Meta-Analysis
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Apr 7, 2022; 28(13): 1362-1376
Published online Apr 7, 2022. doi: 10.3748/wjg.v28.i13.1362
Generic and disease-specific health-related quality of life in patients with Hirschsprung disease: A systematic review and meta-analysis
Veerle Huizer, Naveen Wijekoon, Daniëlle Roorda, Jaap Oosterlaan, Marc A Benninga, LW Ernest van Heurn, Shaman Rajindrajith, Joep PM Derikx
Veerle Huizer, Daniëlle Roorda, LW Ernest van Heurn, Joep PM Derikx, Department of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
Naveen Wijekoon, Department of Surgery, University of Colombo and Department of Paediatric Surgery, Lady Ridgeway Hospital for Children, Colombo 00800, Sri Lanka
Daniëlle Roorda, Jaap Oosterlaan, Marc A Benninga, Department of Pediatrics, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Emma Neuroscience Group, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
Shaman Rajindrajith, Department of Pediatrics, Lady Ridgeway Hospital for Children, Colombo 00800, Sri Lanka
Author contributions: Huizer V, Roorda D, Derikx JPM and Rajindrajith S designed the study and designed data collection; Huizer V and Wijekoon N performed study selection, performed study quality assessment, collected data, carried out the initial analyses, drafted the initial manuscript and revised the manuscript; Roorda D supervised data collection, statistical analyses and critically reviewed the manuscript; Derikx JPM, Rajindrajith S, Benninga MA, van Heurn LWE and Oosterlaan J reviewed the manuscript; all authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Conflict-of-interest statement: All authors have no conflict of interest to disclose.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Danielle Roorda, MD, MSc, Research Scientist, Department of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Reproduction and Development, Meibergdreef 9, Amsterdam 1105 AZ, Netherlands. d.roorda@amsterdamumc.nl
Received: June 9, 2021
Peer-review started: June 9, 2021
First decision: October 3, 2021
Revised: October 15, 2021
Accepted: February 22, 2022
Article in press: February 22, 2022
Published online: April 7, 2022
Processing time: 294 Days and 6.1 Hours
Abstract
BACKGROUND

Patients with Hirschsprung disease (HD) are at risk of persistent constipation, fecal incontinence or recurrent enterocolitis after surgical treatment, which in turn may impact physical and psychosocial functioning. Generic health-related quality of life (HRQoL) and disease-specific health-related quality of life are relevant outcome measures to assess the impact of HD on the QoL of these patients.

AIM

To summarize all available evidence on HRQoL of patients with HD after surgery and the impact of possible moderating factors.

METHODS

Pubmed, Web of Sciences, PsycInfo and Embase were searched with search terms related to ‘Hirschsprung disease’, ‘Pediatrics’ and ‘Quality of life’. Mean and standard deviation of generic HRQoL overall and domain scores were extracted from each study, as well as data describing potential factors associated with QoL. Random effect models were used for meta-analytic aggregation of generic HRQoL scores. Meta-regression was used to assess the relationship between patient and clinical characteristics and generic HRQoL. Disease-specific HRQoL outcomes of patients with HD were systematically reviewed.

RESULTS

Seventeen articles were included in the systematic review (n = 1137 patients) and 15 in the quantitative meta-analysis (n = 1024 patients). Four studies reported disease-specific HRQoL. Patient’s age ranged between 0 and 21 years. Meta-analytic aggregation showed a non-significantly impaired generic HRQoL (d = -0.168 [95%CI: -0.481; 0.145], P = 0.293, I2 = 94.9) in patients with HD compared to healthy controls. Physical (d = -0.042 [95%CI: -0.419; 0.335], P = 0.829, I2 = 95.1), psychosocial (d = -0.159 [95%CI: -0.458; 0.141], P = 0.299, I2 = 93.6) and social HRQoL (d = -0.092 [95%CI: -0.642; 0.457], P = 0.742, I² = 92.3) were also not significantly lower compared to healthy controls. There was no relation between health-related outcomes and the sex of the patients and whether generic HRQoL was measured by parental proxy or self-report. Disease-specific complaints of patients with HD impaired physical HRQoL, but not psychosocial and social HRQoL.

CONCLUSION

In this systematic review and meta-analysis, no evidence was found for impaired generic HRQoL in patients with HD compared to healthy controls, neither for moderating effects of sex, parental proxy or self-report.

Keywords: Hirschsprung disease, Health-related quality of life, Meta-analysis, Systematic review, Pediatrics

Core Tip: Patients with Hirschsprung disease are at risk of constipation, fecal incontinence or enterocolitis after surgery, which in turn may negatively impact physical and psychosocial functioning. In this meta-analysis of 17 available studies representing 1137 patients, we found no evidence that patients with Hirschsprung disease have lower generic health-related quality of life (HRQoL) compared to healthy controls. Generic HRQoL was not dependent of sex of patients and not dependent of whether quality of life was measured by self-report or by parental proxy-report. Disease-specific complaints of patients with HD impaired physical HRQoL, but not psychosocial and social HRQoL.