Outcomes in Hirschsprung's disease with coexisting learning disability

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Davidson , JR , Kyrklund , K , Eaton , S , Pakarinen , MP , Thompson , D , Blackburn , SC , Cross , K , De Coppi , P & Curry , J 2021 , ' Outcomes in Hirschsprung's disease with coexisting learning disability ' , European journal of pediatrics , vol. 180 , no. 12 , pp. 3499-3507 . https://doi.org/10.1007/s00431-021-04129-5

Title: Outcomes in Hirschsprung's disease with coexisting learning disability
Author: Davidson, JR; Kyrklund, K; Eaton, S; Pakarinen, MP; Thompson, D; Blackburn, SC; Cross, K; De Coppi, P; Curry, J
Contributor organization: HUS Children and Adolescents
Children's Hospital
University Management
Lastenkirurgian yksikkö
Date: 2021-12
Language: eng
Number of pages: 9
Belongs to series: European journal of pediatrics
ISSN: 0340-6199
DOI: https://doi.org/10.1007/s00431-021-04129-5
URI: http://hdl.handle.net/10138/335728
Abstract: This study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung's disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala's BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half ofpatients surveyed (44-60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0-23]). Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.
Subject: Hirschsprung's
Learning disability
Down syndrome
Bowel function
Quality of life
Long-term outcomes
3123 Gynaecology and paediatrics
Peer reviewed: Yes
Rights: cc_by
Usage restriction: openAccess
Self-archived version: publishedVersion

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