Spontaneous biliary perforation in infancy: Management strategies and outcomes

Cerine Jeanty1, S Christopher Derderian1, Shinjiro Hirose1

  • 1University of California, San Francisco, Campus Box 0570, 513 Parnassus Avenue, San Francisco, CA 94143-0570, United States.

Insights

Infantile spontaneous biliary perforation is rare but surgical drainage is effective. Persistent biliary fistula requires evaluation for common bile duct obstruction, potentially avoiding extensive reconstruction.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Hepatology

Background:

  • Infantile spontaneous biliary perforation (ISBP) is a rare condition with varied management approaches.
  • Complications like biliary fistula and portal vein thrombosis are known but poorly defined.

Observation:

  • The incidence of ISBP is 1.5 per 1,000,000 live births.
  • A review of 90 reported cases over 25 years showed over half initially managed with surgical drainage.
  • Common bile duct (CBD) obstruction was the most frequent reason for drainage failure.

Findings:

  • Four institutional cases were successfully managed without biliary reconstruction, even with CBD obstruction.
  • Complications occurred in 22% of reported patients, primarily biliary fistula requiring delayed reconstruction.
  • Surgical drainage is an effective initial treatment for ISBP.

Implications:

  • Persistent biliary fistula warrants investigation for distal CBD obstruction.
  • Advances in endoscopic and percutaneous treatments may reduce the need for extensive biliary reconstruction in ISBP.
  • Surgical drainage offers a viable treatment for ISBP, with potential for less invasive future management.
Abstract

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