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Gyrate atrophy (GA) of the choroid and retina is a rare autosomal recessive genetic condition characterized by elevation of the plasma level of the amino acid ornithine due to deficiency of the enzyme ornithine ketoacid aminotransferase. Accumulation of ornithine occurs in various body tissues but leads primarily to characteristic ophthalmic manifestations including myopia, cataract, progressive chorioretinal atrophy, and macular changes. Patients usually present with night blindness that starts in the first decade of life followed by visual field constriction and eventually diminution of the central visual acuity and blindness. The condition has been reported worldwide and its differential diagnosis is broad and includes choroideremia and retinitis pigmentosa. Treatment currently depends on life-long dietary modifications including restriction of the amino acid arginine in diet. This article describes in detail the pathogenesis, clinical features, multimodal imaging findings, and treatment options for GA of the choroid and retina and its complications.

We provide a systematic review of published cases of optic neuropathy following COVID-19 vaccination. We used Ovid MEDLINE, PubMed, and Google Scholar. Search terms included: “COVID-19 vaccination”, “optic neuropathy”, “optic neuritis”, and “ischemic optic neuropathy”. The titles and abstracts were screened, then the full texts were reviewed. Sixty eyes from forty-five patients (28 females) were included. Eighteen eyes from fourteen patients (31.1%) were diagnosed with anterior ischemic optic neuropathy (AION), while 34 eyes from 26 patients (57.8%) were diagnosed with optic neuritis (ON). Other conditions included autoimmune optic neuropathy and Leber hereditary optic neuropathy. Fifteen patients (33.3%) had bilateral involvement. The mean age of all patients was 47.4 ± 17.1 years. The mean age of AION patients was 62.9 ± 12.2 years and of ON patients was 39.7 ± 12.8 years (p < 0.001). The mean time from vaccination to ophthalmic symptoms was 9.6 ± 8.7 days. The mean presenting visual acuity (VA) was logMAR 0.990 ± 0.924. For 41 eyes with available follow-up, the mean presenting VA was logMAR 0.842 ± 0.885, which improved to logMAR 0.523 ± 0.860 at final follow-up (p < 0.001). COVID-19 vaccination may be associated with different forms of optic neuropathy. Patients diagnosed with ON were more likely to be younger and to experience visual improvement. More studies are needed to further characterize optic neuropathies associated with COVID-19 vaccination.

Background. Diabetic macular edema (DME) is a major cause of vision loss in diabetics that is currently mainly treated by antivascular endothelial growth factor (VEGF) agents. The effect of these agents on macular perfusion (MP) is a current concern. Optical coherence tomography angiography (OCTA) is an imaging modality that allows noninvasive high-resolution retinal microvasculature imaging. Several recent studies evaluated the effect of anti-VEGF agents on the MP of DME patients using OCTA. Our aim is to provide a systematic review of these studies. Methods. Multiple databases were searched including PubMed, Ovid Medline, EMBASE, and Google Scholar for relevant studies published between January 2016 and November 2020 which were included in this review. Studies were compared regarding their design, the number of included patients, the machine and scanning protocol used, the inclusion and exclusion criteria, the number of injections given, the type of anti-VEGF agent used, the outcome measures assessed, and the effect of injections on different MP parameters. Results. A total of 16 studies were included. The studies assessed various OCTA parameters that define MP including the foveal avascular zone area and superficial and deep vascular density and yielded conflicting results. Seven studies showed stable or improved MP following treatment, while 7 studies showed worsening MP following treatment, and 2 studies showed inconclusive results. This could have been due to differences in study design, inclusion criteria, type of anti-VEGF agents used, treatment duration, and methods of image analysis and vascular density quantification. All identified studies were noncomparative case series, and 14 of them (87.5%) used the RTVue XR Avanti OCTA machine. Only one study compared OCTA to fluorescein angiography findings. Conclusion. Analysis of MP changes following VEGF inhibition for DME could benefit from a unified scanning protocol and analysis approach that uses similar study designs to eliminate potential sources of bias. This may provide more definitive conclusions regarding the effect of treatment on MP.

