Background: Nontraumatic perforations of the extrahepatic biliary ductal system are a rare albeit recognized occurrence in the cystic duct, choledochus and main hepatic duct; the latter appears to be the rarest.
Methods: Recent experience with such a case prompted a thorough review of 26 similar cases previously reported.
Results: It appears that obstruction of the biliary tract by gallstones results in raised intraductal pressure leading to dilatation of the biliary tree, subsequent stasis and infection, causing ascending cholangitis and thrombosis of intramural vessels, leading to necrosis and perforation of the duct wall. Most patients present with an acute abdomen and are operated upon.
Conclusions: The goals of operation, which should be tailored to the individual patient, are to stop the bile leak, cure choledocholithiasis and cholangitis and reconstruct the bile duct.