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Enterocolitis Complicating Hirschsprung’s Disease

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Abstract

Enterocolitis is a clinical condition with symptoms including diarrhea, abdominal distension, pyrexia, colicky abdominal pain, lethargy and the passage of blood-stained stools [1]. Enterocolitis is a significant complication of Hirschsprung’s disease (HD) both in the pre- and postoperative period [2]. Hirschsprung’s-associated enterocolitis (HAEC) can occur at any time from the neonatal period onwards into adulthood and can be independent of the medical management and surgical procedure performed. Recurrent enterocolitis can occur even in the presence of a diverting colostomy which is termed “diversion enterocolitis”.

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References

  1. Carneiro PMR, Brereton RJ, Drake DP, et al (1992) Enterocolitis in Hirschsprung’s disease. Pediatr Surg Int 7:356–360

    Article  Google Scholar 

  2. Lister T, Tam PKH (1990) Hirschsprung’s disease. In: Lister J, Irving IM (eds) Neonatal surgery. Butterworth, London, pp 523–546

    Google Scholar 

  3. Jung PM (1995) Hirschsprung’s disease: one surgeon’s experience in one institution. J Pediatr Surg 30:646–651

    Article  PubMed  CAS  Google Scholar 

  4. Marty TL, Seo T, Matlak ME, Sullivan JJ, Black RE, Johnson DG (1995) Gastrointestinal function after surgical correction of Hirschsprung’s disease: long-term follow-up in 135 patients. J Pediatr Surg 30:655–658

    Article  PubMed  CAS  Google Scholar 

  5. Swenson O, Sherman JO, Fisher JH, Cohen E (1975) The treatment and postoperative complications of congenital megacolon: a 25 year follow up. Ann Surg 182:266–273

    Article  PubMed  CAS  Google Scholar 

  6. Carcassonne M, Guys JM, Morrison-Lacombe G, Kreitmann B (1989) Management of Hirschsprung’s disease: curative surgery before 3 months of age. J Pediatr Surg 24:1032–1034

    Article  PubMed  CAS  Google Scholar 

  7. Minford JL, Ram A, Turnock RR, et al (2004) Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung’s disease. J Pediatr Surg 39:161–165

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  9. Surana R, Quinn FM, Puri P (1994) Evaluation of risk factors in the development of enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int 9:234–236

    Article  Google Scholar 

  10. Marty TL, Seo T, Sullivan JJ, Matlak ME, Black RE, Johnson DG (1995) Rectal irrigations for the prevention of postoperative enterocolitis in Hirschsprung’s disease. Pediatr Surg 30:652–654

    Article  CAS  Google Scholar 

  11. Foster P, Cowan G, Wrenn EL Jr (1990) Twenty-five years’ experience with Hirschsprung’s disease. J Pediatr Surg 25:531–534

    Article  PubMed  CAS  Google Scholar 

  12. Sherman JO, Snyder ME, Weitzman JJ, et al (1989) A 40-year multinational retrospective study of 880 Swenson procedures. J Pediatr Surg 24:833–838

    Article  PubMed  CAS  Google Scholar 

  13. Suita S, Taguchi T, Ieiri S, et al (2005) Hirschsprung’s dis­ease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatr Surg 40:197–201

    Article  PubMed  Google Scholar 

  14. Kleinhaus S, Boley SJ, Sheran M, et al (1979) Hirschsprung’s disease: a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg 14:588–597

    Article  PubMed  CAS  Google Scholar 

  15. Nixon HH (1982) Hirschsprung’s disease in the newborn. In: Holschneider AM (ed) Hirschsprung’s disease. Hippokrates Verlag, Stuttgart, pp 103–113

    Google Scholar 

  16. Burnard ED (1950) Hirschsprung’s disease in infancy. Br Med J 1:151–156

    PubMed  CAS  Google Scholar 

  17. Fisher JH, Swenson O (1956) Hirschsprung’s disease during infancy. Surg Clin North Am 103:1511–1515

    Google Scholar 

  18. Dorman GW (1957) Hirschsprung’s disease; a lethal problem in early infancy. AMA Arch Surg 75:906–913

    PubMed  CAS  Google Scholar 

  19. Bill AH, Chapman ND (1962) The enterocolitis of Hirschsprung’s disease: its natural history and treatment. Am J Surg 103:70–74

    Article  Google Scholar 

  20. Fujimoto T, Miyano T (1994) Abnormal expression of the blood group antigen (BGA) in colon of Hirschsprung’s dis­ease. Pediatr Surg Int 9:242–247

    Article  Google Scholar 

  21. Wilson-Storey D, Scobie WG (1989) Impaired gastrointestinal mucosal defense in Hirschsprung’s disease: a clue to the pathogenesis of enterocolitis? J Pediatr Surg 24:462–464

