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A national survey of the morphologic features of hepatolithiasis in Japan disclosed that 13 (4.8%) of the 273 cases examined were associated with obvious biliary malformations: Caroli's disease with congenital hepatic fibrosis (one case), adult polycystic disease of the liver and kidneys (one case), choledochal cyst (nine cases), and anomalous pancreaticobiliary ductal union (four cases). The latter two malformations overlapped in two cases. Intrahepatic calculi in all of these cases were of brown pigment stones, suggesting that focal bile stasis as well as bacterial infection might have played an important role in their formation and growth. It seems likely that such phenomena occur in the congenitally dilated segments of the intrahepatic biliary tree. These data imply that biliary malformation is one of the lithogenic factors in hepatolithiasis in Japan.

Eighty-six patients with primary extrahepatic bile duct carcinoma operated on in the Second Department of Surgery at Nagasaki University Hospital during a recent 13.5-year period were reviewed. The patients were divided into five groups depending upon the site of the tumor. The operative mortality, resectability, postoperative survival period, and five-year survival rate in each group were studied. The lower third group had the highest resectability, lowest operative mortality and longest post operative survival period. The hepatic duct, the upper third and the extended groups, however, showed extremely poor results. The pathological features of these three groups are discussed here and an operative procedure for resection of the tumor is proposed. We emphasize that the development of methods of early diagnosis is necessary, and recommend aggressive surgical treatment for tumors of the hepatic duct, upper third, and extended groups.