Abstract
Pancreaticobiliary maljunction (PBM) is defined as a congenital anomaly in which the pancreaticobiliary junction is present outside the duodenal wall. In 2012, Japanese clinical practice guideline for PBM was published. Expert physicians and surgeons of the biliary field must give correctly diagnose and perform appropriate treatment because this congenital anomaly is well known to be a high-risk factor for biliary tract cancer. ERCP is still indispensable to diagnose of PBM and is considered the " gold standard", even though the usefulness of other diagnosis procedures, such as MD-CT, MRCP, and EUS, have been reported. Cholecystectomy and resection of the extrahepatic bile duct, the so-called " flow-diversion surgery", is an established standard surgical treatment for PBM with biliary dilatation (congenital biliary dilatation). However, the appropriate surgical treatment for PBM without biliary dilatation is still unclear. The clinical points of the issue regarding PBM still remain, such as the definition of the normal diameter of a non-dilated biliary duct. Further analysis of case series and basic studies for carcinogenesis of PBM are required.
