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BackgroundThis study aimed to assess the prevalence and causes of vision loss in sub-Saharan Africa (SSA) in 2015, compared with prior years, and to estimate expected values for 2020.MethodsA systematic review and meta-analysis assessed the prevalence of blindness (presenting distance visual acuity In SSA, age-standardised prevalence of blindness, MSVI and MVI in 2015 were 1.03% (80% uncertainty interval (UI) 0.39–1.81), 3.64% (80% UI 1.71–5.94) and 2.94% (80% UI 1.05–5.34), respectively, for male and 1.08% (80% UI 0.40–1.93), 3.84% (80% UI 1.72–6.37) and 3.06% (80% UI 1.07–5.61) for females, constituting a significant decrease since 2010 for both genders. There were an estimated 4.28 million blind individuals and 17.36 million individuals with MSVI; 101.08 million individuals were estimated to have near vision loss due to presbyopia. Cataract was the most common cause of blindness (40.1%), whereas undercorrected refractive error (URE) (48.5%) was the most common cause of MSVI. Sub-Saharan West Africa had the highest proportion of blindness compared with the other SSA subregions.ConclusionsCataract and URE, two of the major causes of blindness and vision impairment, are reversible with treatment and thus promising targets to alleviate vision impairment in SSA.

Background: To contribute to the WHO initiative, VISION 2020: The Right to Sight, an assessment of global vision impairment in 2020 and temporal change is needed. We aimed to extensively update estimates of global vision loss burden, presenting estimates for 2020, temporal change over three decades between 1990–2020, and forecasts for 2050. Methods: We did a systematic review and meta-analysis of population-based surveys of eye disease from January, 1980, to October, 2018. Only studies with samples representative of the population and with clearly defined visual acuity testing protocols were included. We fitted hierarchical models to estimate 2020 prevalence (with 95% uncertainty intervals [UIs]) of mild vision impairment (presenting visual acuity ≥6/18 and

To investigate demographic and clinical factors influencing the longitudinal changes of retinal pigment epithelium (RPE) dehiscence area after RPE tears, including the presence of RPE tear-associated repair proliferation (TARP), and identify factors associated with TARP development over follow-up.Retrospective, single-center, observational cohort study of patients with a history of macular neovascularization and RPE tear. The area of RPE dehiscence was measured on repeated short-wavelength fundus autofluorescence imaging. Associations between covariates and RPE dehiscence areas were tested with multivariable linear mixed models. Associations between TARP development and clinical variables were investigated with Cox regression models. Factors associated with visual acuity changing rates were explored with linear mixed models.Thirty-seven eyes of 36 patients were included in this study and followed for a median time of 18 months. Tear-associated repair proliferation was identified in 27 eyes (73%). The median time for TARP detection was 112 days; none of the investigated factors was significantly associated with TARP occurrence. The presence of TARP (estimate: -0.042 mm2/month; P = 0.001) and female gender (estimate: -0.035 mm2/month; P = 0.006) were associated with slower rates of RPE dehiscence enlargement over time. Faster rates of visual improvement were observed in eyes with TARP compared with those without TARP (estimate = -0.010 logarithm of the minimum angle of resolution/month if TARP was present; P = 0.008).Retinal pigment epithelium tear repair with TARP and female gender were associated with slower RPE degeneration after RPE tears. The presence of TARP was associated better visual prognosis. Additional research on factors promoting TARP development may have therapeutic and prognostic implications.

Introduction: To investigate choroidal thickness changes related to the clinical activity of inflammatory choroidal neovascularization in punctate inner choroidopathy/multifocal choroiditis as compared to myopic choroidal neovascularization. Materials and methods: Consecutive inflammatory choroidal neovascularization secondary to punctate inner choroidopathy/multifocal choroiditis, and myopic choroidal neovascularization were retrospectively reviewed. By means of enhanced-depth imaging optical coherence tomography, choroidal thickness was assessed at the same location before choroidal neovascularization development, at choroidal neovascularization onset (baseline), and after treatment. Results: Eleven eyes with inflammatory choroidal neovascularization and 11 eyes with myopic choroidal neovascularization were analyzed. Choroidal thickness beneath inflammatory choroidal neovascularization significantly increased at baseline and decreased after therapy (“Sponge sign”), reaching preclinical values. In particular, mean choroidal thickness under inflammatory choroidal neovascularization was 145 ± 85 µm at the preclinical stage, increased to 210 ± 103 µm at baseline (p = 0.006), and decreased to 136 ± 87 µm after treatment (p = 0.017). Conversely, no significant choroidal thickness changes were disclosed in myopic choroidal neovascularization eyes, under any location. Conclusion: Optical coherence tomography–based choroidal thickness evaluation may represent an additional useful tool to monitor inflammatory choroidal neovascularization activity. Moreover, choroidal thickness under choroidal neovascularizations could be used to discriminate the origin of choroidal neovascular membrane, either inflammatory or myopic, in doubtful cases and guide the therapeutic management.

