Liver transplantation in a patient with developmental interruption of the inferior vena cava with azygos substitution

Abstract

Infrahepatic interruption of the inferior vena cava (IVC) with azygos or hemiazygos substitution has been reported frequently in children with biliary atresia where this venous abnormality is associated with other venous abnormalities such as preduodenal portal vein or congenital heart disease. It is important to recognize this anomaly pretransplant because the hepatic vein may drain directly into the right atrium rather than into the suprahepatic vena cava. We describe herein the first report of an orthotopic deceased donor liver transplant in an adult patient with an interrupted IVC and azygos continuation. We also review the embryological development of the IVC and the vascular anomalies that can occur.