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To describe the structural changes observed postoperatively in epiretinal membranes (ERM), in particular the alterations in the central cone bouquet (CB), and to identify prognostic factors that might predict postoperative outcome. We included 125 eyes of 117 patients who underwent idiopathic ERM removal with at least a 6-month follow-up. For each patient, spectral-domain optical coherence tomography (SD-OCT) was performed and best-corrected visual acuity (BCVA) was measured, before and after surgery. Before surgery, 44 eyes (35.2%) presented CB alterations: 65.9% a cotton ball sign, 15.9% a foveolar detachment and 18.2% a pseudovitelliform lesion. Median BCVA increased from 20/63 to 20/32 post-operatively (p = .001) with a mean follow-up of 17 months. The disappearance of CB alterations after surgery was observed in 97.7% of eyes. In stage 3 and 4 ERM, ectopic inner foveal layers persisted in 76.7% of eyes after surgery. Postoperative BCVA was correlated with change in central macular thickness and initial BCVA and was not correlated with the presence of preoperative CB alteration, the initial stage of ERM, the presence of postoperative dissociated optical nerve fiber layer, and the disappearance of ectopic inner fiber layers. The combination of cataract surgery and capsulotomy did not seem to change visual outcome and seemed to accelerate visual recovery. Incidentally, general anesthesia was correlated with final BCVA. ERM surgery allowed a significant gain in BCVA and the disappearance of CB alterations in the great majority of cases. CB alteration did not show to be associated with poor visual prognosis.

Purpose To describe the spectral-domain optical coherence tomography (OCT) features of fibrotic lesions associated with neovascular age-related macular degeneration (nAMD) and to outline the progression pathways from initial macular choroidal neovascular lesions (CNVs) to fibrosis. Methods Patients with nAMD were retrospectively included when macular subretinal fibrosis was present. Fibrosis was categorized using spectral-domain OCT with respect to retinal pigment epithelium (RPE) in 836 spectral-domain OCT slices from 44 eyes of 39 patients. In addition, in 47 distinct eyes, 4181 spectral-domain OCT slices were retrospectively reviewed to longitudinally assess progression from the initial lesion to the final fibrosis. Results Cross-sectional analysis classified fibrosis on spectral-domain OCT slices, as type A if located underneath the RPE, as type B if located above the RPE, and as type C if the remaining RPE was undistinguishable. The longitudinal analysis series revealed 3 progression pathways from the original CNV: 1) progression to type A, followed by RPE erosion and subretinal hyperreflective material, then type B and type C fibroglial lesion (FGL; 17/47 eyes); 2) progression to type B then type C FGL (17/47 eyes); and 3) persistence of type A with development of a flat, fibroatrophic lesion (13/47 eyes). Subretinal hyperreflective material, macular hemorrhage, or RPE tear occurred in 14 of 47, 13 of 47, and 10 of 47 eyes, respectively. Conclusion This spectral-domain OCT analysis identified various patterns of macular fibrosis in eyes with nAMD. Three pathways of progression to fibrosis were described including the well-established pathway of type 2 CNV progression to FGL and the progression of type 1 fibrovascular CNV to FGL or fibroatrophic lesion.

The authors present an unusual case of a 6-year-old boy with myopia and foveal hypoplasia who was diagnosed by optical coherence tomography angiography. This case report presents the importance of using optical coherence tomography angiography in the diagnosis of a different etiology. [ J Pediatr Ophthalmol Strabismus . 2022;59(2):e23–e24.]

Purpose: To evaluate anatomical and functional changes in patients with vitreomacular interface disease after internal limiting membrane (ILM) peeling, using microperimetry along with usual clinical and multimodal retinal imaging. Methods: Patients with vitreomacular interface disease requiring vitrectomy underwent multimodal retinal evaluation, including visual acuity assessment, fundus color photograph, Spectral-Domain Optical Coherence Tomography, Optical Coherence Tomography-Angiography, and microperimetry. They were examined at baseline (M0), 6 months (M6) and 18 months (M18) after surgery. Retinal sensitivity was subdivided into three concentric polygons: Large, Medium, Small. Results: Eleven eyes of 11 patients were analyzed, including 10 epiretinal membranes (ERMs). Best-corrected visual acuity (BCVA) improved in all patients from 0.51 logarithm of the minimal angle of resolution (logMAR) to 0.067 ( p = 0.0074). Retinal sensitivity improved between M0 and M6 in all polygons and continued to improve between M6 and M18 for polygons Medium (M) and Small (S) with no statistical significance. At M18, BCVA and retinal sensitivity were similar in the operated eye compared with the fellow eye for all patients. Dissociated optic nerve fiber layer appearance was observed in 8 patients at M18. It was not correlated with either retinal sensitivity or BCVA or microscotomas. No recurrence of ERM or macular hole occurred during follow-up. Conclusion: After surgery, the retinal sensitivity assessed by microperimetry gradually improved until the 18th month and was not different from the values of the fellow eye. These results seemed to confirm that ILM peeling can be an effective and safe technique to treat patients with vitreomacular interface disease.

