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Alexander Ho, Laura Kuehlewein, Amirhossein Hariri, Yulia Wolfson, Rupert Wolfgang Strauss, Hendrik P Scholl, Srinivas R Sadda, ProgSTAR Study Group; Quantitative Characteristics of Spectral-Domain Optical Coherence Tomography (SDOCT) in Corresponding Areas of Decreased Autofluorescence in Patients with Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):5924.
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© ARVO (1962-2015); The Authors (2016-present)
Fundus autofluorescence (FAF) has been proposed as a method to monitor Stargardt disease (STGD), but various patterns of abnormal FAF may be observed, potentially confounding longitudinal assessments. Outer retinal substructure alterations on optical coherence tomography (OCT) have also been demonstrated in STGD patients to correlate with visual outcomes. In this analysis, we explored the relationship between these OCT alterations and areas of definitely and questionably decreased autofluorescence (DDAF, QDAF).
34 eyes from 19 STGD patients with exclusively DDAF or QDAF in the central subfield on confocal scanning laser ophthalmoscope FAF images were identified from the ongoing Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) studies. DDAF was defined as regions of decreased FAF greater than 125 µm in diameter with a blackness level comparable to the optic disc. QDAF was defined as regions of decreased FAF greater than 125 µm in diameter and relatively less dark than the optic disc, but still substantially more hypo-autofluorescent than the background (Figure 1). Corresponding SDOCT B-scans (49-section 20°x20° high resolution volume scans) were independently segmented using proprietary software (OCTOR) for the following layers: inner retina (inner limiting membrane [ILM] to dendritic outer plexiform layer [OPL]), outer nuclear layer (ONL; dendritic OPL to external limiting membrane [ELM]), and combined layers between ELM and Bruch’s membrane (BM). Central subfield layer metrics from DDAF and QDAF were compared using the Mann-Whitney-Wilcoxon test.
The averages and standard deviations of mean thicknesses and preserved areas in the central subfield are shown for DDAF and QDAF in Table 1. The ONL and ELM-BM layer were both significantly thinner in areas of DDAF compared to QDAF (p<0.05).
Thickness and preservation of the ONL and ELM-BM layer appear to be linked to the visible differences in DDAF and QDAF lesions. This finding is consistent with the proposed models for STGD progression, if QDAF is considered a transition state between healthy retina and later stages of STGD.
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