The Progression of Stargardt Disease Using Volumetric Hill of Vision Analyses Over 24 Months: ProgStar Report No.15.
prospective_cohort · Level II
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- Record sourced from PubMed, PMID 33951446.
- Also identified by DOI 10.1016/j.ajo.2021.04.015.
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Abstract
To report the yearly rate of change in macular function in patients with Stargardt disease type 1 (STGD1) over 24 months and to establish a new volumetric visual function index for use in clinical trials investigating the efficacy on retinal sensitivity. Design: International, multicenter, prospective cohort study with 5 study visits every 6 months over 24 months. A total of 233 individuals with genetically confirmed STGD1 (≥1 disease-causing ABCA4 variant). The total volume (V<sub>TOT</sub>) beneath the sensitivity surface of a 3-D model of the hill of vision and mean sensitivity (MS) derived from mesopic microperimetry performed with a white stimulus. Changes of V<sub>TOT</sub> over time and its correlation with the ABCA4 genotype and baseline features. At baseline, 440 eyes (233 patients) with a mean (SD) age of 33.7 (15.0) years, mean (SD) visual acuity of 46.08 (16.03) ETDRS letters were analyzed with an average V<sub>TOT</sub> of 0.91 decibel-steradian (dB-sr) and an MS of 10.73 dB. The overall mean rate of decrease in sensitivity [95% confidence interval] was 0.077 [0.064, 0.090] dB-sr/y for V<sub>TOT</sub> and 0.87 [0.72, 1.02] dB/year for MS. The progression rate of V<sub>TOT</sub> depended on baseline visual function (0.029 dB-sr/year for low and 0.120 dB-sr/year for high baseline V<sub>TOT</sub>; P < .001) and exhibited a difference in the first vs second year of follow-up (0.065 dB-sr/year vs 0.089 dB-sr/year, respectively; P < .001). The absence of pigmentary abnormalities of the retinal pigment epithelium at baseline was found to be associated with a faster progression rate (P < .001), whereas a significant association with the genotype was not detected (P = .7). In STGD1, both microperimetric outcomes demonstrate statistically significant and clinically meaningful changes after relatively short follow-up periods. Volumetric modeling may be useful in future interventional clinical trials that aim to improve retinal sensitivity or to slow down its decline and for structure-function correlations.
Medical subject headings
- Retina
- Visual Fields