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Purpose There is no widely accepted treatment for persistent/chronic central serous chorioretinopathy. The present study aimed to evaluate the efficacy, safety, and factors associated to treatment response to navigated micropulse laser in chorioretinopathy. Methods Retrospective observational case series including consecutive patients presenting with symptomatic persistent and chronic chorioretinopathy. All patients were treated with 5% navigated micropulse laser with the Navilas system (Navilas®, OD-OS GmBH, Teltwo, Germany), by overlying fluorescein angiography, indocyanine green angiography and/or spectral domain-optical coherence tomography images of visible leaking points and/or choroidal hyperpermeability/subretinal fluid to plan the laser treatment. Results Thirty-nine eyes of 36 consecutive patients (29 men and 7 women, with a mean age of 51.87 years) were included. Logarithm of the minimum angle of resolution (LogMar) best-corrected visual acuity increased from 0.39 ± 0.24 at baseline to 0.24 ± 0.22 at 3 months ( p Conclusion Navigated micropulse laser laser treatment was found to be effective and safe for the treatment of chorioretinopathy, with significant improvement in visual and anatomical outcomes, unaccompanied by any adverse event at 3 and 6 months follow-up. Factors associated to subretinal fluid resolution may allow a better selection of likely responders to navigated micropulse laser treatment.

Resume Objectif Etudier l’association entre la DMLA et les parodontites qui sont deux maladies frequentes dans la population âgee, presentant des facteurs de risque de commun. Methodes Etude pilote cas temoin realisee dans un centre d’ophtalmologie specialise dans le diagnostic et le traitement de la DMLA. Nous avons evalue l’etat parodontal chez 43 patients atteints de DMLA et 19 patients non atteints de DMLA. Un examen du fond d’œil et un examen parodontal complet ont ete realises chez tous les participants. Resultats Les patients atteints de DMLA ont un pourcentage de site avec une perte d’attache de 3–4 mm superieure a celle des patients non-DMLA (47 % vs 38 %, respectivement [p = 0,039]). Cependant, aucune difference significative n’a ete observee concernant le diagnostic clinique de parodontite severe. Conclusions Ces resultats suggerent une association entre la DMLA et la perte d’attache caracteristiques des parodontites, et encouragent a la realisation d’autres etudes a plus grandes echelle, necessaires pour soutenir et expliquer l’association potentielle entre ces deux affections.

Purpose To report a spectral-domain (SD) OCT clinical sign, outer foveal microdefect (OFMD), corresponding to a focal disruption of the foveal photoreceptors in association with various macular conditions. Design Retrospective cohort study. Participants Forty-five patients with OFMD. Methods All patients were imaged with color photography and SD OCT, and some were imaged with autofluorescence imaging, fluorescein angiography, indocyanine green angiography, or a combination thereof. Patient demographics, OFMD imaging, courses, and outcomes were analyzed retrospectively. Main Outcome Measures Demographics, conditions associated with OFMD, and diameter of OFMD. Results Fifty-one eyes of 45 patients (15 men and 30 women; age range, 10–88 years) were included. Symptoms included central scotoma, metamorphopsia, and mild to moderate visual loss. Outer foveal microdefects were observed in association with various macular conditions: presumed posterior vitreous detachment, vitreomacular interface changes, or both (24 eyes); sequelae of macular edema (3 eyes); blunt trauma (2 eyes); retinal phototoxicity resulting from laser or solar maculopathy (5 eyes); and macular telangiectasia type 2 (2 eyes). An improvement with a reduction in OFMD diameter was documented in 9 of 14 eyes that could be followed up. Conclusions We suggest renaming the condition outer foveal microdefect instead of macular microhole, which is usually associated with a vitreomacular disorder. The pattern indeed also was observed in traumatic or degenerative conditions. We assumed that various injuries led to changes in foveal morphologic features and that a full recovery of the foveal architecture could be altered, especially in eyes with findings suggestive of the pachychoroid spectrum of disorders.

Presumed solitary circumscribed retinal astrocytic proliferation (pSCRAP) is a rare, benign retinal tumor, typically presenting as a yellow-white, well-defined lesion. It was first qualified as astrocytic, but thanks to the development of optical coherence tomography (OCT), the lesion was identified as a deep glial lesion, as the nerve fiber layer appeared to be spared. Herein, the authors report the case of a 90-year-old man with inferopapillary pSCRAP who benefited from swept-source OCT angiography images. [ Ophthalmic Surg Lasers Imaging Retina. 2021;52:232–235.]

