Skip to main content
SpringerLink
Log in

Long-Term Outcome and Quality of Life After Treatment of Hirschsprung’s Disease

  • Chapter
  • First Online:
Hirschsprung's Disease and Allied Disorders

Abstract

While the treatment results of Hirschsprung’s disease have improved, significant attention is brought to long-term functional, psychosocial, and quality of life issues in children and adults with Hirschsprung’s disease. The overall long-term functional outcome expectancy is relatively optimistic; although many patients have some degree of bowel dysfunction and continence defect, they are able to stay socially continent and report a good quality of life. In contrast, some patients need special bowel management programs to achieve social continence, or some are even left with persistent incontinence and significant psychosocial problems needing additional medical attention. Other long-term problems such as enterocolitis may have a significant impact on long-term functional outcome and quality of life. In this chapter, the long-term outcomes of Hirschsprung’s disease regarding bowel function and continence, enterocolitis, and quality of life are discussed. Furthermore, long-term issues related to specific patient groups, such as syndromic patients and total colonic aganglionosis, are reviewed.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 99.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 129.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. Bowel function and quality of life after transanal endorectal pull-through for hirschsprung disease: controlled outcomes up to adulthood. Ann Surg. 2017;265(3):622–9.

    Article  Google Scholar 

  2. Granstrom AL, Danielson J, Husberg B, Nordenskjold A, Wester T. Adult outcomes after surgery for hirschsprung’s disease: evaluation of bowel function and quality of life. J Pediatr Surg. 2015;50(11):1865–9.

    Article  Google Scholar 

  3. Wester T, Granstrom AL. Hirschsprung disease-bowel function beyond childhood. Semin Pediatr Surg. 2017;26(5):322–7.

    Article  Google Scholar 

  4. Bjørnland K, Pakarinen M, Stenstrøm P, et al. A nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid hirschsprung disease. J Pediatr Surg. 2017;52(9):1458–64.

    Article  Google Scholar 

  5. Khalil M. Long-term health-related quality of life for patients with hirschsprung’s disease at 5 years after transanal endorectal pull-through operation. Qual Life Res. 2015;24(11):2733–8.

    Article  Google Scholar 

  6. Catto-Smith AG, Trajanovska M, Taylor RG. Long-term continence after surgery for hirschsprung’s disease. J Gastroenterol Hepatol. 2007;22(12):2273–82.

    Article  Google Scholar 

  7. Bai Y, Chen H, Hao J, Huang Y, Wang W. Long-term outcome and quality of life after the swenson procedure for hirschsprung’s disease. J Pediatr Surg. 2002;37(4):639–42.

    Article  Google Scholar 

  8. Moore SW, Albertyn R, Cywes S. Clinical outcome and long-term quality of life after surgical correction of hirschsprung’s disease. J Pediatr Surg. 1996;31(11):1496–502.

    Article  CAS  Google Scholar 

  9. Heij HA, de Vries X, Bremer I, Ekkelkamp S, Vos A. Long-term anorectal function after duhamel operation for hirschsprung’s disease. J Pediatr Surg. 1995;30(3):430–2.

    Article  CAS  Google Scholar 

  10. Teitelbaum D, Drongowski R, Chamberlain J, Coran A. Long-term stooling patterns in infants undergoing primary endorectal pull-through for hirschsprung’s disease. J Pediatr Surg. 1997;32(7):1049–52.

    Article  CAS  Google Scholar 

  11. Kim AC, Langer JC, Pastor AC, et al. Endorectal pull-through for hirschsprung’s disease-a multicenter, long-term comparison of results: transanal vs transabdominal approach. J Pediatr Surg. 2010;45(6):1213–20.

    Article  Google Scholar 

  12. Yang L, Tang S, Cao G, et al. Transanal endorectal pull-through for hirschsprung’s disease using long cuff dissection and short V-shaped partially resected cuff anastomosis: early and late outcomes. Pediatr Surg Int. 2012;28(5):515–21.

    Article  Google Scholar 

  13. Mills JL, Konkin DE, Milner R, Penner JG, Langer M, Webber EM. Long-term bowel function and quality of life in children with hirschsprung’s disease. J Pediatr Surg. 2008;43(5):899–905.