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

Treatment-resistant pigment epithelial detachments can be effectively treated with aflibercept switch therapy. Patients were treated with 3 monthly intravitreal injections and then a Q6 regimen. Mean pigment epithelial detachment height and volume were statistically reduced after the switch at primary endpoint (12 weeks), and best-corrected visual acuity also improved steadily throughout the duration of the study., Purpose: To analyze the efficacy of aflibercept switch treatment for regression of pigment epithelial detachment (PED) in patients previously treated with ranibizumab. Methods: Multicenter, prospective, nonrandomized clinical trial. One eye of patients presenting neovascular age–related macular degeneration with PED of more than 250 μm in height, with persistent fluid, was included. Patients had to have received at least six ranibizumab intravitreal injections during the 12 months before enrollment. Patients were switched from ranibizumab pro re nata to aflibercept (fixed regimen, 3 monthly intravitreal injections, and then Q6). Main outcome measure was change in PED height from baseline to Week 12 after switch. Secondary outcomes were best-corrected visual acuity and PED volume changes. Results: Eighty four patients were included. Mean delay between last ranibizumab intravitreal injection and switch was 44.7 days. Mean maximal PED height at baseline visit was 347 μm (±109) and reduced to a mean of 266 μm (±114) at Week 12 (P < 0.001) and 288.2 μm at Week 32 (P < 0.001). Mean PED volume was reduced from 1.3 mm3 to 0.98 mm3 at Week 12 (P < 0.001). Best-corrected visual acuity improved by 3.3 Early Treatment Diabetic Retinopathy Study letters at Week 32 (P = 0.003). Conclusion: Aflibercept switch therapy seems to be effective on large PED in patients previously treated with pro re nata ranibizumab.

To evaluate the diagnostic accuracy of OCT angiography (OCT-A) detecting or predicting choroidal neovascularization (CNV), by ophthalmologists of disparate degrees of skills in retinal diseases, using spectral domain optical coherence tomography (SD-OCT) and fluorescein angiography (FA) as a standard reference. Retrospective observational case series. Patient presenting maculopathy and complete imaging were included. FA, SD-OCT, OCT-A and FA coupled to SD-OCT images were graded independently for presence or absence of CNV by ophthalmologists with varying expertise levels. Overall sensitivity of OCT-A was 85.62% (95% CI 79.04–90.76%) and specificity was 81.51% (95% CI 73.36–88.03). Sensitivity of FA was 74.51% (95% CI 66.84–81.20), and specificity was 82.35% (95% CI 74.30–88.73). Sensitivity of FA + SD-OCT was 92.72% (95% CI 87.34–96.30), and specificity was 90.91% (95% CI 84.31–95.37). OCT-A has good sensitivity and specificity for the detection of CNV in all expertise level groups. OCT-A may soon become a routine tool for CNV diagnosis and follow-up.

Contains fulltext : 220965.pdf (Publisher’s version ) (Closed access)

Item does not contain fulltext PURPOSE: To compare the outcome between high-density subthreshold micropulse laser (HSML) treatment and half-dose photodynamic therapy (PDT) in chronic central serous chorioretinopathy (cCSC) patients, subdivided based on either focal or diffuse leakage on fluorescein angiography (FA). DESIGN: Retrospective analysis of multicenter randomized controlled trial data. METHODS: Patients were treated with either half-dose PDT or HSML (both indocyanine green angiography-guided) and categorized in 2 groups, based on focal or diffuse leakage on FA. Clinical outcomes were evaluated at baseline and during follow-up. RESULTS: In the focal leakage group (63 patients), both at first evaluation and at final visit, more PDT-treated than HSML-treated patients demonstrated a resolution of subretinal fluid (evaluation visit 1: 57% in the PDT group and 17% in the HSML group, P = .007; final visit: 75% and 38%, P = .012). In the diffuse leakage group (93 patients), both at first evaluation and at final visit, more PDT-treated than HSML-treated patients showed a resolution of subretinal fluid (evaluation visit: 1:48% in the PDT group and 16% in the HSML group, P = .002; final visit: 67% and 21%, P = .002). PDT-treated patients in the focal and diffuse leakage group had a higher retinal sensitivity increase, comparing baseline and final visit (+3.1 +/- 3.1 dB vs +1.2 +/- 4.0 dB, P = .048, and +2.7 +/- 3.3 dB vs +1.0 +/- 3.8 dB, P = .036, respectively). Only in the diffuse leakage group, the increase in ETDRS letters was higher in the PDT-treated group when comparing baseline and first evaluation visit (+4.4 +/- 6.1 vs +0.9 +/- 10.0, P = .049). CONCLUSIONS: Half-dose PDT is superior to HSML treatment in cCSC patients, regardless of the presence of focal or diffuse leakage on FA.

BACKGROUND AND OBJECTIVE: To describe and present the reliability and reproducibility of a new software, Retinal Volume Analyzer (ReVAnalyzer), for pigment epithelium detachment (PED) volume quantification. PATIENTS AND METHODS: This is a retrospective study including patients with PEDs secondary to exudative age-related macular degeneration (AMD). Macular volume scans on spectral-domain optical coherence tomography on enhanced depth imaging mode were performed in all eyes. Image batches were then exported in .xml format to the ReVAnalyzer software. A semiautomated PED volume measurement was performed by three independent readers (RBG, VC, OS) twice, at the beginning and end of a 15-day period. Bland-Altman assessment for agreement was used to compare intra- and interobserver observations. RESULTS: Twenty eyes of 20 patients presenting with PED were analyzed. Bland-Altman analysis indicated a good agreement between inter- and intraobserver measurements. The intraclass correlation coefficient for intraobserver PED volume measurements and between the three observers (interobserver) was greater than 0.99, demonstrating high reproducibility and consistency of the methodology. CONCLUSIONS: ReVAnalyzer is a reliable tool that can assist in the analysis of PED volume with high reproducibility. This type of specific retinal volume analysis can be of help for monitoring disease activity and therapeutic response in AMD. [ Ophthalmic Surg Lasers Imaging Retina . 2019;50:e242–e249.]

