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Objective: To evaluate visual outcomes after switching from bevacizumab to ranibizumab or aflibercept in patients with macular edema (ME) secondary to branch retinal vein occlusion (BRVO)., Design: A retrospective, multi-center, observational study., Participants: Patients diagnosed with BRVO and were treated with at least 3 bevacizumab injections, before anti VEGF switch., Methods: The follow-up period was 36 months, and the primary study outcomes assessed changes in best corrected visual acuity (BCVA) after anti VEGF switch., Results: A total of 263 eyes of 263 patients with a mean age of 71.5 ± 11.2 years of which 50% were of male gender met the inclusion criteria. Of them, 175 eyes did not undergo switch, whereas 88 eyes underwent anti-VEGF switch. There was not a significant difference in mean age (p = 0.634) and gender (p = 0.269) between the groups. Baseline BCVA of the no-switch group was 0.47 ± 0.43 logMAR (20/59 Snellen) versus 0.6 ± 0.49 logMAR (20/79 Snellen) (p = 0.031) in the switch group, and at 36-months it was 0.41 ± 0.39 (20/51 Snellen) logMAR versus 0.54 ± 0.49 logMAR (20/69 Snellen) (p = 0.035), respectively. The difference between the rate of change in BCVA per year was insignificant between groups (p = 0.414). In multivariate analysis, baseline BCVA was the single significant predictor for switch (beta 0.137, p = 0.035). Patients with more than one anti-VEGF switch suffer from decrease in BCVA., Conclusions: Worse baseline BCVA is a significant predictor for anti-VEGF switch execution, though the switch has no significant impact on the change in BCVA over time. Multiple anti-VEGF switch is not recommended., (Copyright © 2024 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

In age-related macular degeneration (AMD), large choroidal hypertransmission defects (hyperTDs) are identified on en face optical coherence tomography (OCT) images as bright lesions measuring at least 250 μm in greatest linear dimension (GLD). These choroidal hyperTDs arise from focal attenuation or loss of the retinal pigment epithelium (RPE). We previously reported that once large hyperTDs formed, they were likely to persist compared with smaller lesions that were more likely to be transient. Due to their relative persistence, these large persistent choroidal hyperTDs are a point-of-no-return in the progression of intermediate AMD to the late stage of atrophic AMD. Moreover, the onset of these large choroidal hyperTDs can serve as a clinical trial endpoint when studying therapies that might slow disease progression from intermediate AMD to late atrophic AMD. To confirm the persistence of these large choroidal hyperTDs, we studied an independent dataset of AMD eyes enrolled in an ongoing prospective swept-source OCT (SS-OCT) natural history study to determine their overall persistence. We identified a total of 202 eyes with large choroidal hyperTDs containing 1725 hyperTDs followed for an average of 46.6 months. Of the 1725 large hyperTDs, we found that 1718 (99.6%) persisted while only 7 hyperTDs (0.4%) were non-persistent. Of the 7 non-persistent large hyperTDs in 6 eyes, their average GLD at baseline was 385 μm. Of the large hyperTDs ranging in size between 250 and 300 μm when first detected, only one was not persistent with a baseline GLD of 283 μm. In 6 of the non-persistent hyperTDs, the loss of a detectable large hyperTD was due to the accumulation of hyperreflective material along the retinal pigment epithelium (RPE) and in the retina over the area where the hyperTD was located. This hyperreflective material is thought to represent the migration and aggregation of RPE cells into this focal region where the choroidal hyperTD arose due to attenuated or lost RPE., Competing Interests: Declaration of competing interest Giovanni Gregori, Philip J. Rosenfeld and Ruikang K. Wang received research support from Carl Zeiss Meditec, Inc. Giovanni Gregori and the University of Miami co-own a patent that is licensed to Carl Zeiss Meditec, Inc. Dr. Rosenfeld also received research funding from Gyroscope Therapeutics. He is also a consultant for Abbvie, Annexon, Apellis, Bayer Pharmaceuticals, Boehringer-Ingelheim, Carl Zeiss Meditec, Chengdu Kanghong Biotech, Genentech/Roche, InflammX Therapeutics, Ocudyne, Regeneron Pharmaceuticals, and Unity Biotechnology. He also has equity interest in Apellis, InflammX, Ocudyne, and Valitor. Ruikang K. Wang received other financial support from Colgate Palmolive Company, Estee Lauder lnc, and is a consultant for Carl Zeiss Meditec. He also has several patents: US8, 750, 586, US8, 180, 134, US9, 282,905, US9, 759,544, US10, 354,378, US10, 529,061. The remaining authors have no disclosures., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Purpose: The association between the total macular burden of hyperreflective foci (HRF) in eyes with intermediate AMD (iAMD) and the onset of persistent choroidal hypertransmission defects (hyperTDs) was studied using swept-source optical coherence tomography (SS-OCT)., Design: Post hoc subgroup analysis of a prospective study., Methods: A retrospective review of iAMD eyes from subjects enrolled in a prospective SS-OCT study was performed. All eyes underwent 6×6 mm SS-OCT angiography (SS-OCTA) imaging at baseline and follow-up visits. En face sub-retinal pigment epithelium (subRPE) slabs with segmentation boundaries positioned 64 to 400 µm beneath Bruch's membrane (BM) were used to identify persistent choroidal hyperTDs. None of the eyes had persistent hyperTDs at baseline. The same subRPE slab was used to identify choroidal hypotransmission defects (hypoTDs) attributable to HRF located either intraretinally (iHRF) or along the RPE (rpeHRF) based on corresponding B-scans. A semiautomated algorithm was used by 2 independent graders to validate and refine the HRF outlines. The HRF area and the drusen volume within a 5 mm fovea-centered circle were measured at each visit., Results: The median follow-up time for the 171 eyes from 121 patients included in this study was 59.1 months (95% CI: 52.0-67.8 months). Of these, 149 eyes (87%) had HRF, and 82 (48%) developed at least one persistent hyperTD during the follow-up. Although univariable Cox regression analyses showed that both drusen volume and total HRF area were associated with the onset of the first persistent hyperTD, multivariable analysis showed that the area of total HRF was the sole significant predictor for the onset of hyperTDs (P < .001). ROC analysis identified an HRF area ≥ 0.07 mm² to predict the onset of persistent hyperTDs within 1 year with an area under the curve (AUC) of 0.661 (0.570-0.753), corresponding to a sensitivity of 55% and a specificity of 74% (P < .001)., Conclusions: The total macular burden of HRF, which includes both the HRF along the RPE and within the retina, is an important predictor of disease progression from iAMD to the onset of persistent hyperTDs and should serve as a key OCT biomarker to select iAMD patients at high risk for disease progression in future clinical trials., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: The gradings of complete retinal pigment epithelium and outer retinal atrophy (cRORA) and incomplete retinal pigment epithelium and outer retinal atrophy (iRORA) on spectral domain optical coherence tomography (SD-OCT) B-scans were compared with the grading of persistent choroidal hypertransmission defects (hyperTDs) on swept-source OCT angiography (SS-OCTA) en face images., Design: Comparative diagnostic analysis of prospective study data METHODS: Patients with late nonexudative AMD underwent same day 6 × 6 mm macular scans using both SD-OCT (Spectralis® Heidelberg, 512 × 97, ART:9) and SS-OCTA (PLEX® Elite 9000, Carl Zeiss Meditec, 500 × 500 angio pattern) instruments. SS-OCTA and SD-OCT en face images were generated from a sub-retinal pigment epithelium slab positioned 64-400 μm below Bruch's membrane. SD-OCT B-scan gradings, which included an inspection of neighboring B-scans for the diagnosis of cRORA and iRORA, were performed at the Moran Eye Center, while gradings of en face images to identify persistent choroidal hyperTDs were performed at the Bascom Palmer Eye Institute and Tel Aviv Medical Center., Results: There was a high degree of agreement (99.6%) between the gradings of cRORA lesions and persistent hyperTDs. However, 27.4% of iRORA lesions were found to be contained within persistent hyperTDs. This discrepancy was due to the finding that 27.5% of iRORA lesions were diagnosed as having a greatest linear horizontal dimension of < 250 µm on B-scans, but on en face images, these B-scan defined iRORA lesions were found to have a greatest linear dimensions in the non-horizontal dimension that were ≥ 250 µm., Conclusion: This report demonstrates the benefits of using en face OCT imaging to identify cRORA lesions and highlights the need to acquire dense raster B-scans with the grading neighboring B-scans when identifying iRORA lesions to assess the full extent of the iRORA lesions in the non-horizontal dimension. Even though neighboring B-scans were inspected, 27.5% of iRORA lesions were actually part of larger cRORA lesions when graded using an en face strategy., Competing Interests: Declaration of competing interest Dr. Marc Steffen Schmitz-Valckenberg: Grants or contracts from AlphaRET, Apellis, Bayer, Formycon, Carl Zeiss MediTec, eyeDNA Therapeutics, Galimedix, Katairo, Kubota Vision, Novartis, Perceive Therapeutics, Pixium, Roche/Genentech, SparingVision; Consulting fees from Apellis, Roche/Genentech; and nonfinancial support from Carl Zeiss Meditec and Heidelberg Engineering, outside the submitted work. Dr. Ruikang Wang discloses intellectual property owned by the Oregon Health and Science University and the University of Washington. Dr. Wang also receives research support from Carl Zeiss Meditec Inc, Colgate Palmolive Company and Estee Lauder Inc. He is a consultant to Carl Zeiss Meditec and Cyberdontics. Dr. Philip Rosenfeld and Dr. Giovanni Gregori receive research support from Carl Zeiss Meditec, Inc. and the University of Miami co-own a patent that is licensed to Carl Zeiss Meditec, Inc. Dr. Philip Rosenfeld also received research funding from Gyroscope Therapeutics. He is also a consultant for Abbvie, Annexon, Apellis, Bayer Pharmaceuticals, Boehringer-Ingelheim, Carl Zeiss Meditec, Genentech/Roche, InflammX Therapeutics, Ocudyne, Regeneron Pharmaceuticals, and Unity Biotechnology. He also has equity interest in Apellis, InflammX, Ocudyne, and Valitor. The other authors have no disclosures., (Copyright © 2024. Published by Elsevier Inc.)

Purpose: To assess the effect of blue-light filtering (BLF) intraocular lenses (IOLs) on the development and progression of macular atrophy (MA) in eyes with neovascular age-related macular degeneration (nAMD)., Design: Retrospective, clinical cohort study., Methods: The study included patients with nAMD with anti-vascular endothelial growth factor (VEGF) injections who underwent uneventful cataract surgery between 2007 and 2018 with follow-up until June 2023. Subsequent MA rates were compared between subjects who received a BLF IOL or a non-BLF IOL. All optical coherence tomography scans were manually reviewed in a masked manner regarding patient baseline variables and IOL status by an experienced research technician. By using Heidelberg software, the area of MA was manually evaluated and calculated (mm 2 ) by the program. The overall risk of developing new-onset MA and the effect of IOL type on disease progression were assessed. Death was included as a censoring event., Results: Included were 373 eyes of 373 patients (mean age, 78.6 ± 6.7 years at surgery; 67.4% were female). BLF IOLs were implanted in 206 eyes, and non-BLF IOLs were implanted in 167 eyes with comparable follow-up times (3164 ± 1420 days vs 3180 ± 1403 days, respectively, P = .908) and other baseline parameters (age, gender, corrected distance visual acuity, macular thickness, cumulative number of anti-VEGF injections). Nine preexisting and 77 new-onset MA cases were detected, with similar distribution between BLF and non-BLF eyes (P = .598 and P = .399, respectively). Both univariate Kaplan-Meier (P = .366) and multivariate Cox regression analyses adjusted for age and gender showed that BLF-IOLs were comparable to non-BLF IOLs regarding hazard for new-onset MA (hazard ratio [HR], 1.236; 95% CI, 0.784-1.949; P = .363). Final MA area at the last visit was 5.14 ± 4.71 mm 2 for BLF IOLs and 8.56 ± 9.17 mm 2 for non-BLF IOLs (P = .028), with the mean annual MA area increase of 0.78 ± 0.84 mm 2 and 1.26 ± 1.32 mm 2 , respectively (P = .042)., Conclusions: BLF IOLs did not show added benefit over non-BLF IOLs in terms of MA-free survival but were associated with less progression over time in a cohort of patients with nAMD., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose: Swept-source OCT angiography (SS-OCTA) scans of eyes with age-related macular degeneration (AMD) were used to replace color, autofluorescence, infrared reflectance, and dye-based fundus angiographic imaging for the diagnosis and staging of AMD. Through the use of different algorithms with the SS-OCTA scans, both structural and angiographic information can be viewed and assessed using both cross sectional and en face imaging strategies., Design: Presented at the 2022 Charles L. Schepens, MD, Lecture at the American Academy of Ophthalmology Retina Subspecialty Day, Chicago, Illinois, on September 30, 2022., Participants: Patients with AMD., Methods: Review of published literature and ongoing clinical research using SS-OCTA imaging in AMD., Main Outcome Measures: Swept-source OCT angiography imaging of AMD at different stages of disease progression., Results: Volumetric SS-OCTA dense raster scans were used to diagnose and stage both exudative and nonexudative AMD. In eyes with nonexudative AMD, a single SS-OCTA scan was used to detect and measure structural features in the macula such as the area and volume of both typical soft drusen and calcified drusen, the presence and location of hyperreflective foci, the presence of reticular pseudodrusen, also known as subretinal drusenoid deposits, the thickness of the outer retinal layer, the presence and thickness of basal laminar deposits, the presence and area of persistent choroidal hypertransmission defects, and the presence of treatment-naïve nonexudative macular neovascularization. In eyes with exudative AMD, the same SS-OCTA scan pattern was used to detect and measure the presence of macular fluid, the presence and type of macular neovascularization, and the response of exudation to treatment with vascular endothelial growth factor inhibitors. In addition, the same scan pattern was used to quantitate choriocapillaris (CC) perfusion, CC thickness, choroidal thickness, and the vascularity of the choroid., Conclusions: Compared with using several different instruments to perform multimodal imaging, a single SS-OCTA scan provides a convenient, comfortable, and comprehensive approach for obtaining qualitative and quantitative anatomic and angiographic information to monitor the onset, progression, and response to therapies in both nonexudative and exudative AMD., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: Spectral-domain OCT angiography (SD-OCTA) scans were tested in an algorithm developed for use with swept-source OCT angiography (SS-OCTA) scans to determine if SD-OCTA scans yielded similar results for the detection and measurement of persistent choroidal hypertransmission defects (hyperTDs)., Design: Retrospective study., Participants: Forty pairs of scans from 32 patients with late-stage nonexudative age-related macular degeneration (AMD)., Methods: Patients underwent both SD-OCTA and SS-OCTA imaging at the same visit using the 6 × 6 mm OCTA scan patterns. Using a semiautomatic algorithm that helped with outlining the hyperTDs, 2 graders independently validated persistent hyperTDs, which are defined as having a greatest linear dimension ≥250 μm on the en face images generated using a slab extending from 64 to 400 μm beneath Bruch's membrane. The number of lesions and square root (sqrt) total area of the hyperTDs were obtained from the algorithm using each imaging method., Main Outcome Measures: The mean sqrt area measurements and the number of hyperTDs were compared., Results: The number of lesions and sqrt total area of the hyperTDs were highly concordant between the 2 instruments (r c  = 0.969 and r c  = 0.999, respectively). The mean number of hyperTDs was 4.3 ± 3.1 for SD-OCTA scans and 4.5 ± 3.3 for SS-OCTA scans ( P  = 0.06). The mean sqrt total area measurements were 1.16 ± 0.64 mm for the SD-OCTA scans and 1.17 ± 0.65 mm for the SS-OCTA scans ( P < 0.001). Because of the small standard error of the differences, the mean difference between the scans was statistically significant but not clinically significant., Conclusions: Spectral-domain OCTA scans provide similar results to SS-OCTA scans when used to obtain the number and area measurements of persistent hyperTDs through a semiautomated algorithm previously developed for SS-OCTA. This facilitates the detection of atrophy with a more widely available scan pattern and the longitudinal study of early to late-stage AMD., Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (© 2024 Published by Elsevier Inc. on behalf of the American Academy of Ophthalmology.)

Purpose: To explore visual acuity (VA) outcomes of anti-vascular endothelial growth factor (VEGF) intravitreal injections in treatment-naive eyes with diabetic macular edema (DME), with bevacizumab as first-line treatment., Methods: Retrospective single-center cohort study over a three-year follow-up. Overall, 1765 eyes from 1179 patients treated with intravitreal injections were evaluated. The cohort was divided according to the treatment given: (1) bevacizumab monotherapy, (2) eyes switched to a second-line agent, and (3) eyes switched to a third-line agent., Results: In total, 644 eyes of 444 patients met inclusion criteria. The mean age at presentation was 64.0 ± 11.1 years. The mean follow-up period was 24.6 ± 12.4 months. Furthermore, 67.1% of eyes were treated with bevacizumab monotherapy, 25.45% switched to a second-line agent, and 7.45% were switched to a third-line agent. The mean number of injections decreased significantly during each treatment year in the total cohort and within each treatment group ( P < 0.001). Mean VA for the total cohort and within each treatment group improved significantly throughout follow-up ( P < 0.001). No significant difference in VA was found between the groups ( P = 0.373)., Conclusion: This real-world study demonstrates robust and consistent VA gains over long-term follow-up in eyes with DME treated with either bevacizumab monotherapy or switching to alternative anti-VEGF agents in cases of suboptimal response.

Purpose: To report longitudinal changes in choroidal thickness and the choroidal vasculature using SS-OCT imaging in a patient with superior ophthalmic vein thrombosis (SOVT)., Observations: In a 93-year-old woman with a left-sided SOVT, the choroid in the left eye was thickened and the choroidal vessels were dilated both superiorly and inferiorly, with greater changes evident in the inferotemporal region of the choroid. After the superior ophthalmic vein was decompressed, a decrease in the choroidal thickness and choroidal vessel dilatation was observed both superiorly and inferiorly., Conclusions and Importance: In an eye with thrombosis of the superior ophthalmic vein, longitudinal SS-OCT choroidal imaging showed a greater increase in choroidal thickness and choroidal vessel dilation away from the obstructed quadrant, which improved after treatment. These observations associated with outflow obstruction may be applicable to other choroidal diseases characterized by venous overload., Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: a. Funding/Support: Research supported by grants from Carl Zeiss Meditec, Inc., the Salah Foundation, an unrestricted grant from the Research to Prevent Blindness, Inc. (New York, NY), and the National Eye Institute Center Core Grant (P30EY014801) to the Department of Ophthalmology, University of Miami Miller School of Medicine. The funding organizations had no role in the design or conduct of the present research.b. Financial Disclosures: Giovanni Gregori and Philip J. Rosenfeld received research support from Carl Zeiss Meditec, Inc. Giovanni Gregori and the University of Miami co-own a patent that is licensed to Carl Zeiss Meditec, Inc. Dr. Rosenfeld also received research funding from Gyroscope Therapeutics and Stealth BioTherapeutics. He is also a consultant for Apellis, Bayer Pharmaceuticals, Boehringer-Ingelheim, Carl Zeiss Meditec, Genentech/Roche, InflammX Therapeutics, Ocudyne, Regeneron Pharmaceuticals, and Unity Biotechnology. He also has equity interest in Apellis, InflammX, Ocudyne, and Valitor. Ruikang K. Wang received financial support from Colgate Palmolive Company, Estee Lauder lnc., and is a consultant for Carl Zeiss Meditec and Cyberdontics. He also has several patents: US8, 750, 586, US8, 180, 134, US9, 282,905, US9, 759,544, US10, 354,378, US10, 529,061. The remaining authors have no disclosures., (© 2024 Published by Elsevier Inc.)

Purpose: To evaluate the impact of the changes in treatment patterns before, during and after the COVID-19 pandemic on best-corrected visual acuity (BCVA) in previously treated neovascular age-related macular degeneration (nAMD) patients., Methods: A multi-centre, retrospective, observational study of consecutive nAMD patients during 2019-2021. Data collected included demographics, BCVA, dates of intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections and clinic visits., Results: A total of 1652 eyes of 1652 nAMD patients were included, out of which 850 eyes were assessed in 2019 (pre-COVID-19), 630 eyes were assessed in 2020 (COVID-19) and 974 eyes were assessed in 2021 (post-COVID-19). During the COVID-19 period, the mean number of anti-VEGF injections was significantly lower than the corresponding pre-COVID-19 and post-COVID-19 periods (5.55 compared to 6.13 and 6.60, respectively p < 0.01). A constant lower ratio of injections per patient/month was observed during COVID-19 compared to previous and following years, with a notable decline during March-April, reaching a ratio of 0.4 in 2020 versus 0.65 in 2019 and 0.62 in 2021 (p < 0.01). Baseline BCVA (0.825, p < 0.001), number of injections (-0.007, p < 0.001), gender (-0.027, p = 0.037) and age (0.004, p < 0.001) were shown to be significant predictors of final BCVA., Conclusion: During the COVID-19 period, patients were treated with significantly less intravitreal anti-VEGF injection compared to the previous year with compensation in the following year. These changes in treatment patterns did not have a significant impact on BCVA outcomes. Age, gender, baseline BCVA and number of injections are predictors of final visual outcomes., (© 2023 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: Patients with clinically significant carotid artery stenosis (CAS) undergoing carotid artery endarterectomy (CEA) were imaged with swept-source OCT angiography (SS-OCTA) imaging protocol to determine if there were changes in choroidal blood flow after surgery., Design: Prospective observational study., Participants: Patients with clinically significant CAS undergoing unilateral CEA., Methods: All participants underwent SS-OCTA imaging using a 6 × 6-mm scan pattern on both eyes before CEA and within 1 week after CEA. Previously validated automated algorithms were used to measure the mean choroidal thickness (MCT) and choroidal vascular index (CVI) within 2.5-mm and 5-mm circles centered on the fovea. Multivariable regression analysis was conducted to evaluate the impact of various baseline factors including age, mean arterial blood pressure, and degree of stenosis, on both baseline of MCT and CVI, and the changes in MCT and CVI., Main Outcome Measures: Changes in MCT and CVI., Results: One hundred sixteen eyes from 60 patients with a mean age of 71.57 ± 7.37 years were involved in the study. At baseline, MCT in both the 2.5-mm and 5-mm circles was significantly thinner on the surgical side compared with the nonsurgical side (P = 0.03), while no significant differences were seen in the CVI at baseline between the 2 sides (2.5-mm circle: P = 0.24; 5-mm circle: P = 0.09). Within 1 week after CEA, there were significant increases in the MCT on the surgical side, as compared with the nonsurgical side, in both the 2.5-mm (P < 0.001) and the 5-mm (P < 0.001) circles. No significant change in mean CVI was noted before and after CEA on the surgical side versus the nonsurgical side (2.5-mm circle: P = 0.30; 5-mm circle: P = 0.97). Multivariable regression analysis revealed that baseline MCT before CEA significantly decreased with age on both the surgical (P < 0.001) and nonsurgical sides (P = 0.003) while the changes in MCT and CVI after CEA were not associated with age, mean arterial blood pressure, or degree of stenosis., Conclusion: A rapid and significant increase in MCT was observed on the ipsilateral side of CEA, suggesting an improvement in choroidal perfusion within 1 week after surgery., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

This review covers advancements in geographic atrophy (GA) research. It discusses genetic contributions to AMD, explores treatment strategies, including complement inhibition, and highlights recent FDA approvals, safety concerns and promising future directions., (© 2023 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To determine if macular thickness maps (MTMs) are sufficient to guide management of eyes with exudative age-related macular degeneration (eAMD), we compared the ability to detect change using MTMs with the ability to detect change using the entire optical coherence tomography (OCT) scan in patients undergoing therapy., Design: Retrospective, comparative diagnostic analysis., Methods: Patients with eAMD were imaged using macula-centered 6 × 6-mm OCT scans (CIRRUS HD-OCT 5000; Zeiss). In each case, graders were asked to determine if there were changes that warranted a full clinical assessment after viewing 2 consecutive scans using one of 3 different imaging strategies: MTMs alone, individual foveal-centered B scans alone, or 5 macular B scans including the foveal-centered B scan. Graders were told the 2 scans were taken 2 weeks apart. The consensus ground truth was reached by the graders using a CIRRUS review station to evaluate all the information contained within the OCT scans., Results: A total of 53 eyes were included in this study with 1385 imaging sessions. The Fleiss kappa was highest when graders were given MTMs alone compared with the ground truth. When the averages of all 5 graders were compared with the ground truth, the MTMs alone showed the highest level of agreement (90.05%, SD 0.78%) followed by the central B scans (87.87%, SD 1.59%) and the 5-B scan method (86.512%, SD 0.64%)., Conclusion: MTMs alone provide sufficient information to easily identify recurrent exudation in patients with eAMD, and these maps may be all that is needed for remote monitoring., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: The impact of carotid endarterectomy (CEA) on choriocapillaris (CC) perfusion was investigated using swept-source optical coherence tomography angiography (SS-OCTA) imaging before and after surgery in patients with clinically significant carotid artery stenosis (CAS)., Methods: In this prospective observational study, patients with clinically significant CAS undergoing unilateral CEA had SS-OCTA imaging performed in both eyes before and within 1 week after surgery. The percent CC flow deficits (CC FD%) and CC thickness were assessed using previously validated algorithms. Multivariable regression analysis was conducted to evaluate the impact of variables on the change in CC measurements., Results: A total of 112 eyes from 56 patients with an average age of 72.6 ± 6.9 years were enrolled. At baseline, significantly higher CC FD% and thinner CC thickness were observed on the surgical side (eyes ipsilateral to the side of CEA) versus the nonsurgical side (eyes contralateral to the side of CEA) (P = 0.001 and P = 0.03, respectively). Following CEA, a significant reduction in CC FD% and a significant increase in CC thickness were detected on the surgical as compared with the nonsurgical side (P = 0.008 and P = 0.01, respectively). Smoking status positively affected CC FD% change (coefficient of variation [CV] = 0.84, P = 0.01) on the surgical side and negatively affected CC thickness change on both the surgical side (CV = -0.382, P = 0.009) and the nonsurgical side (CV = -0.321, P = 0.04). The degree of stenosis demonstrated a positive influence on CC FD% change (CV = 0.040, P = 0.02) on the surgical side., Conclusions: Unilateral CEA on the side of clinically significant CAS increases carotid blood flow, which further results in improved CC perfusion.

Purpose: Postintravitreal injection (IVI) endophthalmitis is a rare but devastating complication. Herein, we report the incidence ,and clinical and microbiological characteristics, as well as the visual outcome, in IVIs endophthalmitis in two medical centers., Methods: All patients undergoing intravitreal injections between 1/2018 and 12/2019 in two large medical centers were analyzed for post-IVI endophthalmitis., Results: Of the total of 51,356 IVIs performed, 23 cases of post-IVI endophthalmitis were diagnosed, yielding an overall incidence of 0.045%. The median interval from IVI to symptoms onset was 2 days (IQR: 1-5). Cultures were positive in 56% of the cases (100% Gram-positive bacteria and 76% coagulase-negative staphylococcus ). Parameters associated with higher culture-positive rates included samples taken during vitrectomy, WBC on vitreous smear, the number of IVIs in the 12 months prior to presentation, and the time interval from last IVI to diagnostic sampling. At 6- and 12-month follow-up, the median change in VA (logMAR) was -1.10 (IQR: (-1.32)-(-0.40)) and -1.02 (IQR: (-1.10)-(-0.30)), respectively. Younger age and better BCVA at presentation were associated with better VA outcome, while positive culture result and systemic steroids treatment were each associated with the worse visual outcome. We found no difference in visual outcomes between PPV and TAI as a primary procedure., Conclusion: Post-IVI endophthalmitis is a rare complication, and most patients do not regain their initial VA. Certain parameters (clinical, microbiological, and therapeutic) may help anticipate the outcome and guide decision making regarding diagnosis and treatment., Competing Interests: The authors declare that there are no conflicts of interest., (Copyright © 2023 Bar Davidov et al.)

Purpose: To compare the anatomical and functional outcomes of fovea-on, fovea-off, and fovea-split rhegmatogenous retinal detachment (RRD)., Methods: Retrospective case series of consecutive patients diagnosed with RRD and treated with only pars plana vitrectomy (PPV). Preoperative and postoperative optical coherence tomography (OCT) and functional outcomes were obtained prior to and 6 months after surgery. RRD extending to the edge of the fovea on OCT was termed fovea-split RRD., Results: A total of 152 eyes were included, out of which 89 eyes presented with a fovea-off, 36 with a fovea-on, and 27 with a fovea-split RRD. The mean visual acuity (VA) preoperatively was 1.32 ± 0.58 logMAR (20/400 equivalent on Snellen chart), 0.19 ± 0.20 (20/30), and 0.71 ± 0.56 (20/100) for the fovea-off, fovea-on, and fovea-split groups, respectively (p < 0.001). The mean VA at 6 months of follow-up significantly improved for the fovea-split and fovea-off groups to 0.54 ± 0.79 (20/70) (p < 0.001) and 0.45 ± 0.29 (20/50) (p = 0.01), respectively, and remained stable for the fovea-on group 0.24 ± 0.20 (20/30) (p = 0.25). Differences in alterations of the outer retinal layers (p < 0.001) and in the in-segment/outer-segment ratio (p < 0.001) were found between the groups., Conclusion: Eyes with fovea-split RRD had both a preoperative and a final postoperative VA between those of fovea-on and fovea-off eyes and different anatomical changes on OCT. This new entity warrants different patient expectations for postoperative outcomes., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Choroidal changes before and after anti-VEGF therapy were investigated in eyes with exudative AMD to determine if there was a difference between eyes with macular neovascularization (MNV) that arises from the choroid (type 1 or 2) versus the retinal circulation (type 3)., Methods: Patients with treatment-naïve AMD were imaged with swept-source optical coherence tomography angiography using a 12 × 12-mm scan pattern. The mean choroidal thickness and choroidal vascularity index (CVI) were measured within 5-mm and 11-mm fovea-centered circles before, at the onset of, and after anti-VEGF therapy., Results: Forty-one eyes of 37 patients were included; 24 eyes with type 1 MNV, 4 eyes with type 2 MNV, and 13 eyes with type 3 MNV. Within the 5-mm and 11-mm circles, the mean choroidal thickness and CVI measurements increased from pretreatment to the onset of exudation (P ≤ 0.03). The mean choroidal thickness and CVI measurements decreased from the onset of exudation to after treatment (P < 0.001). No significant changes in mean choroidal thickness or CVI were observed when comparing measurements before or after treatment (P ≥ 0.38). No significant differences in mean choroidal thickness or CVI measurements were observed between eyes with type 1 or 2 MNV and type 3 MNV., Conclusions: In treatment-naïve AMD eyes with MNV, the choroidal thickness and vascularity increased at the onset of exudation and then decreased after anti-VEGF therapy. This finding suggests that these choroidal changes develop in response to the proangiogenic milieu before treatment and in response to treatment, regardless of the site of origin for the MNV.

Purpose: The appearance and growth of persistent choroidal hypertransmission defects (hyperTDs) detected on en face swept-source optical coherence tomography (SS-OCT) images from eyes with intermediate age-related macular degeneration (iAMD) were studied to determine if they could serve as novel clinical trial endpoints., Design: Post hoc subgroup analysis of a prospective study., Methods: Subjects with iAMD underwent 6 × 6 mm SS-OCT angiography imaging at their baseline and follow-up visits. The drusen volumes were obtained using a validated SS-OCT algorithm. Two graders independently evaluated all en face structural images for the presence of persistent hyperTDs. The number and area of all hyperTDs along with drusen volume were obtained from all SS-OCT angiography scans. Eyes were censored from further follow-up once exudative AMD developed., Results: A total of 171 eyes from 121 patients with iAMD were included. Sixty-eight eyes developed at least 1 hyperTD. Within 1 year after developing a hyperTD, 25% of eyes developed new hyperTDs for an average of 0.44 additional hyperTDs. Over 2 years, as hyperTDs appeared, enlarged, and merged, the average area growth rate was 0.220 mm/yr using the square-root transformation strategy. A clinical trial design using the onset and enlargement of these hyperTDs for the study of disease progression in eyes with iAMD is proposed., Conclusions: The appearance and growth of persistent choroidal hyperTDs in eyes with iAMD can be easily detected and measured using en face OCT imaging and can serve as novel clinical trial endpoints for the study of therapies that may slow disease progression from iAMD to late AMD., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: The symmetry of major macular fundus features in both eyes of the same patient with age-related macular degeneration (AMD) was investigated using swept-source(SS)-OCT., Design: Retrospective review of a prospective study., Participants: Patients with AMD., Methods: Grading was performed on the first SS-OCT images obtained on the patients. Two graders diagnosed the presence of drusen, geographic atrophy (GA), and exudative AMD (eAMD) in each eye. Medical records were reviewed to assess prior exudation. To assess symmetry, 1 eye of each patient was randomly selected as the index eye and compared with the fellow eye. The kappa statistic (κ) was used to assess the symmetry of diagnosis. The intraclass correlation coefficient (ICC) was used to assess the symmetry of drusen area and volume., Main Outcome Measures: Interocular symmetry of the AMD stages: drusen, GA, and eAMD., Results: A total of 1310 patients with AMD were included. The average age was 78 years (range, 50-102; 60% women). Of the 1310 subjects, 54% (701) presented with symmetric disease: 20% with bilateral drusen, 11% with bilateral GA, and 22% with bilateral eAMD. Only 0.5% of the subjects had both GA and eAMD in both eyes. Of the randomly selected index eyes, 825 (47%) were right eyes. Overall, limited interocular agreement was observed between the index and fellow eyes (54%; κ = 0.29). Kappa coefficients were poor (< 0.4) for index eyes diagnosed with drusen (κ = 0.27), eAMD (κ = 0.17), and mixed disease (κ = 0.03). There was moderate agreement between the index and fellow eyes for GA (κ = 0.50). Of the 265 patients with bilateral drusen, the symmetry of drusen area measurements had moderate ICC values of 0.70, 0.71, and 0.70 in the 3- and 5-mm diameter foveal-centered circles and in the total scan area, respectively. The ICC values for the drusen volumes were 0.65, 0.66, and 0.64, respectively., Conclusions: Interocular symmetry was poor for eyes with drusen, eAMD, and mixed disease, but moderate for GA. Although the diagnosis of drusen was not very symmetric between eyes, when present in both eyes, the drusen area and volume measurements were moderately symmetric., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To investigate the association of nascent geographic atrophy (GA) preceding the development of exudative type 3 macular neovascularization (MNV) in patients with age-related macular degeneration (AMD)., Design: Retrospective longitudinal study., Participants: Patients with AMD diagnosed with treatment-naive exudative type 3 MNV in 1 or both eyes were evaluated. Inclusion criteria included serial tracked structural OCT examinations for ≥ 2 years before the detection of exudative type 3 MNV., Methods: Clinical characteristics and retinal imaging, including structural OCT at baseline and at each follow-up examination, were analyzed. Eyes showing the presence of nascent GA during the follow-up were selected for analysis of prevalence, and clinical characteristics at the site of subsequent type 3 MNV development., Main Outcome Measures: Description of the prevalence and clinical characteristics of nascent GA at the site of subsequent type 3 MNV development., Results: Overall, 97 eyes affected by type 3 MNV meeting inclusion criteria were analyzed. Of 97 eyes (71 patients), 22 eyes of 21 patients (mean age 82 ± 9 years) showed nascent GA preceding exudative type 3 MNV. The observed prevalence of nascent GA preceding exudative type 3 MNV was 22.7% (95% confidence interval, 14.4%-31.0%). Exudative type 3 MNV developed a mean of 9 ± 6 months after detection of nascent GA. The presence of reticular pseudodrusen in the study eye did not significantly influence the timing of exudative type 3 MNV development after the observation of nascent GA (P > 0.1 in all analyses). Reduced best-corrected visual acuity was recorded at the exudative type 3 stage in comparison with the nascent GA stage (P = 0.003)., Conclusions: As nascent GA may precede the development of exudative type 3 MNV, the detection of nascent GA in eyes with AMD may warrant closer surveillance to identify early exudative type 3 MNV warranting treatment., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: Geographic atrophy (GA) is a late-stage form of age-related macular degeneration (AMD) characterized by the expansion of atrophic lesions in the outer retina. There are currently no approved pharmacological treatments to prevent or slow the progression of GA. This review describes the progression and assessment of GA, predictive imaging features, and complement-targeting investigational drugs for GA., Methods: A literature search on GA was conducted., Results: Expansion of atrophic lesions in patients with GA is associated with a decline in several measures of visual function. GA lesion size has been moderately associated with measures obtained through microperimetry, whereas GA lesion size in the 1-mm diameter area centered on the fovea has been associated with visual acuity. Optical coherence tomography (OCT) can provide 3-dimensional quantitative assessment of atrophy and is useful for identifying early atrophy in GA. Features that have been found to predict the development of GA include certain drusen characteristics and pigmentary abnormalities. Specific OCT features, including hyper-reflective foci and OCT-reflective drusen substructures, have been associated with AMD disease progression. Lesion characteristics, including focality, regularity of shape, location, and perilesional fundus autofluorescence patterns, have been identified as predictors of faster GA lesion growth. Certain investigational complement-targeting drugs have shown efficacy in slowing the progression of GA., Conclusion: GA is a progressive disease associated with irreversible vision loss. Therefore, the lack of treatment options presents a significant unmet need. OCT and drugs under investigation for GA are promising future tools for disease management., (© 2022. The Author(s).)

Introduction: The objective of this study was to evaluate the impact of unplanned treatment gap, secondary to COVID-19 pandemic lockdowns, on visual acuity in previously treated diabetic macular edema (DME) patients., Methods: A multicenter, retrospective study of DME patients, previously treated with anti-VEGF injections, who were followed up during COVID-19 pandemic (2020) compared to pre-CO-VID-19 period (2019)., Results: A total of 634 DME patients with a mean age of 68.4 years met the inclusion criteria, 385 were assessed in 2019 (pre-COVID-19) and 239 patients assessed in 2020 (COVID-19). Baseline best corrected visual acuity (BCVA) among patients in 2019 and 2020 was 0.52 ± 0.44, 0.45 ± 0.43 (logarithm of the minimal angle of resolution, respectively). There was no significant difference between the years 2020 and 2019 in baseline BCVA (p = 0.07). Mean number of anti-VEGF injections was significantly lower (5 vs. 6, p < 0.01), with a major lower ratio of injections per patient in the COVID-19 first lockdown period (March-June 2020) in the COVID-19 group. Baseline BCVA (p < 0.01) was the only significant predictor of final BCVA. Number of injections, age, gender, and the year were not found as predictors of final BCVA., Conclusions: In a large cohort of DME patients, an unplanned delay in treatment with anti-VEGF injections for 2-3 months, due to COVID-19 pandemic lockdown, had no significance impact on visual acuity. For most patients, returning to routine treatment regimen was sufficient for maintaining BCVA., (© 2022 S. Karger AG, Basel.)

Purpose: To evaluate the impact of postponed care attributed to coronavirus disease (COVID-19) pandemic lockdowns on visual acuity and the number of anti-VEGF injections in patients with retinal vein occlusion (RVO)., Methods: A multicenter, retrospective study of consecutive RVO patients previously treated with anti-VEGF injections, which compared data from pre- (2019) and during (2020) COVID-19 lockdown period., Results: A total of 814 RVO patients with a mean age of 72.8 years met the inclusion criteria. Of them, 439 patients were assessed in 2019 and 375 in 2020. There was no significant difference between the COVID-19 and pre-COVID-19 period in terms of baseline and final BCVA (p = 0.7 and 0.9 respectively), but there was a significantly reduced mean number of anti-VEGF injections during the COVID-19 period (5.0 and. 5.9 respectively, p < 0.01), with a constant lower ratio of injections per patient. A noticeable decline was found during March-May (p < 0.01) in 2020. Baseline BCVA (0.69, p < 0.01) and the number of injections (- 0.01, p = 0.01) were predictors of final BCVA., Conclusions: In a large cohort of RVO patients, during 2020 lockdowns imposed due to the COVID-19 pandemic, a significant reduction in the annual number of anti-VEGF injections was noted. The postponed care did not result in a significant impact on the final BCVA. Baseline BCVA and the number of annual injections serve as predictors for final BCVA in RVO patients., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Choriocapillaris (CC) flow deficits (FDs) were measured in the areas exposed by tears of the retinal pigment epithelium (RPE) before and after their onset to determine their change over time., Methods: Patients enrolled in a prospective, swept-source optical coherence tomography angiography (SS-OCTA) study were retrospectively reviewed for RPE tears, and scans were evaluated before and after RPE tear formation. Choriocapillaris flow deficits were measured within the bed of the tear and within a symmetric control region., Results: Three patients with RPE tears were imaged before tear formation and for at least 16 months afterward. When the baseline and first posttear visit were compared, CC FDs decreased by 1.0% in the tear region and 1.7% in the control region ( P = 0.84). When the 16-month follow-up visits were compared with the first post-RPE tear visits, CC FDs decreased by 1.9% in tear regions and increased by 1.3% in control regions ( P = 0.37)., Conclusion: No significant changes in CC FDs were observed before and after RPE tear formation and for 16 months afterward, suggesting that CC FDs can be reliably detected in the presence of an intact RPE and the absence of the RPE did not affect CC perfusion for at least 16 months.

Purpose: Swept-source optical coherence tomography angiography (SS-OCTA) was used to analyze Bruch membrane (BM) and choriocapillaris (CC) abnormalities in undiagnosed family members with Sorsby macular dystrophy (SMD)., Methods: In a family with SMD ( TIMP3 Tyr191Cys), SS-OCTA imaging was performed using the 6 × 6 mm scan patter and previously validated algorithms to detect abnormalities in BM and the CC, as well as the presence of reticular pseudodrusen and macular neovascularization. Genetic analyses were performed for TIMP3 mutations., Results: Of eight family members, two were previously diagnosed with SMD and six were asymptomatic. SS-OCTA imaging of the 33-year-old proband revealed type 1 macular neovascularization in the left eye and bilateral reticular pseudodrusen, thickening of BM, CC thinning, and increases in CC flow deficits. A TIMP3 mutation was confirmed. His niece, despite having no clinical evidence of SMD, showed BM thickening and CC thinning on SS-OCTA. A TIMP3 mutation was confirmed. The proband's younger nephew and niece also carried the TIMP3 mutation without clinical evidence of SMD. Two additional members had normal examinations, unremarkable SS-OCTA findings, and no TIMP3 mutation., Conclusion: Swept-source optical coherence tomography angiography imaging can detect BM and CC abnormalities in vivo in subjects unaware of their TIMP3 status in a family with SMD.

Purpose: Evaluating the impact of delayed care, secondary to coronavirus disease 2019 (COVID-19) pandemic lockdowns, on visual acuity in previously treated neovascular age-related macular degeneration (nAMD) patients., Methods: This was a multicenter, retrospective, study of patients with nAMD previously treated with anti-VEGF injections who were followed up during 2019 (pre-COVID-19) and compared with patients with nAMD during 2020 (COVID-19)., Results: A total of 1,192 patients with nAMD with a mean age of 81.5 years met the inclusion criteria. Of these, 850 patients were assessed in 2019 (pre-COVID-19) and 630 patients were assessed in 2020 (COVID-19). Three hundred eight patients were assessed through both 2019 and 2020 and thus were included in both cohorts. There was no significant difference between 2020 and 2019 in baseline and change in best-corrected visual acuity (BCVA; P = 0.342 and P = 0.911, respectively). The mean number of anti-VEGF injections was significantly lower (5.55 vs. 6.13, P < 0.01), with constant lower ratio of injections per patient in the COVID-19 period. Baseline BCVA (0.859, P < 0.01), number of injections (-0.006, P = 0.01), and age (0.003, P < 0.01) were predictors of final BCVA., Conclusion: In patients with nAMD, delayed care secondary to COVID-19 pandemic lockdowns has no statistically significant impact on BCVA. Best-corrected visual acuity, older age, and lower number of yearly anti-VEGF injections are predictors for decrease BCVA.

Visual rehabilitation after a keratoplasty is often compromised due to high amounts of residual astigmatism. Femtosecond laser-assisted astigmatic keratotomy (FLAK) is gaining popularity in decreasing this astigmatism. Though one set of two incisions suffices in most cases, sometimes the treatment proves insufficient and additional treatment is required. This case series evaluates the outcomes in patients who underwent two consecutive FLAK sets to correct post-keratoplasty residual astigmatism. All repeated FLAK cases performed on post-keratoplasty eyes were reviewed in a high-volume clinic as a non-comparative retrospective case series. The data extracted include the procedure parameters, time between procedures, refraction including sphere, astigmatism, spherical equivalent (SE), and uncorrected and best-spectacle-corrected distance visual acuity (UDVA, BSDVA, respectively). Eleven eyes of eleven patients aged 25.5 ± 10 treated with more than one FLAK procedure were identified. The average follow-up time was 6 months after the first FLAK and 24 ± 10 months after the second. The second FLAK was performed six months after the first. The preoperative mean astigmatism was -9.59 ± 2.36 D. At the final follow-up, the mean astigmatism decreased to -5.38 ± 1.79 D ( p = 0.001). Repeated FLAK procedures achieved a significant and stable reduction of astigmatism in post-keratoplasty eyes. This procedure shows safety and effectivity in these complex eyes and may prevent the need for a further keratoplasty.

