Did Age at Surgery Influence Outcome in Patients With Hirschsprung Disease? A Nationwide Cohort Study in the Netherlands

Danielle Roorda*, Sanne J. Verkuijl, Joep P. M. Derikx, Monika Trzpis, Rob J. Meinds, Cornelius E. J. Sloots, Marieke J. Witvliet, Ivo de Blaauw, Wim G. van Gemert, Lodewijk W. E. van Heurn, Paul M. A. Broens

*Corresponding author for this work

    Research output: Contribution to journalArticleAcademicpeer-review

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    Objectives: Hirschsprung disease (HD) requires surgical resection of affected bowel, but the current evidence is inconclusive regarding the optimal age for resection. The aim of this study was to assess whether age at resection of the aganglionic segment is a determinant for surgical outcomes. Methods: A cross-sectional cohort study was done including all consecutive patients with HD between 1957 and 2015, aged 8 years or older (n = 830), who were treated in 1 of the 6 pediatric surgical centers in the Netherlands. Outcome measures were mortality, postoperative complications, stoma rate and redo surgery rate, retrieved from the medical records. Additionally, constipation and fecal incontinence rate in long term were assessed with the Defecation and Continence Questionnaire (DeFeC and P-DeFeC). Results: The medical records of 830 patients were reviewed, and 346 of the 619 eligible patients responded to the follow-up questionnaires (56%). There was a small increase in the risk of a permanent stoma [odds ratio (OR) 1.01 (95% confidence interval {CI}: 1.00-1.02); P = 0.019] and a temporary stoma [OR 1.01 (95% CI: 1.00-1.01); P = 0.022] with increasing age at surgery, regardless of the length of the aganglionic segment and operation technique. Both adjusted and unadjusted for operation technique, length of disease, and temporary stoma, age at surgery was not associated with the probability and the severity of constipation and fecal incontinence in long term. Conclusions: In this study, we found no evidence that the age at surgery influences surgical outcomes, thus no optimal timing for surgery for HD could be determined.

    Original languageEnglish
    Pages (from-to)431-437
    Number of pages7
    JournalJournal of Pediatric Gastroenterology and Nutrition
    Issue number4
    Publication statusPublished - Oct-2022


    • complications
    • constipation
    • fecal incontinence
    • patient outcome assessment


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