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Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention

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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.

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Authors and Affiliations

  1. Section of Pediatric Surgery, C.S. Mott Children’s Hospital, University of Michigan Health System, 1540 E. Hospital Dr., SPC 4211, Ann Arbor, MI, 48109-4211, USA

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

  2. University of Michigan Medical School, Ann Arbor, MI, USA

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

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  1. Farokh R. Demehri
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  2. Ihab F. Halaweish
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  3. Arnold G. Coran
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  4. Daniel H. Teitelbaum
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Correspondence to Daniel H. Teitelbaum.

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Demehri, F.R., Halaweish, I.F., Coran, A.G. et al. Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int 29, 873–881 (2013). https://doi.org/10.1007/s00383-013-3353-1

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