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Purpose: To correlate structural changes of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with patient age., Design: Retrospective study., Participants: Fifty eyes of 49 patients (age range, 1-74 years) with CHRRPE studied at 9 tertiary vitreoretinal institutions., Methods: We analyzed the clinical findings with respect to lesion topography and pigmentation as well as investigated the OCT findings regarding the thickness, vitreoretinal interface, outer plexiform layer distortion, ellipsoid zone disruption, and retinal pigment epithelium-Bruch's membrane complex involvement of CHRRPE., Main Outcome Measures: Clinical and imaging findings of CHRRPE at different ages., Results: Analysis of 50 CHRRPE patients revealed that younger patients were more likely to demonstrate partial thickness involvement of the retina (P = 0.009) with predominantly inner retinal layer involvement (P = 0.04). The inverse was true for older patients with CHRRPE. In addition, older patients more commonly showed pigmentary changes. Eyes with CHRRPE were more likely to show an increase in central macular thickness independently of tumor location., Conclusions: Based on these findings, we believe that CHRRPE typically begins in the inner retina and continues toward the outer retina over time, with increase in central macular thickness, despite the location of the tumor., (Copyright © 2021 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: By optical coherence tomography (OCT) imaging, hyperreflective foci (HRF) indicate progression risk for advanced age-related macular degeneration (AMD) and are in part attributable to ectopic retinal pigment epithelium (RPE). We hypothesized that ectopic RPE are molecularly distinct from in-layer cells and that their cross-retinal course follows Müller glia., Methods: In clinical OCT (61 eyes, 44 patients with AMD, 79.4 ± 7.7 years; 29 female; follow-up = 4.7 ± 0.9 years), one HRF type, RPE plume (n = 129 in 4 morphologies), was reviewed. Twenty eyes of 20 donors characterized by ex vivo OCT were analyzed by histology (normal, 4; early/intermediate AMD, 7; geographic atrophy, 6; neovascular AMD, 3). Cryosections were stained with antibodies to retinoid (RPE65, CRALPB) and immune (CD68, CD163) markers. In published RPE cellular phenotypes, red immunoreactivity was assessed semiquantitatively by one observer (none, some cells, all cells)., Results: Plume morphology evolved over time and many resolved (40%). Trajectories of RPE plume and cellular debris paralleled Müller glia, including near atrophy borders. RPE corresponding to HRF lost immunoreactivity for retinoid markers and gained immunoreactivity for immune markers. Aberrant immunoreactivity appeared in individual in-layer RPE cells and extended to all abnormal phenotypes. Müller glia remained CRALBP positive. Plume cells approached and contacted retinal capillaries., Conclusions: HRF are indicators not predictors of overall disease activity. Gain and loss of function starts with individual in-layer RPE cells and extends to all abnormal phenotypes. Evidence for RPE transdifferentiation, possibly due to ischemia, supports a proposed process of epithelial-mesenchyme transition. Data can propel new biomarkers and therapeutic strategies for AMD.

Purpose: To longitudinally characterize structural/angiographic findings of retinal-choroidal anastomosis (RCA) in macular telangiectasia Type 2 (MacTel2)., Methods: A combined retrospective/prospective review of patients with MacTel2 with >2 visits and >2 months follow-up, including examination, fundus photography, fluorescein angiography, spectral-domain optical coherence tomography, and projection-resolved optical coherence tomography angiography., Results: There were 24 eyes of 12 patients with a mean age of 61.1 (±7.3 [SD]) years and median follow-up of 447 (range 81-1,511) days. There were five eyes diagnosed with new RCA after not having any previously. Retinal-choroidal anastomosis was diagnosed in 8 (33%) eyes initially and eventually 13 (54%). In the eight eyes initially diagnosed with RCA, the number of RCAs and outer retinal hyperrflective lesions increased over the follow-up. The RCAs were found in clusters, generally temporal to the fovea. Retinal-choroidal anastomosis was uniformly associated with complete retinal subsidence, defined as the loss of outer nuclear layer substance such that the middle retinal layers sank down toward the retinal pigment epithelium (RPE), and was also present in each case. Each RCA colocalized with a region of hyperreflectivity, previously termed an outer retinal hyperreflective lesion. There was a lack of fluorescein leakage, lipid, hemorrhage, or any structural correlates with fluid exudation, and no signs of subretinal/subretinal pigment epithelium neovascularization., Conclusion: De novo RCA development seems to be associated with retinal subsidence, and after RCAs form, they increase in number and topographic distribution over time. Initially after formation, there was no sign of exudation. These data suggest the descent of the deep capillary plexus occurs with RCA, devoid of subretinal/subretinal pigment epithelium neovascularization in MacTel2.

