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Spontaneous biliary perforation: does external drainage constitute adequate therapy?
N Spigland1, R Greco, D Rosenfeld
1Department of Surgery, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, USA.
Insights
Spontaneous biliary perforation (SBP) in infants is rare. Prompt surgical intervention, including biliary intestinal bypass for strictures, is crucial to prevent complications like biliary cirrhosis.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatal Medicine
Background:
- Spontaneous perforation of the biliary tract (SBP) is an uncommon condition in infants, with fewer than 100 reported cases.
- Understanding the etiology and optimal management of SBP is critical for improving infant outcomes.
Observation:
- Two infant cases of SBP are presented, highlighting diverse clinical presentations.
- One infant presented with a retroperitoneal mass and perforation of the pancreatic common bile duct (CBD); the other had jaundice and perforation at the cystic duct/CBD junction with distal CBD stricture.
Findings:
- The study challenges the notion that diminished distal ductal caliber is secondary to perforation.
- Findings suggest distal biliary obstruction may not resolve with drainage alone, especially when associated with primary strictures.
Implications:
- External drainage is advocated for SBP without ductal abnormalities.
- Prompt biliary intestinal bypass is recommended for SBP with underlying strictures to prevent biliary cirrhosis and portal hypertension.
Abstract:
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.