Incidence and clinical significance of fovea plana in the French population with age-related cataract.

BACKGROUND: Fovea plana is defined as an immature macula diagnosed by OCT, showing the unusual shunt of the inner retinal layers into the fovea. The incidence of fovea plana in the adult population remains to be determined. The aim of this study was to determine the incidence of fovea plana in the French population with age-related cataract. METHODS: Consecutive patients who underwent cataract surgery in Rothschild Foundation Hospital, France, between January and March 2021, with preoperative analyzable OCT scans available, were retrospectively screened in order to determine the incidence of fovea plana in these population. Ophthalmological characteristics of patients were reported, and detailed. RESULT: Fovea plana was encountered in 20 out of 204 patients during the 3 months corresponding to an incidence of 9.8%. One of those patients had stage 2 fovea plana. CONCLUSION: Although fovea plana is defined as an immature macula, it is not rare in preoperative population. This macular aspect was not associated with poor visual acuity in our cohort.

Foveal hypoplasia in parents of patients with albinism.

BACKGROUND: Albinism is a group of genetic disorders characterized by general skin and retinal hypopigmentation. It is in most cases an autosomal recessive condition. Foveal hypoplasia (FH) is one of the main criteria for the diagnosis of albinism. The aim of this study was to analyze the macular profile of the parents of patients with albinism. METHODS: This study included a case series of 27 patients with albinism seen in Rothschild Foundation between April 2017 and February 2020. Spectral-domain optical coherence tomography (SD-OCT) and OCT angiography (OCT-A) were performed in every patient when possible and in every available parents. FH was graded according to Thomas' classification based on OCT. Next generation sequencing-based gene panel testing was performed in parents and children when a FH was detected on OCT in a parent. RESULTS: Twenty-seven patients with albinism were examined. Nine parents had FH based on the OCT B-scan (33%). In parents without FH based on the SD-OCT B-scan (67%), OCT-A showed a reduced avascular zone in the deep vascular plexus in 4 parents. Six parents carried variants that could explain their phenotype, including TYR R402Q hypomorphic alleles. CONCLUSION: This study showed the presence of FH in parents of patients with albinism, and aimed to genetically explain this phenotype.

[Delays in the management of ocular complications of giant cell arteritis: A retrospective monocentric study of 33 patients]. / Délais de prise en charge des complications visuelles d'artérite à cellules géantes : étude rétrospective monocentrique de 33 patients.

INTRODUCTION: Ocular complications of giant cell arteritis (GCA) can lead to irreversible bilateral blindness and represent a therapeutic emergency. Recommendations for the management of GCA have recently been updated. The objective of the study was to evaluate delays in appropriate management of the ocular complications of GCA and its determinants. METHOD: Retrospective, monocentric study, conducted over the period January 2013-November 2018. All consecutive patients with a final diagnosis of GCA and related visual impairment (permanent visual loss and/or alteration of visual field) were included. RESULTS: Thirty-three patients were included (women: 21, men: 12; mean age at diagnosis: 79). Twenty-seven patients (82%) presented with symptoms suggestive of ACG prior to the visual complication, ranging from a few weeks to several months. Seventeen patients (52%) had a known biological inflammatory syndrome (median CRP at 64 mg/L) prior to hospital consultation. The median time from the onset of permanent ophthalmologic manifestations to appropriate corticosteroid management was 3 days (range: 0-134). Two of the 21 patients who consulted an out-of-hospital ophthalmologist received corticosteroid therapy before referral to hospital. Three patients (9%) were treated within 24 h of the onset of the disorders. CONCLUSION: There is a significant delay in the appropriate management of ophthalmological complications of ACG and deviations from current recommendations. Numerous actions must therefore be taken to improve the visual prognosis of patients with ACG, both preventively (i.e. early diagnosis and treatment of ACG before the possible occurrence of visual complications), and curatively (rapid recognition and immediate treatment of ocular complications). These elements support the relevance of specific fast-track pathways for GCA.

Approche diagnostique et thérapeutique de la vasculopathie polypoïdale choroïdienne. Recommandations de la Fédération France Macula.

