Subclinical Angioid Streaks with Pseudodrusen: A New Phenotype of Age-Related Macular Degeneration.

INTRODUCTION: To describe subclinical angioid streaks (AS) as a frequent, peculiar age-related macular degeneration (AMD) phenotype, comparing features of eyes with subclinical AS with those of eyes with AMD without AS. METHODS: This was a retrospective, observational study. Among a patient cohort with AMD, we selected patients without known causes for AS whose eyes showed signs of angioid streaks (AS) on structural optical coherence tomography (OCT) but not on fundus examination. Selected OCT features of AS were Bruch's membrane (BM) breaks and large BM dehiscences. RESULTS: Among 543 eyes of 274 patients with AMD (mean ± standard deviation: 82 ± 7 years), 73 eyes of 46 patients (81 ± 7 years; p = 0.432) showed AS features on OCT (OCT AS) that were not visible on fundus examination. Estimated prevalence of subclinical age-related AS was 13.4% (95% confidence interval 10.3-16.3%) in this AMD population. Fifty-three eyes (73%) with AS features were affected by peripapillary atrophy, often with a "petaloid-like" pattern, similar to typical features of AS disease. Almost all cases (97%) presented reticular pseudodrusen (RPD), with (41%) or without (59%) drusen showing a significant difference in RPD prevalence in OCT AS eyes in comparison to AMD eyes without subclinical AS using generalized estimating equations (P < 0.001). Among the 73 subclinical AS cases, 71 were affected by late AMD (57 with macular neovascularization, 14 with geographic atrophy), showing a more advanced AMD stage in comparison with AMD eyes without subclinical AS (P < 0.001). The following OCT features were disclosed: BM breaks in 100% of cases and BM dehiscences in 37%. CONCLUSIONS: Subclinical AS in eyes with AMD is a peculiar phenotype of the disease, with features suggesting a primary involvement of Bruch's membrane and clinical similarities with mild, late-onset pseudoxanthoma elasticum.

No-Dose Photodynamic Therapy Against Half-Dose Photodynamic Therapy for Treatment of Central Serous Chorioretinopathy.

INTRODUCTION: This study aimed to describe the effects of no-dose full-fluence photodynamic therapy without verteporfin (no-dose PDT) and to compare no-dose PDT with half-dose verteporfin full-fluence photodynamic therapy (HDFF PDT) for managing chronic central serous chorioretinopathy (cCSC). METHODS: This retrospective study evaluated 11 patients with chronic recurrent CSC treated with no-dose PDT between January 2019 and March 2022. Most of these patients were also treated with HDFF PDT a minimum of 3 months before and were considered as the control group. We described the changes of best corrected visual acuity (BCVA), maximum subretinal fluid (mSRF), foveal subretinal fluid (fSRF), and choroidal thickness (CT) 8 ± 2 weeks after no-dose PDT, and we compared BVCA, mSRF, fSRF, and CT of no-dose PDT with those of the of same patients previously treated with HDFF PDT. RESULTS: Fifteen eyes of 11 patients (10 male, mean age 54 ± 12 years) received no-dose PDT; among these, 10 eyes of 8 patients (7 male, mean age 53 ± 12 years) also received HDFF PDT. Three eyes showed complete resolution of fSRF after no-dose PDT. No significant differences were disclosed between treatment with and without verteporfin comparing BCVA, mSRF, fSRF, and CT at baseline and 8 ± 2 weeks from the treatment (p > 0.05 in all analyses). CONCLUSION: BVCA and CT significantly improved after no-dose PDT. Short-term functional and anatomical treatment outcomes for cCSC were similar for HDFF PDT and no-dose PDT. We hypothesize that the potential benefits of no-dose PDT may arise from thermal elevation that triggers and enhances photochemical activities by endogenous fluorophores, activating a biochemical cascade response that rescues/replaces sick, dysfunctional retinal pigment epithelial (RPE) cells. Results of this study suggest the potential value of a prospective clinical trial to evaluate no-dose PDT for managing cCSC, especially when verteporfin is contraindicated or unavailable.

The state-of-the-art pharmacotherapeutic management of neovascular age-related macular degeneration.