Inherited retinal dystrophies (IRD) are a heterogeneous group of rare chronic disorders caused by genetically determined degeneration of photoreceptors and retinal pigment epithelium cells. Ultra-widefield (UWF) imaging is a useful diagnostic tool for evaluating retinal integrity in IRD, including Stargardt disease, retinitis pigmentosa, cone dystrophies, and Best vitelliform dystrophy. Color or pseudocolor and fundus autofluorescence images obtained with UWF provide previously unavailable information on the retinal periphery, which correlates well with visual field measurement or electroretinogram. Despite unavoidable artifacts of the UWF device, the feasibility of investigations in infants and in patients with poor fixation makes UWF imaging a precious resource in the diagnostic armamentarium for IRD.

To report our five-year experience on vitreoretinal lymphoma (VRL) as a single-center tertiary hospital.The ophthalmic, cytopathology, and onco-hematologic records of patients with VRL consecutivel...

Purpose To describe the case of a 66-year-old man with bilateral cytologically proven vitreoretinal lymphoma who presented with a pseudo-vitelliform macular lesion and multiple retinal pigment epithelium apertures. Methods The patient underwent comprehensive ophthalmologic evaluation, including best-corrected visual acuity, intraocular pressure, anterior segment and fundus examination, and optical coherence tomography, at baseline and during follow-up. Results A new-onset foveal yellow, ill-defined lesion was noticed in the right eye during the follow-up; best-corrected visual acuity was 20/20, and the patient was asymptomatic. The lesion was isoautofluorescent with the surrounding retina. The optical coherence tomography revealed hyperreflective subretinal material, an irregularly thickened retinal pigment epithelium, and a shallow, hyperreflective retinal pigment epithelium detachment with multiple retinal pigment epithelium apertures. After 15 days, the lesion was completely reabsorbed and replaced by outer retinal atrophy and cystoid macular edema; best-corrected visual acuity dropped to 20/100. Conclusion The macular lesion may be a sign of macular infiltration, a case of paraneoplastic cloudy vitelliform submaculopathy, or coexistence of both. Retinal pigment epithelium apertures are not disease-specific and are described in vitreoretinal lymphoma for the first time.

PurposeTo investigate the intraretinal structural and vascular alterations in patients featuring reticular pseudodrusen (RPD), RPD with outer retinal atrophy (ORA), and drusen.DesignObservational cross-sectional study.MethodsClinical practice study including 68 eyes of 57 patients (22 eyes of 17 patients with RPD; 24 eyes of 21 patients with RPD+ORA; 22 eyes of 19 patients with drusen). Each patient underwent spectral-domain optical coherence tomography (OCT) and OCT angiography (OCT-A). Measurement of retinal layers’ thickness was obtained by the automated segmentation protocol of the Spectralis OCT (Heidelberg Eye Explorer V.1.9.10.0). The superficial capillary plexus (SCP) and the deep capillary plexus (DCP) vessel density, as well as the size of the foveal avascular zone were calculated on 3×3 OCT-A. Main outcome was to compare vessel density at the SCP and DCP among the groups and controls.ResultsAt the SCP, the vessel density was lower in RPD and RPD+ORA patients with respect to controls (P=0.02 and P=0.003, respectively). At the DCP, meaningful disparity was found between the study groups and the healthy subjects in the vessel density (PConclusionSignificant retinal vascular loss is a common feature of patients with non-exudative age-related macular degeneration, more pronounced in those featuring RPD and RPD+ORA. It is associated with retinal thinning, localised particularly at the GCL, compared with controls.

Age-related macular degeneration (AMD) is a leading cause of vision loss in patients .50 years old. The hallmark of the disease is represented by the accumulation of extracellular material between retinal pigment epithelium and the inner collagenous layer of Bruch’s membrane, called drusen. Although identified almost 30 years ago, reticular pseudod­rusen (RPD) have been recently recognized as a distinctive phenotype. Unlike drusen, they are located in the subretinal space. RPD are strongly associated with late AMD, especially geographic atrophy, type 2 and 3 choroidal neovascularization, which, in turn, are less common in typical AMD. RPD identification is not straightforward at fundus examination, and their identification should employ at least 2 different imaging modalities. In this narrative review, we embrace all aspects of RPD, including history, epidemiology, histology, imaging, functional test, natural history and therapy.