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.

Analysis of autofluorescence pattern in birdshot chorioretinopathy.

PURPOSE: To characterize and correlate the different patterns of fundus autofluorescence (FAF) in patients with birdshot chorioretinopathy (BSCR), with functional and anatomical parameters. METHODS: 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. RESULTS: 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%). CONCLUSIONS: Autofluorescence analysis in BRSC patients contributes to evaluating disease activity and could be useful to guide follow-up and treatment.

Macular pigment density variation after supplementation of lutein and zeaxanthin using the Visucam® 200 pigment module: Impact of age-related macular degeneration and lens status.

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 (5mg of Lutein and 1mg 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<0.05). No statistically significant increase in MPOD was noted in either group, even after discontinuation of the supplementation. By multiple regression analysis, age, female gender, lens status and the presence of AMD seemed to significantly affect MPOD measurements. 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.

[Intravitreal ranibizumab for management of choroidal neovascularization secondary to angioid streaks: a case report]. / Traitement par injection intravitréenne de ranibizumab de néovaisseaux choroïdiens compliquant des stries angioïdes : à propos d'un cas.

Angioid streaks are biomicroscopically observable manifestations that frequently lead to choroidal neovascularization. Traditional treatments used to include laser photocoagulation or photodynamic therapy. Over the past few years, anti-VEGF therapies have been used as an alternative treatment. The case of a 54-year-old patient who received anti-VEGF therapy (ranibizumab) for the treatment of choroidal neovascularization secondary to angioid streaks is reported. The patient received two injections that led to complete resolution of intraretinal fluid and reduction in lesion size. After 1 year of follow-up, the patient has presented no recurrence. This case illustrates the efficacy of intravitreal anti-VEGF therapy for choroidal neovascularization in angioid streaks. Further prospective studies on a larger number of patients should help establish the best treatment and follow-up strategies.

[Photodynamic therapy for chronic central serous chorioretinopathy and diffuse retinal epitheliopathy]. / Intérêt de la photothérapie dynamique dans les choriorétinopathies séreuses centrales chroniques et les épithéliopathies rétiniennes diffuses exsudatives.

PURPOSE: To report outcomes in patients with long-standing (more than 6 months) chronic central serous chorioretinopathy (CSC) treated with low-fluence Visudyne(®) photodynamic therapy (LFV-PDT). PATIENTS AND METHODS: The clinical, angiographic and optical coherence tomography (OCT) results of patients with long-standing chronic central serous chorioretinopathy (CCSC) treated with LFV-PDT in the Lyon Centre Rabelais between 2002 and 2008 were retrospectively analyzed. A comprehensive check-up (macular syndrome signs, ETDRS best-corrected visual acuity [BCVA], biomicroscopy, fluorescein [FA] and indocyanine green [ICGA] angiographies, OCT scans) was performed before LFV-PDT treatment and 3 months later. Patients were then followed regularly and retreated in case of recurrence. The LFV-PDT treatment, with a fluence of 25 J/cm(2) at an irradiance of 300 mW, was guided by ICGA. RESULTS: Forty-one eyes of 34 patients (27 males; mean age: 53 years) were included, of which 18 eyes had already been treated with laser photocoagulation. Several leaking points were visible on FA in most of the cases (n=38), mainly in the macula (35 cases). Before treatment, metamorphopsia was noted in 51% of the cases, intraretinal edema (IRE) was present on OCT scans in 71%, serous retinal detachment (SRD) in 85%, and pigment epithelial detachment (PED) in 10%. Thirty-nine eyes had only one treatment session and one eye was retreated once. At 3 months after LFV-PDT, IRE was present in 15% of the cases, SRD in 12%, and PED in 2%. At the end of the 20-month follow-up, IRE was present in 14% of the cases, SRD in 15%, and PED in 0%. Macular atrophy was observed on OCT in most of the cases at the end of the follow-up (mean central thickness, 144.5 µm). Compared to the initial BCVA, at 3 months after LFV-PDT, BCVA decreased in 22% of the cases, stabilized in 39%, and increased in 39%, while at the end of the follow-up, BCVA decreased in 12% of the cases, stabilized in 17%, and increased in 71%. No complication was observed. DISCUSSION: LFV-PDT treatment for patients with long-standing chronic central serous chorioretinopathy results in anatomical and functional improvement (sustained disappearance of the exudative phenomenon in most cases and increased BCVA in more than two-thirds of the cases). The macular atrophy observed may be due to the treatment or the natural course of the disease.

