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Jerome Sherman, Sanjeev Nath, Sarah MacIver, Sherry J Bass, K Bailey Freund; Spectral Domain Optical Coherence Tomography Reveals a Novel Biomarker for Early Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2014;55(13):1432.
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© ARVO (1962-2015); The Authors (2016-present)
To report a novel finding of foveal SD-OCT hyper-reflectivity extending above the external limiting membrane which may precede photoreceptor atrophy in eyes with Stargardt Disease.
This was a retrospective review of SD-OCT and fundus autofluorescence imaging from 3 institutions. Imaging of both Stargardt patients and of their asymptomatic siblings, some having genetic confirmation of their diagnosis, were analyzed.
Six patients were identified as having the novel SD-OCT biomarker including 2 asymptomatic young siblings (4 and 5 yrs old) of an 8-year-old boy with genetically confirmed Stargardt disease and 20/200 VA. Ultra-widefield autofluorescece (UWF-AF) imaging showed mildly increased FAF in the young siblings and marked hyper-AF in the 8 year-old with clinical findings. Additional cases included a 23-year-old (VA 20/25) and a 27-year-old (VA 20/200), both with genetic confirmation of Stargardt disease. The SD-OCT abnormality was not detected in any Stargardt patient over 40 yrs.
The proposed SD-OCT biomarker appears to be present prior to symptomatic vision loss in patients as young as 4 yrs. We hypothesize that cellular degeneration at the level of the outer nuclear layer may produce this finding. The biomarker is likely transient and may be lost with further outer retina degeneration.
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