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Purpose: To describe the natural course of adult-onset foveomacular vitelliform dystrophy using spectral-domain optical coherence tomography (SD-OCT)., Design: Retrospective study., Methods: We reviewed the charts of all consecutive patients with adult-onset foveomacular vitelliform dystrophy who underwent SD-OCT at baseline and at least 12 months later (last visit). Main outcome measures were changes of clinical and SD-OCT features over time., Results: Forty-six eyes (31 patients, 15 male and 16 female; mean age 74.6 ± 8.2 years) were included. Follow-up was 16.2 ± 6 (range, 12-30) months. Visual acuity (VA) reduced from 0.32 ± 0.22 logMAR at baseline to 0.39 ± 0.28 logMAR at last visit (P=.03). The stage of the disease was vitelliform in 28 eyes (60.8%), pseudohypopyon in 7 eyes (15.2%), vitelliruptive in 11 eyes (23.9%) at baseline; vitelliform in 23 eyes (50%), pseudohypopyon in 5 eyes (10.9%), vitelliruptive in 13 eyes (28.2%), and atrophic in 5 eyes (10.9%) at last visit. Stabilization of the disease stage, inner segment/outer segment (IS/OS) interface status, and lesion reflectivity on SD-OCT determined no VA changes (P>.05), while their worsening determined a reduction of VA (P=.03). In eyes that presented a progression of the disease stage, mean central macular thickness, maximal thickness of the lesion, and maximal width of the lesion showed a significant change (from 404.1 ± 107.6 μm to 246.1 ± 74.0 μm, P = .004; from 277.0 ± 80.8 μm to 105.3 ± 92.3 μm, P=.001; from 2324.2 ± 1250.3 μm to 1751.0 ± 858.3 μm, P = .04, respectively)., Conclusions: In adult-onset foveomacular vitelliform dystrophy, progression of the lesion stage (partial/complete resorption of the material) is generally accompanied by IS/OS interface disruption/loss and visual impairment., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To determine the risk of progression to advanced age-related macular degeneration (AMD) conferred by reticular pseudodrusen (RPD), an imaging presentation of reticular macular disease (RMD), in high-risk fellow eyes of subjects with AMD and unilateral choroidal neovascularization (CNV) in a large, prospective study., Design: Cohort study., Participants: Two hundred seventy-one subjects with AMD; 94 with RPD and 177 without RPD., Methods: Images from a cohort of 271 subjects with AMD in the Nutritional AMD treatment phase II (NAT 2) Study, a 3-year prospective study of subjects with unilateral CNV and large soft drusen in the fellow eye, were studied. The fellow eye, at high risk for advanced AMD developing, was the study eye. There were 5 visits per subject. Imaging at each visit consisted of color, red-free, and blue-light photography and fluorescein angiography. The images were analyzed for the presence of RPD, following disease progression throughout the 3-year study., Main Outcome Measures: The development of advanced AMD (CNV or geographic atrophy)., Results: For the 271 subjects who completed the full 3-year study, there was a significantly higher rate of advanced AMD (56% or 53/94) in fellow eyes with RPD at any visit compared with eyes without RPD (32% or 56/177; P < 0.0001, chi-square test; relative risk [RR], 1.8; 95% confidence interval [CI], 1.4-2.4). The chance of developing advanced AMD in the fellow eye in women with RPD (66%) was more than double that of women without RPD (30%; P < 0.00001; RR, 2.2; 95% CI, 1.6-3.1)., Conclusions: To the authors' knowledge, this is the first comprehensive prospective study of RMD, a distinct clinical phenotype of AMD that includes RPD. It provides strong confirmation that RMD, a disease entity with stereotypical presentations across imaging methods, is associated with a high risk of progression to advanced AMD, perhaps on an inflammatory or vascular basis. Reticular macular disease deserves wider recognition and consideration by clinicians caring for patients with AMD., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To investigate the prevalence and clinical significance of cystoid macular degeneration in eyes that underwent intravitreal ranibizumab injections for exudative age-related macular degeneration., Design: Retrospective, interventional case series., Methods: We reviewed the charts of 56 consecutive patients (19 male, 37 female; mean age ± standard deviation, 80.81 ± 4.8 years) with exudative age-related macular degeneration who received the last intravitreal ranibizumab injection at least 6 months before and were judged to have a fibroatrophic scar without signs of progression by fluorescein angiography or spectral-domain optical coherence tomography. Main outcome measures were the estimated prevalence and clinical significance of cystoid macular degeneration., Results: Twenty-two eyes showed various combinations of degenerative pseudocysts, whereas 34 eyes did not show any pseudocysts. The 95% confidence interval for the prevalence estimate was 36.98% to 41.02%. Degenerative pseudocysts appeared square-shaped, did not change their overall appearance over time, and were located just below the internal limiting membrane in 11 eyes (50%), in the inner nuclear layer in 16 eyes (72.7%), in the outer nuclear layer in 8 eyes (36.3%), and in all the retinal layers in 6 eyes (27.2%). Best-corrected visual acuity improved in eyes with and without degenerative pseudocysts and decreased significantly in eyes with degenerative pseudocysts (P = .03). Mean central macular thickness decreased significantly (P < .001) to 324.1 μm and to 328.2 μm in eyes and without degenerative pseudocysts, respectively., Conclusions: Cystoid macular degeneration represents a well-distinguished clinical entity that may be detected in exudative age-related macular degeneration eyes showing a posttreatment fibroatrophic scar and should not be considered as a manifestation of choroidal neovascularization activity., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Aim: To investigate the ability of the preferential hyperacuity perimeter test to assess responsiveness to ranibizumab therapy for exudative age-related macular degeneration (AMD)., Methods: Fourteen consecutive patients with newly diagnosed choroidal neovascularisation underwent a preferential hyperacuity perimeter metamorphopsia test (main outcome measures) 1 h before (baseline) and 1 h, 1 day, 1 week and 1 month after one intravitreal injection of ranibizumab (0.05 ml/0.5 mg). Best corrected visual acuity (BCVA) and several spectral domain optical coherence tomography (OCT) parameters (secondary outcome measures) were compared with the metamorphopsia test., Results: Fourteen eyes (14 patients, 78% women, mean age 83 ± 6.2 years) were included in the analysis. The mean preferential hyperacuity perimeter metamorphopsia test score improved significantly from 20.4 ± 35 at baseline to 9.2 ± 23 after the single ranibizumab injection (p < 0.05). The mean reduction in central macular thickness, maximal retinal thickness at the fovea, maximal height of subretinal fluid, maximal diameter of the largest retinal cyst and maximal height of pigment epithelial detachment, as evaluated by spectral domain OCT, closely reflected the functional improvements as evaluated by preferential hyperacuity perimeter, showing a significant correlation with metamorphopsia changes (Spearman correlation 0.9, p < 0.05). Mean BCVA improved significantly from 20/80 to 20/60 (p < 0.05). A significant correlation was also found between the mean BCVA changes and the mean metamorphopsia changes (Spearman correlation 0.97, p < 0.05). The correlation coefficient between OCT measurements and preferential hyperacuity perimeter score within subjects was 0.51 (p < 0.05)., Conclusion: The improvement in metamorphopsia test score after intravitreal ranibizumab injection, which correlated closely with improvement in several OCT parameters, suggests that the preferential hyperacuity perimeter test may be used to monitor the response to anti-vascular endothelial growth factor (VEGF) treatment in patients with exudative AMD.

