ABSTRACT
Congenital biliary dilatation (CBD), or choledochal cyst, is a cystic or fusiform dilatation of the common bile duct that is uncommon in Caucasians. There is little doubt that CBD is a congenital lesion with a strong hereditary component, which may explain the higher incidence seen in Asia, and its familial occurrence in siblings and twins. Various theories have been proposed for the etiology of CBD, but two factors are known to be causal-weakness of the wall of the common bile duct and obstruction distal to it. In recent years, cholangiography has identified anomalies of the pancreaticobiliary ductal system in association with CBD, which may allow reflux of pancreatic enzymes and subsequent dissolution of duct walls. Clinical manifestations of CBD differ according to age. Neonates and young infants usually present with an abdominal mass, or obstructive jaundice, and acholic stools, depending on the degree of obstruction.
