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Rupert Wolfgang Strauss, Xiangrong Kong, Beatriz E Munoz, Sheila K West, Michael S Ip, Alexander Ho, Anamika Jha, Paul S Bernstein, David G Birch, Artur V Cideciyan, Michel Michaelides, José-Alain Sahel, Janet S Sunness, Elias I Traboulsi, Eberhart Zrenner, Hendrik P Scholl; Progression of Stargardt disease as measured by spectral-domain optical coherence tomography (SD-OCT) in the ProgStar Study. Invest. Ophthalmol. Vis. Sci. 2018;59(9):1568.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate the change of individual retinal layer thicknesses in patients with Stargardt disease type 1 (STGD1) over 12 months of follow-up using SD-OCT in the prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Study.
259 patients with molecularly confirmed STGD1 (disease causing ABCA4 gene mutations) were enrolled. SD-OCT scans using Heidelberg Spectralis (HE, Germany) were obtained at baseline from a 20° x 20° scan area centered on the fovea and repeated at month 6 and 12 using the built-in follow-up mode (where possible) with gradable images available for 427 eyes. Retinal layers were semi-automatically segmented to identify the following bands: retinal pigment epithelium (RPE), outer segments (OS), inner segments (IS), outer nuclear complex (ONC) and inner retina. The mean thicknesses (MT) of individual layers and the areas of damage/tissue loss were calculated by a custom software within the total scanned area (TSA), central subfield (CS; 0.5mm radius from the foveal center), inner ring (IR; 0.5-1.5mm) and outer ring (OR; 1.5-3mm) of the ETDRS grid.
Study demographics are shown in table 1. In CS, 377 eyes (86%) had MT=0 for IS and OS layers, and CS was excluded from further analysis as there would be no further progression. In IR, total retina thickness decreased -3.04 (95% CI: -4.12, -1.96) µm/year, ONC -7.23 (-8.37, -6.09), IS -1.96 (-2.28, -1.64) µm/year, OS -0.55 (-0.75, -0.35) µm/year and RPE -3.42 (-4.00, -2.85) (table 2). In IR, also intact area showed a statistically significant decrease in all layers over a 12-months period; in the outer ring, a statistically significant decrease was found in RPE and ONC (table 2).
A statistically significant loss was observed in the outer retinal layers by SD-OCT over a 12 month period in patients with STGD1. This was especially significant in the IR – with loss of thickness and/or intact area of the RPE, IS, OS and ONL potentially serving as structural endpoints for clinical trials that aim to slow disease progression.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
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