Aim To describe the optical coherence tomography angiography (OCT-A) features of patients affected by acute macular neuroretinopathy (AMN). Methods This is a prospective, observational, cross-sectional study. Multimodal imaging, including spectral domain OCT (Spectralis HRA+OCT; Heidelberg Engineering, Heidelberg, Germany) and 6×6 mm swept source OCT-A (ZEISS PLEX Elite 9000; ZEISS, Dublin, California), was performed on all patients. The primary outcome measure was assessment of the vascular alterations of the superficial (SCP) and deep (DCP) capillary plexuses and choriocapillaris (CC). A segmentation of the AMN lesions using enface OCT images of photoreceptors-retinal pigment epithelium complex was used to discriminate intralesional and extralesional regions on the OCT-A enface slabs of the DCP and CC reconstructions. Each OCT-A slab was imported into ImageJ V.1.50 and digitally binarised for quantitative analyses. Results Overall, seven patients (mean age 19.4±3.2 years, six women) affected by AMN were included. The mean best-corrected visual acuity was 0.00±0.00 logarithm of the minimum angle of resolution. Twelve healthy age-matched subjects (mean age 22±3.4 years, 10 women) represented the control group. The quantitative analysis of global vessel densities showed that the CC vessel density was significantly lower in patients with AMN (0.495±0.03) compared with the age-matched controls (0.545±0.02) (corrected p=0.0003). The intralesional vessel density of the DCP was 0.349±0.04 in AMN and vs 0.497±0.02 in the controls (corrected p=0.0002). Conclusions Our study confirmed inner choroidal vascular flow void as a possible pathogenetic mechanism of AMN. We also found a focal impairment of the DCP within the AMN lesions. Future studies are needed to clarify which is the primary location of the vascular insult in this condition.

Purpose: To report acute macular neuroretinopathy (AMN) in two young women two days after receiving Vaxzevria Coronavirus disease (COVID-19) vaccination. Methods: Observational case reports. Observation: The first patient was an Italian 22-year-old female with acute onset of paracentral scotoma two days post vaccination. The second patient was an Austrian 28-year-old female who presented with sudden onset paracentral scotoma two days after vaccination. Multimodal retinal imaging was consistent with AMN in both cases. Both patients were on long-term oral contraceptives, had no history of COVID-19 and experienced one-day duration fever the day after the vaccination. Conclusions: Vaccination may represent a possible risk factor for AMN onset in women on oral contraceptives. We encourage ophthalmologists to investigate recent vaccination status when dealing with new onset AMN. Further studies are needed to assess the link between vaccinations and AMN.

To describe the photoreceptor-retinal pigment epithelium (RPE) interface changes and to analyze the relationships between these features and hyperreflective material (HRM) with scarring and atrophy at the macula of patients with neovascular age-related macular degeneration (nAMD). Retrospective single-center observational study. A total of 150 eyes from 144 patients with naive nAMD were included. All patients had OCT (HRA-OCT Spectralis, Heidelberg Engineering, Heidelberg, Germany) at baseline and at 1, 3, 6, and 12 months. Macular scar and macular atrophy (MA) were determined on multimodal imaging, including color fundus (CF) and near-infrared imaging at baseline and month 12 (M12). Change in HRM type (undefined and well-defined) and location, development of fibrotic or nonfibrotic macular scar, MA, and best-corrected visual acuity (BCVA) at M12. At baseline, eyes with fibrin on CF had thicker and wider HRM on OCT that correlated strongly with presence of undefined HRM. The proportion of eyes with undefined HRM fell dramatically by month 1 but well-defined HRM increased. At M12 defined HRM was strongly associated with macular scar (chi-square, 82.1; P < 0.001). Ordinal regression showed that both the thickness and the width of HRM were significant risk factors for development of fibrotic scar (P < 0.001 and P = 0.02) but not nonfibrotic scars (P = 0.67 and P = 0.65). Fibrotic macular scar (P = 0.001) but not nonfibrotic scar (P = 0.129) negatively affected visual acuity at M12. Ordinal regression showed that the risk factors for progression to MA were reticular pseudodrusen and thinner HRM (P = 0.017 and P = 0.028, respectively). MA negatively affected BCVA at M12 (P < 0.001). Our study supports the role of HRM as an important biomarker for the evolution of macular scar and atrophy in patients with nAMD undergoing treatment with anti-VEGF therapies. Undefined HRM can resolve with treatment, whereas well-defined HRM likely contains vascular complexes and fibrotic elements.

