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Philipp Lothar Müller, Martin Gliem, Frank G Holz, Peter Charbel Issa; Functional relevance of autofluorescence patterns in Stargardt disease. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5014.
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Stargardt disease is characterized by distinct patterns of increased and decreased signals on near-infrared (NIR) and short wavelength (SW; blue) fundus autofluorescence (AF) imaging. The aim of this study was to evaluate the structure-function correlation between patterns on fundus AF images and maps of retinal sensitivity derived from microperimetry testing.
Fifty eyes of 25 patients with Stargardt disease underwent functional testing using fundus controlled perimetry (MAIA, CenterVue, Italy). A confocal scanning laser ophthalmoscope (HRA2, Heidelberg Engineering, Germany) was used for recording NIR-and SW fundus AF. Disease-related patterns on AF images were categorized as follows: 1) no pattern, 2) granular pattern, 3) bright > dark flecked pattern, 4) dark > bright flecked pattern, 5) dark pattern and 6) atrophic lesions. Retinal sensitivity along a horizontal line of 15° eccentricity through the fovea was compared between these regions.
Pattern-related retinal sensitivity was not different between both eyes of each patient (ANOVA; p=0.16 and p=0.54 for SW- and NIR-fundus AF, respectively). Borders between consecutive patterns on NIR-fundus AF images were more eccentric compared to equivalent borders on SW- fundus AF images. For both SW- and NIR fundus AF retinal sensitivity was different between patterns (ANOVA, p<0.0001; post hoc test, p<0.0001 to p=0.047) except for the comparison between patterns 1 and 2 (p=0.06 and p=0.98 for SW- and NIR fundus AF, respectively), as well as between patterns 2 and 3 (p=0.42 and p=0.05 for SW- and NIR fundus AF, respectively). The largest drop in retinal sensitivity was observed at the border to regions with predominantly dark pattern in both SW- and NIR- fundus AF (mean retinal sensitivity [dB±SEM], SW fundus AF: 1 = 23.33±0.81, 2 = 19.94±1.45, 3 = 16.60±1.53, 4 = 9.27±1.43, 5 = 4.13±1.18, 6 = 0.58±0.35; NIR fundus AF: 1 = 24.08±0.58, 2 = 23.66±0.67, 3 = 19.38±1.33, 4 = 12.62±1.48, 5 = 2.97±0.67, 6 = 0.71±0.41).
Structure-function correlations reveal consistent functional deficits of fundus AF patterns in Stargardt disease. In both, SW- and NIR fundus AF the transition to a predominantly dark pattern is associated with a marked impairment of retinal sensitivity. Areas of equivalent patterns on NIR fundus AF images exceed those on SW fundus AF, suggesting superiority of NIR- fundus AF imaging to indicate early functional alterations.
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