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World J Gastrointest Endosc. Jan 16, 2011; 3(1): 1-5
Published online Jan 16, 2011. doi: 10.4253/wjge.v3.i1.1
Endoscopic diagnosis of pancreaticobiliary maljunction
Terumi Kamisawa, Kensuke Takuma, Fumihide Itokawa, Takao Itoi
Terumi Kamisawa, Kensuke Takuma, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo 113-8677, Japan
Fumihide Itokawa, Takao Itoi, Department of Gastroenterology, Tokyo Medical University, Tokyo 113-8677, Japan
Author contributions: Kamisawa T and Itoi T wrote the paper; and Kamisawa T, Takuma K, Itoi T and Itokawa F gathered the data.
Correspondence to: Terumi Kamisawa, MD, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. kamisawa@cick.jp
Telephone: +81-3-38232101 Fax: +81-3-38241552
Received: October 15, 2010
Revised: December 10, 2010
Accepted: December 17, 2010
Published online: January 16, 2011

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. As 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 biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Endoscopic retrograde cholangiopancreatography (ERCP) is the most effective examination method for close observation of the pattern of the junction site. When the communication between the pancreatic and bile ducts is maintained, despite contraction of the sphincter on ERCP, PBM is diagnosed. In these patients, levels of pancreatic enzymes in the bile are generally elevated, due to continuous pancreatobiliary reflux via a long common channel. Magnetic resonance cholangiopancreatography and 3D-computed tomography can diagnose PBM, based on findings of an anomalous union between the common bile duct and the pancreatic duct, in addition to a long common channel. Endoscopic ultrasonography and intraductal ultrasonography can demonstrate the junction outside the duodenal wall, and are useful for the diagnosis of associated biliary cancer. Gallbladder wall thickness on ultrasonography can be a screening test for PBM.

Keywords: Pancreaticobiliary maljunction, Pancreatobiliary reflux, Congenital choledochal cyst, Endoscopic retrograde cholangiopancreatography, Endoscopic ultrasonography, Magnetic resonance cholangiopancreatography