Nationwide survey of cases of choledochal cyst. Analysis of coexistent anomalies, complications and surgical treatment in 645 cases.

A nationwide survey of cases of choledochal cyst was conducted to obtain the data on cases of choledochal cyst in Japan between 1972 and 1982. A total of 645 cases in 73 institutions, including 52 cases from this hospital, were surveyed. Choledochal cyst was associated with an anomalous arrangement of the pancreaticobiliary ductal system in 90.2% of the cases which were examined extensively. The serum amylase level was elevated in 26.6% of the cases. Calculus disease of the biliary duct was recognized in 28.5% of the cases. Biliary carcinoma developed in 54 (8.4%) of 645 cases. The incidences of biliary carcinoma were 0.3% (1/306) in cases in children and 15.6% (53/339) in adult cases; carcinoma was found in the wall of the choledochus in 30, and in the gallbladder in 16 of these 54 cases. The nationwide survey showed that an anomalous arrangement of the pancreaticobiliary ductal system was closely associated with choledochal cyst and that cases of biliary carcinoma developed with the advance of age. Therefore, early resection of the cyst wall and reconstruction of the biliary duct is proposed as suitable surgical treatment for stopping reflux of pancreatic juice into the biliary duct.