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Emily S Smith, Toco Yuen Ping Chui, Ching-Lung Chen, Joseph Carroll, Alfredo Dubra, Robert F Cooper, Richard B Rosen, Donald Hood, Vivienne C Greenstein; Analysis of the Photoreceptor Mosaic Within, On and Outside the Borders of Hyperautofluorescent Rings in Retinitis Pigmentosa Using Adaptive Optics Scanning Light Ophthalmoscopy. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):4925.
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© ARVO (1962-2015); The Authors (2016-present)
To compare the appearance, density, and spatial organization of photoreceptor cells (PRCs) in patients with retinitis pigmentosa (RP) to measures of visual function and retinal structure within, on and outside the borders of the hyperautofluorescent (hyperAF) ring.
A custom research adaptive optics scanning light ophthalmoscope (AO-SLO)  was used to image the PRC mosaic in 8 eyes of 8 RP patients with visual acuities 20/20-20/30, and hyperAF rings and in 5 healthy eyes. Cone photoreceptor densities and mean nearest neighbor distances were estimated along the horizontal meridian (fovea ± 10°) using custom semi-automated cone counting software. In addition, 10-2 visual fields (Carl Zeiss Meditec Inc.) and dark-adapted cone and rod mediated visual sensitivities were measured (Haag-Streit AG). Fundus autofluorescence and spectral domain-optical coherence tomography (OCT) line scans through the fovea were also obtained (Spectralis HRA+OCT Heidelberg Engineering GmbH). Total receptor layer thickness (R+: Bruch’s membrane to the border between the inner nuclear layer and outer plexiform layer) was measured and compared to values for 30 age-similar normals.
Within the hyperAF ring, the ellipsoid zone band was preserved in all eyes. However, R+ thickness was significantly decreased and cone mediated visual sensitivities ranged from 0 to -8dB of normal. The AO-SLO images showed dark regions, and cone densities were decreased for 3 eyes that had significantly decreased cone sensitivities and R+ thickness values from the fovea to the inner ring border. On the ring border, for all 8 eyes, the clarity of the mosaic decreased, the number and extent of dark regions increased and PRCs appeared misshapen/fragmented. Outside the border, where sensitivities and R+ thickness values were markedly decreased, cones could not be reliably identified, although the size and shape of the cells were consistent with retinal pigment epithelium cells.
In patients with RP, both decreased cone density and cone structure abnormalities were observed, even within the hyperAF ring (i.e. in "relatively healthy" parafoveal regions). 1. Sulai, Dubra. Biomed Opt Express. 2012, 3:1647-61. 2. Garrioch et al. Optom Vis Sci. 2012, 89: 632-43.
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