Skip to main content
SpringerLink
Log in

Hirschsprung-Associated Enterocolitis

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

Abstract

Hirschsprung-associated enterocolitis (HAEC) was described in 1886 by Harald Hirschsprung and is the most feared complication of Hirschsprung disease. HAEC is clinically characterized by abdominal distension, fever, and diarrhea, although there can be a variety of other associated symptoms, including colicky abdominal pain, lethargy, and the passage of bloodstained stools. HAEC can occur preoperatively or postoperatively, is the presenting symptom of HSCR in up to 25% of infants, and carries an overall incidence ranging from 20% to 60%. This chapter reviews our current understanding of HAEC pathophysiology, risk factors, diagnosis, and treatment. Areas of current research or controversy and topics for future investigation are presented.

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

Access this chapter

Log in via an institution
eBook
USD 16.99
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. Akkary S, Sahwy E, Kandil W, Hamdy MH. 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.

    Article  CAS  PubMed  Google Scholar 

  2. Albanese CT, Smith SD, Watkins S, Kurkchubasche A, Simmons RL, Rowe MI. Effect of secretory IgA on transepithelial passage of bacteria across the intact ileum in vitro. J Am Coll Surg. 1994;179(6):679–88.

    CAS  PubMed  Google Scholar 

  3. Ament ME, Bill AH. Persistent diarrhea due to sucrase-isomaltase deficiency in a postoperative child with Hirschsprung’s disease. J Pediatr Surg. 1973;8(4):543–5.

    Article  CAS  PubMed  Google Scholar 

  4. Amiel J, Sproat-Emison E, Garcia-Barcelo M, Lantieri F, Burzynski G, Borrego S, Pelet A, Arnold S, Miao X, Griseri P, Brooks AS, Antinolo G, de Pontual L, Clement-Ziza M, Munnich A, Kashuk C, West K, Wong KK-Y, Lyonnet S, Chakravarti A, Tam PK-H, Ceccherini I, Hofstra RMW, Fernandez R, Hirschsprung Disease Consortium. Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet. 2008;45(1):1–14.

    Article  CAS  PubMed  Google Scholar 

  5. Aslam A, Spicer RD, Corfield AP. 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.

    Article  CAS  PubMed  Google Scholar 

  6. Austin KM. The pathogenesis of Hirschsprung’s disease-associated enterocolitis. Semin Pediatr Surg. 2012;21(4):319–27.

    Article  PubMed  Google Scholar 

  7. Aworanti O, Hung J, McDowell D, Martin I, Quinn F. Are routine dilatations necessary post pull-through surgery for Hirschsprung disease? Eur J Pediatr Surg. 2013;23(5):383–8.

    Article  PubMed  Google Scholar 

  8. Bagwell CE, Langham MR Jr, Mahaffey SM, Talbert JL, Shandling B. Pseudomembranous colitis following resection for Hirschsprung’s disease. J Pediatr Surg. 1992;27(10):1261–4.

    Article  CAS  PubMed  Google Scholar 

  9. Berry CL, Fraser GC. The experimental production of colitis in the rabbit with particular reference to Hirschsprung’s disease. J Pediatr Surg. 1968;3(1):36–42.

    Article  Google Scholar 

  10. Bickelhaupt S, Pazahr S, Chuck N, Blume I, Froehlich JM, Cattin R, Raible S, Bouquet H, Bill U, Rogler G, Frei P, Boss A, Patak MA. Crohn’s disease: small bowel motility impairment correlates with inflammatory-related markers C-reactive protein and calprotectin. Neurogastroenterol Motil. 2013;25(6):467–73.

    Article  CAS  PubMed  Google Scholar 

  11. Bill A, CHapman N. The enterocolitis of Hirschsprung’s disease: its natural history and treatment. Am J Surg. 1962;103:70–4.

    Article  Google Scholar 

  12. Buisine MP, Devisme L, Savidge TC, Gespach C, Gosselin B, Porchet N, Aubert JP. Mucin gene expression in human embryonic and fetal intestine. Gut. 1998;43(4):519–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Burnard ED. Hirschsprung’s disease in infancy. Br Med J. 1950;1(4646):151–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Caniano DA, Teitelbaum DH, Qualman SJ. Management of Hirschsprung’s disease in children with trisomy 21. Am J Surg. 1990;159(4):402–4.

