Congenital biliary dilatation.

Congenital biliary dilatation is commonly associated with pancreaticobiliary malunion. Its etiology remains unknown. With the advent of accurate cholangiography, combined abnormalities of the intrahepatic duct, common channel, and pancreatic duct are being identified more frequently in patients with congenital biliary dilatation. Early diagnosis followed by cyst excision is the treatment of choice, even in asymptomatic cases. The treatment of intrahepatic and intrapancreatic ductal diseases such as intrahepatic duct dilatation and stone debris in the intrahepatic duct and common channel are also discussed. The value of intraoperative endoscopy as an adjunct to cyst excision for the prevention of postoperative complications is explained.