Purpose: To study the reading performance of patients with Stargardt disease (STGD) and the relationship between clinical vision measurements and vision-related quality of life (VRQOL). Methods: We studied both eyes of 16 patients with STGD. Each patient was examined for best-corrected visual acuity (Early Treatment Diabetic Retinopathy Study [ETDRS]), reading ability (MNREAD and REX charts), contrast sensitivity (Pelli-Robson charts), fixation study (MP1 microperimeter), and VRQOL (25-item National Eye Institute Visual Function Questionnaire [NEI VFQ-25]). The correlation pattern among these variables was examined and an exploratory factor analysis was used to investigate dimensionality of both visual function and VRQOL. Results: Mean ETDRS visual acuity was about 20/160 (0.9 logMAR). All studied psychophysical measures were highly or moderately correlated with MNREAD reading speed (p<0.05 level). A similar correlation was found between psychophysical measures and VRQOL, which was higher for MNREAD measures of acuity (r = -0.75) and speed (r = 0.74). Accordingly, exploratory factor analysis suggested that a single latent dimension explained most of the variance of vision psychophysical measures as well as of VRQOL. Conclusions: We propose that reading ability should be assessed in patients with STGD, since we found that both MNREAD reading speed and visual acuity are strong determinants of quality of life. The observed relation between reading ability and VRQOL in STGD suggests that in these patients appropriate low vision rehabilitation can improve both reading performance and consequently VRQOL. Finally, our data support the use of reading speed and visual acuity as important outcome measures for monitoring STGD progression.

Purpose. To investigate the simultaneous association of several psychophysical measures with reading ability in patients with mild and moderate low vision attending rehabilitation services. Methods. Standard measurements of reading ability (Minnesota Reading [MNREAD] charts), visual acuity (Early Treatment of Diabetic Retinopathy Study [ETDRS] charts), contrast sensitivity (Pelli-Robson charts), reading contrast threshold (Reading Explorer [REX] charts), retinal sensitivity, and fixation stability and localization (Micro Perimeter 1 [MP1] fundus perimetry) were obtained in 160 low vision patients with better eye visual acuity ranging from 0.3 to 1.0 logarithm of the minimum angle of resolution and affected by either age-related macular degeneration or diabetic retinopathy. Results. All variables were moderately associated with reading performance measures (MNREAD reading speed and reading acuity and REX reading contrast threshold), as well as among each other. In a structural equation model, REX reading contrast threshold was highly associated with MNREAD reading speed (standardized coefficient, 0.63) and moderately associated with reading acuity (standardized coefficient, −0.30). REX test also mediated the effects of Pelli-Robson contrast sensitivity (standardized coefficient, 0.44), MP1 fixation eccentricity (standardized coefficient, −0.19), and the mean retinal sensitivity (standardized coefficient, 0.23) on reading performance. The MP1 fixation stability was associated with both MNREAD reading acuity (standardized coefficient, −0.24) and MNREAD reading speed (standardized coefficient, 0.23), while ETDRS visual acuity only affected reading acuity (standardized coefficient, 0.44). Conclusions. Fixation instability and contrast sensitivity loss are key factors limiting reading performance of patients with mild or moderate low vision. REX charts directly assess the impact of text contrast on letter recognition and text navigation and may be a useful aid in reading rehabilitation.

Background. The aim of this study is to investigate the ocular thermographic profiles in age-related macular degeneration (AMD) eyes and age-matched controls to detect possible hemodynamic abnormalities, which could be involved in the pathogenesis of the disease.Methods. 32 eyes with early AMD, 37 eyes with atrophic AMD, 30 eyes affected by untreated neovascular AMD, and 43 eyes with fibrotic AMD were included. The control group consisted of 44 healthy eyes. Exclusion criteria were represented by any other ocular diseases other than AMD, tear film abnormalities, systemic cardiovascular abnormalities, diabetes mellitus, and a body temperature higher than 37.5°C. A total of 186 eyes without pupil dilation were investigated by infrared thermography (FLIR A320). The ocular surface temperature (OST) of three ocular points was calculated by means of an image processing technique from the infrared images. Two-samplet-test and one-way analysis of variance (ANOVA) test were used for statistical analyses.Results. ANOVA analyses showed no significant differences among AMD groups (Pvalue >0.272). OST in AMD patients was significantly lower than in controls (P>0.05).Conclusions. Considering the possible relationship between ocular blood flow and OST, these findings might support the central role of ischemia in the pathogenesis of AMD.

