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Purpose: To determine the prevalence and characteristics of foveal hypoplasia (also called fovea plana) in patients with Best disease using spectral-domain (SD) optical coherence tomography (OCT) and OCT-angiography (OCT-A). Design: A retrospective observational study including patients diagnosed with Best disease. Subjects and Participants: Fifty-nine eyes of thirty-two patients (fifteen females (46.9%) and seventeen males (53.1%), p = 0.9) diagnosed with Best disease were included. Patients’ eyes were categorized into two groups: Eyes with a fovea plana appearance (‘FP group’) and eyes without fovea plana appearance (‘no FP group’), based on the foveal appearance on B-scan SD-OCT. Methods and Main Outcome Measures: Cross-sectional OCT images were assessed for the persistence of inner retinal layers (IRL) and OCT-A was analyzed for the presence of a foveal avascular zone (FAZ), the size of which was determined when applicable. Results: Overall, 16 eyes (27.1%) of 9 patients had a fovea plana appearance (‘FP group’) with the persistence of IRL, and 43 eyes (72.9%) of 23 patients did not have fovea plana appearance (‘no FP group’). Among FP eyes, OCT-A performed in 13 eyes showed bridging vessels through the FAZ in 100% of eyes with OCT-A. Using Thomas classification, 14 out of the 16 eyes with fovea plana (87.5%) had atypical foveal hypoplasia, and the 2 others (12.5%) had a grade 1b fovea plana. Conclusion: In our series, foveal hypoplasia was present in 27.1% of patients with Best disease. OCT-A showed bridging vessels through the FAZ in all eyes. These findings highlight the microvascular changes associated with Best disease, which can be an early sign of the disease in patients with a family history.

The aim of this study was to compare primary versus secondary forms of multiple evanescent white dot syndrome (MEWDS) at T0 (baseline) and T1 (1-4 months after the onset of symptoms).A total of 101 eyes in 100 patients were included in a multicentric retrospective study.Secondary MEWDS was defined as MEWDS associated with underlying chorioretinal inflammatory pathologies, mainly multifocal choroiditis and punctuate inner choroidopathy. Patients with secondary MEWDS were older (P = 0.011). The proportion of women (P = 0.8), spherical equivalent (P = 0.3), and best-corrected visual acuity at T0 (P = 0.2) were not significantly different between the two groups. The area of MEWDS lesions on late-phase indocyanine green angiography was significantly smaller in secondary MEWDS (P = 0.001) and less symmetrical with respect to both horizontal (P = 0.003) and vertical (P = 0.004) axis. At T0, neither the clinical (P = 0.5) nor the multimodal imaging (P = 0.2) inflammation scores were significantly different between the groups. At T1, the multimodal imaging inflammation score was higher in secondary MEWDS (P = 0.021).In secondary MEWDS, outer retinal lesions are less extensive and located close to preexisting chorioretinal lesions. Mild signs of intraocular inflammation on multimodal imaging are more frequent in secondary MEWDS during recovery. These findings suggest that chorioretinal inflammation may trigger secondary MEWDS.

Introduction: The performance of “en-face” optical coherence tomography (OCT) in screening for chloroquine (CQ) or hydroxychloroquine (HCQ) retinopathy has not been largely explored. The aim of this study was to determine the concordance of “en-face” OCT with multifocal electroretinography (mfERG) in screening for CQ/HCQ retinopathy. Methods: This is a prospective cohort study conducted at the Rothschild Foundation Hospital – Paris between August 2016 and February 2021. Patients taking hydroxychloroquine were followed up over 2 consecutive years and received an “en-face” OCT and a mfERG on each visit. Results: A total of 91 patients (182 eyes) were analyzed. mfERG and “en-face” OCT were concordant in 147 eyes (86.3%). Cohen’s Kappa coefficient for concordance between mfERG and “en-face” OCT was considered weak with a value 0.61 (95% CI: 0.50-0.72). The sensitivity and specificity of “en-face” OCT are 70% (95% CI: 59-79%) and 91% (95% CI: 83-96%) respectively, relatively to mfERG. Proportion of abnormal R2/R5 and R3/R5 ratios did not differ between patients with normal and abnormal “en-face” OCT (p=0.2). Discussion/Conclusion: “En-face” OCT and mfERG have low concordance and cannot be used interchangeably as each investigation evaluates a different facet of CQ/HCQ retinopathy. “En-face” OCT could be used as a complement in screening for CQ/HCQ retinal toxicity if the anomalies detected on “en-face” OCT are confirmed by B-scan OCT sections.

Purpose Numerous small hyperreflective dots (HRDs) can be seen within the hyporeflective layer between the ellipsoid zone (EZ) and the interdigitation zone (IZ) on C‐scan spectral‐domain optical coherence tomography (SD‐OCT) with a yet unknown variation under light conditions. The aim of this study was to explore light‐induced SD‐OCT changes in these HRDs. Methods The study subjects were randomly assigned to two groups: Group 1 experienced a dark adaptation protocol followed by intense retinal photobleaching, while Group 2, serving as the control group, was exposed to constant ambient light without any variation. The number of HRDs was automatically counted. Results Twenty healthy volunteers were prospectively included. The number of HRDs differed significantly over time (p = 0.0013). They decreased in Group 1 after dark adaptation and retinal photobleaching before returning to baseline levels 30 min later; conversely, they remained relatively constant in Group 2 throughout the study (p

Purpose. To highlight the advantages of optical coherence tomography angiography (OCTA) in delineating the morphological features of the retinal and choroidal vascular network during acute, relapsing, and quiescent stages of macular toxoplasma retinochoroiditis. Methods. This prospective study included patients presenting with both active and quiescent ocular toxoplasmoses. OCTA was obtained to diagnose and follow the subsequent vascular network changes at diagnosis and six months after acute presentation. Results. Twenty-three eyes of 23 patients were included. In active lesions, OCTA showed extensive, well-delineated areas of intense hyposignal and perifoveal capillary arcade disruption in the parafoveal superficial capillary plexus (pSCP) and less extensive hyposignal in the parafoveal deep capillary plexus (pDCP). Signals of decreased deep capillary density and disorganization were also seen in the choroid. In nonactive lesions, OCTA demonstrated a homogenous and equally attenuated grayish hyposignal of the pSCP and pDCP and a partial restoration of the nonperfused choroidal areas. Conclusion. OCTA is a useful technique for vascular network analysis in toxoplasma retinochoroiditis. It allows the visualization of the different network changes and behaviors during the different stages of the infection.

