• Tatsuru KajiEmail author
  • Waka Yamada
  • Tokuro Baba
  • Seiro Machigashira


Hirschsprung’s disease has been usually classified by the extent of the aganglionic segment. However, there are no unified classification criteria in the world yet. Generally, it is classified as “short-segment aganglionosis” when the aganglionic segment extends to the sigmoid colon; especially, the aganglionic segment extends only to the rectum under peritoneal reflection, which is termed as “rectal aganglionosis” (Fig. 8.1a), and extends to the sigmoid colon, which is termed as “rectosigmoid aganglionosis” (Fig. 8.1b); “long-segment aganglionosis” when the aganglionic segment extends beyond the sigmoid colon to the splenic flexure or transverse colon or ascending colon (Fig. 8.1c); and “total colon aganglionosis” when the aganglionic segment extends beyond the cecum to the distal ileum (Fig. 8.1d), and “extensive aganglionosis” when the aganglionic segment extends to the jejunum (Fig. 8.1e). Almost 80% of the patients with typical Hirschsprung’s disease have aganglionic segment limited to the rectum and the sigmoid colon [1–4]. Previous studies presented that rectal aganglionosis accounts for 5.7–14.6%, rectosigmoid aganglionosis accounts for 64.9–67.0%, long-segment aganglionosis accounts for 12.0–26.0%, and total colon aganglionosis accounts for 2.8–12.0% (Table 8.1) [1, 5–10]. Almost all studies were institutional experiences in North America or Europe; however, only Singh et al. presented the nationwide survey of Australia from 1997 to 2000 [10]. Table 8.2 shows the nationwide survey of Japan for four series from 1978 [11–13]. Total patients’ number has been gradually decreased followed by the decrease of birth. Recent study presented that the percentage of rectal aganglionosis, rectosigmoid aganglionosis, long-segment aganglionosis, total colon aganglionosis, and extensive aganglionosis presented 11.1%, 63.1%, 14.9%, 7.9%, and 3.1%, respectively [13].


Short-segment aganglionosis Long-segment aganglionosis Total colon aganglionosis Extensive aganglionosis 


  1. 1.
    Menezes M, Corbally M, Puri P. Long-term results of bowel function after treatment for Hirschsprung’s disease: a 29-year review. Pediatr Surg Int. 2006;22(12):987–90.CrossRefPubMedGoogle Scholar
  2. 2.
    Bruder E, Meier-Ruge WA. Twenty years diagnostic competence center for Hirschsprung’s disease in Basel. Chirurg. 2010;81(6):572–6.CrossRefPubMedGoogle Scholar
  3. 3.
    Teitelbaum DH, Cilley RE, Sherman NJ, Bliss D, Uitvlugt ND, Renaud EJ, Kirstioglu I, Bengston T, Coran AG. A decade of experience with the primary pull-through for Hirschsprung disease in the newborn period: a multicenter analysis of outcomes. Ann Surg. 2000;232(3):372–80.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Ryan ET, Ecker JL, Christakis NA, Folkman J. Hirschsprung’s disease: associated abnormalities and demography. J Pediatr Surg. 1992;27(1):76–81.CrossRefPubMedGoogle Scholar
  5. 5.
    Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg. 1973;8(5):587–94.CrossRefPubMedGoogle Scholar
  6. 6.
    Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung’s disease – a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg. 1979;14(5):588–97.CrossRefPubMedGoogle Scholar
  7. 7.
    Orr JD, Scobie WG. Presentation and incidence of Hirschsprung’s disease. Br Med J (Clin Res Ed). 1983;287(6406):1671.CrossRefGoogle Scholar
  8. 8.
    Sherman JO, Snyder ME, Weitzman JJ, Jona JZ, Gillis DA, O’Donnell B, Carcassonne M, Swenson O. A 40-year multinational retrospective study of 880 Swenson procedures. J Pediatr Surg. 1989;24(8):833–8.CrossRefPubMedGoogle Scholar
  9. 9.
    Klein MD, Philippart AI. Hirschsprung’s disease: three decades’ experience at a single institution. J Pediatr Surg. 1993;28(10):1291–3.CrossRefPubMedGoogle Scholar
  10. 10.
    Singh SJ, Croaker GD, Manglick P, Wong CL, Athanasakos H, Elliott E, Cass D. Hirschsprung’s disease: the Australian Paediatric Surveillance Unit’s experience. Pediatr Surg Int. 2003;19(4):247–50.CrossRefPubMedGoogle Scholar
  11. 11.
    Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg. 1984;199(4):400–5.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung’s disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatr Surg. 2005;40(1):197–201.CrossRefPubMedGoogle Scholar
  13. 13.
    Taguchi T, Obata S, Ieiri S. Current status of Hirschsprung’s disease: based on a nationwide survey of Japan. Pediatr Surg Int. 2017;33(4):497–504.CrossRefPubMedGoogle Scholar
  14. 14.
    Davidson M, Bauer CH. Studies of distal colonic motility in children. IV. Achalasia of the distal rectal segment despite presence of ganglia in the myenteric plexuses of this area. Pediatrics. 1958;21(5):746–61.PubMedGoogle Scholar
  15. 15.
    Neilson IR, Yazbeck S. Ultrashort Hirschsprung’s disease: myth or reality. J Pediatr Surg. 1990;25(11):1135–8.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Tatsuru Kaji
    • 1
    Email author
  • Waka Yamada
    • 1
  • Tokuro Baba
    • 1
  • Seiro Machigashira
    • 1
  1. 1.Department of Pediatric Surgery, Research Field in Medicine and Health Sciences, Medical and Dental Sciences Area, Research and Education AssemblyKagoshima UniversityKagoshimaJapan

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