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

Purpose: To describe the characteristics and potential differences between focal and diffuse phenotypes of untreated chronic central serous chorioretinopathy (cCSC). Methods: For this study, patients were divided in two groups. Focal leakage was defined as 1 “hot spot” of leakage, whereas diffuse leakage was defined as either > 1 hot spot or a larger area of widespread leakage on FA. Clinical characteristics were assessed at presentation. After Bonferroni correction, P values < 0.00125 were deemed statistically significant. Results: The focal leakage group included 68 eyes (53 males), and the diffuse leakage group included 105 eyes (88 males). Mean best-corrected visual acuity (BCVA) was 77.1 ± 8.1 Early Treatment of Diabetic Retinopathy Study (ETDRS) letters in the focal group and 76.0 ± 9.6 ETDRS letters in the diffuse group (p = 0.440). In the focal group, mean age was 46.9 ± 8.8years, whereas this was 49.7 ± 8.3years in the diffuse group (p = 0.033). Mean central foveal thickness was 107.1 ± 21.3μm in the focal group and 106.2 ± 27.3μm in the diffuse group (p = 0.818). Mean choroidal thickness was 407.5 ± 114.8μm in the focal group and 419.1 ± 113.9μm in the diffuse group (p = 0.578). In the focal group, subretinal fluid was present in the fellow eye in 16% of the patients, as compared to 29% in the diffuse group (p = 0.067). Conclusions: In untreated cCSC patients with focal or diffuse leakage on FA, no marked differences in clinical characteristics were found. Extensive choroidal abnormalities may be present in both groups, which are presumed to lie at the basis of the development of cCSC.

PURPOSE We hypothesized that severe forms of neovascular age-related macular degeneration (AMD) such as large pigment epithelial detachments poorly responding to anti-vascular endothelial growth factor therapy might present a distinct genotype compared with overall series of neovascular AMD. METHODS This is a multicenter genetic association study. Sixty-eight patients presenting pigment epithelial detachments resistant to ranibizumab (issued from ARI2 study, register number NCT02157077 on clinicaltrials.gov) were compared with two series of patients derived from previously published clinical studies, presenting neovascular AMD (NAT2 study n = 300 and PHRC study n = 1,127), and with healthy controls (n = 441). The phenotype of neovascular AMD groups was based on visual acuity measurement, fundus examination, spectral-domain optical coherence tomography, and angiographic data. All samples were genotyped for three single-nucleotide polymorphisms: CFH (rs1061170), ARMS2 (rs10490924), and C3 (rs2230199). Significant difference in allele frequency between participants with neovascular AMD and control was the main outcome measurement. RESULTS The GG genotype of the C3 rs2230199 was significantly more frequent in the ARI2 group (55.9%) than the PHRC group (6.0%, P < 0.0001; odds ratio = 24.0 [95% confidence interval 10.4-55.0]) and the NAT2 group (5.1%, P < 0.0001; odds ratio = 16.1 [95% confidence interval 5.0-51.9]). The repartition of patients carrying a T allele of the ARMS2 (rs10490924) or patients carrying a C allele of the CFH (rs1061170) was similar in the ARI2 group when compared with the NAT2 and PHRC groups. CONCLUSION In our series, the genotype GG of C3 rs2230199 was more significantly associated with the phenotype of large vascularized pigment epithelial detachment poorly responding to anti-vascular endothelial growth factor therapy than in global AMD series.

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

Contains fulltext : 196622.pdf (Publisher’s version ) (Open Access) PURPOSE: To compare the anatomic and functional efficacy and safety of half-dose photodynamic therapy (PDT) versus high-density subthreshold micropulse laser (HSML) treatment in patients with chronic central serous chorioretinopathy (cCSC). DESIGN: Open-label, multicenter, randomized controlled clinical trial. PARTICIPANTS: Patients with cCSC whose disease had to be confirmed by both clinical characteristics and findings on multimodal imaging. METHODS: Eligible patients were randomized in a 1:1 allocation ratio. Treatment was evaluated during a follow-up visit, and the same treatment was repeated in patients who still demonstrated subretinal fluid (SRF). MAIN OUTCOME MEASURES: The primary end point was the complete disappearance of SRF at the first evaluation visit at 6 to 8 weeks after treatment. As a secondary outcome measure, we assessed this anatomic result at the final evaluation visit at 7 to 8 months after treatment. Other secondary outcomes covered functional improvement and included change in best-corrected visual acuity (BCVA; measured in Early Treatment Diabetic Retinopathy Study [ETDRS] letters), retinal sensitivity (measured using microperimetry), and vision-related quality of life using a validated questionnaire. RESULTS: Between November 2013 and September 2016, 179 patients were included: 89 patients were assigned randomly to half-dose PDT, and 90 were assigned randomly to HSML treatment. At their first evaluation visit, SRF had resolved in 51.2% and 13.8% of patients, respectively (P < 0.001). At their final evaluation visit, a significantly higher percentage of PDT-treated patients demonstrated no SRF (67.2% vs. 28.8%; P < 0.001). Moreover, at the first evaluation visit, the PDT-treated patients showed a significantly higher increase in BCVA (+4.60+/-6.62 ETDRS letters vs. +1.39+/-8.99 ETDRS letters; P = 0.011), and a significantly higher increase in retinal sensitivity on microperimetry (+2.01+/-3.04 dB vs. +0.92+/-3.65 dB; P = 0.046); however, the improvement in vision-related quality of life was similar (score of +2.87+/-8.35 vs. +2.56+/-7.36, respectively; P = 0.800). CONCLUSIONS: Half-dose PDT is superior to HSML for treating cCSC, leading to a significantly higher proportion of patients with complete resolution of SRF and functional improvement.

