Current understanding of Hirschsprung-associated enterocolitis: Pathogenesis, diagnosis and treatment

Semin Pediatr Surg. 2022 Apr;31(2):151162. doi: 10.1016/j.sempedsurg.2022.151162. Epub 2022 Apr 8.

Abstract

Hirschsprung-associated enterocolitis (HAEC) was described in 1886 by Harald Hirschsprung and is a potentially deadly complication of Hirschsprung Disease. HAEC is classically characterized by abdominal distension, fever, and diarrhea, although there can be a variety of other associated symptoms, including colicky abdominal pain, lethargy, and the passage of blood-stained stools. HAEC occurs both pre-operatively and post-operatively, is the presenting symptom of HSCR in up to 25% of infants and varies in overall incidence from 20 to 60%. This article reviews our current understanding of HAEC pathogenesis, diagnosis, and treatment with discussion of areas of ongoing research, controversy, and future investigation.

Keywords: Dysmotility; Enterocolitis; Hirschsprung disease; Microbiome; Mucosal immunity; Treatment.

Publication types

  • Review

MeSH terms

  • Enterocolitis* / diagnosis
  • Enterocolitis* / etiology
  • Enterocolitis* / therapy
  • Hirschsprung Disease* / complications
  • Hirschsprung Disease* / diagnosis
  • Hirschsprung Disease* / surgery
  • Humans
  • Infant