Joseph R Davidson1, Kristiina Kyrklund2, Simon Eaton3, Mikko P Pakarinen2, David S Thompson4, Kate Cross4, Simon C Blackburn4, Paolo De Coppi5, Joe Curry6. 1. Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK; Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, UK. Electronic address: joseph.davidson@doctors.org.uk. 2. Department of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Finland. 3. Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, UK. 4. Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK. 5. Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK; Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, UK. 6. Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK. Electronic address: joe.curry@gosh.nhs.uk.
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.
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.
Authors: Riyad Peeraully; Jason Langley; Nicola Hayes; Kate Storry; Louise Richardson; Demelza Burridge; Bharat More Journal: Pediatr Surg Int Date: 2022-04-08 Impact factor: 1.827
Authors: Marina L Reppucci; Lea A Wehrli; Duncan Wilcox; Jill Ketzer; Alberto Pena; Luis de la Torre; Andrea Bischoff; Dan Wood Journal: Pediatr Surg Int Date: 2022-09-23 Impact factor: 2.003
Authors: See Wan Tham; Michael D Rollins; Ron W Reeder; Katelyn E Lewis; Casey M Calkins; Jeffrey R Avansino; Tonya M Palermo Journal: J Pediatr Surg Date: 2022-04-23 Impact factor: 2.549
Authors: Joseph R Davidson; Annika Mutanen; Malla Salli; Kristiina Kyrklund; Paolo De Coppi; Joe Curry; Simon Eaton; Mikko P Pakarinen Journal: BJS Open Date: 2022-01-06
Authors: Joseph R Davidson; Kristiina Kyrklund; Simon Eaton; Mikko P Pakarinen; David Thompson; Simon C Blackburn; Kate Cross; Paolo De Coppi; Joe Curry Journal: Eur J Pediatr Date: 2021-06-11 Impact factor: 3.183