Purpose: To describe the presence of subretinal lipid globules (SLG), analyze the multimodal imaging features inherent in their optical properties, and provide a means to distinguish them from other retinal structures and clinical signs., Design: Retrospective cohort study., Methods: The clinical data and multimodal imaging features of 39 patients (49 eyes) showing SLG were evaluated. Patients underwent color fundus photography, near-infrared reflectance (NIR), spectral-domain (SD) and swept-source (SS) optical coherence tomography (OCT) and OCT angiography. In vitro phantom models were used to model OCT optical properties of water, mineral oil, and intralipid droplets and to investigate the optical mechanisms producing hypertransmission tails beneath SLG., Results: The SLG were not visible in color fundus photographs or in NIR images. With both SD- and SS-OCT B-scans, SLG appeared as 31-157 μm, round, hyporeflective structures demonstrating a characteristic hypertransmission tail previously described with lipid globules found in the choroid and in neovascular membranes. Similarly, with en face OCT, SLG appeared as small, round, hyporeflective structures. SLG were encountered most often in eyes with neovascular age-related macular degeneration (AMD) that had type 1 macular neovascularization (MNV) (91.1%). Of those eyes, 93.3% were receiving intravitreal antivascular endothelial growth factor (VEGF) therapy (median of 15 injections) with a mean follow-up of 52.6 months. The number of prior injections positively correlated with the number of SLG. The detection of MNV preceded the presence of SLG in 66.7% of cases. En face OCT showed that, in many eyes (49%), SLG appeared in clusters of >10. In 38.8% of eyes, SLG were found overlying type 1 MNV, and in 44.9% of eyes, often those with more numerous SLG, the SLG were located near the lesion border. In 2 eyes with AMD followed for nonexudative type 1 MNV, SLG were detected prior to the detection of other imaging signs of exudation. SLG were observed in several other exudative macular diseases. Phantom models demonstrated that the hypertransmission tail beneath SLG is related to a lensing effect produced by these hyporeflective spherical structures., Conclusions: SLG are a newly recognized OCT feature frequently seen in eyes receiving intravitreal anti-VEGF therapy for type 1 MNV due to AMD. OCT B-scans show SLG as small, round, hyporeflective structures with a characteristic hypertransmission tail. This OCT signature is influenced by the OCT focal plane, and it relates to reduced signal attenuation through oil and a lensing effect created by a higher refractive index compared to surrounding tissue., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To present multimodal imaging of multifocal chorioretinitis secondary to endogenous candida infection in a young adult., Methods: A 49-year-old woman who presented for evaluation of bilateral endogenous candida chorioretinitis underwent complete ophthalmic examination, in addition to fundus photography (FP), enhanced depth imaging optical coherence tomography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA)., Results: Multimodal imaging of both eyes of the patient affected by endogenous candida chorioretinitis was performed. FP showed multiple white chorioretinal lesions at the posterior pole, FAF showed dark dot at the posterior pole surrounded by hyperautofluorescence area, FA showed early hyperfluorescence round perifoveal lesion at the posterior pole and small hyperfluorescence dots under the inferior retinal vessels. Early ICGA showed hypofluorescence dots at the posterior pole. Late ICGA showed dark hypofluorescence dots at the posterior pole surrounded by faint hyperautofluorescent ring. OCTA showed dark areas corresponded to hypoperfusion areas seen with early ICGA., Conclusion: We reported multimodal imaging of an unusual occurrence of multifocal chorioretinitis due to immunosuppression. These findings suggested that the infection resulted from choroidal infiltration via the short posterior ciliary arteries with resultant breakthrough into the retina, rather than via the central retinal artery. By comparing findings on OCTA with data obtained from traditional systems, we are gaining essential information on the pathogenesis of endogenous candida chorioretinitis.