To describe the phenotype and genotype of a 10-year-old boy affected with enhanced S-cone syndrome associated with neovascularization.Fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, full-field electroretinogram and NR2E3 molecular testing were performed.Best-corrected visual acuity was measured as 20/32, right eye and 20/20, left eye. Fluorescein and indocyanine green angiographies showed unilateral macular retinochoroidal anastomosis on his right eye, and spectral domain optical coherence tomography showed typical signs of subretinal exudation and foveolar pseudoschisis consistent with the diagnosis of enhanced S-cone syndrome. Genetic analysis revealed biparental transmission of mutations in the enhanced S-cone syndrome-causing gene, NR2E3, namely, c.194_202del (p.Asn65_Cys67del), and c.932 GA (p.Arg311Gln), supporting an autosomal recessive inheritance. The patient received three intravitreal injections of anti-VEGF agents.Evidence of retinochoroidal anastomosis in an individual affected with enhanced S-cone syndrome supports the view that neovascularization can occur early in the course of the disease, and raises the question to know whether it might be responsible for previously described enhanced S-cone syndrome-associated hemorrhage-induced fibrosis.

Background/purpose Neovascular age-related macular degeneration (nAMD) is frequently associated with vascularized pigment epithelial detachment (v-PED). We observed a peculiar characteristic of v-PED characterized by small lacy folds of the retinal pigment epithelium, appearing as a wrinkled PED (w-PED) on spectral domain optical coherence tomography (SD-OCT). Our purpose was to describe the visual prognosis and number of intravitreal injections in w-PED compared with non-w-PED. Methods In this retrospective, case-control series, we reviewed retrospectively medical records of 52 eyes of 51 patients who were consecutively included between November 1 and 30, 2015 with a previous minimum 3-year follow-up. Inclusion criteria were: neovascular age-related macular degeneration, affected with w-PED. Baseline characteristics, best-corrected visual acuity (BVCA), number of intravitreal anti-vascular endothelial growth factor injections (anti-VEGF IVT) and maximal recurrence-free interval, that is, without intravitreal anti-vascular endothelial growth factor injection, were analyzed. A w-PED was defined as a v-PED ≥200 μm in height on SD-OCT imaging, presenting with at least 4 small lacy folds on the surface of the retinal pigment epithelium. Patients were compared with a control group, that is, patients harboring PED without wrinkle shape (non-w-PED). All patients had been treated by intravitreal anti-vascular endothelial growth factor injection of either ranibizumab (IVR) or aflibercept (IVA) using a pro re nata (PRN) protocol after three initial monthly treatments, with a minimum of follow-up of 3 years. Results Two groups of patients were compared, w-PED (29 eyes, from 29 patients), and non-w-PED (23 eyes from 22 patients). In the w-PED group, mean BCVA evolved from 0.28 (±0.18) log MAR (20/40, range 20/25-20/63) at baseline, to 0.29 (±0.21) log MAR (20/40, range 20/25-20/63) at 1 year (P = 0.41), 0.34 (±0.26) log MAR (20/40, range 20/25-20/80) at 2 years (P = 0.49), 0.35 (±0.28) log MAR (20/40, range 20/25-20/80) at 3 years (P = 0.54). In the non-w-PED group, mean BCVA was 0.40 (±0.28) log MAR (20/50, range 20/25-20/100) at baseline and decreased to 0.48 (±0.46) log MAR (20/63, range 20/20-20/160) at 1 year (P = 0.19), 0.48 (±0.35) log MAR (20/63, range 20/25-20/125) at 2 years (P = 0.02), 0.60 (±0.38) log MAR (20/80, range 20/32-20/200) at 3 years (P = 0.002). In the w-PED group, the mean maximal documented recurrence-free interval was 7.87 (±2.94) months at Year 1, 13.5 (±7.52) at Year 2 and 14.78 (±10.70) at Year 3, versus 4.59 (±2.95) months at Year 1, 7.83 (±6.62) at Year 2, 8.57 (±11.18) at Year 3 in the non-w-PED group (P = 0.0004; 0.0101; 0.0168 respectively at Years 1, 2 and 3). Discussion The evolution of v-PED after intravitreal anti-vascular endothelial growth factor injection is still difficult to predict despite intense clinical research in this topic. In our study, we noticed that w-PED might be a phenotypic prognosis factor for better visual acuity and longer maximal recurrence-free interval.

