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Purpose: To characterize morphological changes in the retina and to report the frequency and natural history of non-exudative macular neovascularization (MNV) in a cohort of pseudoxanthoma elasticum (PXE)., Methods: A single-center, retrospective study was complemented by a cross-sectional examination. Consecutive patients with a definitive genetic and/or clinical diagnosis of PXE, visiting our department between January 2019 and December 2019, and with a minimum follow-up of 6 months were recruited. Baseline data were retrieved from each patient file. Additionally, a cross-sectional examination comprising color fundus photography, spectral-domain optical coherence tomography (SD-OCT), OCT-Angiography (OCT-A), and fundus autofluorescence was performed. The presence of typical PXE-related findings, as well as related complications, was multimodally evaluated. The prevalence and natural history of non-exudative MNV were assessed. All images were graded by two independent graders., Results: Forty-eight eyes from 24 patients (mean age 59.11 ± 18.14) with a median follow-up of 53.00 months were included. Angioid streaks and peau d'orange were observed in 46/48 and 42/48 eyes, while MNV was present in 75.00% of the cohort. The prevalence of non-exudative MNV was 33.33% (6/18). In the 2 eyes that developed exudation, time to conversion was 9.50 ± 4.95 months. No significant difference in visual acuity was found between eyes with non-exudative MNV and those with no signs of MNV., Conclusion: We have shown that non-exudative MNV is a frequent finding in PXE but the majority of eyes did not develop exudation during follow-up. Our results are a clear evidence of the utility of OCT-A in the management of PXE.

Purpose: To assess the development of macular atrophy, according to the new Classification of Atrophy Meetings criteria, in patients with treatment-naïve neovascular age-related macular degeneration during the first year of treatment with ranibizumab or aflibercept, and to determine baseline factors predictive of atrophy development., Methods: Retrospective subanalysis of three prospective clinical trials that included eyes with treatment-naïve neovascular age-related macular degeneration. Multimodal evaluation was performed with spectral-domain optical coherence tomography, fluorescein angiography, fundus autofluorescence and color fundus photography at baseline and after 12 months of treatment. The main outcome was the macular atrophy type, classified according to Classification of Atrophy Meeting criteria. Logistic regression models were built to test predictors of macular atrophy development., Results: A total of 85 eyes of 85 patients (63% female; mean age: 78.5 ± 6.3 years old) were included. After 12 months of antiangiogenic therapy, all four Classification of Atrophy Meeting types of macular atrophy developed de novo . The atrophy type with highest incidence at end of follow-up was incomplete retinal pigment epithelium and outer retinal atrophy (63.6%; 95% confidence interval: 45.9%-86.0%). A significant association was observed between development at 12 months and the presence of incomplete retinal pigment epithelium and outer retinal atrophy at baseline (odds ratio (95% confidence interval): 22.4 (1.6, 323.5)). The number of injections was predictive of complete outer retinal atrophy development at end of follow-up (odds ratio (95% confidence interval) 1.5 (1.1, 2.1), p = 0.011)., Conclusion: Predictors of atrophy development have the potential to change treatment practices. Further research is warranted.

Purpose: The aim of the study was to characterize the morphological features of polypoidal choroidal vasculopathy (PCV) in a large Caucasian population., Methods: We conducteda multicenter, cross-sectional study of treatment-naïve patients with PCV. Baseline fundus photography, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and indocyanine green angiography (ICGA) were assessed by trained medical graders. Typical PCV features were explored, and retinal thickness (RT) and choroidal thickness (CT) measurements were performed., Results: Seventy-nine eyes of 73 patients (mean age, 72.6 ± 11.9 years) were included. ICGA identified macular polyps in 89.9% of cases. SD-OCT revealed mostly subretinal fluid (93.6%) and a retinal pigment epithelium (RPE) detachment in 91.4%, with sharp protrusion in 67.0% of cases. Polyp-like structures were seen in 74.3% of cases, mostly adherent to an elevated RPE (69.6%). Type 1 neovascularization (NV) was identified in 74.7% of patients, while 16.5% had a mixed NV. The mean macular CT was 220.9 ± 83.2 μm (range, 67.9-403.6). Diffuse and focal pachychoroid were observed in 26.6 and 30.4% of patients, respectively. Soft drusen were reported in 62.0% of cases, but retinal hemorrhage occurred in only 19.0% of cases., Conclusion: The morphological features of PCV in Caucasians are similar to those reported in Asians. Pachychoroid signs were found in nearly half of our cohort. However, the mean age at presentation, high prevalence of soft drusen, and low prevalence of large subretinal hemorrhages make PCV closer to age-related macular degeneration in this ethnic group., (© 2021 S. Karger AG, Basel.)

