Spectral-Domain Optical Coherence Tomography Analysis of Fibrotic Lesions in Neovascular Age-Related Macular Degeneration.

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.

RETINOCHOROIDAL ANASTOMOSIS ASSOCIATED WITH ENHANCED S-CONE SYNDROME.

PURPOSE: To describe the phenotype and genotype of a 10-year-old boy affected with enhanced S-cone syndrome associated with neovascularization. METHODS: Fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, full-field electroretinogram and NR2E3 molecular testing were performed. RESULTS: 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 G>A (p.Arg311Gln), supporting an autosomal recessive inheritance. The patient received three intravitreal injections of anti-VEGF agents. CONCLUSION: 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.

Parafoveal OCT Angiography Features in Diabetic Patients without Clinical Diabetic Retinopathy: A Qualitative and Quantitative Analysis.

PURPOSE: To evaluate the capacity of OCT angiography (OCTA) for detecting infraclinical lesions in parafoveal capillaries in diabetic patients without diabetic retinopathy (DR). METHODS: This prospective observational cross-sectional case-control study analyzed the superficial and deep capillary plexuses (SCP and DCP) on macular OCTA scans (3 × 3 mm) centered on the fovea. We compared 22 diabetic patients (34 eyes included) without DR diagnosis on color fundus photographs, with 22 age- and gender-matched nondiabetic controls (40 eyes included). Qualitative analysis concerned morphological ischemic capillary alterations. Quantitative analysis measured foveal avascular zone (FAZ) size, parafoveal capillary density, and enlargement coefficient of FAZ between SCP and DCP. RESULTS: Neither the qualitative nor quantitative parameters were significantly different between both groups. No microaneurysms or venous tortuosity was observed in any of the analyzed images. On the SCP, the mean FAZ area was 0.322 ± 0.125 mm2 in diabetic patients and 0.285 ± 0.150 mm2 in controls, P = 0.31. On the DCP, the mean FAZ area was 0.444 ± 0.153 mm2 in cases and 0.398 ± 0.138 mm2 in controls, P = 0.20. CONCLUSION: OCTA did not detect infraclinical qualitative or quantitative differences in parafoveal capillaries of diabetic patients without DR in comparison with nondiabetic controls.

NEOVASCULARIZATION SECONDARY TO HIGH MYOPIA IMAGED BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To describe the optical coherence tomography angiography (OCTA) characteristics of active myopic choroidal neovascularization (CNV) and to compare its sensitivity versus fluorescein angiography and spectral-domain optical coherence tomography. METHODS: Consecutive highly myopic patients complicated with active myopic CNV were prospectively included. The OCTA features were analyzed and correlated with the findings of conventional imaging (spectral-domain optical coherence tomography and fluorescein angiography). RESULTS: Twenty eyes of 19 patients (mean age: 59.6 ± 12.1 years, mean spherical equivalent: -13.5 ± 3.6 diopters) presenting with both treatment-naive CNV and recurrent CNV were included in the analysis. The OCTA showed a 90% sensitivity for myopic CNV detection in 18 of 20 eyes, revealing a high-flow neovascular network accurately visible using a 30-µm manual segmentation underneath Bruch membrane. Mean selected area of myopic CNV on OCTA images was 0.34 ± 0.45 mm, whereas the mean vessel area was 0.22 ± 0.27 mm. Two neovascular phenotypes prevailed in our series: disorganized vascular loops and organized interlacing patterns. CONCLUSION: The OCTA seems to be a valuable tool in detecting myopic CNV with a high sensitivity. However, its specificity needs to be investigated in further studies.

Prevalence and quantification of geographic atrophy associated with newly diagnosed and treatment-naïve exudative age-related macular degeneration.

OBJECTIVE: To identify and quantify geographic atrophy (GA) associated with neovascular age-related macular degeneration (AMD) at initial presentation using a fundus autofluorescence (FAF) semi-automated software and to correlate the results with demographic and clinical data. DESIGN: Retrospective, observational study. METHODS: The study population consisted of treatment-naïve patients with newly diagnosed neovascular AMD. Best-corrected visual acuity, fundus photographs, infrared reflectance, FAF and spectral-domain optical coherence tomography were performed, associated with fluorescein and indocyanine green angiographies. Identification of GA was independently performed by three readers. Quantification of atrophy areas was done using RegionFinder Software (RFA), a semi-automated software embedded in Spectralis device (Heidelberg Engineering, Germany). RESULTS: We included 206 eyes of 173 consecutive patients (72% female, mean age: 79.7±9.1 years). Type I choroidal neovascularisation (CNV) was observed in 44.2% of eyes, type II CNV was observed in 20.9% and mixed CNV lesion was observed in 11.7%. Polypoidal choroidal vasculopathy was diagnosed in 7.7% and type III CNV was diagnosed in 15.5%. Analysis of FAF frames showed that GA was associated with nAMD in 46/206 eyes (22.3%). Taking into account data both from Region Finder and multimodal imaging, our results suggest that GA was present in 24.3% of eyes newly diagnosed with exudative AMD. Mean size of GA was 1.23±1.76 mm2 (range 0.03-7.39). CONCLUSION: GA is associated with nAMD in 1/4 of cases at initial presentation. Combined imaging, including RFA is an effective tool to identify and quantify GA at diagnosis.

