Journal of American Association for Pediatric Ophthalmology and Strabismus
Major articleSpectrum of Foveal Development in Albinism Detected with Optical Coherence Tomography
Section snippets
Methods
Eleven patients with a diagnosis of albinism and binocular best-corrected visual acuity (BCVA) measuring at least 20/60 were recruited for this prospective study between April and August in 2004. We selected this criterion for BCVA to include a group of patients who were more likely to have foveal development. We excluded uncooperative patients and those who refused to participate. Diagnosis of albinism was made with clinical phenotype determined by a pediatric ophthalmologist (C.G.S.) and
Results
Patient demographics are summarized in Table 1. Patients 3 and 4 are brother and sister, as are patients 10 and 11. Although entry criterion for the study was binocular BCVA ≥20/60, all subjects had BCVA of 20/50 or better. Mean binocular BCVA was 20/39. Two patients in this study (no. 4, no. 11) did not have nystagmus detected with clinical examination. Neither of these patients had indication of foveal development with ophthalmoscopy or OCT. All patients except one (no. 8) had a grade 3
Discussion
The spectrum of foveal hypoplasia in albinism can be detected with binocular indirect ophthalmoscopy.2, 3 To our knowledge, this is the first study to show objective, in vivo evidence of foveal development in albinism with OCT. Both ophthalmoscopy and OCT are limited due to the often associated nystagmus and photosensitivity in persons with albinism. While indirect ophthalmoscopy is subjective, it seems to be more sensitive to detection of rudimentary foveal development by permitting detection
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Albinism
Vision in albinism
Trans Am Ophthalmol Soc
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Supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY.