Large focal nodular hyperplasia and extrahepatic portosystemic shunt in a male patient: multi-modality imaging features.

Congenital extrahepatic portocaval shunt is a rare condition that is described mostly in female patients. We report an unusual case of a young adult male patient with type 1 congenital extrahepatic portocaval shunt with associated development of a focal nodular hyperplasia on a background of regenerative nodules. With multi-slice CT utilisation, there is increased detection of portocaval malformation in asymptomatic patients. This congenital variant is clinically significant with associated development of hepatocellular lesions, hepatic dysfunction and/or encephalopathy.