To report a case of peripapillary subretinal fluid associated with a ridge-shaped morphology surrounding the optic disc, which we termed ridge-shaped peripapilla.Case report.A 6-year old girl with mild to moderate myopia was referred for an abnormal fundus appearance of the left eye. Fundus examination of the left eye showed a vertical whitish elevation just temporal to the disc with pigment clumping. Spectral domain optical coherence tomography (OCT) of the left eye showed an elevation of the fundus at the temporal edge of the disc with thinning of the choroid overlying the thickened scleral protrusion and a serous subretinal fluid. Fluorescein angiography (FA) of the left eye showed a hyperfluorescent area without leakage at the temporal edge of the disc, indicative of retinal pigment epithelium (RPE) atrophy. There was no sign of choroidal neovascularization. Based on the FA and OCT findings, the protrusion of the sclera seemed to result in overlying choroidal thinning with choroidal blood flow disturbances, and consequent RPE atrophy, leading to the subretinal fluid.This case highlights an unusual presentation of ridge-shaped peripapilla, characterized by inward convexity of the peripapillary area with a ridge-shaped morphology and localized thickening of the peripapillary sclera, in eyes with myopia.

Purpose To establish a process to evaluate and standardize a state-of-the-art nomenclature for reporting neovascular age-related macular degeneration (AMD) data. Design Consensus meeting. Participants An international panel of retina specialists, imaging and image reading center experts, and ocular pathologists. Methods During several meetings organized under the auspices of the Macula Society, an international study group discussed and codified a set nomenclature framework for classifying the subtypes of neovascular AMD and associated lesion components. Main Outcome Measures A consensus classification of neovascular AMD. Results The study group created a standardized working definition of AMD. The components of neovascular AMD were defined and subclassified. Disease consequences of macular neovascularization were delineated. Conclusions The framework of a consensus nomenclature system, a definition of AMD, and a delineation of the subtypes of neovascular AMD were developed. Establishing a uniform set of definitions will facilitate comparison of diverse patient groups and different studies. The framework presented is modified and updated readily, processes that are anticipated to occur on a periodic basis. The study group suggests that the consensus standards outlined in this article be used in future reported studies of neovascular AMD and clinical practice.

Purpose To report intraretinal hyperreflective lines related to various macular conditions. Methods All cases were imaged with color photographs, autofluorescence images, and spectral-domain optical coherence tomography, some with fluorescein and/or indocyanine green angiography. Demographic data, imaging, course and outcome were retrospectively analyzed. Results Forty-nine eyes of 43 patients (16 men and 27 women) were included. Hyperreflective vertical lines (38 eyes) or curvilinear lines along the Henle fiber layer (11 eyes) were present in association with various macular conditions: adult vitelliform dystrophy or pattern dystrophy (24 eyes) frequently associated with an epiretinal membrane (six eyes) and/or thick choroid (nine eyes), age-related maculopathy or macular degeneration (nine eyes), partial resorption of subretinal or intraretinal hemorrhages (five eyes), idiopathic macular microhole (two eyes), vitreomacular traction (three eyes), multiple evanescent white dot syndrome (three eyes), fundus flavimaculatus (two eyes), and pachychoroid pigment epitheliopathy (one eye). The lines fully vanished in cases of hemorrhages, multiple evanescent white dot syndrome or resolution of vitreomacular traction, but usually persisted with gradual thinning in the other conditions. Conclusion The present series showed that intraretinal hyperreflective lines could occur in various inflammatory, degenerative, or tractional conditions. They could reflect a previously unrecognized reaction to various photoreceptor, Muller cell, and/or retinal pigment epithelium damage.

To quantitatively analyze characteristics of choriocapillaris flow using spectral domain optical coherence tomography angiography (SD-OCTA) in eyes with chronic central serous chorioretinopathy (CSC) before and after treatment by photodynamic therapy (PDT).Retrospective interventional study. Macular 3X3 SD-OCT scans were analyzed in eyes diagnosed with chronic CSC before and after treatment with half-fluence PDT. The choriocapillaris en face slabs were extracted from the SD-OCTA device after manual segmentation. En face choriocapillaris flow images were compensated with en face choriocapillaris structure images. Phansalkar local thresholding method was then used with a radius of 4 and 8 pixels. Percentage of flow deficits (FD%), number, size, and total area of FDs were computed for comparison, before and after treatment by half-fluence PDT.Mean choriocapillaris FD% before PDT was of 58.36 + / - 11.88 and of 60.82 + / - 11.08 after PDT using radius 4 pixels with no significant difference (p = 0.140). Mean choriocapillaris FD% was of 58.63 + / - 11.08 before PDT and of 60.87 + / - 10.36 after PDT using radius 8 pixels with no significant difference (p = 0.200). Similarly, no significative difference was found in number, size, and total area of FDs, before and after half-fluence PDT, using radius 4 and 8 pixels in patients with chronic CSC.Using Phansalkar local thresholding method, quantitative analysis of choriocapillaris with SD-OCTA found no significant change in choriocapillaris flow deficits before and after successful half-fluence PDT in patients with chronic CSC. Therefore, it seems that half-fluence PDT may not alter choriocapillaris perfusion, at least on a relative short-term basis.