Purpose: To describe the morphologic and functional characteristics of subclinical Best vitelliform macular dystrophy (VMD) in subjects with mutation in the BEST1 gene., Methods: Best-corrected visual acuity (BCVA), funduscopic appearance, fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT), and electro-oculography (EOG) were assessed in 23 consecutive subjects from nine unrelated families with known mutations in the BEST1 gene (eight distinct BEST1 mutations)., Results: Six subjects were identified with BEST1 mutations (three male, three female; aged 8 to 30 years) without clinically detectable (subclinical) Best VMD (absence of both symptoms and funduscopic lesions). All six subjects showed 20/20 BCVA and normal FAF findings. In these 6 of 26 subjects from five different families, we found five distinct mutations in the BEST1 gene. In three (six eyes) out of these six subjects with BEST1 gene mutations (two families: p.G15D; p.A243V), SD-OCT showed overall normal findings. In the other three subjects (six eyes) with BEST1 gene mutations (three families: p.V9A; p.R92C; p.I230T), we found, on SD-OCT, a thicker and more reflective appearance of the layer between the retinal pigment epithelium and the interface of inner segments and outer segments of the photoreceptor (the Verhoeff's membrane). EOG showed a reduced light-peak:dark-trough ratio in 5 of 12 eyes. Changes on SD-OCT were present in the absence of EOG abnormalities (two of six eyes), and vice versa (one of six eyes)., Conclusions: Subclinical Best VMD (absence of both symptoms and funduscopic lesions) in subjects with BEST1 mutation may vary from the absence of any morphologic and functional abnormalities to the presence of specific SD-OCT and EOG changes.

Purpose: To determine the visual field patterns obtained by the preferential hyperacuity perimetry (PHP) in patients with Best vitelliform macular dystrophy with mutations in the BEST1 gene., Methods: Consecutive patients with Best vitelliform macular dystrophy underwent a complete ophthalmologic examination, including functional assessment by best-corrected visual acuity and Foresee PHP and morphologic assessment by fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography. The functional "PHP visual field defect index" (which is the max peak value of the metamorphopsia [maximal distortion value at the visual field] + the max peak value of the scotoma [maximal scotoma value at the visual field]) and best-corrected visual acuity were analyzed about the disease stage., Results: Thirty eyes of 15 consecutive patients (8 men and 7 women; mean age 39 ± 24 years) were included for analysis. Based on fundus biomicroscopy, fundus autofluorescence, and spectral-domain optical coherence tomography, the macular lesions could be counted as follows: previtelliform lesions in 5 eyes of 3 patients (Stage 1), vitelliform lesions in 2 eyes of 2 patients (Stage 2), pseudohypopyon lesions in 6 eyes of 5 patients (Stage 3), vitelliruptive lesions in 4 eyes of 3 patients (Stage 4), atrophic lesions in 7 eyes of 5 patients (Stage 5), and fibrotic lesions in 6 eyes of 4 patients (Stage 6). Best-corrected visual acuity and PHP visual field defect index were averaged for each stage. Best-corrected visual acuity showed a good correlation (P = 0.01) with the morphologic severity (stage) of the disease (Pearson correlation = -0.88). Similarly, the PHP visual field defect index showed a good correlation (P = 0.03) with the morphologic severity (stage) of the disease (Pearson correlation = 0.78). Finally, best-corrected visual acuity showed a good correlation (P = 0.02) with the functional PHP visual field defect index (Pearson correlation = -0.83) about the morphologic stage of the disease., Conclusion: Preferential hyperacuity perimetry could be considered an adjunctive useful tool in the evaluation of functional impairment and disease progression in patients with Best vitelliform macular dystrophy.

Background: Conventional risk factors for retinal vein occlusion (RVO) are well established through large epidemiological studies, but triggering factors remain poorly known., Patients and Methods: A prospective observational study through a questionnaire was completed between January and October 2009 by patients with RVO and controls., Results: Sixty-one patients, including 42 central retinal vein occlusions (CRVO) and 19 branch vein occlusions (BRVO) as well as 118 controls were included. Of the CRVO patients, 77% discovered visual loss upon awakening in contrast to only 33% of BRVO patients. The comparison between RVO and controls showed a higher proportion in the RVO group for (in order of the highest risk): migraine headache (CRVO, 21 %; BRVO, 47 %; controls, 13 %; p=0.008), hypertension (CRVO, 52%; BRVO, 63%; controls, 37%; p=0.012), glaucoma (CRVO, 33%; BRVO, 22%; controls, 16%; p=0.034), antiplatelet or anticoagulant medication (CRVO, 42%; BRVO, 33%; controls, 26%; p=0.074), hyperlipidemia, ocular trauma, sudden deafness, or vertigo. The prevalence was similar in both groups for body mass index, history of phlebitis or peptic ulcer, smoking habits, stress, fasting, dehydration, vasodilator intake, and altitude stay., Conclusion: In addition to the already known risk factors, this study underlines certain underlying conditions or circumstances related to the onset of RVO, such as migraine. In CRVO, three-quarters of the patients on average discovered visual loss upon awakening, which may indicate that nocturnal events may play a significant role in the pathogenesis of the occlusion., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Age-related macular degeneration is the primary cause of blindness in developed countries. Current treatments of this degenerative disease mainly include laser, photodynamic therapy with verteporfin and administration of anti-vascular endothelial growth factors. The LUEUR (LUcentis® En Utilisation Réelle) study is composed of a cross-sectional part (LUEUR1), which examined the current management of wet AMD in France, and a follow-up part (LUEUR2), which will assess the development of patients treated for wet AMD over 4 years. Here we describe the results of LUEUR1., Methods: Patients with wet AMD were enrolled during a routine medical examination in LUEUR1, a cross-sectional, observational, prospective, multicentre study. Investigators recorded patient demographics, visual acuity, characteristics of wet AMD lesions, date of AMD diagnosis, comorbidities, previous treatments, treatments prescribed at inclusion, and low vision rehabilitation., Results: A total of 72 investigators recruited 1,019 patients with wet AMD, corresponding to 1,405 eyes affected by the disease. The mean age of patients was 78.7 ± 7.3 years. Most were female (62.3%) and non-smokers (66.9%). The mean visual acuity was 49.12 ± 24.18 Early Treatment Diabetic Retinopathy Study letters. Most eyes showed occult (52.8%) and subfoveal (84.6%) choroidal neovascularisation. Bilateral wet AMD affected 37.9% of patients. The median time since diagnosis was 12 months. Ranibizumab-based therapy (67.3%) and photodynamic therapy (29.8%) were the most frequent previous treatments. Prior to inclusion, 5.6% of patients had low vision rehabilitation. When a treatment was prescribed on the day of inclusion, it was most often ranibizumab (89.0% of all treatments at inclusion)., Conclusions: The results of this study illustrate the impact of anti-vascular endothelial growth factor therapies on the treatment of wet AMD in a real-life context. Specifically, ranibizumab-based therapy appears to have largely replaced laser photocoagulation and verteporfin-based photodynamic therapy.

