Neurodevelopmental Outcomes Among Nonsyndromic Craniosynostosis Subtypes

Nonsyndromic craniosynostosis (NSCS) has been associated with higher rates of cognitive, developmental, and motor delays. This study compares neurodevelopmental outcomes among different NSCS subtypes.

A retrospective cohort study was performed using a large multi-institutional database. Patients with sagittal (SCS), metopic (MCS), and coronal (CCS) craniosynostosis were propensity-score matched 1:1 with age-matched unaffected controls. Outcomes included psychiatric and behavioral disorders, intellectual disability, speech and language disorders, neurodevelopmental disorders [autism spectrum disorder, attention-deficit hyperactivity disorder (ADHD)], learning disorders, motor disorders, and intracranial pressure (ICP) abnormalities.

Final cohorts included 3,517 children with SCS (mean age: 7.9 ± 4.4 y), 1776 with MCS (9.0 ± 5.2 y), and 379 with CCS (9.8 ± 6.1 y). All subtypes had significantly higher rates of intellectual disability (SCS: RR = 6.17; MCS: RR = 7.00; CCS: RR = 9.20) and motor delays (SCS: RR = 1.26; MCS: RR = 1.63; CCS: RR = 1.70) compared with controls. Metopic craniosynostosis demonstrated the most severe profile, with elevated rates of speech and language disorders (RR = 1.39), overall neurodevelopmental disorders (RR = 1.22), specific learning disorders (RR = 1.82), and autism spectrum disorder (RR = 1.98). Intracranial pressure abnormalities were highest in SCS (RR = 16.20), yet SCS showed rates of psychiatric disorders and neurodevelopmental diagnoses comparable to or lower than controls.

Intellectual disability and motor delays are elevated across all NSCS subtypes. Metopic craniosynostosis carries the most severe neurodevelopmental phenotype. Despite the highest ICP rates, SCS is associated with the mildest developmental and behavioral profile.