PURPOSE To describe a case of a 68-year-old man with macular telangiectasia (MacTel) Type 1 in the right eye, showing an increase in capillary ischemia after intravitreal ranibizumab. METHODS The patient underwent complete ophthalmologic evaluation, including best-corrected visual acuity, intraocular pressure, anterior segment and fundus examination, optical coherence tomography (OCT), and OCT angiography at baseline and on each visit. Fluorescein angiography was performed at baseline. The patient was followed up on monthly bases for 22 months. RESULTS The patient presented a best-corrected visual acuity of 20/80 in the right eye and of 20/25 in the left eye at baseline. In the right eye, the fluorescein angiography images showed perifoveal capillary ectasia, late-frames dye leakage, and enlargement of the foveal avascular zone. The OCT showed intraretinal pseudocysts and microaneurysms, and the OCT angiography showed vascular rarefaction, capillary dropout, and capillary ectasia of the superficial plexus. After 16 months of follow-up and four ranibizumab injections, the best-corrected visual acuity was 20/60, and the OCT angiography disclosed a further enlargement of the foveal avascular zone area and increased capillary obliteration in the perifoveal nasal area. CONCLUSION Optical coherence tomography angiography may represent an indispensable diagnostic technique, complementary to traditional imaging, in the evaluation of the effects of anti-vascular endothelial growth factor therapy in patients with MacTel Type 1.

Purpose: To compare efficacy of treat and extend (T&E) versus fixed regimen treatment protocols in neovascular age-related macular degeneration (nAMD). Methods: Randomized clinical trials (RCTs) comparing T&E versus fixed regimen protocols for nAMD were systematically searched. Primary outcome was to compare the mean best corrected visual acuity (BCVA) change in T&E regimen versus fixed regimen. Secondary outcomes were change in the mean optical coherence tomography (OCT) central retinal thickness (CRT) and mean number of injections. Standardized mean difference (SMD) along with 95% confidence intervals (CIs) were calculated. Random-effect models were used for meta-analyses. Results: Four RCTs were included, with a total of 649 and 621 eyes in the T&E and fixed regimen cohort at 12 months, and 267 and 249 eyes at 24 months. Pooled analysis of mean BCVA change included all four RCTs at 12 months and two RCTs at 24 months, showing no difference between the two groups (12-month: SMD = 0.08, 95% CI: −0.20 to 0.35, p = 0.55; 24-month: SMD = 0.04, 95% CI: −0.13 to 0.21, p = 0.64). Pooled analysis of OCT CRT change at 12 months included three studies, showing no difference between the two groups (SMD = 0.03, 95% CI: −0.46 to 0.51, p = 0.91). Pooled analysis of mean injection number included all four RCTs at 12 months and two RCTs at 24 months, showing significant difference between the two groups (12-month: SMD = −1.11, 95% CI: −1.67 to −0.56, p Conclusion: A T&E regimen proved as effective as a fixed dosage regimen throughout a 24-month follow-up and with a lower number of injections.

This study was aimed at reporting a set of complications associated with retinal pigment epithelium (RPE) humps in high myopia. Data included three eyes from three different patients. Complications observed over RPE humps were the development of choroidal neovascularization, active inflammatory lesions of multifocal choroiditis, and simple bleeding. Regular follow-up with appropriate examinations can help to recognize these events and offer the most adequate treatment in a timely manner. [ Ophthalmic Surg Lasers Imaging Retina . 2020;51:119–123.]

Aims To describe the vascular abnormalities in patients affected by Stargardt disease (STGD1) by means of optical coherence tomography angiography (OCT-A). Methods Cross-sectional case series, with the following inclusion criteria: diagnosis of STGD1, clear ocular media, and stable fixation. Patients underwent best-corrected visual acuity (BCVA), biomicroscopy, applanation tonometry, short-wavelength fundus autofluorescence (SW-FAF) (HRA Heidelberg, Germany), 3×3 Swept Source OCT-A (Topcon Corporation, Japan). Foveal avascular zone (FAZ) area was manually outlined and removed from the vessel density analysis (ImageJ). Main outcome was vessel density assessment in the superficial capillary plexus (SCP), in the deep capillary plexus (DCP), and in the choriocapillaris (CC) of patients with STGD1. Results Nineteen patients (36 eyes) were recruited for the study (10 females, 52.6%). Mean age was 33±5.7 years and mean BCVA was 0.6±0.3 logarithm of the minimum angle of resolution. Thirty-six healthy age-matched subjects (one eye for each patient) acted as a control group. Qualitative analysis of OCT-A revealed areas of reduced vascular density in superficial and DCPs. CC showed focal defects partially corresponding to the flecks on SW-FAF imaging. Quantitative analysis of OCT-A disclosed a statistically significant difference in the density of the SCP (0.302±0.062 vs 0.365±0.042; p=0.0002) and the DCP (0.303±0.081 vs 0.399±0.045; p