INTRODUCTION: Age-related macular degeneration (AMD) is a major cause of central visual loss in the developed world. Although the pathogenesis is not fully understood, vascular endothelial growth factor (VEGF) is considered the most important growth factor involved in angiogenesis and exudation in neovascular AMD eyes. Advances in anti-VEGF agents have changed the treatment approach for wet AMD, allowing better outcomes in visual acuity and retinal anatomy. AREAS COVERED: The present review describes the main pharmacological and clinical characteristics of anti-VEGF drugs, focusing firstly on the molecules commonly in use and then on the new candidate therapies. We performed a comprehensive literature search using the PubMed database from 1 January 1993 to 1 June 2022, with search terms including anti-VEGF, biosimilar, neovascular macular degeneration, AMD, and safety. EXPERT OPINION: The management of AMD is still onerous for both the physician and patient due to the great number of required injections. Current research is underway to resolve not only the economic burden but also the discomfort of patients, aiming to develop a drug with a different or a multiple target, increasing the potency whilst maintaining a good safety profile. Furthermore, clinical research is currently investigating different forms of drug administration.

Bilateral asymptomatic macular hypopigmentation in a young woman: multimodal imaging and pathogenetic hypothesis.

Purpose: Macular retinal pigment epithelium (RPE) hypopigmentation is a recently described very rare condition and its pathogenesis is not completely understood. We report the case of a 23-year-old female who presented with bilateral whitish, oval-shaped foveal lesions and we speculated about the possible etiopathogenetic origin. Observations: A 23-year-old female presented to our consideration for a routine ophthalmology visit. Visual acuity was 20/20 in both eyes. The fundus examination revealed a perifoveal choroidal nevus in the right eye and a bilateral yellowish, oval-shaped lesion centered on the fovea. Imaging tests (Spectral Domain-Optical Coherence Tomography, short wavelength and near-infrared autofluorescence) and functional tests (microperimetry and multifocal electroretinogram) were within normal limits, supporting the diagnosis of macular hypopigmentation without functional loss. Conclusions: A complex dysregulation of both choroidal and RPE with melanin loss may be responsible for this condition.

Sub-retinal pigment epithelium tubules in non-neovascular age-related macular degeneration.

To describe a novel optical coherence tomography (OCT) signature resembling sub-retinal pigment epithelium (RPE) tubules (SRT) in non-neovascular age-related macular degeneration (AMD). Patients suffering from non-neovascular AMD with complete medical records and multimodal imaging were retrospectively revised in three different tertiary care centers. Multimodal imaging included color fundus photograph, spectral-domain OCT (Spectralis, Heidelberg Engineering, Germany), fundus autofluorescence, OCT angiography (RTVue XR Avanti, Optovue, Inc., Fremont, CA). A total of 7 eyes of 7 patients with drusenoid pigment epithelium detachment (PED) were consecutively analyzed. The sub-RPE tubules appeared as ovoidal structures with a hyperreflective contour and hyporeflective interior appreciable in the sub-RPE-basal lamina (BL) space on OCT B-scan. The anatomical location of the sub-RPE formations was lying above the Bruch's membrane in 5/7 cases (71.4%) or floating in the sub-RPE-BL space in 2/7 cases (28.6%). En-face OCTA revealed a curvilinear tubulation-like structure corresponding to SRT without flow signal. Sub-RPE tubules represent a newly identified OCT signature observed in eyes with drusenoid PED. The presumed origin may include a variant of calcified structure or alternatively activated RPE cells with some residual BL or basal laminar deposits attracted to BrM for craving oxygen.

A COMMON FINDING IN FOVEAL-SPARING EXTENSIVE MACULAR ATROPHY WITH PSEUDODRUSEN IMPLICATES BASAL LAMINAR DEPOSITS.

