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Resume Les stries angioides sont une pathologie oculaire qui se complique frequemment par la survenue de neovaisseaux choroidiens. Leur prise en charge a longtemps ete dominee par des traitements conventionnels tels le laser thermique ou la phototherapie dynamique. Plus recemment, les anti-VEGF sont devenus une alternative therapeutique de choix. Nous rapportons le cas d’un patient de 54 ans ayant presente des neovaisseaux choroidiens en rapport avec la presence de stries angioides et pour lesquels un traitement par anti-VEGF (ranibizumab) a ete realise. Deux injections ont ete necessaires pour stopper le processus neovasculaire. Le patient a ete suivi pendant plus de 12 mois sans qu’aucune recidive ne soit notee. Ce cas illustre l’interet des anti-VEGF dans la prise en charge des neovaisseaux choroidiens compliquant les stries angioides. Des etudes prospectives et randomisees incluant un plus grand nombre de patients sont necessaires afin d’etablir le rythme d’injections et de surveillance optimal dans cette pathologie.

To characterize and correlate the different patterns of fundus autofluorescence (FAF) in patients with birdshot chorioretinopathy (BSCR), with functional and anatomical parameters. Twenty-one BSCR patients were prospectively studied in 2013 and 2014. Each patient underwent visual acuity (VA) and visual field (SITA standard 30.2) testing as well as fluorescein and indocyanine green angiography, spectral-domain optical coherence tomography (SD-OCT) B scan, enhanced depth imaging (EDI), and fundus autofluorescence (FAF) imaging. The disease was classified as active, chronic, or quiescent. The patients’ mean age was 60.3 ± 9.2 years and 60% were female. Disease duration was 5.7 ± 3.7 years. Autofluorescence imaging showed punctiform hyper-FAF spots in 23 out of the 29 eyes (79%), which was significantly associated with a greater visual field mean deviation (−7 ± 7 versus −3 ± 2 dB, p = 0.04). Hypo-FAF was defined as peripapillary (n = 25; 86.2%), macular (n = 10; 34.5%), lichenoid (n = 17; 58.6%), and/or diffuse (n = 13; 44.8%). Lichenoid hypo-FAF was significantly associated with worse VA (0.18 ± 0.24 vs. 0.05 ± 0.07 LogMAR, p = 0.04). Macular hypo-FAF was associated with a history of macular edema (62.5%; p = 0.06). Diffuse hypo-FAF was observed more frequently (p = 0.01) in chronic disease (66.7%) than in active (0%) or quiescent disease (27.3%). Autofluorescence analysis in BRSC patients contributes to evaluating disease activity and could be useful to guide follow-up and treatment.

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.Clinical and molecular study.A total of 45 patients from 3 generations belonging to a family originating from Martinique with an autosomal dominant MCRPE were examined.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.The MCRPE natural history in view of MAPKAPK3 function and Mapkapk3(-/-) mouse retinal lesions.Eighteen patients had the c.518TC mutation. One heterozygous woman aged 20 years was asymptomatic with normal fundus and SD OCT (stage 0). All c.518TC 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.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.

Resume Objectifs Rapporter les resultats du traitement par phototherapie dynamique a la Visudyne® (V-PDT) en fluence reduite applique a des patients atteints de chorioretinite sereuse centrale (CRSC) chronique (CRSC evoluant depuis plus de six mois). Patients et methodes L’ensemble des donnees cliniques, angiographiques et OCT des patients atteints de CRSC chronique traites par V-PDT entre 2002 et 2008 au centre Rabelais de Lyon a ete analyse retrospectivement. Un bilan complet (recherche de syndrome maculaire, acuite visuelle ETDRS, biomicroscopie, AG a la fluoresceine et au vert d’indocyanine, OCT) etait realise avant le traitement par V-PDT et trois mois apres. Les patients etaient ensuite regulierement suivis et retraites en cas de recidives. Le traitement par V-PDT etait realise avec un spot de surface egale a la surface des zones hyperfluorescentes a bords flous reperees sur les temps tardifs de l’angiographie ICG, de puissance reduite de moitie (300 mW/cm2 au lieu de 600). Resultats Quarante et un yeux de 34 patients (27 hommes) d’âge moyen 53 ans ont ete inclus. Dix-huit yeux avaient deja ete traites par photocoagulation au laser. Plusieurs points de diffusion caracteristiques des CRSC etaient visibles sur l’angiographie a la fluoresceine dans la plupart des cas (n = 38), maculaires dans 35 cas. Avant traitement : des metamorphopsies etaient notees dans 51 % des cas, un œdeme intraretinien (OIR) etait present sur l’OCT dans 71 % des cas, un DSR dans 85 % et un DEP dans 10 %. Trente-neuf yeux n’ont beneficie que d’une seance de traitement et un œil a ete retraite une fois. A M+3 post-V-PDT, un OIR etait present dans 15 % des cas, un DSR dans 12 % et un DEP dans 2 %. A l’issue d’une periode de suivi de 20 mois, un OIR etait present dans 14 % des cas, un DSR dans 15 % des cas et un DEP dans 0 %. Une atrophie maculaire etait observee sur l’OCT dans la plupart des cas a l’issue du suivi (epaisseur centrale mesuree en moyenne a 144,5 microns). Comparee a l’AV initiale, l’AV a M+3 post-V-PDT etait abaissee dans 22 % des cas, stabilisee dans 39 % et amelioree dans 39 % alors que l’AV finale a l’issue du suivi etait abaissee dans 12 % des cas, stabilisee dans 17 % et amelioree dans 71 %. Aucune complication n’a ete observee. Discussion Le traitement par V-PDT des formes chroniques de CRSC est a l’origine d’une amelioration anatomique et fonctionnelle (disparition durable des phenomenes exsudatifs dans la plupart des cas et augmentation de l’acuite visuelle dans plus de deux cas sur trois dans cette etude). L’atrophie observee peut etre induite par le traitement ou etre liee a l’evolution naturelle de la maladie.