Purpose To compare the area of Type 1 neovascularization in patients with age-related macular degeneration calculated on spectral domain optical coherence tomography angiography (SD-OCTA), swept-source OCTA, and indocyanine green angiography (ICGA). Methods Cross-sectional study enrolling patients with neovascular age-related macular degeneration with Type 1 neovascularization. During the same visit, ICGA (Spectralis HRA, Heidelberg, Germany), 3 × 3-mm or 6 × 6-mm SD-OCTA (CIRRUS AngioPlex model 5000; Carl Zeiss Meditec, Inc, Dublin, OH), and 3 × 3-mm or 6 × 6-mm swept-source OCTA (Plex Elite 9000; Carl Zeiss Meditec, Inc) were performed. Neovascularization areas were compared among the three instruments. The degree of consistency between measurements was investigated through the two-way mixed intraclass correlation, whereas the intermethod agreement was expressed by the Bland-Altman analysis. Mean difference and 95% confidence intervals are provided. Results Eighteen eyes of 14 white patients (10 females, 83.3%) were included in the study. The neovascularization area measured on ICGA was higher compared to that measured on both SD-OCTA (P = 0.008) and swept-source OCTA (P = 0.008), whereas no differences were found between the two OCTA. Similar results were achieved analyzing 3 × 3-mm and 6 × 6-mm scan separately. Lowest reliability resulted from the ICGA versus SD-OCTA pair (intraclass correlation = 0.786, confidence interval = 0.500-0.915). Spectral domain OCTA and swept-source OCTA exhibited an excellent agreement (mean difference = 0.2). Swept-source OCTA offered qualitatively better images of the neovascularization, compared with SD-OCTA. Conclusion Better visualization of the extent of neovascularization is obtained using SS-OCT or SD-OCT compared with ICGA, which may be influenced by choroidal permeability and dye leakage. Neovascularization area on OCTA may become an objective parameter in the follow-up of age-related macular degeneration patients, along with traditional imaging techniques.

Purpose To report a rare case of choroidal neovascularization (CNV) developed 2 years after successful treatment of ocular syphilis, identified by optical coherence tomography angiography. Methods Case report. Results A 31-year-old man with a history of syphilitic chorioretinitis developed a CNV 2 years after clinical remission of the infection. Structural optical coherence tomography (OCT) and optical coherence tomography angiography were helpful in providing detailed evidence of an extrafoveal CNV in an easy and noninvasive way. In comparison, the identification of CNV on fluorescein angiography was difficult because of the retinal blood barrier breakdown and intense choroidal background fluorescence for diffuse chorioretinal scarring of syphilitic chorioretinitis. The patient underwent 3 intravitreal injections of anti-vascular endothelial growth factor in addition to 25 mg/day of oral prednisone, with the restoration of previous visual acuity. Conclusion Choroidal neovascularization is a rare, but sight-threatening complication of syphilitic chorioretinitis. The combination of different imaging modalities, and in particular optical coherence tomography angiography, allowed reaching a definite diagnosis of CNV. Combined treatment of systemic steroid and intravitreal anti-vascular endothelial growth factor was effective in controlling the CNV and improving the visual outcome.

Purpose: To evaluate the efficacy of intravitreal anti-VEGF injections in choroidal neovascularization (CNV) related to pattern dystrophy-like deposit in pseudoxanthoma elasticum (PXE). Methods: One-year prospective, interventional study. Nine eyes were recruited in the ophthalmology departments of San Raffaele University and University of Barcelona. Each patient underwent best corrected visual acuity (BCVA) measurement on ETDRS chart, slit-lamp biomicroscopy, fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). The protocol included a first anti-VEGF injection, followed by monthly evaluations with re-treatments based on new funduscopic hemorrhages, fluid on OCT or leakage on FA and/or ICGA. Primary outcome measures were the mean BCVA changes. Secondary outcomes included central macular thickness (CMT) variations and the number of injections needed. Results: At month 12, mean BCVA significantly improved from 20/45 to 20/35 Snellen equivalent, with 3 eyes gaining at least 3 ETDRS lines. Mean CMT decreased from 297 ± 22 to 262 ± 13 μm, after 5.5 ± 4.0 injections. No leakage was observed at the end of follow-up. Conclusions: Intravitreal anti-VEGF injections represent an effective treatment for CNV related to pattern dystrophy-like deposit in PXE, with an improvement of BCVA and CMT. Mean injection number is in line with other studies performed in CNV secondary to angioid streaks.

