Anomaly of the portal vein with an anomalous hepatic vein--the first case report.

An anomaly of the portal vein associated with an anomalous hepatic vein is described as the first reported case. A 44-year-old woman was incidentally found to have a huge hemangioma by ultrasonography. Computed tomography revealed an anomalous portal system with a normally located gallbladder and round ligament. Arterial portography revealed anomalous branching of the portal vein, with absence of the left umbilical portion, the curved right portal branch mimicked the right-sided umbilical portion. Hepatic venography demonstrated patent umbilical veins communicating with the left hepatic vein. Intraoperatively the hepatic vein branch was found on the surface of the right hepatic lobe and the common bile duct was dorsal to the hepatic artery. A limited excision of the involved liver was performed safely with the help of the preoperative definition of the abnormal liver anatomy.