Clinical analysis of congenital bile duct dilatation: Experience with 160 patients over a 7-year period

From July 1989, to June 1996, 160 patients with congenital bile duct dilatation (CBD) were treated at our institution. The incidence of CBD at our institution has increased annually. In approximately 90% of patients with CBD there was associated anomalous arrangement of the pancreaticobiliary ductal system, and we concluded that this was one of the main causes of CBD. Pediatric endoscopic retrograde cholangiopancreatography was first introduced to China in 1989, and has been successfully performed in 90.2% of 92 patients at our institution. We found that age at diagnosis was closely related to the subtype, complications, and mortality of CBD. The subtype also correlated with the presenting symptoms. One hundred and forty-seven of the 160 patients underwent surgery. Of these 147 patients, 136 (92.5%) were cured with normal liver function and 9 (6.1%) improved with liver impairment. Two patients died, one of postoperative pneumonia and one of liver failure. We conclude that early radical surgery and careful postoperative follow-up are essential in the prevention of CBD complications such as cholangitis, pancreatitis, biliary stones, and development of carcinoma. This article is based on a special lecture delivered before the 19th meeting of the Japanese Society of Pancreatico-biliary Maljunction, on September 14, 1996, in Tokyo