BackgroundTo assess prevalence and causes of vision loss in Central and South Asia.MethodsA systematic review of medical literature assessed the prevalence of blindness (presenting visual acuityResultsIn Central and South Asia combined, age-standardised prevalences of blindness, MSVI and MVI in 2015 were for men and women aged 50+years, 3.72% (80% uncertainty interval (UI): 1.39–6.75) and 4.00% (80% UI: 1.41–7.39), 16.33% (80% UI: 8.55–25.47) and 17.65% (80% UI: 9.00–27.62), 11.70% (80% UI: 4.70–20.32) and 12.25% (80% UI:4.86–21.30), respectively, with a significant decrease in the study period for both gender. In South Asia in 2015, 11.76 million individuals (32.65% of the global blindness figure) were blind and 61.19 million individuals (28.3% of the global total) had MSVI. From 1990 to 2015, cataract (accounting for 36.58% of all cases with blindness in 2015) was the most common cause of blindness, followed by undercorrected refractive error (36.43%), glaucoma (5.81%), age-related macular degeneration (2.44%), corneal diseases (2.43%), diabetic retinopathy (0.16%) and trachoma (0.04%). For MSVI in South Asia 2015, most common causes were undercorrected refractive error (accounting for 66.39% of all cases with MSVI), followed by cataract (23.62%), age-related macular degeneration (1.31%) and glaucoma (1.09%).ConclusionsOne-third of the global blind resided in South Asia in 2015, although the age-standardised prevalence of blindness and MSVI decreased significantly between 1990 and 2015.

PURPOSE. To assess the changes in hyperreflective foci (HF) by means of spectral-domain optical coherence tomography (SD-OCT) in patients undergoing anti-VEGF treatment for subfoveal choroidal neovascularization (CNV) secondary to angioid streaks (AS). METHODS. Fifteen eyes with diagnosis of AS-related CNV (8 males) and 15 control eyes with uncomplicated AS were consecutively recruited. Patients and controls underwent complete ophthalmologic examination and SD-OCT. Patients were subjected to a pro re nata treatment regimen, including monthly examinations and intravitreal aflibercept injection in case of fluid detection on SD-OCT. HF were measured on horizontal scans of the six-line radial SD-OCT, in the fovea and parafovea and the subdivided as retinal or choroidal. Specifically, HF were analyzed at the following time points: baseline, dry on SD-OCT, 1 month before its reactivation, and the time of CNV reactivation. RESULTS. HF numbers resulted higher in all CNV phases with respect to controls, except during inactive phase. Moreover, foveal and parafoveal HF were found significantly increased in active, prereactive, and reactive phases when compared with inactive phase (P < 0.05). A similar trend was detected for choroidal HF. Interestingly, a subanalysis revealed that only foveal choroidal HF are significantly higher in a prereactive phase if compared with an inactive phase (P = 0.03). Our correlational analysis unveiled negative associations between intraretinal HF numbers and logMAR best-corrected visual acuity. CONCLUSIONS. Our findings suggest that HF represent useful markers to monitor CNV activity. Choroidal HF appear already increased in the fovea 1 month before CNV reactivation. Validation of our results might lead to earlier anti-VEGF reinjection and possibly better visual outcomes.

WOS: 000473042100004, PubMed: 30209083, Objective To estimate the prevalence and causes of blindness and vision impairment for distance and near in Latin America and the Caribbean (LAC) in 2015 and to forecast trends to 2020. Methods A meta-analysis from a global systematic review of 283 cross-sectional, population-representative studies from published and unpublished sources from 1980 to 2014 in the Global Vision Database included 17 published and 6 unpublished studies from LAC. Results In 2015, across LAC, age-standardised prevalence was 0.38% in all ages and 1.56% in those over age 50 for blindness; 2.06% in all ages and 7.86% in those over age 50 for moderate and severe vision impairment (MSVI); 1.89% in all ages and 6.93% in those over age 50 for mild vision impairment and 39.59% in all ages and 45.27% in those over 50 for near vision impairment (NVI). In 2015, 117.86 million persons were vision impaired; of those 2.34 million blind, 12.46 million with MSVI, 11.34 million mildly impaired and 91.72 million had NVI. Cataract is the most common cause of blindness. Undercorrected refractive-error is the most common cause of vision impairment. Conclusions These prevalence estimates indicate that one in five persons across LAC had some degree of vision loss in 2015. We predict that from 2015 to 2020, the absolute numbers of persons with vision loss will increase by 12% to 132.33 million, while the all-age age-standardised prevalence will decrease for blindness by 15% and for other distance vision impairment by 8%. All countries need epidemiologic research to establish accurate national estimates and trends. Universal eye health services must be included in universal health coverage reforms to address disparities, fragmentation and segmentation of healthcare, Brien Holden Vision Institute, This study was partially funded by the Brien Holden Vision Institute.

