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Enterocolitis

  • Keiichi UchidaEmail author
  • Mikihiro Inoue
  • Yuhki Koike
  • Kohei Matsushita
  • Yuka Nagano
  • Masato Kusunoki
Chapter

Abstract

Hirschsprung’s disease-associated enterocolitis is the most common complication of Hirschsprung’s disease. Clinical symptoms of Hirschsprung’s disease-associated enterocolitis vary from mild to severe, and it sometimes becomes a serious and life-threatening condition. Historically, surgical procedures for Hirschsprung’s disease have significantly progressed, but Hirschsprung’s disease-associated enterocolitis remains a frequent complication before and after surgery. The incidence of Hirschsprung’s disease-associated enterocolitis widely varies because of different diagnostic criteria. Historically, distal obstruction due to aganglionosis has been considered as the sole causative factor in the development of Hirschsprung’s disease-associated enterocolitis. Recent studies have shown that the pathogenesis of Hirschsprung’s disease-associated enterocolitis is complex and is associated with other factors, such as genetics, the gut mucosal barrier, mucosal immunity, the microbiome, and surgical factors. The development of management strategies for this condition can be applied before and after definitive pull-through surgery. These include prompt application of preventive strategies for Hirschsprung’s disease-associated enterocolitis, parenteral and enteral nutrition, and probiotics, which might contribute to the decreased mortality rate. In this chapter, we review the pathogenesis, diagnosis, therapeutic management, and prevention of Hirschsprung’s disease-associated enterocolitis.

Keywords

Hirschsprung’s disease Enterocolitis 

Notes

Acknowledgment

We thank Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.

