Objective: To estimate the global prevalence of neurodevelopmental impairment in children with Robin sequence (RS) at one year or more of age.
Study Design: Electronic databases such as PubMed, Embase, CINAHL, APA PsycInfo, Emcare, MedNAR and Cochrane library were searched systematically from inception to 31st May 2024. Studies reporting on the neurodevelopmental (global, cognitive, or motor) outcomes in children with RS were included. Data was extracted using a standardized form by two independent reviewers. Overall and subgroup-specific prevalence (95% CI) of neurodevelopmental impairment was estimated with random-effects meta-analysis. Subgroup analyses were performed for three categories of RS: isolated (no other associated abnormalities), syndromic RS (associated with a genetic syndrome), and RS plus (associated with non-syndromic congenital abnormalities).
Results: A total of 2919 records were screened. Seventeen studies were included in the systematic review, of which data from 16 studies (n = 1008) were pooled for meta-analysis. The overall prevalence of neurodevelopmental impairment was 19 % (12-26 %). Neurodevelopmental impairment prevalence in isolated RS was 10 % (5 to16%), syndromic RS 19 % (02 to44%), and RS plus 63 % (39 to84%). The overall prevalence in non-isolated RS (syndromic and plus) was 35 % (22 to49%).
Conclusion: This is first systematic review and meta-analysis to report on the global prevalence of neurodevelopmental impairment in children with RS. Children with RS are at high risk of neurodevelopmental impairment and should be considered for long-term neurodevelopmental follow up. These findings will guide clinician counselling of parents, resource allocation, facilitate benchmarking, and enable the assessment of treatment impact in future studies.
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