Macular Atrophy Research Articles

Comparative analysis of macular characteristics in mCNV and contralateral eyes.

To illustrate the characteristics of perforating scleral vessels in macular regions between mCNV eyes and contralateral eyes in unilateral mCNV patients. This was a retrospective study that included patients with unilateral naive mCNV. The study aimed to identify and analyze the distribution of perforating scleral vessels (PSVs) in the macular region of mCNV eyes and contralateral eyes. The central macular choroidal thicknesses (mChT) were measured using optical coherence tomography angiography (OCTA). The grades of myopic atrophic maculopathy (MAM) and macular myopic diffuse chorioretinal atrophy (DCA) were evaluated within groups. The number of PSVs and mChT were compared between contralateral and mCNV eyes based on the grade of DCA. The ROC curves were utilized to explore the diagnostic indexes for mCNV. A total of 102 eyes from 51 patients with unilateral mCNV were included. There was no significance in the severity of MAM or the grade of DCA between mCNV eyes and contralateral eyes (p = 0.074, p = 0.054, respectively). The mean number of PSVs in mCNV eyes was fewer than the contralateral eyes [1.00 (1.00-2.00) vs. 2.00 (0.75-3.00), p = 0.030]. The mChT in mCNV eyes was thinner than the contralateral eyes [36.00 (25.00-53.75) μm vs. 46.00 (31.00-75.25) μm, p = 0.001]. The mean grade of DCA in mCNV eyes was higher than that in contralateral eyes [3.00 (3.00-3.00) vs. 3.00 (2.00-3.00), p = 0.004]. When DCA involved the macular region, there were more PSVs in contralateral eyes than in mCNV eyes [1.50 (1.00-2.00) vs. 2.00 (1.00-3.00), p = 0.042]. Similarly, when DCA involved the foveal region, there were more PSVs in contralateral eyes than in mCNV eyes [1.50 (1.00-2.00) vs. 3.00 (2.00-4.00), p = 0.004]. The grade of DCA and mChT were valuable factors for predicting mCNV eyes (AUC = 0.6566, p = 0.021; AUC = 0.6304, p = 0.029; respectively). When the extent of DCA exceeded the foveal region, the count of PSVs was a good diagnostic factor for predicting mCNV (AUC = 0.7430, p = 0.003). The mean amount of PSVs was significantly lower in the mCNV eyes compared to the contralateral eyes. When the extent of DCA exceeded the foveal region, the count of PSVs was a good diagnostic factor for predicting mCNV. Myopic eyes with a higher grade of DCA and a thinner mChT were more likely to develop mCNV.

Clinical characterizations and molecular genetic study of two co-segregating variants in PDZD7 and PDE6C genes leading simultaneously to non-syndromic hearing loss and achromatopsia

BackgroundAutosomal recessive non-syndromic hearing loss (NSHL) and cone dystrophies (CODs) are highly genetically and phenotypically heterogeneous disorders. In this study, we applied the whole exome sequencing (WES) to find the cause of HL and COD in an Iranian consanguineous family with three affected individuals.MethodsThree members from an Iranian consanguineous family who were suffering from NSHL and visual impairment were ascertained in this study. Comprehensive clinical evaluations and genetic analysis followed by bioinformatic and co-segregation studies were performed to diagnose the cause of these phenotypes. Data were collected from 2020 to 2022.ResultsAll cases showed congenital bilateral NSHL, decreased visual acuity, poor color discrimination, photophobia and macular atrophy. Moreover, cornea, iris and anterior vitreous were within normal limit in both eyes, decreased foveal sensitivity, central scotoma and generalized depression of visual field were seen in three cases. WES results showed two variants, a novel null variant (p.Trp548Ter) in the PDE6C gene causing COD type 4 (Achromatopsia) and a previously reported variant (p.Ile84Thr) in the PDZD7 gene causing NSHL. Both variants were found in the cis configuration on chromosome 10 with a genetic distance of about 8.3 cM, leading to their co-inheritance. However, two diseases could appear independently in subsequent generations due to crossover during meiosis.ConclusionsHere, we could successfully determine the etiology of a seemingly complex phenotype in two adjacent genes. We identified a novel variant in the PDE6C gene, related to achromatopsia. Interestingly, this variant could cooperatively cause visual disorders: cone dystrophy and cone-rod dystrophy.