[Role of intolerance to hypoxia in the occurrence of anterior bilateral ischaemic optic neuropathy at high altitude]. / Survenue d'une neuropathie optique ischémique antérieure aiguë bilatérale en haute altitude: rôle de l'intolérance à l'hypoxie.

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).

[Outer retinal cysts in exudative age-related macular degeneration: a spectral domain OCT study]. / Kystes rétiniens externes dans la dégénérescence maculaire liée à l'âge exsudative: un nouvel aspect de l'OCT.

PURPOSE: To describe the optical coherence tomography (OCT) findings and progression of peculiar retinal cysts that we identified in patients being followed up after anti-vascular endothelial growth factor (anti-VEGF) treatment. METHODS: This is an observational case series. All relevant data (including best-corrected visual acuity and spectral domain OCT scans) concerning exudative age-related macular degeneration (AMD) patients treated with at least three anti-VEGF intravitreal injections (IVT) during the previous 12 months were collected over a period of four months. RESULTS: A total of 376 consecutive choroidal neovascularization (CNV) patients (398 eyes) were examined. Of these patients, 18 (18 eyes, 4.5%), who underwent a mean of five (range, 3 to 15) anti-VEGF IVTs, had a cystic appearance of the retina on OCT scans. These cysts were usually multiple (2 to 7) and presented as optically empty spaces bordered by a mildly reflective rim. Tiny punctate spots were seen inside or along the inner border of the cyst. The presence of these two features allowed the differentiation of these cysts from CME cavities. Usually round, the cysts could be elongated in shape and simulate a serous retinal detachment (SRD). A thin layer of degenerate retina below the cysts helps differentiate them from SRD. The cysts, varying in size from 60 to 600 µm, were always located below the outer plexiform layer and visualized over or contiguous to a fibrous and hyperreflective thickening of the choriocapillary/retinal pigment epithelial (CC/RPE) complex or over an atrophic portion of the CC/RPE complex. Their size did not change over time. CONCLUSIONS: These retinal cysts are a newly reported SD-OCT finding in anti-VEGF-treated exudative AMD. They could correspond to active scavenger macrophages and must be differentiated from CME and SRD in order to avoid unnecessary anti-VEGF retreatment.

[Treatment of circumscribed choroidal haemangiomas by photodynamic therapy: a review]. / Traitement des hémangiomes choroïdiens circonscrits par photothérapie dynamique : revue de la littérature.

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.

[Impact of eating habits on macular pathology assessed by macular pigment optical density measure]. / Impact des habitudes nutritionnelles sur la pathologie maculaire évalué par mesure de la densité optique du pigment maculaire.

INTRODUCTION: Low levels of lutein and zeaxanthin in blood or food are associated with an increased risk of age-related macular degeneration (AMD). These molecules, provided by food, form the macular pigment. PATIENTS AND METHODS: Patients included in this pilot study where categorized into four groups : (1) < 50 years with drusen, (2) > or = 50 years without drusen, (3) > or = 50 years with drusen, and (4) > or = 50 years with drusen and neovascularization. During consultation, macular pigment optical density was measured and information on pathology and eating habits were collected. RESULTS: Assessment of macular pigment optical density considering eating habits and groups showed that it was lower in group 1 patients when they ate less than five portions of fruits and vegetables per day and less than two portions of cabbage, broccoli, pepper, corn, or spinach a week. In groups 3 and 4, food supplement intake was related to an increase in optical density. Food supplements were consumed by 58.5 % of patients in group 4. CONCLUSION: Analysis of mean optical density measured by the MPS 9000 QuantifEYE considering eating habits confirmed the impact of food supplement intake on optical density, especially in patients > or = 50 years with drusen and with or without neovascularization.