PURPOSE: To characterize structural and clinical alterations preceding the diffuse macular atrophy in extensive macular atrophy with pseudodrusen (EMAP) and their evolution toward atrophic changes. METHODS: A retrospective chart review was performed of patients with early-onset reticular pseudodrusen (i.e., pre-EMAP) younger than 55 years and EMAP with foveal sparing. Patients were included if they had complete medical records and multimodal imaging. RESULTS: A total of 12 patients were reviewed, of whom 4 of 12 patients (7 eyes) presented a pre-EMAP stage, characterized by the presence of pseudodrusen-like deposits without atrophic changes, while the remaining 8 of 12 patients (10 eyes) exhibited EMAP with foveal sparing (60.1 ± 6.4 years). Subretinal deposits of various stages tended to fade, leaving subretinal pigment epithelium accumulation of hyperreflective material with a physical separation between the retinal pigment epithelium-basal lamina and the Bruch membrane, along with the persistence of hyperreflective material after retinal pigment epithelium loss. These findings preceded atrophy development in a pre-EMAP stage and the EMAP stage with foveal sparing. CONCLUSION: Our findings presented distinct multimodal imaging features in eyes with reticular pseudodrusen depicting a peculiar phenotype of rapidly progressing atrophy in midlife. The disease spectrum may include other forms of geographic atrophy allied by thickened basal laminar deposits.

The COVID-19 Pandemic Has Had Negative Effects on Baseline Clinical Presentation and Outcomes of Patients with Newly Diagnosed Treatment-Naïve Exudative AMD.

PURPOSE: To investigate whether the coronavirus disease 2019 (COVID-19) pandemic-associated postponement in care had effects on the baseline clinical presentation of patients with newly diagnosed treatment-naïve exudative neovascular age-related macular degeneration (AMD). METHODS: We included the first 50 consecutive patients referred within the COVID-19 pandemic with a diagnosis of treatment-naïve exudative neovascular AMD. Two groups of fifty consecutive patients with newly diagnosed neovascular exudative AMD presenting in 2018 and 2019 (control periods) were also included for comparisons. RESULTS: Baseline visual acuity was statistically worse in patients referred during the COVID-19 pandemic period (0.87 ± 0.51 logarithm of the minimum angle of resolution (LogMAR)) as compared with both the "2019" (0.67 ± 0.48 LogMAR, p = 0.001) and "2018" (0.69 ± 0.54 LogMAR, p = 0.012) control periods. Data on the visual function after a loading dose of anti-vascular endothelial growth factor (VEGF) was available in a subset of patients (43 subjects in 2020, 45 in 2019 and 46 in 2018, respectively). Mean ± SD best corrected visual acuity (BCVA) at the 1-month follow-up visit after the third anti-VEGF injection was still worse in patients referred during the COVID-19 pandemic (0.82 ± 0.66 LogMAR) as compared with both the "2019" (0.60 ± 0.45 LogMAR, p = 0.021) and "2018" (0.55 ± 0.53 LogMAR, p = 0.001) control periods. On structural optical coherence tomography (OCT), the maximum subretinal hyperreflective material (SHRM) height and width were significantly greater in the COVID-19 pandemic patients. CONCLUSIONS: We demonstrated that patients with newly diagnosed treatment-naïve exudative neovascular AMD referred during the COVID-19 pandemic had worse clinical characteristics at presentation and short-term visual outcomes.

Subthreshold laser treatment for reticular pseudodrusen secondary to age-related macular degeneration.

There is a lack of treatment aimed at the regression of reticular pseudodrusen (RPD) secondary to age-related macular degeneration (AMD). The aim of this prospective, pilot study is to evaluate the safety and short-term efficacy of subthreshold laser treatment (SLT) in patients affected by RPD secondary to dry AMD (dAMD). Twenty eyes of 20 patients (mean age 78.4 ± 6.8 years) with RPD secondary to dAMD were prospectively enrolled. All patients were treated in an extrafoveal area of 1.27 mm2 using end-point management yellow subthreshold laser and followed for 3 months. Best-corrected visual acuity was 0.140 ± 0.09 LogMAR at the baseline and no changes were observed during the follow-up (p = 0.232). No significant worsening was disclosed before and after the treatment analyzing the macular sensitivity of the treated area (p = 0.152). No topical and/or systemic side effects were disclosed during the 3-month follow-up. The distribution among the RPD stages changed after the treatment (p < 0.001). In detail, in the treated area, we observed a significant increase in the number of Stage 1 RPD during the follow-up (p = 0.002), associated with a significant decrease of Stage 3 RPD (p = 0.020). Outer nuclear layer (ONL) thickness analysis showed a significant increase after the treatment associated with RPD regression (p = 0.001). End-point management SLT appears a safe treatment for RPD secondary to dAMD, showing short-term safety outcomes. Our results suggest that SLT could be effective in inducing a RPD regression in terms of RPD stage and ONL thickening.

