Hirschsprung-associated enterocolitis in children treated at US children's hospitals

J Pediatr Surg. 2020 Mar;55(3):535-540. doi: 10.1016/j.jpedsurg.2019.10.060. Epub 2019 Nov 20.

Abstract

Background: The incidence of and risk factors for Hirschsprung's-associated enterocolitis (HAEC) following pull-through have been limited to single institutions studies. We characterized the incidence of, risk factors for, and consequences of post-operative HAEC.

Methods: We identified children with Hirschsprung's Disease (HD) at US Children's Hospitals from 2007 to 2017 with and an associated pull-through operation at less than 1 year of age. HAEC readmissions were identified using ICD9/10 Diagnosis Codes and antibiotic administration. Hierarchical logistic regression models were developed for the risk factors for HAEC after pull-through and effects of recurrent HAEC on HD-related reoperations.

Results: We identified 2030 children with HD, and 138 (7%) who had two or more readmissions related to HAEC. The frequency of recurrent HAEC by hospital ranged from 0 to 33%. Pre-operative HAEC, history of central nervous system infection, and congenital neurologic anomalies were associated with increased risk of recurrent HAEC. Recurrent HAEC was associated with HD-specific re-operation (OR 5.2, CI 3.3-8.1, p < 0.001); however, it was not associated with risk of in-hospital mortality (OR 3.3, CI 0.88-12.1, p = 0.08).

Conclusions: HAEC following pull-through occurs in a large proportion of infants with HD and predicts reoperation. Multicenter studies are needed to develop prediction models and treatment protocols for HAEC.

Level of evidence: II TYPE OF STUDY: Retrospective cohort study.

Keywords: Enterocolitis; Hirschsprung disease; Outcome.

MeSH terms

  • Enterocolitis* / epidemiology
  • Enterocolitis* / etiology
  • Hirschsprung Disease* / complications
  • Hirschsprung Disease* / epidemiology
  • Hirschsprung Disease* / surgery
  • Hospitals, Pediatric
  • Humans
  • Incidence
  • Infant
  • Postoperative Complications / epidemiology*
  • Retrospective Studies
  • Risk Factors