Purpose: To compare the choroidal thickness before and after pars plana vitrectomy for rhegmatogenous retinal detachment repair., Methods: A retrospective case series of rhegmatogenous retinal detachment patients presenting between January 2015 and September 2020. Subfoveal choroidal thickness (SFCT) and anatomical success were measured in operated eyes and fellow eyes at presentation, as well as 3 months and 6 months after pars plana vitrectomy for rhegmatogenous retinal detachment repair., Results: A total of 93 patients (males 59%) with a mean age of 61.8 ± 15.2 years were included. Eighty-one patients were anatomically successful (Group 1) and 12 redetached (Group 2). The mean SFCT of the operated eye at presentation was 258.3 ± 82.0 µm in comparison with 257.5 ± 83.7 µm in the fellow eye (P = 0.96). Group 2 presented with thicker SFCT than Group 1 at baseline (309.2 ± 56.2 vs. 250.7 ± 82.8 µm; P = 0.01). Both groups demonstrated thinning trend throughout follow-up. At 6-month follow-up, the mean SFCT was 225.6 ± 75.5 µm (P = 0.05). Fellow-eye SFCT was stable throughout follow-up (257 ± 83.7 at baseline vs. 255 ± 80.2 µm at 6 months)., Conclusion: Eyes with rhegmatogenous retinal detachment demonstrated thinning in the SFCT after vitrectomy surgery. Eyes with recurrent retinal detachment presented with a thicker choroid at baseline. Thicker SFCT at presentation may play a role in retinal redetachment.

Purpose: En face OCT imaging was investigated as a method for the detection and monitoring of calcified drusen in eyes with nonexudative age-related macular degeneration (AMD)., Design: Retrospective case series of a prospective study., Participants: Patients with nonexudative AMD., Methods: A retrospective review was performed of same-day color fundus (CF), fundus autofluorescence (FAF), near-infrared (NIR), and en face swept-source (SS) OCT images to identify eyes with nonexudative AMD and calcified drusen. The appearance and progression of these lesions were compared using the different imaging methods., Main Outcome Measures: Comparison between the presence of calcified drusen observed on CF images with the detection of these lesions on FAF, NIR, and en face SS OCT images., Results: Two hundred twenty eyes from 139 patients with nonexudative AMD were studied, with 42.7% of eyes containing calcified drusen either at baseline or during follow-up visits. On the en face SS OCT images, calcified drusen appeared as dark focal lesions referred to as choroidal hypotransmission defects (hypoTDs) that were detected in the choroid using a sub-retinal pigment epithelium (RPE) slab. The corresponding B-scans showed drusen with heterogenous internal reflectivity, hyporeflective cores, and hyperreflective caps. In most calcified drusen, choroidal hypertransmission defects (hyperTDs) were observed to develop over time around the periphery of the hypoTDs, giving them the appearance of a donut lesion on the en face SS OCT images. These donut lesions were associated with significant attenuation of the overlying retina, and the corresponding FAF images showed hypoautofluorescence at the location of these lesions. The donut lesions fulfilled the requirement for a persistent hyperTD, which is synonymous with complete RPE and outer retinal atrophy (cRORA). Six eyes displayed regression of the calcified drusen without cRORA developing. B-scans at the location of these regressed calcified drusen showed deposits along the RPE, with outer retinal thinning in the regions where the calcified lesions previously existed., Conclusions: En face OCT imaging is a useful method for the detection and monitoring of calcified drusen and can be used to document the evolution of these drusen as they form donut lesions or foci of cRORA.

Purpose: The impact of cataract surgery on low luminance visual acuity deficit (LLVAD) measurements was investigated by measuring the LLVAD before and after cataract surgery., Design: Prospective, longitudinal study., Participants: Patients undergoing cataract surgery., Methods: Photopic luminance (PL)-best-corrected visual acuity (BCVA) and low luminance (LL)-BCVA were obtained using the ETDRS chart. Low luminance visual acuity deficit scores were calculated by subtracting the LL-BCVA letter score from the PL-BCVA letter score. To demonstrate the reproducibility of these visual acuity measurements, we used data from drusen-only eyes previously published in the Complement Inhibition with Eculizumab for the Treatment of Nonexudative Age-Related Macular Degeneration (COMPLETE) study. The PL-BCVA, LL-BCVA, and LLVAD measurements obtained at an interval of 3 months in this cohort were used for comparison. In the current study, the impact of cataract surgery on LLVAD measurements was analyzed by comparing the PL-BCVA, LL-BCVA, and LLVAD measurements before and after cataract surgery., Main Outcome Measures: The reproducibility of the visual acuity measurements and the changes in LLVAD measurement after cataract surgery., Results: In the COMPLETE study, no clinically significant differences were found in the PL-BCVA, LL-BCVA, or LLVAD measurements between baseline and the 3-month follow-up visits with a change of -1.1 letters, -1.3 letters, and 0.1 letters, respectively ( P  = 0.02, P  = 0.11, and P  = 0.88, respectively). In the current study, significant increases were found in the PL-BCVA and LL-BCVA measurements, with a change of 7.3 letters and 10.2 letters after cataract surgery ( P < 0.001 for both), and a statistically significant decrease in LLVAD measurements was found, with a change of -3.0 letters after cataract surgery ( P  = 0.002)., Conclusions: Because of the variable effect of cataracts on LL-BCVA measurements and the significant change in LLVAD measurements after cataract surgery, investigators should be aware that cataract surgery during a trial will have an unpredictable impact on LLVAD measurements, and pseudophakic and phakic patients should be analyzed separately., (© 2022 Published by Elsevier Inc. on behalf of the American Academy of Ophthalmology.)

Purpose: A training exercise was performed to study the ability of graders to reliably identify precursor lesions to geographic atrophy (GA), known as persistent choroidal hypertransmission defects (hyperTDs), using en face OCT images from eyes with nonexudative age-related macular degeneration (AMD)., Design: Intergrader agreement study., Participants: Eleven graders participated in this exercise., Methods: Formal training on how to identify persistent hyperTDs on en face OCT images was provided to the graders. A persistent hyperTD was defined as a bright lesion having a greatest linear dimension (GLD) of at least 250 μm. Training consisted of a tutorial session followed by the grading of 3 pretest exercises, each consisting of 3 cases. After all graders scored 100% on the pretest exercises, they performed a final exercise consisting of 30 en face OCT images from 29 eyes with nonexudative AMD containing 107 hyperTDs that each grader needed to evaluate. The cases contained a variety of AMD-related atrophic lesions., Main Outcome Measures: The sensitivity, positive predictive value (PPV), and modified accuracy were assessed for each grader., Results: A total of 1177 hyperTDs from 30 en face OCT images were reviewed by the graders. The mean sensitivity, PPV, and modified accuracy for all the graders were calculated to be 99.0%, 99.2%, and 98.2%, respectively. There was a 97% agreement observed between all the graders (first-order agreement coefficient [AC 1 ] = 0.97). Internal graders from the Bascom Palmer Eye Institute had a slightly higher agreement compared with the external graders (AC 1  = 0.98 vs. AC 1  = 0.96). The hyperTDs most often incorrectly identified included the following features: (1) hyperTDs containing hypotransmission defect cores, (2) single hyperTDs that were incorrectly graded as 2 separate lesions, and (3) hyperTDs with borderline GLDs that were close to 250 μm., Conclusions: The accurate detection of persistent hyperTDs on en face OCT images by graders demonstrates the feasibility of using this OCT biomarker to identify disease progression in eyes with nonexudative AMD, especially when used as a clinical trial end point in studies designed to test new therapies that may slow disease progression from intermediate AMD to GA., (Copyright © 2022 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To provide an update on the hemodynamic model of age-related macular degeneration (AMD)., Design: Evidence-based perspective., Methods: Review of the literature and experience of the authors., Results: Choroidal hemodynamics are not the primary cause of AMD as proposed by Ephraim Friedman in 1997. However, evidence is accumulating to suggest that choroidal perfusion is an important environmental influence that contributes to our understanding of disease progression in this complex genetic disorder. Although early and intermediate AMD seem to be influenced to a large extent by the underlying genetics, the asymmetry of disease progression to the later stages of AMD cannot be explained by genetics alone. The progression of disease and the asymmetry of this progression seem to correlate with abnormalities in choroidal perfusion that can be documented by optical coherence tomography. These perfusion abnormalities in the setting of a thickened Bruch's membrane are thought to exacerbate the impaired nutritional exchange between the retinal pigment epithelium and the choriocapillaris. We propose that the genetic susceptibility to develop AMD combined with age-related changes in macular choroidal hemodynamics, such as increasing choriocapillaris perfusion deficits and decreasing choroidal vascular densities, play an important role in disease progression and may help to explain the asymmetry between eyes, particularly in the later stages of AMD., Conclusions: This updated hemodynamic model of AMD focuses on disease progression and highlights the importance of age-related changes in the choroidal circulation as a major environmental influence on disease severity in eyes that are genetically susceptible to develop AMD., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: Multimodal imaging was used to identify and characterize the cause of hyperpigmentation seen on color fundus images (CFIs) of eyes with intermediate age-related macular degeneration (iAMD)., Design: Retrospective review of a prospective study., Participants: Patients with iAMD., Methods: Color fundus images with macular hyperpigmentation were compared with same-day images obtained using fundus autofluorescence (FAF), near infrared reflectance (NIR), and swept-source (SS) OCT imaging. Two SS OCT en face slabs were generated: a retinal slab to identify hyperreflective foci within the retina and a slab from beneath the retinal pigment epithelium (RPE; the sub-RPE slab) that was used to detect regions that cause decreased light transmission into the choroid, also known as hypotransmission defects. All images were registered to allow for qualitative comparisons by 2 independent graders., Main Outcome Measures: Comparison between foci of macular hyperpigmentation seen on CFIs with the detection of these regions on FAF, NIR, and SS OCT en face images., Results: Compared with CFIs, FAF imaging seemed to be the least sensitive method for the detection of hyperpigmentation, whereas NIR and SS OCT imaging reliably detected these hyperpigmented areas. Although NIR imaging detected most of the hyperpigmentation seen in CFIs, SS OCT imaging detected all the areas of hyperpigmentation and anatomically localized these areas by using both en face and B-scan images. En face OCT slabs of the retina and sub-RPE region were registered to the CFIs, and areas of hyperpigmentation were shown to correspond to hyperreflective foci in the retina and regions of thickened RPE seen on OCT B-scans. Although both hyperpigmentation and early atrophic lesions appeared bright on NIR imaging, en face SS OCT imaging was able to distinguish these lesions because hyperpigmentary changes appeared dark and early atrophic lesions appeared bright on the sub-RPE slab., Conclusions: En face OCT imaging in conjunction with OCT B-scans were able to identify and localize the hyperpigmentation seen in CFIs reliably. This hyperpigmentation was not only associated with intraretinal hyperreflective foci, but also corresponded to areas with a thickened RPE., (© 2022 by the American Academy of Ophthalmology.)