Purpose: The purpose of this study was to analyze the integrated infrared reflectance, fundus autofluorescence, and fluorescein angiography (integrated confocal scanning laser ophthalmoscopy fundus imaging) features of reticular pseudodrusen and eye-tracked Spectralis high-resolution spectral domain optical coherence tomography (Spectralis SD-OCT; Heidelberg Engineering, Heidelberg, Germany)., Methods: Twenty-two consecutive patients with reticular pseudodrusen were prospectively enrolled and evaluated regarding confocal scanning laser ophthalmoscopy fundus imaging and eye-tracked SD-OCT findings., Results: Integrated fundus imaging revealed a "target" aspect of most reticular pseudodrusen in the 42 included eyes (22 patients; 12 women, 10 men; mean age 81.38 ± 6.47 years). On fundus autofluorescence and infrared reflectance, the center of most reticular pseudodrusen appeared as an area of isoautofluorescence/reflectance surrounded by halos of reduced autofluorescence/reflectance. Similarly, on fluorescein angiography, the center of reticular pseudodrusen appeared as an area of decreased fluorescence surrounded by a faint halo of increased fluorescence. Spectral domain optical coherence tomography showed a well-defined round or triangular hyperreflective deposit localized between, externally, the retinal pigment epithelium layer, and, internally, the external limiting membrane or the outer plexiform layer. Moreover, SD-OCT showed the loss of both outer segment/retinal pigment epithelium interface and inner segment/outer segment interface over the hyperreflective lesions, as well as an abrupt interruption of both these interfaces at the border of the hyperreflective lesions., Conclusion: The peculiar confocal scanning laser ophthalmoscopy fundus imaging and tracked SD-OCT of reticular pseudodrusen suggest the presence of central lipofuscin-like retinal deposits localized above the retinal pigment epithelium. These findings give insights to other possible aspects of age-related retinal changes.

Aims: Drusen are rarely observed in patients < 50 years of age. Two types of early onset drusen (EOD) are commonly described: basal laminar drusen (BLD) and drusen associated with Malattia Leventinese (ML). Our purpose was to classify the phenotype of EOD on the basis of fundus examination, and fluorescein angiography (FA) and indocyanine green angiography (ICGA) features., Methods: We performed a prospective study including 48 consecutive EOD patients. All of them had a complete ophthalmologic examination including FA and ICGA., Results: BLD (67%) were extremely hyperfluorescent on FA and ICGA. ML (10%) was characterised by a combination of small radial and large round drusen with differences in staining in both FA and ICGA. We evidenced a third type of EOD (23%) harbouring an aspect of large colloid drusen (LCD), mildly hyperfluorescent in the early phases of FA, with a progressive staining in late phases. In intermediate and late phases of ICGA, LCD presented as hypofluorescent dot surrounded by a hyperfluorescent halo bordered by a thin hypofluorescent ring., Conclusion: Three types of EOD are distinguished by their FA and ICGA features. We report a new kind of juvenile drusen, distinct from BLD and ML, named LCD, associated with a good vision and absence of complications.

Purpose: The determination of homogeneous subgroups of age-related macular degeneration (AMD) is necessary for clinical and genetic studies; therefore, the development of a simple, reproducible, and discriminating classification is essential. In this second part of our study (SPA-2), we evaluated a selected list of items for atrophic AMD based on color photographs of fundus, red-free frames, autofluorescence, fluorescein angiography, indocyanine angiography, and Spectral-Domain OCT., Methods: Ten items for atrophy were chosen from the literature and clinical experience. Twenty eyes of 20 patients with atrophic AMD were included. For each patient, the grid was completed by five independent, experienced readers from our reading center and by an expert. The Kappa coefficient was calculated for each item., Results: The greatest agreement between observers was found for the item "presence of atrophy" (Kappa=1). The worst concordance was recorded for the item "size of atrophy" (Kappa=-0.0286±0.0769 to 0.1813±0.0835)., Conclusion: The classification of atrophic AMD is complex and currently not very consensual, hence the need for a discriminant and reproducible classification grid. The evaluation of our grid for atrophic AMD shows satisfactory agreement between observers for the majority of the items. Some modifications are proposed to make it more discriminant and reproducible., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Purpose: To determine if genetic variants that have been associated with age-related macular degeneration (AMD) have a differential effect on the risk of choroidal neovascularization (CNV) and geographic atrophy., Design: Genetic association study., Setting: Multicenter study., Study Population: Seven hundred forty-nine participants with geographic atrophy and 3209 participants with CNV were derived from 4 AMD studies with similar procedures from Tufts Medical Center, the Age-Related Eye Disease Study, University of Utah, and Hopital Intercommunal de Creteil., Procedures: AMD grade was assigned based on fundus photography and examination using the clinical age-related maculopathy staging system. All samples were genotyped for single nucleotide polymorphisms (SNPs) previously associated with AMD. Allele frequencies were compared between participants with CNV and geographic atrophy using PLINK within each cohort and Mantel-Haenszel meta-analysis was performed to combine odds ratios (OR)., Main Outcome Measures: Differences in allele frequencies between participants with geographic atrophy and CNV., Results: The frequency of the T allele of ARMS2/HTRA1 rs10490924 was significantly higher in participants with CNV than in those with geographic atrophy (OR, 1.37; 95% confidence interval, 1.21-1.54; P value = 4.2 × 10(-7)). This result remained statistically significant when excluding individuals who had geographic atrophy in 1 eye and CNV in the contralateral eye (P = 2.2 × 10(-4)). None of the other SNPs showed a significant differential effect for CNV vs geographic atrophy, including CFH, C2/CFB, C3, CFI, LIPC, and TIMP3., Conclusions: Genetic variation at the ARMS2/HTRA1 locus confers a differential risk for CNV vs geographic atrophy in a well-powered sample., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Over the last decade, several epidemiological studies based on food frequency questionnaires suggest that omega-3 polyunsaturated fatty acids could have a protective role in reducing the onset and progression of retinal diseases. The retina has a high concentration of omega-3, particularly DHA, which optimizes fluidity of photoreceptor membranes, retinal integrity, and visual function. Furthermore, many studies demonstrated that DHA has a protective, for example antiapoptotic, role in the retina. From a nutritional point of view, it is known that western populations, particularly aged individuals, have a higher than optimal omega-6/omega-3 ratio and should enrich their diet with more fish consumption or have DHA supplementation. This paper underscores the potential beneficial effect of omega-3 fatty acids on retinal diseases.

Clinical Question: Is there any new knowledge about the pathogenesis and treatment of age-related macular degeneration (AMD)?, Results: We now understand better the biochemical and pathological pathways involved in the genesis of AMD. Treatment of exudative AMD is based on intravitreal injection of new antivascular endothelial growth factor drugs for which there does not yet exist a unique recognized strategy of administration. No therapies are actually available for atrophic AMD, despite some experimental new pharmacological approaches., Implementation: strategy of administration, safety of intravitreal injection.

Purpose: To study the ability to appreciate qualitative features that indicate disease activity in patients with neovascular age-related macular degeneration (AMD) and to analyze the differences in automated retinal thickness measurement, using 1 time domain optical coherence tomography (TD-OCT) and 2 different spectral-domain OCT (SD-OCT) machines., Methods: Thirty-three consecutive naïve patients with neovascular AMD underwent Stratus TD-OCT, Cirrus SD-OCT and Spectralis SD-OCT, at baseline, 1 h, 1 day, 1 week and 1 month after intravitreal ranibizumab injection., Results: As regards the ability to detect retinal cysts, subretinal fluid and pigment epithelium detachment, at each follow-up visit, there was a significant correlation among all 3 OCT devices (p < 0.05), even though Cirrus SD-OCT and Spectralis SD-OCT showed the highest level of intermachine agreement. At each follow-up visit, automated retinal thickness measurements showed a greater mean central macular thickness (CMT) for both Spectralis SD-OCT and Cirrus SD-OCT, compared with Stratus TD-OCT. However, the mean paired differences in CMT among the 3 OCT devices were not statistically significant (p > 0.05). Overall, Cirrus SD-CT showed fewer segmentation errors, compared with both Spectralis SD-OCT and Stratus TD-OCT., Conclusion: SD-OCT showed a greater ability to evaluate qualitative features indicating disease activity and fewer errors in automated segmentation. However, differences in CMT changes were similar between TD-OCT and SD-OCT systems during follow-up., (Copyright © 2011 S. Karger AG, Basel.)

