Kikuyama Masataka, Kamisawa Terumi, Kuruma Sawako, Chiba Kazuro, Koizumi Satomi, Tabata Taku, Honda Goro
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan.
Department of Endoscopy, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
Clin J Gastroenterol. 2017 Apr;10(2):196-199. doi: 10.1007/s12328-017-0723-6. Epub 2017 Feb 22.
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.
胰胆管合流异常（PBM）是一种先天性畸形，其中胰管和胆管在十二指肠壁外进行解剖学上的汇合，通常形成一条异常长的共同通道。在PBM中，由于长的共同通道削弱了Oddi括约肌的作用，胰胆管反流经常发生，导致胆道癌的高发病率。我们报告了一例68岁女性，患有晚期胆囊癌并伴有与PBM相关的胆管癌，且无胆管扩张，其具有极其罕见的形态，显示出一条没有长共同通道的连接导管。选择性插管至主胰管进行的胰管造影通过一条异常连接导管显示了胆总管的末端部分。选择性插管至低位胆管进行的胆管造影通过连接导管显示了主胰管和副胰管。胆汁淀粉酶水平显著升高。这种具有无长共同通道的连接导管的PBM罕见形态的病例是英文文献中首次报道的此类病例。