TriPla Regimen: A new treatment approach for patients with neovascular age-related macular degeneration in the COVID-19 "era".

In the last months, a rapidly increasing number of people have been infected with severe acute respiratory syndrome coronavirus 2, the virus causing coronavirus disease 2019 (COVID-19). Due to the risk of cross-infections, the number of visits and injections was dramatically reduced in the last months, and the time between visits has been rescheduled from every 15 to 45 min, significantly impairing the total number of available visits. Although continuity of care has been allowed, a series of measures to diminish the risk of contamination need to be adopted until the end of this pandemic outbreak, which may persist until the development of an effective vaccine. For these reasons, we have introduced a new treatment regimen that is aimed at reducing the number of in-person visits and achieving continuity of treatment. This regimen is named "Triple and Plan" (TriPla). The main advantage of the TriPla regimen is to reduce the number of visits of patients in comparison to the pro re nata and treat and extend regimen. Using the TriPla regimen, the risk of contamination would be reduced. Furthermore, by reducing the number of scheduled visits, physicians could guarantee an adequate number of examinations for each patient, lengthening the interval between visits, and reducing the risk of cross-infections.

Short-term outcomes of patients with neovascular exudative AMD: the effect of COVID-19 pandemic.

PURPOSE: To estimate the impact of delayed care during the coronavirus disease 2019 (COVID-19) pandemic on the outcomes of patients with neovascular age-related macular degeneration (AMD). METHODS: Consecutive patients with diagnosis of neovascular AMD were consecutively enrolled between March 9, 2020, and June 12, 2020, (during and immediately after the Italian COVID-19 quarantine). During the inclusion (or pandemic) visit (V0), patients received a complete ophthalmologic evaluation, including optical coherence tomography (OCT). Best-corrected visual acuity (BCVA) and OCT findings from the two preceding visits (V-1 and V-2) were compared with data at V0. RESULTS: One-hundred patients (112 eyes) were enrolled in this study. The time interval between following visits was 110.7 ± 37.5 days within V0 and V-1 and 80.8 ± 39.7 days within V-1 and V-2, respectively (P < 0.0001). BCVA was statistically worse at the V0 visit as compared with the immediately preceding (V-1) visit (0.50 ± 0.43 LogMAR and 0.45 ± 0.38 LogMAR at the V0 and V-1 visits, respectively; P = 0.046). On structural OCT, 91 out of 112 (81.2%) neovascular AMD eyes displayed the evidence of exudative disease activity at the V0 visit, while 77 (68.7%) eyes exhibited signs of exudation at the V-1 visit (P = 0.022). No differences in terms of BCVA and OCT findings were detected between the V-1 and V-2 visits. In multiple regression analysis, the difference in BCVA between V0 and V-1 visits was significantly associated with the interval time within these two visits (P = 0.026). CONCLUSION: The COVID-19 pandemic-related postponement in patient care proved to be significantly associated with worse short-term outcomes in these patients.

Impact of COVID-19 on outpatient visits and intravitreal treatments in a referral retina unit: let's be ready for a plausible "rebound effect".

PURPOSE: To quantify the shrinking in outpatient and intravitreal injections' volumes in a tertiary referral retina unit secondary to virus causing coronavirus disease 2019 (COVID-19). METHODS: In this retrospective cross-sectional study, we reviewed the charts of all patients who had a visit at a medical retina referral center during the Italian quarantine (from 9th of March 2020 to 3rd of May 2020). Number and characteristics of these data were compared with data from the same period in 2019 (from 9th of March 2019 to 3rd of May 2019). RESULTS: In the 2019 study period, there were 303 patients attending clinic (150 males, 153 females). In the 2020 study period, patients decreased to 75 (48 males, 27 females; P = 0.022 comparing gender prevalence between the two periods) with an overall reduction of 75.2%. Mean ± SD age was 71.4 ± 14.3 years (range 25-93 years) in the 2019 study period and 66.7 ± 13.1 years (range 32-91 years) in the 2020 study period (P = 0.005). The largest drop in outpatient volume was recorded in AMD patients (- 79.9%). Regarding the intravitreal treatments, there were 1252 injections in the 2019 period and 583 injections in the 2020 period (- 53.6% in injections). The drop in intravitreal treatments was larger in patients with posterior uveitis, retinal vein occlusion, and diabetes (- 85.7%, - 61.9%, and - 59.6%, respectively). CONCLUSION: The volume of outpatient visits and intravitreal injections declined during the COVID-19 quarantine. The short- and long-term impacts are that routine in-person visits and intravitreal injections are expected to increase after the quarantine and, even more, after the pandemic.

Choroidal Rift: A New OCT Finding in Eyes with Central Serous Chorioretinopathy.

Central serous chorioretinopathy (CSC) is a complex and not entirely understood retinal disease. The aim of our research was to describe a novel optical coherence tomography (OCT) finding named "choroidal rift", which may be identified in the choroid of eyes with CSC. We collected data from 357 patients (488 eyes) with CSC who had structural OCT and OCT angiography (OCTA) scans obtained. Choroidal rifts were identified as polygonal (and not round-shaped) hyporeflective lesions without hyperreflective margins. Choroidal rifts had to be characterized by a size superior to that of the largest choroidal vessel. Finally, hyporeflective lesions were graded as choroidal rifts only if these lesions had a main development perpendicular to the retinal pigment epithelium. OCT analysis allowed the identification of choroidal rifts in ten eyes from nine patients, all with chronic CSC, with an estimated prevalence rate of 2.1%. In three out of ten cases with choroidal rifts, these lesions spanned all the choroidal layers. In the remaining cases, choroidal rifts only partially spanned the choroidal thickness. In OCTA, choroidal rifts were characterized by the absence of flow. Combining structural OCT and OCTA information, we hypothesized that choroidal rifts may represent interruptions of the choroidal stroma in correspondence of fragile regions (in between expanded larger-sized choroidal vessels). Choroidal rift represents a novel OCT feature, which may characterize eyes with chronic CSC and may have a role in the development of irreversible chorio-retinal changes.

Spectrally Resolved Fundus Autofluorescence in Healthy Eyes: Repeatability and Topographical Analysis of the Green-Emitting Fluorophores.

The aim of this study was to report normal measurements of green-emitting fluorophores in the macula of healthy young individuals and to assess the repeatability of these quantitative metrics. To do so, healthy young volunteers were imaged twice (7 ± 3 days apart) using a confocal blue-light fundus autofluorescence (FAF) device with a shorter excitation wavelength (peak at 450 nm) and the capability for separately detecting the red and green components of the emission spectrum. The main outcome measure was the percentage of area occupied by green-emitting fluorophores in the macula. In addition, this measure was performed in separate regions providing a topographical assessment in the foveal, parafoveal and perifoveal regions. Furthermore, the level of agreement between repeated measurements was evaluated. Thirty eyes from 30 healthy volunteers were included in this analysis. Mean age was 26.2 ± 2.8 years (median: 25.0 years; range: 23.0-32.0 years). Median (interquartile range-IQR) area occupied by green-emitting fluorophores was 3.6% (1.9-4.7%) in the macular region. In the topographical analysis, this percentage was higher in the foveal area (median = 33.3%, IQR = 21.9-41.2%), as compared with both the parafoveal (median = 5.3%; IQR = 2.4-8.1%; p < 0.0001) and perifoveal (median = 0.5%, IQR = 0.2-0.8%; p < 0.0001) regions. The coefficient of variation (CV; ranging from 1.1% to 1.7% in the analyzed regions) and the intraclass correlation coefficient (ICC; ranging from 0.93 to 0.97) indicated high levels of repeatability. In conclusion, the assessment of green-emitting fluorophores is repeatable. The distribution of these fluorophores is highest in the foveal region. Assuming that flavin adenine dinucleotide (FAD) emits in the green autofluorescence spectrum, this variability could be secondary to an increased quantity of mitochondria in the foveal region.