Purpose: The purpose of our study was to describe ultra-widefield (UWF) imaging and optical coherence tomography angiography (OCT-A) findings in affected and fellow eyes of patients with Coats’ disease. Methods: Consecutive patients affected by Coats’ disease were prospectively recruited at the Department of Ophthalmology, San Raffaele Hospital, Milan, Italy in this cross-sectional, observational study. Patients underwent UWF color fundus photographs, UWF green autofluorescence, UWF fluorescein angiography (FA), optical coherence tomography (OCT), with 3 × 3 mm and 6 × 6 mm OCT-A scans of the macula. Images were qualitatively evaluated by two independent operators for the presence of pathology. Results: Eleven patients affected by Coats’ disease (eight males, mean age 17.1 ± 6.7 years). Nine and two patients had a clinical diagnosis of unilateral and bilateral disease, respectively. Five eyes had macular fibrosis. All clinically affected eyes exhibited retinal pathology at UWF imaging with the temporal sector most involved followed by the inferior, nasal, superior and macula. In all eyes with macular fibrosis, OCT-A revealed replacement of the foveal avascular zone with coarse vessels suggestive of vascularized fibrosis and flow void area in the choriocapillaris due to a masking effect; type 3 neovascularization was seen in 75% of cases. Seven out of nine clinically unaffected fellow eyes showed retinal pathology at UWF FA with the temporal quadrant most involved. Conclusion: We demonstrated that Coats’ disease is a highly asymmetric bilateral disease and that UWF imaging is able to identify more retinal pathology than standard fundus imaging, thus guiding proper retinal photocoagulation. OCT-A allowed easy identification of type 3 neovascularization in a proportion of patients with macular fibrosis.

Pathologic myopia is associated with degenerative changes of the globe, especially at the posterior pole. Eyes affected by pathologic myopia have higher odds to undergo posterior segment surgery and, in those eyes, vitreoretinal surgery is challenging. Many practical tips and tricks can make the surgical procedures simpler, significantly preventing sight-threatening intra- and post-operative complications. Moreover, novel surgical techniques and technological advancements (i.e. ad-hoc instrumentation, minimally invasive vitreoretinal surgery, filters, dye staining, intraoperative optical coherence tomography and 3-dimensional surgery) may play role in highly myopic eyes. The aim of the present work is to review practical tips and tricks, novel surgical techniques and technological advancements. Electronic supplementary material The online version of this article (doi:10.1186/s40942-017-0090-y) contains supplementary material, which is available to authorized users.

A 58-year-old man with bilateral pigmented paravenous retinochoroidal atrophy (PPRCA) associated with macular coloboma in the right eye underwent color fundus photography and fundus autofluorescence with the California ultra-widefield retinal imaging system (Optos, Dunfermline, UK), spectral-domain optical coherence tomography (SD-OCT) (Heidelberg Spectralis HRA + OCT; Heidelberg Engineering, Heidelberg, Germany), and en face OCT angiography (OCTA) (AngioPlex, Cirrus HD-OCT 5000; Carl Zeiss Meditec, Dublin, CA). The patient presented with a visual acuity of counting fingers in the right eye and 20/32 in the left eye. Fundus examination and SD-OCT showed typical PPRCA alterations in both eyes and a macular coloboma in the right eye. The OCTA showed relative sparing of the retinal capillary plexuses, with diffuse defects in the choriocapillaris. The authors concluded OCTA imaging of PPRCA suggests more insights of the pathogenesis of this disease, showing that the disease primarily affects the choroidal vascular network, with a relative sparing of the retinal vasculature. [ Ophthalmic Surg Lasers Imaging Retina . 2018;49:381–383.]

PURPOSE:: To quantify the foveal avascular zone area at superficial capillary plexus (SCP) and deep capillary plexus (DCP) and the global, parafoveal, and perifoveal vessel densities at SCP, DCP, and choriocapillaris using optical coherence tomography angiography in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD). METHODS:: Twenty eyes of 20 consecutive patients (10 females, 50%) with AOFVD and 20 eyes of 20 healthy controls presenting at the Department of Ophthalmology of San Raffaele Hospital, Milan, Italy were enrolled. All patients underwent a complete ophthalmic examination, including 3-mm × 3-mm optical coherence tomography angiography centered on the macula. The vessel density was calculated in the three plexuses (i.e., SCP, DCP, and choriocapillaris) by mean of image binarization, and foveal avascular zone area was manually outlined at SCP and DCP with ImageJ software. RESULTS:: In the SCP, patients with AOFVD disclosed a significant reduction of global (P < 0.001), parafoveal (P = 0.0017), and perifoveal (P = 0.0019) vessel density. In the DCP, parafoveal vessel density was higher in patients with AOFVD (P = 0.0026), whereas no significant difference was appreciated for both the global image (P = 0.5) and the perifoveal area (P = 0.5). Patients with AOFVD showed less dense choriocapillaris (P = 0.012) and perifoveal circle (P = 0.0152), whereas no difference was observed in the perifoveal zone (P = 0.07). Foveal avascular zone area was significantly enlarged at the DCP (P = 0.0184), but not at the SCP. CONCLUSION:: Patients with AOFVD have quantitative vascular alterations in all vascular layers.

