Risk factors for enterocolitis in patients with Hirschsprung disease: A retrospective observational study

Daniëlle Roorda*, Jaap Oosterlaan, Ernest van Heurn, Joep P.M. Derikx

*Corresponding author for this work

Research output: Contribution to JournalArticleAcademicpeer-review


Introduction: Hirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD). The aim of this study was to identify incidence of pre- and postoperative HAEC in our consecutive cohort and to identify patient and clinical characteristics that are associated with developing postoperative HAEC and HAEC-free interval. Material and methods: A retrospective cohort study was performed with all 146 HD patients treated between 2000 and 2017. Data were retrieved from the medical records. HAEC was defined as presence of clinical signs of bowel inflammation, that required treatment with intravenous antibiotics and admittance to the hospital during at least two days. To identify risk factor for HAEC, patients with and without a history of postoperative HAEC were compared. Kaplan-Meier and Cox-regression were used to assess HAEC free intervals before and after surgery. Results: Out of 146 patients, 12 patients had pre-operative HAEC (8%) and 31 patients had postoperative HAEC (21%). Median preoperative HAEC free interval was 112 days (IQR 182 days). Length of hospital stay due to readmissions was longer for patients with a history of postoperative HAEC compared to patients without a history of postoperative HAEC (9.5 vs 16 days, U = 1872.5, p = 0.047). Median postoperative HAEC free interval was 226 days. Of the patients who had postoperative HAEC, 66% had their first episode within the first year after surgery and that the incidence of HAEC declined over follow-up. Conclusions: HAEC incidence was relatively low in our population. No patient or clinical characteristics were associated with the risk of postoperative HAEC.

Original languageEnglish
Pages (from-to)1791-1798
Number of pages8
JournalJournal of Pediatric Surgery
Issue number10
Early online date26 Apr 2021
Publication statusPublished - Oct 2021

Bibliographical note

Funding Information:
This study was supported with a grant from the Dutch patient association for Hirschsprung disease. The funding source had nog involvement in the study design; in the collection, analysis and interpretation of data; in the writing of the report; and in the decision to submit the article for publication.

Publisher Copyright:
© 2021 The Authors

Copyright 2021 Elsevier B.V., All rights reserved.


  • Complications
  • Enterocolitis
  • Etiology
  • Hirschsprung disease
  • Risk factors
  • Surgery


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