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, and this anormaly occur various pathology including carcinogenesis due to reflux and stasis of bile mixed with pancreatic juice. We herein review epidemiology and clinical features of PBM, in particular, based on nationwide survey (n=2,561) of Japanese study group of PBM.
Almost cases had clinical symptoms. Particularly, acute pancreatitis was most frequent complication in pediatric patients. Regarding cancer occurrence, in pediatric patients, only one case with biliary dilatation was combined with a bile duct cancer. In adult patients, the occurrence rates of biliary cancers were 21.6% in patients with biliary dilatation and 42.4% in patients without biliary dilatation. Overall incidence of biliary cancers with PBM is from 1,000 to 3,000 times higher comparing to the risk in the general population.
Further evaluation and follow-up survey were still necessary for the elucidation of pathology and adequate diagnosis and treatment of PBM.