Purpose: To evaluate the long-term progression of myopic maculopathy and functional outcome after treatment of myopic choroidal neovascularization (CNV) with photodynamic therapy (PDT) and/or intravitreal ranibizumab (IVR)., Methods: Retrospective study with a cross-sectional evaluation. Eyes were assigned to 4 groups (PDT, IVR, PDT + IVR, dry myopic maculopathy) and evaluated with best-corrected visual acuity, color fundus photography and spectral-domain optical coherence tomography. Chorioretinal atrophy progression was quantified., Results: Fifty-four eyes were included with a mean follow-up of 80.6 ± 28.0 months. The prevalence of diffuse, patchy and macular atrophy increased during the follow-up, in contrast with tessellated fundus, lacquer cracks and active CNV. Progression of macular atrophy was significant in the 3 treatment groups (p < 0.05) and predictive of visual acuity. It depended on age, degree of myopia and presence of staphyloma, but not on the type of treatment., Conclusions: The long-term functional outcome of eyes with myopic CNV is more dependent on the progression of macular atrophy, and not on the type of treatment., (© 2014 S. Karger AG, Basel.)

Purpose: To evaluate choroidal thickness in highly myopic eyes with choroidal neovascularization (CNV), 3 or more years after treatment with photodynamic therapy (PDT), intravitreal ranibizumab (IVR), or both (PDT + IVR)., Methods: The medical records of patients with high myopia and CNV treated with PDT or IVR in our department were reviewed. Eyes meeting the inclusion criteria were assigned to 3 groups: PDT, IVR, and PDT + IVR. A fourth group, "dry myopic maculopathy," included the contralateral highly myopic eyes that never developed CNV. All patients performed a cross-sectional evaluation with best-corrected 
visual acuity (BCVA), measurement of axial length, color fundus photography, and enhanced depth imaging with spectral domain optical coherence tomography., Results: Forty-two eyes (21 patients) were included: 11 eyes (26.2%) in the PDT group, 8 (19.0%) in the IVR group, 9 (21.4%) in the PDT + IVR group, and 14 (33.3%) in the dry maculopathy group. Subfoveal choroidal thickness showed no significant differences between groups (p>0.05). Positive correlation was found between BCVA and macular choroidal thickness (r = +0.293, p<0.001). Regression analysis showed that age (p<0.001), axial length (p<0.001), sex (p = 0.001), and myopic lesions such as tessellated fundus (p = 0.046) and patchy atrophy (p = 0.008) were predictive of choroidal thickness. Type of treatment was not predictive of choroidal thickness., Conclusions: Older age and greater axial length are the major factors associated with macular choroidal thinning in highly myopic eyes submitted to CNV treatment. The type of treatment performed for myopic CNV had no predictive contribution for choroidal thickness.

Purpose: To evaluate the long-term safety and efficacy of intravitreal ranibizumab in the treatment of myopic choroidal neovascularization (CNV)., Methods: Three-year retrospective, nonrandomized, interventional case series. Forty eyes of 39 patients with myopic CNV were included; 15 with previous photodynamic therapy, and 25 naïve eyes. Best-corrected visual acuity (BCVA) changes, central foveal thickness (CFT), and number of treatments were assessed, from baseline to month 36., Results: Mean visual acuity improved from 55.4 Early Treatment Diabetic Retinopathy Study (ETDRS) letters at baseline to 59.7 letters at 12 months (p = 0.07), 61.8 letters at 24 months (p = 0.008) and 63.4 letters at 36 months (p = 0.039). Twenty-five percent of the patients gained ≥15 letters (3 lines) at 12 months, 30% at 24 months and 35% at 36 months. There was a mean reduction of 80 μm in CFT (p < 0.001). A mean of 4.1 injections were performed in the first year, 2.4 in the second year and 1.1 in the third year. Fifty-three percent of the eyes had no need for treatment during the third year of follow-up., Conclusions: Intravitreal ranibizumab seems to be an effective and safe therapeutic procedure to treat CNV in highly myopic eyes, with a high proportion of patients gaining or stabilizing BCVA at a 3-year follow-up., (Copyright © 2011 S. Karger AG, Basel.)