Objective HCQ is an essential medication in SLE, proven to lengthen survival and reduce flares. Its use, however, is limited by its rare but severe ophthalmological complications. Here, we aimed to analyse factors associated with HCQ retinopathy including HCQ blood levels. Methods This case–control study compared SLE patients with and without HCQ retinopathy, defined by abnormal results for at least two of the following ophthalmological tests: automated visual fields, spectral-domain optical coherence tomography (SD-OCT), multifocal electroretinogram (mfERG) and fundus autofluorescence. We compared clinical and laboratory findings to assess risk factors for HCQ retinopathy. Results The study included 23 patients with confirmed retinopathy (cases) and 547 controls. In the univariate analysis, age (P Conclusion SLE patients on HCQ should be closely monitored for retinopathy, especially those from the West Indies or sub-Saharan Africa, or with renal insufficiency, longer HCQ intake or a high cumulative dose. Although reducing the daily dose of HCQ in patients with persistently high HCQ blood levels seems logical, these concentrations were not associated with retinopathy in this study with controls adherent to treatment.

Purpose The aim of this study is to compare the one year outcome of neovascular age-related macular degeneration (nAMD) patients treated by a PRN regimen of Anti-vascular endothelial growth factor (VEGF) intravitreal injections (IVTs), using optical coherence tomography B-scan (OCT-B) or OCT Angiography (OCT-A) imaging modalities during follow-up. Methods Patients older than 50 years with nAMD currently treated by PRN regimen of Anti-VEGF IVTs were recruited from Rothschild Foundation Hospital – Paris and Centre Ophtalmologique Maison Rouge – Strasbourg and followed-up for a year. Patients were randomized in two groups: one group was followed by OCT-B while the other was followed by OCT-A. Results Thirty-three patients were followed by OCT-A and 31 patients were followed by OCT-B. Twenty-nine patients in the OCT-A group and 27 patients in the OCT-B group attended the last visit. No statistically significant difference was found between the two groups at 1 year concerning: improvement or stabilization of the best corrected distance visual acuity (BCVA) (p > 0.9), exudative signs (p > 0.9), number of injections (p = 0.8) and the delay until the first reinjection was performed (p = 0.5). Conclusion The use of OCT-A or OCT-B as imaging modalities in nAMD treated by a PRN regimen of Anti-VEGF IVTs seem to be comparable at one year.

To report the occurrence of paracentral acute middle maculopathy (PAMM) in giant cell arteritis (GCA), describe its features and outcomes, and identify risk factors associated with PAMM in patients with GCA.Review of medical records of patients with GCA who were examined in the Rothschild Foundation Hospital. Patients were divided into three groups: GCA with PAMM (Group 1), GCA with ophthalmic involvement but without PAMM (Group 2), and GCA without ophthalmic involvement (Group 3). We analyzed the data for age, sex, medical history, laboratory testing, visual acuity, and posterior segment vascular involvement.Among the 96 patients who met the inclusion criteria, 52 had ophthalmic involvement, and 16 patients were included in Group 1 (GCA with PAMM). In this subgroup, the mean age was 81.6 years and was found to be older than other groups. The visual prognosis was similar between Groups 1 and 2. Of the 20 eyes with PAMM, 35% were also associated with homolateral anterior ischemic optic neuropathy. No statistical difference was found in initial symptoms, signs, and laboratory testing.Paracentral acute middle maculopathy is frequently observed lesions in ocular GCA. Patients can present with isolated findings of PAMM as the only indication of GCA. Optical coherence tomography of the macula should be routinely performed in patients with suspected GCA, specifically if they complain of visual changes, to look for signs of ischemia in the middle layers of the retina. Isolated PAMM should raise suspicion for GCA in patients at risk.

The aim of the study is to report on the indocyanine green angiography (ICGA) and OCT findings in patients hospitalized for severe COVID infection. In this observational prospective monocentric cohort study, we included patients hospitalized for severe COVID infection. The main outcomes were ICGA and OCT findings. A total of 14 patients with a mean age of 58.2 ± 11.4 years and a male predominance (9/14 patients, 64%) were included. The main ICGA findings included hypofluorescent spots in 19 eyes (68%), intervortex shunts in 10 eyes (36%), and characteristic “hemangioma-like” lesions in five eyes (18%). “Hemangioma-like” lesions were both unique and unilateral, and showed no washout on the late phase of the angiogram. The main OCT findings included focal choroidal thickening in seven eyes (25%), caverns in six eyes (21%) and paracentral acute middle maculopathy lesions in one eye (4%). All patients hospitalized for severe COVID infection had anomalies on ICGA and OCT. Lesions to both retinal and choroidal vasculature were found. These anomalies could be secondary to vascular involvement related directly or indirectly to the SARS-CoV2 virus.