    Article  PubMed  CAS  Google Scholar 

  22. Wilson-Storey D, Scobie WG, Raeburn JA (1988) Defective white blood cell function in Hirschsprung’s disease: a possible predisposing factor to enterocolitis. J R Coll Surg Edinb 33:185–188

    PubMed  CAS  Google Scholar 

  23. Ikeda K, Goto S (1984) Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg 199:400–405

    Article  PubMed  CAS  Google Scholar 

  24. Caniano DA, Teitelbaum DH, Qualman SJ (1990) Management of Hirschsprung’s disease in children with trisomy 21. Am J Surg 159:402–404

    Article  PubMed  CAS  Google Scholar 

  25. Ament ME, Bill AH (1973) Persistent diarrhea due to sucrase-isomaltase deficiency in a postoperative child with Hirschsprung’s disease. J Pediatr Surg 8:543–545

    Article  PubMed  CAS  Google Scholar 

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

    Article  Google Scholar 

  27. Lloyd-Still JD, Demers LM (1978) Hirschsprung’s enterocolitis, prostaglandins and response to cholestyramine. J Pediatr Surg 13:417–418

    Article  PubMed  CAS  Google Scholar 

  28. Szulman AE, Marcus DM (1973) The histologic distribution of the blood group substances in man as disclosed by immunofluorescence. VI. The Le and Le antigens during fetal development. Lab Invest 28:565–574

    PubMed  CAS  Google Scholar 

  29. Sieber WK (1986) Hirschsprung’s disease In: Welch KJ, Randolph JG, Ravitch MM, et al (eds) Pediatric surgery. Year Book Medical Publishers, Chicago, pp 995–1016

    Google Scholar 

  30. Akkary S, Sahwy E, Kandil W, Hamdy MH (1981) A histochemical study of the mucosubstances of the colon in cases of Hirschsprung’s disease with and without enterocolitis. J Pediatr Surg 16:664–668

    Article  PubMed  CAS  Google Scholar 

  31. Fujimoto T, Puri P (1988) Persistence of enterocolitis following diversion of the faecal stream in Hirschsprung’s disease. A study of mucosal defence mechanism. Pediatr Surg Int 3:141–146

    Article  Google Scholar 

  32. Teitelbaum DH, Caniano DA, Qualman SJ (1989) The pathophysiology of Hirschsprung’s-associated enterocolitis: importance of histologic correlates. J Pediatr Surg 24:1271–1277

    Article  PubMed  CAS  Google Scholar 

  33. Aslam A, Spicer RD, Corfield AP (1997) Children with Hirschsprung’s disease have an abnormal colonic mucus defensive barrier independent of the bowel innervation status. J Pediatr Surg 32:1206–1210

    Article  PubMed  CAS  Google Scholar 

  34. Aslam A, Spicer RD, Corfield AP (1998) Turnover of radioactive mucin precursors in the colon of patients with Hirschsprung’s disease correlates with the development of enterocolitis. J Pediatr Surg 33:103–105

    Article  PubMed  CAS  Google Scholar 

  35. Aslam A, Spicer RD, Corfield AP (1999) Histochemical and genetic analysis of colonic mucin glycoproteins in Hirschsprung’s disease. J Pediatr Surg 34:330–333

    Article  PubMed  CAS  Google Scholar 

  36. Gork AS, Usui N, Ceriati E, et al (1999) The effect of mucin on bacterial translocation in I-407 fetal and Caco-2 adult enterocyte cultured cell lines. Pediatr Surg Int 15:155–159

    Article  PubMed  CAS  Google Scholar 

  37. Buisine MP, Devisme L, Savidge TC, et al (1998) Mucin gene expression in human embryonic and fetal intestine. Gut 43:519–524

    Article  PubMed  CAS  Google Scholar 

  38. Mattar AF, Coran AG, Teitelbaum DH (2003) Hirsch­sprung’s disease: possible association with enterocolitis development. J Pediatr Surg 38:417–421

    Article  PubMed  Google Scholar 

  39. Albanese CT, Smith SD, Watkins S, et al (1994) Effect of secretory IgA on transepithelial passage of bacteria across the intact ileum in vitro. J Am Coll Surg 179:679–688

    PubMed  CAS  Google Scholar 

  40. Richardson J (1975) Pharmacologic studies of Hirsch­sprung’s disease on a murine model. J Pediatr Surg 10:875–884

    Article  PubMed  CAS  Google Scholar 

  41. Webster W (1974) Aganglionic megacolon in piebald-lethal mice. Arch Pathol 97:111–117

    PubMed  CAS  Google Scholar 

  42. Bulock A, Vallant C, Dockray GJ (1984) Selective depletion of substance P immunoreactive neurons in the transitional zone of the colon in piebald lethal mice. Neurochem Int 6:55–61