    Article  CAS  PubMed  Google Scholar 

  15. Carcassonne M, Guys JM, Morrison-Lacombe G, Kreitmann B. Management of Hirschsprung’s disease: curative surgery before 3 months of age. J Pediatr Surg. 1989;24(10):1032–4.

    Article  CAS  PubMed  Google Scholar 

  16. Cheng LS, Schwartz DM, Hotta R, Graham HK, Goldstein AM. Bowel dysfunction following pullthrough surgery is associated with an overabundance of nitrergic neurons in Hirschsprung disease. J Pediatr Surg. 2016;51(11):1834–8.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Cheng Z, Dhall D, Zhao L, Wang HL, Doherty TM, Bresee C, Frykman PK. Murine model of Hirschsprung-associated enterocolitis. I: phenotypic characterization with development of a histopathologic grading system. J Pediatr Surg. 2010;45(3):475–82.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Cheng Z, Wang X, Dhall D, Zhao L, Bresee C, Doherty TM, Frykman PK. Splenic lymphopenia in the endothelin receptor B-null mouse: implications for Hirschsprung associated enterocolitis. Pediatr Surg Int. 2011;27(2):145–50.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Coran AG, Teitelbaum DH. Recent advances in the management of Hirschsprung’s disease. Am J Surg. 2000;180(5):382–7.

    Article  CAS  PubMed  Google Scholar 

  20. Dalkey N, Helmer O. An experimental application of the Delphi method to the use of experts. Manag Sci. 1963;9(3):458–67.

    Article  Google Scholar 

  21. De Filippo C, Pini-Prato A, Mattioli G, Avanzini S, Rapuzzi G, Cavalieri D, Di Paola M, Stefanini I, Ceccherini I, Mavilio D, Lionetti P, Jasonni V. Genomics approach to the analysis of bacterial communities dynamics in Hirschsprung’s disease-associated enterocolitis: a pilot study. Pediatr Surg Int. 2010;26(5):465–71.

    Article  PubMed  Google Scholar 

  22. Dorman GW. Hirschsprung’s disease; a lethal problem in early infancy. AMA Arch Surg. 1957;75(6):906–13.

    Article  CAS  PubMed  Google Scholar 

  23. El-Sawaf M, Siddiqui S, Mahmoud M, Drongowski R, Teitelbaum DH. 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.

    Article  PubMed  Google Scholar 

  24. Elhalaby EA, Coran AG, Blane CE, Hirschl RB, Teitelbaum DH. Enterocolitis associated with Hirschsprung’s disease: a clinical-radiological characterization based on 168 patients. J Pediatr Surg. 1995;30(1):76–83.

    Article  CAS  PubMed  Google Scholar 

  25. Fisher JH, Swenson O. Hirschsprung’s disease during infancy. Surg Clin North Am. 1956:1511–5.

    Google Scholar 

  26. Foster P, Cowan G, Wrenn EL. Twenty-five years’ experience with Hirschsprung’s disease. J Pediatr Surg. 1990;25(5):531–4.

    Article  CAS  PubMed  Google Scholar 

  27. Frykman PK, Cheng Z, Wang X, Dhall D. Enterocolitis causes profound lymphoid depletion in endothelin receptor B- and endothelin 3-null mouse models of Hirschsprung-associated enterocolitis. Eur J Immunol. 2015a;45(3):807–17.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Frykman PK, Kim S, Wester T, Nordenskjöld A, Kawaguchi A, Hui TT, Teitelbaum DH, Granström AL, Rogatko A, HAEC Collaborative Research Group (HCRG). Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: a multicenter study of 116 children with Hirschsprung disease. J Pediatr Surg. 2018;53(4):708–17.

