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Current status of Hirschsprung’s disease: based on a nationwide survey of Japan

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Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Purpose

The diagnosis and surgical treatments of Hirschsprung’s disease (HD) have undergone various changes in the last few decades because of establishment of laparoscopic procedures. A retrospective nationwide survey for 4 decades was performed to study the changing profile of HD in Japan.

Methods

The patient data were collected in 4 phases: Group 1, between 1978 and 1982; Group 2, between 1988 and 1992; Group 3, between 1998 and 2002; and Group 4, between 2008 and 2012.

Results

The incidence and the male/female ratio remained almost the same over time (1/4, 895 in newborns and 2.9:1 in Group 4). The patients with a family history increased to 7.1% in Group 4, in comparison to 2.8–6.0% in other groups. Regarding the extent of aganglionosis, sigmoid colon increased to 63.1% in Group 4, compared to 51.9% in Group 3. Manometry was performed less frequently in Group 4 (45.8%) than in Group 3 (66.1%). Transanal endorectal pull-through (TAEPT) was the most popular operation in Group 4 (49.6%). In addition, laparoscopy-assisted operations increased to 46.9% in Group 4, in comparison to 29.7% in Group 3. The incidence of preoperative enterocolitis and the mortality rate in Group 4 were 17.2% and 2.4%, respectively, and were markedly decreased in comparison to Group 1 (29.2% and 6.5%, respectively). The mortality rate decreased over time to 2.4% in Group 4. Over the last decade, there has been remarkable improvement in the mortality rate associated with the small intestine (aganglionosis extending orally to more than 30 cm of the terminal ileum). The rates were 25.5% in Group 4, 53.6% in Group 1, 33.3% in Group 2, and 35.5% in Group 3. In addition, the mortality rates of the remaining aganglionosis subgroups also improved.

Conclusion

Primary operations without laparotomy, including TAEPT and laparoscopy-assisted operations, have become the first choice for the definitive surgical treatment of HD in Japan. The mortality rate has decreased over time. However, the mortality rate of small intestinal aganglionosis is still relatively high. The development of new treatment strategy for small intestinal aganglionosis is called for.

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Acknowledgements

The authors thank Mr. Brian Quinn for editing the manuscript. The authors wish to thank all of the doctors in the 156 pediatric surgery departments at major universities and hospitals throughout Japan for replying to the questionnaires (Appendix). This study was supported by a grant from The Ministry of Health, Labour and Welfare of Japan [Health and Labour Sciences Research Grants for Research on Intractable Diseases (H26-045)].

Author information

Authors and Affiliations

  1. The Japanese Study Group for Hirschsprung’s Disease, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Tomoaki Taguchi, Satoshi Obata & Satoshi Ieiri

  2. Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Fukuoka, 812-8582, Japan

    Tomoaki Taguchi & Satoshi Obata

  3. Department of Pediatric Surgery, Field of Developmental Medicine, Kagoshima University, Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan

    Satoshi Ieiri

Authors
  1. Tomoaki Taguchi
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Corresponding author

Correspondence to Tomoaki Taguchi.

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Additional information

T. Taguchi and S. Obata have contributed equally in this paper.

Appendix

Appendix

Aichi Children’s Health and Medical Center

Aichi

Aichi Prefectural Colony Central Hospital

Aichi

Aso Iizuka Hospital

Fukuoka

Chiba Children’s Hospital

Chiba

Chiba University Hospital

Chiba

Department of Gastroenterological Surgery I Hokkaido University Graduate School of Medicine

Hokkaido

Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University

Tokyo

Department of Surgery, Asahikawa Medical University

Hokkaido

Department of Surgery, Iwate Medical University School of Medicine

Iwate

Department of Surgery, Miyagi Children’s Hospital

Miyagi

Department of Surgery, Shiga University of Medical Science

Shiga

Department of Surgery, Tokyo Metropolitan Ohtsuka Hospital

Tokyo

Division of Pediatric Surgery, Integrative Center of Surgery, Gunma University Hospital