Purpose. - Extensive macular atrophy with pseudodrusen-like appearance (EMAP) is a recently described entity. We describe the first observations of choroidal neovascularization (CNV) associated with EMAP in 3 patients. Methods. - Nineteen consecutive patients with EMAP were retrospectively investigated for the presence of CNV and treatment outcomes. Each patient underwent a complete ophthalmologic examination including color fundus photograpy, fluorescein angiography (FA), indocyanine green angiography (ICG) and spectral-domain optical coherence tomography (SD-OCT). Results. - Retrospective analysis revealed choroidal neovascularization in 3 patients (4 eyes) out of 19 patients with EMAP. In these patients, laser photocoagulation or intravitreal injections of ranibizumab led to resolution of retinal exudation with limited functional improvement. Conclusion. - CNV is a possible complication of EMAP, a recently reported form of macular atrophy resembling geographic atrophy. Laser photocoagulation and anti-VEGF treatment appear to be two valuable therapeutic options. (C) 2014 Elsevier Masson SAS. All rights reserved.

Purpose: We observed hyperreflective dome-shaped or pyramidal structures (HPS) on spectral domain optical coherence tomography (SD-OCT) in patients affected with geographic atrophy (GA). Our purpose was to describe the multimodal imaging features of HPS identified in areas of GA in patients with age-related macular degeneration. Methods: This is a retrospective case series of patients with GA harboring HPS in atrophic areas. Multimodal imaging examination including infrared reflectance, fundus autofluorescence, and SD-OCT, was performed for each patient. Infrared and fundus autofluorescence appearance and mean SD-OCT height of HPS in GA were analyzed. Results: A total of 36 eyes of 25 patients (20 women; mean age, 82.3 +/- 5.9 years, range, 73-92 years) with GA were included. A total of 96 HPS in GA were analyzed by SD-OCT. In all HPS (96/96, 100%), the peripheral part was hyperreflective. In 66 of 96 HPS (69%), the center was heterogeneously hyperreflective, whereas in 30 of 96 HPS (31%), the center was hyporeflective. On infrared reflectance images, HPS in GA appeared as hyporeflective lesions surrounded by hyperreflective halos, within an area of background hyperreflectivity because of GA in all eyes. On fundus autofluorescence, 39 of 96 HPS (41%) were heterogeneously hyperautofluorescent, whereas 57 of 96 HPS (59%) were hypoautofluorescent. Mean height of HPS was 91 +/- 50.9 mu m in the foveal scan (range, 42-291 mu m). Conclusion: We describe a multimodal imaging of distinctive lesions that presented as hyperreflective pyramidal structures on SD-OCT. We suggest the name "ghost drusen" because these HPS appear in GA areas, and because of their pyramidal or dome-shaped aspect on SD-OCT.

Purpose To investigate the appearance of medium–large drusen and reticular pseudodrusen on adaptive optics (AO). Methods In 14 consecutive patients, AO infrared (IR) images were overlaid with confocal scanning-laser-ophthalmoscope IR reflectance images and IR-referenced spectral-domain optical coherence tomography. Results In eight eyes of six patients, a total of 19 images of medium–large drusen were investigated by AO imaging. En face AO revealed medium–large drusen as highly hyper-reflective round/oval lesions, always centred and/or surrounded by a continuous/discontinuous hyporeflectivity. Cone photoreceptors were detected overlying drusen, appearing either as continuous ‘bright’ hyper-reflective dots over a ‘dark’ hyporeflective background, or as continuous ‘dark’ hyporeflective dots over a ‘bright’ hyper-reflective background. In eight eyes from eight patients, a total of 14 images of pseudodrusen were investigated by AO imaging. En face AO revealed reticular pseudodrusen as isoreflective lesions, always surrounded by a continuous/discontinuous hyporeflectivity. Cone photoreceptors were detected overlying pseudodrusen as ‘bright’ hyper-reflective dots over either a hyporeflective or isoreflective background. No ‘dark’ hyporeflective dots were detected in eyes with reticular pseudodrusen only. Cone photoreceptors were counted on the border of the drusen and pseudodrusen, respectively, and in a visibly healthy zone in its absolute vicinity. A similar decrease in cone appearance was observed for drusen and pseudodrusen (15.7% vs 16.2%). Conclusions AO allows differences in reflectivity between medium–large drusen and reticular pseudodrusen to be appreciated. The cone mosaics may be detected as continuous ‘bright’ hyper-reflective dots overlying/on the border of drusen and pseudodrusen deposits, and possibly as continuous ‘dark’ hyporeflective dots overlying drusen only.

To assess the anatomical and functional efficacy of ranibizumab on vascularized pigment epithelial detachment (V-PED) secondary to neovascular age-related macular degeneration (nAMD). One hundred and nine patients (116 eyes) were retrospectively selected from medical records of 2097 patients who benefited from intravitreal injection between January 2011 and June 2013 in a tertiary-care University-based Department of Ophthalmology. Inclusion criteria were: nAMD, treatment-naive eyes, presence of V-PED higher than 250 μm, intravitreal ranibizumab with a loading phase, followed by a pro-re-nata regimen, and 1-year follow-up. Baseline characteristics and type of choroidal neovascularization (CNV) were analyzed. PED height, central macular thickness (CMT) and best-corrected visual acuity (BCVA, logMAR) were measured at baseline, months 3, 6 and 12. CNV was of type 1 in 91 eyes (78.4 %), type 2 in seven (6 %), type 3 in six (5.2 %), and polypoidal choroidal vasculopathy in 12 (10.3 %). Mean CMT at baseline was 572.1 μm and decreased to 396.6 μm (p