Age-related macular degeneration (AMD) is a complex, multifactorial disease associated with environmental and genetic factors. This review emphasizes the clinical impact of the major genetic factors mainly located in the complement factor H gene and on the 10q26 locus, and their current and future implications for the management of AMD., (Copyright © 2011 S. Karger AG, Basel.)

Purpose: The possibility of applying long-lasting steroids, such as triamcinolone and subsequently dexamethasone implant, directly in the eye, without the systemic side effects observed after their oral or intravenous administration, aroused great enthusiasm among ophthalmologists., Methods and Results: The SCORE study, a multicenter clinical trial, compared the efficacy and safety of 1-mg and 4-mg doses of preservative-free intravitreal triamcinolone with observation in central retinal vein occlusion (CRVO) participants with macular edema secondary to perfused CRVO. This study marked a turning point in the management of retinal vein occlusion (RVO), since it was the first report on an effective treatment of macular edema due to CRVO. But in branch retinal vein occlusion (BRVO), the SCORE study showed no difference in visual acuity for the standard care group compared with the triamcinolone groups; however, the rates of adverse events (particularly elevated intraocular pressure and cataract) were highest in the 4-mg group. The authors concluded that laser grid photocoagulation should remain the benchmark against which other treatments should be compared. The Geneva Study, a randomized, controlled, clinical trial, conducted to evaluate the safety and efficacy of an intravitreal implant that delivers sustained levels of dexamethasone (Ozurdex™), studied the largest group of RVO patients with macular edema (1267 patients), including 35% CRVO and 65% BRVO. The study demonstrated that this slow-release device could both reduce the risk of vision loss and improve the speed and incidence of visual improvement in eyes with macular edema secondary to CRVO and BRVO, with fewer side effects, such as elevation of intraocular pressure or cataract. Other ongoing studies evaluate the safety and efficacy of anti-vascular endothelial growth factor injection; preliminary results showed that these therapies are effective in decreasing macular edema and improving visual acuity, with fewer ocular adverse effects, but their duration of action seems limited., Conclusions: These recent studies result in a great change in the management of macular edema from RVO. Unfortunately, long-term studies on safety and efficacy are not yet available, and further studies will have to show whether the short-term benefits are only transient or may finally lead to a long-lasting improvement in vision.

Purpose: To analyze retrospectively the efficacy of intravitreal ranibizumab injections for the management of choroidal neovascularization (CNV) in patients with angioid streaks., Design: Nonrandomized, double-center, retrospective, interventional case series., Methods: A consecutive series of patients affected with CNV associated with angioid streaks were treated with intravitreal ranibizumab injections (0.5 mg/0.05 mL). Best-corrected visual acuity, fundus photography results, optical coherence tomography (OCT) results, and fluorescein angiography results were examined before and after treatment. The primary end point was the percentage of eyes with stable or improved visual acuity at the end of follow-up. Secondary end points were the percentage of eyes with stable or decreased macular thickness on optical coherence tomography and the percentage of eyes with persistent leakage on fluorescein angiography at the last follow-up examination., Results: Thirty-five eyes of 27 patients were treated with repeated intravitreal ranibizumab injections (mean, 5.7 injections; range, 2 to 14 injections) for a mean of 24.1 months (range, 6 to 37 months). At the end of follow-up, visual acuity was stabilized or improved in 30 (85.7%) of 35 eyes. Macular thickness had stabilized or decreased in 18 (51.5%) of 35 eyes. At the last follow-up examination, on fluorescein angiography, no further leakage was observed in 23 (65.7%) of 35 eyes., Conclusions: In this large series of angioid streaks-associated CNV, ranibizumab injections allowed stabilization of visual acuity. Ranibizumab seems to be a safe therapeutic option in CNV associated with angioid streaks., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Purpose: To assess morphologic changes associated with retinal flecks in fundus flavimaculatus using spectral-domain optical coherence tomography (SD-OCT)., Design: Observational case series., Methods: Simultaneous recordings of SD-OCT and confocal scanning laser ophthalmoscope (cSLO) fundus autofluorescence images were obtained in fundus flavimaculatus patients. Morphologic aspects of the retinal flecks were analyzed and classified., Results: Thirty-one eyes of 17 consecutive patients (8 male, 9 female; mean age 47.9 +/- 17.1 years) were included for analysis. SD-OCT revealed 5 distinct types of lesions. Group A lesions were limited to the outer segment (OS) of the photoreceptors, the retinal pigment epithelium (RPE) interdigitations, and the RPE/Bruch membrane complex. Group B showed a protrusion of the hyper-reflective material through the interface of inner segment (IS)/OS of the photoreceptors up to the external limiting membrane. A further protrusion of the hyper-reflective material into the outer nuclear layer was seen in group C lesions. Group D lesions were characterized by an accumulation of the hyper-reflective material limited to the outer nuclear layer. Type E lesions can be described as drusen-like retinal pigment detachments. No significant correlation between the different types of flecks and visual acuity was observed (P > .05)., Conclusions: SD-OCT allows one to distinguish at least 5 different types of lesions associated with retinal flecks in fundus flavimaculatus. The ability to characterize the different types of flecks and to analyze the photoreceptor layers surrounding these lesions suggests that SD-OCT might have a potential clinical role in the evaluation and follow-up of the structural changes in fundus flavimaculatus., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

Purpose: To describe the different morphological features in adult onset foveomacular vittelliform dystrophy (AOFVD) using high-resolution spectral domain optical coherence tomography (OCT)., Design: Prospective observational case series., Methods: Complete ophthalmologic examination, including spectral domain OCT, was performed in 49 consecutive AOFVD patients (60 eyes)., Results: In 28/60 eyes, spectral domain OCT showed hyper-reflective clumps within the outer plexiform and outer nuclear layers. In 9/60 eyes, the photoreceptor inner segment/outer segment (IS/OS) interface appeared highly reflective like a shell all around the vitelliform material, and appeared irregular and discontinued in 27/60 eyes. The Verhoeff membrane was clearly visible at the border of the lesion, disappeared over the vitelliform lesion in 20/60 eyes, became thickened and less defined on the outer aspect of the lesion in 11/60 eyes, appeared without noticeable alterations in 10/60 eyes and not well defined in 19/60 eyes. The vitelliform material appeared as a highly reflective dome-shaped lesion (homogeneous in 14/60 eyes and heterogeneous in 36/60 eyes) located between the photoreceptor layer and the retinal pigment epithelium (RPE). In 10/60 eyes, the macular lesion appeared as hypo/a-reflective. The RPE appeared irregular in 14/60 eyes, with hyper-reflective mottling on its inner aspect. We observed discrete RPE detachments in 29/60 eyes., Conclusions: We hypothesise that early changes involve the layer between RPE and the IS/OS interface, first with vitelliform material accumulation beneath the sensory retina, and then with IS/OS alterations, pigments migration towards inner layers and fluid accumulation. These changes come with RPE alterations such as hypertrophy or sub-RPE deposits.

Purpose: To report an unusual association between cancer-associated retinopathy (CAR) associated with invasive thymoma and choriodal neovascularization (CNV), treated by photodynamic therapy (PDT)., Methods: A 39-year-old man affected with thymoma and paraneoplastic syndrome (myasthenia gravis and diarrhoea) was observed between October 1997 and September 2007. The patient developed progressive visual dysfunction including bilateral visual acuity loss and concentric constriction of visual fields. Ophthalmological, immunological and systemic examinations were performed. Immunological evaluations included an assessment of antibody activity by indirect immunohistochemistry on sectioned rhesus monkey eye, and Western blot reactions upon an extract of pig retina., Results: Fundus ophthalmoscopy and fluorescein angiography revealed retinal vessel attenuation and retinal pigment epithelium degeneration. Electroretinogram suggested both rod and cone dysfunction. Indirect immunohistochemistry identified antibody activity within the photoreceptor outer segments. Western blots on the retina revealed that most of the patient's antibody activity was focused upon a retinal protein antigen approximating 145 kD. These findings share the commonalities of size and retinal distribution of the interphotoreceptor retinoid-binding protein (IRBP), a recognized autoantigen. The surgically resected mediastinal tumour was diagnosed as invasive thymoma. No other malignancy has since been found throughout nearly 10 years of follow-up. In March 2006, the patient developed a subfoveal CNV in his left eye, which was treated by PDT., Conclusion: We describe the third case of paraneoplastic retinopathy associated with invasive thymoma. This is the first example of CAR involving autoantibodies reactive with a retinal protein having the characteristics of the IRBP, and is also the first complicated by CNV treated by PDT.

Background: We analyzed the effects of a docosahexaenoic acid (DHA) supplementation in patients affected with late onset Stargardt disease (STGD)., Methods: DHA (840 mg/day) was given to 20 STGD patients for six months. A complete ophthalmologic examination, including best-corrected visual acuity (BCVA) and multifocal electroretinogram (mfERG), was performed at inclusion day 0 (D0) and at month 6 (M6)., Results: Overall, no statistical differences have been observed at M6 vs D0 as regards BCVA and mfERG (P > 0.05). Mild Improvement of BCVA and improvement of mfERG was noted in seven/40 eyes of four/20 patients. In the first patient, the peak of the a wave increased from 66 nV/deg(2) to 75.4 nV/deg(2) in the right eye (RE) and 24.5 nV/deg(2) to 49.1 nV/deg(2) in the left eye (LE). The peak of the b wave improved from 122 nV/deg(2) to 157 nV/deg(2) in the RE, and 102 nV/deg(2) to 149 nV/deg(2) in the LE. In the second patient peaks of the a and b waves respectively increased from 11.8 nV/deg(2) to 72.1 nV/deg(2) and 53 nV/deg(2) to 185 nV/deg(2) in the RE. In the third patient the peak of the a wave increased from 37 nV/deg(2) to 43 nV/deg(2) in the RE, and from 31 nV/deg(2) to 45 nV/deg(2) in the LE; the peak of the b wave improved from 70 nV/deg(2) to 89 nV/deg(2) in the RE, and from 101 nV/deg(2) to 108 nV/deg(2) in the LE. In the fourth patient, the peak of the a wave increased from 39 nV/deg(2) to 42 nV/deg(2) in the RE, and from 40 nV/deg(2) to 43 nV/deg(2) in the LE; the peak of the b wave improved from 86 nV/deg(2) to 94 nV/deg(2) in the RE, and from 87 nV/deg(2) to 107 nV/deg(2) in the LE., Conclusion: DHA seems to influence some functional parameters in patients affected with STGD. However, no short-term benefit should be expected from DHA supplementation.

Purpose: Major genetic factors for age-related macular degeneration (AMD) have recently been identified as susceptibility risk factors, underlying the role of the complement pathway in AMD. Our purpose was to analyze the role of the R102G polymorphism of the complement component (C3) gene in a French population, in a case-control study., Methods: A total of 1,080 patients with exudative AMD and 406 controls were recruited and genotyped for Y402H of complement factor H (CFH), rs10490924 of age-related maculopathy susceptibility 2 (ARMS2), and R102G of the C3 gene., Results: The distribution of the R102G genotypes was significantly different in the AMD patients compared to controls (p=0.02). The Odds Ratio compared to C/C individuals was 1.4 (95% CI 1.1-1.8) for C/G individuals and 1.4 (95% CI 0.8-2.4) for G/G individuals. In a dominant model, the adjusted Odds Ratio for carriers of the G allele is 1.4 (95% CI 1.0-1.9; p=0.03)., Conclusions: Our study shows C3 to be a moderate susceptibility gene for exudative AMD in the French population.

Purpose: To assess the 1-year functional outcome and to evaluate the morphological changes after intravitreal injections of ranibizumab in eyes affected with retinal angiomatous proliferation (RAP) due to age-related macular degeneration (AMD)., Methods: A prospective, non-randomized, interventional study was conducted on 26 consecutive patients with newly diagnosed RAP. All eyes were treatment naive and were randomized to receive intravitreal injections of ranibizumab for a 12-month period. After the first three monthly injections, re-treatment was performed in case of best-corrected visual acuity (BCVA) loss of at least five letters associated with fluid within the macula, central macular thickness (CMT) increase of at least 100 microm, and/or persistence of fluid within the macula as evaluated by optical coherence tomography, new onset macular haemorrhages, persistence of leakage from the lesions on fluorescein angiography., Results: All patients completed the 12-month follow-up: 25 of the 29 treated eyes (86.2%) were stabilized, with a loss of less than 15 letters. Nineteen eyes (65.5%) maintained or improved their BCVA, and three eyes (10.3%) gained three lines or more. Overall, mean BCVA remained stable at the 12-month follow-up (-0.07 letters; P>0.05). Mean CMT significantly decreased from 386+/-147 to 216+/-74 microm at the 12-month follow-up. No significant adverse events were observed during the study. The mean number of injections was 5.8+/-1.7 during the follow-up period., Conclusion: The 1-year follow-up outcomes in our series suggest that ranibizumab is an effective treatment for RAP in AMD, allowing stabilization of BCVA and reduction of CMT.

Purpose: To report on a patient with retinal astrocytic hamartoma, who developed a choroidal neovascularization (CNV), effectively treated by intravitreal ranibizumab injections., Methods: A 74-year-old woman who, 12 years before, had been diagnosed with a yellow-gray lesion in the left eye (OS) presented in our department for OS decreased vision of recent onset., Results: Upon a complete ophthalmologic examination including ultrasonography, fluorescein angiography (FA), and spectral domain optical coherence tomography (SD-OCT), the patient was diagnosed with retinal astrocytic hamartoma and coincident CNV on its foveal border. Six months after 3 monthly intravitreal ranibizumab injections, FA and OCT revealed the CNV closure and absence of intraretinal and subretinal fluid on the foveal border of the retinal astrocytic hamartoma., Conclusions: Associations between retinal astrocytic hamartoma and CNV have not been previously reported. Intravitreal ranibizumab injection appears an attractive therapeutic option for patients showing such an unusual association.

Purpose: Major genetic risk factors have recently been identified for age-related macular degeneration (AMD), including the ARMS2/LOC387715 and CFH at-risk polymorphisms. The study was conducted to establish correlations between the AMD genotype and both the phenotype and severity of AMD., Methods: In a prospective cohort of 1216 AMD patients, four genotypic homozygous groups were identified (n = 264): double homozygous for wild-type alleles (group 1, n = 49), homozygous for the at-risk allele of ARMS2/LOC387715 only (group 2, n = 57), homozygous for the at-risk allele of CFH only (group 3, n = 106), and double homozygous for both at-risk alleles (group 4, n = 52). The phenotypic classification of exudative AMD was based on fluorescein angiography., Results: Mean age at presentation was significantly lower in group 4 than in group 1 (P < 0.014). Patients in group 4 presented more often with bilateral CNV and fibrovascular scars than did patients in group 1 (P < 0.001 and < 0.0031 respectively) and with significantly lower visual acuity (VA) in the first affected eye than did patients in group 1 (P < 0.02). Patients in group 2 presented with worse VA than did patients in group 3 (P < 0.003). Classic CNV was more commonly associated with the at-risk allele of the ARMS2/LOC387715 locus than with the at-risk allele of the CFH gene (P < 0.026)., Conclusions: This study demonstrates an association between the at-risk allele of the ARMS2/LOC387715 locus and classic CNV, fibrovascular lesions, and poor VA. Individuals double homozygous for both at-risk alleles had a higher risk of being affected with a severe form of AMD at an earlier age.