PURPOSE To report a new toxic retinopathy related to the use of hair dye. METHODS Case reports of three patients with follow-up after exposure and until resolution. RESULTS There were three middle-aged women (32-66 year old) all of whom had bilateral moderate to severe vision loss and normal slit-lamp examination at presentation. Fundus examination showed bilateral multiple serous retinal detachments predominantly located in the posterior pole, with some pigment epithelial hypertrophy in chronic cases. Optical coherence tomography showed similar features as in MEK-inhibitor retinopathy. Electrooculogram performed in one patient showed abnormal Arden ratio. During follow-up, visual acuity improved with regression of the serous retinal detachments. The speed of resolution was proportional to the acuteness of the exposure to aromatic amines. CONCLUSION Hair dyes containing aromatic amines can be responsible for bilateral toxic retinopathy mimicking MEK-inhibitor retinopathy.

Purpose. To examine choroidal neovascularization (CNV) characteristics in patients with exudative age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV), using swept-source optical coherence tomography angiography (SS-OCTA), and investigate agreement with OCT B-scan, fundus fluorescein angiography (FFA), and indocyanine green angiography (ICGA) by two different examiners. Methods. This is a retrospective multicentric study that involved patients with a history of AMD and PCV. Examiner A, who had access to OCTA, B-scan OCT, FFA, and ICGA imaging, had to differentiate between AMD and PCV, study the activity of AMD using Coscas’ criteria (active vs. quiescent), and categorize PCV subtypes, while examiner B had only access to OCTA. Then, the diagnostic concordance was assessed between both examiners. Results. A total of 27 patients (11 females (40.7%) and 16 males (59.3%), P = 0.231 ) were included in the analysis. Among those, 13 patients presented with neovascular AMD and 14 patients with PCV. There were 92.3% of correct answers regarding appropriate diagnosis and lesion characterization among AMD patients, against 61.5% of correct answers among PCV patients. The overall interrater reliability agreement between examiners, using Cohen’s kappa coefficient ( κ ) was 0.70 (0.5082-0.8916). Disagreement was found with one active AMD misdiagnosed as inactive AMD, three inactive PCV misdiagnosed as inactive AMD, and one inactive PCV misdiagnosed as active AMD. Conclusion. SS-OCTA alone remains limited in some specific phenotypes of PCV, which suggests the ongoing role of B-scan OCT associated with FFA and ICGA in the diagnosis of these conditions.

INTRODUCTION To date, corticosteroids remain the cornerstone treatment of ocular involvement of GCA, and no other treatment has proven to be effective in this setting. We herein report on a unique case of GCA with ocular involvement worsening despite high dose corticosteroids and recovering with intravenous iloprost. CASE REPORT A 70-year-old man presented with acute vision loss in his left eye related to anterior ischemic optic neuropathy. The diagnosis of giant-cell arteritis was confirmed by a temporal artery biopsy. Despite intravenous pulse methylprednisolone for 3 days then oral prednisone at 60 mg/day, the patient developed from day 5 fluctuating vision loss in the right eye, related to ocular ischemia by occlusion of the ophthalmic artery, and responsive to hyperhydration. Iloprost, an analog of prostacyclin PGI2, was then administered intravenously for 5 days and resulted in a stable improvement in visual acuity in the right eye. CONCLUSION This case highlights the potential role of vasodilatator agents in giant cell arteritis with ocular involvement.

Background: This paper reports the case of a young man who presented with syphilis masquerading as multiple evanescent white dots syndrome (MEWDS), which turned out to be an acute syphilitic posterior placoid chorioretinopathy (ASPPC) during follow-up. Case presentation: A 59-year-old healthy male consulted for a three days’ history of visual impairment in both eyes. On multimodal imaging, he was diagnosed as MEWDS. Fundus fluorescein angiography (FFA) showed early peripheral bilateral granular hyperfluorescence that correlated with the yellow-white dots found on fundus exam. Indocyanine green angiography (ICGA) depicted hypofluorescent dots on late phase. Spectral-domain optical coherence tomography (SD-OCT) revealed numerous inner retinal highly reflective deposits in the outer nuclear layer and disruption of the ellipsoid zone. After initial improvement, he presented again for a sudden visual loss at 3 weeks. FFA, ICGA and SD-OCT demonstrated the same but more numerous and outer lesions suggesting an ASPPC. A full inflammatory work-up revealed highly positive titers of rapid plasma regain (RPR) and fluorescent treponemal antibody absorption (FTA-Abs), suggesting a syphilis infection. The ophthalmological manifestations dramatically improved after the patient was admitted for high-dose intravenous penicillin G 24 million per day for 2 weeks. Conclusion: This is the first case that reports an ocular syphilitic infection masquerading as MEWDS at presentation and that turns to be an ASPPC. Syphilis serology should be routinely done in every case of atypical MEWDS especially when unusually presented in a young healthy man, with bilateral involvement and a bad clinical evolution.

Purpose To report the use of Spectralis High Magnification Module (HMM) as part of multimodal imaging in Multiple Evanescent White Dot Syndrome (MEWDS). Observations HMM imaging showed a blurry mosaic pattern corresponding to MEWDS-related photoreceptors’ lesions. These abnormalities remained detectable at later stages of the disease while other imaging modalities were negative. Conclusions and importance HMM can be a useful technique to monitor the structure of the outer retina during the different stages of MEWDS.

To collect information about the retinal blood flow variations and other choroidal and retinal parameters during a prolonged effort such as marathon running.Non-randomized prospective cohort study.Patients were recruited through an information campaign at the Rothschild Foundation Hospital (Paris, France). A first visit (V1) was planned in the month before the marathon. All participants underwent blood pressure measurement, fundus photography, spectral domain-optical coherence tomography (SD-OCT) and OCT-angiography (OCT-A). A second visit (V2) was scheduled within one hour of crossing the finish line. The same tests were repeated, using the same equipment.Of the 31 runners who were included, 29 finished the marathon and attended V2. At baseline, various ophthalmological abnormalities were found in 45.2% of the 58 eyes, among which almost a third concerned the optic nerve and a quarter the pachychoroid spectrum. A significant decrease in retinal vascular plexus density was found between V1 and V2 (p0.01). While median macular and retinal nerve fiber layer (RNFL) thicknesses significantly increased after the marathon (p0.01), median choroidal thickness significantly decreased (p0.01). Both systolic and diastolic blood pressures significantly decreased (p0.01 and p=0.021 respectively).Prolonged physical effort impacts the structure and vascularization of the retina and the choroid. Hypoxia and dehydration due to such an effort may induce a low ocular blood flow rate resulting in a choroidal thinning, contrasting with a transient subclinical ischemic edema of the inner retina and optic nerve head.NCT03864380.