BACKGROUND AND PURPOSE To the best of our knowledge, there is no study of patients with central serous chorioretinopathy associated with chorioretinal folds, since a short mention in Gass' stereoscopic atlas. We report here six cases with this association. METHODS Six patients with both conditions were examined in our institution and underwent fluorescein angiography and optical coherence tomography. RESULTS Patients were 3 men and 3 women, aged 44 years to 82 years. All patients were hyperopic and two received corticosteroids. Fluorescein angiography showed pigmentary changes, diffuse leakage areas typical of chronic central serous chorioretinopathy, and chorioretinal folds mainly located in the upper temporal part of the fundus. Enhanced depth imaging optical coherence tomography was performed in 5 cases and revealed a thick choroid in all cases (mean subfoveal choroidal thickness: 381 µm, range: 280-510 µm). CONCLUSION Although possibly coincidental, the presence of chorioretinal folds in hyperopic central serous chorioretinopathy eyes could be due to the excessive thickness of the choroid in eyes with hyperopia related to short axial length.

In a prospective study, spectral domain optical coherence tomography and fluorescein angiography, combined with color fundus photograph used as a first diagnostic test in patients aged 50 years or older, had a similar sensitivity and specificity for the diagnosis of choroidal neovascularization in age-related macular degeneration., Purpose: To determine the sensitivity and specificity of different retinal imaging combinations for the diagnosis of choroidal neovascularization (CNV) in age-related macular degeneration. Methods: Patients aged 50 years or older referred for suspicious recent-onset CNV related to age-related macular degeneration were prospectively included for 6 months. Data recorded included color fundus photographs (CFPs), spectral domain optical coherence tomography (SD-OCT), and fluorescein angiography (FA) images. Five retina specialists randomly interpreted SD-OCT combined with CFP, and then FA combined with CFP. The reference diagnosis of CNV was based on the agreement of two readers in the interpretation of the SD-OCT + FA + CFP combination. Results: One hundred and forty-eight patients (148 eyes) were included. For the diagnosis of CNV, the sensitivity of both SD-OCT + CFP and FA + CFP was of 90.9%. Type 2 CNV was diagnosed in 98% to 100% of cases with SD-OCT + CFP or FA + CFP, whereas Type 1 CNV was diagnosed in 82.9% of cases with SD-OCT + CFP and 81.6% with FA + CFP. Conclusion: When used as a first diagnostic test, SD-OCT combined with CFP had sensitivity and specificity similar to those of FA combined with CFP, for the diagnosis of CNV in age-related macular degeneration. This shows the increasingly important role of SD-OCT as a first-line test in the diagnosis of CNV.

Introduction: To evaluate the effects of the Navilas system guided by optical coherence tomography angiography for advanced macular neovascularization (MNV) secondary to age-related macular degeneration (AMD). Methods: Prospective case-series including nine eyes presenting with advanced MNV with persistence of exudative signs, no longer responding to anti-VEGF therapy, best-corrected visual acuity at least of 1.3 logMar. All patients were treated with Navilas guided by overlaid optical coherence tomography angiography (OCTA) images at the site of branching large neovascular trunks. Results: Occlusion of large neovascular trunks successfully occurred in all nine included patients. OCTA analysis revealed, at 1 month follow up, MNV total area decreasing from 6.2 ± 3.1 to 2.6 ± 3.4 mm2. At 6 months follow up, mean MNV area was 3.3 ± 3.4 mm2 ( p = 0.008). Conclusion: This preliminary study showed that Navilas treatment guided by OCTA may represent an attractive therapeutic option in advanced neovascular lesions secondary to AMD.

The aim of this study was to report unusual progression of type 2 macular neovascularization (MNV) associated with age-related macular degeneration (AMD), high myopia or angioid streaks. Retrospective multicentric observational case series data were used. Eyes that progressed from type 2 MNV secondary to AMD, high myopia or angioid streaks to fibrovascular pigment epithelial detachment (PED) were included. A total of 29 treatment-naive eyes from 29 patients with type 2 MNV secondary to AMD (n = 14), high myopia (n = 10) or angioid streaks (n = 5) that progressed to a fibrovascular PED on Spectral Domain-Optical Coherence Tomography were used. This progression occurred within 3 months after anti-VEGF therapy initiation. Logarithm of minimum angle of resolution (LogMAR) visual acuity improved significantly after anti-VEGF therapy, from 0.55 (SD ± 0.30) (20/63–20/80) at baseline to 0.30 (20/40) at 3 months, and 0.33 (20/40) at the final follow-up (mean follow up: 3.68 years). Mean number of intravitreal injections per year for patients with a total follow-up ≥ 12 months (n = 24) was 4.3 ± 2.1 per year. Progression from type 2 MNV to a fibrovascular PED may occur in patients suffering from AMD, high myopia or angioid streaks. This progression appears early after initiation of anti-VEGF therapy and is associated with a favorable visual and anatomical outcome, at least on a short follow up basis.

