Congenital choledochal cyst: Occurrence of primary intrahepatic stones and prevention of carcinogenesis

Intrahepatic biliary stones and carcinogenesis are two major complications seen after corrective surgery for congenital choledochal cyst. The incidence of these two complications was reviewed in patients seen at out-patient clinics at a mean follow-up time of 13 years and 6 months. Intrahepatic biliary stones developed in 5 of 56 patients who had undergone a standard excisional operation; in 4 of the 5 patients, the stones were found in the preexisting intrahepatic biliary dilatation (with or without a downstream stenosis), 7–15 years postoperatively. Biliary stricture with dilatation is a putative cause of intrahepatic bile duct stones, and this hypothesis was supported here, since stones developed in all 3 of the patients who had the peculiar configuration of intrahepatic biliary dilatation and stenosis. Biliary carcinoma was not observed in any of the 56 patients who had undergone standard excision of a choledochal cyst followed by hepatico-jejunostomy, but developed in 1 of 12 patients in whom complete excisional resection of the dilated extrahepatic bile duct had not been performed. The occurrence of carcinoma in choledochal cysts is reviewed.