Visual Surveillance in Craniosynostoses

Abstract

Craniosynostosis is the premature fusion of one or more cranial sutures that may be isolated or syndromic. These children can have multiple developmental issues including speech, hearing, and vision, in addition to the aesthetic issue of an abnormally shaped skull and midfacial hypoplasia. As the aesthetic outcomes of craniofacial surgery have improved, attention has turned on the functional outcomes and visual loss is a well-known problem with these patients. In the past 15 years, a greater understanding of the causes of visual loss has developed. Factors such as amblyopia, corneal exposure, and optic neuropathy are all now looked for to prevent or reduce visual loss. Optic neuropathy is caused by craniocerebral disproportion (though to a lesser extent than originally thought), cerebral hypo perfusion, hydrocephalus, and obstructive sleep apnea. Amblyopia is due to increased incidence of strabismus, anisometropia, astigmatism, and ametropia in these cases. A comprehensive approach to managing these children's visual function allows the clinician to reduce potential visual loss in children with craniosynostoses especially the syndromic variety.

• craniosynostosis
• amblyopia
• obstructive sleep apnea
• Apert syndrome
• Crouzon syndrome
• Pfeiffer syndrome