Purpose To report a case of bilateral idiopathic chorioretinal folds that seemed to be related to an atypical staphyloma. Observations A 49-year old man without medical history consulted for slight vision loss and metamorphopsia in the left eye. The ophthalmologic examination revealed moderate myopia and bilateral chorioretinal folds in the posterior pole, confirmed by multimodal imaging. Orbital and systemic examinations ruled out all the known etiologies of chorioretinal folds. 3-D optical coherence tomography and B-scan suggested that the folds were related to an atypical staphyloma that developed in the temporal part of the fundus, while sparing the peripapillary area. The peripapillary area, spared by the staphyloma process, appeared as a “dome-shaped disc” compared to the staphylomatous area. Conclusion and importance This case suggests that myopic patients with unusual staphyloma located outside the peripapillary area could develop chorioretinal folds.

Tilted disc syndrome (TDS) is considered a congenital anomaly due to a delayed closure of the embryonic fissure. It is characterized by an oblique orientation of the axis of the optic disc, associated with other posterior pole anomalies such as inferior crescent, situs inversus and inferior staphyloma. The aim of this review was to summarize the data supporting the current hypotheses for the pathogenesis of TDS, and its anatomical and functional clinical consequences. Recent imaging techniques, such as magnetic resonance imaging, wide-field fundus imaging, and 2- and 3-D optical coherence tomography have provided a new perspective on TDS and its complications. Different abnormalities have previously been reported, both in the anterior and posterior segments. The focus was on vision-threatening chorioretinal changes or complications, including choroidal neovascularization and serous retinal detachments and their therapeutic options. Based on clinical observations, assumptions were proposed to understand the occurrence of complications such as chorioretinal degenerative changes, choroidal neovascularization and polypoidal choroidal vasculopathy, macular serous retinal detachment, myopic foveoschisis and chorioretinal folds. These hypotheses could be referred to as the curvature "breaking point" hypothesis, the uneven growth "tractional" hypothesis, the "container-content" imbalance hypothesis, and the "choroidal funnel" hypothesis. Because these complications could also occur in other contexts, understanding the pathogenesis of TDS complications could help to understand their pathophysiology.

Resume Objectif Mettre a jour les recommandations de la Federation France Macula (FFM) pour le diagnostic de la degenerescence maculaire liee a l’âge (DMLA) exsudative. Methode Analyse de la litterature et avis d’experts. Resultats La FFM recommande d’aboutir au diagnostic de la DMLA exsudative par l’association des donnees de l’examen du fond d’œil (ou de retinographies couleur ou monochromatiques), de la tomographie a coherence optique (OCT), montrant des manifestations exsudatives et par un examen morphologique qui peut etre non invasif (OCT-angiographie) ou invasif (angiographie a la fluoresceine ou au vert d’indocyanine). Dans les conditions optimales ou tous ces examens sont praticables, elle suggere de debuter par les examens non invasifs en premiere ligne et de recourir aux angiographies avec colorant en cas de doute diagnostic residuel. Conclusion Le diagnostic de la DMLA exsudative suit l’evolution actuelle de l’imagerie medicale, qui privilegie les examens non invasifs et reserve les autres examens aux cas de diagnostic plus difficile.

Diabetic macular edema (DME) frequently presents bilaterally. In case of bilateral retinal disease, the visual impairment (VI) and the visual acuity (VA) are strongly correlated to the better eye. The aim of this study was to assess the impact of ranibizumab intravitreal injections (IVR) on VI in patients with simultaneous VA loss due to DME. This was a retrospective two-center study including consecutive DME patients with visual loss treated with ranibizumab since November 2011 and with a minimum follow-up of 6 months. Patients with bilateral visual decrease from DME undergoing IVR within 6 months of each other were included. Twenty-nine DME patients who received bilateral IVR within a 6-month interval in the second eye were included. At baseline, 82.8% (n = 24) of patients had a VA

