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”.
Access provided by Autonomous University of Puebla. Download to read the full chapter text
Chapter PDF
Similar content being viewed by others
Keywords
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
References
Carneiro PMR, Brereton RJ, Drake DP, et al (1992) Enterocolitis in Hirschsprung’s disease. Pediatr Surg Int 7:356–360
Lister T, Tam PKH (1990) Hirschsprung’s disease. In: Lister J, Irving IM (eds) Neonatal surgery. Butterworth, London, pp 523–546
Jung PM (1995) Hirschsprung’s disease: one surgeon’s experience in one institution. J Pediatr Surg 30:646–651
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
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
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
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
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
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
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
Foster P, Cowan G, Wrenn EL Jr (1990) Twenty-five years’ experience with Hirschsprung’s disease. J Pediatr Surg 25:531–534
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
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
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
Nixon HH (1982) Hirschsprung’s disease in the newborn. In: Holschneider AM (ed) Hirschsprung’s disease. Hippokrates Verlag, Stuttgart, pp 103–113
Burnard ED (1950) Hirschsprung’s disease in infancy. Br Med J 1:151–156
Fisher JH, Swenson O (1956) Hirschsprung’s disease during infancy. Surg Clin North Am 103:1511–1515
Dorman GW (1957) Hirschsprung’s disease; a lethal problem in early infancy. AMA Arch Surg 75:906–913
Bill AH, Chapman ND (1962) The enterocolitis of Hirschsprung’s disease: its natural history and treatment. Am J Surg 103:70–74
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
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
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
Ikeda K, Goto S (1984) Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg 199:400–405
Caniano DA, Teitelbaum DH, Qualman SJ (1990) Management of Hirschsprung’s disease in children with trisomy 21. Am J Surg 159:402–404
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
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
Lloyd-Still JD, Demers LM (1978) Hirschsprung’s enterocolitis, prostaglandins and response to cholestyramine. J Pediatr Surg 13:417–418
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
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
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
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
Teitelbaum DH, Caniano DA, Qualman SJ (1989) The pathophysiology of Hirschsprung’s-associated enterocolitis: importance of histologic correlates. J Pediatr Surg 24:1271–1277
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
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
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
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
Buisine MP, Devisme L, Savidge TC, et al (1998) Mucin gene expression in human embryonic and fetal intestine. Gut 43:519–524
Mattar AF, Coran AG, Teitelbaum DH (2003) Hirschsprung’s disease: possible association with enterocolitis development. J Pediatr Surg 38:417–421
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
Richardson J (1975) Pharmacologic studies of Hirschsprung’s disease on a murine model. J Pediatr Surg 10:875–884
Webster W (1974) Aganglionic megacolon in piebald-lethal mice. Arch Pathol 97:111–117
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
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
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
Imamura A, Puri P, O’Briain DS, Reen DJ (1992) Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut 33:801–806
Brown WR, Isobe Y, Nakane PK (1976) Studies on translocation of immunoglobulins across the intestinal epithelium. Gastroenterology 71:985–995
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
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
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
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
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
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
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
Burgio GR, Ugazio A, Nespoli L, Maccario R (1983) Down syndrome: a model of immunodeficiency. Birth Defects Orig Artic Ser 19:325–327
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
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
Thomas DF, Fernie DS, Malone M, et al (1982) Association between Clostridium difficile and enterocolitis in Hirschsprung’s disease. Lancet 1:78–79
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
Hardy SP, Bayston R, Spitz L (1993) Prolonged carriage of Clostridium difficile in Hirschsprung’s disease. Arch Dis Child 69:221–224
Wilson-Storey D, Scobie WG, McGenity KG (1990) Microbiological studies of the enterocolitis of Hirschsprung’s disease. Arch Dis Child 65:1338–1339
Wilson-Storey D (1994) Microbial studies of enterocolitis in Hirschsprung’s disease. Pediatr Surg Int 9:248–250
Bagwell CE, Langham MR, Mahaffey SM, et al (1992) Pseudomembranous colitis following resection for Hirschsprung’s disease. J Pediatr Surg 27:1261–1264
Golderman L, Kaplan B, Rubinstein E (1985) Escherichia coli adherence to the intestine of mice. Isr J Med Sci 21:410–414
Hirschsprung H (1887) Stuhtragheit Neugeborener infolge Dilatationen und hypertrophie des Colons. Jahruch Kinderheikunde 27:1
Teitelbaum DH, Qualman SJ, Caniano DA (1988) Hirschsprung’s disease. Identification of risk factors for enterocolitis. Ann Surg 207:240–244
Nixon HH (1985) Hirschsprung’s disease: progress in management and diagnostics. World J Surg 9:189–202
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
Wildhaber BE, Teitelbaum DH, Coran AG (2005) Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg 40:203–206
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
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
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
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
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
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
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
Burgio GR, Ugazio A, Nespoli L, Maccario R (1983) Down syndrome: a model of immunodeficiency. Birth Defects Orig Artic Ser 19:325–327
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
Klein MD, Coran AG, Wesley JR, et al (1984) Hirschsprung’s disease in the newborn. J Pediatr Surg 19:370–374
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
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
Menardi G (1982) Hirschsprung’s disease in the newborn. In: Holschneider AM (ed) Hirschsprung’s disease. Hippokrates Verlag, Stuttgart, pp 125–131
Shim WK, Swenson O (1966) Treatment of congenital megacolon in 50 infants. Pediatrics 38:185–193
Menezes M, Puri P (2006) Longterm outcome of patients with enterocolitis complicating Hirschsprung’s disease. Pediatr Surg Int 22:316–318
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
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
Murphy F, Puri P (2005) New insights into the pathogenesis of Hirschsprung’s associated enterocolitis. Pediatr Surg Int 21:773–779
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2008 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-540-33935-9_10
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-33934-2
Online ISBN: 978-3-540-33935-9
eBook Packages: MedicineMedicine (R0)