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

Autor: Kristiina Kyrklund, Joseph R Davidson, Simon Eaton, Simon Blackburn, Joe Curry, Kate Cross, David S Thompson, Paolo De Coppi, Mikko P. Pakarinen
Přispěvatelé: HUS Children and Adolescents, Children's Hospital, Clinicum, Lastenkirurgian yksikkö
Rok vydání: 2021
Předmět:
Adult
Male
Background information
Pediatrics
medicine.medical_specialty
Functional impairment
ENDORECTAL PULL-THROUGH
Fecal soiling
CHILDHOOD
Duhamel
Disease
Single Center
03 medical and health sciences
Postoperative Complications
0302 clinical medicine
Quality of life
QUALITY-OF-LIFE
3123 Gynaecology and paediatrics
030225 pediatrics
Hirschsprung s
medicine
Humans
Long-term outcomes
Hirschsprung Disease
Patient Reported Outcome Measures
Bowel function
HEALTH SURVEY QUESTIONNAIRE
CONTINENCE
Child
10. No inequality
Hirschsprung's
business.industry
URINARY-TRACT SYMPTOMS
ADULTS
General Medicine
3126 Surgery
anesthesiology
intensive care
radiology
3. Good health
Treatment Outcome
030220 oncology & carcinogenesis
Pediatrics
Perinatology and Child Health
Quality of Life
Female
Surgery
FOLLOW-UP
business
Fecal Incontinence
Zdroj: Journal of Pediatric Surgery. 56:1502-1511
ISSN: 0022-3468
DOI: 10.1016/j.jpedsurg.2021.01.043
Popis: 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 longterm 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;eta(2) = 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. (C) 2021 Elsevier Inc. All rights reserved.
Databáze: OpenAIRE
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