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Grace K Han, Tomas S Aleman, Jessica Ijams Wolfing Morgan; Adaptive optics and high-resolution imaging of structure and function in retinitis pigmentosa patients with verses without a hyper-autofluorescent ring. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):4927.
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© ARVO (1962-2015); The Authors (2016-present)
Hyper-autofluorescent (AF) rings are prevalent in patients with retinitis pigmentosa (RP). This study uses multimodal high-resolution imaging, including confocal adaptive optics scanning light ophthalmoscopy (AOSLO), to compare the structural and functional phenotypes in RP patients with verses without hyper-AF rings.
10 RP patients aged 11-63 years were imaged using AOSLO (Canon, Inc. and/or a custom system), spectral domain optical coherence tomography (SD-OCT) and AF (Heidelberg Engineering, HRA), fundus photography (8 patients), and microperimetry (Nidek MP1, 8 patients). One patient participated in retinal densitometry (10 deg, temporal retina) using the Optos P200C-AF SLO. Images from all modalities were registered in Photoshop. Cell density was measured using a semi-automated Matlab script.
AF imaging revealed a hyper-AF ring in parafoveal retina in 6 of 10 patients. 2/4 who did not show the hyper-AF ring were diagnosed as X-linked RP. Foveal sensitivity was reduced 7.3 ± 5.5dB and was not significantly different in patients with vs. without a hyper-AF ring. Outside of the hyper-AF ring, OCTs showed loss of the IS/OS layer and thinning of the outer nuclear layer (ONL). The retinal area corresponding to the ring appeared as a transition zone to photoreceptor layer loss. OCTs of patients without the hyper-AF ring showed severe ONL thinning with a smoother transition in thickness. AOSLO revealed a regular cone mosaic central to the parafoveal hyper-AF rings. Images of retinal areas corresponding to the hyper-AF ring showed sparsely remaining cones. Outside of the hyper-AF ring, the cone mosaic was not visible. In images showing a contiguous cone mosaic, parafoveal cone density was normal between 1 and 1.5 mm eccentricities. At 0.5mm eccentricity one third of locations showed reduced cone density while two thirds were normal. The increase in reflectance from retinal densitometry was ~1/6 of the normal response.
AOSLO imaging demonstrates a dramatic transition in the organization of the cone photoreceptor mosaic co-localizing with the hyper-AF rings commonly described in retinal degenerations. The outer boundary of the hyper-AF ring demarcates the transition zone to loss of the cone photoreceptor mosaic, a region that may be used to monitor disease severity and progression.
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