Purpose: To describe novel cystic structures (‘outer retinal cysts’ or ORC) found in the outer retina in age-related macular degeneration (AMD). Methods: One hundred and seventy-three consecutive eyes of 88 AMD patients were prospectively examined with spectral domain optical coherence tomography (SD-OCT). The prevalence of ORCs was searched, and their sizes and shapes were determined. Results: SD-OCT revealed round or ovoid, intraretinal, hyporeflective cystic structures with a hyperreflective border in 60 eyes (56%) with neovascular AMD and in six eyes (21%) with atrophic AMD. These cystic structures were of different sizes and shapes. They remained stable in all the patients after a follow-up period of 6 months. Conclusions: Outer retinal cyst is a new type of cystic structure recently identified in AMD patients. ORCs should not be confused with intraretinal exudates or cystoid cavities and therefore do not require any treatment. The histopathological nature of ORC remains to be determined. Further studies are necessary to determine their true origin.

To determine the specific location of the initial lesion in acute retinal pigment epitheliitis.Four patients diagnosed with acute retinal pigment epitheliitis were studied. Fundus photographs, fluorescein angiography and indocyanine green angiography, and spectral-domain optical coherence tomography findings were reviewed.Four healthy young patients presented with acute onset of unilateral decreased vision. Ophthalmoscopy showed macular pigment mottling with surrounding yellow hypopigmented areas at the level of the retinal pigment epithelium (RPE). Fluorescein angiography revealed transmission hyperfluorescence. Early-phase and midphase indocyanine green angiography images showed a patchy macular hyperfluorescence. At late phase of indocyanine green angiography, a hyperfluorescent halo with a cockadelike appearance of the macular area was observed. Spectral-domain optical coherence tomography showed a disruption of the photoreceptors' inner segment and outer segment interface associated with a wider disruption of the RPE inner band. These disrupted lines were replaced by a dome-shaped highly reflective lesion involving the RPE inner layer, the photoreceptors' inner segment and outer segment layers, and, in two cases, the outer nuclear layer. With time, indocyanine green angiography showed resolution of the observed lesions. Spectral-domain optical coherence tomography showed restored and continuous inner segment and outer segment layers and RPE inner band.Spectral-domain optical coherence tomography findings suggest that the initial lesion in acute retinal pigment epitheliitis is located at the junction between the photoreceptor outer segments and the apical side of the RPE cells. Indocyanine green angiography and spectral-domain optical coherence tomography show that the RPE appears to be more widely involved than the neurosensory retina.

The pathophysiology of nonarteritic anterior ischemic optic neuropathy (NA-AION) is still imperfectly understood, but arterial hypertension seems to play a decisive role. Intolerance to hypoxia is defined as desaturation of hemoglobin more than 15% at rest and/or elevation of blood pressure at rest or after exercise over 20mmHg in hypoxia. We report the case of a 66-year-old woman who presented bilateral NA-AION during a trek at high altitude (>2500m). The etiological check up was negative. Due to the circumstances of occurrence, we requested a tolerance to hypoxia test, which was positive. In this case, the combined effects of altitude and effort probably led to prolonged desaturation of oxyhemoglobin associated with an excessive blood pressure increase upon exercise leading to ischemia of the optic nerve head. This case showed the value of a systematic search for hypoxia tolerance in patients with nonarteritic anterior ischemic optic neuropathy occurring during a situation of prolonged hypoxia (long-distance flight, high altitude).

Resume Introduction L’utilisation recente des spectral domain (SD) a permis de mettre en evidence la presence de formations kystiques intraretiniennes chez des patients presentant une degenerescence maculaire liee a l’âge (DMLA) neovasculaire ou atrophique. L’objectif de notre etude a ete de decrire ce nouvel aspect de tomographie en coherence optique (OCT) chez des patients presentant une DMLA exsudative traitee par injections intravitreennes (IVT) d’anti-vascular endothelial growth factor (anti-VEGF). Methode Il s’agit d’une etude prospective portant sur une serie de patients presentant une DMLA exsudative vus sur une periode de quatre mois. Pour chaque patient ont ete analyses l’OCT en spectral domain (SD-OCT) et l’acuite visuelle. Tous les patients avaient beneficie d’au moins trois IVT d’anti-VEGF au cours des 12 derniers mois. Resultats Trois cent quatre-vingt-dix-huit DMLA exsudatives (de 376 patients) ont ete examinees. Dans 4,5 % des yeux (18 cas), ayant recu en moyenne cinq injections d’anti-VEGF, le SD-OCT a permis de retrouver des lesions intraretiniennes, arrondies, a contenu hypo-reflectif et entourees d’une collerette reguliere hyper reflective. Ces kystes sont en general multiples (de 2 a 7) et leur taille peut varier de 60 a 600 μm. Des ponctuations moyennement reflectives ont ete observees au sein et le long du bord interne des kystes. Ces lesions sont situees au niveau de la couche nucleaire externe et se localisent en regard d’une zone de fibrose neovasculaire ou en bordure d’un territoire d’atrophie chorio-retinienne. Les kystes retiniens externes (KRE) ne sont pas sensibles aux injections d’anti-VEGF et ne semblent pas se modifier pendant quatre mois d’observation. Conclusion Les KRE sont un nouvel aspect de l’OCT, frequemment rencontre dans les DMLA. Ils correspondent probablement a des macrophages actives digerant des lipoproteines oxydees provenant de la degenerescence retinienne observee au cours des DMLA neovasculaires. Les KRE doivent etre differencies des œdemes intraretiniens ou des decollements sereux retiniens afin d’eviter tout traitement anti-angiogenique inutile.

