Journal of Pediatric Surgery Case Reports · Published 2026-06-08 · DOI 10.1016/j.epsc.2026.103281
Raissa Li, Megan Z. Chiu, Kate Templeton, Amit S. Grover, Heung Bae Kim, Arin L. Madenci
Introduction: Choledochal cysts are characterized by dilation of the biliary tree with uncertain etiology. It has been theorized that an anomalous pancreaticobiliary junction (APBJ), a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall, can cause reflux of pancreatic enzymes into the biliary duct leading to cystic dilation. We present a series of three pediatric patients with previously normal caliber common bile ducts (CBD) who developed choledochal cysts in the setting of APBJ. Case presentation: Three patients with APBJ and subsequent choledochal cyst formation were identified at our institution. Patient 1 is a 16-month-old girl who presented with severe pancreatitis and was initially noted to have normal biliary anatomy. MRCP later demonstrated new CBD dilation and ERCP demonstrated an APBJ. Persistent dilation was noted on follow-up imaging, consistent with a choledochal cyst. Patient 2 is a 5-year-old boy who initially presented with gallstone pancreatitis and was found to have an APBJ seen on ERCP during sphincterotomy and stone removal. A year later, he presented with pancreatitis and persistent ductal dilation, confirming choledochal cyst formation. Patient 3 is a 7-year-old boy who experienced three episodes of pancreatitis over two years with progressive CBD dilation and APBJ seen on ERCP. All patients underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. Conclusion: These cases highlight the role of the APBJ in the development of choledochal cysts after pancreatitis. Surveillance for choledochal cysts should be considered among any individual known to have an APBJ.
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Li, R., Chiu, M., Templeton, K., et al. (2026). Anomalous pancreaticobiliary junction and new-onset biliary dilation: A case series. Journal of Pediatric Surgery Case Reports. https://doi.org/10.1016/j.epsc.2026.103281