Presentation
Hepatic encephalopathy.
Patient Data
Age: 55
Gender: Male
From the case:
Congenital extrahepatic portosystemic shunt
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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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