Introduction: Bile duct perforation (BDP) with resultant biliary ascites in children is a rare clinical condition. The aetiopathogenesis is still an enigma, with increasing evidence suggesting anomalous union of pancreaticobiliary ductal (AUPBD) system as the prime causative factor.
Clinical picture: We report 2 cases of spontaneous perforation of the bile duct confirmed on histopathological examination as choledochal cyst, in a 6-month-old female child and a 4-year-old boy who presented with subtle clinical symptoms.
Treatment and outcome: Both patients were successfully managed by excision of the gall bladder and common bile duct and Roux-en- Y hepaticojejunostomy. This procedure was performed following initial cholecystostomy drainage in the second case.
Conclusions: From the available literature and experience with our patients, BDP is not merely spontaneous but may be related to AUPBD and choledochal cyst.