[A surgical treatment for congenital bile duct dilatation with intrahepatic calculi]

A total of 141 cases of congenital bile duct dilatation (excluding those with cancer) were treated at the Department of gastroenterological Surgery of Tokyo Women's Medical College in the past 27 years. The lesion was morphologically classified as Todani type I in 93 patients, type II in 1, type III in 2, type IV-A in 42, and type V or multiple dilatation of the peripheral bile ducts in 3. Cholangiectasis was accompanied by intrahepatic stones in 1 type I patient, 18 type IVA patients, and 2 type V patients. None of type II and type III patients had intrahepatic stones. Of the 21 patients with intrahepatic stones, only 2 (both type IVA) had no history of surgery. In these patients, a common bile duct stone was considered to be the cause of intrahepatic stone formation. Nineteen patients had undergone surgery, comprising cholecystectomy in 2 type V patients, bypass surgery in 5 type IVA patients, and excision of the extrahepatic bile ducts with biliary tract reconstruction in 1 type I and 11 type IVA patients. Of the 12 patients who underwent excision of the extrahepatic bile ducts with biliary tract reconstruction, intrahepatic stone formation was considered to be ascribable to stenosis at the choledocho-jejunostomy site in 3 and to insufficient excision of the extrahepatic bile ducts in 4. Therefore, when performing excision of the extrahepatic bile ducts and biliary tract reconstruction in patients with congenital cholangiectasis, we now take care to achieve thorough excision of the extrahepatic bile ducts and not leave any stenotic region in the porta hepatis. For choledocho-jejunostomy, we join the left and right hepatic ducts as necessary or incise the hepatic duct axialy so as to make the anastomotic orifice large enough. In the 5 remaining patients, intrahepatic stone formation was considered to be due to persistence of intrahepatic bile duct dilatation or stenosis, although there were no technical problem with excision of the extrahepatic ducts and biliary tract reconstruction. In 4 of the 5 patients, partial hepatectomy was performed to remove the intrahepatic stones, but new stones formed again in 2 of them. When intrahepatic bile duct dilatation or stenosis is localized at the periphery of lobule, hepatectomy is indicated. However, when intrahepatic bile duct dilatation or stenosis is extensive in both lobes, no curative technique is available and bile duct dilatation or stenosis wil persist in the liver after surgery. In such cases, the disease may become further complicated by cholangitis and intrahepatic stone formation, making it very difficult to control. Therefore, patients with extensive intrahepatic bile duct dilatation or stenosis should be monitored very carefully even after surgery.