Bruch's Membrane Opening Changes in Eyes With Myopic Macular Degeneration: AIER-SERI Adult High Myopia Study.

The purpose of this study was to assess the choroidal thickness and the Bruch's membrane opening size and their relationship to visual acuity in eyes with myopic macular degeneration (MMD). This was a population-based, cross-sectional study. Patients over the age of 30years with high myopia (spherical equivalent ≤-5 diopters [D]) were recruited. The eyes were grouped according to the International Meta-Analysis for Pathologic Myopia (META-PM) classification based on fundus photographs and diffuse atrophy was subdivided into peripapillary diffuse choroidal atrophy (PDCA) or macular diffuse choroidal atrophy (MDCA). Swept-source optical coherence tomography imaging was performed and then the subfoveal choroidal thickness (SFCT) and Bruch's membrane opening diameter (BMOD) were measured. Of the 470 study participants recruited, 373 patients (691 eyes), with a mean age of 42.8 ± 7.2years, were eligible for the study and included in the analysis. There was no significant difference in SFCT between MDCA and patchy atrophy (M3) groups (P = 1.000), and the BMOD enlarged significantly from no myopic macular lesions to M3 (the P values of multiple comparison tests were all <0.005). Simple linear regression analysis showed that BMOD correlated positively with age (P < 0.001) and axial length (P < 0.001). Multiple linear regression analysis showed that best corrected visual acuity (BCVA) was significantly correlated with age (P = 0.041), axial length (P = 0.001), and BMOD (P = 0.017), but not with SFCT (P = 0.231). The significant variation of BMOD among MMD groups and the correlation between BMOD and BCVA in MMD eyes suggest that BMOD may be an imaging biomarker for monitoring MMD.

Myopic Macular Atrophy in the Two-Continent Population-Based Study.

To examine the prevalence of Bruch's membrane defects (BMDs) and subretinal proliferations (SRPs) in highly myopic eyes with myopic macular atrophy (myopic macular degeneration [MMD] stage 4) and myopic patchy atrophies (MMD stage 3) in three ethnically different cohorts recruited in a population-based manner. The Ural Eye and Medical Study (UEMS) and Beijing Eye Study (BES) included individuals aged 40+years, and the Ural Very Old Study (UVOS) examined individuals aged 85+years. Main outcome measures were the prevalence of BMDs and SRPs. Among 5794 UEMS participants, 19 eyes had MMD stage 4, with 17 (89%) eyes showing a foveal BMD; two eyes could not fully be explored. All 19 eyes showed localized SRPs. Among 21 eyes with MMD stage 3, BMD and SRP prevalence was 9 of 21 (44%) and 7 of 21 (33%), respectively. Among 930 UVOS participants, 17 eyes had MMD stage 4, with 16 (94%) eyes showing foveal BMDs and SRPs; one eye could not be assessed. Among 18 eyes with MMD stage 3, BMD and SRP prevalence was 3 of 18 (17%) and 2 of 18 (11%), respectively. Among 3468 BES participants, 8 eyes had MMD stage 4, with all eyes showing foveal BMDs and SRPs. Among 14 eyes with MMD stage 3, BMD and SRP prevalence was 10 of 14 (71%) and 7 of 21 (33%), respectively. All eyes with assessable myopic macular atrophy showed foveal BMDs associated with SRPs, while patchy atrophies could be differentiated into those with BMDs and SRPs and those without BMDs and without SRPs. Independent of the MMD stage, the prevalences of BMDs and SRPs were highly significantly associated with each other.

