Idiopathic perforation of the extrahepatic bile duct in infancy: Pathogenesis, Diagnosis, and Management

Three infants with idiopathic perforation of the extrahepatic bile ducts were treated at this hospital during the last 6 years. Two patients presented with jaundice and biliary ascites, with high bilirubin in the ascitic fluid, and one patient with peritonitis. The site of perforation was repaired and abdomen drained in one patient. Simple drainage was performed in two others, with good overall results. Perforation of the bile ducts should be suspected in every neonate and infant with ascites and jaundice. Confirmation is obtained by paracentesis and bilirubin measurement in the ascitic fluid or by combination of sonography and scintigraphy. Simple drainage is the treatment of choice provided the distal obstruction is excluded.