Choledochal Cyst with Pancreas Divisum – Surgical Insight into A Rare Association

Congenital pancreaticobiliary anomalies like pancreatic divisum (PD), choledochal cysts (CDC), anomalous pancreaticobiliary ductal union (APBDU) have been reported in 5.7% of patients undergoing magnetic resonance cholangiopancreatography. CDC’s are characterized by abnormal dilatations of the intrahepatic and/or extrahepatic portion of the biliary tree and can be complicated by cystolithiasis, cholangitis, pancreatitis and malignant transformation necessitating surgical managemnet. While CDCs are commonly associated with APBDU, combination of CDC with PD is rare and a potential surgical challenge. We report a case of recurrent cholangitis in a patient with CDC and coincidental classic PD and illustrate how preoperative identification, fastidious dissection technique to safeguard both the pancreatic ducts and simple intraoperative preemptive strategies may decrease consequences of distal stump blowout. PD, pancreatic divisum; CBD, common bile duct; CDC, choledochal cysts; APBDU, anomalous pancreaticobiliary ductal union; MRCP, magnetic resonance cholangiopancreatography; DOS, Duct of Santorini; DOW, Duct of Wirsung; PTBD, percutaneous transhepatic biliary drainage; RYHJ, roux en Y hepaticojejunostomy.