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A case of pancreaticobiliary maljunction with a connecting duct without a long common channel

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Abstract

Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall, usually forming an abnormally long common channel. In PBM, since the long common channel defeats the effect of the sphincter of Oddi, pancreatobiliary reflux frequently occurs, resulting in high rates of biliary tract cancers. We present the case of a 68-year-old female with advanced gallbladder cancer concomitant with bile duct cancer associated with PBM without biliary dilatation that had an extremely rare configuration showing a connecting duct without a long common channel. Pancreatography in the selectively cannulated main pancreatic duct showed the terminal portion of the common bile duct via an abnormal connecting duct. Cholangiography in the selectively cannulated lower bile duct showed the main and accessory pancreatic ducts via the connecting duct. The bile amylase level was markedly elevated. This case of a rare configuration of PBM with a connecting duct without a long common channel is the first such reported case in the English literature.

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Correspondence to Terumi Kamisawa.

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The authors declare that they have no conflict of interest.

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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration.

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Informed consent was obtained from all patients for being included in the study.

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Kikuyama, M., Kamisawa, T., Kuruma, S. et al. A case of pancreaticobiliary maljunction with a connecting duct without a long common channel. Clin J Gastroenterol 10, 196–199 (2017). https://doi.org/10.1007/s12328-017-0723-6

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  • DOI: https://doi.org/10.1007/s12328-017-0723-6

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