Congenital choledochal cyst with intrahepatic involvement.

We reviewed congenital choledochal cyst with intrahepatic involvement in 38 patients aged 52 days to 29 years. Dilatations were classified into the following three forms, based on the shape of the extrahepatic and intrahepatic bile duct: cystic-cystic, cystic-cylindrical, and cylindrical-cylindrical. Stenosis of the hepatic duct near the hilum probably does not cause dilatation of the intrahepatic duct. The anomalous arrangement of the pancreatobiliary union seems to determine only the form of extrahepatic dilatation, and cystic dilatation of the intrahepatic duct may be caused by some other factors. Total excision of the extrahepatic bile duct with the creation of a wide anastomotic stoma apparently is the treatment of choice. Hepatectomy would be recommended for some patients with the cystic-cystic form.