Pancreaticobiliary Maljunction: A Multidimensional Exploration of Pathophysiology, Diagnosis, Classification, Management and Research Prospects.

Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall, usually forming a markedly long common channel, which can cause reciprocal reflux between pancreatic juice and bile. Cholangiography, endoscopic ultrasonography, surgery, and autopsy can be used to diagnose pancreaticobiliary maljunction. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of pancreaticobiliary maljunction. The regurgitation may lead to the development of various hepatobiliary and pancreatic disorders such as pancreatitis and biliary carcinoma. The pathogenesis of pancreaticobiliary maljunction is the result of a series of pathophysiological changes caused by reflux. Surgery is recommended for patients diagnosed with pancreaticobiliary maljunction irrespective of the presence or absence of symptoms because of its high biliary carcinogenicity, but the treatment strategy is quite different between adult patients with and without biliary dilatation.