A multi-center cross-sectional comparison of parent-reported quality of life and bowel function between anorectal malformation and Hirschsprung's disease patients with versus those without Down syndrome
Document Type
Article
Publication Date
7-1-2024
Abstract
Purpose Down syndrome (DS) is a common abnormality associated with anorectal malformation (ARM) and Hirschsprung's disease (HD). However, quality of life (QOL) in ARM and HD patients with DS is under-researched. This study compares parent-reported QOL and bowel function in ARM and HD patients with DS to those without. Methods Between December 2020 to February 2023, parents of ARM and HD patients with and without DS aged 3-17 years who had undergone surgery > 12 months prior at four tertiary referral centers were recruited. We used the Pediatric Quality of Life Inventory (TM) (PedsQL (TM)) Generic Core Scales, General Well-Being (GWB) Scale and Family Impact (FI) Module questionnaires, and the Rintala bowel function score (BFS). Results There were 101 ARM, 9 (8.9%) of whom had DS; and 87 HD, of whom 6 (6.9%) had DS. Parent-reported Core scores in ARM and HD with DS were comparable to those without DS. However, ARM and HD with DS had worse scores in the FI Module and bowel function than those without DS. Conclusion Although parent-reported QOL in ARM and HD with DS is similar to those without DS, family impact and BFS are worse. Our findings are limited by small sample size in proportion of DS patients.
Keywords
Congenital, Colorectal, Quality of life, Down syndrome
Divisions
surgerydept
Funders
UMSC CA.R.E Fund Research Grant (PV023-2020)
Publication Title
Pediatric Surgery International
Volume
40
Issue
1
Publisher
Springer Verlag
Publisher Location
ONE NEW YORK PLAZA, SUITE 4600, NEW YORK, NY, UNITED STATES