3 years ago

Quantifying the Rate of Ellipsoid Zone Loss in Stargardt Disease

To determine a reliable method of using the ellipsoid zone (EZ) on optical coherence tomography (OCT) to track disease progression in Stardgardt disease (STGD). DESIGN retrospective reliability study METHODS STGD patients with genetically confirmed ABCA4 gene mutations seen at the Wilmer Eye Institute with follow-up visits separated by at least 12 months were identified. Spectral domain optical coherence tomography (SD-OCT) macula volume scans centered at the fovea and fundus autofluorescence (FAF) images were obtained. The area of EZ loss was calculated from the SD-OCT and the area of RPE loss from the FAF. Scans were re-analyzed by the primary grader to assess intra-grader reliability, and re-analyzed by a second grader to assess inter-grader reliability. RESULTS 16 STGD patients (total of 31 eyes) were followed for a mean of 2 years (range 1-4.7 years). The mean rate EZ loss, 0.31± 0.31mm2/year, was similar to the average rate of RPE loss, 0.33±0.38mm2/year. The average area of EZ loss at the initial examination, 4.18±1.9mm2/year, was larger than the initial area of RPE loss, 2.25±1.66mm2 (p < 0.01). The absolute difference of the area of EZ loss on test-retest for the first grader was 0.12±0.10mm2, and between graders 0.21±0.21mm2. The intraclass correlation (ICC) of both intra-grader as well as inter-grader reliability for EZ loss was excellent at 0.99. CONCLUSIONS Tracking the area of EZ loss on SD-OCT macular volume scans longitudinally is a reliable way of monitoring disease progression in STGD. This could be used as a sensitive anatomic outcome measure in clinical trials related to STGD.

Publisher URL: www.sciencedirect.com/science

DOI: S0002939417304713

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