Hirschsprung-Associated Enterocolitis

  • Farokh R. DemehriEmail author
  • Ihab F. Halaweish
  • Arnold G. Coran
  • Daniel H. Teitelbaum
Living reference work entry


Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung’s disease (HD). Presenting either before or after definitive surgery for HD, HAEC may manifest clinically as abdominal distension and explosive diarrhea, along with emesis, fever, lethargy, and even shock. The pathogenesis of HAEC, the subject of ongoing research, likely involves a complex interplay between a dysfunctional enteric nervous system, abnormal mucin production, insufficient immunoglobulin secretion, and unbalanced intestinal microflora. Early recognition of HAEC and preventative practices, such as rectal washouts following a pull-through, can lead to improved outcomes. Treatment strategies for acute HAEC include timely resuscitation, colonic decompression, and antibiotics. Recurrent or persistent HAEC requires evaluation for mechanical obstruction or residual aganglionosis and may require surgical treatment with posterior myotomy/myectomy (POMM) or redo pull-through. This chapter describes the incidence, pathogenesis, treatment, and preventative strategies in the management of HAEC.


Hirschsprung’s disease Enterocolitis HAEC Pathogenesis Prevention Treatment 


  1. Akkary S, Sahwy E, et al. A histochemical study of the mucosubstances of the colon in cases of Hirschsprung’s disease with and without enterocolitis. J Pediatr Surg. 1981;16(5):664–8.CrossRefPubMedGoogle Scholar
  2. Aslam A, Spicer RD, et al. Turnover of radioactive mucin precursors in the colon of patients with Hirschsprung’s disease correlates with the development of enterocolitis. J Pediatr Surg. 1998;33(1):103–5.CrossRefPubMedGoogle Scholar
  3. Aslam A, Spicer RD, et al. Histochemical and genetic analysis of colonic mucin glycoproteins in Hirschsprung’s disease. J Pediatr Surg. 1999;34(2):330–3.CrossRefPubMedGoogle Scholar
  4. Austin KM. The pathogenesis of Hirschsprung’s disease-associated enterocolitis. Semin Pediatr Surg. 2012;21(4):319–27.CrossRefPubMedGoogle Scholar
  5. Bagwell CE, Langham Jr M, et al. Pseudomembranous colitis following resection for Hirschsprung’s disease. J Pediatr Surg. 1992;27(10):1261–4.CrossRefPubMedGoogle Scholar
  6. Belzer C, de Vos W. Microbes inside – from diversity to function: the case of Akkermansia. ISME J. 2012;6:1449–58.CrossRefPubMedPubMedCentralGoogle Scholar
  7. Bill AH, Chapman N. The enterocolitis of Hirschsprung’s disease: its natural history and treatment. Am J Surg. 1962;103:70–4.CrossRefGoogle Scholar
  8. Caniano DA, Teitelbaum DH, et al. The piebald-lethal murine strain: investigation of the cause of early death. J Pediatr Surg. 1989;24(9):906–10.CrossRefPubMedGoogle Scholar
  9. Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung’s disease. Am J Surg. 2000;180(5):382–7.CrossRefPubMedGoogle Scholar
  10. Corman ML. Classic articles in colonic and rectal surgery. Am Soc Colon Rectal Surg. 1981;24(5):408–10.Google Scholar
  11. De Filippo C, Cavalieri D, et al. Impact of diet in shaping gut microbiota revealed by a comparative study in children from Europe and rural Africa. Proc Natl Acad Sci USA. 2010;107(33):14691–6.CrossRefPubMedPubMedCentralGoogle Scholar
  12. Demehri FR, Halaweish IF, et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int. 2013;29(9):873–81.CrossRefPubMedGoogle Scholar
  13. Elhalaby E, Coran A, et al. Enterocolitis associated with Hirschsprung’s disease: a clinical-radiological characterization based on 168 patients. J Pediatr Surg. 1995a;30(1):76–83.CrossRefPubMedGoogle Scholar
  14. Elhalaby EA, Teitelbaum DH, et al. Enterocolitis associated with Hirschsprung’s disease: a clinical histopathological correlative study. J Pediatr Surg. 1995b;30(7):1023–6; discussion 1026–7.Google Scholar
  15. El-Sawaf M, Siddiqui S, et al. Probiotic prophylaxis after pullthrough for Hirschsprung disease to reduce incidence of enterocolitis: a prospective, randomized, double-blind, placebo-controlled, multicenter trial. J Pediatr Surg. 2013;48(1):111–7.CrossRefPubMedGoogle Scholar
  16. Engum SA, Grosfeld JL. Long-term results of treatment of Hirschsprung’s disease. Semin Pediatr Surg. 2004;13(4):273–85.CrossRefPubMedGoogle Scholar