References

  1. 1.
    Taguchi T, Obata S, Ieiri S. Current status of Hirschsprung’s disease: based on a nationwide survey of Japan. Pediatr Surg Int. 2017;33:497–504.CrossRefPubMedGoogle Scholar
  2. 2.
    Berger M, Muensterer O, Harmon CM. Tales from previous times: important eponyms in pediatric surgery. Pediatr Surg Int. 2014;30:1–10.CrossRefPubMedGoogle Scholar
  3. 3.
    Bill J, Chapman AH, Chapman ND. The enterocolitis of Hirschsprung’s disease: its natural history and treatment. Am J Surg. 1962;103:70–4.CrossRefGoogle Scholar
  4. 4.
    Demehri FR, Halaweish IF, Coran AG, et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int. 2013;29:873–81.CrossRefPubMedGoogle Scholar
  5. 5.
    Lui VC, Li L, Sham MH. 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;28:89–100.CrossRefGoogle Scholar
  6. 6.
    Vieten D, Spicer R. Enterocolitis complicating Hirschsprung’s disease. Semin Pediatr Surg. 2004;13:263–72.CrossRefPubMedGoogle Scholar
  7. 7.
    Pini-Prato A, Rossi V, Avanzini S, et al. Hirschsprung’s disease: what about mortality? Pediatr Surg Int. 2011;27:473–8.CrossRefPubMedGoogle Scholar
  8. 8.
    Gosain A, Fryman P, Cowles RA, et al. Guidelines for the diagnosis and management of Hirschsprung-associate enterocolitis. Pediatr Surg Int. 2017;33:517–21.  https://doi.org/10.1007/s00383-017-4065-8.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Haricharan RN, Seo JM, Kelly DR, et al. Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis. J Pediatr Surg. 2008;43:1115–23.CrossRefPubMedGoogle Scholar
  10. 10.
    El-Sawaf M, Siddiqui S, Mahmoud M, 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;48:111–7.CrossRefPubMedGoogle Scholar
  11. 11.
    Marty TL, Seo T, Matlak ME, et al. Gastrointestinal function after surgical correction of Hirschsprung’s disease: long-term follow-up in 135 patients. J Pediatr Surg. 1995;30:655–8.CrossRefPubMedGoogle Scholar
  12. 12.
    Polly TZ, Coran AG, Wesley JR. A ten-year experience with ninety-two cases of Hirschsprung’s disease. Including sixty-seven consecutive endorectal pull-through procedures. Ann Surg. 1985;202:349–55.CrossRefGoogle Scholar
  13. 13.
    Zhao L, Dhall D, Cheng Z, et al. Murine model of Hirschsprung-associated enterocolitis II: surgical correction of aganglionosis does not eliminate enterocolitis. J Pediatr Surg. 2010;45:206–11.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Teitelbaum D, Qualman S, Caniano DA. Hirschsprung’s disease. Identification of risk factors enterocolitis. Ann Surg. 1988;207:240–4.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Thakkar HS, Bassett C, Hsu A, et al. Functional outcomes in Hirschsprung disease: a single institution’s 12-year experience. J Pediatr Surg. 2017;52:277–80.CrossRefPubMedGoogle Scholar
  16. 16.
    Kwendakwema N, Al-Dulaimi R, Presson AP, et al. Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21. J Pediatr Surg. 2016;51:2001–4.CrossRefPubMedGoogle Scholar
  17. 17.
    Wang X, Li Z, Xu Z, et al. Probiotics prevent Hirschsprung’s disease-associated enterocolitis: a prospective multicenter randomized controlled trial. Int J Color Dis. 2014;30:105–10.CrossRefGoogle Scholar
  18. 18.
    Jiao CL, Chen XY, Feng JX. Novel insights into the pathogenesis of Hirschsprung’s-associated enterocolitis. Chin Med J. 2016;129:1491–7.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Caniano DA, Teitelbaum DH, Qualman SJ, et al. The piebald-lethal murine strain: Investigation of the cause of early death. J Pediatr Surg. 1989;24:906–10.CrossRefPubMedGoogle Scholar
  20. 20.
    Menezes M, Puri P. Long-term outcome of patients with enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int. 2006;22:316–8.CrossRefPubMedGoogle Scholar
  21. 21.
    Cheng Z, Dhall D, Zhao L, et al. murine model of Hirschsprung-associated enterocolitis. 1: phenotypic characterization with development of a histopathologic grading system. J Pediatr Surg. 2010;45:475–82.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Hardy SP, Bayston R, Spitz L. Prolonged carriage of Clostridium difficile in Hirschsprung’s disease. Arch Dis Child. 1993;69:221–4.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Veiga-Fernandes H, Coles MC, Foster KE, et al. Tyrosine kinase receptor RET is a key regulator of Peyer’s patch organogenesis. Nature. 2007;446:547–51.CrossRefPubMedGoogle Scholar
  24. 24.
    Lacher M, Fitze G, Helmbrecht J, et al. Hirschsprung-associated enterocolitis develops independently of NOD2 variants. J Pediatr Surg. 2010;45:1826–31.CrossRefPubMedGoogle Scholar
