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Mital Shah, Laura K Young, Susan M. Downes, Hannah Smithson; Investigating the clinical use of adaptive optics scanning laser ophthalmoscopy in patients with Stargardt disease. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4580.
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© ARVO (1962-2015); The Authors (2016-present)
Adaptive optics (AO) retinal imaging is increasingly being used during interventional clinical trials in patients with inherited retinal diseases. Although there are a number of planned/ongoing clinical trials in patients with Stargardt disease, the published literature on AO retinal imaging in these patients remains limited. This prospective observational study investigates the clinical use of AO scanning laser ophthalmoscopy (AOSLO) in patients with Stargardt disease.
14 patients aged 18 to 53 with clinically and molecularly confirmed Stargardt disease underwent ophthalmic examination and retinal imaging, including autofluorescence (AF) imaging, spectral domain optical coherence tomography (SDOCT), microperimetry and confocal adaptive optics scanning laser ophthalmoscopy (AOSLO) with a custom-built AOSLO.
The clinical phenotype of participants was variable in this cohort. LogMAR visual acuity ranged from 0.00 to 1.04. The oldest participant presented with a foveal-sparing Stargardt phenotype and no retinal atrophy. All other participants presented with loss or disruption of foveal architecture on SDOCT and varying degrees of retinal atrophy. A reduced retinal sensitivity was measured with microperimetry in areas with disruption or loss of the photoreceptor inner segment/outer segment (IS/OS) junction on SDOCT. Areas that retained normal retinal sensitivity sometimes showed early disruption of the IS/OS junction. Retinal sensitivity was absent in areas of retinal atrophy.AOSLO images showed a regular photoreceptor mosaic in areas of normal or near normal retinal structure. In areas with retinal degeneration, the photoreceptor mosaic was disorganised. Discrete hyperreflective structures were observed of similar size (mean full width half max 4.0 µm, SD 0.7 µm) across retinal eccentricities (range 0.5 – 16 degrees) and across participants.
Using the AOSLO, we were able to resolve a regular photoreceptor mosaic in participants with Stargardt disease. However, the interpretation of AOSLO images is challenging in these patients, particularly in areas of retinal degeneration. Further work to correlate structure and function at the cellular level is required to enable interpretation of these images.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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