Isolated Hypoganglionosis, Acquired

  • Satoshi Obata
  • Kosuke Kirino
  • Tomoaki TaguchiEmail author


Isolated hypoganglionosis (IH) is proposed to be one of the allied disorders of Hirschsprung’s disease (ADHD), which is rare and resembles the symptoms of Hirschsprung’s disease (HD), and associated with decreased numbers of intestinal ganglion cells [1]. Historically, the existence of this entity has been questioned [2, 3].



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. 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 (H23-042, H24-037, and H26-045)).

Ethical approval: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. And, for this type of study, formal consent was not required. This retrospective study was also approved by the ethics committee for clinical research of Kyushu University Hospital (No. 28-155).

Conflict of interest: None.


  1. 1.
    Tiffin ME, Chandler LR, Faber HK. Localized absence of the ganglion cells of the myenteric plexus in congenital megacolon. Am J Dis Child. 1940;59(5):1071–82.Google Scholar
  2. 2.
    Martucciello G, Pini Prato A, Puri P, Holschneider AM, Meier-Ruge W, Jasonni V, Tovar JA, Grosfeld JL. Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung’s disease and related neurocristopathies. J Pediatr Surg. 2005;40(10):1527–31. Scholar
  3. 3.
    Schäppi MG, Staiano A, Milla PJ, Smith VV, Dias JA, Heuschkel R, Husby S, Mearin ML, Papadopoulou A, Ruemmele FM, Vandenplas Y, Koletzko S. A practical guide for the diagnosis of primary enteric nervous system disorders. J Pediatr Gastroenterol Nutr. 2013;57(5):677–86. Scholar
  4. 4.
    Taguchi T, Masumoto K, Ieiri S, Nakatsuji T, Akiyoshi J. New classification of hypoganglionosis: congenital and acquired hypoganglionosis. J Pediatr Surg. 2006;41(12):2046–51. Scholar
  5. 5.
    Taguchi T, Ieiri S, Miyoshi K, Kohashi K, Oda Y, Kubota A, Watanabe Y, Matsufuji H, Fukuzawa M, Tomomasa T The incidence and outcome of allied disorders of Hirschsprung’s disease in Japan: results from a nationwide survey. Asian J Surg. 2015.
  6. 6.
    Watanabe Y, Kanamori Y, Uchida K, Taguchi T. Isolated hypoganglionosis: results of a nationwide survey in Japan. Pediatr Surg Int. 2013;29(11):1127–30. Scholar
  7. 7.
    Friedmacher F, Puri P. Classification and diagnostic criteria of variants of Hirschsprung’s disease. Pediatr Surg Int. 2013;29(9):855–72. Scholar
  8. 8.
    Iantorno G, Bassotti G, Kogan Z, Lumi CM, Cabanne AM, Fisogni S, Varrica LM, Bilder CR, Munoz JP, Liserre B, Morelli A, Villanacci V. The enteric nervous system in chagasic and idiopathic megacolon. Am J Surg Pathol. 2007;31(3):460–8. Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Satoshi Obata
    • 1
    • 2
  • Kosuke Kirino
    • 1
    • 2
  • Tomoaki Taguchi
    • 1
    • 2
    Email author
  1. 1.The Japanese Study Group for Allied Disorders of Hirschsprung’s DiseaseKyushu UniversityFukuokaJapan
  2. 2.Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical SciencesKyushu UniversityFukuokaJapan

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