Choroidal thickness can undergo considerable variations in response to different substances. The aim of this study was to assess the change in choroidal thickness after the ingestion of taurine and caffeine contained in the Red Bull energy drink.Enhanced Depth Imaging - Spectral Domain Optical Coherence Tomography was used to measure subfoveal choroidal thickness (SFCT) variations in healthy volunteers at several time points after drinking a Red Bull can (25 cl).Forty eyes of 20 volunteers were enrolled. A significant reduction in SFCT (-14 μm, p 0.0001) occurred at 1 h after the Red Bull intake, and was even more important (-20.14 μm, p 0.0001) in eyes with particularly thick choroids (≥ 395 μm). SFCT measurements at 4 h were comparable to baseline.A concomitant ingestion of caffeine and taurine can induce a transient choroidal thinning that is more marked in eyes with thick choroids.NCT02856256.

Wilson’s disease (WD) is an autosomal recessive genetic disorder due to a mutation of the ATP7B gene, resulting in impaired hepatic copper excretion and accumulation in various tissues. Ocular findings are one of the hallmarks of the disease. Many ophthalmological manifestations have been described and new techniques are currently available to improve their diagnosis and to follow their evolution. We have performed a systematic PubMed search to summarize available data of the recent literature on the most frequent ophthalmological disorders associated with WD, and to discuss the newest techniques used for their detection and follow-up during treatment. In total, 49 articles were retained for this review. The most common ocular findings seen in WD patients are Kayser–Fleischer ring (KFR) and sunflower cataracts. Other ocular manifestations may involve retinal tissue, visual systems and eye mobility. Diagnosis and follow-up under decoppering treatment of these ocular findings are generally easily performed with slit-lamp examination (SLE). However, new techniques are available for the precocious detection of ocular findings due to WD and may be of great value for non-experimented ophthalmologists and non-ophthalmologists practitioners. Among those techniques, anterior segment optical coherence tomography (AS-OCT) and Scheimpflug imaging are discussed.

In central serous chorioretinopathy (CSC), the macula is detached because of fluid leakage at the level of the retinal pigment epithelium. The fluid appears to originate from choroidal vascular hyperpermeability, but the etiology for the fluid is controversial. The choroidal vascular findings as elucidated by recent optical coherence tomography (OCT) and wide-field indocyanine green (ICG) angiographic evaluation show eyes with CSC have many of the same venous patterns that are found in eyes following occlusion of the vortex veins or carotid cavernous sinus fistulas (CCSF). The eyes show delayed choroidal filling, dilated veins, intervortex venous anastomoses, and choroidal vascular hyperpermeability. While patients with occlusion of the vortex veins or CCSF have extraocular abnormalities accounting for the venous outflow problems, eyes with CSC appear to have venous outflow abnormalities as an intrinsic phenomenon. Control of venous outflow from the eye involves a Starling resistor effect, which appears to be abnormal in CSC. Similar choroidal vascular abnormalities have been found in peripapillary pachychoroid syndrome. However, peripapillary pachychoroid syndrome has intervortex venous anastomoses located in the peripapillary region while in CSC these are seen to be located in the macular region. Spaceflight associated neuro-ocular syndrome appears to share many of the pathophysiologic problems of abnormal venous outflow from the choroid along with a host of associated abnormalities. These diseases vary according to their underlying etiologies but are linked by the venous decompensation in the choroid that leads to significant vision loss. Choroidal venous overload provides a unifying concept and theory for an improved understanding of the pathophysiology and classification of a group of diseases to a greater extent than previous proposals.

Introduction: Polypoidal choroidal vasculopathy (PCV) is a choroidal pathology characterized by frequent occurrences of subretinal hemorrhages and resistance to monotherapies such as ranibizumab or bevacizumab intravitreal injections (IVT). The purpose of this study is to evaluate both the anatomical and functional efficacy of aflibercept IVT as a monotherapy in PCV in a Caucasian population. Methods: We conducted a prospective multicenter study in either treatment-naïve patients with PCV or PVC patients who had not been treated with anti-VEGF within the previous 3 months or with photodynamic therapy (PDT) within the previous 6 months. All patients had been treated with 3 initial monthly loading doses of aflibercept followed by a Q8 regimen for 28 weeks in total. All patients underwent a complete ophthalmic examination including the measurement of best-corrected visual acuity (BCVA) before each IVT and after 28 weeks as well as an optical coherent tomography (OCT) of the macula. At baseline and 28 weeks, the polypoidal dilations were analyzed with indocyanine green angiography. Results: Thirty-four eyes of 34 patients were included in this study. All patients were followed for 28 weeks and received 5 aflibercept IVT. The mean baseline BCVA was 55 letters. After 28 weeks, significant +13 letters in BCVA and a regression of exudative signs on OCT in all patients were observed. In 62% of the cases, polyp disappearance was observed on indocyanine green angiography. Discussion: In this study on a Caucasian population, we showed that aflibercept as a monotherapy provided both a significant visual gain and the regression of polypoidal dilations. Aflibercept use in monotherapy may contribute to reduce the hemorrhagic risk and atrophy linked to PDT.