In this study, an attempt is made to assess in-vivo, the location of retinal crystals in a 39-year-old woman with Bietti's crystalline dystrophy using spectral domain optical coherence tomography (Spectralis SD-OCT). In this patient, it was possible to show the exact correspondence between the retinal crystals, as visualized by infrared frame, and the tiny hyper-reflective SD-OCT lesions that appeared localized in all retinal layers, from the RPE to the retinal nerve fibre layer., (Copyright 2010, SLACK Incorporated.)

Purpose: The purpose of this study was to evaluate the efficacy of intravitreal injections of ranibizumab in choroidal neovascularization secondary to pathologic myopia., Methods: A prospective case series of 32 eyes of 32 patients affected with choroidal neovascularization secondary to pathologic myopia treated by intravitreal injections of ranibizumab. Best-corrected visual acuity, fundus examination, fluorescein angiography, indocyanine green angiography, and spectral domain-optical coherence tomography were performed for the diagnosis of myopic choroidal neovascularization. Best-corrected visual acuity and central retinal thickness measurement were performed monthly during the follow-up., Results: The median number of injections was 3 with a median follow-up of 17 months. The median visual acuity at baseline was 20/100 and improved to 20/50 at final examination (P < 0.0001). Best-corrected visual acuity improved by > or = 3 lines in 15 of 32 eyes (46.8%). The median central thickness was 336 microm (range, 179-663 microm) at baseline and 233 microm (range, 125-465 microm) at final examination (P < 0.0001). No severe drug-related side effect was reported., Conclusion: In our series of myopic choroidal neovascularization, intravitreal injections of ranibizumab showed visual acuity improvement and retinal thickness reduction. Further prospective multicentric clinical trials are needed to evaluate the safety and the efficacy of this treatment.

Purpose: To describe the simultaneous presentation of soft confluent drusen and type 2 idiopathic macular telangiectasia (IMT) in both eyes of one patient., Methods: A 79-year-old man with bilateral metamorphopsia and gradual reduction of central vision underwent a complete ophthalmologic examination., Results: In this patient, fundus biomicroscopy revealed soft confluent drusen and a cystic appearance within the fovea, and fluorescein angiography (FA) showed late dye leakage. Interestingly, indocyanine green angiography (ICGA) showed absence of late hypercyanescence, and spectral domain optical coherence tomography (Spectralis SD-OCT) clearly revealed the presence of bilateral foveal cysts with thinning and loss of the normal architecture of the outer retina, as well as absence of retinal thickening within the parafoveolar area showing discrete late dye leakage on FA. Based on these findings, the patient was diagnosed with nonexudative age-related macular degeneration with foveal soft confluent drusen, and coincident nonproliferative type 2 IMT., Conclusions: To our knowledge, there is no previously reported case of simultaneous presentation of soft confluent drusen and type 2 IMT. This report highlights the importance of ICGA and OCT in the correct diagnosis of such cases.

Background: To analyse the 24-month outcomes of intravitreal ranibizumab injections for choroidal neovascularisation (CNV) secondary to age-related macular degeneration (AMD)., Methods: The authors reviewed the charts of all consecutive eyes with CNV secondary to AMD, who underwent one intravitreal ranibizumab injection (followed by a pro re nata (1+PRN) decision to retreat or to not retreat) at least 24 months before. Best-corrected visual acuity (BCVA) changes and central macular thickness (CMT) were retrospectively assessed, from baseline (m0) to month 12 (m12), and 24 (m24)., Results: Ninety-six eyes of 79 patients (23 male, 56 female, aged 63-90 years) were included for analysis. The number of intravitreal injections administered ranged from 1 to 16. The mean BCVA significantly improved from m0 (0.78+/-0.33) to m12 (0.61+/-0.39, p<0.001), and m24 (0.65+/-0.38, p<0.001). The mean CMT significantly decreased from m0 (323.7+/-118.1) to m12 (254.6+/-92.3, p<0.001), and m24 (259.0+/-89.9, p<0.001). At m24, subretinal fluid, cystoid macular oedema and pigment epithelium detachment were present in fewer eyes (13, 31 and 31 eyes respectively), compared with m0 (33, 61 and 72 eyes, respectively). Overall, at m12 and m24, 91 eyes (94.8%) and 84 eyes (87.5%) lost fewer than 15 letters, and 25 (26%) eyes and 24 eyes (25%) improved by 15 letters or more, respectively; five eyes (5.2%) and 12 eyes (12.5%) lost more than 15 letters, at m12 and m24, respectively., Conclusion: In this study, similarly to other studies of variable dosing regimen over 24 months, intravitreal ranibizumab was effective in significantly increasing BCVA and reducing CMT.

Purpose: The purpose of this study was to analyze the angiographic (confocal scanning laser ophthalmoscopy technology) and corresponding (eye-tracked) spectral-domain optical coherence tomography (SD-OCT) features and to propose a classification for the progressive phases establishing retinal-choroidal anastomosis (RCA)., Methods: We reviewed all consecutive eyes with RCA that underwent Heidelberg Retina Angiograph angiography and tracked Spectralis SD-OCT at the University Eye Clinic of Creteil between September 2007 and March 2009., Results: Twenty-six eyes of 23 patients (8 men and 15 women, aged 70-88 years) showing RCA naïve to any treatment were included for analysis. In 6 of 7 eyes showing a discrete focal hyperfluorescence (focal staining), the corresponding (eye-tracked) SD-OCT scan showed a focal retinal pigment epithelium (RPE) erosion ("erosion sign") over a small, localized RPE elevation (which appeared filled with a hyperreflective material); in 7 of 8 eyes showing a typical "hot spot" in the late angiographic frames (focal leakage) and absence of a serosanguineous pigment epithelium detachment, the corresponding (eye-tracked) SD-OCT scan showed a focal RPE break leaving 2 free RPE flaps ("flap sign") at the level of a small, localized RPE elevation. In 10 of 11 eyes showing a typical hot spot in the late angiographic frames and presence of a serosanguineous pigment epithelium detachment, the corresponding (eye-tracked) SD-OCT scan showed, at the level of a large serosanguineous RPE detachment, a focal funnel-shaped RPE joining (kissing) an inverted focal funnel-shaped inner neuroepithelium ("kissing sign")., Conclusion: An early neovascularization (a discrete focal hyperfluorescence) arising from the choroid initially simply erodes the basement membrane/RPE (erosion sign; Phase 1) and later breaks the basement membrane/RPE (flap sign), infiltrating first into the outer retina forming an early RCA (Phase 2, a typical hot spot without a serosanguineous pigment epithelium detachment) and later into the inner retina (kissing sign) forming an established RCA (Phase 3, a typical hot spot with a serosanguineous pigment epithelium detachment).

Purpose: To analyze functional and clinical data of Best vitelliform macular dystrophy (VMD) patients with mutations in the BEST1 gene., Methods: Best VMD patients with BEST1 mutations were evaluated prospectively regarding age, age of onset, best-corrected visual acuity (BCVA), fundus autofluorescence, fluorescein angiography, optical coherence tomography, and electro-oculography. Mutations in BEST1 were established by direct sequencing., Results: Forty-six eyes of 23 patients (10 male, 13 female) were included in the study. We identified nine different BEST1 mutations (3/9 novel), in ten unrelated families. The age of patients ranged between 3 and 75 years; age of onset varied between 2 and 67 years. BCVA ranged between 20/20 and 20/200. On the basis of fundus biomicroscopy with direct illumination, using one widely accepted classification, the macular lesions could be counted as follows: 1. no lesion (normal fovea): eight eyes, five patients carrying a mutation on the BEST1 gene; 2. previtelliform lesions: six eyes, three affected patients; 3. vitelliform lesions: four eyes, two affected patients; 4. pseudohypopyon: three eyes, three affected patients; 5. vitelliruptive lesions (scrambled egg aspect with dispersion of the vitelliform material without sign of atrophy or fibrosis): ten eyes, six affected patients; 6. atrophic lesions (atrophy with or without residual dispersed material): seven eyes, five patients; 7. fibrotic lesions: eight eyes, five patients. Two patients presented unilateral Best VMD. Both eyes of two patients presented multifocal Best VMD features on fundus examination. Six eyes of four patients have been treated for choroidal neovascularization by thermic photocoagulation [one eye], photodynamic therapy [three eyes], and intravitreal ranibizumab injection [two eyes]. Comparison of interfamilial and intrafamilial clinical data between patients did not reveal differences in age, BCVA, and stage of the disease as evaluated by fundus autofluorescence, fluorescein angiography, and optical coherence tomography (p>0.05). Mean BCVA impairment showed a statistically significant correlation to a more advanced stage of the disease (p<0.001)., Conclusions: BEST1 mutations were not correlated with the severity of the functional and clinical data in the Best VMD patients examined.

Age-related macular degeneration (AMD) is the main cause of vision loss in the elderly in developed countries. The exudative AMD is the most frequent and severe form of the disease, leading to a rapid loss of vision. The results of MARINA and ANCHOR studies, two multicentric clinical trials analyzing the efficacy of anti-VEGF in exudative AMD, have showed that this modality of treatment results in a stabilisation of the visual acuity in most cases (70%), and in visual improvement in 30 % of the cases. Up till now, the anti-VEGF approach represents a major step in the therapeutic strategy of this disease.

Purpose: Basal laminar drusen (BLD) is a rare retinal disorder that may be complicated with choroidal neovascularisation (CNV). Here we describe four patients treated with verteporfin photodynamic therapy (PDT) for CNV secondary to BLD., Methods: Four consecutive patients presented with subfoveal CNV secondary to BLD. Mean visual acuity was 20/50. CNV was treated with one single PDT session., Results: Exsudative features of CNV were resolved after one session for all patients. After 6 months, mean visual acuity was 20/25 and remained stable., Conclusion: In this series, PDT was an effective treatment for CNV secondary to BLD.

Major genetic factors for age-related macular degeneration (AMD) have recently been identified as susceptibility risk factors, including variants in the CFH gene and the ARMS2 LOC387715/HTRA1locus. Our purpose was to perform a case-control study in two populations among individuals who did not carry risk variants for CFHY402H and LOC387715 A69S (ARMS2), called "study" individuals, in order to identify new genetic risk factors. Based on a candidate gene approach, we analyzed SNP rs5888 of the SCARB1 gene, coding for SRBI, which is involved in the lipid and lutein pathways. This study was conducted in a French series of 1241 AMD patients and 297 controls, and in a North American series of 1257 patients with advanced AMD and 1732 controls. Among these individuals, we identified 61 French patients, 77 French controls, 85 North American patients and 338 North American controls who did not carry the CFH nor ARMS2 polymorphisms. An association between AMD and the SCARB1 gene was seen among the study subjects. The genotypic distribution of the rs5888 polymorphism was significantly different between cases and controls in the French population (p<0.006). Heterozygosity at the rs5888 SNP increased risk of AMD compared to the CC genotypes in the French study population (odds ratio (OR) = 3.5, CI95%: 1.4-8.9, p<0.01) and after pooling the 2 populations (OR = 2.9, 95% CI: 1.6-5.3, p<0.002). Subgroup analysis in exudative forms of AMD revealed a pooled OR of 3.6 for individuals heterozygous for rs5888 (95% CI: 1.7-7.6, p<0.0015). These results suggest the possible contribution of SCARB1, a new genetic factor in AMD, and implicate a role for cholesterol and antioxidant micronutrient (lutein and vitamin E) metabolism in AMD.

Purpose: Choroidal neovascularization (CNV) secondary to traumatic rupture of Bruch's membrane is a rare condition, without standardized treatment. Here we describe one case of CNV related to traumatic rupture of Bruch's membrane which was successfully treated with intravitreal injection of ranibizumab., Methods: A 14-year-old patient was referred for ocular contusion, complicating interpapillomacular rupture of Bruch's membrane in left eye. Indeed, a correct initial visual acuity, juxtafoveolar CNV appeared 4 months later on the border of Bruch's membrane rupture. The patient was treated with an off-label intravitreal ranibizumab because of worsening of visual acuity., Results: One month after intravitreal injection, visual acuity improved, from 20/40 to 20/25. At 12-month follow-up, visual acuity remained at 20/25, fundus examination. Fluorescein angiography, indocyanine green angiography and optic coherence tomography showed fibrotic evolution of CNV. The Bruch's membrane rupture remained stable. No side-effect of intravitreal injection of ranibizumab was observed., Conclusion: For this patient affected with CNV secondary to traumatic Bruch's membrane, one single intravitreal ranibizumab injection was efficient, with 1-year follow-up.

Purpose: Central areolar choroidal dystrophy is an inherited autosomal dominant macular disease characterized by a central atrophy of the retinal pigment epithelium and choriocapillaris. Our purpose was to describe fluorescein angiography and confocal indocyanine green angiography features of central areolar choroidal dystrophy., Methods: We performed a complete ophthalmologic examination including best corrected visual acuity, fundus examination, color fundus photographs, red free frames, fluorescein angiography, infrared, confocal indocyanine green, and electroretinography in a cohort of patients with a family history of central areolar choroidal dystrophy., Results: Eleven patients (22 eyes) affected were prospectively included. Indocyanine green differentiated two distinct phenotypes. In 9/11 patients, atrophy area was hyperfluorescent or normofluorescent. In the two other patients, the lesion was hypofluorescent from early to late phases and pinpoints were observed on the late phases., Conclusion: In our small series, indocyanine green angiography distinguished two phenotypes of central areolar choroidal dystrophy, correlated with fluorescein angiography features.

Purpose: To describe a patient treated with ranibizumab for juxtafoveal choroidal neovascularization secondary to choroidal nevus., Methods: A 71-year-old man presented with juxtafoveal choroidal neovascularization secondary to choroidal nevus. Choroidal neovascularization was initially treated with a single intravitreal injection of ranibizumab., Results: The treatment was successful and best-corrected visual acuity improved from 20/50 to 20/25 and remained stable during 7 months. A recurrence was observed 9 months after initial treatment., Conclusion: Ranibizumab seems to be an effective treatment for choroidal neovascularization secondary to choroidal nevus. However, late recurrence may occur.

Age-related macular degeneration (ARMD) is a multifactorial and polygenic disease and is the main cause of vision loss in developed countries. The environmental factors of ARMD can modify prevalence and incidence of this disease. This article is a review of the main environmental factors currently recognized as at risk or protective factor for ARMD. Modification of these factors is of crucial importance because it could delay the onset of exudative or atrophic forms of the disease.

