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Hirschsprung-Associated Enterocolitis

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Abstract

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

Daniel H. Teitelbaum passed away in 2016

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Authors and Affiliations

  1. Section of Pediatric Surgery, C.S. Mott Children’s Hospital and the University of Michigan School of Medicine, 1500 E. Medical Center Dr., Ann Arbor, MI, 48109, 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 Farokh R. Demehri .

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Editors and Affiliations

  1. Our Lady’s Children's Hospital, National Children’s Research Centre, Dublin, Ireland

    Prem Puri

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Demehri, F.R., Halaweish, I.F., Coran, A.G., Teitelbaum, D.H. (2017). Hirschsprung-Associated Enterocolitis. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_37-1

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  • DOI: https://doi.org/10.1007/978-3-642-38482-0_37-1

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  • Print ISBN: 978-3-642-38482-0

  • Online ISBN: 978-3-642-38482-0

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