Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma.
We report a case of congenital absence of the portal vein (CAPV) in an adult male who underwent resection for hepatocellular carcinoma. CAPV is a rare anomaly; only 29 cases, mostly in children, have been presented to date. An association between CAPV and cardiac abnormalities, polysplenia syndrome, skeletal abnormalities and liver tumors has been seen in children. To the best of our knowledge this is the first case of CAPV in an adult male resected for hepatocellular carcinoma. However, since CAPV can be without symptoms, the number of undiagnosed cases is not known.