Central retinal vein occlusion (CRVO) leads to poor visual outcome in most eyes. Abnormal hemorheology was suspected to play a major role in its pathogenesis. CRVO treatment is still a matter of debate but several studies have pointed out the efficacy of isovolumic hemodilution. The aim of this study was to assess the feasibility and efficacy of hemodilution using automated erythrocytapheresis in recent-onset CRVO.In this prospective randomized controlled multicenter study, 61 consecutive CRVO patients were enrolled when they met the following criteria: CRVO lasting for 3 weeks or less, visual acuity ranging from 20/200 to 20/32, age between 18 and 85 years, no diabetes, no uncontrolled systemic hypertension, no antiplatelet or anticoagulant therapy, hematocrit higher than 38%, and signed informed consent. Patients were randomly assigned to the hemodilution group (n = 31) or to the control group (n = 30). Hemodilution therapy consisted of one session of erythrocytapheresis on outpatient basis, followed by additional session(s) for 6 weeks if needed. Target hematocrit was 35%. Follow-up was 12 months.No statistical differences in age, associated risk factors, or CRVO characteristics were observed at baseline between both groups. Mean visual acuity was equivalent to 20/80 in the hemodilution group and to 20/63 in the control group (non-significant difference). In the treated group, mean number of hemodilution sessions was 3.3 (range, 1 to 6), and no major side-effects occurred. At the 12-month follow-up visit, 64.5% of the hemodilution group had visual acuity of 20/40 or better compared to 40% of the control group (p = .048). Visual change was a gain of 1.7 ETDRS line in the hemodilution group versus a loss of 2.3 lines in the control group (p = .007). There was less conversion into an ischemic form in the hemodilution group (11%) than in the control group (50%, p = .004). Mean final retinal thickness was 289 μm in the hemodilution group versus 401 μm in the control group (p = .068).This multicenter controlled randomized study demonstrated that automated erythrocytapheresis is a safe and effective tool for performing hemodilution and confirmed that hemodilution therapy can improve the final prognosis of CRVO when applied in the early phase of the disease.

Circumscribed choroidal hemangioma (CCH) is an uncommon benign vascular tumor of the choroid that is usually diagnosed when it causes a decrease in vision due to exudative retinal detachment. The classical treatments of CCH were photocoagulation and radiotherapy, which induced additional chorioretinal damage, explaining the poor visual prognosis even in adequately treated patients. It is difficult to compare these two therapeutic options, laser photocoagulation having been abandoned for many years due to the retractile retinal scars that it induced. Conventional radiotherapy and proton therapy with a total dose of 20 Gy gave and give good results but can be performed only by teams used to these types of treatment. Recently, photodynamic therapy (PDT), which allows a selective occlusion of vascular lesions without damaging overlying retinal structures, has emerged as a sound alternative for the treatment of CCH. Most of the clinicians used the standard treatment of age related macular degeneration by photodynamic therapy (TAP) study protocol with or without variations. The main variations brought to the TAP study protocol were the duration of the verteporfin infusion, and/or the increase of the laser power settings, and/or the increase of the duration of exposure, and/or the number of PDT spots (single spot or multiple spots, overlapping or not). Mid-term PDT results are very encouraging. PDT has been reported to bring about resolution of subretinal fluid and to reduce tumor thickness in almost all cases. Visual acuity was improved or stabilized in at least 80% of the cases. Several studies reported on minor local side effects following PDT, consisting of atrophy and proliferation of the retinal pigment epithelium, atrophy of the choroid, transient choroidal effusion, and mild subretinal fibrosis. Published data demonstrated that PDT is a safe and effective alternative to radiotherapy or photocoagulation as first-line therapy for CCH.

Resume Introduction De faibles taux de luteine et zeaxanthine dans le sang ou l’alimentation sont associes a un risque accru de degenerescence maculaire lie a l’âge. Ces molecules dont l’apport est assure par l’alimentation constituent le pigment maculaire. Patients et Methodes Les patients inclus dans cette etude pilote francaise ont ete repartis en quatre groupes : « Resultats L’evaluation des densites optiques du pigment maculaire en fonction des habitudes alimentaires et des groupes montrait que celle-ci etait plus faible dans le groupe « Conclusion L’analyse des densites optiques moyennes mesurees par l’appareil MPS 9000 QuantifEYE™ en fonction des habitudes alimentaires confirme l’impact de la prise d’un complement alimentaire sur celle-ci, notamment chez les patients âges de 50 ans et plus avec drusen qu’ils presentent ou non des neovaisseaux.