    Google Scholar 

  29. Frykman PK, Nordenskjold A, Kawaguchi A, Hui TT, Granstrom AL, Cheng Z, Tang J, Underhill DM, Iliev I, Funari VA, Wester T, H. C. R. Group. Characterization of bacterial and fungal microbiome in children with Hirschsprung disease with and without a history of Enterocolitis: a multicenter study. PLoS One. 2015b;10(4):e0124172.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  31. Fujimoto T. Natural history and pathophysiology of enterocolitis in the piebald lethal mouse model of Hirschsprung’s disease. J Pediatr Surg. 1988a;23(3):237–42.

    Article  CAS  PubMed  Google Scholar 

  32. Fujimoto T, Reen DJ, Puri P. Inflammatory response in enterocolitis in the piebald lethal mouse model of Hirschsprung’s disease. Pediatr Res. 1988;24(2):152–5.

    Article  CAS  PubMed  Google Scholar 

  33. Gao Y, Li G, Zhang X, Xu Q, Guo Z, Zheng B, Li P, Li G. Primary transanal rectosigmoidectomy for Hirschsprung’s disease: preliminary results in the initial 33 cases. J Pediatr Surg. 2001;36(12):1816–9.

    Article  CAS  PubMed  Google Scholar 

  34. Gosain A, Barlow-Anacker AJ, Erickson CS, Pierre JF, Heneghan AF, Epstein ML, Kudsk KA. Impaired cellular immunity in the murine neural crest conditional deletion of Endothelin receptor-B model of Hirschsprung’s disease. PLoS One. 2015;10(6):e0128822.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  35. Gosain A, Frykman PK, Cowles RA, Horton J, Levitt M, Rothstein DH, Langer JC, Goldstein AM, American Pediatric Surgical Association Hirschsprung Disease Interest Group. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int. 2017;33(5):517–21.

    Article  PubMed  PubMed Central  Google Scholar 

  36. 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(6):830–3.

    Article  CAS  PubMed  Google Scholar 

  37. Hardy SP, Bayston R, Spitz L. Prolonged carriage of Clostridium difficile in Hirschsprung’s disease. Arch Dis Child. 1993;69(2):221–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Haricharan RN, Seo J-M, Kelly DR, Mroczek-Musulman EC, Aprahamian CJ, Morgan TL, Georgeson KE, Harmon CM, Saito JM, Barnhart DC. 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.

    Article  PubMed  Google Scholar 

  39. Harrison MW, Deitz DM, Campbell JR, Campbell TJ. Diagnosis and management of Hirschsprung’s disease. A 25 year perspective. Am J Surg. 1986;152(1):49–56.

    Article  CAS  PubMed  Google Scholar 

  40. 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  PubMed  Google Scholar 

  41. Heuckeroth RO. Hirschsprung disease – integrating basic science and clinical medicine to improve outcomes. Nat Rev Gastroenterol Hepatol. 2018;15(3):152–67.

    Article  Google Scholar 

  42. Hirschsprung H. Stuhltragheit neugeborener infolge von dilatation and hypertrophies des colons. Jahrb Kinderheilkd. 1887;27:1–42.

    Google Scholar 

  43. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Imamura A, Puri P, O’Briain DS, Reen DJ. Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut. 1992;33(6):801–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Kobayashi H, Hirakawa H, Puri P. Overexpression of intercellular adhesion molecule-1 (ICAM-1) and MHC class II antigen on hypertrophic nerve trunks suggests an immunopathologic response in Hirschsprung’s disease. J Pediatr Surg. 1995;30(12):1680–3.

    Article  CAS  PubMed  Google Scholar 

  46. Langer JC, Rollins MD, Levitt M, Gosain A, Torre L, Kapur RP, Cowles RA, Horton J, Rothstein DH, Goldstein AM. American Pediatric Surgical Association Hirschsprung Disease Interest Group. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int. 2017;33(5):523–6.

    Article  CAS  PubMed  Google Scholar 

  47. Lloyd-Still JD, Demers LM. Hirschsprung’s enterocolitis, prostaglandins, and response to cholestyramine. J Pediatr Surg. 1978;13(4):417–8.

    Article  CAS  PubMed  Google Scholar 

  48. Marty TL, Matlak ME, Hendrickson M, Black RE, Johnson DG. Unexpected death from enterocolitis after surgery for Hirschsprung’s disease. Pediatrics. 1995;96(1 Pt 1):118–21.