Optical Coherence Tomography Parameters as Predictors of Treatment Response to Eplerenone in Central Serous Chorioretinopathy.

Purpose: To present data on clinical response to eplerenone over a 1-year period in patients with central serous chorioretinopathy (CSC), and to evaluate optical coherence tomography (OCT) variables as predictors of treatment response at 3- and 12-month follow-up visits. Methods: Patients with acute or chronic CSC treated with eplerenone were retrospectively included. Clinical and imaging characteristics were recorded at baseline and at the 3-month and 12-month follow-up visits. Changes from baseline in quantitative measurements were calculated at each follow-up. Logistic regression analysis was computed to correlate clinical and OCT parameters at baseline with response to treatment at 3 and 12 months of follow-up. Results: A total of 50 eyes of 50 patients were included in the study. Mean ± SD best corrected visual acuity (BCVA) was 0.20 ± 0.14 Logarithm of the Minimum Angle of Resolution (LogMAR) at baseline and significantly improved at both the 3-month (0.12 ± 0.13 LogMAR, p < 0.0001) and 12-month (0.10 ± 0.12 LogMAR, p < 0.0001) follow-up visits. At the 3-month follow-up visit, 25 out of 50 eyes (50.0%) demonstrated macular complete subretinal fluid (SRF) resolution, while 13 eyes (26.0%) showed macular partial SRF resolution, and 12 eyes (24%) had neither partial nor complete macular SRF resolution. Among those patients with macular partial or complete SRF resolution at 3 months and thus not shifted to photodynamic therapy, 36 out of 38 cases had macular complete SRF resolution at the 12-month follow-up visit. There was a significant change from baseline at both follow-up visits in all anatomical OCT parameters (except for reduction in choroidal thickness that did not reach the statistical significance at the 12-month follow-up visit). Several OCT parameters at baseline were independently significant predictors for macular subretinal fluid complete resolution at 3 months, including (i) a thicker subfoveal choroidal thickness; (ii) a smaller subretinal fluid maximum diameter; (iii) a lower number of serous pigment epithelium detachments; and (iv) a lower number of intraretinal hyperreflective foci. Conclusion: Treatment with eplerenone in CSC patients is confirmed to be beneficial for both anatomical and functional outcomes. We identified attractive OCT metrics that could potentially be used as a tool to select patients who might mainly benefit from this treatment.

Spectrum of choroidal neovascularisation associated with dome-shaped macula.

AIMS: To investigate the clinical spectrum of choroidal neovascularisation (CNV) in patients with dome-shaped macula with the aid of multimodal imaging. METHODS: In this cross-sectional, retrospective, observational study, we reviewed charts and imaging studies of 144 eyes of 79 consecutive patients with dome-shaped macula evaluated at a tertiary referral centre, including optical coherence tomography angiography (OCTA), fluorescein angiography (FA) and indocyanine green angiography (ICGA). Three masked graders evaluated the prevalence and type of CNV. Primary outcome measures were the diagnostic agreement between OCTA and FA±ICGA, the multimodal imaging characteristics and prevalence of CNV associated with dome-shaped macula. RESULTS: We identified 30 eyes with dome-shaped macula complicated by CNV with an estimated prevalence of 20.8% (95% CI 14.2 to 27.4). Twenty-two eyes (73%) showed a type 2 CNV while eight eyes (27%) showed a type 1 CNV. Serous macular detachment was present in 39 eyes (27%) and harboured a CNV in five cases (13%). The overall diagnostic agreement between OCTA and FA±ICGA was excellent (k=0.894, p<0.001), but only the use of both techniques allowed the identification of the whole pool of CNVs. CONCLUSION: Eyes with dome-shaped macula may either develop typical myopic CNV (ie, type 2 CNV) or pachychoroid-associated CNV (ie, type 1 CNV), similar to those observed in central serous chorioretinopathy. Accordingly, in case of serous macular detachment complicating dome-shaped macula, the presence of an associated CNV should be considered. When suspecting a CNV associated with dome-shaped macula, the application of both OCTA and FA±ICGA is superior to the use of one technique alone.

Intravitreal avastin for choroidal neovascularization associated with stargardt-like retinal abnormalities in pseudoxanthoma elasticum.

