Quantifying the Burden of Cerebellar and Ophthalmologic Disease in Lambdoid Craniosynostosis

Abstract

OBJECTIVE: Lambdoid craniosynostosis may restrict posterior brain development, particularly involving the cerebellum and occipital lobe, with potential implications for vision and balance. However, due to its rarity, evidence linking lambdoid craniosynostosis to adverse ophthalmologic and intracranial outcomes remains limited. This study describes the prevalence and timing of visual deficits, Chiari malformation, and balance disturbances in children with lambdoid craniosynostosis, using the largest multi-institutional cohort to date. METHODS: A retrospective descriptive study was conducted using the Epic Cosmos database (2017-2025) to identify children diagnosed with lambdoid craniosynostosis. Outcomes included ophthalmologic disorders (amblyopia, strabismus, hemianopia, cortical blindness, diplopia, and visual field defects) and cerebellar conditions (Chiari malformation and balance disturbances). RESULTS: Among 356 children with lambdoid craniosynostosis, strabismus was identified in 39 (11.0%), amblyopia in 21 (5.9%), balance disturbances in 17 (4.8%), and Chiari malformation in 6 (1.70%). No cases of hemianopia, cortical blindness, diplopia, or other visual field defects were identified. Strabismus was diagnosed at the earliest median age (0.8 y), followed by Chiari malformation (0.9 y), balance disturbances (1.6 y), and amblyopia (2.0 y). CONCLUSIONS: These findings highlight the need for early ophthalmologic referral, consideration of neuroimaging, and close longitudinal follow-up to detect and mitigate intracranial sequelae in children with lambdoid craniosynostosis.