Abstract
Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. Since the action of the sphincter of Oddi does not functionally affect the junction in PBM patients, continuous pancreatobiliary reflux occurs, resulting in a high incidence of carcinoma in the biliary tract. The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Diagnosis of PBM with endoscopic retrograde cholangiopancreatography (ERCP) is made when the communication between the pancreatic and bile ducts is maintained even during contraction of the sphincter. Characteristic pancreatographic findings of PBM are as follows : a dilated long common channel, duct derived from a common channel, dominant Santorini's duct, and frequent association with incomplete pancreas divisum. Endoscopic ultrasonography (EUS) and intraductal ultrasonography (IDUS) can demonstrate junction of the pancreatic and bile ducts outside the duodenal wall, and are useful to diagnose associated biliary carcinoma. Some cases with a relatively long common channel that are not classified as PBM because the sphincter of Oddi includes the pancreaticobiliary ductal junction (high confluence of pancreaticobiliary ducts) show pathophysiological changes similar to PBM.
