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