RDH5 and RLBP1-Associated Inherited Retinal Diseases: Refining the Spectrum of Stationary and Progressive Phenotypes

To investigate the clinical, functional, and imaging characteristics in patients affected by inherited retinal diseases associated with RDH5 and RLBP1 gene variants, and to report novel genotype-phenotype correlations. Retrospective single-center cohort study. Twenty-two patients with molecularly confirmed RLBP1-associated retinopathy and 5 with RDH5-associated retinopathy. Medical records were reviewed to obtain data on family history and ophthalmologic examinations, including retinal imaging and full-field electroretinography (ffERG). Genotype was determined by targeted next-generation sequencing followed by confirmation and familial segregation by Sanger sequencing. The median (interquartile ranges) age at baseline for the RDH5 and RLBP1 cohort was 44.6 (38.2-67.9) years and 36.9 (23.1-45.2) years, respectively. Macular atrophy (MA) was found in approximately 80% of eyes from both cohorts. The RLBP1 genotype was associated with a lower macular volume by 0.28 mm3 (95% CI, -0.46 to -0.11; P = .005) compared to the RDH5 genotype. In both genotypic cohorts, we found a significant annual rate of macular volume loss, estimated at -0.007 mm3/y (95% CI, -0.012 to -0.001; P = .02), without any significant difference between the two genotypes. Three unrelated patients homozygous for the c.361C>T p.(Arg121Trp) RLBP1 variant showed minimal impairment of both the rod and cone systems function on ffERG and absence of MA. Progressive MA in addition to congenital night blindness can be identified in adult patients with RDH5-associated retinopathy. Vice versa, hypomorphic RLBP1 variants may cause milder retinal phenotypes rather than the typical severe rod-cone dystrophy with MA. These findings could prove beneficial to improve the prognostication of patients and help in designing future interventional trials.

Treatment Impact on the Long-Term Ocular Outcomes in Behçet’s-Related Uveitis

ABSTRACT Purpose To examine the demographic characteristics, findings and complication rates in patients with Behçet’s uveitis (BU) and to investigate the effect of early biological therapy on the development of complications. Methods Medical records of 94 patients with BU were retrospectively reviewed. Demographic data, ocular findings and complications at presentation, complications during follow-up, and treatments received during follow-up were analyzed. Patients who were followed for at least 24 months were divided into two groups according to the time of presentation as Group 1 (between 2009 and 2015) and Group 2 (between 2016 and 2021). Complications at the time of presentation and during follow-up, and treatments were compared. Results We enrolled 94 patients with a male-to-female ratio of 1.94 with a mean age of 30 ± 12 years. Median follow-up was 58.1(12–163) months. There were 35 patients (66 eyes) in Group 1 and 33 patients (61 eyes) in Group 2. At the time of presentation, end-stage disease, cataract, epiretinal membrane, and optic atrophy were significantly more common in Group 1 than in Group 2 (p < 0.05). A significantly higher proportion of eyes in Group 1 developed macular edema, cataract, epiretinal membrane, and macular atrophy during-follow-up (p < 0.05). Median time to initiation of biological treatment was 17.29 months in Group 1 and 3.33 months in Group 2 (p < 0.001). The overall complication rate was significantly lower in Group 2. Conclusions Prognosis of BU is improved after the introduction of biological treatment. Early use of biological agents in BU is effective in decreasing sight-threatening ocular complications.

Approved AI-based fluid monitoring to identify morphological and functional treatment outcomes in neovascular age-related macular degeneration in real-world routine

AimTo predict antivascular endothelial growth factor (VEGF) treatment requirements, visual acuity and morphological outcomes in neovascular age-related macular degeneration (nAMD) using fluid quantification by artificial intelligence (AI) in a real-world...

Subretinal fluid in macular edema secondary to branch retinal vein occlusion

We identified characteristics of patients with subretinal fluid (SRF) in macular edema (ME) secondary to branch retinal vein occlusion (BRVO) and determined their clinical outcomes after anti-vascular endothelial growth factor (VEGF) treatment. Fifty-seven eyes of BRVO patients with ME were divided into two groups according to the presence or absence of SRF at diagnosis. We compared the aqueous profiles, ocular and systemic characteristics at baseline, and the clinical outcomes. The SRF group had significantly greater central subfield thickness (CST) values and poorer best-corrected visual acuity (BCVA) at baseline compared to the non-SRF group. The former group had significantly higher aqueous levels of interleukin-8, VEGF, and placental growth factor. CST reduction and BCVA improvement during treatment were significantly greater in the SRF group than in the non-SRF group. Consequently, CST values were significantly lower in the SRF group than in the non-SRF group at 12 months, when BCVA did not differ significantly between the two groups. The SRF group required more frequent anti-VEGF treatment over 12 months and exhibited a higher rate of macular atrophy. Based on the aqueous profiles and the number of treatments required, the presence of SRF in BRVO patients appears to be associated with higher disease activity.