Purpose To assess the difference in best-corrected visual acuity (BCVA) at presentation between the first and second eye in patients with bilateral neovascular age-related macular degeneration (AMD). Methods We reviewed the charts of all patients who had a clinical examination for neovascular AMD at the University Eye Clinic of Creteil in January 2013. We retrospectively analyzed demographic and clinical data for 264 patients. Results In the fellow eye, choroidal neovascularization (CNV) developed in 75/264 patients (28.4%) with a time interval between the 2 events of 30.3 months (range 6-145). Data were available on 65 patients: 14/65 (21.5%) were asymptomatic, 24/65 (36.9%) had BCVA >20/40, whereas at the time of CNV diagnosis in the first eye, no patient was asymptomatic (p20/40 (pConclusions The BCVA at the time of diagnosis of CNV was higher in the second eye than in the first affected eye. This was possibly due to several factors including systematic bilateral examination in follow-up of unilateral exudative AMD that allowed detection of 20% of cases.

Contains fulltext : 154838.pdf (Publisher’s version ) (Open Access) BACKGROUND: Chronic central serous chorioretinopathy (cCSC) is an eye disease characterized by an accumulation of serous fluid under the retina. It is postulated that this fluid accumulation results from hyperpermeability and swelling of the choroid, the underlying vascular tissue of the eye, causing a dysfunction of the retinal pigment epithelium. This fluid accumulation causes neuroretinal detachment. A prolonged neuroretinal detachment in the macula can lead to permanent vision loss. Therefore, treatment is aimed primarily at achieving resolution of subretinal fluid, preferably within the first 4 months after diagnosis of the disease. A broad spectrum of treatment modalities has been investigated in cCSC, but no consensus exists on the optimal treatment of cCSC. Currently, photodynamic therapy (PDT) and high-density subthreshold micropulse laser treatment (HSML) are among the most frequently cited treatments in obtaining successful neuroretinal reattachment. METHODS/DESIGN: This is a randomized, controlled, open-label, multicenter trial comparing the efficacy of half-dose PDT to HSML in treating patients with cCSC. A total of 156 patients will be recruited, 78 patients in each treatment arm, with a maximum follow-up duration of 8 months after the first treatment. A complete ophthalmological examination with vision-related quality of life (NEI VFQ-25) and stress questionnaires, will be performed at baseline, 6 to 8 weeks after the first treatment, 6 to 8 weeks after a second treatment (if necessary), and at the final follow-up visit at 7 to 8 months after the first treatment. Treatment visits will be scheduled within 3 weeks after the baseline visit, and within 3 weeks after the first control visit, if a second treatment is required. DISCUSSION: Both half-dose PDT and HSML may be effective treatments in cCSC, but because of the lack of prospective randomized controlled trials, which treatment should be the first choice remains unclear. The aim of this study is to compare the efficacy of half-dose PDT to HSML. The primary endpoint to evaluate efficacy will be a complete absence of subretinal fluid on optical coherence tomography after treatment. Secondary functional endpoints include change in Early Treatment Diabetic Retinopathy Study (ETDRS) best-corrected visual acuity, retinal sensitivity on microperimetry, and NEI VFQ-25 questionnaire of visual functioning. Registration number Institutional Review Board (CMO Arnhem-Nijmegen, the Netherlands): 2013/203 NL nr.: 41266.091.13 TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT01797861 . Date of registration: 21 February 2013.

Background:To describe adaptive optics (AO) imaging of foveal sparing in geographic atrophy (GA) secondary to age-related macular degeneration.Methods:Flood-illumination AO infrared (IR) fundus images were obtained in four consecutive patients with GA using an AO retinal camera (rtx1; Imagine Eyes). Adaptive optics IR images were overlaid with confocal scanning laser ophthalmoscope near-IR autofluorescence images to allow direct correlation of en face AO features with areas of foveal sparing. Adaptive optics appearance of GA and foveal sparing, preservation of functional photoreceptors, and cone densities in areas of foveal sparing were investigated.Results:In 5 eyes of 4 patients (all female; mean age 74.2 11.9 years), a total of 5 images, sized 4 degrees x 4 degrees, of foveal sparing visualized on confocal scanning laser ophthalmoscope near-IR autofluorescence were investigated by AO imaging. En face AO images revealed GA as regions of inhomogeneous hyperreflectivity with irregularly dispersed hyporeflective clumps. By direct comparison with adjacent regions of GA, foveal sparing appeared as well-demarcated areas of reduced reflectivity with less hyporeflective clumps (mean 14.2 vs. 3.2; P = 0.03). Of note, in these areas, en face AO IR images revealed cone photoreceptors as hyperreflective dots over the background reflectivity (mean cone density 3,271 +/- 1,109 cones per square millimeter). Microperimetry demonstrated residual function in areas of foveal sparing detected by confocal scanning laser ophthalmoscope near-IR autofluorescence.Conclusion:Adaptive optics allows the appreciation of differences in reflectivity between regions of GA and foveal sparing. Preservation of functional cone photoreceptors was demonstrated on en face AO IR images in areas of foveal sparing detected by confocal scanning laser ophthalmoscope near-IR autofluorescence.

