The long-term quality of life outcomes in adolescents with Hirschsprung disease

Shreya Sood; Reuben Lim; Lucy Collins; Misel Trajanovska; John M Hutson; Warwick J Teague; Sebastian K King

doi:10.1016/j.jpedsurg.2018.08.036

The long-term quality of life outcomes in adolescents with Hirschsprung disease

J Pediatr Surg. 2018 Dec;53(12):2430-2434. doi: 10.1016/j.jpedsurg.2018.08.036. Epub 2018 Sep 5.

Authors

Shreya Sood¹, Reuben Lim¹, Lucy Collins¹, Misel Trajanovska², John M Hutson³, Warwick J Teague¹, Sebastian K King⁴

Affiliations

¹ Department of Paediatric Surgery, The Royal Children's Hospital, Melbourne; Department of Paediatrics, University of Melbourne; Surgical Research, Murdoch Children's Research Institute, Melbourne.
² Centre for Community and Child Health, The Royal Children's Hospital, Melbourne.
³ Department of Paediatrics, University of Melbourne; Surgical Research, Murdoch Children's Research Institute, Melbourne; Department of Urology, The Royal Children's Hospital, Melbourne.
⁴ Department of Paediatric Surgery, The Royal Children's Hospital, Melbourne; Department of Paediatrics, University of Melbourne; Surgical Research, Murdoch Children's Research Institute, Melbourne; Department of Gastroenterology and Clinical Nutrition, The Royal Children's Hospital, Melbourne. Electronic address: sebastian.king@rch.org.au.

PMID: 30244941
DOI: 10.1016/j.jpedsurg.2018.08.036

Abstract

Background: Postoperative outcomes for Hirschsprung disease (HD) remain variable, with many patients affected by constipation and/or fecal incontinence. The long-term impact upon quality of life (QoL) for HD patients is unclear. We measured long-term QoL outcomes in adolescents with HD using validated questionnaires.

Methods: Patients with HD, managed at a large tertiary pediatric institution between 1997 and 2004, were identified. Patients and/or their proxy completed validated questionnaires. Results were compared with published healthy population controls. Two questionnaires assessed QoL: Pediatric Quality of Life Inventory (PedsQL) and Fecal Incontinence and Constipation Quality of Life (FICQOL). Three measures assessed functional outcomes: Baylor Continence Scale, Cleveland Clinic Constipation Scoring System, and Vancouver Dysfunctional Elimination Symptom Survey.

Results: Interviews were completed for 58 (70% response rate) patients [M:F, 49:9; median age, 14.5 years (11.1-18.7)]. No significant differences were found in general QoL scores between patients and healthy controls (84.84 versus 81.49, p = 0.28). Disease-specific questionnaires revealed reduced QoL in patients and families, with 17% of parents reporting the bowel dysfunction stopped their child from socializing and 47% of parents experiencing some degree of anxiety/depression regarding their child's bowel condition. Fecal incontinence (r = -0.59, p < 0.01), constipation (r = -0.36, p = 0.01), and dysfunctional elimination (r = -0.59, p < 0.01) all negatively correlated with QoL scores.

Conclusions: In this study, generic QoL in the adolescent HD population was comparable to healthy populations. However, children with HD have ongoing bowel dysfunction which negatively impacts upon their QoL.

Level of evidence: Prognosis study:- level II (prospective cohort study).

Keywords: Constipation; Fecal incontinence; Genitourinary; Hirschsprung disease; Psychosocial functioning; Quality of life.

MeSH terms

Adolescent
Child
Constipation / etiology
Constipation / psychology
Digestive System Surgical Procedures / adverse effects
Digestive System Surgical Procedures / methods*
Fecal Incontinence / etiology
Fecal Incontinence / psychology
Female
Hirschsprung Disease / complications
Hirschsprung Disease / psychology
Hirschsprung Disease / surgery*
Humans
Male
Parents / psychology
Prospective Studies
Quality of Life*
Surveys and Questionnaires
Treatment Outcome