    CAS  PubMed  Google Scholar 

  49. Marty TL, Seo T, Matlak ME, Sullivan JJ, Black RE, Johnson DG. Gastrointestinal function after surgical correction of Hirschsprung’s disease: long-term follow-up in 135 patients. J Pediatr Surg. 1995;30(5):655–8.

    Article  CAS  PubMed  Google Scholar 

  50. Marty TL, Seo T, Sullivan JJ, Matlak ME, Black RE, Johnson DG. Rectal irrigations for the prevention of postoperative enterocolitis in Hirschsprung’s disease. J Pediatr Surg. 1995;30(5):652–4.

    Article  CAS  PubMed  Google Scholar 

  51. Mattar AF, Coran AG, Teitelbaum DH. MUC-2 mucin production in Hirschsprung’s disease: possible association with enterocolitis development. J Pediatr Surg. 2003;38(3):417–21; discussion 417-421

    Article  PubMed  Google Scholar 

  52. Medrano G, Cailleux F, Guan P, Kuruvilla K, Barlow-Anacker AJ, Gosain A. B-lymphocyte-intrinsic and -extrinsic defects in secretory immunoglobulin A production in the neural crest-conditional deletion of endothelin receptor B model of Hirschsprung-associated enterocolitis. FASEB J. 2019:fj201801913R. https://doi.org/10.1096/fj.201801913R.

  53. Menezes M, Puri P. Long-term clinical outcome in patients with Hirschsprung’s disease and associated Down’s syndrome. J Pediatr Surg. 2005;40(5):810–2.

    Article  PubMed  Google Scholar 

  54. 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.

    Article  CAS  PubMed  Google Scholar 

  55. Moore SW, Sidler D, Zaahl MG. The ITGB2 immunomodulatory gene (CD18), enterocolitis, and Hirschsprung’s disease. J Pediatr Surg. 2008;43(8):1439–44.

    Article  PubMed  Google Scholar 

  56. Nair MP, Schwartz SA. Association of decreased T-cell-mediated natural cytotoxicity and interferon production in Down’s syndrome. Clin Immunol Immunopathol. 1984;33(3):412–24.

    Article  CAS  PubMed  Google Scholar 

  57. Nakamura H, Lim T, Puri P. Probiotics for the prevention of Hirschsprung-associated enterocolitis: a systematic review and meta-analysis. Pediatr Surg Int. 2018;34(2):189–93.

    Article  CAS  PubMed  Google Scholar 

  58. Nunez R, Torres A, Agulla E, Moreno C, Marin D, Santamaria JI. Rectal irrigation and bowel decontamination for the prevention of postoperative enterocolitis in Hirschsprung’s disease. Cir Pediatr. 2007;20(2):96–100.

    CAS  PubMed  Google Scholar 

  59. Ohama T, Hori M, Fujisawa M, Kiyosue M, Hashimoto M, Ikenoue Y, Jinno Y, Miwa H, Matsumoto T, Murata T, Ozaki H. Downregulation of CPI-17 contributes to dysfunctional motility in chronic intestinal inflammation model mice and ulcerative colitis patients. J Gastroenterol. 2008;43(11):858–65.

    Article  PubMed  Google Scholar 

  60. Pastor AC, Osman F, Teitelbaum DH, Caty MG, Langer JC. Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg. 2009;44(1):251–6.

    Article  PubMed  Google Scholar 

  61. Patrus B, Nasr A, Langer JC, Gerstle JT. Intrasphincteric botulinum toxin decreases the rate of hospitalization for postoperative obstructive symptoms in children with Hirschsprung disease. J Pediatr Surg. 2011;46(1):184–7.

    Article  PubMed  Google Scholar 

  62. Pierre JF, Barlow-Anacker AJ, Erickson CS, Heneghan AF, Leverson GE, Dowd SE, Epstein ML, Kudsk KA, Gosain A. Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung’s disease. J Pediatr Surg. 2014;49(8):1242–51.

