Abstract

Choledochal cyst (CDC) and annular pancreas (AP) are rare congenital anomalies of the pancreatobiliary system, and their coexistence is exceedingly uncommon, with only a few pediatric cases reported. Recognition of this association is important, as it may influence clinical presentation, surgical planning, and long-term outcomes. Herein, we reported a two-year-old female who presented with recurrent abdominal pain and nonbilious vomiting. Laboratory investigations revealed elevated pancreatic enzyme levels, and magnetic resonance cholangiopancreatography demonstrated a fusiform dilatation of the common bile duct consistent with Todani type 1 CDC. The child underwent laparoscopic cyst excision, during which an AP encircling the duodenum was unexpectedly identified. To avoid the risk of future duodenal obstruction, hepaticoduodenostomy was not performed, and laparoscopic Roux-en-Y hepaticojejunostomy was completed. The postoperative course was uneventful, and at the three-year follow-up, the child remained asymptomatic. A review of the literature revealed only 10 previously reported pediatric cases of CDC associated with AP, most presenting in the neonatal period with duodenal obstruction. This case is unique in that the AP was clinically silent and discovered only intraoperatively, underscoring the need for surgical vigilance when managing such rare associations.

Citation: Ahmad MF, Malik MA. Coexistence of choledochal cyst and annular pancreas in a child: A rare case report and literature review. Turkish J Ped Surg 2026;40(x):ivii. https://doi.org/10.62114/JTAPS.2026.194.