Abernethy Syndrome Type 1B in a Young Male presenting with acute gastrointestinal bleeding: A Case report

Abstract

Abernethy syndrome is a rare congenital extrahepatic portosystemic shunt (CEPS) in which splanchnic blood flows straightly into the inferior vena cava (IVC). Compared to type 1, in which the intra hepatic portal tree is not developed, in type 2 the portal vein is intact, but forms an anastomosis with the IVC. In the subtype 1a, superior mesenteric and splenic veins do not merge into the portal vein, while in type 1b they anatomically form the portal vein. CEPS can cause a wide spectrum of clinical manifestations including hepatic, pulmonary, metabolic and neurological symptoms and is usually diagnosed before adulthood. The diagnosis is established using the radiological imaging methods. Whileliver transplantation is the treatment of choice for type 1, surgical or minimally invasive endovascular shunt closure are treatment options for type 2 Abernethy syndome.