BackgroundMultiple evanescent white dot syndrome (MEWDS) is an inflammatory disease that can be associated with choroidalneovascularisation (CNV). However, few studies in the literature have described the occurrence of MEWDS in association with CNV. This paper discusses whether CNV can trigger MEWDS in a predisposed eye.MethodsA retrospective multicentric case series of six eyes in six patients with acute onset of MEWDS and evidence of previous CNV was conducted between January 2015 and January 2017. All patients underwent ophthalmic examination including multimodal imaging at baseline and during follow-up.ResultsThe mean age was 32.2±12.2 years. The majority of patients were women (5/1). In each case, MEWDS was diagnosed during a recurrence or occurrence of CNV secondary to choriocapillaritis, central serous chorioretinopathy or atrophic scar, presumably due to congenital toxoplasmosis. All patients were treated with intravitreal injections of antivascular endothelial growth factor (anti-VEGF) with good anatomical and functional responses (mean gain of 0.3±0.31logMAR). The mean duration of follow-up was 13.5±10.65 months.ConclusionThis study highlights a sequence in the development of MEWDS, following the occurrence or recurrence of CNV. CNV may trigger MEWDS, possibly due to the proinflammatory environment created by the retinal tissue surrounding the CNV.

// Thibaud Mathis 1 , Stephane Vignot 2 , Cecila Leal 3 , Jean-Pierre Caujolle 3 , Celia Maschi 3 , Martine Mauget-Faysse 4 , Laurent Kodjikian 1 , Stephanie Baillif 3 , Joel Herault 5 and Juliette Thariat 5, 6 1 Department of Ophthalmology, Croix-Rousse University Hospital, 69004 Lyon, France 2 Department of Medical Oncology, Jean Godinot Institute, 51100 Reims, France 3 Department of Ophthalmology, Pasteur II Hospital, 06000 Nice, France 4 Rothschild Ophthalmologic Foundation, 75019 Paris, France 5 Proton Therapy Center, Universite Nice Sophia Antipolis, 06200 Nice, France 6 Department of Radiation Therapy, Centre Francois Baclesse, ARCHADE, 14000 Caen, France Correspondence to: Thibaud Mathis, email: mathisthibaud@hotmail.fr Keywords: phosphenes, radiation therapy, eye tumors, choroidal melanoma, proton beam therapy Received: April 04, 2017 Accepted: May 15, 2017 Published: June 28, 2017 ABSTRACT Anomalous visual perceptions have been reported in various diseases of the retina and visual pathways or can be experienced under specific conditions in healthy individuals. Phosphenes are perceptions of light in the absence of ambient light, occurring independently of the physiological and classical photonic stimulation of the retina. They are a frequent symptom in patients irradiated in the region of the central nervous system (CNS), head and neck and the eyes. Phosphenes have historically been attributed to complex physical phenomena such as Cherenkov radiation. While phosphenes are related to Cherenkov radiation under high energy photon/electron irradiation conditions, physical phenomena are unlikely to be responsible for light flashes at energies used for ocular proton therapy. Phosphenes may involve a direct role for ocular photoreceptors and possible interactions between cones and rods. Other mechanisms involving the retinal ganglion cells or ultraweak biophoton emission and rhodopsin bleaching after exposure to free radicals are also likely to be involved. Despite their frequency as shown in our preliminary observations, phosphenes have been underreported probably because their mechanism and impact are poorly understood. Recently, phosphenes have been used to restore the vision and whether they might predict vision loss after therapeutic irradiation is a current field of investigation. We have reviewed and also investigated here the mechanisms related to the occurrence of phosphenes in irradiated patients and especially in patients irradiated by proton therapy for ocular tumors.

Summary Purpose To assess the evolution of macular pigment optical density (MPOD) following supplementation with various macular formulations obtained with the Visucam® 200, and to study the factors affecting MPOD measurements. Materials and methods In this prospective, randomized, double-masked multicenter study, patients were divided into 2 groups: group A (patients without retinal pathology who underwent cataract surgery 1 month previously) and group B (patients with neovascular age-related macular degeneration [AMD] in one eye). In each group, half of the patients were randomly assigned to receive a food supplementation either with or without carotenoids (5 mg of Lutein and 1 mg of Zeaxanthin). Outcome measures included MPOD responses obtained with the Visucam® 200 for one year. Results In total, 126 subjects (52 men, 74 women) with a mean age of 75.3 ± 7.61 years were enrolled. Mean MPOD values at the time of inclusion were statistically lower in group A (0.088 density unit [DU]) compared to group B (0.163 DU, P Conclusion No significant improvement in MPOD seems to be detected with the Visucam® 200 after carotenoid supplementation. The MPOD measurement seems to be highly affected by cataract extraction and the presence of AMD.

Objective To describe a new stage of multiple evanescent white dot syndrome (MEWDS), occurring at a very early phase of the disease. Methods Retrospective analysis of clinical, angiographic and tomographic findings in four patients with ‘hyper-early’ stage MEWDS. Results In four patients seen within 1 week of the onset of symptoms, fundus analysis revealed macular granity and the classic yellow–white dots, some having no corresponding hyperautofluorescent pattern. Spectral-domain optical coherence tomography (SD-OCT) showed central foveal disruption of the ellipsoid zone (EZ) and interdigitation layer with a hyper-reflective dome-shaped lesion. In two patients, fluorescein angiography (FA) revealed an intermediate hypofluorescent perimacular halo, whereas late indocyanine green angiography (ICGA) showed a hyperfluorescent halo as well as the classic MEWDS features. After a few days, the EZ disruption appeared complete on OCT and fundus autofluorescence (FAF) in all patients. Visual acuity, OCT and FAF findings had fully recovered within 3 months. Conclusions We have shown a new feature of MEWDS on FAF, OCT, FA and ICGA, corresponding to a very early stage of the disease.

