Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: A case report

Congenital absence of the portal vein (CAPV) is a rare congenital anomaly in which the superior mesenteric veins (SMV) and splenic veins converge and bypass the liver, effectively draining directly into the systemic venous circulation via the inferior vena cava (IVC), or alternatively the renal or iliac vein, creating a native portosystemic shunt. Portosystemic shunting results in clinical manifestations of hepatic encephalopathy as well as a predisposition to focal nodular hyperplasia and tumors, including adenomas, hepatoblastoma, and hepatocellular carcinoma (HCC), by the disruption of enterohepatic blood flow. Historically, CAPV has been thought to be a rare condition found mainly at autopsy, however, in recent years due to advances in radiological techniques, CAPV detection has increased. Herein we describe a patient with known CAPV who initially underwent hepatic resection for HCC. During surveillance, additional masses were discovered and were identified as recurrent HCC. Unfortunately, this patient was not a candidate for further resection or locoregional therapy. We demonstrate that transplantation is a challenging but technically viable option for treatment of HCC complicating adenomatosis-associated CAPV.