The technique of intravitreous injections has been well documented for several years. Recently, a descriptive article on the intravitreous injections procedure was published in the Journal Francais d'Ophtalmologie, and the AFSSAPS (French agency for drug safety) released recommendations concerning this matter on the occasion of commercial launch of pegaptanib. Since that time, the number of intravitreal injections has considerably increased, because anti-VEGF drugs had been made available to ophthalmologists, and several teams have performed a large number of procedures, allowing them to better comprehend intravitreous injections. The present paper describes our current practice of intravitreous injections. Several specialists have exchanged their experiences and issued a common synthesis. Detailed modifications of the initial recommendations have been suggested, with such basic changes such as abandoning preoperative pupil dilatation and easing postsurgical monitoring. Follow-up examinations should be adapted to each patient rather than being systematic. The suggested modifications do not change the procedure of intravitreous injections substantially, but they simplify many steps and detail the various procedures when consensus is lacking.

Macular edema from various etiologies P.-L. Cornut, C. Francais-Maury, M. Mauget-Faysse Macular edema typically occurs as the final ocular manifestation of many intraocular or systemic diseases with fluid collection within the macular layers due to the breakdown of the blood–retinal barrier. This could be chemically mediated by VEGF, because increased VEGF has been shown to increase blood–retinal barrier permeability, or mechanically induced by vitreomacular traction causing hemodynamic or retinal pigment epithelium pump disturbances. This article reviews the different clinical entities of macular edema, focusing on their physiopathological concepts and on the current therapeutic strategies for their management.

Examination of vitreomacular traction syndrome C. Francais-Maury, P.-L. Cornut, M. Mauget-Faysse Vitreomacular traction syndrome is a complication of partial posterior vitreous detachment: vitreous is separated from the retina throughout the peripheral fundus but remains adherent posteriorly with anteroposterior traction on the macular area and the optic nerve. The functional and anatomical examinations of this condition are based on the following triad: clinical examination, angiography, and OCT.

International audience; Purpose:To determine the rate of reticular pseudodrusen (RPD) in age-related macular degeneration using multimodal imaging, including color fundus photography, the blue channel image of fundus photography, infrared reflectance, fundus autofluorescence, multicolor imaging, and spectral domain optical coherence tomography, as well as to compare the sensitivities and specificities of these modalities for detecting RPD.Methods:This prospective study included 243 eyes from 125 consecutive patients with age-related macular degeneration. They underwent fundus examination including color fundus photography, blue channel, infrared reflectance, fundus autofluorescence, multicolor imaging, and spectral domain optical coherence tomography in both eyes. To be considered as having RPD, eyes had to have reticular patterns on spectral domain optical coherence tomography in a large studied cube of 30 degrees x 25 degrees or on infrared reflectance with at least one other examination.Results:The mean age of the 125 patients was 81.1 years (8.1). Eighty-six patients (68.8%) were diagnosed with RPD. Spectral domain optical coherence tomography, infrared reflectance, and multicolor imaging had the highest sensitivity (99.3, 84.6, and 87.1%, respectively) and specificity (100%). The color fundus photography, blue channel, and fundus autofluorescence had lower sensitivity to detect RPD.Conclusion:Reticular pseudodrusen is frequently associated with soft drusen in patients with age-related macular degeneration. As RPD may be rarely located only in the perifoveal area, spectral domain optical coherence tomography with a larger cube (30 x 25 degrees) than that usually used (20 x 20 degrees) had the highest sensitivity and specificity to detect RPD and is recommended to optimize the rate of detection.

But Les anti-VEGF constituent les traitements les plus recents de la neovascularisation choroidienne compliquant la DMLA. Leur mode d’administration est l’injection intravitreenne. Differentes etudes cliniques ont valide l’interet de ces produits. Cependant, les modalites de surveillance par examen complementaire de l’efficacite de ces traitements n’ont pas ete definies par ces memes etudes cliniques. Le but de cet article est de faire le point sur les pratiques realisees actuellement en France, par differents specialistes, et les examens complementaires necessaires au suivi therapeutique et aux decisions de retraitement. Methode Plusieurs reunions ont ete effectuees entre plusieurs specialistes exercant en milieu hospitalier et/ou en pratique liberale, pour definir leurs modalites actuelles de suivi et leur proposition pour un suivi rationnel et une harmonisation des pratiques. Resultats La tomographie a coherence optique (OCT) occupe une place croissante dans la surveillance post-injection intravitreenne d’anti-VEGF. Tous les auteurs reconnaissent la valeur de l’angiographie a la fluoresceine a l’initiation du traitement, mais aussi trois mois apres instauration du traitement, associee, pour certains d’entre nous, a la realisation d’une angiographie au vert d’indocyanine. Ils recommandent l’utilisation de l’angiographie a la fluoresceine chaque fois que les resultats fonctionnels ne sont pas a la hauteur de ce qu’en attendent le medecin ou le patient. Les auteurs proposent un organigramme decisionnel qui pourrait guider les decisions de retraitement. Conclusion Les auteurs suggerent l’utilisation d’un organigramme decisionnel visant a preciser, en fonction, essentiellement, de l’evolution de l’acuite visuelle et des resultats de l’OCT, les indications de retraitement les plus rationnelles pour eviter aux patients tout risque d’injection intra-vitreenne inutile.