    Article  CAS  Google Scholar 

  43. Fujimoto T (1988) Natural history and pathophysiology of enterocolitis in the piebald lethal mouse model of Hirschsprung’s disease. J Pediatr Surg 23:237–242

    Article  PubMed  CAS  Google Scholar 

  44. Fujimoto T, Reen DJ, Puri P (1988) Inflammatory response in enterocolitis in the piebald lethal mouse model of Hirschsprung’s disease. Pediatr Res 24:152–155

    Article  PubMed  CAS  Google Scholar 

  45. Imamura A, Puri P, O’Briain DS, Reen DJ (1992) Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut 33:801–806

    Article  PubMed  CAS  Google Scholar 

  46. Brown WR, Isobe Y, Nakane PK (1976) Studies on translocation of immunoglobulins across the intestinal epithelium. Gastroenterology 71:985–995

    PubMed  CAS  Google Scholar 

  47. Turnock RR, Spitz L, Strobel S (1992) A study of mucosal gut immunity in infants who develop Hirschsprung’s-associated enterocolitis. Pediatr Surg 27:828–829

    Article  CAS  Google Scholar 

  48. O’Briain DS, Dayal Y (1981) The pathology of the gastrointestinal endocrine cells. In: De Lellis RA (ed) Diagnostic immunocytochemistry. Masson, New York, pp 75–109

    Google Scholar 

  49. Wiedenmann B, Waldherr R, Buhr H, et al (1988) 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 95:1364–1374

    PubMed  CAS  Google Scholar 

  50. Soeda J, O’Briain DS, Puri P (1992) Mucosal neuroendocrine cell abnormalities in the colon of patients with Hirschsprung’s disease. J Pediatr Surg 27:823–827

    Article  PubMed  CAS  Google Scholar 

  51. Soeda J, O’Briain DS, Puri P (1993) Regional reduction in intestinal neuroendocrine cell populations in enterocolitis complicating Hirschsprung’s disease. J Pediatr Surg 28:1063–1068

    Article  PubMed  CAS  Google Scholar 

  52. Levin S (1987) The immune system and susceptibility of infections in Down’s syndrome. In: McCoy EE, Epstein CJ (eds) Oncology and immunology in Down’s syndrome. Liss, New York, pp 143–162

    Google Scholar 

  53. Nair MPN, Schwartz SA (1984) Association of decreased T cell mediated natural cytotoxicity and inferno production in Down’s Syndrome. Clin Immunol Immunopathol 33:412–424

    Article  PubMed  CAS  Google Scholar 

  54. Burgio GR, Ugazio A, Nespoli L, Maccario R (1983) Down syndrome: a model of immunodeficiency. Birth Defects Orig Artic Ser 19:325–327

    PubMed  CAS  Google Scholar 

  55. Kobayashi H, Hirakawa H, O’Briain DS, Puri P (1994) Intracellular adhesion molecule-1 (ICAM-1) in the pathogenesis of enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int 9:237–241

    Article  Google Scholar 

  56. Suzuki T, Won KJ, Horiguchi K, Kinoshita K, et al (2004) Muscularis inflammation and the loss of interstitial cells of Cajal in the endothelin ETB receptor null rat. Am J Physiol Gastrointest Liver Physiol 287:638–646

    Article  Google Scholar 

  57. Thomas DF, Fernie DS, Malone M, et al (1982) Association between Clostridium difficile and enterocolitis in Hirschsprung’s disease. Lancet 1:78–79

    Article  PubMed  CAS  Google Scholar 

  58. Thomas DF, Fernie DS, Bayston R, et al (1986) Enterocolitis in Hirschsprung’s disease: a controlled study of the etiologic role of Clostridium difficile. J Pediatr Surg 21:22–25

    Article  PubMed  CAS  Google Scholar 

  59. Hardy SP, Bayston R, Spitz L (1993) Prolonged carriage of Clostridium difficile in Hirschsprung’s disease. Arch Dis Child 69:221–224

    PubMed  CAS  Google Scholar 

  60. Wilson-Storey D, Scobie WG, McGenity KG (1990) Microbiological studies of the enterocolitis of Hirschsprung’s dis­ease. Arch Dis Child 65:1338–1339

    Article  PubMed  CAS  Google Scholar 

  61. Wilson-Storey D (1994) Microbial studies of enterocolitis in Hirschsprung’s disease. Pediatr Surg Int 9:248–250

    Article  Google Scholar 

  62. Bagwell CE, Langham MR, Mahaffey SM, et al (1992) Pseudomembranous colitis following resection for Hirschsprung’s disease. J Pediatr Surg 27:1261–1264

