Fabry disease (FD) is an X-linked lysosomal storage disorder, causing Gb-3 (globotriaosylceramide) buildup in cellular lysosomes throughout the body, in particular in blood vessel walls, neuronal cells, and smooth muscle. The gradual accumulation of this glycosphingolipid in numerous eye tissues causes conjunctival vascular abnormalities, corneal epithelial opacities (cornea verticillata), lens opacities, and retinal vascular abnormalities. Although a severe vision impairment is rare, these abnormalities are diagnostic indicators and prognostics for severity. Cornea verticillata is the most common ophthalmic feature in both hemizygous men and heterozygous females. Vessel tortuosity has been linked to a faster disease progression and may be useful in predicting systemic involvement. New technologies such as optical coherence tomography angiography (OCTA) are useful for monitoring retinal microvasculature alterations in FD patients. Along with OCTA, corneal topographic analysis, confocal microscopy, and electro-functional examinations, contributed to the recognition of ocular abnormalities and have been correlated with systemic involvement. We offer an update regarding FD ocular manifestations, focusing on findings derived from the most recent imaging modalities, to optimize the management of this pathology.

Introduction Choroidal thickness (CT) plays an important role in the pathogenesis of various ocular diseases, including neovascular age-related macular degeneration (nAMD). Previous studies evaluated the CT variations after anti-vascular endothelial growth factor (VEGF) injections in patients with nAMD, but the results are still controversial. The present study aimed to evaluate the CT at different times (15, 30, 60, 90, and 365 days) after intravitreal aflibercept injections and its correlation with the baseline CT in treatment-naïve patients with nAMD. Secondly, the study evaluated the correlation between CT variation at 365 days and the number of intravitreal injections received. Methods This was a prospective, open-label, single-arm pilot study. Twenty-one treatment-naïve nAMD eyes were enrolled. The study population underwent three monthly aflibercept injections (loading phase) and additional injections as needed (pro re nata regimen). A complete ophthalmological examination, including optical coherence tomography (OCT) was performed at each visit. CT was measured manually by two independent observers. All patients were evaluated at baseline and at 15, 30, 60, 90, and 365 days after the first intravitreal injection. Results CT showed a statistically significant reduction at days 15, 90, and 365 in comparison to baseline. However, the major reduction of CT was observed at day 15 and in eyes with a thicker choroid at baseline. No significant correlation between CT variation and the number of injections performed was found. Conclusion Our findings contribute to clarifying the role of aflibercept injections in choroidal vasculature, confirming its effect after the first 2 weeks. Moreover, CT can be considered as a potential biomarker, as it reflects the pharmacological effect of anti-VEGF drugs.

Background: To investigate macular microvascular changes after uncomplicated phacoemulsification surgery according to the cataract severity grade. Methods: Retrospective, cross-sectional study involving 23 eyes of 23 patients who underwent elective cataract extraction. All patients underwent routine ophthalmologic examination, including optical coherence tomography angiography (OCTA) at baseline (preoperative visit, T0) and seven days postoperatively (T7). OCTA scans were obtained with the spectral domain system Cirrus 5000 (Carl Zeiss Meditec, Inc., Dublin, CA, USA), and 3 mm × 3 mm raster fovea-centered scans were obtained to evaluate the superficial capillary plexus (SCP) vessel density, perfusion density, and foveal avascular zone (FAZ) parameters. Results: SCP perfusion density significantly increased from 28.3 ± 5.73% to 33.74 ± 4.13% after the surgery (p < 0.001). Similarly, SCP vessel density significantly increased from 15.14 ± 3.41 mm⁻¹ to 18.14 ± 2.57 mm⁻¹ after surgery (p < 0.001). The mean preoperative FAZ area significantly increased from 0.27 ± 0.12 mm to 0.24 ± 0.11 mm seven days postoperatively (p = 0.008). When comparing softer and harder cataracts, no significant variations in SCP vessel density, as well as SCP perfusion density parameters and the FAZ area, perimeter, and circularity index, were noted before and after surgery. Conclusions: Macular SPC vessel density and macular SCP perfusion density increase after uncomplicated cataract surgery regardless of the cataract severity.

