Skip to main content

Advertisement

SpringerLink
Log in

A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood

  • Original Article
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Background

Hirschsprung’s disease (HD) is characterised by an absence of ganglion cells in the distal bowel, beginning at the internal sphincter and extending proximally to varying distances. It is usually diagnosed in the newborn period, with usual presentation of delayed passage of meconium and abdominal distension, with or without bilious vomiting. HD in adults is rare and is thus often undiagnosed or misdiagnosed. The purpose of this meta-analysis was to review the presentation, treatment and clinical outcome of HD presenting after childhood.

Methods

A systematic review and meta-analysis of all cases of HD presenting after childhood in the English literature was performed from 1950 to 2009. Detailed information regarding demographics, clinical presentation and methods of diagnosis, surgical procedure, complications and the outcome at time of follow up was recorded.

Results

There were 490 cases of HD presenting after childhood in the English literature, 341 (69.5%) males, 129 (26.4%) females and 20 (4.1%) cases where gender was not specified. As much as 390 (79.6%) were confined to the rectum, 60 (12.3%) had recto-sigmoid disease, 4 (0.8%) had disease extending to the descending colon and there were 2 (0.4%) cases that extended to the transverse colon and 2 (0.4%) cases of total colonic disease. The extent of disease was not specified in the remaining 32 (6.5%) cases. A total of 49 (10%) patients had the Swenson procedure, 231 (47.2%) patients had the Duhamel procedure, 40 (8.2%) patients had the Soave procedure, 45 (9.2%) patients had a myectomy only, 3 (0.6%) patients had a myectomy combined with colectomy, 14 (2.9%) patients had a myectomy combined with anterior resection. As much as 26 (5.3%) patients had a lower anterior resection (LAR), 28 (5.7%) patients had LAR combined with colectomy, 10 (2%) patients had a colectomy, 1 (0.2%) patient had an anopexy and 4 (0.9%) patients had a colostomy only. A total of 13 (2.7%) patients refused surgery and managed with conservative treatment, and in 25 (5.1%) patients, the specific procedure was not identified. There were 2 (0.4%) deaths reported; 1 patient died prior to surgery due to colonic perforation and sepsis and the 2nd patient died post-operatively due to appendix stump dehiscence, peritonitis and sepsis. The time of follow up ranged from 1 to 25 years and all, but 6 (1.3%) had a very good clinical outcome.

Conclusions

Hirschsprung’s disease should be considered in patients who have had chronic constipation since birth. This review suggests that the vast majority of patients in whom HD is diagnosed after childhood have normal bowel function after pull-through surgery.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Chen F et al (2006) Hirschsprung’s disease in a young adult: report of a case and review of the literature. Ann Diagn Pathol 10(6):347–351

    Article  PubMed  Google Scholar 

  2. Rosin JD, Bargen JA, Waugh JM (1950) Congenital megacolon of a man 54 years of age: report of case. Proc Staff Meet Mayo Clin 25(26):710–715

    CAS  PubMed  Google Scholar 

  3. Grove K, Ahlawat SK (2009) Hirschsprung disease in adults. South Med J 102(2):127–128

    Google Scholar 

  4. Miyamoto M et al (2005) Hirschsprung’s disease in adults: report of a case and review of the literature. J Nippon Med Sch 72(2):113–120

    Article  PubMed  Google Scholar 

  5. Barnes PR et al (1986) Hirschsprung’s disease and idiopathic megacolon in adults and adolescents. Gut 27(5):534–541

    Article  CAS  PubMed  Google Scholar 

  6. Vorobyov GI, Achkasov SI, Biryukov OM (2009) Clinical features diagnostics and treatment of Hirschsprung’s disease in adults. Colorectal Dis. doi:10.1111/j.1463-1318.2009.02031.x

  7. Hiatt RB (1951) The surgical treatment of congenital megacolon. Ann Surg 133(3):321–329

    Article  CAS  PubMed  Google Scholar 

  8. Kempton JJ (1954) Hirschsprung’s disease in a man aged 23. Proc R Soc Med 47(7):545–546

    CAS  PubMed  Google Scholar 

  9. Rich AJ, Lennard TW, Wilsdon JB (1983) Hirschsprung’s disease as a cause of chronic constipation in the elderly. Br Med J (Clin Res Ed) 287(6407):1777–1778

    Article  CAS  Google Scholar 

  10. Kim HJ et al (2008) Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation. Radiology 247(2):428–434

    Article  PubMed  Google Scholar 

  11. Meier-Ruge W and Bruder E (2008) Histopathological diagnosis and differential diagnosis of Hirschsprung’s disease. In: Holschneider A, Puri P (eds) Hirschsprung’s disease and allied disorders. Springer, pp 185–186

  12. Langer JC et al (1999) Transanal one-stage soave procedure for infants with Hirschsprung’s disease. J Pediatr Surg 34(1):148–152

    Article  CAS  PubMed  Google Scholar 

  13. Duhamel B (1956) New operation for congenital megacolon: retrorectal and transanal lowering of the colon, and its possible application to the treatment of various other malformations. Presse Med 64(95):2249–2250

    CAS  PubMed  Google Scholar 

  14. Duhamel B (1964) Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung’s disease. Dis Colon Rectum 7:455–458

    Article  CAS  PubMed  Google Scholar 

  15. Steichen FM, Talbert JL, Ravitch MM (1968) Primary side-to-side colorectal anastomosis in the Duhamel operation for Hirschsprung’s disease. Surgery 64(2):475–483

    CAS  PubMed  Google Scholar 

  16. Elliot MS, Todd IP (1985) Adult Hirschsprung’s disease: results of the Duhamel procedure. Br J Surg 72(11):884–885

    Article  CAS  PubMed  Google Scholar 

  17. Puri P (2008) Swenson’s Procedure. In: Holschneider A, Puri P (eds) Hirschsprung’s disease and allied disorders. Springer, pp 329–330

  18. Soave F (1964) A new surgical technique for treatment of Hirschsprung’s disease. Surgery 56:1007–1014

    CAS  PubMed  Google Scholar 

  19. Boley SJ (1968) An endorectal pull-through operation with primary anastomosis for Hirchsprung’s disease. Surg Gynecol Obstet 127(2):353–357

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Ireland

    Reshma Doodnath & Prem Puri

  2. School of Medicine and Medical Science and Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin, Ireland

    Reshma Doodnath & Prem Puri

Authors
  1. Reshma Doodnath
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Prem Puri
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Prem Puri.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Doodnath, R., Puri, P. A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood. Pediatr Surg Int 26, 1107–1110 (2010). https://doi.org/10.1007/s00383-010-2694-2

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-010-2694-2

Keywords

Search

Navigation