What is the classification of Congenital Extrahepatic Portosystemic Shunt?
Type 1 (end-to-side shut) where Portal vein is directly communicating to 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.
What be other conditions associated with the congenital extrahepatic portosystemic shunt?
Cardiovascular anomalies, biliary atresia, and various other abdominal visceral anomalies. Patients are also prone to develop intrahepatic tumors (focal nodular hyperplasia, adenoma, hepatocellular carcinoma) and hepatic encephalopathy.
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).