Visual Outcomes of Children With Craniosynostosis

Craniosynostosis can impact the visual development of a child. Historically, children with craniosynostosis, particularly when associated with a syndrome, had a significant risk of vision loss. The authors aimed to study the incidence of ophthalmic pathology in a modern, multidisciplinary craniosynostosis practice. Children aged 7 to 13 years attending face-to-face ophthalmic craniofacial clinics between February 2020 and June 2021 were included in a retrospective case note review. Visual acuity, ocular alignment, optic nerve function, and retinal nerve fiber layer (RNFL) condition using optical coherence tomography (OCT) were recorded. Forty-three children (30 girls) were assessed at a median age of 10.3 years (7.8-13.1). Eleven children had unicoronal synostosis, 15 had single-suture synostosis not involving the coronal, 14 had multisuture synostosis involving the coronal, and 3 had multisuture synostosis not involving the coronal. Thirty-two out of 43 had craniofacial surgery. Sixty-seven percent required glasses. Forty-nine percent had strabismus, 11/43 (26%) had squint surgery, and 2/43 (5%) had tarsorrhaphy for corneal protection. Four out of 43 (9%) had papilloedema detected; however, at the final review, 15/68 (22%) eyes showed RNFL changes on OCT imaging, none of whom had optic atrophy. Two children did not meet UK driving standards due to refractive amblyopia; no children were registered as sight impaired. In this cohort, optic atrophy and visual loss due to exposure keratopathy were not seen. A high incidence of strabismus, glasses wear, and amblyopia is persistent. Binocular visual impairment was rare in this cohort: 95% met UK driving standards. Visual outcomes appear to be improving coinciding with improved craniofacial care alongside multidisciplinary team working.