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Neurodevelopmental impairment in children with Robin sequence: A systematic review and meta-analysis
Dimple Goel1, Andrew Wilson1, Gareth Baynam2
1Perth Children's Hospital, Perth, Western Australia, Australia; University of Western Australia, Perth, Western Australia, Australia.
Insights
Children with Robin sequence (RS) have a 19% risk of neurodevelopmental impairment. Non-isolated RS significantly increases this risk, highlighting the need for long-term follow-up.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Genetics
Background:
- Robin sequence (RS) is a congenital condition affecting craniofacial development.
- Neurodevelopmental outcomes in children with RS require further investigation.
Purpose of the Study:
- To estimate the global prevalence of neurodevelopmental impairment (NDI) in children with RS aged one year or older.
- To analyze NDI prevalence across different RS classifications.
Main Methods:
- Systematic review and random-effects meta-analysis of 17 studies (1008 children).
- Searched major electronic databases from inception to May 2024.
- Subgroup analyses for isolated, syndromic, and RS plus categories.
Main Results:
- Overall NDI prevalence in children with RS was 19% (95% CI: 12-26%).
- Prevalence varied by RS type: isolated RS (10%), syndromic RS (19%), and RS plus (63%).
- Non-isolated RS (syndromic and RS plus) showed a 35% NDI prevalence.
Conclusions:
- Children with RS face a substantial risk of NDI.
- Long-term neurodevelopmental follow-up is crucial for all children with RS.
- Findings aid clinical counseling, resource allocation, and future research.
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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