    Article  PubMed  PubMed Central  Google Scholar 

  63. 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  PubMed  Google Scholar 

  64. Polley 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. 1985a;202(3):349–55.

    Article  PubMed  PubMed Central  Google Scholar 

  65. 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  PubMed  Google Scholar 

  66. Rescorla FJ, Morrison AM, Engles D, West KW, Grosfeld JL. Hirschsprung’s disease. Evaluation of mortality and long-term function in 260 cases. Arch Surg. 1992;127(8):934–41; discussion 941–932

    Article  CAS  PubMed  Google Scholar 

  67. 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.

    Article  CAS  PubMed  Google Scholar 

  68. Shim WK, Swenson O. Treatmentof congenital megacolon in 50 infants. Pediatrics. 1966;38(2):185–93.

    CAS  PubMed  Google Scholar 

  69. Soeda J, O’Briain DS, Puri P. Mucosal neuroendocrine cell abnormalities in the colon of patients with Hirschsprung’s disease. J Pediatr Surg. 1992;27(7):823–7.

    Article  CAS  PubMed  Google Scholar 

  70. Soeda J, O’Briain DS, Puri P. Regional reduction in intestinal neuroendocrine cell populations in enterocolitis complicating Hirschsprung’s disease. J Pediatr Surg. 1993;28(8):1063–8.

    Article  CAS  PubMed  Google Scholar 

  71. Spiller RC. Role of nerves in enteric infection. Gut. 2002;51(6):759–62.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  72. 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; discussion 201-192

    Article  PubMed  Google Scholar 

  73. Swenson O. Hirschsprung’s disease—a complicated therapeutic problem: some thoughts and solutions based on data and personal experience over 56 years. J Pediatr Surg. 2004;39(10):1449–53.

    Article  PubMed  Google Scholar 

  74. Swenson O, Davidson FZ. Similarities of mechanical intestinal obstruction and aganglionic megacolon in the newborn infant: a review of 64 cases. N Engl J Med. 1960;262:64–7.

    Article  CAS  PubMed  Google Scholar 

  75. Swenson O, Fisher JH, Scott JE. Diarrhea following rectosigmoidectomy for Hirschsprung’s disease. Surgery. 1960;48:419–21.

    CAS  PubMed  Google Scholar 

  76. Teitelbaum DH, Caniano DA, Qualman SJ. The pathophysiology of Hirschsprung’s-associated enterocolitis: importance of histologic correlates. J Pediatr Surg. 1989;24(12):1271–7.

    Article  CAS  PubMed  Google Scholar 

  77. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  78. Teitelbaum DH, Drongowski RA, Chamberlain JN, Coran AG. Long-term stooling patterns in infants undergoing primary endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg. 1997;32(7):1049–52; discussion 1052-1043

    Article  CAS  PubMed  Google Scholar 

  79. Teitelbaum DH, Qualman SJ, Caniano DA. Hirschsprung’s disease. Identification of risk factors for enterocolitis. Ann Surg. 1988;207(3):240–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. 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(1):209–12.

    Article  PubMed  Google Scholar 

  81. Thiagarajah JR, Yildiz H, Carlson T, Thomas AR, Steiger C, Pieretti A, Zukerberg LR, Carrier RL, Goldstein AM. Altered goblet cell differentiation and surface mucus properties in Hirschsprung disease. PLoS One. 2014;9(6):e99944.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  82. Thomas DF, Fernie DS, Bayston R, Spitz L, Nixon HH. Enterocolitis in Hirschsprung’s disease: a controlled study of the etiologic role of Clostridium difficile. J Pediatr Surg. 1986;21(1):22–5.

    Article  CAS  PubMed  Google Scholar 

  83. Thomas DF, Fernie DS, Malone M, Bayston R, Spitz L. Association between Clostridium difficile and enterocolitis in Hirschsprung’s disease. Lancet. 1982;1(8263):78–9.

    Article  CAS  PubMed  Google Scholar 

  84. van Leeuwen K, Teitelbaum DH, Elhalaby EA, Coran AG. Long-term follow-up of redo pull-through procedures for Hirschsprung’s disease: efficacy of the endorectal pull-through. J Pediatr Surg. 2000;35(6):829–33; discussion 833-824

    Article  PubMed  Google Scholar 

  85. Wang X, Li Z, Xu Z, Wang Z, Feng J. Probiotics prevent Hirschsprung’s disease-associated enterocolitis: a prospective multicenter randomized controlled trial. Int J Color Dis. 2015;30(1):105–10.

