein is directly communicating with the IVC with absent of intrahepatic portal branches
  • type 2 (side-to-side shunt) where intrahepatic are intact, however, blood flow is diverted to systemic veins
  • the term Abernethy malformation is an eponym for CEPS, and type 1 shunts are occasionally called the congenital absence of the portal vein

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