    Article  Google Scholar 

  14. Yanchar NL, Soucy P. Long-term outcome after hirschsprung’s disease: patients’ perspectives. J Pediatr Surg. 1999;34(7):1152–60.

    Article  CAS  Google Scholar 

  15. Diseth TH, Bjornland K, Novik TS, Emblem R. Bowel function, mental health, and psychosocial function in adolescents with hirschsprung’s disease. Arch Dis Child. 1997;76(2):100–6.

    Article  CAS  Google Scholar 

  16. Heikkinen M, Rintala R, Luukkonen P. Long-term anal sphincter performance after surgery for hirschsprung’s disease. J Pediatr Surg. 1997;32(10):1443–6.

    Article  CAS  Google Scholar 

  17. Bjornland K, Diseth TH, Emblem R. Long-term functional, manometric, and endosonographic evaluation of patients operated upon with the duhamel technique. Pediatr Surg Int. 1998;13(1):24–8.

    Article  CAS  Google Scholar 

  18. Baillie CT, Kenny SE, Rintala RJ, Booth JM, Lloyd DA. Long-term outcome and colonic motility after the duhamel procedure for hirschsprung’s disease. J Pediatr Surg. 1999;34(2):325–9.

    Article  CAS  Google Scholar 

  19. Jarvi K, Koivusalo A, Rintala RJ, Pakarinen MP. Anorectal manometry with reference to operative rectal biopsy for the diagnosis/exclusion of hirschsprung’s disease in children under 1 year of age. Int J Colorectal Dis. 2009;24(4):451–4.

    Article  Google Scholar 

  20. Rintala RJ, Pakarinen MP. Long-term outcomes of hirschsprung’s disease. Semin Pediatr Surg. 2012;21(4):336–43.

    Article  Google Scholar 

  21. Collins L, Collis B, Trajanovska M, et al. Quality of life outcomes in children with hirschsprung disease. J Pediatr Surg. 2017;52(12):2006–10.

    Article  Google Scholar 

  22. Tannuri AC, Ferreira MA, Mathias AL, Tannuri U. Long-term results of the duhamel technique are superior to those of the transanal pullthrough: a study of fecal continence and quality of life. J Pediatr Surg. 2017;52(3):449–53.

    Article  Google Scholar 

  23. Ludman L, Spitz L, Tsuji H, Pierro A. Hirschsprung’s disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis. Arch Dis Child. 2002;86(5):348–51.

    Article  CAS  Google Scholar 

  24. Reding R, de Ville de Goyet J, Gosseye S, et al. Hirschsprung’s disease: a 20-year experience. J Pediatr Surg. 1997;32(8):1221–5.

    Article  CAS  Google Scholar 

  25. Jarvi K, Laitakari EM, Koivusalo A, Rintala RJ, Pakarinen MP. Bowel function and gastrointestinal quality of life among adults operated for hirschsprung disease during childhood: a population-based study. Ann Surg. 2010;252(6):977–81.

    Article  Google Scholar 

  26. Conway SJ, Craigie RJ, Cooper LH, et al. Early adult outcome of the duhamel procedure for left-sided hirschsprung disease – a prospective serial assessment study. J Pediatr Surg. 2007;42(8):1429–32.

    Article  Google Scholar 

  27. Frykman PK, Short SS. Hirschsprung-associated enterocolitis: prevention and therapy. Semin Pediatr Surg. 2012;21(4):328–35.

    Article  Google Scholar 

  28. Swenson O, Sherman JO, Fisher JH, Cohen E. The treatment and postoperative complications of congenital megacolon: a 25 year followup. Ann Surg. 1975;182(3):266–73.

    Article  CAS  Google Scholar 

  29. Pini Prato A, Rossi V, Avanzini S, Mattioli G, Disma N, Jasonni V. Hirschsprung’s disease: what about mortality? Pediatr Surg Int. 2011;27(5):473–8.

