Abstract
Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a union of the pancreatic and biliary ducts that is located outside the duodenal wall. The PBM results in free reflux of pancreatic juice into the bile duct and inflammatory changes in the epithelium of the bile duct, and may be a key factor in the pathogenesis of malignant changes in bile duct. The incidence of bile duct carcinoma with PBM is approximately 1,000 times greater than the incidence of bile duct carcinoma in the general population. Excision of extrahepatic biliary tract and hepaticojejunostomy is the standard operation for choledochal cysts. However, reports of bile duct carcinoma after cyst excision are gradually increasing. Careful long-term follow-up is very important, even after complete excision.
