OPHTHALMOLOGICAL MANAGEMENT IN CRANIOSYNOSTOSIS

ABSTRACT Introduction: In published series, a large proportion of patients with craniosynostosis show impaired vision. Materials and methods: A literature review was performed, using the PubMed and Google Scholar databases, to identify original and review articles on the consequences of craniosynostosis for the eyes and visual pathways, and on ophthalmological management of craniosynostosis. Results and discussion: Many ophthalmic potentially sight-threatening complications occur in patients with craniosynostosis, especially when syndromic. Optic neuropathy, mostly resulting from the papilledema causing optic atrophy, secondary to raised intracranial pressure (ICP), should be diagnosed early, in order to promptly lower the ICP. Cyclovertical and horizontal strabismus and refractive errors are frequent in unicoronal synostosis (anterior plagiocephaly) and syndromic craniosynostosis. Exorbitism, encountered in some cases of syndromic craniofacial synostosis, leads to exposure keratopathy, which requires aggressive management to avoid severe irremediable corneal complications. Amblyopia can complicate asymmetrical optic neuropathy, corneal opacity, strabismus, or refractive errors. If undiagnosed and untreated at a young age, it results in permanent visual impairment. Conclusion: Children with craniosynostosis require a multidisciplinary care network including a pediatric ophthalmologist. Systematic ophthalmological follow-up enables papilledema to be diagnosed and amblyopia to be diagnosed and treated, in order to avoid visual impairment.