Congenital absence of the portal vein: Define the portosystemic shunt, avoid liver transplantation.

Liver transplantation has been historically recommended for patients with congenital absence of the portal vein associated with extrahepatic congenital portosystemic shunts. Here, based on a case report of a 2-year-old girl and a thorough review of all published cases from 1974 to 2020, we show that such a diagnosis most often conceals a hypoplastic portal vein, which can be successfully re-permeabilized through the closure of the shunt in order to re-establish a physiological vascular anatomy. This highlights the importance of achieving a detailed anatomical description of extrahepatic congenital portosystemic shunts with a balloon occlusion test in order to plan the best surgical approach and avoid unnecessary liver transplantation.