Elsevier

Journal of Pediatric Surgery

Volume 52, Issue 12, December 2017, Pages 2006-2010
Journal of Pediatric Surgery

Colorectal
Quality of life outcomes in children with Hirschsprung disease

https://doi.org/10.1016/j.jpedsurg.2017.08.043Get rights and content

Abstract

Background

Morbidity following repair of Hirschsprung disease (HD) is common. However, quality of life (QoL) results focused on HD children are contradictory. We aimed to measure QoL outcomes in HD children using validated questionnaires.

Methods

Patients with HD, managed at a large tertiary pediatric institution between 2004 and 2013, were identified. Parents completed validated questionnaires. Results were compared with published healthy population controls. QoL outcomes were measured using Pediatric Quality of Life (PedsQL) and Fecal Incontinence and Constipation Quality of Life (FIC QOL). Functional outcomes were assessed using Baylor Continence Scale, Cleveland Clinic Constipation Scoring System, and Vancouver Dysfunctional Elimination Syndrome Survey.

Results

Parents of 60 HD patients [M:F 49:11; median age 6.4 years (2.3–10.9)] were interviewed (59% participation). The majority (47/60, 78%) had rectosigmoid disease. There was significant reduction in psychosocial (social and emotional) QoL compared with healthy children (p = 0.03). Psychosocial functioning was affected by increasing age (r =  2.72, p < 0.001), fecal incontinence (r =  0.475, p = 0.007), constipation (r =  1.58, p = 0.006), and dysfunctional elimination (r =  2.94, p = 0.004). Fecal incontinence also reduced physical functioning QoL (r =  0.306, p = 0.007). Children with HD had significantly higher levels of fecal incontinence (p < 0.01).

Conclusions

We have demonstrated that HD children have significant reductions in psychosocial QoL and functional outcomes.

Level of evidence

Prognosis Study – Level II (Prospective cohort study).

Section snippets

Methods

Patients with HD, who were managed at our tertiary pediatric surgical center between 2004 and 2013, were identified from admissions coded by Health Information Systems. Hospital medical records were reviewed for demographic and clinical data. Parents of patients completed questionnaires via phone interview during a three-month study period. Data regarding age, gender, associated anomalies, level of aganglionosis, operative management and complications were collected from patient records and a

Results

A total of 102 HD patients were identified and invited to participate in the study. Twenty-eight families were unable to be contacted, two families were unwilling to be involved and one family was excluded due to language difficulties (non-English speaking background). Of the available 71 families, 60/71 (85%) families completed the questionnaires. Median age at the time of study was 6.4 years (2.3–10.9), and 82% were male. Patient demographics, concurrent disease, length of aganglionic segment

Discussion

This study has demonstrated that children with HD experience significantly lower psychosocial QoL compared with healthy children. However, in physical and total QoL, there were no significant differences between healthy children and those with HD. Increasing age and increasing severity of fecal incontinence, constipation and dysfunctional elimination negatively affected QoL. Gender, associated anomalies, the presence of a stoma, the severity of disease and the number of enterocolitis episodes

Ethics

HREC 36003A Human Research Ethics Committee.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or non-for-profit sectors.

Disclosures

The authors have no conflict of interests to declare.

Acknowledgements

Associate Professor Sebastian King is the grateful recipient of a Career Development Award (Murdoch Children's Research Institute) and Senior Lecturer Fellowship (Royal Australasian College of Surgeons). His position as an Academic Pediatric Surgeon is generously supported by The Royal Children's Hospital Foundation. Associate Professor Warwick Teague's position as an Academic Pediatric Surgeon and Director of Trauma Services is generously supported by The Royal Children's Hospital Foundation.

References (29)

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