  25. 25.
    Gosain A. Established and emerging concepts in Hirschsprung’s-associated enterocolitis. Pediatr Surg Int. 2016;32:313–20.CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Gariepy CE, Cass DT, Yanagisawa M. Null mutation of endothelin receptor type B gene in spotting lethal rats causes aganglionic megacolon and white coat color. Proc Natl Acad Sci U S A. 1996;23:867–72.CrossRefGoogle Scholar
  27. 27.
    Gosain A, Brinkman AS. Hirschsprung’s associated enterocolitis. Curr Opin Pediatr. 2015;27:364–9.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Keita ÅV, Söderholm JD. The intestinal barrier and its regulation by neuroimmune factors. Neurogastroenterol Motil. 2010;22:718–33.CrossRefPubMedGoogle Scholar
  29. 29.
    Johansson ME, Sjövall G, Hansson GC. The gastrointestinal mucus system in health and disease. Nat Rev Gastroenterol Hepatol. 2013;10:352–561.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Pelaseyed T, Bergström JH, Gustafsson JK, et al. The mucus and mucins of the goblet cells and enterocytes provide the first defense line of the gastrointestinal tract and interact with the immune system. Immunol Rev. 2014;260:8–20.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Mattar AF, Coran AG, Teitebaum DH. MUC-2 mucin production in Hirschsprung’s disease: possible association with enterocolitis development. J Pediatr Surg. 2003;38:417–21.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Thiagarajah JR, Yildiz H, Carlson T, et al. Altered goblet cell differentiation and surface mucus properties in Hirschsprung disease. PLoS One. 2014;9:e99944.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Tomuschat C, O’Donnell AM, Coyle D, et al. Altered expression of ATP-sensitive K(+) channels in Hirschsprung’s disease. J Pediatr Surg. 2016;51:948–52.CrossRefPubMedGoogle Scholar
  34. 34.
    Tomuschat C, O’Donnell AM, Coyle D, et al. Increased Act1/IL-17R expression in Hirschsprung’s disease. Pediatr Surg Int. 2016;32:1201–7.CrossRefPubMedGoogle Scholar
  35. 35.
    Tomuschat C, O’Donnell AM, Coyle D, et al. Altered expression of IL36γ and IL36 receptor (IL1RL2) in the colon of patients with Hirschsprung’s disease. Pediatr Surg Int. 2017;33:181–6.CrossRefPubMedGoogle Scholar
  36. 36.
    Iwasaki A, Medzhitov R. Control of adaptive immunity by the innate immune system. Nat Immunol. 2015;16:343–53.CrossRefPubMedPubMedCentralGoogle Scholar
  37. 37.
    Azzali G. Structure, lymphatic vascularization and lymphocyte migration in mucosa-associated lymphoid tissue. Immunol Rev. 2003;195:178–89.CrossRefPubMedGoogle Scholar
  38. 38.
    Mowat AM, Agace WW. Regional specialization within the intestinal immune system. Nat Rev Immunol. 2014;14:667–85.CrossRefPubMedGoogle Scholar
  39. 39.
    Imamura A, Puri P, O’Briain DS. Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut. 1992;33:801–6.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Gosain A, Barlow-Anacker AJ, Erickson CS, et al. Impaired cellular immunity in the murine neural crest conditional deletion of endothelin receptor-B model of Hirschsprung’s disease. PLoS One. 2015;10:e0128822.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Moore SW, Johnson G, Schneider JW. Elevated tissue immunoglobulins in Hirschsprung’s disease—indication of early immunologic response. Eur J Pediatr Surg. 2000;10:106–10.CrossRefPubMedGoogle Scholar
  42. 42.
    Vulchanova L, Casey MA, Crabb GW, et al. Anatomical evidence for enteric neuroimmune interactions in Peyer’s patches. J Neuroimmunol. 2007;185:64–74.CrossRefPubMedPubMedCentralGoogle Scholar
  43. 43.
    Karlsson J, Pütsep K, Chu H, et al. Regional variations in Paneth cell antimicrobial peptide expression along the mouse intestinal tract. BMC Immunol. 2008;9:37.CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Yan Z, Poroyko V, Gu S. Characterization of the intestinal microbiome of Hirschsprung’s disease with and without enterocolitis. Biochem Biophys Res Commun. 2014;445:269–74.CrossRefPubMedGoogle Scholar
  45. 45.
    Li Y, Poroyko V, Yan Z, 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:e0162079.CrossRefPubMedPubMedCentralGoogle Scholar
  46. 46.
    Frykman PK, Nordenskjöld A, Kawaguchi A. 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:e0124172.CrossRefPubMedPubMedCentralGoogle Scholar
  47. 47.
    Pierre JF, Barlow-Anacker AJ, Erickson CS. Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung’s disease. J Pediatr Surg. 2014;49:1242–51.CrossRefPubMedPubMedCentralGoogle Scholar
  48. 48.