We describe the abnormalities seen in the mid periphery and posterior pole of two patients with multifocal vitelliform macular dystrophy as evaluated by high-definition spectral domain optical coherence tomography (HD-OCT). In patient 1, HD-OCT scans revealed, in the central area, a thicker and more reflective layer compared with the normal macula, located between the retinal pigment epithelium and the interface of the inner segment/outer segment, corresponding to the Verhoeff's membrane. Moreover, HD-OCT macular scans, as well as C-scans, revealed a slight hyper-reflective lesion just above an area of reduced reflectivity between the photoreceptor layer (interface of the inner segment and outer segment) and the Verhoeff's membrane. In patient 2, on HD-OCT macular scans, the layer corresponding to the interface of inner segment and outer segment of the photoreceptor, and the Verhoeff's membrane, appeared disrupted, whereas the retinal pigment epithelium layer appeared preserved. On the other hand, in both patient 1 and 2, the clinically evident vitelliform lesions outside the macular area appeared on HD-OCT scans either as small focal hyper-reflective lesions at the level of the retinal pigment epithelium/photoreceptor complex, either as a more pronounced diffuse thickening of the retinal pigment epithelium/photoreceptor complex, facing the deposition of lipofuscin reported on the histopathologic examination. These new findings would help in a further understanding of multifocal vitelliform macular dystrophy.

Purpose: To describe the optical coherence tomography (OCT) features of X-linked choroideremia (CHM)., Design: Prospective, observational case series., Patients and Methods: Color fundus photography, fluorescein angiography, indocyanine green angiography, and OCT were performed for four patients (eight eyes) with CHM., Results: In areas of angiographic choriocapillaris loss, OCT revealed retinal thinning (mean, 124 μm [range, 97-150 μm) and increased reflectivity of the choroid. On the contrary, in areas of preserved choriocapillaris, retinal thickness was within normal limits (mean, 256 μm [range, 238-284 μm]; P < 0.001)., Conclusion: OCT revealed a correlation between retinal thickness and choriocapillaris preservation in CHM.

Aim: Wet age-related macular degeneration (AMD) represents a heterogeneous group of phenotypes, all defined by fluorescein angiography features (FA). Imaging of wet AMD is extensively described in literature, including colour pictures, FA, indocyanine green angiography (ICG) and optical coherence tomography (OCT). The purpose of this study was to describe features of infrared (IR) pictures of a homogeneous subgroup of classic choroidal neovascularisation (CNV) associated with wet AMD, METHODS: We analysed 22 eyes of 22 consecutive patients with classic CNV. All patients underwent a complete ophthalmological examination including colour fundus photography, infrared picture, fluorescein angiography, indocyanine green angiography and an optical coherence tomography., Results: Infrared pictures revealed a whitish ring surrounding the neovascular lesion in all eyes (22/22). The whitish ring corresponded in all cases to the borders of the CNV defined on the early phase of FA and ICG pictures. The ring had an "O-shape" in 15/22 cases (68%) and a "U-shape" in 7/22 cases (32%)., Conclusion: Analysis of infrared pictures in classic CNV constantly revealed a whitish ring that is correlated to the limits of the lesion. IR picture is a non invasive imaging of the macula, but the specificity of the features needs to be investigated in further studies.

Purpose: To create a pilot study in order to evaluate the feasibility of a prospective case-control study of oral supplementation with fish oil (docosahexaenoic acid [DHA]; eicosapentaenoic acid [EPA]) in a population with age-related macular degeneration (AMD)., Methods: A homogeneous group of 38 patients with drusenoid pigment epithelial detachment in one eye (PED) without choroidal new vessels (CNV) was selected. A complete ophthalmologic examination, and a complete profile of fatty acids in serum (S) and in red blood cell membranes (RBCM), were recorded at day 0 and month 6. In group 1, 22 patients were orally supplemented with EPA (720 mg/day) and DHA (480 mg/day) during 6 months. In group 2, 16 patients were followed as controls. Nutritional recommendations on fish consumption were given to both groups., Results: In group 1, after 6 months supplementation we observed a significant blood enrichment in EPA (EPA-S: 2.20 vs 0.79, p<0.0001 and EPA-RBCM: 2.24 vs 0.85, p<0.0001) and in DHA (DHA-S: 2.47 vs 1.56, p<0.0001 and DHA-RBCM: 6.47 vs 4.67, p<0.0001). No change was observed in group 2 despite nutritional recommendations. In this short followup, no evolution to CNV was noted in either of the two groups. Neither side effects nor dropouts were observed in either of the groups., Discussion: This study supports the feasibility of a long-term double-masked prospective case-control study in an AMD population in order to evaluate a potential benefit from oral supplementation with DHA.

Aim: To describe a patient with type 3 choroidal neovascularization (CNV) associated with fundus flavimaculatus (FFM), who underwent treatment with intravitreal ranibizumab., Methods: A 78-year-old woman diagnosed with FFM presented at our department complaining of decreased vision and metamorphopsia in her left eye. Upon a complete ophthalmologic examination, including best corrected visual acuity (BCVA), fundus autofluorescence, fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT), the patient was diagnosed with type 3 CNV associated with FFM, and was submitted to intravitreal ranibizumab injections at monthly intervals., Results: Six months after 3 monthly injections of ranibizumab, the patient's BCVA improved from 20/64 to 20/32. FA and ICGA revealed a type 3 CNV closure, and the SD-OCT scan showed a fibrous scar replacing the type 3 CNV, with resolution of serous retinal detachment., Conclusion: This case represents the first demonstration of type 3 CNV associated with FFM. Based on our findings, intravitreal ranibizumab may be considered as a therapeutic option for this rare association.

Introduction: To assess photoreceptor (PR) layer morphology in patients with Stargardt's disease (STGD) and fundus flavimaculatus (FFM) using high resolution spectral domain optical coherence tomography (HD-OCT; OCT 4000 Cirrus, Humphrey-Zeiss, San Leandro, CA)., Methods: This was a prospective observational case series. Sixteen consecutive patients with STGD and FFM underwent a complete ophthalmologic examination. Optical coherence tomography examination was performed with HD-OCT, a high-speed (27,000 axial scans per second) OCT system using spectral/Fourier domain detection, with an axial image resolution of 5 mum., Results: A total of 31 eyes were included in the study. Transverse loss of the PR layer in the foveal region was shown by HD-OCT. Twenty eyes with clinically evident central atrophy had a disruption of either the Verhoeff's membrane (VM) or the layer corresponding to the interface of inner segment (IS) and outer segment (OS) of PR in the foveal region. Among these eyes, 12/20 eyes had a loss of the PR layer (loss of both VM and IS-OS interface) in the foveal region. Eleven eyes (11/31) without clinically evident central atrophy had an intact interface of IS and OS of PR centrally. Moreover, we observed hyperreflective deposits: type 1 lesions located within the retinal pigment epithelium (RPE) layer and at the level of the outer segments of PR, and type 2 lesions located at the level of the outer nuclear layer and clearly separated from the RPE layer. Type 1 lesions alone were associated with absence of loss of the PR layer in the foveal region in all eyes; type 2 lesions were always associated with presence of type 1 lesions, and often (8/12 eyes) associated with loss of the PR layer within the foveal region. Mean best-corrected visual acuity (BCVA) was significantly correlated with loss of the PR layer in the foveal region (P < 0.001), as well as to presence of type 2 flecks (P = 0.03)., Conclusion: Type 2 deposits in STGD/FFM patients seem to represent a marker of the possible evolution towards foveal atrophy.