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.
Purpose:
Infantile spontaneous biliary perforation is rare with variable management strategies ranging from nonoperative treatment to complex operations such as biliary-enteric reconstruction. Biliary fistula and portal vein thrombosis are known complications, though outcomes are poorly defined.
Methods:
We assessed the incidence of spontaneous biliary perforation in infants <1 year old using a population database. Next, we describe 4 patients treated at our institution and review all reported cases within the past 25 years.
Results:
The incidence of spontaneous biliary perforation is 1.5 in 1,000,000 live births. Over the past 25 years, 90 cases were reported, over half of which were initially managed with a surgical drainage procedure. The most common reason for failure of this strategy was CBD obstruction. Our 4 patients were successfully managed without biliary reconstruction despite 2 presenting with CBD obstruction. Reported complications occurred in 22% of patients, most frequently biliary fistula requiring delayed biliary reconstruction.
Conclusions:
Surgical drainage is an effective method for treatment of infantile spontaneous biliary perforation; however a persistent biliary fistula should prompt evaluation for distal CBD obstruction. Though biliary-enteric anastomosis is the historic procedure of choice for persistent fistula, with improvements in endoscopic and percutaneous treatment, extensive biliary reconstruction may be avoided in the future.
Related Concept Videos
Esophageal Perforation-II: Clinical Manifestations and Management
Clinical Manifestations:
Esophageal Perforation-I: Introduction
The location of esophageal perforation can vary, occurring anywhere along the esophagus....
Chronic Bowel Disorders: Introduction
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Inflammatory Bowel Disease V: Surgical Management
Here are some common surgical interventions for IBD:
Appendicitis-II: Diagnostic Studies and Management
Diagnosing Appendicitis
It requires a multifaceted approach, starting with a detailed physical examination to pinpoint the location and nature of the pain and identify any associated symptoms. Laboratory tests play a crucial role. A complete Blood Count (CBC) typically reveals leukocytosis (an increased number of...