    Article  PubMed  CAS  Google Scholar 

  63. Golderman L, Kaplan B, Rubinstein E (1985) Escherichia coli adherence to the intestine of mice. Isr J Med Sci 21:410–414

    PubMed  CAS  Google Scholar 

  64. Hirschsprung H (1887) Stuhtragheit Neugeborener infolge Dilatationen und hypertrophie des Colons. Jahruch Kinderheikunde 27:1

    Google Scholar 

  65. Teitelbaum DH, Qualman SJ, Caniano DA (1988) Hirschsprung’s disease. Identification of risk factors for enterocolitis. Ann Surg 207:240–244

    Article  PubMed  CAS  Google Scholar 

  66. Nixon HH (1985) Hirschsprung’s disease: progress in management and diagnostics. World J Surg 9:189–202

    Article  PubMed  CAS  Google Scholar 

  67. Menezes M, Corbally M, Puri P (2006) Long-term results of bowel function after treatment of Hirschsprung’s dis­ease: a 29-year review. Pediatr Surg Int 22:987-990

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  71. Polley TZ, Coran AG, Wesley JR (1986) The definitive management of Hirschsprung’s disease with endorectal pull through procedure. Pediatr Surg Int 1:90–94

    Article  Google Scholar 

  72. Menezes M, Puri P (2005) Long-term clinical outcome in patients with Hirschsprung’s disease and associated Down’s syndrome. J Pediatr Surg 40:810–812

    Article  PubMed  Google Scholar 

  73. Quinn FM, Surana R, Puri P (1994) The influence of trisomy 21 on outcome in children with Hirschsprung’s dis­ease. J Pediatr Surg 29:781–783

    Article  PubMed  CAS  Google Scholar 

  74. Levin S (1987) The immune system and susceptibility of infections in Down’s Syndrome. In: McCoy EE, Epstein CJ (eds) Oncology and immunology in Down’s syndrome. Liss, New York, pp 143–162

    Google Scholar 

  75. Nair MPN, Schwartz SA (1984) Association of decreased T cell mediated natural cytotoxicity and inferno production in Down’s Syndrome. Clin Immunol Immunopathol 33:412–424

    Article  PubMed  CAS  Google Scholar 

  76. Burgio GR, Ugazio A, Nespoli L, Maccario R (1983) Down syndrome: a model of immunodeficiency. Birth Defects Orig Artic Ser 19:325–327

    PubMed  CAS  Google Scholar 

  77. Hackam DJ, Filler RM, Pearl RH (1998) Enterocolitis after the surgical treatment of Hirschsprung’s disease: risk factors and financial impact. J Pediatr Surg 33:830–833

    Article  PubMed  CAS  Google Scholar 

  78. Klein MD, Coran AG, Wesley JR, et al (1984) Hirschsprung’s disease in the newborn. J Pediatr Surg 19:370–374

    Article  PubMed  CAS  Google Scholar 

  79. Puri P, Lake BD, Nixon HH, et al (1977) Neuronal colonic dysplasia: an unusual association of Hirschsprung’s disease. J Pediatr Surg 12:681–685

    Article  PubMed  CAS  Google Scholar 

  80. Kobayashi H, Hirakawa H, Surana R, et al (1995) Intestinal neuronal dysplasia is a possible cause of persistent bowel symptoms after pull-through operation for Hirschsprung’s disease. J Pediatr Surg 30:253–257

    Article  PubMed  CAS  Google Scholar 

  81. Menardi G (1982) Hirschsprung’s disease in the newborn. In: Holschneider AM (ed) Hirschsprung’s disease. Hippokrates Verlag, Stuttgart, pp 125–131

    Google Scholar 

  82. Shim WK, Swenson O (1966) Treatment of congenital megacolon in 50 infants. Pediatrics 38:185–193

    PubMed  CAS  Google Scholar 

  83. Menezes M, Puri P (2006) Longterm outcome of patients with enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int 22:316–318

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  85. Rintala RJ, Lindahl H (2001) Sodium cromoglycate in the management of chronic or recurrent enterocolitis in patients with Hirschsprung’s disease. J Pediatr Surg 36:1032–1035

    Article  PubMed  CAS  Google Scholar 

  86. Murphy F, Puri P (2005) New insights into the pathogenesis of Hirschsprung’s associated enterocolitis. Pediatr Surg Int 21:773–779

    Article  PubMed  Google Scholar 

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Murphy, F., Menezes, M., Puri, P. (2008). Enterocolitis Complicating Hirschsprung’s Disease. In: Holschneider, A., Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-33935-9_10

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