Risk factors for complications in patients with Hirschsprung disease while awaiting surgery: Beware of bowel perforation.

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  • Additional Information
    • Source:
      Publisher: Saunders Country of Publication: United States NLM ID: 0052631 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1531-5037 (Electronic) Linking ISSN: 00223468 NLM ISO Abbreviation: J Pediatr Surg Subsets: MEDLINE
    • Publication Information:
      Publication: Philadelphia, PA : Saunders
      Original Publication: New York.
    • Subject Terms:
      Enterocolitis*/epidemiology ; Enterocolitis*/etiology ; Hirschsprung Disease*/complications ; Hirschsprung Disease*/surgery ; Intestinal Perforation*/epidemiology ; Intestinal Perforation*/etiology ; Intestinal Perforation*/surgery; Humans ; Infant ; Laxatives ; Postoperative Complications/epidemiology ; Postoperative Complications/etiology ; Retrospective Studies ; Risk Factors
    • Abstract:
      Background: Patients with Hirschsprung disease (HD) mostly undergo surgery around the age of three to six months. While awaiting surgery, therapy to treat the obstruction such as transanal irrigation (TAI) or laxatives is applied. The aim of this study was to gain insight in the prevalence and severity of complications occurring while awaiting surgery and to identify patient characteristics associated with the development of these complications.
      Methods: This study retrospectively analyzed data of patients with HD operated in our center between 2000 and 2021. Complications emerging while awaiting surgery were graded using Clavien-Dindo (CD). Patient characteristics as predictor of a complication were tested using logistic regression analysis.
      Results: Twenty-two of 132 (17%) included patients (preoperative treatment: 94% TAI; 2% laxatives; 2% other therapy) developed 45 complications while awaiting surgery, including predominantly major complications (91%). Bowel perforation occurred most frequently (n = 9, 7%) wherefrom six caused by TAI (5%), including three patients with total colon aganglionosis (TCA) (2%) counting one life-threatening and one lethal perforation. The other perforations were caused by meconium ileus (n = 2) and Hirschsprung associated enterocolitis (HAEC) (n = 1). Other frequent complications were: sepsis (5%), ileus (4%) and persistent obstruction (4%). Predictive factor for developing complication was TCA (OR 9.905, CI 2.994-32.772, p < 0.001).
      Conclusion: We found a complication rate of 17% in patients while awaiting surgery, reporting bowel perforation most frequently. We found this complication in patients with TCA being highly dangerous causing one life-threatening and one lethal perforation. Therefore, we advise in patients with (suspected) TCA to limit the time awaiting surgery.
      Level of Evidence: level III.
      Competing Interests: Declaration of Competing Interest None of the authors have any conflicts-of-interest to disclose.
      (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)
    • Contributed Indexing:
      Keywords: Clavien-Dindo; Complications; Hirschsprung disease; Preoperative obstructive therapy; Transanal irrigation
    • Accession Number:
      0 (Laxatives)
    • Publication Date:
      Date Created: 20220331 Date Completed: 20221018 Latest Revision: 20221018
    • Publication Date:
      20221213
    • Accession Number:
      10.1016/j.jpedsurg.2022.02.022
    • Accession Number:
      35354528