Thirteen patients with congenital biliary dilatation treated in our department were analyzed in relation to its complications and other features. Two of the 13 patients were associated with biliary tract cancer and Both lesions were unresectable, because of their advanced stages. The dilated bile ducts appeared cystic, and the cancers involved the intrahepatic bile ducts and dilated extrahepatic bile ducts. The level of amylase in bile was high, suggesting that prolonged regurgitation of pancreatic juice into the bile ducts had induced the canceration. Concomitant cholelithiasis was found in 5 patients, 3 of whom had bilirubin calciferous stones. Morpholog ically, the dilated bile duct was classified as type IV-A in 3 and type Ia and Ib in each one. Stones were intrahepatic in 2 of the 3 cases of type IV-A. Pancreatic lithiasis was found in 3 patients; stones were present in the Wirsung's canal, Santorini's canal and both canals in each one. Two of the 3 had no positive stones upon roentgenography. The pancreatic stones were removed by papilloplasty or accessory papilloplasty. The 3 patients were treated by resection of the dilated extrahepatic bile duct with bile duct reconstruction. Postoperative pancreatic fistula occurred in 2 patients undergone a second operation after cystoduodenostomy or cystojejunostomy, who could be successfully managed by conservative treatment alone. Prognosis was favorable in all patients except those accompanied by cancers.
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