Drugs to treat retinal diseases are often injected intravitreally. This procedure can cause severe complications. We present ways to minimize the risk for complications. Rigorous preoperative antisepsis with povidone iodine, a sterile environment (using gloves and a mask for the injector, a lid speculum, and a drape on the eye to be injected), and immediate follow-up after injection are key issues of the injection technique.

To demonstrate the usefulness of spectral domain optical coherence tomography (SD-OCT) in investigating the evolution of the retinal outer lesions seen in acute macular neuroretinopathy (AMN).A 32-year-old woman presented with a 1-day history of paracentral dark spot in the left eye, preceded by a flulike illness. A full ophthalmologic examination, color vision, visual field examination, and conventional imaging of the retina were done. Scanning laser ophthalmoscopic infrared imaging and SD-OCT of the macular region were performed.Best-corrected visual acuity was 20/20 in both eyes. Slit-lamp examination as well as retinal photographs were unrevealing. Scanning laser ophthalmoscopic infrared examination showed a dark, sharp, oval area in the macular region that corresponded to a rounded patch drawn by the patient on an Amsler grid. The SD-OCT showed focal highly reflective lesion of outer plexiform layer (OPL) extending to the outer nuclear layer. After recovery of the OPL, follow-up SD-OCT showed an associated disruption of the external limiting membrane and the retinal pigment epithelium inner band as well as thinning of the inner segment/outer segment junction. Retinal inner layers were otherwise normal.Spectral domain optical coherence tomography is of particular interest in the diagnosis of AMN. It is also very useful in featuring the chronological events happening in the outer retina. As the lesions shown are confined to the outer layers, this condition should be called acute macular outer retinopathy rather than AMN.

Purpose To report the occurrence and study the characteristics of macular hematoma after ranibizumab (anti-VEGF) intravitreal injection for subfoveal choroidal neovascularization in age-related macular degeneration (AMD). Methods The charts of 6000 patients treated with ranibizumab (0.5 mg) for exudative AMD were reviewed. Inclusion criteria were a minimum follow-up of 1 year after the first injection and the occurrence of a large macular hematoma involving the fovea in patients with macular lesions considered stabilized or still active. All patients had a complete ophthalmologic assessment including Early Treatment of Diabetic Retinopathy Study visual acuity (VA) measurement, fundus photography, fundus fluorescein angiography, scanning laser ophthalmoscopy–infracyanine green angiography, and spectral-domain optical coherence tomography. Results Of the 6000 eyes, 24 (0.4%) developed macular hematoma during follow-up. There were 8 men (33.3%) and 16 women (66.7%). The mean age at the time of initial presentation was 76.7 ± 3.8 years (range 61-81 years). The mean time to occurrence of macular hematoma after the last injection was 4.8 months. Spectral-domain optical coherence tomography showed the presence of a retinal pigment epithelium (RPE) tear in 19 eyes (79.1%). Vitreomacular traction (VMT) was only present in 4 eyes (17%). Final VA after macular hematoma resorption was Conclusions Macular hematoma may follow intravitreal anti-VEGF injection for exudative AMD with large occult neovascularization, especially if a large RPE tear is found. The occurrence does not seem to be linked to anticoagulation treatment or the presence of VMT.

Purpose: To comprehensively evaluate the retinal and choroidal changes in eyes with Type 2 idiopathic macular telangiectasia using “en face” and B-scan spectral domain optical coherence tomography (OCT), and to compare their respective contributions to this evaluation. Methods: Eyes with a diagnosis of proliferative or nonproliferative Type 2 macular telangiectasia were prospectively studied. All patients underwent an extensive ophthalmologic examination including biomicroscopic fundus examination, color photography, fundus autofluorescence, fluorescein angiography, B-scan and en face spectral domain OCT. Results: Twenty eyes of 10 patients were included in this study. En face OCT C-scans and conventional B-scans were both able to show inner crystalline deposits (15%), retinal capillary anomalies (100%), intraretinal cysts (80%), hyperreflective spots in the outer nuclear layer (100%) and external limiting membrane (80%), hyperplastic pigment plaques (30%), intraretinal neovascularization (20%), photoreceptor loss (100%), and choroidal cavitations (30%). En face OCT C-scans provided more information than B-scans on intraretinal neovascularization, photoreceptor loss, and choroidal cavitations. Also, en face OCT C-scans provided better visualization of the retinal vessels and telangiectasia than fluorescein angiography. Conclusion: En face OCT is a noninvasive and reproducible technique that helps to better assess and follow up retinal and choroidal processes in Type 2 macular telangiectasia.