Choroidal neovascularisation secondary toX-linked retinoschisis

AimsChoroidal neovascularisation (CNV) in patients with X-linked retinoschisis (XLRS) has been poorly documented. This study aims to investigate the prevalence and clinical characteristics of CNV in patients with XLRS, as...

The central retinal thickness and its related genotype in ABCA4-related retinopathy.

To further explore the influence of genotype, including mutation type and structural domain, on the severity of macular atrophy, we measured the central retinal thickness (CRT) in patients with ABCA4-related retinopathy. A total of 66 patients were included in the cohort. This was a retrospective investigation. The patients were tested using whole exon sequencing and ophthalmic exams, including slip lamp exams, best-corrected visual acuity, optical coherence tomography, fundus photo, and fundus autofluorescence. In this study, we discovered that mutations on nucleotide binding domains (NBD) lead to less CRT (45.00 ± 25.25μm, 95% CI: 31.54-58.46) had significantly less CRT than the others (89.75 ± 71.17μm, 95% CI: 30.25-149.25, p = 0.032), and could accelerate the rate of CRT decrease. Our study provides new perspectives in the understanding of ABCA4-related retinopathy.

Differentiating glaucoma from chiasmal compression using optical coherence tomography: the macular naso-temporal ratio

Background/aimsThe analysis of visual field loss patterns is clinically useful to guide differential diagnosis of visual pathway pathology. This study investigates whether a novel index of macular atrophy patterns can...

The Effect of Blue-light Filtering Intraocular Lenses on the Development and Progression of Macular Atrophy in Eyes With Neovascular Age-related Macular Degeneration

PurposeTo 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). DesignRetrospective clinical cohort study. MethodsnAMD patients 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 OCT scans were manually reviewed in a masked manner regarding patient baseline variables and IOL status by an experienced research technician. Using the Heidelberg software, the area of MA was manually evaluated and calculated (mm2) 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. ResultsIncluded were 373 eyes of 373 patients (mean age 78.6±6.7 years at surgery, 67.4% females). 206 eyes were implanted with BLF IOLs and 167 with non-BLF IOLs with comparable follow-up times (3164±1420 days vs. 3180±1403 days, respectively, P=0.908), and other baseline parameters (age, gender, corrected distance visual acuity, macular thickness, cumulative number of anti-VEGF injections). Nine pre-existing and 77 new-onset MA cases were detected, with similar distribution between BLF and non-BLF eyes (P=0.598 and P=0.399, respectively). Both univariate Kaplan-Meier (P=0.366) and multivariate Cox regression analysis adjusted for age and gender showed that BLF-IOLs were comparable to non-BLF IOLs regarding hazard for new-onset MA (HR 1.236, 95% CI 0.784-1.949, P=0.363). Final MA area at the last visit was 5.14±4.71mm2 for BLF IOLs and 8.56±9.17mm2 for non-BLF IOLs (P=0.028), with the mean annual MA area increase of 0.78±0.84mm2 and 1.26±1.32mm2, respectively (P=0.042). ConclusionsBLF 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 nAMD patients.