Identify predictors for response to anti-vascular endothelial growth factor (VEGF) therapy in patients with neovascular (wet) age-related macular degeneration (nAMD).Retrospective, post hoc analysis of VIEW 1/2. Patients were randomized 1:1:1:1 to 0.5 mg intravitreal aflibercept (IVT-AFL) injection every 4 weeks (0.5q4); 2 mg IVT-AFL every 4 weeks (2q4); 2 mg IVT-AFL every 8 weeks (2q8) after an initial three injections at weeks 0, 4 and 8 or 0.5 mg intravitreal ranibizumab every 4 weeks (0.5q4).1815 patients [IVT-AFL 2q4 (n = 613); IVT-AFL 2q8 (n = 607); ranibizumab 0.5q4 (n = 595)] were included. Baseline demographics/characteristics were evenly balanced. Younger age (49-69 years), lower visual acuity (VA) [10.0-≤45.0 Early Treatment Diabetic Retinopathy Study (ETDRS) letters] and smaller choroidal neovascularization (CNV) size [0.0-≤3.1 disc areas (DA)] at baseline were associated with the most vision gain (≥15 letters) over 52 weeks (all nominal p 0.0001).Younger age, higher baseline VA (64.0-≤83.0 letters) and smaller CNV size were associated with a VA ≥20/40 at week 52. Predominantly classic CNV at baseline (nominal p = 0.0007), older age (≥90 years), lower baseline VA (10.0-≤ 45.0 ETDRS letters) and larger CNV size (10.1-≤32.6 DA) were all associated with a VA ≤20/200 at week 52 (all nominal p 0.0001). Along with treatment (nominal p 0.0001), lower VA (p = 0.0166) and smaller central retinal thickness (both nominal p = 0.0190) were predictors for dry retina development.Younger age, lower VA and smaller CNV size at baseline were all associated with greater vision gains over 52 weeks while younger age, higher VA and smaller CNV size at treatment start were more likely to achieve best-corrected VA 20/40 or better after a year's treatment, suggesting the benefit of early anti-VEGF treatment.

A review of the literature from 2014 to 2016 was conducted, focusing on the results of optical coherence tomography angiography in different chorioretinal diseases. In only 3 years, optical coherence tomography angiography has been shown to be an effective tool for diagnosing choroidal neovascularization complicating age-related macular degeneration, pathologic myopia, and inflammatory conditions. The technique has sometimes been considered superior to conventional multimodal imaging, for example, in choroidal neovascularization associated with chronic central serous chorioretinopathy or multifocal choroiditis. In retinal vascular diseases, optical coherence tomography angiography has helped to understand the condition described as paracentral acute middle maculopathy and has been considered highly effective for the analysis of retinal vascular macular changes secondary to retinal vein occlusion or macular telangiectasia. Changes in the foveal avascular zone, also reported in diabetic maculopathy, have been shown to occur before any angiographic signs. A reduction in capillary vascular density has been reported in the fovea of eyes with malignant melanoma, but not in eyes with choroidal nevus. However, optical coherence tomography angiography is a recent technique that probably needs refinements and further studies. Nevertheless, the first 3 years of optical coherence tomography angiography use suggest its clinical relevance and useful applications in daily clinical practice.

Purpose: To describe atypical cases of multiple evanescent white dot syndrome (MEWDS) associated with foveal exudation, increased choroidal thickness, and secondary Type 2 (subretinal) neovascularization. Methods: Four cases of atypical MEWDS were studied at a retina referral center. Patients underwent evaluation with multimodal retinal imaging, including fluorescein angiography, indocyanine green angiography, spectral-domain and enhanced depth imaging optical coherence tomography (OCT). Two patients were imaged with OCT angiography. Results: Four patients (3 female, 1 male) with a median age of 23.5 years presented with acute onset, painless, decreased central vision. All cases demonstrated fundus findings consistent with MEWDS on color photography, indocyanine green angiography, fluorescein angiography, fundus autofluorescence, and structural OCT imaging. On structural OCT, all 4 patients were noted to have hyperreflective subretinal material and increased subfoveal choroidal thickness ranging from 307 mm to 515 mm. Type 2 neovascularization was diagnosed in all four patients using fluorescein angiography, indocyanine green angiography, and/or OCT angiography. Two patients had poor visual acuity at the last follow-up despite resolution of characteristic clinical findings of MEWDS. Conclusion: A subset of patients with atypical MEWDS may develop persistent poor vision due to subfoveal exudation and secondary Type 2 neovascularization. Patients showing increased choroidal thickness at presentation may be more susceptible to this unusual presentation.

To investigate the changes in imaging tool practice for the diagnosis of neovascular age-related macular degeneration (nAMD). Retrospective analysis of consecutive patients diagnosed with nAMD in a tertiary care center, over a 6-month period in 2014, 2016, and 2018. Patient demographics were compared. Imaging modalities used in 2014 were fundus photography, fluorescein angiography (FA), and structural spectral-domain optical coherence tomography (SD-OCT), while OCT-angiography (OCT-A) was available from 2015. Imaging tools used in our practice were compared in the 3 cohorts. The 3 cohorts included 163, 99, and 167 patients, respectively. There was no difference in age or gender (mean age 81.7 years). OCT-A images were analyzable in 60.5% and 89.7% of patients respectively in 2016 and in 2018. In the 3 cohorts, all patients were imaged with fundus photography and structural OCT. FA was performed in 70.2, 28.8, and 22.1% of patients, respectively. This study showed a shift in practice of imaging tools used for the diagnosis of nAMD, non-invasive tools being increasingly used as the first-line imaging, and FA as the second-line imaging.