PURPOSE: To assess retinal and choroidal changes in drug-induced retinal toxicity using "en-face" and BScan OCT images. METHODS: All patients underwent complete ophthalmologic examination, including fundus examination and autofluorescence, color photography, fluorescein and indocyanin green angiography. Type, amount and time of drug intake was precisely assessed. RESULTS: 8 patients were analysed and included retrospectively. Retinal toxicity was induced by tamoxifen in 2 cases, by synthetic antimalarial (SAM) drug in 3 cases and by inhaled amyl nitrite ("poppers") in 3 cases. The angiographic features, B-Scan and "en-face" OCT scans findings were correlated. In all cases, "en-face" OCT provided precise anatomic location of the retinal layers involved, the extend of retinal damage, as well as follow-up of these changes. CONCLUSION: "En-face" OCT allows full imaging and better understanding of retinal toxicity induced by tamoxifen, SAM drugs and amyl nitrite.

To evaluate the retinal and choroidal changes in White Dot syndromes (WDS) using ‘’en face’’ and B-scan OCT images. All the study patients underwent a comprehensive ophthalmologic examination including biomicroscopic fundus examination, color photography, fundus autofluorescence, fluorescein angiography, indocyanin green angiography and B scan and "en face" SD-OCT. A total of 10 patients with Multiple evanescent white dot syndrome (MEWDS), 4 cases diagnosed with acute posterior multifocal placoid pigment epitheliopathy (AMPPE), 1 case of serpiginous choroidopathy, 4 cases of multifocal choroiditis, 3 cases of punctuate inner choroidopathy (PIC) and 3 cases diagnosed with Birdshot disease were analysed and included in the study. The B-scan findings were correlated with the angiography findings as well as with the ‘’en face’’ OCT scans. In all the cases the ‘’en face’’ OCT, providing a layer-by-layer view of the involved retina, enabled depicting in detail the IS/OS envolvement and the extent of the outer retinal damage. The patho-physiologic mechanism of the WDS remains poorly understood. All these entities probably share a common pathway leading to external retinal involvem

PURPOSE : To evaluate the retinal and choroidal aspect in retinal or choroidal vascular abnormalities using « en face » OCT images. METHODS : All the study patients underwent a complete ophthalmologic examination including biomicroscopic fundus examination, color photography, fundus autofluorescence, fluorescein angiography, indocyanin green angiography , B-scan and « en face » SD-OCT. RESULTS : A total of 11 patients with choroidal or retinal vascular abnormalities, 4 cases diagnosed with submacular choroidal varix, 2 cases of central serous chorioretinopathy with choroidal cavitations, 2 cases of retinal vein occlusion (RVO) with perivenular whitening and retinal ischemia, 3 cases of retinal arterial macroaneurysm were analysed. The B scan findings were correlated with the angiography findings as well as with the « en face » OCT scans. In all the cases the « en face » OCT providing a layer-by layer view of the involved retina and choroid (with EDI mode: enhanced-depth imaging). CONCLUSIONS : « En face » OCT imaging enables an easy analyse of the structural retinal and choroidal vascular abnormalities which are correlated with B scan OCT and angiography findings.

The authors report the case of a healthy 56-year-old man presenting with bilateral vision loss. Clinical features were consistent with the diagnosis of acute exudative polymorphous vitelliform maculopathy (AEPVM). The patient returned 10 days later with bilateral anterior granulomatous uveitis, and the inflammatory work-up revealed treponemal antibodies in the serum and spinal fluid, consistent with a diagnosis of active neurosyphilis. The patient received standard treatment for neurosyphilis with intravenous penicillin G. Two months later, the intraocular inflammation had resolved, but the resolution of the vitelliform lesions was more gradual. An immune process could be a plausible explanation for these clinical findings. Clinicians should be aware that syphilis can produce AEPVM. [ Ophthalmic Surg Lasers Imaging Retina. 2014;45:e29–e31.]

Devices for time-domain optical coherence tomography and, more recently, spectral-domain optical coherence tomography (spectral OCT) with an improved depth resolution of 7 μm (27,000–400,000 scans per second) carry out a noninvasive and in vivo visualization of the retinal layers, particularly the outer nuclear layer (ONL), the photoreceptor inner segment/outer segment junction (IS/OS), and the retinal pigment epithelium (RPE). Hence, the integrity or abnormality of the retinal pigment epithelium, the inner/outer segment junction, and the external limiting membrane can be assessed and graduated even during early stages of inherited retinal diseases, when the fundus appears normal on examination.

PURPOSE Optic atrophy constitutes the final stage in the evolution of optic neuropathy. The aim of this study was to describe the presence of macular microcystic changes or pseudocysts in patients with advanced optic atrophy. METHODS The medical records of 24 patients who had retinal pseudocysts in association with optic atrophy have been analyzed. All patients underwent a complete neuro-ophthalmologic assessment; peripapillary retinal nerve fiber layer thickness and macular screening with spectral-domain optical coherence tomography and optical coherence tomography "en face" imaging analysis were also performed. RESULTS A total of 36 eyes were included in the study. Patients' mean age was 37 years. The major cause of optic atrophy was glaucoma (12 cases). The retinal pseudocysts were observed as hyporeflective lesions in the internal nuclear layer. Infrared images revealed a hyporeflective circular or semilunar shape corresponding to the location of the pseudocysts in all cases. In eyes with pseudocysts, mean thickness of the peripapillary retinal nerve fiber layer was statistically significantly less than that of fellow eyes (P = 0.0003), whereas macular thickness was statistically significantly higher compared with fellow eyes (P < 0.005). CONCLUSION The presence of pseudocystic lesions always associated with severe optic nerve fiber loss is reported. The reason why pseudocystic lesions develop within the retina is not well understood. They might constitute the translation of degeneration of Muller cells in severe optic nerve fiber loss. Recognizing these pseudocysts is crucial because they may be confused with cystoid macular edema. Their prognostic value and role in the therapeutic process need to be further evaluated with prospective studies and molecular experiments in vivo.