    Article  Google Scholar 

  30. Friedmacher F, Puri P. Hirschsprung’s disease associated with down syndrome: a meta-analysis of incidence, functional outcomes and mortality. Pediatr Surg Int. 2013;29(9):937–46.

    Article  Google Scholar 

  31. Wildhaber BE, Teitelbaum DH, Coran AG. Total colonic hirschsprung’s disease: a 28-year experience. J Pediatr Surg. 2005;40(1):203–6; discussion 206.

    Article  Google Scholar 

  32. Menezes M, Pini Prato A, Jasonni V, Puri P. Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review. J Pediatr Surg. 2008;43(9):1696–9.

    Article  Google Scholar 

  33. Dong Q, Li G, Dong J. Identification of risk factors for postoperative recurrent hirschsprung associated enterocolitis. J Pediatr Surg. 2018.

    Google Scholar 

  34. Nakamura H, Lim T, Puri P. Inflammatory bowel disease in patients with hirschsprung’s disease: a systematic review and meta-analysis. Pediatr Surg Int. 2018;34(2):149–54.

    Article  CAS  Google Scholar 

  35. Nakamura H, Tomuschat C, Coyle D, O’Donnel A, Lim T, Puri P. Altered goblet cell function in hirschsprung’s disease. Pediatr Surg Int. 2018;34(2):121–8.

    Article  Google Scholar 

  36. Prideaux L, Kang S, Wagner J, et al. Impact of ethnicity, geography, and disease on the microbiota in health and inflammatory bowel disease. Inflamm Bowel Dis. 2013;19(13):2906–18.

    Article  Google Scholar 

  37. Frykman P, Nordenskjöld A, Kawaguchi A, et al. Characterization of bacterial and fungal microbiome in children with hirschsprung disease with and without a history of enterocolitis: a multicenter study. PLoS One. 2014;10(4):e0124172.

    Article  Google Scholar 

  38. Neuvonen MI, Korpela K, Kyrklund K, Rintala RJ, Pakarinen MP. Intestinal microbiota in hirschsprung disease. J Pediatr Gastroenterol Nutr. 2018; epub ahead of print.

    Google Scholar 

  39. Sherman JO, Snyder ME, Weitzman JJ, et al. A 40-year multinational retrospective study of 880 swenson procedures. J Pediatr Surg. 1989;24(8):833–8.

    Article  CAS  Google Scholar 

  40. Lof Granstrom A, Amin L, Arnell H, Wester T. Increased risk of inflammatory bowel disease in a population-based cohort study of patients with hirschsprung disease. J Pediatr Gastroenterol Nutr. 2018;66(3):398–401.

    Article  Google Scholar 

  41. Levin DN, Marcon MA, Rintala RJ, Jacobson D, Langer JC. Inflammatory bowel disease manifesting after surgical treatment for hirschsprung disease. J Pediatr Gastroenterol Nutr. 2012;55(3):272–7.

    Article  Google Scholar 

  42. Marquez TT, Acton RD, Hess DJ, Duval S, Saltzman DA. Comprehensive review of procedures for total colonic aganglionosis. J Pediatr Surg. 2009;44(1):257–65; discussion 265.

    Article  Google Scholar 

  43. Laughlin DM, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int. 2012;28(8):773–9.

    Article  Google Scholar 

  44. Roorda D, Witvliet MJ, Wellens LM, et al. Long-term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands. Colorectal Dis. 2018;20(8):719–26.

    Article  CAS  Google Scholar 

  45. Escobar MA, Grosfeld JL, West KW, et al. Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg. 2005;40(6):955–61.

    Article  Google Scholar 

  46. Hoehner JC, Ein SH, Shandling B, Kim PC. Long-term morbidity in total colonic aganglionosis. J Pediatr Surg. 1998;33(7):961–5; discussion 965.

    Article  CAS  Google Scholar 

  47. Urla C, Lieber J, Obermayr F, et al. Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome. BMC Surg. 2018;18(1):58–018.

    Article  Google Scholar 

  48. Quinn FM, Surana R, Puri P. The influence of trisomy 21 on outcome in children with hirschsprung’s disease. J Pediatr Surg. 1994;29(6):781–3.

