Spontaneous perforation of the extra-hepatic bile ducts in neonates and infants

Biliary ascites due to "spontaneous" perforation of the extra-hepatic bile ducts is uncommon, and should be suspected in an infant who, after a period of good health, develops jaundice and progressive abdominal distension. When the presentation is acute without jaundice the diagnosis is unlikely to be considered unless paracentesis is performed. The perforation is typically located in the common bile duct near its junction with the cystic duct and simple peritoneal drainage without suture is recommended unless the bile duct is obstructed, in which case T tube drainage or a bypass procedure is required. The prognosis is good. Four patients are presented who illustrate the spectrum of clinical presentation and the different methods of management. Two patients in whom the perforation was in the cystic duct were successfully treated by cholecystectomy. In most cases the aetiology is not apparent, but important factors may be weakening of the common bile duct wall due to ischaemia or pancreatic juice, reflux, associated with a rise in choledochal pressure. In older children the onset of biliary ascites may be preceded by an acute gastrointestinal illness, which may be a predisposing factor.