Central retinal vein occlusion (CRVO) is a common retinal disease. Recent works mentioned spontaneous retinal arterial pulsations (SRAPs) as a feature of some CRVOs. This is a retrospective study on patients presenting with CRVO who were followed up for at least 6 months. The objective was to identify SRAP in the acute phase of the disease and determine their relationship with patients’ characteristics and visual prognosis. A 10-second infrared film centered on the optic disc was recorded within a month of the onset of symptoms, and SRAPs were detected in two-thirds of the cases. Patients with SRAP were significantly younger than those without SRAP. Mean central macular thickness was significantly higher in the absence of SRAP, which was translated into a more severe macular edema; however, this difference faded with time. BCVA tended to be higher in the presence of SRAP at the 6-month follow-up when adjusted to baseline. This study demonstrates that SRAPs are a frequent finding, easily detected by infrared fundus video recording, and associated with a younger age and lesser macular edema.

Importance Long-term data of intravitreal injections of vascular endothelial growth factor (VEGF) inhibitors are lacking. Background This study aims to assess visual and anatomic outcomes of eyes with neovascular age-related macular degeneration (nAMD) after 10 years of anti-VEGF therapy. Design Retrospective analysis of data from a prospectively designed database. Participants One hundred and sixteen eyes with nAMD (94 participants) that started anti-VEGF therapy at least 10 years earlier. Methods Eyes were tracked by the Fight Retinal Blindness! registry. Main outcome measures Mean change in visual acuity at 10 years vs baseline. Visual acuity was assessed by the number of letters read on a logarithm of the minimum angle of resolution chart. Results Eyes received a median of 27.5 injections over 10 years. Mean visual acuity was 57.5 letters (SD 17.5) at baseline. It increased slightly at 1 year, then dropped steadily by 18 letters (95% CI: 13.7; 22.3) at 10 years. Overall, 10% of eyes gained ≥10 letters, 64% lost ≥10 letters and 23% remained stable (±5 letters from baseline). Geographic atrophy and subretinal fibrosis were found in 93% and 71%, respectively, after 10 years, both mostly affecting the centre of the fovea. Pre-treated eyes (47.5%) had significantly worse visual acuity than treatment-naive eyes at baseline and during follow-up and were significantly more likely to have atrophy and fibrosis. Conclusions and relevance Despite short-term stabilization, long-term visual outcomes of nAMD eyes under anti-VEGF therapy may be poor. Development of atrophy and fibrosis, resulting from the natural progression of the disease, may partly explain this evolution.

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 describe and interpret the features of pachychoroid neovasculopathy (PNV) using optical coherence tomography angiography (OCTA) technique. Methods This is an observational case series of patients who presented with PNV. Best-corrected visual acuity (BCVA), anterior segment examination, dilated funduscopic examination, infrared and autofluorescence fundus images and spectral-domain optical coherence tomography (SD-OCT; B-scan, ‘en-face’ and OCTA) were carried out for all patients. Choroid thickness was measured using enhanced depth imaging (EDI) mode. Colour fundus photographs, fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were not performed systematically. Optical coherence tomography angiography (OCTA) features of PNV are described and interpreted. Results Five eyes of five patients with a mean age of 62.2 years (range, 53–73 years) presenting with PNV were analysed. They all presented pachychoroid pigment epitheliopathy (PPE) with choroidal thickening and dilated choroidal vessels as seen with EDI-OCT. Fluorescein angiography (FFA) was performed in three patients showing multiple areas of retinal pigment epithelium atrophy and fundus lesions suggestive of chronic central serous chorioretinopathy. Indocyanine green angiography (ICGA) in one patient confirmed the presence of large choroidal veins and choroidal hyperpermeability seen beneath the area of the neovascular tissue. Two eyes had the appearance of polypoidal structures within the neovascular tissue, with the characteristic aspect of the polypoidal lesions on B-scan and ‘en-face’. Optical coherence tomography angiography (OCTA) showed the appearance of tangled filamentous vascular network in all eyes. Conclusion Optical coherence tomography angiography (OCTA) is a safe, highly sensitive and specific examination for the detection of type 1 neovascularization associated with PPE. Features are characteristic of tangled filamentous vessels overlying a focal area of thickened choroid.

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 update the medical literature on the diagnostic and therapeutic approach to polypoidal choroidal vasculopathy (PCV) and to propose a treatment algorithm in agreement with French market approval, supported by the France Macula Federation (FFM). METHODS: Literature review and expert opinion. RESULTS: The diagnosis of PCV is based on multimodal imaging, including indocyanine green angiography (ICGA), which is considered the gold standard for the diagnosis of PCV. Regarding the therapeutic management of PCV, the FFM recommends treating PCV first-line either by monotherapy with intra-vitreal anti-vascular endothelial growth factor (anti-VEGF) injections, or by a combined treatment of photodynamic therapy (PDT) with Verteporfin and intra-vitreal anti-VEGF injections, depending on the location of the PCV.; Objectif : Mettre à jour la littérature médicale sur l’approche diagnostique et thérapeutique de la vasculopathie polypoïdale choroïdienne (VPC) et proposer un algorithme de traitement adapté aux recommandations françaises et à la bonne utilisation des autorisations de mise sur le marché (AMM), soutenu par la Fédération France Macula (FFM).Méthodes : Analyse de la littérature et avis d’experts.Résultats : Le diagnostic de la VPC repose sur l’imagerie multimodale, néanmoins, l’angiographie au vert d’indocyanine est recommandée par la FFM et reste le Gold Standard. Concernant la prise en charge thérapeutique de la VPC, la FFM recommande de traiter la VPC en 1ère intention soit par monothérapie avec des injections intra-vitréennes d’anti-VEGF, soit par traitement combiné associant photothérapie dynamique (PDT) à la Verteporfine et injections intra-vitréennes d’anti-VEGF en fonction de la localisation de la VPC.

