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Pediatric Surgery pp 1031-1043 | Cite as

Hirschsprung-Associated Enterocolitis

  • Farokh R. DemehriEmail author
  • Ihab F. Halaweish
  • Arnold G. Coran
  • Daniel H. Teitelbaum
Reference work entry

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.

Keywords

Hirschsprung’s disease Enterocolitis HAEC Pathogenesis Prevention Treatment 

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  • Farokh R. Demehri
    • 1
    Email author
  • Ihab F. Halaweish
    • 1
  • Arnold G. Coran
    • 1
  • Daniel H. Teitelbaum
    • 1
  1. 1.Section of Pediatric SurgeryC.S. Mott Children’s Hospital and the University of Michigan School of MedicineAnn ArborUSA

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