Purpose: The local growth rates of geographic atrophy (GA) adjacent to non-exudative type 1 macular neovascularization (MNV) were investigated to determine if MNV influenced GA growth., Methods: Eyes with GA and non-exudative type 1 MNV were followed for at least 1 year. Both GA and the MNV were imaged and measured using swept-source optical coherence tomography angiography (SS-OCTA) scans. Pearson correlations were computed between local growth rates of GA, which were estimated using a biophysical GA growth model, and local distances-to-MNV. Corresponding P values for the null hypothesis of no Pearson correlation were computed using a Monte Carlo approach that adjusts for spatial autocorrelations., Results: Nine eyes were included in this study. There were positive correlations (Pearson's r > 0) between distance-to-MNV and local GA growth in eight (89%) of the eyes; however, in all but one eye (11%), correlations were relatively weak and statistically nonsignificant after Bonferroni correction (corrected P > 0.05)., Conclusions: SS-OCTA imaging combined with GA growth modeling and spatial statistical analysis enabled quantitative assessment of correlations between local GA growth rates and local distances-to-MNV. Our results are not consistent with non-exudative type 1 MNV having a strong inhibitory effect on local GA growth rates.

Purpose: Swept-source optical coherence tomography angiography was used to investigate choroidal changes and their association with pigment epithelial detachments (PEDs) in eyes with polypoidal choroidal vasculopathy (PCV) after treatment with vascular endothelial growth factor (VEGF) inhibitors., Methods: Patients with treatment-naïve PCV were included and underwent anti-VEGF therapy. Mean choroidal thickness (MCT), choroidal vascularity index (CVI), and PED volume measurements were obtained before and after treatment., Results: Thirty-four treatment-naïve PCV eyes from 33 patients were included. The PED volume decreased after treatment (P < 0.05). The MCT decreased from 223.0 ± 79.6 µm at baseline to 210.9 ± 76.2 µm after treatment (P < 0.001). The CVI at baseline was 0.599 ± 0.024, and the CVI after treatment was 0.602 ± 0.023 (P = 0.16). There was a correlation between the decreased PED volumes and the decreased MCT measurements (r = 0.47; P = 0.006). Also, there was a correlation between the decreased PED volumes and the increased CVI measurements (r = -0.63; P < 0.001)., Conclusions: In treatment-naïve eyes with PCV, the decreases in PED volumes were correlated with the decrease in MCT and the increase in CVI measurements. We propose that, at baseline, the PCV lesions serve as high-volume arteriovenous shunts between choroidal arterial and venous circulation, causing transudation into the choroidal stroma. We propose that, after treatment, the blood flow through the vascular shunt is reduced, the excess stromal transudation is resorbed, and the exudation from the neovascular lesion is reduced, resulting in thinning of the choroid, resolution of the PEDs, and an increase in the CVI due to the resorption of excess choroidal transudation.

Purpose: The purpose of this study was to assess the results of a sulcus intraocular lens (Sulcoflex) for pseudophakic refractive errors following phacoemulsification cataract surgery., Methods: This retrospective clinical observational cohort study included consecutive eyes in which a Sulcoflex was implanted. Uncorrected distance visual acuity and corrected distance visual acuity as well as refractive outcomes were assessed. The minimum follow-up time required for inclusion was 3 months., Results: In total, 15 eyes ( n  = 15) were evaluated. The mean follow-up was 14 months (range: 3-18 months). The Sulcoflex aspheric (653L) was implanted in 13 eyes and the Sulcoflex toric (653T) in two eyes. The preoperative mean logMAR (Snellen) uncorrected distance visual acuity and corrected distance visual acuity were 0.88 (20/150) and 0.27 (20/40), respectively. The postoperative mean logMAR (Snellen) corrected distance visual acuity was 0.15 (20/30). The preoperative mean spherical equivalent was -0.22 ± 5.95 D and the postoperative mean spherical equivalent was -1.59 ± 1.45 D. There was a significant and strong correlation ( r  = 0.64, p  < 0.001) between the attempted and the achieved spherical equivalent., Conclusion: The Sulcoflex is a safe and viable option for patients with residual refractive error following cataract surgery.

Limited information is known about the extent of canthaxanthin crystalline retinopathy on the retinal layers. The authors describe a 51-year-old woman who was taking canthaxanthin for tanning purposes for 7 years. Three years after cessation of this agent, she presented with asymmetric crystalline retinopathy affecting both eyes. She was lost to follow-up, and upon returning 4 years later, the crystalline retinopathy persisted but the number of crystals had decreased. Using swept-source optical coherence tomography, the authors showed that the crystalline retinopathy affected all retinal layers. In addition, retinal pigmented epithelial detachments were present suggesting persistent damage caused by the canthaxanthin. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:727-731.]., (Copyright 2020, SLACK Incorporated.)

Purpose: The clinic efficiency and cost savings achieved by eliminating formal visual acuity (VA) and dilated fundus examinations (DFEs) were assessed for established patients receiving optical coherence tomography (OCT)-guided intravitreal injections., Design: Comparative cost analysis., Methods: Two different treatment models were evaluated. The first model included patients undergoing routine VA assessment, DFEs, OCT imaging, and intravitreal injections. The second model eliminated the routine VA assessment and DFE while using OCT imaging through an undilated pupil followed by the intravitreal injection. The 2 models incorporated both bevacizumab and aflibercept. The number of patients per clinic day, the cost per visit, and the daily revenues were compared between the 2 models., Results: Optimized schedules with and without VA assessments and DFEs allowed for 48 and 96 patients to be injected per day, respectively. Excluding drug costs, the cost per encounter for the visits with and without a DFE were $39.33 and $22.63, respectively. Including the drug costs, the costs per encounter for the visits with and without a DFE were $85.55 and $68.85 for bevacizumab and $1787.58 and $17770.88 for aflibercept, respectively. Once the reimbursements for each visit type were included, the clinics that eliminated the VA and DFEs were more cost efficient., Conclusion: Eliminating both VA assessments and DFEs for patients undergoing OCT-guided retreatment with intravitreal injections resulted in decreased exposure times between patients and clinic staff, decreased cost per encounter, and increased patient volumes per clinic day, resulting in improved clinic efficiency and safety while seeing more patients in a clinic day., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To describe novel findings of hyperreflective material in the silicone-retina interphase on spectral domain optical coherence tomography (SD-OCT) imaging in eyes with silicone oil tamponade., Methods: Retrospective observational clinical study of consecutive patients who underwent primary pars plana vitrectomy with silicone oil tamponade for rhegmatogenous retinal detachment. Repeat clinical examination and spectral domain optical coherence tomography macular imaging performed 3 months after surgery were evaluated to identify any macular pathologies, including formation of epiretinal membranes, intraretinal changes, subretinal fluid, and edema before scheduled secondary vitrectomy for silicone oil removal., Results: Eighty-two patients (mean age 54 years, range 22-89) were included. Twelve eyes (14%) showed discrete preretinal hyperreflective organized coarse material in the silicone-retina interphase on spectral domain optical coherence tomography. The material was scattered in the posterior pole, with several foci showing additional hyperreflectivity of the inner retinal layers beneath. These findings did not resemble silicone oil emulsification in size, shape, or reflectivity., Conclusion: This is the first report on hyperreflective material detected by spectral domain optical coherence tomography in the silicone-retina interphase in eyes with silicone oil tamponade. These findings may represent an inflammatory response to silicone oil exposure that may be the initial manifestation of a future proliferative process, warranting a rigorous follow-up protocol for affected patients.

