Dilatation of the intrahepatic bile duct associated with congenital anomalous junction of the cystic duct--a new disease entity.

We describe 3 children with dilatation of the intrahepatic bile duct, who had anomalous junctions of the cystic duct, 2 high and 1 low, without pancreaticobiliary maljunction. They were all male, and underwent excision of the gallbladder and the extrahepatic bile duct followed by a Roux-en-Y hepaticojejunostomy as a definitive surgery. Postoperatively, the dilated intrahepatic bile duct became normal in size. From these 3 cases, we propose a new disease entity-dilatation of the intrahepatic bile duct because of congenital anomalous junction of the cystic duct.