Clinical features of pancreaticobiliary maljunction: Update analysis of 2nd Japan-nationwide survey

Yuji Morine, Mitsuo Shimada, Hideo Takamatsu, Tatsuo Araida, Itaru Endo, Masayuki Kubota, Akira Toki, Takuo Noda, Toshinobu Matsumura, Shuichi Miyakawa, Hiroki Ishibashi, Terumi Kamisawa, Hiroshi Shimada

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107 Citations (Scopus)


Introduction: Pancreaticobiliary maljunction (PBM) is a congenital anomaly, which can be defined as a union of the pancreatic and biliary ducts located outside off the duodenal wall. We herein investigate clinical features of PBM including as the 2nd report of a Japanese nationwide survey. Patients and methods: During a period of 18 years (from 1990 to 2007), 2,561 patients with PBM were registered at 141 medical institutions in Japan. Among them, eligible patients (n = 2,529) were divided into two groups: adult (n = 1,511) and pediatric patients (n = 1,018). Comparisons of clinical features including associated biliary cancers were performed according to the biliary dilatation (BD), age factor, and time era. Results: Only one case in pediatric patients with BD combined with a bile duct cancer (0.1 %). In adult patients, the bile duct cancer and the gallbladder cancer was seen in 6.9 and 13.4 % patients with BD and in 3.1 and 37.4 % patients without BD, respectively. In adult patients with BD, the occurrence rates of biliary cancers were increased in latter period (00'-07') compared with former period (90'-99'). The ratio of biliary cancer localization was changed between former and latter period, and the bile duct cancer was increased in latter period (from 5.5 to 9.3 %). Conclusions: The largest series of PBM were evaluated to clarify the clinical features including the associated biliary cancer in this Japan-nationwide survey. This report could be widely used in the future as a reference data for diagnosis and treatment of PBM.

Original languageEnglish
Pages (from-to)472-480
Number of pages9
JournalJournal of Hepato-Biliary-Pancreatic Sciences
Issue number5
Publication statusPublished - Jun 2013
Externally publishedYes


  • Amylase level
  • Biliary cancer
  • Biliary dilatation
  • Time era

ASJC Scopus subject areas

  • Surgery
  • Hepatology


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