  17. Estevao-Costa J, Carvalho JL, et al. Risk factors for the development of enterocolitis after pull-through for Hirschsprung’s disease. J Pediatr Surg. 1999;34(10):1581–2. [comment].CrossRefPubMedGoogle Scholar
  18. Fisher JH, Swenson O. Hirschsprung’s disease during infancy. Surg Clin North Am. 1956;103:1511–5.Google Scholar
  19. Frykman PK, Nordenskjöld 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. 2015;10(4):e0124172.CrossRefPubMedPubMedCentralGoogle Scholar
  20. Fujimoto T, Reen DJ, et al. Inflammatory response in enterocolitis in the piebald lethal mouse model of Hirschsprung’s disease. Pediatr Res. 1988;24(2):152–5.CrossRefPubMedGoogle Scholar
  21. Hackam DJ, Filler RM, et al. Enterocolitis after the surgical treatment of Hirschsprung’s disease: risk factors and financial impact. J Pediatr Surg. 1998;33(6):830–3. [see comment].CrossRefPubMedGoogle Scholar
  22. Hardy SP, Bayston R, et al. Prolonged carriage of Clostridium difficile in Hirschsprung’s disease. Arch Dis Child. 1993;69(2):221–4.CrossRefPubMedPubMedCentralGoogle Scholar
  23. Haricharan RN, Seo JM, et al. Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not. J Pediatr Surg. 2008;43(6):1115–23.CrossRefPubMedGoogle Scholar
  24. Heikkinen M, Lindahl H, et al. Long-term outcome after internal sphincter myectomy for internal sphincter achalasia. Pediatr Surg Int. 2005;21(2):84–7.CrossRefPubMedGoogle Scholar
  25. Imamura A, Puri P, et al. Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut. 1992;33(6):801–6.CrossRefPubMedPubMedCentralGoogle Scholar
  26. Kaul A, Hyman P, et al. Colonic hyperactivity results in frequent fecal soiling in a subset of children after surgery for Hirschsprung disease. J Pediatr Gastroenterol Nutr. 2011;52(4):433–6.CrossRefPubMedGoogle Scholar
  27. Lawal TA, Chatoorgoon K, et al. Redo pull-through in Hirschsprung’s [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel. J Pediatr Surg. 2011;46(2):342–7.CrossRefPubMedGoogle Scholar
  28. Levitt MA, Dickie B, et al. Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg. 2010;19(2):146–53.CrossRefPubMedGoogle Scholar
  29. Li Y, Poroyko V, et al. Characterization of intestinal microbiomes of Hirschsprung’s disease patients with or without enterocolitis using Illumina-MiSeq high-throughput sequencing. PLoS One. 2016;11(9):e0162079.CrossRefPubMedPubMedCentralGoogle Scholar
  30. Lui VC, Li L, et al. CDX-1 and CDX-2 are expressed in human colonic mucosa and are down-regulated in patients with Hirschsprung’s disease associated enterocolitis. Biochim Biophys Acta. 2001;1537(2):89–100.CrossRefPubMedGoogle Scholar
  31. Marty TL, Matlak ME, et al. Unexpected death from enterocolitis after surgery for Hirschsprung’s disease. Pediatrics. 1995a;96(1 Pt 1):118–21.PubMedGoogle Scholar
  32. Marty TL, Seo T, et al. Rectal irrigations for the prevention of postoperative enterocolitis in Hirschsprung’s disease. J Pediatr Surg. 1995b;30(5):652–4.CrossRefPubMedGoogle Scholar
  33. Mattar A, Drongowski R, et al. MUC-2 mucin production in Hirschsprung’s disease: possible association with enterocolitis development. J Pediatr Surg. 2003;38(3):417–21.CrossRefPubMedGoogle Scholar
  34. Messina M, Amato G, et al. Topical application of isosorbide dinitrate in patients with persistent constipation after pull-through surgery for Hirschsprung’s disease. Eur J Pediatr Surg. 2007;17(1):62–5.CrossRefPubMedGoogle Scholar
  35. Minkes RK, Langer JC. A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung’s disease. J Pediatr Surg. 2000;35(12):1733–6.CrossRefPubMedGoogle Scholar
  36. Miyahara K, Kato Y, et al. Neuronal immaturity in normoganglionic colon from cases of Hirschsprung disease, anorectal malformation, and idiopathic constipation. J Pediatr Surg. 2009;44(12):2364–8.CrossRefPubMedGoogle Scholar
  37. Moore SW, Millar AJ, et al. Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung’s patient. J Pediatr Surg. 1994;29(1):106–11.CrossRefPubMedGoogle Scholar
  38. Moore SW, Sidler D, et al. The ITGB2 immunomodulatory gene (CD18), enterocolitis, and Hirschsprung’s disease. J Pediatr Surg. 2008;43(8):1439–44.CrossRefPubMedGoogle Scholar
