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

  • Topics
  • Recent advances in pediatric hepatobiliary surgery
  • Published:
Journal of Hepato-Biliary-Pancreatic Sciences

Abstract

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.

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Abbreviations

PBM:

Pancreaticobiliary maljunction

JSPBM:

The Japanese Study Group on Pancreaticobiliary Maljunction

BD:

Biliary dilatation

CI:

Confidence interval

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Conflict of interest

The authors have no conflict of interest to declare regarding the contents of this manuscript.

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Authors and Affiliations

  1. Japanese Study Group on Pancreaticobiliary Maljunction, Tokushima, Japan

    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

  2. Department of Surgery, Institute of Health Biosciences, The University of Tokushima Graduate School, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan

    Yuji Morine, Mitsuo Shimada & Hiroki Ishibashi

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  1. Yuji Morine
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  2. Mitsuo Shimada
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  3. Hideo Takamatsu
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  4. Tatsuo Araida
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  5. Itaru Endo
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  6. Masayuki Kubota
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  7. Akira Toki
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  9. Toshinobu Matsumura
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  12. Terumi Kamisawa
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  13. Hiroshi Shimada
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Correspondence to Yuji Morine.

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Morine, Y., Shimada, M., Takamatsu, H. et al. Clinical features of pancreaticobiliary maljunction: update analysis of 2nd Japan-nationwide survey. J Hepatobiliary Pancreat Sci 20, 472–480 (2013). https://doi.org/10.1007/s00534-013-0606-2

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  • DOI: https://doi.org/10.1007/s00534-013-0606-2

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