Background: Visual impairment in terms of reduced visual acuity and “visual loss” has been reported as an atypical symptom in patients with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. This systematic review and meta-analysis aims to assess the cumulative incidence of “visual loss” during coronavirus disease 2019 (COVID-19) and review the current evidence regarding “visual loss” caused by SARS-CoV-2 infection. Methods: We performed a systematic review and meta-analysis of studies following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We systematically searched the PubMed, Embase, and Scopus databases for relevant studies published that clearly described “vision loss” and SARS-CoV-2 infection. All studies reporting concomitant “vision loss” and laboratory-confirmed SARS-CoV-2 infection were included. Meta-analyses were conducted using the measurement of risk and a 95% confidence interval for each study. Results: Our search identified 1143 manuscripts published in the English language. After study screening, twenty-nine articles were selected: two cross-sectional studies, twenty-four case reports, and three case series. A random-effect meta-analysis demonstrated that the pooled “visual loss” cumulative incidence in COVID-19 patients was 0.16 (95% CI 0.12–0.21). The quality rating of the cross-sectional studies averaged four out of the maximum score on the Newcastle–Ottawa scale. Conclusions: COVID-19 infection might cause “visual loss”. Even if the current evidence is limited, ophthalmological assessment should be promptly provided to all patients experiencing visual impairment symptoms during SARS-CoV-2 infection.

Background: Endothelial cells damage and thromboinflammation are considered key elements in the generation of organ impairment in patients with COVID-19 disease. The endothelial function is evaluated by measuring flow-mediated dilation (FMD). We aimed to analyze the association between FMD impairment and retinal vascular parameters in early post-COVID-19 patients. 00118-00199Tomography (OCT), OCT Angiography (OCTA) and slit lamp examination were performed. FMD ≤ 7% was considered as pathological. Our primary outcome was to assess potential differences in the radial peripapillary capillary plexus flow index (RPCP-FI) and RPCP density (RPCP-D) values between post-COVID-19 patients with and without FMD impairment. The associations of other retinal vascular parameters with FMD impairment were assessed as secondary endpoints. Results: FMD impairment was detected in 31 patients (37.8%). RPCP-FI (p = 0.047), age (p = 0.048) and prevalence of diabetes (p = 0.046) significantly differed in patients with FMD ≤ 7% in regression analysis. RPCP-FI was linearly correlated with FMD values (R = 0.244, p =0.027). SCT was found to be lower in patients with impaired FMD (p = 0.004), although this difference was only a trend in binary logistic regression output (p = 0.07). Conclusions: Early post-COVID-19 patients showed a higher prevalence of FMD impairment compared to the general population. Age, diabetes and RPCP-FI were independently correlated with the presence of endothelial impairment in the early post-infective period.

(1) Purpose: To determine the characteristics of macular epiretinal membranes (ERM) using non-invasive retromode imaging (RMI) and to compare retromode images with those acquired via fundus autofluorescence (FAF) and fundus photography. (2) Methods: Prospective observational case-series study including patients with macular ERM with no other ocular disease affecting their morphology and/or imaging quality. We compared RMI, FAF and fundus photography features by cropping and overlapping images to obtain topographic correspondence. (3) Results: In total, 21 eyes (21 patients) affected by ERM were included in this study. The mean area of retinal folds detected by RMI was significantly higher than that detected by FAF (11.85 ± 3.92 mm² and 5.67 ± 2.15 mm², respectively, p < 0.05) and similar to that revealed by fundus photography (11.85 ± 3.92 mm² and 10.58 ± 3.45 mm², respectively, p = 0.277). (4) Conclusions: RMI appears to be a useful tool in the evaluation of ERMs. It allows for an accurate visualization of the real extension of the retinal folds and provides a precise structural assessment of the macula before surgery. Clinicians should be aware of RMI’s advantages and should be able to use them to warrant a wide range of information and, thus, a more personalized therapeutic approach.