ObjectiveTo evaluate 12-month outcomes in treatment-naïve patients with neovascular (wet) age-related macular degeneration (AMD) stratified by intravitreal aflibercept (IVT-AFL) regimen.Methods and analysisPatients included in the 12-month interim analysis of Real life of intravitreal Aflibercept In FraNce: oBservatiOnal Study in Wet AMD (RAINBOW), a 4-year, ongoing observational study conducted in France, were stratified by IVT-AFL dosing regimen. Safety (n=593) and effectiveness (n=428) data were analysed. Regimens included a regular cohort (three initial monthly IVT-AFL injections and ≥6 injections) and irregular cohorts (ResultsMean number of IVT-AFL injections was 6.0 (all patients, n=513), 7.2 (regular cohort, n=102), 6.1 (irregular cohort with three initial monthly injections, n=266) and 5.2 (irregular cohort without three initial monthly injections, n=60). Overall mean gain in BCVA at 12 months was 5 letters; +7.1 letters (regular cohort) and +5.6 letters (irregular cohort with three initial monthly injections), both pConclusionTreatment-naïve patients with neovascular AMD receiving three initial monthly injections followed by regular or irregular injections over 12 months experienced better visual acuity outcomes than those receiving irregular treatment without three initial monthly injections.Trial registration numberNCT02279537.

To establish a process to evaluate and standardize a state-of-the-art nomenclature for reporting neovascular age-related macular degeneration (AMD) data.Consensus meeting.An international panel of retina specialists, imaging and image reading center experts, and ocular pathologists.During several meetings organized under the auspices of the Macula Society, an international study group discussed and codified a set nomenclature framework for classifying the subtypes of neovascular AMD and associated lesion components.A consensus classification of neovascular AMD.The study group created a standardized working definition of AMD. The components of neovascular AMD were defined and subclassified. Disease consequences of macular neovascularization were delineated.The framework of a consensus nomenclature system, a definition of AMD, and a delineation of the subtypes of neovascular AMD were developed. Establishing a uniform set of definitions will facilitate comparison of diverse patient groups and different studies. The framework presented is modified and updated readily, processes that are anticipated to occur on a periodic basis. The study group suggests that the consensus standards outlined in this article be used in future reported studies of neovascular AMD and clinical practice.

Purpose: To evaluate the efficacy of switching from intravitreal ranibizumab to intravitreal aflibercept in choroidal neovascularization secondary to angioid streaks. Design: Multicenter retrospective interventional case series. Methods: Patients previously treated with intravitreal ranibizumab with at least 12-month follow-up (M12) after switching (M0) to intravitreal aflibercept. Switch to intravitreal aflibercept was decided in cases of refractory or recurrent choroidal neovascularization. Primary endpoint: Change of best-corrected visual acuity using the Early Treatment Diabetic Retinopathy Study letters. Secondary endpoints: Mean change of central macular thickness, absence of intraretinal/subretinal fluid on spectral domain optical coherence tomography and the percentage of eyes with absence of leakage on fluorescein angiography. Results: Fourteen eyes of 13 patients were included. Mean best-corrected visual acuity was 65.0 ± 21.03 letters at M0 and 63.5 ± 17.30 letters at M12 (p = 0.5). Secondary endpoints: Mean central macular thickness was 344 ± 194.65 µm at M0 and 268 ± 79.97 µm at M12 (p = 0.008). Absence of intraretinal/subretinal fluid was observed in 71%. Fluorescein angiography (nine eyes) showed absence of leakage in 77% (seven eyes). Conclusion: Switching from intravitreal ranibizumab to intravitreal aflibercept represents a therapeutic option in patients with refractory or recurrent choroidal neovascularization secondary to angioid streaks.

Pathologic myopia is a major cause of visual impairment worldwide. Pathologic myopia is distinctly different from high myopia. High myopia is a high degree of myopic refractive error, whereas pathologic myopia is defined by a presence of typical complications in the fundus (posterior staphyloma or myopic maculopathy equal to or more serious than diffuse choroidal atrophy). Pathologic myopia often occurs in eyes with high myopia, however its complications especially posterior staphyloma can also occur in eyes without high myopia. Owing to a recent advance in ocular imaging, an objective and accurate diagnosis of pathologic myopia has become possible. Especially, optical coherence tomography has revealed novel lesions like dome-shaped macula and myopic traction maculopathy. Wide-field optical coherence tomography has succeeded in visualizing the entire extent of large staphylomas. The effectiveness of new therapies for complications have been shown, such as anti-VEGF therapies for myopic macular neovascularization and vitreoretinal surgery for myopic traction maculopathy. Myopia, especially childhood myopia, has been increasing rapidly in the world. In parallel with an increase in myopia, the prevalence of high myopia has also been increasing. However, it remains unclear whether or not pathologic myopia will increase in parallel with an increase of myopia itself. In addition, it has remained unclear whether genes responsible for pathologic myopia are the same as those for myopia in general, or whether pathologic myopia is genetically different from other myopia.

