Topics on pathophysiology and treatment of pancreaticobiliary maljunction

Abstract

Pancreaticobiliary maljunction (PBM) is defined as a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall. Congenital biliary dilatation (CBD) is defined as a malformation which has both extrahepatic biliary dilatation and PBM. PBM causes reciprocal regurgitation of bile and pancreatic juice, which produces intermittent symptoms during childhood and biliary carcinoma in adulthood. Symptoms of abdominal pain and vomiting are caused by transient obstruction in the common bile duct with protein plugs. Plugs consist of insoluble lithostathine cleaved by trypsin, both of which are regurgitated from the pancreas. Biliary cancers are caused by chronic irritation of hazardous substances, produced by mixture of bile and pancreatic juice. Prevention of regurgitation and resection of organs tend to suffer from biliary cancer is necessary for treatment. Treatment of CBD involves bile duct resection and separation of bile and pancreatic juice. Complete excision of intrapancreatic bile duct and treatment of congenital intrahepatic bile duct stenoses are necessary to prevent later complication.