Background and Objective: To use quantitative fundus autofluorescence (qAF) to analyze different stages of non-neovascular age-related macular degeneration (AMD)., Patients and Methods: In this cohort study, 38 pseudophakic patients and 36 age-matched controls participated. We performed near-infrared, spectral-domain optical coherence tomography and qAF imaging on 31 pseudophakic eyes and controls of participants older than 60 years with non-neovascular AMD phenotypes using the Spectralis HRA + OCT (Heidelberg Engineering, Heidelberg, Germany)., Results: The patients included in this study had a mean age of 83.9 years, and 35.7% patients were men. Mean qAF was higher in control participants than in all patients with AMD (P < .001). According to non-neovascular AMD phenotype, mean qAF levels were significantly lower in eyes with subretinal drusenoid deposits than in control eyes (P < .05). The lowest mean qAF was in patients with geographic atrophy., Conclusion: Quantitative fundus autofluorescence of non-neovascular AMD decreases from normal to early to late AMD, suggesting that loss of lipofuscin fluorophores, not increase, signifies AMD progression. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:S34-S42.]., (Copyright 2018, SLACK Incorporated.)

Purpose: To investigate the utility of optical coherence tomography angiography (OCTA) for detecting pathologic vascularization within pigment epithelial detachments (PEDs)., Methods: This was a retrospective, cross-sectional, consecutive case series. Multimodal imaging (structural OCT, fluorescein, and indocyanine green angiography) was used as the gold standard to classify PEDs as nonvascularized or vascularized. Optical coherence tomography angiography imaging of the PED was subsequently and independently evaluated to classify PEDs as vascularized or nonvascularized. Specifically, OCTA images were evaluated for the presence of abnormal flow on cross-sectional OCTA and the presence of a vascular complex on en face OCTA. Comparisons between OCTA and the gold standard were determined., Results: Sixty-four eyes of 49 patients were evaluated. A total of 18 eyes were classified as nonvascularized PED, and 46 eyes were classified as vascularized PED using the gold standard. Optical coherence tomography angiography was found to have a sensitivity of 76%, specificity of 61%, positive predictive value of 83%, and negative predictive value of 50% for detecting vascularized PEDs. False positive cases in the nonvascularized PED group were due to projection or flow artifacts from hyperreflective material overlying the PED. False negative cases were seen in eyes with minimal exudation on structural OCT and also those manifesting retinal pigment epithelial tears., Conclusion: Our proposed two-step approach of OCTA interpretation, first using cross-sectional OCTA and then en face OCTA, may allow the detection of vascularization within PEDs and, in some cases, reduce the need for conventional angiography. Increased awareness about potential artifacts and limitations of OCTA may help clinicians interpret OCTA more accurately.

