Neurodevelopmental impairment in children with Robin sequence: A systematic review and meta-analysis

Dimple Goel; Andrew Wilson; Gareth Baynam; Karen Waters; Jane Pillow; Shripada Rao

doi:10.1016/j.earlhumdev.2024.106185

Neurodevelopmental impairment in children with Robin sequence: A systematic review and meta-analysis

Early Hum Dev. 2024 Dec 21:201:106185. doi: 10.1016/j.earlhumdev.2024.106185. Online ahead of print.

Authors

Dimple Goel¹, Andrew Wilson², Gareth Baynam³, Karen Waters⁴, Jane Pillow⁵, Shripada Rao⁶

Affiliations

¹ Perth Children's Hospital, Perth, Western Australia, Australia; University of Western Australia, Perth, Western Australia, Australia. Electronic address: [email protected].
² Perth Children's Hospital, Perth, Western Australia, Australia; University of Western Australia, Perth, Western Australia, Australia. Electronic address: [email protected].
³ Perth Children's Hospital, Perth, Western Australia, Australia; Curtin University, Perth, Western Australia, Australia. Electronic address: [email protected].
⁴ The Children's Hospital Westmead, Sydney, New South Wales, Australia. Electronic address: [email protected].
⁵ University of Western Australia, Perth, Western Australia, Australia; Telethon Kids Institute, Perth, Western Australia, Australia. Electronic address: [email protected].
⁶ Perth Children's Hospital, Perth, Western Australia, Australia; University of Western Australia, Perth, Western Australia, Australia. Electronic address: [email protected].

PMID: 39733593
DOI: 10.1016/j.earlhumdev.2024.106185

Abstract

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.

Keywords: Cognitive outcomes; Educational achievements; Neurodevelopmental impairment; Pierre Robin sequence; Robin sequence; School performance; Upper airway obstruction; meta-analysis.