Transition Zone Pull-through in Patients with Hirschsprung Disease: Is Redo Surgery Beneficial for the Long-term Outcomes?

Lieke Beltman, Hosnieya Labib, Hafsa Ahmed, Marc Benninga, Joris Roelofs, Patrick van der Voorn, Joost van Schuppen, Jaap Oosterlaan, Ernest van Heurn, Joep Derikx

Research output: Contribution to JournalArticleAcademicpeer-review

Abstract

Introduction: Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients. Methods: We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up. Results: Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12–260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63). Conclusion: Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.
Original languageEnglish
Pages (from-to)1903-1909
JournalJournal of Pediatric Surgery
Volume58
Issue number10
DOIs
Publication statusPublished - 1 Oct 2023
Externally publishedYes

Funding

None of the authors have any conflicts-of-interest to disclose. This study was supported with a grant from the Dutch patient association for Hirschsprung disease .

FundersFunder number
Dutch patient association for Hirschsprung disease

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