    Article  Google Scholar 

  86. Ward NL, Pieretti A, Dowd SE, Cox SB, Goldstein AM. Intestinal aganglionosis is associated with early and sustained disruption of the colonic microbiome. Neurogastroenterol Motil. 2012;24(9):874–e400.

    Article  CAS  PubMed  Google Scholar 

  87. Webster W. Aganglionic megacolon in piebald-lethal mice. Arch Pathol. 1974;97(2):111–7.

    CAS  PubMed  Google Scholar 

  88. Wiedenmann B, Waldherr R, Buhr H, Hille A, Rosa P, Huttner WB. Identification of gastroenteropancreatic neuroendocrine cells in normal and neoplastic human tissue with antibodies against synaptophysin, chromogranin a, secretogranin I (chromogranin B), and secretogranin II. Gastroenterology. 1988;95(5):1364–74.

    Article  CAS  PubMed  Google Scholar 

  89. Wildhaber BE, Pakarinen M, Rintala RJ, Coran AG, Teitelbaum DH. Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung’s disease. J Pediatr Surg. 2004;39(6):920–6; discussion 920–926

    Article  PubMed  Google Scholar 

  90. Wildhaber BE, Teitelbaum DH, Coran AG. Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg. 2005;40(1):203–6; discussion 206-207

    Article  PubMed  Google Scholar 

  91. Wiles TJ, Jemielita M, Baker RP, Schlomann BH, Logan SL, Ganz J, Melancon E, Eisen JS, Guillemin K, Parthasarathy R. Host gut motility promotes competitive exclusion within a model intestinal microbiota. PLoS Biol. 2016;14(7):e1002517.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  92. Wilson-Storey D, Scobie WG. Impaired gastrointestinal mucosal defense in Hirschsprung’s disease: a clue to the pathogenesis of enterocolitis? J Pediatr Surg. 1989;24(5):462–4.

    Article  CAS  PubMed  Google Scholar 

  93. Wilson-Storey D, Scobie WG, McGenity KG. Microbiological studies of the enterocolitis of Hirschsprung’s disease. Arch Dis Child. 1990;65(12):1338–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  94. Wilson-Storey D, Scobie WG, Raeburn JA. Defective white blood cell function in Hirschsprung’s disease: a possible predisposing factor to enterocolitis. J R Coll Surg Edinb. 1988;33(4):185–8.

    CAS  PubMed  Google Scholar 

  95. Yan Z, Poroyko V, Gu S, Zhang Z, Pan L, Wang J, Bao N, Hong L. Characterization of the intestinal microbiome of Hirschsprung’s disease with and without enterocolitis. Biochem Biophys Res Commun. 2014;445(2):269–74.

    Article  CAS  PubMed  Google Scholar 

  96. Zaitoun I, Erickson CS, Barlow AJ, Klein TR, Heneghan AF, Pierre JF, Epstein ML, Gosain A. Altered neuronal density and neurotransmitter expression in the ganglionated region of Ednrb null mice: implications for Hirschsprung’s disease. Neurogastroenterol Motil. 2013;25(3):e233–44.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  97. Zhao L, Dhall D, Cheng Z, Wang HL, Doherty TM, Bresee C, Frykman PK. Murine model of Hirschsprung-associated enterocolitis II: surgical correction of aganglionosis does not eliminate enterocolitis. J Pediatr Surg. 2010;45(1):206–11; discussion 211-202

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, TN, USA

    Laura V. Veras & Ankush Gosain

  2. Children’s Foundation Research Institute, Le Bonheur Children’s Hospital, Memphis, TN, USA

    Ankush Gosain

Authors
  1. Laura V. Veras
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ankush Gosain
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ankush Gosain .

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

Veras, L.V., Gosain, A. (2019). Hirschsprung-Associated Enterocolitis. In: Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-15647-3_13

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-15647-3_13

  • 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