Hypoxia tolerance and retinal vein occlusion: a pilot evaluation.

PURPOSE: To determine if hypoxia tolerance in patients with retinal vein occlusion (RVO) following exposure to transient hypoxia is different from the hypoxia tolerance of healthy patients without retinal vein occlusion. METHODS: Consecutive patients presenting with RVO following exposure to transient hypoxia (Group I) were compared with healthy subjects (Group II). In addition to cardiovascular and plasma tests, functional respiratory evaluation was performed at rest and during exercise at both normal oxygen levels (21% O2) and in hypoxia (11.6% O2). We used the Wilcoxon test for statistical analysis. RESULTS: Both groups of eight males had similar mean ages: Group I, 47.5 years and Group II, 53 years. In Group I, three patients had glucose or lipid abnormalities, one had hypertension, and one minor thalassanemia. In Group II, one patient had hypertension. At rest in hypoxia, the oxyhemoglobinic desaturation was significantly different (p=0.03) in Group I in comparison with Group II (-13.8 versus -9.3). At exercise in hypoxia, the oxyhemoglobinic desaturation was similar in both groups but there was a statistically significant increase in both systolic (189 versus 155 mmHg; p=0.01) and diastolic (94 versus 77 mmHg; p=0.03) blood pressure in Group I. Ventilation rate and increased heart rate during hypoxia were higher in Group I compared with Group II but were not statistically significant. CONCLUSIONS: In our pilot study, patients with RVO following exposure to transient hypoxia demonstrated intolerance to hypoxia and were significantly different from healthy subjects in their response to hypoxia. A larger study is required to confirm these preliminary results.

[Evolving intravitreous injection technique]. / Evolution des pratiques pour la réalisation des injections intravitréennes.

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 Français 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.

[Outcome of photodynamic therapy for subretinal neovascularization secondary to choroidal nevi]. / Résultats de la photothérapie dynamique dans le traitement des néovaisseaux sous-rétiniens secondaires aux naevi choroïdiens.

INTRODUCTION: Choroidal nevi are the most common benign intraocular tumors. However, the occurrence of subretinal neovascularization secondary to choroidal nevus is very rare. Our goal was to assess the efficacy and the safety of photodynamic therapy (PDT) for treatment of subretinal neovascularization related to choroidal nevi. PATIENTS AND METHOD: We conducted a retrospective study including subretinal neovascularization secondary to choroidal nevi treated by PDT from 1999 to 2005. PDT was performed according to the standard protocols used for treating neovascularization due to age-related macular degeneration (ARMD). Post-PDT follow-up was also done according to the guidelines used in ARMD. Moreover, tumors were followed up every 3 months. RESULTS: Six subretinal neovascularizations secondary to choroidal nevi were included in our study. All nevi were localized in the posterior choroids. The median diameter of tumors was 3mm and their thickness was less than 2mm in all cases. Subretinal neovascularizations were classic in five cases. The last case was a small vascularized pigment epithelium detachment. Locations were subfoveal in two cases, juxtafoveal in three cases, and extrafoveal in one case. In half the cases, the size of subretinal neovascularization was 1-disk-diameter or less. A mean of 3.3 sessions of PDT were necessary to obtain the stabilization of subretinal neovascularization on both fluorescein angiography and OCT. The average follow-up period was 35 months with no patients lost to follow-up. Visual outcomes were extremely variable. Large or subfoveal neovascularization but also a history of atrophic ARMD were related to worse functional results. No growth of the nevi was observed. CONCLUSIONS: Subretinal neovascularization is reassuring from the oncologist's point of view because it is considered a relative indicator of benignity of the nevus. However, it often marks a definitive turning point in visual function. PDT appears to be a safe and effective procedure for the treatment subretinal neovascularization secondary to choroidal nevus, with results similar to those observed in ARMD.

[Examination of vitreomacular traction syndrome]. / Bilan actuel d'un syndrome de l'interface maculaire.

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.

[Macular edema from various etiologies]. / Diversité des oedèmes maculaires.

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.