Effectiveness of EScoop® lenses on a Select Group of Veterans

Purpose: The purpose of this study was to perform a retrospective chart review to see if the use of EScoop® lenses improved the referred veterans' contrast acuity and subjective visual discomfort of glare, photophobia, or difficulty with night driving. Methods: We retrospectively reviewed examinations of 23 veterans aged 36 to 91 who chose EScoop ® for their spectacle prescription. The EScoop ® set available in our clinic included two shades, yellow or orange, two center lens thicknesses (LT), 6 or 9 mm, and with or without 4 prism diopters yoked base up (BU) prism. Results: The largest diagnostic group tested was with traumatic brain injury (12 of 23), and they preferred the orange-tinted lens (10 of 12). The orange 9 mm LT with 4 prism diopters BU was the most commonly chosen EScoop® lens overall (5 of 23). Pre and post lens visual acuity testing with Sloan EDTRS LogMAR Low Contrast Chart at 25% contrast was available for 13 of the 23 patients. A small but statistically significant improvement of 0.11 decimal mean acuity (about one-line Snellen) under a low contrast condition was noted while wearing the preferred EScoop ® lenses (Wilcoxon Signed Rank P=0.015). Conclusion: Our retrospective study reviewed visually impaired patients with varied clinical conditions, including traumatic brain injury, age-related macular degeneration, glaucoma, and optic atrophy. Upon informal questioning, the patients reported improvements in glare, photophobia, and night glare symptoms. Objectively, we measured approximately one line of improvement in contrast Snellen acuity, which was statistically significant. Further research is needed to discern the actual effectiveness of this lens.

Effectiveness of EScoop® lenses on a Select Group of Veterans

Purpose: The purpose of this study was to perform a retrospective chart review to see if the use of EScoop® lenses improved the referred veterans' contrast acuity and subjective visual discomfort of glare, photophobia, or difficulty with night driving. Methods: We retrospectively reviewed examinations of 23 veterans aged 36 to 91 who chose EScoop ® for their spectacle prescription. The EScoop ® set available in our clinic included two shades, yellow or orange, two center lens thicknesses (LT), 6 or 9 mm, and with or without 4 prism diopters yoked base up (BU) prism. Results: The largest diagnostic group tested was with traumatic brain injury (12 of 23), and they preferred the orange-tinted lens (10 of 12). The orange 9 mm LT with 4 prism diopters BU was the most commonly chosen EScoop® lens overall (5 of 23). Pre and post lens visual acuity testing with Sloan EDTRS LogMAR Low Contrast Chart at 25% contrast was available for 13 of the 23 patients. A small but statistically significant improvement of 0.11 decimal mean acuity (about one-line Snellen) under a low contrast condition was noted while wearing the preferred EScoop ® lenses (Wilcoxon Signed Rank P=0.015). Conclusion: Our retrospective study reviewed visually impaired patients with varied clinical conditions, including traumatic brain injury, age-related macular degeneration, glaucoma, and optic atrophy. Upon informal questioning, the patients reported improvements in glare, photophobia, and night glare symptoms. Objectively, we measured approximately one line of improvement in contrast Snellen acuity, which was statistically significant. Further research is needed to discern the actual effectiveness of this lens.

Optical Coherence Tomography in Inherited Macular Dystrophies: A Review.

Macular dystrophies (MDs) constitute a collection of hereditary retina disorders leading to notable visual impairment, primarily due to progressive macular atrophy. These conditions are distinguished by bilateral and relatively symmetrical abnormalities in the macula that significantly impair central visual function. Recent strides in fundus imaging, especially optical coherence tomography (OCT), have enhanced our comprehension and diagnostic capabilities for MD. OCT enables the identification of neurosensory retinal disorganization patterns and the extent of damage to retinal pigment epithelium (RPE) and photoreceptor cells in the dystrophies before visible macular pathology appears on fundus examinations. It not only helps us in diagnostic retinal and choroidal pathologies but also guides us in monitoring the progression of, staging of, and response to treatment. In this review, we summarize the key findings on OCT in some of the most common MD.

Influence of submacular hemorrhage at baseline on the long-term outcomes of aflibercept treatment for typical neovascular age-related macular degeneration and polypoidal choroidal vasculopathy.