Purpose. To report an association between reticular pseudodrusen, located above the retinal pigment epithelium (RPE), and Early Onset Drusen (EOD) as described using Spectral-Domain Optical Coherence Tomography (SD-OCT). Methods. Eight patients (16 eyes) with EOD were examined. EOD were classified into three entities called Large Colloid Drusen (LCD), Malattia Leventinese (ML), and Cuticular Drusen (CD). Best-corrected visual acuity, fundus examination, color fundus photographs, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and SD-OCT were performed in all study patients. Results. Four patients had LCD, 2 had ML, and 2 had CD. Reticular pseudodrusen were observed with SD-OCT in all study patients; all these patients had hyperreflective lesions above and below the RPE. Conclusion. Early Onset Drusen appear to be associated with reticular pseudodrusen. SD-OCT is helpful in distinguishing the location of the deposits that are above and below the RPE in EOD. Further studies are needed to understand the role of reticular pseudodrusen in the pathophysiology of EOD.

The term ‘white dot syndromes’ (WDS) refers to several inflammatory diseases of the retina and choroid caused by immune dysregulation. They consist of the following disorders, with overlapping clinical features: • Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) • Serpiginous choroidopathy • Multiple evanescent white dot syndrome (MEWDS) • Birdshot retinochoroidopathy • Acute retinal pigment epitheliitis (ARPE) • Multifocal choroiditis and panuveitis syndrome (MCP) • Punctuate inner choroidopathy (PIC), and • Acute zonal occult outer retinopathy (AZOOR) These conditions usually occur following an influenza-like illness, but their patho-physiologic mechanism remains poorly understood. The white dot syndromes affect more frequently young females and individuals with mild myopia, and present as white or yellow, deep, round lesions in the central fundus. Their size and number can vary between each entity, as well as their unior bilateral involvement.

International audience; Objective: To evaluate the relative efficacy and safety profile of bevacizumab versus ranibizumab intravitreal injections for the treatment of neovascular age-related macular degeneration (AMD). Design: Multicenter, prospective, noninferiority, double-masked, randomized clinical trial performed in 38 French ophthalmology centers. The noninferiority limit was 5 letters. Participants: Patients aged >= 50 years were eligible if they presented with subfoveal neovascular AMD, with best-corrected visual acuity (BVCA) in the study eye of between 20/32 and 20/320 measured on the Early Treatment of Diabetic Retinopathy Study chart and a lesion area of less than 12 optic disc areas (DA). Methods: Patients were randomly assigned to intravitreal administration of bevacizumab (1.25 mg) or ranibizumab (0.50 mg). Hospital pharmacies were responsible for preparing, blinding, and dispensing treatments. Patients were followed for 1 year, with a loading dose of 3 monthly intravitreal injections, followed by an asneeded regimen (1 injection in case of active disease) for the remaining 9 months with monthly follow-up. Main Outcome Measures: Mean change in visual acuity at 1 year. Results: Between June 2009 and November 2011, 501 patients were randomized. In the per protocol analysis, bevacizumab was noninferior to ranibizumab (bevacizumab minus ranibizumab +1.89 letters; 95% confidence interval [CI], -1.16 to +4.93, P< 0.0001). The intention-to-treat analysis was concordant. The mean number of injections was 6.8 in the bevacizumab group and 6.5 in the ranibizumab group (P=0.39). Both drugs reduced the central subfield macular thickness, with a mean decrease of 95 mm for bevacizumab and 107 mm for ranibizumab (P=0.27). There were no significant differences in the presence of subretinal or intraretinal fluid at final evaluation, dye leakage on angiogram, or change in choroidal neovascular area. The proportion of patients with serious adverse events was 12.6% in the bevacizumab group and 12.1% in the ranibizumab group (P=0.88). The proportion of patients with serious systemic or ocular adverse events was similar in both groups. Conclusions: Bevacizumab was noninferior to ranibizumab for visual acuity at 1 year with similar safety profiles. Ranibizumab tended to have a better anatomic outcome. The results are similar to those of previous head-to-head studies. (C) 2013 by the American Academy of Ophthalmology.

Purpose. “En face” is an emerging imaging technique derived from spectral domain optical coherence tomography (OCT). It produces frontal sections of retinal layers, also called “C-scan OCT.” Outer retinal tubulations (ORTs) in age-related macular degeneration (AMD) are a recent finding evidenced by spectral-domain OCT. The aim of this study is to characterize the morphology of ORT according to the form of AMD, using “en-face” spectral domain OCT.Methods. “En face” OCT imaging was prospectively performed in 26 consecutive eyes with AMD that also had ORT.Results. There were 15 neovascular, 8 atrophic, and 3 eyes with a mixed (fibrotic and atrophic) form of AMD. Among the neovascular group, the most frequent tubulation pattern on “en-face” OCT was a branching network emanating from a fibrovascular scar; we term this pattern as “pseudodendritic.” It did not require treatment when observed as an isolated finding. In all cases of atrophic AMD, the tubular network was located at the edge of the geographic atrophy area, and formed a “perilesional” pattern. Six atrophic cases showed tubular invaginations inside this area.Conclusion. “En face” OCT is a valuable technique in the diagnosis and followup of macular disease. It revealed the main characteristic patterns of ORT associated with neovascular and atrophic AMD.