Background: To report the use of En-face optical coherence tomography (OCT) in a patient treated with docetaxel and tamoxifen for breast cancer for the detection of macular edema (ME) without evidence of leakage on fluorescein angiography (FA). Case Presentation: A 52-year-old woman treated for breast cancer presented with bilateral visual loss for 2 months. FA showed no significant leakage while spectral-domain OCT scans of both eyes showed foveolar and parafoveolar cystic spaces in a moderately thickened macula. En-face OCT segmented at the inner retina showed the petaloid arrangement of cystic cavities, comparable to a cystoid ME. Conclusions: The combined use of tamoxifen could have potentiated the toxic effect of docetaxel on the macula. En-face OCT images may reveal a petaloid aspect of the macula due to cysts in the inner retina segmentation, when FA shows no leakage.

PURPOSE To assess the influence of tilted disk syndrome (TDS) and inferior staphyloma on the macular clinical expression of MFC. METHODS Medical charts of patients with MFC examined since 2009 were reviewed retrospectively. All patients underwent standard examination and multimodal imaging including fundus color photography, fundus autofluorescence, and spectral domain optical coherence tomography. Two groups of patients were compared : Group 1 with TDS and inferior staphyloma and Group 2 without. The number of MFC spots were counted and normalized to the surface area in a circle centered about the fovea. In Group 1, the number of lesions inside the staphyloma was compared with that outside the staphyloma. RESULTS At baseline, 8 eyes in Group 1 had a mean (±SD) higher number of chorioretinal lesions outside the staphyloma (14.5 [±7.2]) than within the staphyloma (5 [±5.5]) (P = 0.017). All 11 eyes in Group 2 had chorioretinal lesions homogeneously distributed in the posterior pole. Eyes with TDS and inferior staphyloma developed choroidal neovascularization more frequently (6/8 eyes) than eyes without TDS (5/11 eyes) (P = 0.026). CONCLUSION Inferior staphyloma associated with TDS could modulate the clinical expression of the inflammatory and neovascular process in eyes with MFC.

Introduction: Diabetic retinopathy (DR), hypertensive retinopathy (HR) and retinal vein occlusion (RVO) are among the most common retinal vascular diseases, causing severe vision loss in affected patients worldwide. However the spectrum of retinal vascular diseases comprises a wider, more heterogeneous group of clinical entities, that, though less frequent, can prove to be a diagnostic and therapeutical challenge in a clinical setting.Areas covered: In this review we describe the findings of some uncommon retinal vascular disease using a multimodal approach. In the last 50 years, fluorescein angiography (FA) has been the gold standard for retinal vascular diseases. However, in case of choroidal involvement or masking effect generated by haemorrhage, indocyanine green angiography (ICGA) proved to be very useful. Differently from previous examinations, the newly introduced OCT-Angiography (OCT-A) is less invasive (dyeless) depth-resolved tool and thus may particularly useful for the diagnosis and th...

Purpose To reappraise the autosomal dominant Martinique crinkled retinal pigment epitheliopathy (MCRPE) in light of the knowledge of its associated mutated gene mitogen-activated protein kinase–activated protein kinase 3 ( MAPKAPK3 ), an actor in the p38 mitogen-activated protein kinase pathway. Design Clinical and molecular study. Participants A total of 45 patients from 3 generations belonging to a family originating from Martinique with an autosomal dominant MCRPE were examined. Methods Best-corrected visual acuity, fundus photographs, and spectral-domain optical coherence tomography (SD OCT) of all clinically affected patients and carriers for the causal mutation were reviewed at the initial visit and 4 years later for 10 of them. Histologic retinal lesions of Mapkapk3 −/− mice were compared with those of the human disease. Main Outcome Measures The MCRPE natural history in view of MAPKAPK3 function and Mapkapk3 −/− mouse retinal lesions. Results Eighteen patients had the c.518T>C mutation. One heterozygous woman aged 20 years was asymptomatic with normal fundus and SD OCT (stage 0). All c.518T>C heterozygous patients older than 30 years of age had the characteristic dried-out soil fundus pattern (stages 1 and 2). Complications (stage 3) were observed in 7 cases, including polypoidal choroidal vasculopathy (PCV) and macular fibrosis or atrophy. One patient was homozygous and had a form with severe Bruch's membrane (BM) thickening and macular exudation with a dried-out soil pattern in the peripheral retina. The oldest heterozygous patient, who was legally blind, had peripheral nummular pigmentary changes (stage 4). After 4 years, visual acuity was unchanged in 6 of 10 patients. The dried-out soil elementary lesions radically enlarged in patients with a preferential macular extension and confluence. These findings are in line with the progressive thickening of BM noted with age in the mouse model. During follow-up, there was no occurrence of PCV. Conclusions MCRPE is an autosomal dominant, fully penetrant retinal dystrophy with a preclinical stage, an onset after the age of 30 years, and a preserved visual acuity until occurrence of macular complications. The natural history of MCRPE is in relation to the role of MAPKAPK3 in BM modeling, vascular endothelial growth factor activity, retinal pigment epithelial responses to aging, and oxidative stress.

