Childhood outcomes in children with Hirschsprung disease: a population-based data linkage study in England
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Objective
Hirschsprung disease (HSCR) is a rare congenital intestinal condition that, despite lifesaving surgery, can result in increased hospitalisations and a lower quality of life throughout childhood and into adulthood. There is a lack of population-level research on the reasons for these admissions, or on the level of support required at school age.
Design/Methods
We used linked administrative data from health and education services (ECHILD) to create a cohort of births from 2002-2020. We evaluated admission and mortality rates, number of surgical procedures, and reasons for admissions for children with and without HSCR at ages 0-4, 5-9 and 10-14. We assessed how many children had recorded Special Educational Needs by Year 1 of primary school (age 6).
Results
Of the 11,261,227 children in our cohort, 3227 (0.03%) had HSCR. 95.0% of children with HSCR were readmitted by age 4 compared with 40.2% of those without. Across ages and sexes, children with HSCR were frequently admitted for constipation, gastroenteritis, intestinal infections, abdominal pain, and nausea and vomiting. 3.2% of children with HSCR died by age 4 compared with 0.5% of children without HSCR. 44.0% of children with HSCR had recorded Special Educational Needs by age 6 compared with 17.9% of those without HSCR.
Conclusion
Children with HSCR have more frequent hospital admissions, surgical procedures, and higher mortality rates up to age 14 than their peers, and are more likely to have Special Educational Needs at school entry, irrespective of common comorbidities such as Down Syndrome. Improvements to treatment success, including novel or complementary approaches, are required in order to improve quality of life for this group of children. Further research is needed to understand the impact of HSCR and its treatment at the transition from paediatric to adult services, and on child development, progress in school, and other psychosocial factors including mental health.
What is already known on this topic
Hirschsprung disease (HSCR) is a rare congenital condition treated surgically in infancy, but long-term outcomes remain poorly understood. Existing studies have provided limited insight into broader population impacts of HSCR. Hence, comprehensive national data are needed to clarify ongoing health and developmental challenges in children with HSCR.
What this study adds
Using linked national health and education data from over 11 million children in England, this study provides the first population-level evidence on long-term outcomes in HSCR. Children with HSCR had significantly higher rates of hospital admissions, mortality, and Special Educational Needs compared with peers. These findings highlight the persistent health and developmental burdens of HSCR despite early surgical intervention.
How this study might affect research, practice or policy
Our study emphasises the need for coordinated long-term, multidisciplinary, care for children with HSCR. Our data can inform clinical pathways and policy planning to better address ongoing medical and educational needs for children and families living with HSCR.
