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01-09-2013 | Review Article

Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention

Authors: Farokh R. Demehri, Ihab F. Halaweish, Arnold G. Coran, Daniel H. Teitelbaum

Published in: Pediatric Surgery International | Issue 9/2013

Abstract

Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung 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 or redo pull-through. This chapter describes the incidence, pathogenesis, treatment, and preventative strategies in management of HAEC.

Aslam A, Spicer RD et al (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(1):103–105PubMedCrossRef

Aslam A, Spicer RD et al (1999) Histochemical and genetic analysis of colonic mucin glycoproteins in Hirschsprung’s disease. J Pediatr Surg 34(2):330–333PubMedCrossRef

Austin KM (2012) The pathogenesis of Hirschsprung’s disease-associated enterocolitis. Semin Pediatr Surg 21(4):319–327PubMedCrossRef

Bagwell CE, Langham M Jr et al (1992) Pseudomembranous colitis following resection for Hirschsprung’s disease. J Pediatr Surg 27(10):1261–1264PubMedCrossRef

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

Caniano DA, Teitelbaum DH et al (1989) The piebald-lethal murine strain: investigation of the cause of early death. J Pediatr Surg 24(9):906–910PubMedCrossRef

Coran AG, Teitelbaum DH (2000) Recent advances in the management of Hirschsprung’s disease. Am J Surg 180(5):382–387PubMedCrossRef

De Filippo C, Cavalieri D et al (2010) Impact of diet in shaping gut microbiota revealed by a comparative study in children from Europe and rural Africa. Proc Natl Acad Sci USA 107(33):14691–14696PubMedCrossRef

El-Sawaf M, Siddiqui S et al (2013) Probiotic prophylaxis after pullthrough for Hirschsprung disease to reduce incidence of enterocolitis: a prospective, randomized, double-blind, placebo-controlled, multicenter trial. J Pediatr Surg 48(1):111–117PubMedCrossRef

10.

Elhalaby E, Coran A et al (1995) Enterocolitis associated with Hirschsprung’s disease: a clinical-radiological characterization based on 168 patients. J Pediatr Surg 30(1):76–83PubMedCrossRef

11.

Elhalaby EA, Teitelbaum DH et al (1995) Enterocolitis associated with Hirschsprung’s disease: a clinical histopathological correlative study. J Pediatr Surg 30(7):1023–1026 (discussion 1026–1027)

12.

Engum SA, Grosfeld JL (2004) Long-term results of treatment of Hirschsprung’s disease. Semin Pediatr Surg 13(4):273–285PubMedCrossRef

13.

Estevao-Costa J, Carvalho JL et al (1999) Risk factors for the development of enterocolitis after pull-through for Hirschsprung’s disease (comment). J Pediatr Surg 34(10):1581–1582PubMedCrossRef

14.

Fisher JH, Swenson O (1956) Hirschsprung’s disease during infancy. Surg Clin North Am 36:1511–1515

15.

Hackam DJ, Filler RM et al (1998) Enterocolitis after the surgical treatment of Hirschsprung’s disease: risk factors and financial impact (see comment). J Pediatr Surg 33(6):830–833PubMedCrossRef

16.

Hardy SP, Bayston R et al (1993) Prolonged carriage of Clostridium difficile in Hirschsprung’s disease. Arch Dis Child 69(2):221–224PubMedCrossRef

17.

Haricharan RN, Seo JM et al (2008) Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not. J Pediatr Surg 43(6):1115–1123PubMedCrossRef

18.

Heikkinen M, Lindahl H et al (2005) Long-term outcome after internal sphincter myectomy for internal sphincter achalasia. Pediatr Surg Int 21(2):84–87PubMedCrossRef

19.

Imamura A, Puri P et al (1992) Mucosal immune defence mechanisms in enterocolitis complicating Hirschsprung’s disease. Gut 33(6):801–806PubMedCrossRef

20.

Lawal TA, Chatoorgoon K et al (2011) Redo pull-through in Hirschsprung’s (corrected) disease for obstructive symptoms due to residual aganglionosis and transition zone bowel. J Pediatr Surg 46(2):342–347PubMedCrossRef

21.

Levitt MA, Dickie B et al (2010) Evaluation and treatment of the patient with Hirschsprung disease who is not doing well after a pull-through procedure. Semin Pediatr Surg 19(2):146–153PubMedCrossRef

22.

Lui VC, Li L et al (2001) 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 1537(2):89–100PubMedCrossRef

23.

Marty TL, Matlak ME et al (1995) Unexpected death from enterocolitis after surgery for Hirschsprung’s disease. Pediatrics 96(1 Pt 1):118–121PubMed

24.

Marty TL, Seo T et al (1995) Rectal irrigations for the prevention of postoperative enterocolitis in Hirschsprung’s disease. J Pediatr Surg 30(5):652–654PubMedCrossRef

25.

Mattar A, Drongowski R et al (2003) MUC-2 mucin production in Hirschsprung’s disease: possible association with enterocolitis development. J Pediatr Surg 38(3):417–421PubMedCrossRef

26.

Messina M, Amato G et al (2007) Topical application of isosorbide dinitrate in patients with persistent constipation after pull-through surgery for Hirschsprung’s disease. Eur J Pediatr Surg 17(1):62–65PubMedCrossRef

27.