Purpose To assess the association of clinical and biological factors with extensive macular atrophy with pseudodrusen (EMAP) characterized by bilateral macular atrophy occurring in patients aged 50 to 60 years and a rapid progression to legal blindness within 5 to 10 years. Design A national matched case-control study. Participants Participants were recruited in 10 French Departments of Ophthalmology and their associated clinical investigation centers. All 115 patients with EMAP had symptoms before the age of 55 years due to bilateral extensive macular atrophy with a larger vertical axis and diffuse pseudodrusen. Three controls without age-related macular degeneration (AMD) or retinal disease at fundus examination were matched for each patient with EMAP by gender, age, and geographic area (in total 415). Methods Subjects and controls underwent an eye examination including color, red-free autofluorescent fundus photographs and spectral-domain optical coherence tomography with macular analysis. The interviews collected demographic, lifestyle, family and personal medical history, medications, and biological data. Associations of risk factors were estimated using conditional logistic regression. Main Outcome Measures Extensive macular atrophy with pseudodrusen status (cases vs. controls). Results Extensive macular atrophy with pseudodrusen most frequently affected women (70 women, 45 men). After multivariate adjustment, family history of glaucoma or AMD was strongly associated with EMAP (odds ratio [OR], 2.3, P = 0.008 and OR, 1.5, P = 0.01, respectively). No association was found with cardiac diseases or their risk factors. Mild and moderate kidney disease and higher neutrophil rate were associated with a reduced risk of EMAP (OR, 0.58, P = 0.04; OR, 0.34, P = 0.01; and OR, 0.59, P = 0.003, respectively). On the contrary, eosinophilia (OR, 1.6; P = 0.0002), lymphocytosis (OR, 1.84; P = 0.0002), increased erythrocyte sedimentation rate (OR, 6.5; P = 0.0005), decreased CH50 ( P = 0.001), and high plasma C3 level ( P = 0.023) were significantly associated with a higher risk of EMAP. Conclusions This study documents an association between EMAP and family history of AMD and glaucoma, a clear female predominance, and a systemic inflammatory profile. The reduced CH50 and increased C3 plasma values could reflect a more severe complement pathway dysfunction than in AMD, leading to early pseudodrusen and rapid development of geographic atrophy. There is no association of EMAP with AMD cardiac diseases or cardiac risks, including cigarette smoking.

PURPOSE To analyze the subfoveal choroidal thickness (SFCT) in patients with cuticular drusen. METHODS Retrospective, monocentric, study of consecutive patients examined with enhanced depth imaging spectral domain optical coherence tomography (EDI SD-OCT, Cirrus, Zeiss) between 2009 and 2014 in a tertiary care center. Measurements of the height of the subfoveal vitelliform detachment and SFCT were manually performed. RESULTS Thirteen patients, 3 men and 10 women, aged from 35 to 73 (mean: 53.6 years) were selected. For the 24 eyes without macular atrophy at first visit, SFCT ranged from 195 to 559 µm (mean ± SD = 317.5 ± 93). The SFCT was significantly thicker in 12 eyes with vitelliform macular detachment at presentation (369 ± 96, median = 368.5) than in 12 eyes without (266 ± 58, median = 257.5) (P = 0.007), whereas the 2 groups did not differ in age (P = 0.35) or refractive error (P = 0.56). No correlation was observed between SFCT and the height of the foveal detachment. For 10 eyes followed up longer than 24 months (mean: 38.9 months), the SFCT significantly decreased over time, from 375 ± 96 (median = 368.5) to 303 ± 138 (median = 319) µm (P = 0.022). CONCLUSION Eyes with cuticular drusen combined with vitelliform macular detachment present with choroidal thickening, suggesting that the choroidal vasculature may play a role in the occurrence of macular detachments in patients with cuticular drusen. The life cycle of these vitelliform lesions evolves from translucent subretinal fluid to the accumulation of yellowish material eventually resolving and leading to atrophy with marked and rapid thinning of the choroid.