    Rolig AS, Mittge EK, Ganz J, et al. The enteric nervous system promotes intestinal health by constraining microbiota composition. PLoS Biol. 2017;15:e2000689.CrossRefPubMedPubMedCentralGoogle Scholar
  49. 49.
    Wiles TJ, Jemielita M, Baker RP. Host gut motility promotes competitive exclusion within a model intestinal microbiota. PLoS Biol. 2016;14:e1002517.CrossRefPubMedPubMedCentralGoogle Scholar
  50. 50.
    Langer JC, Rollins MD, Levitt M, et al.; Hirschsprung Disease Interest Group. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int. 2017;33:523–6.CrossRefPubMedGoogle Scholar
  51. 51.
    Minford JL, Ram A, Turnock RR, et al. Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung’s disease. J Pediatr Surg. 2004;39:161–5.CrossRefPubMedGoogle Scholar
  52. 52.
    Hackam DJ, Filler RM, Pearl RH. Enterocolitis after the surgical treatment of Hirschsprung’s disease: risk factors and financial impact. J Pediatr Surg. 1998;33:830–3.CrossRefPubMedGoogle Scholar
  53. 53.
    Church JT, Gadepalli SK, Talishinsky T, et al. Ultrasound-guided intrasphincteric botulinum toxin injection relieves obstructive defecation due to Hirschsprung’s disease and internal anal sphincter achalasia. J Pediatr Surg. 2017;52:74–8.CrossRefPubMedGoogle Scholar
  54. 54.
    Chumpitazi BP, Nurko S. Defecation disorders in children after surgery for Hirschsprung disease. J Pediatr Gastroenterol Nutr. 2011;53:75–9.CrossRefPubMedGoogle Scholar
  55. 55.
    Demehri FR, Frykman PK, Cheng Z, et al. Altered fecal short chain fatty acid composition in children with a history of Hirschsprung-associated enterocolitis. J Pediatr Surg. 2016;51:81–6.CrossRefPubMedGoogle Scholar
  56. 56.
    Pastor A, Osman F, et al. Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg. 2009;44:251–6.CrossRefPubMedGoogle Scholar
  57. 57.
    Bell MJ, Ternberg JL, Feigin RD, et al. Neonatal necrotizing enterocolitis. Therapeutic decisions based upon clinical staging. Ann Surg. 1978;187:1–7.CrossRefPubMedPubMedCentralGoogle Scholar
  58. 58.
    Teitelbaum DH, Cilley R Sherman NJ, 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;232:372–80.CrossRefPubMedPubMedCentralGoogle Scholar
  59. 59.
    Levitt MA, Dickie B, Peña A, 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;19:146–53.CrossRefPubMedGoogle Scholar
  60. 60.
    Moore SW, Millar AJ Cywes S. Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung’s patient. J Pediatr Surg. 1994;29:106–11.CrossRefPubMedGoogle Scholar
  61. 61.
    Lawal TA, Chatoorgoon K, Collins MH. Redo pull-through in Hirschsprung’s (corrected) disease for obstructive symptoms due to residual aganglionosis and transition zone bowel. J Pediatr Surg. 2011;46:342–7.CrossRefPubMedGoogle Scholar
  62. 62.
    Patrus B, Nasr A, Langer JC, et al. Intrasphincteric botulinum toxin decreases the rate of hospitalization for postoperative obstructive symptoms in children with Hirschsprung disease. J Pediatr Surg. 2011;46:184–7.CrossRefPubMedGoogle Scholar
  63. 63.
    Friedmacher F, Puri P. Comparison of posterior internal anal sphincter myectomy and intrasphincteric botulinum toxin injection for treatment of internal anal sphincter achalasia: a meta-analysis. Pediatr Surg Int. 2012;28:765–71.CrossRefPubMedGoogle Scholar
  64. 64.
    Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung’s disease. Am J Surg. 2000;180:382–7.CrossRefPubMedGoogle Scholar
  65. 65.
    Frykman PK, Short SS. Hirschsprung-associated enterocolitis: prevention and therapy. Semin Pediatr Surg. 2012;21:328–35.CrossRefPubMedPubMedCentralGoogle Scholar
  66. 66.
    Marty TL, Matlak ME, Hendrickson M, et al. Unexpected death from enterocolitis after surgery for Hirschsprung’s disease. Pediatrics. 1995;96:118–21.PubMedGoogle Scholar
  67. 67.
    Temple SJ, Shawyer A, Langer JC. Is daily dilatation by parents necessary after surgery for Hirschsprung disease and anorectal malformations? J Pediatr Surg. 2012;47:209–12.CrossRefPubMedGoogle Scholar
  68. 68.
    Marty TL, Seo T, Sullivan JJ, et al. Rectal irrigations for the prevention of postoperative enterocolitis in Hirschsprung’s disease. J Pediatr Surg. 1995;30:652–4.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Keiichi Uchida
    • 1
    Email author
  • Mikihiro Inoue
    • 1
  • Yuhki Koike
    • 1
  • Kohei Matsushita
    • 1
  • Yuka Nagano
    • 1
  • Masato Kusunoki
    • 1
  1. 1.Department of Gastrointestinal and Pediatric SurgeryMie University Graduate School of MedicineTsuJapan

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