The management of diabetic retinopathy (DR) has significantly improved over the past decade. New imaging techniques along with more appropriate and tailored treatments have been introduced in an attempt to simplify the diagnostic and therapeutic pathways of patients affected by DR. Although the rate of involvement will increase in the near future, we hope that an improved approach can provide helpful answers to the remaining clinical and therapeutic questions.

To analyze the optical coherence tomography angiography features of eyes affected with angioid streaks (AS) and to evaluate their ability to predict choroidal neovascularization (CNV) activity.Angioid streaks were individuated from a pool of consecutive patients. Eyes with and without CNV were evaluated by multimodal imaging.Thirty-eight eyes of 19 consecutive patients diagnosed with AS were included. Thirty of 38 eyes with CNV and 8 of 38 eyes without CNV were included. In the majority of cases, CNV showed on optical coherence tomography angiography tangled appearance always associated with signs of neovascular inactivity on multimodal imaging (100%-0%, inactive-active, respectively). Choroidal neovascularization cases showing interlacing appearance were often associated with signs of neovascular activity on multimodal imaging (71.4%-28.6%, active-inactive, respectively). Optical coherence tomography angiography revealed a total of 27 AS, of which 20 appeared as a choriocapillary rarefaction, and in 7 AS, optical coherence tomography angiography choriocapillary segmentation revealed an irregular vascular network, possibly representing fibrovascular tissue over the crack-like breaks in Bruch membrane.Optical coherence tomography angiography is a noninvasive tool to detect the presence of CNV secondary to AS and to evaluate CNV activity. Optical coherence tomography angiography is able to add a novel element to the multimodal imaging characterization of AS.

Aims To describe the vascular abnormalities in patients affected by retinitis pigmentosa (RP) by means of optical coherence tomography angiography (OCT-A). Methods Cross-sectional case series; patients with RP presenting at the Medical Retina Service of the Department of Ophthalmology, University Vita-Salute San Raffaele in Milan were recruited. Inclusion criteria were: diagnosis of RP, clear ocular media, adequate pupillary dilation, and stable fixation. Patients underwent best-corrected visual acuity (BCVA), biomicroscopy, short-wavelength fundus autofluorescence (SW-FAF), and 3×3 Swept Source OCT-A. 30 healthy subjects were chosen as controls. The main outcome was identification of abnormalities in density of the superficial capillary plexus (SCP) and deep capillary plexus (DCP), along with abnormalities of the choriocapillaris (CC). Results 16 patients (32 eyes) were recruited (6 females, 37.4%). Mean age was 53±18 years; mean BCVA was 0.5±0.3 LogMAR. Vessel density analysis disclosed a statistical significant difference in the SCP (29.5±6.8 vs 34.1±4.3; p=0.009) and in the DCP (28.7±7.5 vs 35.5±5.7; p=0.001) between the patients and the controls. No difference was found at the level of the CC (51±4.4 vs 51.3±2.2; p=0.716). RP patients showed a bigger foveal avascular zone at the DCP level compared to controls (p

Purpose To investigate the foveal avascular zone (FAZ) by OCT angiography (OCT-A) in patients affected by diabetic retinopathy and its correlation with peripheral retinal ischemic index. Design Observational, cross-sectional study, prospectively designed. Participants Consecutive patients with treatment-naive diabetic retinopathy were prospectively recruited between October 2015 and January 2017. Methods All patients underwent a comprehensive ocular examination including OCT, OCT-A, ultra-widefield (UWF) color fundus images, and UWF fluorescein angiography. Main Outcome Measures Variables analyzed included best-corrected visual acuity (BCVA) expressed in logarithm of the minimal angle of resolution (logMAR); diabetic retinopathy grading; FAZ area at full-thickness (internal limiting membrane to Bruch's membrane) OCT-A angiogram; superficial capillary plexus; deep capillary plexus; ischemic index; and central macular thickness (CMT). Results Twenty-two eyes of 22 patients (11 male, mean age 54.9±15.8 years) were included. Mean FAZ areas at full thickness, superficial plexus, and deep plexus were 0.331±0.137 mm 2 , 0.340±0.140 mm 2 , and 1.028±0.447 mm 2 , respectively. Mean ischemic index was 13.6% (range, 0%–50.2%). A significant correlation was found between ischemic index and FAZ area at both full-thickness (r = 0.60, P = 0.0035) and superficial (r = 0.68, P = 0.0005) layers. Disease severity correlated to ischemic index (r = 0.49, P = 0.0204), and FAZ area at full-thickness (r = 0.53, P = 0.0108) and superficial (r = 0.47, P = 0.0292) plexuses. No significant correlation between ischemic index and FAZ at deep plexus was found. BCVA correlated only to CMT (r = 0.66, P = 0.0008). Conclusions The association between peripheral and macular perfusion found in this study supports the hypothesis that both conditions share a common pathogenic mechanism that leads to capillary nonperfusion.