PURPOSE: To investigate the effects of ranibizumab 0.5 mg on gray hyper-reflective subretinal lesions diagnosed by spectral-domain optical coherence tomography (SD OCT) in patients with exudative age-related macular degeneration (AMD). DESIGN: Retrospective interventional study. METHODS: Data from 28 consecutive patients affected with neovascular AMD that presented subretinal hyper-reflective lesions as visualized by SD OCT were collected. Gray hyper-reflective subretinal lesion characteristics were analyzed before and after intravitreal ranibizumab 0.5 mg injection. RESULTS: Thirty eyes of 28 patients (5 male, 23 female, aged 57-91 years) were included. At study entry, gray lesion was associated with exudative features in 24 of 30 eyes (80%), including subretinal fluid (SRF) in 20 of 30 eyes (67%) and retinal cystoid spaces in 11 of 30 eyes (37%). Twenty-four eyes with exudative features at study entry received prompt treatment; 6 eyes without exudative features at study entry received deferred treatment (after 1 month observation), when exudative signs emerged (SRF in 3/6 eyes and retinal cystoid spaces in 5/6 eyes). Ninety-three percent of the gray lesions responded to ranibizumab treatment at 2 months and 77% at 6 months. Gray hyper-reflective subretinal lesion thickness was significantly reduced after treatment at both 2 months (from 482 +/- 116 mu m to 367 +/- 102 mu m, P < .0001) and 6 months (from 482 +/- 116 mu m to 369 +/- 71 mu m, P < .0001). CONCLUSION: Our findings suggest that gray hyper-reflective subretinal lesions might be considered as a qualitative criterion for retreatment of exudative AM:D. They may represent an early sign of active choroidal neovascularization, and should prompt to early treatment. ((C) 2014 by Elsevier Inc. All rights reserved.)

Purpose: To assess intraobserver and interobserver agreement among physicians with different degrees of clinical experience, using a novel fundus autofluorescence semiautomated software for quantification of geographic atrophy in clinical setting. Methods: Fundus autofluorescence frames (excitation: 488 nm; emission: 500-700 nm) of 29 eyes (20 patients; mean age, 79.6 +/- 6.2 years) with geographic atrophy secondary to age-related macular degeneration, and no signs of choroidal neovascularization, were analyzed using Region Finder, a semiautomated software embedded in Spectralis (Heidelberg Engineering, Heidelberg, Germany). For each study eye, semiautomated atrophy identification and quantification were independently performed, twice (in a 2-week time frame), by 3 readers with different degrees of clinical experience (2 fellows, and 1 resident). Intraobserver and interobserver agreements were assessed. Results: Mean difference in intraobserver agreement (Bland-Altman statistics) ranged from -0.17 mm(2) to 0.13 mm(2). Intraobserver agreement was excellent until the geographic atrophy threshold value of 15.72 mm(2). Variability correlated with the size of atrophy. Mean difference in interobserver agreement (Bland-Altman statistics) ranged from -0.25 mm(2) to 0.27 mm(2), with no significant difference between senior and junior readers. Multifocal lesion or foveal involvement in atrophy was not the cause of disagreement. Conclusion: Region Finder is a reliable tool for the identification and quantification of geographic atrophy in patients with age-related macular degeneration, in a clinical setting even when performed by junior reader.

Background Our purpose was to describe the different morphological features in adult onset foveomacular vitelliform dystrophy (AOFVD), using en face enhanced depth imaging (EDI) spectral-domain optical coherence tomography (SD-OCT). Methods Thirty eyes of 22 consecutive patients presenting with diagnosis of AOFVD were enrolled. Diagnosis of AOFVD was concluded based on fundus examination, autofluorescence imaging, fluorescein angiography and SD-OCT. En face OCT imaging was obtained with the Spectralis EDI SD-OCT; 97 inverted sections (nine averaged B-scans per image) were acquired. Results On en face OCT, vitelliform lesions appeared as regular concentric rings of different reflectivity. From the periphery to center of the ring, we observed: (1) the hypereflective ring representing the inner segment/outer segment (IS/OS) junction, which was continuous in 23 out of 30 eyes, and (2) a well-detectable hyporeflective ring between the IS/OS junction and vitelliform material in 20 out of 30 eyes; the innermost composant of the lesion was hypereflective, and it corresponded to vitelliform material. In eight out of 30 eyes, a hyporeflective "croissant"-shaped lesion with inferior concavity in the upper part of the hyperreflective material was present. Hypereflective retinal pigment epithelium (RPE) elevations or bumps were detected in 25 out of 30 eyes. These areas of focal RPE thickening or bumps appeared to be intensely hypereflective on infrared reflectance imaging. Conclusion En face imaging of the retina helps visualizing the distribution of vitelliform material in AOFVD. The sedimentation of vitelliform lesions is characterized by a upper "croissant"-shaped hypoflectivity. The bumps/thickening of RPE appeared as hypereflective lesions on IR imaging.

