Congenital Absence of the Portal Vein

A 59-year-old woman presented with abdominal bloating, elevated alkaline phosphatase and transaminases, and computed tomography abdomen/pelvis demonstrating large right-sided hepatic masses. A percutaneous fine needle aspiration demonstrated hepatocellular neoplasm concerning for hepatocellular carcinoma. Preoperative imaging demonstrated possible porto-caval shunt. She underwent uneventful right hepatic lobectomy with confirmation of porto-systemic shunt. Congenital porto-systemic shunt, or Abernethy malformation, is rare and is associated with congenital cardiac and gastrointestinal abnormalities. Additionally, congenital porto-systemic shunt is associated with increased risk of hepatic neoplasms including hepatocellular carcinoma. Recommended surveillance for these patients is not well defined.