Purpose: To correlate presence of hyperreflective material (HRM) on spectral-domain optical coherence tomography (SD-OCT) with color fundus photography (CFP) in patients with different subtypes of neovascular age-related macular degeneration (n-AMD). Methods: Retrospective assessments were made at baseline and months 1, 3, and 12 after initiation of treatment. At baseline, CFP images were graded for the presence of blood, fibrin and lipid exudates, and retinal angiograms for n-AMD subtype. At the four selected visits, SD-OCT scans were graded for HRM type (well-defined or undefined) and location (subretinal, intraretinal, and subretinal pigment epithelium [RPE]), integrity of RPE, ellipsoid zone, and external limiting membrane (ELM). Results: A total of 121 eyes with active n-AMD from 117 patients were included. At baseline, undefined HRM was strongly associated with fibrin on CFP (χ² = 39.87; P < 0.001). The overall prevalence of HRM decreased from 85.9% at baseline to 52.9% by month 12. From baseline to month 12, undefined HRM decreased (53.7% vs. 7.4%, respectively) and well-defined HRM increased (32.2% vs. 45.5%, respectively). Sub-RPE HRM, which was infrequent at baseline, increased up to 30.6% by month 12. At month 12, eyes with no HRM had the best mean final best-corrected visual acuity (BCVA), and those with undefined HRM the worst. Multivariate regression analysis showed that ELM disruption at both baseline and month 12 was a negative predictive factor for final BCVA (P = 0.001 and P < 0.001, respectively), whereas presence of subretinal fluid at month 12 and number of treatments were positive predictors for final BCVA (P = 0.007 and P = 0.041, respectively), but the covariates describing HRM did not reach statistical significance in these models. Conclusions: In eyes with n-AMD, location and morphology of HRM changed after anti-VEGF treatment, and differences were observed in the various choroidal neovascularization (CNV) subtypes. After anti-VEGF treatment, it was well-defined HRM in the sub-RPE space that was observed mostly.

To investigate the clinical course, genetic findings, and phenotypic spectrum of autosomal recessive bestrophinopathy (ARB) in a large cohort of children and adults. Retrospective case series. Patients with a detailed clinical phenotype consistent with ARB, biallelic likely disease-causing sequence variants in the BEST1 gene, or both identified at a single tertiary referral center. Review of case notes, retinal imaging (color fundus photography, fundus autofluorescence, OCT), electrophysiologic assessment, and molecular genetic testing. Visual acuity (VA), retinal imaging, and electrophysiologic changes over time. Fifty-six eyes of 28 unrelated patients were included. Compound heterozygous variants were detected in most patients (19/27), with 6 alleles recurring in apparently unrelated individuals, the most common of which was c.422G→A, p.(Arg141His; n = 4 patients). Mean presenting VA was 0.52 ± 0.36 logarithm of the minimum angle of resolution (logMAR), and final VA was 0.81 ± 0.75 logMAR (P = 0.06). The mean rate of change in VA was 0.05 ± 0.13 logMAR/year. A significant change in VA was detected in patients with a follow-up of 5 years or more (n = 18) compared with patients with a follow-up of 5 years or less (n = 10; P = 0.001). Presence of subretinal fluid and vitelliform material were early findings in most patients, and this did not change substantially over time. A reduction in central retinal thickness was detected in most eyes (80.4%) over the course of follow-up. Many patients (10/26) showed evidence of generalized rod and cone system dysfunction. These patients were older (P < 0.001) and had worse VA (P = 0.02) than those with normal full-field electroretinography results. Although patients with ARB are presumed to have no functioning bestrophin channels, significant phenotypic heterogeneity is evident. The clinical course is characterized by a progressive loss of vision with a slow rate of decline, providing a wide therapeutic window for anticipated future treatment strategies.