Spontaneous biliary perforation: does external drainage constitute adequate therapy?

Spontaneous perforation of the biliary tract in infancy is rare; fewer than 100 cases have been reported. The authors report on two patients with spontaneous biliary perforation (SBP) who were treated during the past 5 years. One of the patients, a 10-month-old boy, presented with an extensive retroperitoneal mass, without jaundice. He was found to have a perforation in the pancreatic portion of the common bile duct (CBD). The second patient, a 6-week-old boy, had insidious onset of jaundice, abdominal distension, and acholic stools. He was found to have a perforation at the cystic duct/CBD junction and distal CBD stricture. Both patients initially underwent cholecystostomy and drainage. The second patient had a persistent external biliary fistula and high-grade stricture, and subsequently underwent CBD excision and Roux-en-Y hepaticojejunostomy, at age 12 weeks. The authors' findings do not support the widely held concepts proposing that (1) the diminished distal ductal caliber often associated with these lesions occurs secondary to the perforation rather than is a primary causative factor and (2) the distal biliary obstruction does not require treatment because it resolves with adequate drainage. In the absence of ductal abnormalities, the authors advocate external drainage. For SBP associated with an underlying stricture, prompt biliary intestinal bypass is necessary to avoid biliary cirrhosis and portal hypertension.