Purpose.: To investigate the reliability of the Reading Explorer (REX) charts and to assess the impact of text contrast reduction (1.5 cycle/degree) on reading speed in subjects with normal and low vision. Methods.: Standard visual acuity (ETDRS charts), reading speed (MNread charts), and contrast sensitivity (Pelli-Robson charts) measurements were obtained in 3 groups of subjects stratified by visual acuity level in the better eye from 0.0 to 1.0 logMAR, with intermediate cutoffs at 0.3 and 0.6 logMAR. Measurements of reading speed for decreasing levels of text contrast were obtained with the REX charts using a 1.5 cycle/degree text. Results.: Since in many patients with lower vision a plateau of maximum reading speed across different levels of text contrast was not found, reliability indexes were computed for average reading speed and reading contrast threshold. In the group with lower visual acuity, 95% limits of agreement were ±0.134 log word/minute and ±0.175 log contrast sensitivity, suggesting good reliability. The proportion of subjects with a 20% loss of reading speed from 90% to 45% text contrast was estimated to be 1/3 at 0.6 logMAR visual acuity level and 2/3 at 1.0 logMAR. Conclusions.: The adverse effect of decreased text contrast, which may be found in ordinary reading material, on the reading performance of subjects with advanced and initial low vision is probably underestimated. The REX test proved to be a reliable investigation tool for this phenomenon.

Purpose: To evaluate the long-term outcomes of intravitreal anti-vascular endothelial growth factor (VEGF) drugs with a pro re nata (PRN) regimen for the treatment of choroidal neovascularization (CNV) secondary to angioid streaks (AS). Methods: This is a retrospective, multicenter, noncomparative case series of consecutive AS eyes affected by treatment-naïve CNV. A complete ophthalmologic examination was performed every 30-45 days after the loading phase, including fluorescein angiography and/or optical coherence tomography. Results: In all, 52 eyes of 39 patients were treated with intravitreal bevacizumab and/or ranibizumab and followed up for a mean of 33.8 months. The best corrected visual acuity at baseline was 20/40, and it deteriorated by an average of 6.8 ETDRS letters per year (p < 0.001). We performed an average of 5.1, 6.5, and 6.8 injections at the 1-, 2-, and 3-year follow-up, respectively. Conclusions: Intravitreal anti-VEGF drugs in a PRN regimen with close monitoring appear to slow the progression of CNV in AS, but they do not prevent the affected eyes from progressive visual loss.

To investigate saccadic movements in subjects with eccentric fixation due to a deep central scotoma in Stargardt disease (STGD). We studied 10 patients with STGD and 10 healthy subjects (control group). Saccadic movements of all the 20 subjects were assessed by using the eye tracker technique Tobii Glasses Pro 2. Standard measurements of reading ability (MNREAD charts), visual acuity (ETDRS charts), contrast sensitivity (Pelli-Robson charts), reading contrast threshold and speed (REX charts), retinal sensitivity and stability and localization of the fixation (MP1 fundus perimetry) were obtained in all subjects. The saccadic movements time was significantly slower in STGD than in healthy subjects (699 ± 193 ms vs 299 ± 40 ms, p < 0.001). When STGD patients moved fixation to the target localized in retinal scotomatous areas, the movement was significantly slower compared to non scotomatous areas in the retina (1103 ± 798 ms vs 524 ± 187 ms, p = 0.039). There was a trend toward a correlation between slow saccadic movements in STGD subjects and the reading performance indices, although statistical significance was not achieved. Ocular saccades guided by eccentric fixation in STGD patients are significantly slower than in the control group, especially when the target corresponds to retinal areas with a deep scotoma. These results can explain the worse reading performance in STGD subjects, in particular when a non-viewing area on the right part of the text is present.