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.
In the absence of signs of chronic liver disease/portal hypertension, the findings are suggestive of a congenital extrahepatic portosystemic shunt (type 2).
2 case question available
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
- Gallego C, Velasco M, Marcuello P, Tejedor D, De Campo L, Friera A. Congenital and acquired anomalies of the portal venous system. (2002) Radiographics : a review publication of the Radiological Society of North America, Inc. 22 (1): 141-59. doi:10.1148/radiographics.22.1.g02ja08141 - Pubmed
- Wai-Kit Lee, Silvia D. Chang, Vinay A. Duddalwar, Jules M. Comin, Warren Perera, Wing-Fai E. Lau, Elhamy K. Bekhit, Oliver F. Hennessy. Imaging Assessment of Congenital and Acquired Abnormalities of the Portal Venous System. (2011) RadioGraphics. 31 (4): 905-26. doi:10.1148/rg.314105104 - Pubmed
- Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. (2011) Radiographics : a review publication of the Radiological Society of North America, Inc. 31 (3): 707-22. doi:10.1148/rg.313105070 - Pubmed