Increased risk of clinically relevant neurodevelopmental disorders in survivors of congenital diaphragmatic hernia: a population-based study
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In brief
In brief
This Swedish population-based study of 641 CDH survivors found significantly elevated risks for neurodevelopmental disorders compared to controls, including 2.2-fold increased risk for any ND and 10.6-fold increased risk for intellectual disability. The findings highlight the importance of long-term neurodevelopmental surveillance in CDH survivors beyond the neonatal period.
- CDH survivors show 2.2× higher risk of any neurodevelopmental disorder compared to general population (11.5% vs 5.6%, p<0.001).
- Intellectual disability risk is dramatically elevated in CDH survivors: 10.6× higher than controls (6.6% vs 0.7%, p<0.001).
- Autism spectrum disorder incidence nearly doubles in CDH survivors: 3.9% vs 2.1% in controls (1.9× risk, p=0.011).
- Neurodevelopmental sequelae persist as the most common long-term morbidity in CDH, warranting systematic developmental surveillance.
- Population-based data confirms clinically significant ND risk in CDH independent of screening bias—counsel families accordingly.
Written by the GCMD Library team from the article.
Abstract
Purpose
Neurodevelopmental disorders (ND) have been recognized as the most common and potentially most disabling outcome of congenital diaphragmatic hernia (CDH). Our aim was to evaluate the incidence of ND in patients with a history of CDH referred for ND assessment on the basis of clinically relevant symptoms rather than through a screening process.
Methods
The study was a nationwide, population-based prospective nested case–control study within a cohort of newborn children who survived the newborn period in Sweden during the observational period from 1st January 1982 until 31st December 2015.
Results
In the study period, 641 non-syndromic CDH survivors were identified, and 3080 control cases were selected. Significantly higher incidence of clinically relevant any ND (11.5% vs 5.6%,p < 0.001), ASD (3.9% vs 2.1%,p:0.011), and intellectual disorder (6.6% vs 0.7%,p < 0.001) were observed in CDH compared to controls. When compared with controls, those with CDH were found to have a 2.179 times higher risk to develop any ND (p < 0.001), 1.867 times higher risk for ASD (p = 0.008), and 10.63 times higher for intellectual disorder (p < 0.001).
Conclusion
CDH survivors have a higher risk of clinically relevant ND than the general population, independent of the effect of screening.