Gunma

Dokkyo Medical University Koshigaya Hospital

Saitama

Ehime Prefectural Central Hospital

Ehime

First Department of Surgery, Dokkyo Medical University

Tochigi

Fujita Health University Hospital

Aichi

Fukuoka Chidren’s Hospital

Fukuoka

Fukuoka University

Fukuoka

Fukushima Medical University

Fukushima

Gunma Children’s Medical Center

Gunma

Hepato-Biliary-Pancreatic Surgery and Transplantation, Kyoto University Hospital

Kyoto

Hiroshima City Hiroshima Citizens Hospital

Hiroshima

Hiroshima Prefectural Hospital

Hiroshima

Hyogo College of Medicine

Hyogo

Hyogo Prefectural Kobe Children’s Hospital

Hyogo

Hyogo Prefectural Tsukaguchi Hospital

Hyogo

Ibaraki Children’s Hospital

Ibaraki

International University of Health and Welfare Hospital

Tochigi

Ishii Memorial Aizen-en Aizenbashi Hospital

Osaka

Ishikawa Medical Center for Maternal and Child Health

Ishikawa

Iwate Prefectural Central Hospital

Iwate

Japanese Red Cross Kumamoto Hospital

Kumamoto

Japanese Red Cross Kyoto Daiichi Hospital

Kyoto

Japanese Red Cross Society Fukaya Red Cross Hospital

Saitama

Japanese Red Cross Society Nagaoka Red Cross Hospital

Niigata

Japanese Red Cross Society Sendai Red Cross Hospital

Miyagi

Jichi Medical School

Tochigi

Juntendo University Hospital

Tokyo

Juntendo University Nerima Hosptal

Tokyo

Juntendo University Urayasu Hosptal

Chiba

Kagawa University Hospital

Kagawa

Kagoshima City Hospital

Kagoshima

Kagoshima University Graduate School of Medical and Dental Sciences

Kagoshima

Kakogawa West City Hospital

Hyogo

Kanagawa Children’s Medical Center

Kanagawa

Kanazawa Medical University

Ishikawa

Kansai Medical University Hirakata Hospital

Osaka

Kawaguchi Municipal Medical Center

Saitama

Kawasaki Medical School

Okayama

Kimitsu Chuo Hospital

Chiba

Kitakyushu Municipal Medical Center

Fukuoka

Kitano Hospital

Osaka

Kitasato University Hospital

kanagawa

Kochi Health Sciences Center

Kochi

Kumamoto City Hospital

Kumamoto

Kumamoto University Hospital

Kumamoto

Kurume University School of Medicine

Fukuoka

Kyorin University School of Medicine

Tokyo

Kyoto Prefectural University of Medicine

Kyoto

Kyushu University Hospital

Fukuoka

Matsudo City Hospital

Chiba

Mie University Faculty of Medicine

Mie

Miyazaki Prefectural Miyazaki Hospital

Miyazaki

Nagano Children’s Hospital

Nagano

Nagasaki University Hospital

Nagasaki

Nagoya City University Hospital

Aichi

Nagoya City West Medical Center

Aichi

Naha City Hospital

Okinawa

Nara Hospital Kinki University Faculty of Medicine

Nara

National Center for Child Health and Development

Tokyo

National Hospital Organization Kokura Medical Center

Fukuoka

National Hospital Organization Nagara Medical Center

Gifu

Niigata City General Hospital

Niigata

Niigata University Graduate School of Medicine and Dental Sciences

Niigata

Ohta Nishinouchi Hospital

Fukushima

Oita Prefectural Hospital

Oita

Okayama Medical Center

Okayama

Osaka City General Hospital

Osaka

Osaka Medical Center and Research Institute for Maternal and Child Health

Osaka

Osaka University Hospital

Osaka

Oumihachiman Community Medical Center

Shiga

Saga-ken Medical Centre Koseikan

Shiga

Saitama Children’s Medical Center

Saitama

Saitama City Hospital

Saitama

Saitama Medical Center

Saitama

Saitama Medical University Hospital

Saitama

Second Department of Surgery, Wakayama Medical University

Wakayama

Seirei Hamamatsu General Hospital

Shizuoka

Shimane University Hospital

Shimane

Shizuoka Children’s Hospital

Shizuoka

Social Medical Corporation BOKOI Tenshi Hospital

Hokkaido

St. Luke’s International Hospital

Tokyo

St. Marianna University Hospital

Kanagawa

St. Marianna University School of Medicine Yokohama City Seibu Hospital

Kanagawa

St. Mary’s Hospital

Fukuoka

Takatsuki General Hospital

Osaka

Tohoku University Hospital

Miyagi

Tokai University Hachioji Hospital

Tokyo

Tokai University Hospital

Kanagawa

Tokyo Metropolitan Children’s Medical Center

Tokyo

Tokyo Women’s Medical University

Tokyo

Tokyo Women’s Medical University Yachiyo Medical Center

Tokyo

Tsuchiura Kyodo General Hospital

Ibaraki

University of Tsukuba

Ibaraki

Yamaguchi Prefectural Grand Medical Center

Yamaguchi

Yamanashi Prefectural Central Hospital

Yamaguchi

Yodogawa Christian Hospital

Osaka

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Taguchi, T., Obata, S. & Ieiri, S. Current status of Hirschsprung’s disease: based on a nationwide survey of Japan. Pediatr Surg Int 33, 497–504 (2017). https://doi.org/10.1007/s00383-016-4054-3

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