Purpose: To evaluate the use of intravitreal bevacizumab injections for the treatment of intraretinal cystic spaces associated with gyrate atrophy of the choroid and retina. Methods: Retrospective chart review of 5 eyes of 3 patients with intraretinal cystic spaces associated with gyrate atrophy and treated with intravitreal bevacizumab injections was performed. Information obtained included history, examination findings, optical coherence tomography (OCT), OCT angiography, fluorescein angiography, and microperimetric findings before and after the injections. Results: The mean age of patients was 11 ± 4.6 years. All patients received three monthly bevacizumab injections. The mean corrected distance visual acuity was 0.27 ± 0.10 at baseline and improved to 0.36 ± 0.12 after the injections ( P = .015). The mean central macular thickness was 569 ± 127 µm at baseline and improved to 422 ± 123 µm after the injections ( P = .067). Microperimetry and OCT angiography performed in 1 patient before and after the three injections showed improved macular sensitivity and vascular density measurements following the injections. Conclusions: Intravitreal bevacizumab is safe and effective in the treatment of intraretinal cystic spaces associated with gyrate atrophy. [ J Pediatr Ophthalmol Strabismus . 2020;57(6):400–406.]

Objective. To evaluate macular perfusion changes following intravitreal bevacizumab injections for diabetic macular edema (DME) using spectral domain optical coherence tomography angiography (SD-OCTA). Methods. This study was a prospective noncomparative interventional case series. Treatment naïve patients with DME underwent full ophthalmological examination and SD-OCTA scanning at baseline and after 3 intravitreal bevacizumab injections. Both the 6 × 6 and 3 × 3 mm macular scan protocols were used. Pretreatment and posttreatment OCTA images were automatically aligned using a commercially available retina alignment software (i2k Align Retina software); then the fractal dimension (FD), vascular density (VD), and skeleton VD changes were obtained at the full retinal thickness (Full) and superficial (SCP) and deep (DCP) capillary plexuses after processing images using a semiautomated program. The foveal avascular zone (FAZ) was manually measured and FD was calculated using the FracLac plugin of ImageJ. Results. Forty eyes of 26 patients were included. Following injections, there were an 8.1% increase in FAZ, 1.3% decrease in FD-Full and FD-SCP, 1.9% decrease in FD-DCP, 8% decrease in VD-Full, 9.1% decrease in VD-SCP, 10.6% decrease in VD-DCP, 13.3% decrease in skeleton VD-Full, 12.5% decrease in skeleton VD-SCP, and 16.3% decrease in skeleton VD-DCP in the 6 × 6 mm macular area and a 2.6% decrease in FD-Full, 3.4% decrease in FD-SCP, 11.5% decrease in VD-Full, 14.3% decrease in VD-SCP, and 25.1% decrease in skeleton VD-SCP in the 3 × 3 mm macular area which were all statistically significant p<0.05. Using univariate and multivariate analysis, the pretreatment FD, VD, and skeleton VD at each capillary layer significantly negatively correlated with the change in FD, VD, and skeleton VD at the corresponding capillary layer, respectively p<0.05. Conclusion. OCTA is a useful noninvasive tool for quantitative evaluation of macular perfusion changes following DME treatment. This trial is registered with NCT03246152.

A 53-year-old female patient with center-involving diabetic macular edema affecting the left eye was imaged using optical coherence tomography angiography (OCTA) in both eyes. She underwent three monthly intravitreal bevacizumab injections in the left eye only and OCTA was repeated in both eyes one month following the last injection and showed decreased vascular density (VD) in the treated left eye but not in the untreated right eye compared to baseline. No further injections were required in either eye, and OCTA was done in both eyes 4 months following the last injection which showed improved VD of the left eye with stable VD in the right. Three monthly intravitreal bevacizumab injections were then required in both eyes; then OCTA was repeated following the last injection and revealed decreased VD in both eyes compared to previous scan. OCTA could be a useful tool for detecting VD changes following bevacizumab injections in diabetics.