Long-term surgical and patient-reported outcomes of Hirschsprung Disease

AbstractBackground Information is needed regarding the complex relationships between long-term functional outcomes and health-related quality of life (HRQoL) in Hirschsprung's Disease (HSCR). We describe across multiple domains, completing a core outcome set through to adulthood. Methods HSCR patients operated at single center over 35-year period (1978–2013) were studied. Patients completed detailed questionnaires on bowel urologic function, HRQOL. with learning disability (LD) excluded. Outcomes compared normative data. Data are reported as median [IQR] or mean (SD). Results 186 (median age 28 [18–32] years; 135 males) surveys. Bowel function was reduced (BFS 17 [14–19] vs. 19 [19–20], p < 0•0001;η2 = 0•22). Prevalence severity fecal soiling awareness improved (p 0•05 for both). Urinary incontinence more frequent than controls, most all 13–26y females (65% 31%,p 0•003). In adults, this correlated independently constipation symptoms (OR 3.18 [1.4–7.5],p 0.008). HRQoL strongly outcome: 42% children demonstrated clinically significant reductions overall PedsQL score, poor associated impaired QOL (B 22•7 [12•7–32•7],p 0•001). GIQLI scores often impacted extended segment disease. SF-36 relative population level data large effect sizes noted General Health (g 1.19) Social Wellbeing 0.8). Conclusion Functional impairment common after pull-through, but improves age. Clustering domains identifies need early recognition support these patients.