Interventional, surgical, and clinical management of Abernethy malformations: A case series and novel anatomic variant

Abernethy malformation is a rare congenital extrahepatic portosystemic shunt resulting from the persistence of embryonic vessels in the splanchnic venous system. There are 2 known types of Abernethy malformations: type I and II; both of which involve aberrant drainage of the portal venous blood into the inferior vena cava. Here, we detail the anatomy as well as clinical, surgical, and interventional management of 4 patients with Abernethy malformations. In addition, we detail a patient with a previously undescribed anatomic variant of the Abernethy type II malformation: a portosystemic shunt into an interrupted inferior vena cava in which central venous return was provided through the azygous/hemiazygos system.