To describe the vascular changes in patients affected by non-neovascular age-related macular degeneration (AMD), featuring reticular pseudodrusen (RPD), drusen, or both RPD and drusen by means of optical coherence tomography angiography (OCT-A).Cross-sectional observational case series. Patients with non-neovascular AMD presenting at the Medical Retina Service of the Department of Ophthalmology, University Vita-Salute San Raffaele in Milan were recruited. Patients underwent best-corrected visual acuity, biomicroscopy, infrared reflectance, short-wavelength fundus autofluorescence and OCT-A (AngioPlex, CIRRUS HD-OCT 5000, Carl Zeiss Meditech, Dublin, USA). Main outcome was quantification of vessel density, stromal tissue, and vascular/stromal (V/S) ratio at the choriocapillaris (CC), the Sattler and Haller's and the whole choroid layers among different groups of patients with non-neovascular AMD by means of binarised OCT-A scans.45 eyes of 34 patients were enrolled (15 eyes of 11 patients with RPD, group 1; 15 eyes of 11 patients with drusen, group 2; 15 eyes of 12 patients with mixed phenotype, group 3). The CC, the Sattler and Haller's and the whole choroid vessel density were reduced in all groups of patients (p=0.023, p=0.007 and p=0.011 in group 1, group 2 and group 3 for the CC; p=0.021, p=0.037 and p=0.043 in group 1, group 2 and group 3 for the Sattler and Haller's density; p=0.016, p=0.002 and p0.001 in group 1, group 2 and group 3 for the choroidal density), with significantly lower V/S ratios compared with healthy controls.Patients with non-neovascular AMD show significant choroidal vascular depletion and fibrotic replacement, suggesting a possible role in the pathogenesis and progression of the disease.

Inherited retinal dystrophies and cone dysfunction syndromes may show a sharp hyporeflective interruption in the outermost retinal layers on optical coherence tomography (OCT), known as foveal cavitation (FC). The aim of the study was to describe the morpho-functional features of FC in patients affected by retinal dystrophies by means of multimodal imaging. A consecutive series of patients affected by FC were prospectively recruited for the study. Patients underwent short-wavelength (SW) and near-infraRed (NIR) fundus autofluorescence (FAF), spectral domain OCT (SD-OCT), microperimetry (MP), and multifocal electroretinogram (mfERG). Mean size of FC on OCT was correlated with best-corrected visual acuity (BCVA). Overall, 15 patients (30 eyes) were enrolled. Mean age was 38.2 ± 14.5 years (range: 10-60), with nine females (60 %). Mean BCVA was 0.5 ± 0.4 LogMAR. SD-OCT revealed focal loss of outer retinal layers and disruption of inner segment ellipsoid zone. Vertical height of FC (mean 27.77 ± 18.77 μm) was indirectly related to BCVA; complete forms of FC, with total loss of outer OCT bands, showed a poorer visual outcome. The FC size on NIR-FAF turned out to be larger with respect to SD-OCT and SW-FAF. Our data indicate that the presence of FC worsens functional outcome in patients affected by retinal disorders; complete and higher lesions are associated with a worse morpho-functional prognosis in these eyes.

PURPOSE To describe a peculiar flecked-retina phenotype in a young female affected by early-onset retinal dystrophy due to a heterozygous mutation in the cone-rod transcription factor CRX gene. CASE REPORT A 5-year-old girl presented with poor vision and nystagmus from the first month after birth. Opththalmologic examination at baseline revealed an altered foveal reflex, epiretinal membrane, and yellow fleck-like retinal deposits in the mid- and extreme periphery bilaterally that disappeared after 3 years of follow-up. Electoretinogram was non-recordable in both rods and cones components bilaterally. Genomic sequencing identified a heterozygous missense mutation -c.425A > G (Tyr142Cys) in CRX. CONCLUSIONS We identified a novel early-onset retinal dystrophy-related heterozygous CRX mutation associated with early and severe rod and cone dysfunction and regressive flecked-retina appearance on ophthalmoscopy.