Childhood outcomes in children with Hirschsprung disease: a population-based data linkage study in England
Abstract
Objective Hirschsprung disease (HSCR) is a rare congenital intestinal condition that, despite corrective surgery, can lead to recurrent hospitalisations and reduced quality of life throughout childhood. There is limited population-level evidence on the causes of these admissions or associated educational needs. Design/methods Using linked health and education data from the Education and Child Health Insights from Linked Data database, we created a cohort of all children born in England between 2002 and 2020. We compared admission rates, mortality, surgical procedures and causes of admission for children with and without HSCR at ages 0–4, 5–9 and 10–14 years. We also assessed the prevalence of recorded Special Educational Needs and Disability (SEND) by Year 1 of primary school (age 6). Results Among 11 261 227 children, 3227 (0.03%) had HSCR. By age 4, 95.0% of children with HSCR had been readmitted, compared with 40.2% without. Common admission reasons included constipation, gastroenteritis, intestinal infection, abdominal pain and nausea or vomiting. 81.4% of children with HSCR had two or more surgical procedures between ages 0 and 4, compared with 2.9% in children without HSCR. Mortality by age 4 was 3.2% for HSCR versus 0.5% for non-HSCR children. By age 6, 44.0% of children with HSCR had recorded SEND compared with 17.9% of those without. Conclusion Children with HSCR experience substantially higher hospital readmission rates, more surgeries and greater mortality up to…
How strong is the evidence?
Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence. Reported size: 11 participants or records.
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