Nine children aged between 2 months and 11 years, who had congenital dilatation of extrahepatic biliary tract, with or without intrahepatic dilatation, are presented. The radiological studies performed in eight patients demonstrated abnormal choledochopancreatico ductal junction. Thus, a long common channel is formed out of the duodenal wall, leading to a continuous reflux of pancreatic juice into the biliary system, chronic inflammation of biliary tract wall, fibrosis of muscular layer and finally different types of dilatations. The main clinical findings were upper abdominal pain, vomiting, or obstructive jaundice. In only one child the dilatation was cystic type and the clinical manifestation was palpable abdominal mass. In eight patients the initial diagnosis of biliary tract dilatation was made through abdominal ultrasound and in one case by direct visualization through emergent exploratory laparotomy. The final confirmation of the presence of long common channel was made by preoperative endoscopic retrograde cholangiopancreatography or intraoperative cholangiogram. The radiologic investigation was not performed in only one case due to bad clinical conditions. The surgical treatment indicated to all cases was Roux- en- Ÿ hepatic- jejunostomy. The late follow up demonstrated that all patients are well and assymptomatic.
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