Minkes RK, Langer JC (2000) A prospective study of botulinum toxin for internal anal sphincter hypertonicity in children with Hirschsprung’s disease. J Pediatr Surg 35(12):1733–1736PubMedCrossRef

28.

Miyahara K, Kato Y et al (2009) Neuronal immaturity in normoganglionic colon from cases of Hirschsprung disease, anorectal malformation, and idiopathic constipation. J Pediatr Surg 44(12):2364–2368PubMedCrossRef

29.

Moore SW, Millar AJ et al (1994) Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung’s patient. J Pediatr Surg 29(1):106–111PubMedCrossRef

30.

Moore SW, Sidler D et al (2008) The ITGB2 immunomodulatory gene (CD18), enterocolitis, and Hirschsprung’s disease. J Pediatr Surg 43(8):1439–1444PubMedCrossRef

31.

Murphy F, Puri P (2005) New insights into the pathogenesis of Hirschsprung’s associated enterocolitis. Pediatr Surg Int 21(10):773–779PubMedCrossRef

32.

Pastor A, Osman F et al (2009) Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg 44(1):251–256PubMedCrossRef

33.

Polley T Jr, Coran A et al (1985) A ten-year experience with ninety-two cases of Hirschsprung’s disease. Including sixty-seven consecutive endorectal pull-through procedures. Ann Surg 202(3):349–355PubMedCrossRef

34.

Rescorla F, Morrison A et al (1992) Hirschsprung’s disease. Evaluation of mortality and long-term function in 260 cases. Arch Surg 127(8):934–941 (discussion 941–932)

35.

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(7):1032–1035PubMedCrossRef

36.

Shen DH, Shi CR et al (2009) Detection of intestinal bifidobacteria and lactobacilli in patients with Hirschsprung’s disease associated enterocolitis. World J Pediatr 5(3):201–205PubMedCrossRef

37.

Soeda J, O’Briain DS et al (1993) Regional reduction in intestinal neuroendocrine cell populations in enterocolitis complicating Hirschsprung’s disease. J Pediatr Surg 28(8):1063–1068PubMedCrossRef

38.

Staiano A, Santoro L et al (1999) Autonomic dysfunction in children with Hirschsprung’s disease. Dig Dis Sci 44(5):960–965Swenson O, Bill AH. (1948) Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing mega colon. Surgery. 24:212-220.PubMedCrossRef

39.

Swenson O, Bill AH (1948) Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing mega colon. Surgery 24:212–220PubMed

40.

Swenson O, Fisher J et al (1960) Diarrhea following rectosigmoidectomy for Hirschsprung’s disease. Surgery 48:419–421PubMed

41.

Swenson O, Sherman J et al (1975) The treatment and postoperative complications of congenital megacolon: a 25 year follow-up. Ann Surg 182:266–273PubMedCrossRef

42.

Teitelbaum D, Caniano D et al (1989) The pathophysiology of Hirschsprung’s-associated enterocolitis: importance of histologic correlates. J Pediatr Surg 24(12):1271–1277PubMedCrossRef

43.

Teitelbaum D, Qualman S et al (1988) Hirschsprung’s disease. Identification of risk factors for enterocolitis. Ann Surg 207(3):240–244PubMedCrossRef

44.

Teitelbaum DH, Coran AG (2013) Hirschprung disease. In: Spitz L, Coran AG (eds) Operative pediatric surgery, 7th edn. CRC Press, Taylor & Francis Group, LLC, Boca Raton, p 578

45.

Teitelbaum DH, Cilley R et al (2000) A decade experience with the primary pull-through for Hirschsprung’s disease in the newborn period: a multi-center analysis of outcomes. Ann Surg 232(3):372–380PubMedCrossRef

46.

Temple SJ, Shawyer A et al (2012) Is daily dilatation by parents necessary after surgery for Hirschsprung disease and anorectal malformations? J Pediatr Surg 47(1):209–212PubMedCrossRef

47.

Thomas DF, Fernie DS et al (1982) Association between Clostridium difficile and enterocolitis in Hirschsprung’s disease. Lancet 1(8263):78–79PubMedCrossRef

48.

Turnock RR, Spitz L et al (1992) A study of mucosal gut immunity in infants who develop Hirschsprung’s-associated enterocolitis. J Pediatr Surg 27(7):828–829PubMedCrossRef

49.

Vieten D, Spicer R (2004) Enterocolitis complicating Hirschsprung’s disease. Semin Pediatr Surg 13(4):263–272PubMedCrossRef

50.

Wildhaber B, Pakarinen M et al (2004) Posterior myotomy/myectomy for persistent stooling problems in Hirschsprung’s disease. J Ped Surg 39(6):920–926CrossRef

51.

Wilson-Storey D, Scobie W (1989) Impaired gastrointestinal mucosal defense in Hirschsprung’s disease: a clue to the pathogenesis of enterocolitis? J Pediatr Surg 24(5):462–464PubMedCrossRef

52.

Wilson-Storey D, Scobie WG et al (1990) Microbiological studies of the enterocolitis of Hirschsprung’s disease. Arch Dis Child 65(12):1338–1339PubMedCrossRef

Title: Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention
Authors: Farokh R. Demehri
Ihab F. Halaweish
Arnold G. Coran
Daniel H. Teitelbaum
Publication date: 01-09-2013
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Surgery International / Issue 9/2013
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-013-3353-1

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Springer Medicine

Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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