Pancreaticobiliary malunion and incomplete pancreas divisum: an unusual cause of common bile duct obstruction

Abstract

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. 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. Treatment with choledochoduodenostomy and cholecystectomy provided thorough relief and resolution of symptoms. This is the first report of coexistent PBM and incomplete pancreas divisum in a Caucasian patient with unusually late clinical manifestation.