To investigate the influence of submacular hemorrhage (SMH) at baseline on long-term visual outcomes of patients with typical age-related macular degeneration (tAMD) and polypoidal choroidal vasculopathy (PCV) treated with intravitreal aflibercept (IVA). In this retrospective study, eyes of treatment-naïve patients with tAMD and PCV who initiated IVA under a treat-and-extend regimen and were followed up for ≥ 5years were classified into the tAMD-SMH ( +), tAMD-SMH (-), PCV-SMH ( +), and PCV-SMH (-) groups based on the presence of SMH at baseline. Best-corrected visual acuity (BCVA) changes and macular fibrosis and atrophy incidences were assessed. This study included 127 eyes (127 patients), including 51 with tAMD and 76 with PCV; 18 eyes had SMH at baseline. In the tAMD-SMH ( +) group (n = 6), the mean logMAR BCVA significantly deterioratedfrom 0.59 ± 0.45 at baseline to 0.88 ± 0.47 at the final visit (P = 0.024). No significant BCVA changes were observed in the tAMD-SMH (-) (n = 45), PCV-SMH ( +) (n = 12), or PCV-SMH (-) (n = 64) groups (all P > 0.05). The tAMD-SMH ( +) group showed a significantly higher incidence of macular fibrosis at the final visit than did the tAMD-SMH (-) group (P = 0.042). There was no influence of baseline SMH on the macular fibrosis incidence in eyes with PCV and the macular atrophy incidence in eyes with tAMD and PCV. The presence of SMH at baseline resulted in poorer long-term visual acuity in eyes with tAMD, even with aflibercept treatment. However, no such influence was observed in eyes with PCV.

Effect of Pro Re Nata Regimen with Anti-VEGF on Type 3 Macular Neovascularization: Long-Term Outcomes

Introduction: The purpose of this study was to investigate long-term outcomes of intravitreal injections (IVI) of antivascular endothelial growth factor (VEGF) in neovascular age-related macular degeneration (nAMD) with type 3 macular neovascularization (MNV). Methods: This retrospective study included 19 eyes of 17 patients with nAMD and type 3 MNV treated with anti-VEGF IVI with a loading dose and a PRN regimen. Best corrected visual acuity (BCVA), central macular thickness (CMT), presence of macular intraretinal fluid (IRF) and subretinal fluid (SRF), flow area (FA), subfoveal choroidal thickness (CT), and macular atrophy (MA) were assessed at baseline (T0) and during follow-up (T1, post-loading phase; T2, 1 year; T3, 2 years; T4 >2 years). The correlations between MA at the last follow-up and standard deviation (SD) values of CMT and CT during follow-up were assessed. The influence of the number of injections on the change in MA over time was also analyzed. MA differences at T4 were assessed for pseudodrusen presence. Results: BCVA improved significantly during follow-up (p = 0.013) particularly increasing from baseline to post-loading phase and then did not modify significantly thereafter. CMT significantly reduced from T0 to T1 and remained stable during follow-up (p = <0.001). MNV flow area showed a trend toward an increase in the post-loading phase that was not statistically significant (p = 0.082) and CT decreased significantly during follow-up (p < 0.001). MA changed significantly during follow-up (p < 0.001) with a significant increase from T0 to T3 and from T0 to T4 (p < 0.010). A Cochran-Armitage test for trend showed a significant reduction (p = 0.001) of macular IRF and SRF during follow-up. MA at T4 showed a significant positive correlation with SD (standard deviation) values of CMT (p = 0.040) and CT (p = 0.020). Indeed, the number of injections did not influence the change over time of MA (p = 0.709). MA at T4 was not statistically significantly different between patients with pseudodrusen at baseline (p = 0.497). Conclusions: Intravitreal anti-VEGF injections with PRN regimen in MNV type 3 showed functional and anatomical benefits. Variations of retinal thickness and choroidal thickness during treatment were related to MA modification over time.

The incidence of macular atrophy in patients with long-term use of anti-vascular endothelial growth factor (VEGF) in age-related macular degeneration (AMD): a systematic review and meta-analysis

Background: Age-related macular degeneration (AMD) is the chief factor in sightlessness in affluent nations. Visual symptoms at a late stage of AMD are frequently severe and irreversible and might include drastically reduced center vision in both eyes. Patients with nAMD are mostly preserved with anti-vascular endothelial growth factor (VEGF) medications. Chronic anti-VEGF treatment, however, has also been connected to the emergence of macular atrophy (MA). This study aims to determine the prevalence of MA in AMD patients who have been on anti-VEGF medications for a long time. Methods: The literature research was performed by systematically searching PubMed, Cochrane, ScienceDirect, and Google Scholar using the search terms “macular atrophy”, “Anti-VEGF”, “age-related macular degeneration”, and “trial”. The Review Manager v.5.4 and R statistical software v.3.3 are used in this study. Results: The study includes 2094 individuals from 7 clinical trial trials. Anti-VEGF therapy had an overall proportion of MA episodes in MDA patients of 0.29 (95% CI 0.24 - 0.34). Between the anti-VEGF and control groups, there is no discernible difference in the risk of MA (OR 1.44; 95% CI 0.34 - 6.04; p=0.39). Additionally, there is no discernible difference between the comparison group and anti-VEGF regarding mean MA area scores (MD 0.03; 95% CI -0.28 - 0.34; p=0.85). Conclusion: The incidence of MA in long-term anti-VEGF use with other comparators in AMD was not significantly different.

Extensive Macular Atrophy with Pseudodrusen-like appearance: Progression Kinetics and Late-Stage Findings

To describe the clinical outcome and late-stage findings of extensive macular atrophy with pseudodrusen-like appearance (EMAP). Retrospective cohort study. Seventy-eight patients (156 eyes) affected by EMAP. We collected data on best-corrected visual acuity, kinetic perimetry, OCT, short-wavelength autofluorescence, and near-infrared autofluorescence findings. Genetic testing for the TIMP3 and C1QTNF5 genes was performed via Sanger sequencing for 58 patients, with no pathogenic variants identified. The primary outcomes were best-corrected visual acuity at the last examination, visual field at the last examination, and incidence rates and time-to-event curves for blindness with the United States Social Security Administration and World Health Organization (WHO) criteria, foveal involvement, and atrophy enlargement beyond the 30° and 55° field of view. Imaging findings at the last examination were secondary outcomes. At the most recent visit, mean age was 70.9 ± 5.2 years. Using United States criteria, 58.1% of the patients were blind, and 25.8% were blind according to WHO criteria. All eyes showed large central scotomas, which were associated with visual field constriction in 22.2% of eyes. We detected focal openings or large dehiscences of Bruch's membrane (BM) in 25.4% of eyes. Near-infrared autofluorescence showed increased visibility of the choroidal vessels beyond the atrophy in 87.2% of eyes. The incidence rates for blindness were 3.95 per 100 patient-years with United States criteria and 1.54 per 100 patient-years according to WHO criteria. The incidence rates were 22.8 per 100 eye-years for foveal involvement, 12.0 per 100 eye-years for atrophy enlargement beyond 30°, and 6.6 per 100 eye-years for atrophy enlargement beyond 55°. The estimates were not influenced by the age at onset. We identified characteristic imaging findings, including BM ruptures, in elder patients with EMAP and calculated incidence rates for different functional and anatomic outcomes. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Adaptive Optics Retinal Imaging in RDH12-Associated Early Onset Severe Retinal Dystrophy.

RDH12 is among the most common genes found in individuals with early-onset severe retinal (EOSRD). Adaptive optics scanning light ophthalmoscopy (AOSLO) enables resolution of individual rod and cone photoreceptors in the retina. This study presents the first AOSLO imaging of individuals with RDH12-associated EOSRD. Case series of patients who attended Moorfields Eye Hospital (London, UK). Spectral-domain optical coherence tomography, near-infrared reflectance (NIR), and blue autofluorescence imaging were analyzed. En face image sequences of photoreceptors were recorded using either of two AOSLO modalities. Cross-sectional analysis was undertaken for seven patients and longitudinal analysis for one patient. Nine eyes from eight patients are presented in this case series. The mean age at the time of the assessment was 11.2 ± 6.5years of age (range 7-29). A subfoveal continuous ellipsoid zone (EZ) line was present in eight eyes. Posterior pole AOSLO revealed patches of cone mosaics. Average cone densities at regions of interest 0.5° to the fovea ranged from 12,620 to 23,660 cells/mm2, whereas intercell spacing ranged from 7.0 to 9.7µm. This study demonstrates that AOSLO can provide useful high-quality images in patients with EOSRD, even during childhood, with nystagmus, and early macular atrophy. Cones at the posterior pole can appear as scattered islands or, possibly later in life, as a single subfoveal conglomerate. Detailed image analysis suggests that retinal pigment epithelial stress and dysfunction may be the initial step toward degeneration, with NIR being a useful tool to assess retinal well-being in RDH12-associated EOSRD.