Objective: To identify morphological and/or functional early markers of choroidal neovascularization (CNV) development in fellow eyes of patients with exudative age-related macular degeneration (AMD)., Design: This is a single-center, prospective, observational, longitudinal 2-year study., Patients: Patients were enrolled with the diagnosis of neovascular AMD in 1 eye and early age-related maculopathy (ARM) in the fellow eye. Intervention or Methods: All patients completed the baseline assessment and were followed up for up to 24 months with repeated ophthalmic and imaging assessments performed at 6-month intervals., Main Outcome Measures: Each patient underwent a detailed ocular and medical history, a complete ophthalmologic examination with color fundus photography, fluorescein angiography, indocyanine green angiography (ICG), optical coherence tomography (OCT), fundus autofluorescence (FAF) imaging and retinal leakage analysis (RLA)., Results: Sixty-two patients were enrolled in the study. Large or intermediate drusen were present in 100% of the study eyes and hyperpigmentation in 46% (24 eyes). Fifty-two patients completed the 2-year study follow-up. Large soft drusen (>125 μm) were observed in 15 out of 17 eyes (88%) that converted and developed CNV during the study and in 25 out of 35 eyes (71.4%) that did not develop CNV. Among the 17 eyes that developed CNV, 9 (53%) showed abnormal findings before conversion, on ICG. No particular FAF pattern was found to be correlated with conversion to wet AMD. OCT was able to document the presence of intra- or subretinal fluid at the time of conversion in all 17 eyes that developed CNV during the study. Alterations of the blood-retinal barrier were identified by RLA before conversion in 76% of the eyes that converted and 23% of the eyes that did not convert during the study., Conclusions: Characterization of early ARM phenotypes is challenging. By combining different imaging modalities of the macula and correlating this information, we were able to determine the presence of functional macular alterations in the fellow eye of patients with this disease before development of CNV., (Copyright © 2010 S. Karger AG, Basel.)

Purpose: To evaluate the long-term efficacy of ranibizumab in the treatment of retinal angiomatous proliferation (RAP) and to identify predictors of functional outcome. Methods: Retrospective case series comprised 79 eyes of 68 consecutive patients with RAP followed up ≥36 months. Primary end-points were best-corrected visual acuity (BCVA) and central macular thickness (CMT) variation at 36 months and at the last visit. Results: Mean follow-up time was 59.8 ± 16.0 months. All eyes were treated with pro re nata ranibizumab, with (n = 33) or without (n = 46) photodynamic therapy (PDT). Stabilization or improvement in BCVA was observed in 50.6% of the patients at 36 months, and in 40.5% at the end of the follow-up, where 20.3% preserved reading vision. A significant decrease in CMT was observed at 36 months (p < 0.001), but not at the end of the follow-up. Geographic atrophy (GA) was present in 59.5% of the eyes at the final visit. Baseline subretinal fluid was associated with better visual outcomes (p = 0.001). Results of combination treatment with intravitreal ranibizumab and PDT did not significantly differ from ranibizumab monotherapy. Conclusion: Modest functional outcomes can be expected from the long-term treatment of RAP lesions in clinical practice, most likely due to the advent of GA. Baseline subretinal fluid positively correlated with final BCVA. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the 36-month efficacy of intravitreal ranibizumab injections for choroidal neovascularization secondary to age-related macular degeneration (AMD) in real world clinical practice. Methods: Retrospective study involving 84 eyes of 77 patients; 52 eyes completed 3 years of follow-up. Subjects were observed initially on a monthly basis and with extended follow-up intervals if signs of quiescence were detected, according to an established protocol. A comprehensive ophthalmologic examination was performed, including best-corrected visual acuity (BCVA) determined with Early Treatment Diabetic Retinopathy Study charts, stereoscopic macular biomicroscopy and optical coherence tomography (OCT) with fluorescein angiography and indocyanine green angiography if considered necessary. Treatment was given if signs of active lesions were present. Results: The mean baseline BCVA was 49.33 and 49.52 letters at the 36-month visit. The average of treatments was 8.6 at 3 years. At this time point, 77% of treated eyes stabilized or improved their vision (VA loss ≤5 letters). A predictive value for better VA was found for younger age, better baseline VA, good response on OCT and more frequent treatments. Conclusion: At 3 years, intravitreal ranibizumab is able to maintain baseline VA in exudative AMD patients, with a reduced number of injections, but not to show VA improvement, in clinical practice. Copyright © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the long-term safety and efficacy of intravitreal ranibizumab in the treatment of myopic choroidal neovascularization (CNV). Methods: Three-year retrospective, nonrandomized, interventional case series. Forty eyes of 39 patients with myopic CNV were included; 15 with previous photodynamic therapy, and 25 naïve eyes. Best-corrected visual acuity (BCVA) changes, central foveal thickness (CFT), and number of treatments were assessed, from baseline to month 36. Results: Mean visual acuity improved from 55.4 Early Treatment Diabetic Retinopathy Study (ETDRS) letters at baseline to 59.7 letters at 12 months (p = 0.07), 61.8 letters at 24 months (p = 0.008) and 63.4 letters at 36 months (p = 0.039). Twenty-five percent of the patients gained ≥15 letters (3 lines) at 12 months, 30% at 24 months and 35% at 36 months. There was a mean reduction of 80 μm in CFT (p < 0.001). A mean of 4.1 injections were performed in the first year, 2.4 in the second year and 1.1 in the third year. Fifty-three percent of the eyes had no need for treatment during the third year of follow-up. Conclusions: Intravitreal ranibizumab seems to be an effective and safe therapeutic procedure to treat CNV in highly myopic eyes, with a high proportion of patients gaining or stabilizing BCVA at a 3-year follow-up. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]