Rationale: Crystal sorting histiocytosis (CSH) is a rare disorder that is morphologically characterized by the accumulation of monoclonal immunoglobulin crystals, predominantly of a kappa light chain type, within lysosomes of macrophages. CSH may result in a variety of clinical manifestations depending on the involved organs. In this case report, we aim to describe a patient with ophthalmic manifestations which lead to the diagnosis of multiple myeloma with crystal-storing histiocytosis, crystalline podocytopathy, and light chain proximal tubulopathy. Patient concerns: A 60-year-old male patient presented with progressive bilateral decreased vision for 2 years. Diagnosis: Ophthalmic explorations showed bilateral macular and papillary edema, and multiple crystalline deposits in the anterior stromal cornea and in the retina. Laboratory tests showed nephrotic syndrome and renal dysfunction. Further work-up revealed IgG kappa multiple myeloma, with biopsy-proven combined crystalline podocytopathy and tubulopathy. Interventions: The patient received chemotherapy (bortezomib, cyclophosphamide, and dexamethasone for 3 cycles, then bortezomib, lenalidomide, and dexamethasone). Outcomes: Despite partial hematologic response and improvement of the papilledema and macular edema, the patient developed dialysis-dependent end-stage renal failure. Lessons: This report, highlighting the protean presentation of paraprotein-mediated injuries, provides additional information on the ocular anomalies not previously described that may be associated with crystal-storing histiocytosis.

Purpose To describe clinical, angiographic, and tomographic features and the therapeutic management of patients with retinal arterial macroaneurysm (RAM). Methods Retrospective analysis of patients diagnosed with RAM between January 2011 and September 2014 in Professor Sahel's Department of Ophthalmology, Rothschild Foundation. Retinal arterial macroaneurysms were classified as hemorrhagic, exudative, or quiescent. Patient demographics and comorbidities were recorded. All patients underwent complete ophthalmologic examination including initial and final visual acuity (Early Treatment Diabetic Retinopathy Study chart), slit-lamp biomicroscopy, fundus examination, color and autofluorescence fundus imaging, fluorescein and indocyanine green angiography, and spectral-domain optical coherence tomography. Treatments were recorded. Results Fourteen RAMs of 14 patients were included: 9 hemorrhagic and 5 exudative. The most severe clinical picture was associated with the hemorrhagic form. Exudative cases had a progressive onset and a better visual prognosis. Direct laser photocoagulation was performed in 4 cases, anti-vascular endothelial growth factor (VEGF) intravitreal injection in 4 cases, combined treatment (YAG laser + argon laser + anti-VEGF intravitreal injection) in 1 case, and 5 cases received no treatment. Conclusions Retinal arterial macroaneurysm management should be specialized and rapid. Multimodal imaging should be performed for classifying it, assessing its prognosis and determining the most suitable treatment.

The authors report, for the first time, an association between large colloid drusen (LCD) and choroidal neovascularization in a 58-year-old man. Multimodal imaging confirmed the diagnosis of LCD in both eyes and polypoidal choroidal vasculopathy in the left eye. The patient was treated with monthly intravitreal injections of aflibercept (Eylea; Regeneron, Tarrytown, NY). The authors hypothesize that these deposits are probably associated with retinal pigment epithelium dysfunction and could thus lead to delayed neovascularization and atrophy. [ Ophthalmic Surg Lasers Imaging Retina . 2016;47:1154–1156.]

Purpose: To describe abnormal dark (hyposignal) and white (hypersignal) lesions observed on optical coherence tomography angiography in central serous chorioretinopathy. Methods: Prospective, multicenter, and descriptive study including patients with active or quiescent central serous chorioretinopathy. All patients had undergone a complete ophthalmic examination. Results: Abnormal dark lesions were detected as “dark spots” and “dark areas” on optical coherence tomography angiography. A “dark spot” could correspond to six different abnormalities: pigment epithelium detachment, subretinal deposit, “Lucency” within surrounding subretinal fibrin, choroidal cavitation, choroidal excavation, and choroidal fluid. A “dark area” could be related to a serous retinal detachment or choriocapillary compression. Abnormal white lesions were also detected: A “white spot” could correspond with the leaking point on fluorescein angiography or with hyper-reflective dots; A “white filamentous pattern” at the Brüch’s membrane level corresponded to abnormal choroidal neovascular vessels. Conclusion: A semiology is described using optical coherence tomography angiography in central serous chorioretinopathy as abnormal dark and white lesions. Multimodal imaging is mandatory in addition to optical coherence tomography angiography to diagnose non-neovascular retinal and choroidal central serous chorioretinopathy lesions. However, optical coherence tomography angiography alone is helpful in detecting choroidal neovascular membrane in central serous chorioretinopathy.

EMAP (Extensive Macular Atrophy with Pseudodrusen) is a maculopathy we recently described that shares pseudodrusen and geographic atrophy with Age-related Macular Disease (AMD). EMAP differs from AMD by an earlier age of onset (50-55 years) and a characteristic natural history comprising a night blindness followed by a severe visual loss. In a prospective case-control study, ten referral centers included 115 EMAP (70 women, 45 men) patients and 345 matched controls to appraise dietary, environmental, and genetic risk factors. The incidence of EMAP (mean 2.95/1.106) was lower in Provence-Côte d’Azur with a Mediterranean diet (1.9/1.106), and higher in regions with intensive farming or industrialized activities (5 to 20/1.106). EMAP patients reported toxic exposure during professional activities (OR 2.29). The frequencies of common AMD complement factor risk alleles were comparable in EMAP. By contrast, only one EMAP patient had a rare AMD variant. This study suggests that EMAP could be a neurodegenerative disorder caused by lifelong toxic exposure and that it is associated with a chronic inflammation and abnormal complement pathway regulation. This leads to diffuse subretinal deposits with rod dysfunction and cone apoptosis around the age of 50 with characteristic extensive macular atrophy and paving stones in the far peripheral retina.