    Article  CAS  Google Scholar 

  49. Ahola JA, Koivusalo A, Sairanen H, Jokinen E, Rintala RJ, Pakarinen MP. Increased incidence of hirschsprung’s disease in patients with hypoplastic left heart syndrome – a common neural crest-derived etiology? J Pediatr Surg. 2009;44(7):1396–400.

    Article  Google Scholar 

  50. Makitie O, Heikkinen M, Kaitila I, Rintala R. Hirschsprung’s disease in cartilage-hair hypoplasia has poor prognosis. J Pediatr Surg. 2002;37(11):1585–8.

    Article  Google Scholar 

  51. Asai N, Jijiwa M, Enomoto A, et al. RET receptor signaling: dysfunction in thyroid cancer and hirschsprung’s disease. Pathol Int. 2006;56(4):164–72.

    Article  CAS  Google Scholar 

  52. Amiel J, Lyonnet S. Hirschsprung disease, associated syndromes, and genetics: a review. J Med Genet. 2001;38(11):729–39.

    Article  CAS  Google Scholar 

  53. Pakarinen MP, Rintala RJ, Koivusalo A, Heikkinen M, Lindahl H, Pukkala E. Increased incidence of medullary thyroid carcinoma in patients treated for hirschsprung’s disease. J Pediatr Surg. 2005;40(10):1532–4.

    Article  Google Scholar 

  54. Virtanen VB, Pukkala E, Kivisaari R, et al. Thyroid cancer and co-occurring RET mutations in hirschsprung disease. Endocr Relat Cancer. 2013;20(4):595–602.

    Article  CAS  Google Scholar 

  55. Hartman EE, Oort FJ, Aronson DC, Sprangers MA. Quality of life and disease-specific functioning of patients with anorectal malformations or hirschsprung’s disease: a review. Arch Dis Child. 2011;96(4):398–406.

    Article  CAS  Google Scholar 

  56. Hartman EE, Oort FJ, Sprangers MA, et al. Factors affecting quality of life of children and adolescents with anorectal malformations or hirschsprung disease. J Pediatr Gastroenterol Nutr. 2008;47(4):463–71.

    Article  Google Scholar 

  57. Onishi S, Nakame K, Kaji T, et al. The bowel function and quality of life of hirschsprung disease patients who have reached 18 years of age or older - the long-term outcomes after un... - PubMed - NCBI J Pediatr Surg. 2017;52(12):2001–5.

    Google Scholar 

  58. Heikkinen M, Rintala R, Louhimo I. Bowel function and quality of life in adult patients with operated hirschsprung’s disease. Pediatr Surg Int. 1995;10:342–4.

    Article  Google Scholar 

  59. Ieiri S, Nakatsuji T, Akiyoshi J, et al. Long-term outcomes and the quality of life of hirschsprung disease in adolescents who have reached 18 years or older – a 47-year single-institute experience. J Pediatr Surg. 2010;45(12):2398–402.

    Article  Google Scholar 

  60. Gunnarsdottir A, Sandblom G, Arnbjornsson E, Larsson LT. Quality of life in adults operated on for hirschsprung disease in childhood. J Pediatr Gastroenterol Nutr. 2010;51(2):160–6.

    Article  Google Scholar 

  61. Hartman EE, Oort FJ, Visser MR, et al. Explaining change over time in quality of life of adult patients with anorectal malformations or hirschsprung’s disease. Dis Colon Rectum. 2006;49(1):96–103.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland

    Annika Mutanen, Mikko P. Pakarinen & Risto J. Rintala

Authors
  1. Annika Mutanen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mikko P. Pakarinen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Risto J. Rintala
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Risto J. Rintala .

Editor information

Editors and Affiliations

  1. National Children’s Research Centre, Our Lady’s Childrens Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Mutanen, A., Pakarinen, M.P., Rintala, R.J. (2019). Long-Term Outcome and Quality of Life After Treatment of Hirschsprung’s Disease. In: Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-15647-3_32

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-15647-3_32

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-15646-6

  • Online ISBN: 978-3-030-15647-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation