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 rev
ealed an anomalous portal system with a normally located gallbladder and ro
und ligament. Arterial portography revealed anomalous branching of the port
al vein, with absence of the left umbilical portion, the curved right porta
l branch mimicked the right-sided umbilical portion. Hepatic venography dem
onstrated patent umbilical veins communicating with the left hepatic vein.
Intraoperatively the hepatic vein branch was found on the surface of the ri
ght 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 hel
p of the preoperative definition of the abnormal liver anatomy.