Purpose: To analyze the genetic and environmental factors associated with reticular pseudodrusen (RPD) in age-related macular degeneration (AMD). Methods: In a large population, AMD patients (n = 519) with and without RPD were assessed with a standardized examination including infrared images and spectral domain optical coherence tomography scans. Three groups were defined: Group 1: AMD patients with RPD (n = 105); Group 2: AMD patients without RPD (n = 414); and Group 3: controls with no AMD and no RPD (n = 430). Four genes associated with AMD (CFH, ARMS2/HTRA1, C3, apolipoprotein E) and environmental factors were assessed between the 3 groups. Results: None of the environmental factors studied were more significantly associated to either Group 1 or Group 2. The odds ratios and 95% confidence intervals for individuals homozygous for the CFH risk allele were 4.0 (2.1-7.7) ([95% confidence interval: 2.1-7.7]; P < 0.0004) in Group 1 and 4.3 ([2.6-7.1]; P < 0.0004) in Group 2, compared with Group 3. The odds ratios for individuals homozygous for the ARMS2 risk allele for Groups 1 and 2 compared with Group 3 were 16.3 ([7.6-35.4]; P < 0.0004) and 11.9 ([6.3-22.3]; P < 0.0004), respectively. None of the genotypes studied were more significantly associated to Group 1 than Group 2. Conclusion: Genotypes known to be associated with AMD were similarly observed in patients with and without RPD. RETINA 33: 998-1004, 2013

Purpose To investigate the influence of at least 12 hours of fasting followed by acute water ingestion on macular choroidal thickness in eyes of healthy subjects. Methods Prospective case series study including healthy volunteers from April 1, 2015 to May 31, 2015. Macular choroidal thickness was measured on enhanced depth imaging optical coherence tomography (EDI-OCT) and on swept-source (SS)-OCT after 12 hours of fasting, and 60 minutes after acute water ingestion (750 mL in 5 minutes). Results A total of 52 eyes of 26 healthy volunteers were included in this study. The mean age for participants was 32 years old. After 12 hours of fasting, the mean (±SD) subfoveal choroidal thickness was 335.43 (±104.82) μm on EDI-OCT and 286.16 (±80.32) μm on SS-OCT. The mean (±SD) subfoveal choroidal thickness after acute water intake was 352.32 (±120.36) μm and 290.5 (±85.86) μm, respectively. There was no significant difference between the mean subfoveal thickness at baseline and 60 minutes after acute water ingestion for any of the choroidal imaging techniques (p = 0.1386 for EDI-OCT and p = 0.0659 for SS-OCT). Conclusions These results suggest that choroidal circulation can physiologically quickly adapt to systemic vascular changes in healthy eyes. Choroidal thickness did not depend upon the hydration status for the subjects participating in this study.

Purpose To analyze specific outer retinal and retinal pigment epithelium (RPE) features in reticular pattern dystrophy using spectral-domain optical coherence tomography (SD-OCT). Methods Consecutive patients with reticular pattern dystrophy underwent a complete ophthalmologic examination, including assessment of best-corrected visual acuity (BCVA), fundus biomicroscopy, and SD-OCT (Spectralis SD-OCT, Heidelberg Engineering). Outer retinal and RPE macular features, as evaluated by SD-OCT (scan passing through the fovea) were analyzed by 3 authors (JZ, NM and GQ). Results 24 eyes of 13 patients (5 males, 8 women, mean age 71.4) were included. Mean visual acuity was measured at 0.30 (logMAR). The RPE layer in the foveal area appeared normal in 41.7% of scans, while small RPE elevations and RPE bumps were detected in 33.3% and 20.8% of scans, respectively. Inner segment/outer segment (IS/OS) junction appeared disrupted in 50% of scans, and absent in 58.3% of scans. SD-OCT showed a slight IS/OS elevation in 54.2% of scans. The external limiting membrane (ELM), appeared disrupted in 50% of scans, absent in 37.5% of scans, and elevated in 66.7% of scans. Hypereflective subretinal material accumulation or hyporeflective subretinal lesions, were detected in 33.3% and in 12.5% of scans, respectively. SD-OCT showed hyporeflective retinal pseudocysts in 16.7% of scans. Conclusion In this series of reticular pattern dystrophy, SD-OCT showed disruption of RPE layer and RPE bumps. Outer retinal changes included absence and disruption of both IS/OS junction and ELM. Hypereflective subretinal material accumulation or hyporeflective subretinal lesions, and hyporeflective retinal pseudocysts were also noticed.

Purpose: Optical coherence tomography angiography is a novel and noninvasive technique for imaging retinal microvasculature by detecting changes in reflectivity that is related to blood flow. The purpose of this study was to describe Type 2 neovascularization characteristics in age-related macular degeneration using optical coherence tomography angiography. Methods: Fourteen eyes of 14 consecutive patients with Type 2 neovascularization were prospectively included. All patients underwent a complete ophthalmological examination, including color and infrared fundus photography, fluorescein and indocyanine green angiography, spectral domain optical coherence tomography angiography, and optical coherence tomography angiography. Results: In all cases, Type 2 lesions could be detected by optical coherence tomography angiography, presenting as a hyperflow lesion in the outer retina, with a glomerulus (4/14) or medusa shape (10/14), surrounded by a dark halo. The superficial layer and the deep retina showed no abnormal flow. Surprisingly, the Type 2 lesions could also be observed in the presumed choriocapillaris layer. These glomerulus- or medusa-shaped lesions were connected, in 10/14 eyes, to a thicker main branch, which seemed to continue deep into the choroidal layers. Conclusion: Optical coherence tomography angiography may be a new imaging method for the diagnosis of Type 2 neovascularization in clinical routine. However, the specificity of the features needs to be investigated in further studies.