Classification of pancreaticobiliary maljunction and its clinical features in adults.

In pancreaticobiliary maljunction (PBM), reflux of pancreatic juice and bile produces various pathological conditions in the biliary tract and pancreas. Clinical features according to the classification of PBM by confluence between the distal bile duct and the main pancreatic duct proposed in 2015 were evaluated in children.Clinical features and complicating diseases according to the PBM classification were evaluated in 168 adult PBM patients. Patency of Santorini duct and associated biliary carcinomas were evaluated in 123 patients.Similar to children, there were significant differences in age (P<0.01) and type of common bile duct (P<0.01) between the groups of the classification. Unlike in children, there was no significant difference in the incidence of abdominal pain and hyperamylasemia. There were 87 associated biliary carcinomas (79 gallbladder carcinomas and 8 cholangiocarcinomas). PBM patients with a cudgel-type Santorini duct, which is greater than 2 mm in diameter, did not develop biliary carcinomas, compared to 61.1% of those with other types of Santorini duct (P<0.01).Clinical features according to the PBM classification in adults were different from those in children. Although biliary carcinomas were frequently seen in adult PBM patients, none of those with a cudgel-type Santorini duct developed biliary carcinoma.