Purpose: To evaluate the effect of pterygium excision on the posterior corneal surface and analyze the factors associated with those changes., Methods: A prospective, interventional study including 33 eyes of 31 patients who underwent pterygium excision at the Tel Aviv Medical Center (Tel Aviv, Israel). Exclusion criteria included corneal dystrophy, pseudopterygium, corneal scarring, or previous ocular surgery in the treated eye. Data were obtained by using the Galilei dual Scheimpflug analyzer. Recorded posterior corneal data included steep keratometry, flat keratometry, mean keratometry, corneal astigmatism, best-fit sphere, and the squared eccentricity index (e). Posterior surgically induced astigmatism (SIA) was calculated to demonstrate the astigmatic effect of surgery. Anterior-segment high resolution optical coherence tomography was used to measure pterygium dimensions (depth and horizontal/vertical size)., Results: The mean age was 53.7 ± 16.7 years. Posterior corneal SIA was 0.9 ± 1.1 D (P < 0.001) and was significantly correlated with age (r = 0.568, P = 0.002), horizontal pterygium size (r = 0.387, P = 0.046), and preoperative posterior astigmatism (r = 0.688, P < 0.001). In a multivariable analysis, only age (coefficient = 0.010, P = 0.038) and preoperative posterior astigmatism (coefficient = 0.648, P = 0.002) remained significant. Pterygium dimensions were not significantly associated with SIA magnitude. Flat keratometry steepened by 0.5 ± 1.1 D (P = 0.019), mean keratometry steepened by 0.3 ±0.6 D (P = 0.035), posterior astigmatism was reduced by 0.4 ± 1.2 D (P = 0.072), and e decreased by 5.1 ± 17.3 (P = 0.021)., Conclusions: Pterygium excision has a significant astigmatic effect on the posterior corneal surface. The astigmatic effect increases with age and with higher preoperative posterior astigmatism. Pterygium depth and size are not associated with the degree of surgical astigmatic effect.

Purpose : To evaluate the visual outcome and patient satisfaction after a unilateral multifocal IOL implantation in the non-dominant eye of emmetropic patients with presbyopia. Methods : An interventional case series of consecutive patients who underwent unilateral phacoemulsification with femto-laser assisted cataract surgery (FLACS) and refractive lens exchange (RLE), followed by an implantation of a trifocal diffractive IOL (FineVision Micro F), was performed in the non-dominant eyes of emmetropic patients with presbyopia. After 6 months of follow-up, the main outcome measures were uncorrected distance visual acuity (UDVA), uncorrected intermediate visual acuity (UIVA), and uncorrected near visual acuity (UNVA). Secondary outcomes included spherical equivalent (SE), refraction, contrast sensitivity, patient questionnaire and presence of visual side effects. Results : A total of 26 eyes of 26 patients, with an average age of 53.8 ± 4.1 years, were included in this study. Preoperative mean UDVA was 0.13 ± 0.04 logMAR (Snellen 20/27), UIVA was 0.46 ± 0.12 logMAR (Snellen 20/58), and UNVA was 0.66 ± 0.17 logMAR (Snellen 20/91), in comparison to postoperative mean UDVA of 0.18 ± 0.32 logMAR (Snellen 20/30) (p = 0.32), UIVA of 0.17 ± 0.21 logMAR (Snellen 20/30) (p < 0.005), and UNVA of 0.02 ± 0.10 logMAR (Snellen 20/21) (p < 0.005). Monocular UNVA of 20/25 or better in the operated eye was achieved in 23 (88%) patients. Twenty-four (96%) patients said they would recommend this procedure to family and friends. There were no intraoperative complications and no IOL exchange was required. Conclusions : A unilateral RLE of the non-dominant eye with FLACS and a trifocal diffractive IOL (FineVision Micro F) implantation in emmetropic, presbyopic patients is provides satisfactory distance, intermediate, and near visual outcomes with no complications reported in this preliminary case series.

Purpose: To analyze the visual and refractive outcome of the bioptics procedure combining multifocal intraocular lens implantation and excimer laser surgery in young patients with high hyperopic eyes not suitable for a single surgical procedure., Methods: This retrospective case series included 10 eyes of five patients (age range 18-30 years) with high hyperopia (spherical equivalent +8.51 ± 0.85 diopters (D)). They had been treated with serial multifocal intraocular lens implantation followed 6 weeks later by laser in situ keratomileusis for residual hyperopia. Uncorrected distance visual acuity, uncorrected near visual acuity, corrected distance visual acuity, corrected near visual acuity, and manifest refraction were evaluated before surgeries, after multifocal intraocular lens implantation, and 3 months post laser in situ keratomileusis., Results: No patients were lost to follow-up (6 months). The mean spherical equivalent decreased to +2.05 ± 1.33 D after multifocal intraocular lens implantation and to -0.10 ± 0.58 D after the laser in situ keratomileusis procedure. Success of the procedures was determined by uncorrected visual acuity. LogMAR uncorrected distance visual acuity improved by a total of more than six lines from 1.05 ± 0.18 LogMAR to 0.46 ± 0.12 LogMAR post multifocal intraocular lens implantation and to 0.15 ± 0.06 LogMAR after both surgeries. The LogMAR uncorrected near visual acuity increased by 0.81 ± 0.82 LogMAR after lens implantation due to loss of accommodation, and all eyes reached a LogMAR of 0 at 1 month postoperatively following laser in situ keratomileusis., Conclusions: A bioptics approach involving multifocal intraocular lens followed 6 weeks later by a laser in situ keratomileusis procedure for the correction of very high hyperopia enabled the resolution of the residual refractive error in young very high hyperopic patients.

Purpose: To characterize cat-scratch disease (CSD) ocular manifestations and visual outcome and evaluate the effect of systemic antibiotics and corticosteroids on final visual acuity (VA)., Methods: Multicentre retrospective cohort study. Medical records of 86 patients with ocular disease (107 eyes) of 3222 patients identified in a national CSD surveillance study were reviewed., Results: Mean age was 35.1 ± 14.2 years. Median follow-up was 20 weeks (range 1-806 weeks). Of 94/107 (88%) eyes with swollen disc, 60 (64%) had neuroretinitis at presentation, 14 (15%) developed neuroretinitis during follow-up, and 20 (21%) were diagnosed with inflammatory disc oedema. Optic nerve head lesion, uveitis, optic neuropathy and retinal vessel occlusion were found in 43 (40%), 38 (36%), 34 (33%) and 8 (7%) eyes, respectively. Good VA (better than 20/40), moderate vision loss (20/40-20/200) and severe vision loss (worse than 20/200) were found in 26/79 (33%), 35/79 (44%) and 18/79 (23%) eyes at baseline and in 63/79 (80%), 11/79 (14%) and 5/79 (6%) eyes at final follow-up, respectively (p < 0.001). Significant VA improvement (defined as improvement of ≥3 Snellen lines at final follow-up compared to baseline) occurred in 12/24 (50%) eyes treated with antibiotics compared with 14/16 (88%) eyes treated with antibiotics and corticosteroids (p = 0.02). Multivariate logistic regression was suggestive of the same association (odds ratio 7.0; 95% CI 1.3-37.7; p = 0.024)., Conclusion: Optic nerve head lesion is a common and unique manifestation of ocular CSD. Most patients improved and had final good VA. Combined antibiotics and corticosteroid treatment was associated with a better visual outcome., (© 2018 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To report a cluster of high incidence of noninfectious inflammatory reaction shortly after bevacizumab injection., Methods: Data were collected from medical records of consecutive patients who underwent bevacizumab injections and presented with acute uveitis within 1-4 days., Results: All 7 patients were injected intravitreally with bevacizumab from different batches and presented with visual deterioration. One patient with anterior uveitis complained of pain. None had hypopyon. Three patients with vitritis underwent a vitreous tap with an intravitreal injection of vancomycin and ceftazidime for possible infectious endophthalmitis. All patients received intensive topical antibiotic drops and steroids, and the intraocular inflammation resolved completely within 1 month. Five patients continued to receive bevacizumab injections without recurrent episodes., Conclusions: Our series demonstrates an incidence of noninfectious inflammatory reaction shortly after bevacizumab injection that is higher than that reported in the literature. The cause is more likely the preparation process in the same compounding pharmacy., (© 2018 S. Karger AG, Basel.)

Background: Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis of the small vessels occurring at a young age and considered as a benign self-limited disease. Due to its low prevalence, there are limited data on the presentation and complications of this disease., Methods: All computerized files of children who were hospitalized at a tertiary pediatric center due to AHEI over a 10 year period were reviewed. Clinical, laboratory and histopathological data were collected., Results: Twenty-six patients were included in our study, accounting for 0.7 cases per 1000 admissions of children aged 2 years or less. Mean age was 12.9 months. More than two thirds of the children had preceding symptoms compatible with a viral infection. Upon admission, all patients presented with typical findings of a rash and edema. Edema was most profound over the lower extremities (73%). Concomitant viral or bacterial infections were found in six children. Skin biopsy was performed in six patients revealing leukocytoclastic vasculitis. Thirteen children (50%) had systemic involvement including joint involvement (n=9), gastrointestinal hemorrhage (n=4), microscopic hematuria (n=1) and compartment syndrome of the limb (n=1). The latter was diagnosed in a patient with familial Mediterranean fever., Conclusions: Our largest data series highlighted what is known regarding clinical and histological findings in children with AHEI. However, contrary to what was previously reported, we found a higher rate of systemic involvement. Although AHEI is a rare entity, pediatricians should be familiar with its presentation, management and our reported complications.