Childhood outcomes in children with Hirschsprung disease

“Many of the families we’ve spoken to have described their frustration at receiving mixed information regarding how their child will be affected after their surgery. Information is power, and while there is lots more work to do, this study takes important steps towards handing that to the Hirschsprung community.”
— Dr Ben Jevans, Senior Research Fellow

This study is being led by Prof Katie Harron, Dr Ben Jevans and Dr Conor McCann (UCL) and was funded by NIHR. The study started in 2024 and aims to finish in 2028.

‍This project was reviewed and approved through ECHILD's data access process. Learn how ECHILD data is kept safe.‍

  • We want to understand the health needs of children with Hirschsprung disease, and how they progress through school as they grow up.

  • Hirschsprung disease is a rare condition that affects less than 1 in 5,000 babies and young children. Although we know how to treat Hirschsprung disease, we know little about the long-term impact of the condition on the children’s health and whether they need extra support at school.  

  • We will use health information from NHS hospitals and education information collected by schools to understand how children with Hirschsprung disease are affected as they grow up.

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  • Why does this matter?

    Hirschsprung disease is a serious condition that some babies are born with. It happens when part of their gut is missing the nerve cells needed to push food through. This can cause severe problems like constipation, blockages, and infections. Surgery is needed to remove the affected section, but many children still have difficulties with digestion, bathroom issues, and even social challenges as they grow up. Most research focuses on the surgery itself, but not much is known about the long-term effects on children’s health, emotions, and daily life.

    What do we want to find out?

    Families of children with Hirschsprung often struggle with stress, worry, and financial difficulties because of their child’s medical needs and hospital visits. The disease can make it hard for children to attend school regularly, make friends, and feel confident. Parents sometimes feel overwhelmed, and siblings may also be affected because so much attention is needed for the child with Hirschsprung.

    What we will do

    We will use health information collected by NHS hospitals and education information collected by schools to understand how children with Hirschsprung disease are affected as they grow up. The information collected by hospitals tells us about health conditions that children with Hirschsprung disease who attend hospital have. The information collected by schools tells us about how long children have been absent from school, how well they do in exams, and whether they are provided with extra support. By understanding the biggest challenges children and families face, we can help doctors, hospitals, and schools provide better support and assistance. 

  • Introduction:

    Hirschsprung disease (HD) is a life-threatening congenital condition affecting approximately 1 in 5,000 live births, caused by the absence of the enteric nervous system in the distal bowel. This results in intestinal obstruction necessitating surgical intervention. Despite surgery, a substantial proportion of children with suffer ongoing physical, psychological, and social complications. Current evidence is limited, with existing studies focusing on short-term surgical outcomes. There is therefore a pressing need for robust, population-level data to evaluate long-term outcomes, inform care strategies, and improve the quality of life for individuals with HD and their families.

    We aim to generate a comprehensive evidence base on the long-term impacts of HD by analysing linked hospital, education, and mental health datasets. The overarching aim is to inform clinical care pathways, policy development, and support strategies that improve quality of life and health equity for affected individuals and their families.

    Methods and analysis:

    We will use ECHILD to create a retrospective national cohort of children born in NHS hospitals in England from 2003 onwards. Children with HD will be flagged using ICD-10 code Q43.1. We will assess healthcare use, mortality, educational attainment, school attendance, special educational needs and mental health service use for children aged up to 16. We will use multi-level generalised linear regression models to estimate the relative risk of adverse outcomes compared to unaffected children, accounting for demographic and socioeconomic confounders.

    ECHILD data used:

    HES:  Admitted Patient Care (2002/03 onwards), ONS Mortality Data

    NPD: Spring Census data, EYFSP, Key Stage 1, Key Stage 2 and Key Stage 4 data from 2006/07 onwards.

  • ‍Published paper: Childhood outcomes in children with Hirschsprung disease

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