International audience

Purpose To examine the association of low serum 25-hydroxyvitamin D (25OHD) concentration with age-related macular degeneration (AMD). Based on a case–control study. 31 patients with AMD (i.e., early, intermediate and advanced AMD) and 34 control patients without AMD were prospectively recruited. Ophthalmological examination, and funduscopic analysis was performed to determine AMD stage and serum 25OHD concentration was measured for each patient. Methods Hypovitaminosis D was defined by serum 25OHD concentration < 50nmol/L. Age, gender and season of blood collection were used as potential confounders. Results Compared to 28 subjects with normal 25OHD status (i.e., ≥50nmol/mL), subjects with hypovitaminosis D (n=37) had more often AMD (P=0.029) all stages confused. Hypovitaminosis D was associated with AMD (unadjusted odds ratio (OR)=3.10 with P=0.031; adjusted OR=3.03 with P=0.041 for full model; adjusted OR=3.10 with P=0.031 for stepwise backward model). Conclusion Hypovitaminosis D may be associated with AMD.

To evaluate the efficacy and safety of verteporfin photodynamic therapy (V-PDT) for young adults and children with subfoveal choroidal neovascularization (CNV) associated with toxoplasmic retinochoroiditis.Patients with subfoveal CNV associated with toxoplasmic retinochoroiditis were treated with V-PDT and prospectively followed up. Before V-PDT and during follow-up, patients underwent visual acuity testing, complete ophthalmic examination including color photography, angiography with fluorescein and/or indocyanine green, and optical coherence tomography. The decision to retreat CNV was based on the criteria used in the Treatment of Age-Related Macular Degeneration with Photodynamic Therapy investigation.Eight patients (5 males and 3 females) were treated at a mean age of 15.3 years (range, 5-31 years). CNV was 100% classic or predominantly classic in all study patients. Mean visual acuity increased from 20/225 (range, 20/400 to 20/50) to 20/123 (range, 20/200 to 20/25) during a mean follow-up period of 25 months (range, 5-49 months). Persistent closure of CNV was achieved in all eight patients (mean number of treatments, 1.75). Vascular anastomosis developed in the treated area in two patients, but there was no additional visual loss. No significant adverse effects of V-PDT were observed.V-PDT for subfoveal CNV associated with toxoplasmic retinochoroiditis appears to be effective and safe even in young adults and children. However, a longer follow-up is recommended to confirm our observations.

Purpose To describe polypoidal choroidal vasculopathy as a complication of tilted disk syndrome and high myopia with staphyloma. Design Retrospective interventional case series. Methods This report was a multicenter evaluation of six patients (eight eyes) with tilted disk syndrome or high myopia that was complicated by posterior staphyloma. Complete ophthalmic examination that included fluorescein angiography, optical coherence tomography (OCT), and indocyanine green angiography (ICG-A) was performed in all patients. Results All patients had macular abnormalities and visual loss. Fundus examination and fluorescein angiography showed typical features of tilted disk syndrome (five patients; six eyes) or high myopia (one patient; two eyes) with staphyloma that was associated with polypoidal choroidal vasculopathy. OCT and ICG-A confirmed the presence of polypoidal dilations in the choroid. Seven eyes were treated with laser photocoagulation or verteporfin-photodynamic therapy (V-PDT), although one eye did not require treatment. Visual acuity at the final visit had improved in three eyes, deteriorated in three eyes, and remained unchanged in two eyes. Conclusion Polypoidal choroidal vasculopathy is a potential cause of visual loss in tilted disk syndrome and high myopia. We postulate that choroidal abnormalities at the border of staphylomas induce blood-flow disturbances that are similar to those disturbances that are observed in chronic central serous chorioretinopathy, which is another condition that occasionally is associated with polypoidal choroidal vasculopathy. The pathogenesis remains unclear, and further study is required to better understand the formation of choroidal polypoidal dilations in these conditions.

Idiopathic central serous chorioretinopathy (ICSC) is a disease that typically affects middleaged adults and involves the sensory retina, the retinal pigment epithelium (RPE) and the choroid. Patients usually have mild visual loss. ICSC generally resolves without therapy, although the disease can become chronic with ensuing RPE decompensation. Some patients, particularly older adults, can also develop choroidal subretinal neovascularisations (CNV), which may lead to a severe loss in visual acuity. Although the aetiopathogenesis of the disease is still incompletely understood, a correlation with psychophysical stress supports the idea that the disease may be "adrenergically conditioned", leading to the development of one or several defects in the RPE, with subsequent focal leakage of serous fluid and its retention in the subretinal space. An association between ICSC and the Helicobacter pylori (HP) infection has also been recently documented, suggesting that this organism may possibly be involved in the development of some cases of ICSC. Pathogenetic mechanisms that may explain the contribution of HP in the development of ICSC are postulated.