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Congenital extrahepatic portosystemic shunt

Case contributed by Ameen Rageh
Diagnosis probable

Presentation

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.

Conclusion:

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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