Abstract
Purpose: :
To describe retinal changes observed by Fourier-Domain Optical Coherence Tomography (OCT) in patients with Stargardt disease.
Methods: :
Descriptive, transversal study. Patients with clinical diagnosis of Stargardt disease confirmed by means of fluorescein angiography and electrophysiology studies were included. All patients underwent a complete ophthalmological examination and best corrected visual acuity assessment. A Fourier-domain OCT of the macular area (Cirrus HD OCT, Zeiss) was performed in all cases, using a 512x128 macular cube. The central macular thickness (CMT), the structural characteristics of the retina and retinal pigment epithelium (EPR) were analyzed.
Results: :
Thirty-eight eyes of 19 patients (9 male, 10 female) were included. Age ranged from 12 to 51 years. Mean CMT: 173.96 µm. OCT findings showed RPE irregularities in 29 eyes (76.31%), photoreceptor layer loss in 23 eyes (60.53%), inner retinal layer disorganization in 12 eyes (31.58%), increased visualization of choroidal vessels in 16 eyes (42.10%), hyper-reflective intraretinal deposits in 12 eyes (31.58%) and macular atrophy in 9 eyes (23.68%). Foveal Thinning (<200µm) was observed in 28 eyes (73.68%) which correlates with mean BCVA of 20/200.
Conclusions: :
Our results suggest that the Fourier domain OCT could be a useful tool in the analysis of the atrophy extent in the RPE-photoreceptor complex and its relation with the choriocapillaris in patients with Stargardt disease.
Keywords: retinal degenerations: hereditary • retina • imaging/image analysis: clinical