Idiopathic perforation of the biliary tract in infancy

Abstract

Idiopathic perforation of the bile duct is rare in children. Sixty-seven cases were reported in the English literature to 1980. It is, nevertheless, the second commonest surgical cause of jaundice in the neonate. The etiology is unknown though distal obstruction and weakness in the bile duct wall have been postulated. Limited surgical treatment with external drainage is the preferred therapy. In isolated cases internal drainage procedures or repeated aspiration have been successful. The diagnosis should be suspected in the presence of jaundice and ascites with or without abdominal pain and signs of peritoneal irritation. We describe a 3-month-old girl presenting with anemia, vomiting, jaundice, and ascites. This was initially diagnosed as hepatitis but bilious fluid was found on paracentesis. Computerized tomography with cholangiography and 99 MTC Diisopropyl IDA cholescintigraphy confirmed the diagnosis. The latter seems to be more accurate than I-131 Rose Bengal. The perforation was at the junction of the hepatic and cystic ducts. It was treated successfully by external drainage and a cholecystostomy. Direct attempts to close the perforation, or more complicated surgical procedures, are unnecessary while nonoperative treatment carries a high mortality. At follow-up after 1 year the IV cholangiogram and liver-function tests are normal. Cholecystostomy provided good drainage of the biliary ducts as well as easy access for follow-up cholangiography.