Spontaneous Bile Duct Perforation in Children: A Systematic Review of 209 Cases and Practical Diagnostic and Management Considerations

Background/Objectives: Pediatric spontaneous or non-traumatic extrahepatic bile duct perforation (SBDP) is a rare cause of bile ascites and biliary peritonitis. We aimed to characterize presentation, diagnosis, management, outcomes, and summarize practical management considerations. Methods: A systematic review was performed in accordance with PRISMA 2020, we searched PubMed/MEDLINE, EMBASE, and Google Scholar from inception to February 2026, and completed backward citation tracking in June 2026. We included pediatric SBDP cases and excluded traumatic, iatrogenic, isolated gallbladder, and perforated choledochal cyst cases. Case-level data were extracted when available, and exploratory subgroup analyses were performed by age and management approach. Results: A total of 139 studies reported 208 cases; with one institutional case, final cohort of 209 patients. Median age was 3.0 months (IQR, 1.15–15.0; n = 174), and 70.5% of patients were ≤1 year old (124/176). Bilious ascites or bile-stained fluid was confirmed in 95.2%. The cystic duct–CHD/CBD junction was the most frequently reported site (82/180, 45.5%), and biliary anomalies were reported in 39.7% (83/209). A step-up initial management was used in 60.3% and immediate surgery in 38.8%. Reported survival was 97.0% (197/203), although publication bias limits interpretation. Clinical complications and reinterventions were reported in 75/198 (37.9%) and 66/209 (31.6%), respectively. Conclusions: Pediatric SBDP is age-dependent and anatomy-driven; infants more often presented with cholestatic-ascitic features, whereas older children more often had acute-abdominal features. Bilious ascites is an important diagnostic clue. Management should prioritize stabilization and source control, followed by anatomical definition and selective reconstruction.