Ophthalmic sequelae of Crouzon syndrome

Purpose: To document the frequency of ophthalmic sequelae in patients with Crouzon syndrome before the influence of craniofacial surgery. Design: Retrospective observational case series. Participants: Seventy-one consecutive patients with a clinical diagnosis of Crouzon syndrome assessed before craniofacial or ophthalmic surgery at the Australian Craniofacial Unit between 1984 and 2000. Methods: Review of clinical records with documentation of patient age, gender, visual acuity, refractive error, diagnosis of amblyopia, squint, eye movement dysfunction, nystagmus, fundus examination, examination of the anterior segment, interpupillary distance, and intercanthal distance. Main Outcome Measures: The frequency of ophthalmic signs and visual impairment, defined as a visual acuity of 6/12 or less. Results: Visual impairment in at least 1 eye occurred in 35% of patients and was bilateral in 9%. The most common cause of visual impairment was amblyopia, which was present in 21% of patients, followed by optic atrophy in 7%. Ametropia occurred in 77% of patients; 57% had hypermetropia of >=+2 diopters (D) and 20% had myopia of >=-0.5 D. Strabismus occurred in 39% of patients. Although exposure keratopathy was observed in 15% of patients, this complication was well managed and caused no reduction in visual acuity. Conclusions: Early detection to reduce amblyopia by correction of refractive errors, timely treatment of strabismus, and patching should be a priority for ophthalmologists and a goal of the craniofacial teams managing patients with Crouzon syndrome. Optic atrophy remains an important cause of visual impairment in these patients before decompressive craniectomy.