Purpose: Pancreaticobiliary malunion (PBM) is a distinct disease entity of the pancreatic and biliary ductal system defined as a condition in which the junction of the pancreatic and biliary ducts occurs above the duodenal wall. PBM may be combined with a stenosis of the distal common bile duct and pathological changes in the common bile duct wall (congenital cyst of bile duct), being a potentially malignant condition. Pancreas divisum, resulting from a fusion failure of the ventral and dorsal pancreatic buds, and characterized by a dominant Santorine duct, is considered to be a predisposing factor to recurrent attacks of acute pancreatitis. In incomplete pancreas divisum, the ventral and dorsal pancreas are connected by a segmental branch.
Material and methods: We report a case of a 33-year-old female patient with PBM associated with incomplete pancreas divisum, who had presented episodes of acute cholangitis due to a benign distal common bile duct stricture.
Results: Treatment with choledochoduodenostomy and cholecystectomy provided thorough relief and resolution of symptoms.
Conclusion: This is the first report of coexistent PBM and incomplete pancreas divisum in a Caucasian patient with unusually late clinical manifestation.