Quality of life outcomes in children with Hirschsprung disease

Abstract

BackgroundMorbidity 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. MethodsPatients 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. ResultsParents of 60 HD patients [M:F 49:11; median age 6.4years (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). ConclusionsWe have demonstrated that HD children have significant reductions in psychosocial QoL and functional outcomes. Level of evidencePrognosis Study – Level II (Prospective cohort study).