  39. Murphy F, Puri P. New insights into the pathogenesis of Hirschsprung’s associated enterocolitis. Pediatr Surg Int. 2005;21(10):773–9.CrossRefPubMedGoogle Scholar
  40. Pastor A, Osman F, et al. Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg. 2009;44(1):251–6.CrossRefPubMedGoogle Scholar
  41. Polley Jr T, Coran A, et al. A ten-year experience with ninety-two cases of Hirschsprung’s disease. Including sixty-seven consecutive endorectal pull-through procedures. Ann Surg. 1985;202(3):349–55.CrossRefPubMedPubMedCentralGoogle Scholar
  42. Rescorla F, Morrison A, et al. Hirschsprung’s disease. Evaluation of mortality and long-term function in 260 cases. Arch Surg. 1992;127(8):934–41; discussion 941–932.Google Scholar
  43. Rintala RJ, Lindahl H. Sodium cromoglycate in the management of chronic or recurrent enterocolitis in patients with Hirschsprung’s disease. J Pediatr Surg. 2001;36(7):1032–5.CrossRefPubMedGoogle Scholar
  44. Shen DH, Shi CR, et al. Detection of intestinal bifidobacteria and lactobacilli in patients with Hirschsprung’s disease associated enterocolitis. World J Pediatr. 2009;5(3):201–5.CrossRefPubMedGoogle Scholar
  45. Soeda J, O’Briain DS, et al. Regional reduction in intestinal neuroendocrine cell populations in enterocolitis complicating Hirschsprung’s disease. J Pediatr Surg. 1993;28(8):1063–8.CrossRefPubMedGoogle Scholar
  46. Staiano A, Santoro L, et al. Autonomic dysfunction in children with Hirschsprung’s disease. Dig Dis Sci. 1999;44(5):960–5.CrossRefPubMedGoogle Scholar
  47. Swenson O, Fisher J, et al. Diarrhea following rectosigmoidectomy for Hirschsprung’s disease. Surgery. 1960;48:419–21.PubMedGoogle Scholar
  48. Swenson O, Sherman J, et al. The treatment and postoperative complications of congenital megacolon: a 25 year follow-up. Ann Surg. 1975;182:266–73.CrossRefPubMedPubMedCentralGoogle Scholar
  49. Teitelbaum DH, Coran AG. Hirschsprung’s disease. In: Spitz L, Coran AG, editors. Operative pediatric surgery. 7th ed. Boca Raton: CRC Press/Taylor & Francis Group, LLC; 2013. p. 578.Google Scholar
  50. Teitelbaum D, Qualman S, et al. Hirschsprung’s disease. Identification of risk factors for enterocolitis. Ann Surg. 1988;207(3):240–4.CrossRefPubMedPubMedCentralGoogle Scholar
  51. Teitelbaum D, Caniano D, et al. The pathophysiology of Hirschsprung’s-associated enterocolitis: importance of histologic correlates. J Pediatr Surg. 1989;24(12):1271–7.CrossRefPubMedGoogle Scholar
  52. Teitelbaum DH, Cilley R, et al. A decade experience with the primary pull-through for Hirschsprung’s disease in the newborn period: a multi-center analysis of outcomes. Ann Surg. 2000;232(3):372–80.CrossRefPubMedPubMedCentralGoogle Scholar
  53. Temple SJ, Shawyer A, et al. Is daily dilatation by parents necessary after surgery for Hirschsprung disease and anorectal malformations? J Pediatr Surg. 2012;47(1):209–12.CrossRefPubMedGoogle Scholar
  54. Thomas DF, Fernie DS, et al. Association between Clostridium difficile and enterocolitis in Hirschsprung’s disease. Lancet. 1982;1(8263):78–9.CrossRefPubMedGoogle Scholar
  55. Turnock RR, Spitz L, et al. A study of mucosal gut immunity in infants who develop Hirschsprung’s- associated enterocolitis. J Pediatr Surg. 1992;27(7):828–9.CrossRefPubMedGoogle Scholar
  56. Vieten D, Spicer R. Enterocolitis complicating Hirschsprung’s disease. Semin Pediatr Surg. 2004;13(4):263–72.CrossRefPubMedGoogle Scholar
  57. Wildhaber B, Pakarinen M, et al. Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung’s disease. J Pediatr Surg. 2004;39(6):920–6.CrossRefPubMedGoogle Scholar
  58. Wilson-Storey D, Scobie W. Impaired gastrointestinal mucosal defense in Hirschsprung’s disease: a clue to the pathogenesis of enterocolitis? J Pediatr Surg. 1989;24(5):462–4.CrossRefPubMedGoogle Scholar
  59. Wilson-Storey D, Scobie WG, et al. Microbiological studies of the enterocolitis of Hirschsprung’s disease. Arch Dis Child. 1990;65(12):1338–9.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  • Farokh R. Demehri
    • 1
    Email author
  • Ihab F. Halaweish
    • 1
  • Arnold G. Coran
    • 1
  • Daniel H. Teitelbaum
    • 1
  1. 1.Section of Pediatric SurgeryC.S. Mott Children’s Hospital and the University of Michigan School of MedicineAnn ArborUSA

Personalised recommendations