Catalog

Congenital extrahepatic portosystemic shunt

No MMS code

Link to WHO Definition

Congenital extrahepatic portosystemic shunt – http://id.who.int/icd/entity/385708613

Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins, such as renal, hepatic veins or the inferior vena cava (IVC). Abernethy malformation can be subdivided into two types. In type I, there is complete diversion of portal blood into the IVC and the extrahepatic portal vein is absent, whereas in type II, there is partial portal flow to the liver through a hypoplastic portal vein. Subvariants of both types exist.