Purpose. To identify spectral-domain optical coherence tomography (SD-OCT) predictive morphological features for the outcome of Ranibizumab therapy for neovascular age-related macular degeneration (AMD). Methods. This is a retrospective multicentric study that involved 64 eyes with naïve AMD. Patients who received three monthly intravitreal injections of Ranibizumab were stratified into (1) “responders” [≥ 5 letters gain on Early Treatment Diabetic Retinopathy Study (ETDRS) scale] and (2) “nonresponders” (< 5 letters gain). Best-corrected visual acuity (BCVA) and SD-OCT morphological features were compared at baseline and one month after three consecutive injections of Ranibizumab. Univariate and multivariate analyses were carried out to correlate these morphological features with the change in BCVA. Results. Among the 64 patients enrolled, 40 (62.5%) were “responders” and 24 (37.5%) “nonresponders”. Age, sex, and BCVA were comparable between both groups. A multivariate correlational analysis found that subfoveal choroidal thickness (SFCT) and the presence of pigment epithelial detachment (PED) > 250 μm at baseline were two independent prognostic indicators of final BCVA. No other SD-OCT morphological studied features seem to affect final BCVA after Ranibizumab treatment. Conclusion. SFCT and the presence of PED > 250 μm are two significant biomarkers that may predict improvement after Ranibizumab therapy for AMD. These markers may guide ophthalmologists' treatment decision under financial constraints and limited time.

Pericentral visual field changes and disruption of the ellipsoid layer on spectral domain optical coherence tomography (SD-OCT) are the main features of antimalarial retinal toxicity. C-Scan OCT or “en face” enables a topographic frontal view of the changes observed within the different retinal layers in particular the ellipsoid layer. The aim of this prospective study was to compare multifocal ERG (mfERG) responses with the results of C-Scan OCT (“en face” OCT) in patients with abnormal visual field and to analyze relationships between the structural and functional abnormalities. In 354 consecutive patients screened for antimalarial toxicity between January 1, 2014 and December 31, 2016, central visual field, mfERG recording, C-Scan OCT and short-wavelength fundus autofluorescent imaging were performed. Among the 17/354 patients with abnormal central visual field results, all presented with abnormalities on the mfERG at least in one eye. In 16/33 eyes, there was a good concordance between focal loss of the mfERG response and the disruption of the ellipsoid layer on C-Scan OCT. In one eye with characteristic changes in the ellipsoid layer on the C-Scan OCT, the mfERG was normal, whereas in three eyes the mfERG was abnormal in eyes with a normal C-Scan OCT. The contribution of the C-Scan OCT changes remains difficult to establish as there is no strict concordance with the local ERG responses. Although C-Scan OCT technology provides a new approach in analyzing focal abnormalities in the photoreceptor–retinal pigment epithelium interface, the sensitivity of this method compared with mfERG and other tests (central visual field, B-Scan OCT) needs to be evaluated. This study is still ongoing on a larger cohort.

To describe multimodal imaging findings in patients with dark or white without pressure lesions of the fundus.Retrospective observational case series of 10 patients with white or dark without pressure lesions. We analyzed multimodal imaging using spectral domain optical coherence tomography, color and near-infrared fundus photography, and fundus autofluorescence imaging to explore the findings associated with these lesions.All patients had geographic dark or white lesions on clinical examination and color photography, which were either hyporeflective or hyperreflective on near-infrared reflectance imaging, respectively. On optical coherence tomography, these lesions correlated with an abrupt change of the photoreceptor reflectivity, with relative hyporeflectivity of photoreceptor zones (ellipsoid and interdigitation zones, as well as outer segments) within the dark, and relative hyperreflectivity within white lesions. Ten patients underwent fundus autofluorescence, which showed well-defined zones of relative hypo-autofluorescence within the lesion, compared with neighboring uninvolved regions, whether dark or white without pressure. In two patients who had a lesion combining white and dark without pressure, we observed the transition in photoreceptor reflectivity from the dark lesion (hyporeflective) to the white lesion (hyperreflective), relative to the surrounding retina.Both white and dark without pressure lesions are associated with changes in outer retinal reflectivity on optical coherence tomography, which occur in opposite directions compared with the surrounding unaffected areas. In the face of normal visual field testing to date, the clinical significance of this finding remains uncertain. Recognition of the optical coherence tomography appearance will help clinicians avoid unnecessary workup of these patients for outer retinal dystrophy or degeneration.

Aims To describe retinal and choroidal changes in acute and quiescent central serous chorioretinopathy (CSC) observed with ‘En face’ spectral domain optical coherence tomography (SD OCT) combined with enhanced-depth imaging (EDI). Methods A prospective and descriptive study at the Rothschild Ophthalmologic Foundation (Paris, France) between September 2011 and February 2012. Eyes with a clinical diagnosis of CSC were examined using SD OCT with EDI, fluorescein and indocyanine green angiography. 3D reconstruction of 197 transverse sections with SD OCT, spaced of 30 μ, provided a virtual macular brick through which 496 sections in the coronal plane resulted in a C-scan or En face OCT image. Results 23 of 29 eyes (79%) had serous retinal detachment (all active CSC) and 22 had pigment epithelial detachment (75%). Pigment epithelial hyperplasia was visualised in nine eyes (31%). Posterior cystoid retinal degenerations were present in five eyes (17%). The mean choroidal thickness was 491.5 μ. In 11 eyes (38%), En face OCT showed multiple hyper reflective points located at the level of the choriocapillary layer and choroidal cavitations were found in two patients. Conclusions En face OCT imaging using SD OCT is an easy, reproducible, non-invasive and effective tool to understand choroidal changes in acute and quiescent CSCR. It provides complementary morphological information, describes new semiological entities and might substitute other exams in the future.