Ophthalmic sequelae of Crouzon syndrome.
OBJECTIVE
To document the frequency of ophthalmic sequelae in patients with Crouzon syndrome before the influence of craniofacial surgery.


METHODS
Retrospective observational case series.


METHODS
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.


METHODS
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 > or =+2 diopters (D) and 20% had myopia of > or =-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.
Gray
Timothy L
TL
Department of Ophthalmology, Women's and Children's Hospital, North Adelaide, Australia. timgray@cyllene.uwa.edu.au
Casey
Theresa
T
Selva
Dinesh
D
Anderson
Peter J
PJ
David
David J
DJ
D000293:Adolescent; D000328:Adult; D000550:Amblyopia / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000150:complications*; D005128:Eye Diseases / Q000453:epidemiology / Q000209:etiology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009216:Myopia / Q000209:etiology; D009896:Optic Atrophy / Q000209:etiology; D015995:Prevalence; D012030:Refractive Errors / Q000209:etiology; D012189:Retrospective Studies; D013021:South Australia / Q000453:epidemiology; D013285:Strabismus / Q000209:etiology; D014792:Visual Acuity