The aim of the study was to describe a patient with pseudoxanthoma elasticum (PXE), showing Stargardt-like retinal abnormalities, who underwent treatment with intravitreal bevacizumab for subfoveal choroidal neovascularization (CNV) of the right eye (RE). A 57-year-old woman with diagnosis of angioid streaks, retinal flecks, and chorioretinal Stargardt-like atrophy due to PXE was referred to our department for sudden decreased vision in her RE (20/160). Upon a complete ophthalmologic examination, including fluorescein angiography (FA), and optical coherence tomography (OCT), the patient was diagnosed with subfoveal CNV of the RE. Owing to the subfoveal localization of the CNV, the patient was submitted to intravitreal bevacizumab injection. At the 1-month follow-up, visual acuity (VA) improved (20/40), and FA and OCT revealed the CNV closure. Twelve months after the treatment, the patient's VA remained stable with no recurrence of active CNV. On the basis of our findings, a single intravitreal bevacizumab injection seems to induce total regression of CNV complicating PXE, in a patient showing Stargardt-like retinal abnormalities. Further investigations are required to confirm our results.

Late-onset Leber hereditary optic neuropathy mimicking Susac's syndrome.

Leber hereditary optic neuropathy (LHON) is a mitochondrial disorder characterized by bilateral painless optic atrophy and blindness. It usually occurs in young men in association with three major mutations in the mitochondrial genome (mtDNA). We report a patient with a history of alcohol abuse who developed at age 63 years visual impairment, sensorineural hearing loss, and memory dysfunction, suggestive of Susac's syndrome. The patient carried the heteroplasmic mt. 11778G>A mutation on the T2e mtDNA haplogroup. It remains unclear if chronic alcohol abuse combined with the mitochondrial genetic background prompted an aged-related neurodegeneration or deferred the onset of the LHON disease.

Very Large Bilateral Lesions Obscuring Diagnosis of Adult-Onset Foveomacular Vitelliform Dystrophy.

Adult-onset foveomacular vitelliform dystrophy (AFVD) is a clinically heterogeneous and pleomorphic disease originally described by Gass as bilateral with symmetrical, solitary, round or oval, slightly elevated, yellowish subretinal lesions, one third to one disc diameter in size, often with a central pigmented spot. A possible AFVD patient showing very large bilateral macular lesions was reported.

Bevacizumab compared with macular laser grid photocoagulation for cystoid macular edema in branch retinal vein occlusion.

INTRODUCTION: To evaluate the outcome of cystoid macular edema treated with intravitreal injections of bevacizumab and macular grid laser photocoagulation (GLP), in patients with perfused branch retinal vein occlusion. METHODS: Thirty eyes of 30 consecutive patients with cystoid macular edema secondary to nonischemic branch retinal vein occlusion were assigned to either GLP group or to intravitreal bevacizumab (IB) group. Complete ophthalmologic examinations were performed just before GLP and IB injection at 1, 3, 6, and 12 months after treatment. Changes in logarithm of minimum angle of resolution (logMAR) best-corrected visual acuity (BCVA), central macular thickness (CMT) shown by optical coherence tomography-3 were evaluated. RESULTS: Baseline BCVA (logMAR) and CMT were, respectively, 0.89 +/- 0.13 and 650 +/- 140 microm for the GLP group, 0.87 +/- 0.16 and 690 +/- 120 microm for the IB group. After the treatment, at 1, 3, 6, and 12 months in the GLP group, BCVA had improved by 0.19, 0.22, 0.21, and 0.20 logMAR, CMT had decreased by 40%, 41.3%, 40.5%, and 42%. In the IB group, BCVA had improved by 0.31, 0.32, 0.30, and 0.31 logMAR and CMT had decreased by 59.5%, 59%, 60%, and 60.3%. The group receiving bevacizumab had better BCVA and lower CMT values at all time points (P < 0.05). CONCLUSION: Intravitreal bevacizumab injection improves BCVA and reduces CMT more than GLP. Intravitreal bevacizumab injection was well tolerated and could be used as primary treatment in patients with cystoid macular edema secondary to perfused branch retinal vein occlusion.