Progression of Macular Atrophy in Pattern Dystrophies.

BACKGROUND AND OBJECTIVE: To quantify the progression of macular atrophy associated with pattern dystrophies (PD). PATIENTS AND METHODS: Retrospective, observational study including patients with reticular PD and macular atrophy. A detailed ophthalmologic exam was performed, and progression of macular atrophy areas was evaluated on fundus autofluorescence frames using RegionFinder software, a semiautomated software embedded in Spectralis device (Heidelberg Engineering, Heidelberg, Germany). RESULTS: We included 19 eyes of 12 patients. The median follow-up was 4.5 years (interquartile range [IQR]: 2.7-5.5). Three eyes (16%) had choroidal neovascularization. Atrophy involved foveal area in 21% (four of 19) of cases. Decreased vision occurred in three eyes (16%). The median atrophy progression rate evaluated by RegionFinder software was 0.101 mm(2)/year (IQR: 0.054-0.257). CONCLUSION: The progression of macular atrophy in PDs appears to be relatively slow. Further studies are necessary to correlate the progression of atrophy in PDs with genetic data. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:652-658.].

Optical coherence tomography angiography characteristics of polypoidal choroidal vasculopathy.

PURPOSE: To analyse the morphological characteristics of polypoidal choroidal vasculopathy (PCV) on optical coherence tomography angiography (OCT-A). METHODS: Prospective study with consecutive patients affected with PCV were included. All patients underwent a complete ophthalmological examination including fundus photography, fluorescein angiography, indocyanine green angiography, spectral-domain OCT and OCT-A. RESULTS: Twelve eyes of 12 patients (mean age 72.6±10.5 years; 4 men and 8 women) were included for analysis. In all eyes (12/12) the segmentation of the choriocapillaris layer on OCT-A revealed the branching vascular network (BVN) as a hyperflow lesion. OCT-A segmentation of the choriocapillaris layer in correspondence of the polypoidal lesion showed in 3/12 eyes (25%) a hyperflow round structure, surrounded by a hypointense halo, and in 9/12 eyes (75%) a hypoflow round structure. CONCLUSIONS: The OCT-A is a non-invasive imaging modality allowing the visualisation of different structures in PCV. The BVN is constantly clearly detected. The hypoflow round structure appearance of the polyp in OCT-A, is probably due to an unusual blood flow inside the polypoidal lesions, contrasting with the BVN. Further improvement in OCT-A knowledge will provide information on the specificity of the different intensity characteristics in PCV.

Unusual retinopathy associated with hemochromatosis.

PURPOSE: To describe unusual retinal findings of a patient affected by hemochromatosis. METHODS: Case report of a 49-year-old patient who presented a progressive loss of vision. Fundus photography, fluorescein angiography, full-field electroretinogram, autofluorescence imaging, and spectral domain optical coherence tomography were performed. The patient was known to be homozygous for the C282Y mutation in the HFE gene. RESULTS: Visual acuity was measured at 20/20 on his right eye and 20/25 on his left eye. Retinal imaging showed alterations of the retinal pigment epithelium clearly visible on fundus autofluorescence and fluorescein angiography. The spectral domain optical coherence tomography showed retinal pigmentary epithelial atrophy associated with irregularities and focal interruption of the ellipsoid zone. A thin retina was also observed in the foveolar region associated to a thickened choroid. Full-field electroretinogram showed a decrease of rods and cones responses. CONCLUSION: Here, the authors describe the retinal findings of a patient affected by hemochromatosis, characterized by unusual retinal pigment epithelium changes associated to altered visual function. The authors hypothesize that the retinopathy could be linked to hemochromatosis because of the pathophysiology of iron homeostasis and the toxicity of iron overload for the photoreceptors.