Purpose: To analyze the changes in visual acuity and subfoveal choroidal thickness in patients with non-neovascular age-related macular degeneration (AMD) and drusenoid pigment epithelium detachments (PED)., Design: Consecutive observational case series., Methods: Observational retrospective review of eyes diagnosed with drusenoid PED in a single clinical setting. Demographic and clinical data included age, sex, laterality, best-corrected visual acuity (BCVA), and subfoveal choroidal thickness measured at baseline. before and after the collapse of the PED, and at the last available follow-up. The presence of geographic atrophy (GA) was also assessed., Results: Thirty-seven eyes of 25 patients (18 female) were included in the analysis. Mean age at baseline was 71 ± 8.4 years. During a mean follow-up period of 4.9 ±1.9 years, PED collapse was observed in 25 eyes (68%). Mean BCVA, mean maximum PED height, and mean subfoveal choroidal thickness significantly decreased from baseline to the last available follow-up (P < .001) in patients showing PED collapse. Choroidal thinning was faster during the PED collapse (speed rate of 35.9 μm/year). From those, 23 eyes (92%) developed GA. A significant correlation between the area of GA and the decrease in choroidal thickness was found (P = .010)., Conclusions: Choroidal thickness significantly decreased in eyes showing drusenoid PED collapse, but not in eyes in which the PED persisted. A significant correlation with resultant GA area following PED collapse and the magnitude of choroidal thinning was found. Further studies are warranted to better understand the mechanisms involved in the occurrence of choroidal changes during the life cycle of drusenoid PEDs., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The term aneurysmal type 1 neovascularization is derived from terminology, which is established in the literature but has fallen out of use. We believe that aneurysmal type 1 neovascularization accurately describes the lesions which define the entity known as polypoidal choroidal vasculopathy (PCV). Over the last three decades, the clinical spectrum of PCV has expanded to recognize the occurrence of the aneurysmal (polypoidal) lesions in different contexts, resulting in a complex and unwieldy taxonomy based sometimes on circumstantial findings rather than mechanistic considerations. Advances in multimodal imaging provides increasingly convincing evidence that the lesions which define various forms of PCV are indeed vascular and arise from type 1 neovascular networks. The understanding of PCV as type 1 neovascularization with aneurysms renews focus on the question as to why some patients with type 1 neovascularization develop aneurysms while others do not. Conceptual themes and potential for further study are discussed., (© 2017 The Authors Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To examine the alterations in retinal vascular morphology over an extended follow-up in eyes with macular telangiectasis Type 2 (MacTel2)., Methods: Eyes with high-quality digital photographs were evaluated. The geometric distortion in baseline images required to emulate the follow-up images was determined and vectors were made that represented the direction and magnitude of changes, to create a warp field. Optical coherence tomography and optical coherence tomography angiography evaluation of the retina was performed., Results: There were 7 eyes of 4 patients, who had a mean age of 70.25 years, which were followed for a mean of 8.8 years. The eyes showed increasing grayish opacification in the temporal macula with straightening and displacement of the macular vessels, even those in the nasal macula. The warp field vectors pointed to the temporal juxtafoveal macula. There was never any cavitation at the epicenter of the retinal distortion in any patient, although cavitations were found around this area. Optical coherence tomography imaging showed a circumscribed region of hyperreflectivity in the temporal macula. Optical coherence tomography angiography showed a deep angular condensed network of vessels within the hyperreflective region. One eye showed marked atrophic changes including full-thickness macular hole formation, but no increase in graying of the retina, loss of retinal laminations, pigmentary infiltration, or alteration in the retinal vessels., Conclusion: Tissue contraction with retinal vascular displacement and contortion seem to be integral aspects of disease manifestation in MacTel2. The induced vascular changes may lead to secondary effects that increase morbidity in this disease.

Purpose: To define the range and life cycles of cuticular drusen phenotypes using multimodal imaging and to review the histologic characteristics of cuticular drusen., Design: Retrospective, observational cohort study and experimental laboratory study., Participants: Two hundred forty eyes of 120 clinic patients with a cuticular drusen phenotype and 4 human donor eyes with cuticular drusen (n = 2), soft drusen (n = 1), and hard drusen (n = 1)., Methods: We performed a retrospective review of clinical and multimodal imaging data of patients with a cuticular drusen phenotype. Patients had undergone imaging with various combinations of color photography, fluorescein angiography, indocyanine green angiography, near-infrared reflectance, fundus autofluorescence, high-resolution OCT, and ultrawide-field imaging. Human donor eyes underwent processing for high-resolution light and electron microscopy., Main Outcome Measures: Appearance of cuticular drusen in multimodal imaging and the topography of a cuticular drusen distribution; age-dependent variations in cuticular drusen phenotypes, including the occurrence of retinal pigment epithelium (RPE) abnormalities, choroidal neovascularization, acquired vitelliform lesions (AVLs), and geographic atrophy (GA); and ultrastructural and staining characteristics of druse subtypes., Results: The mean age of patients at the first visit was 57.9±13.4 years. Drusen and RPE changes were seen in the peripheral retina, anterior to the vortex veins, in 21.8% of eyes. Of eyes with more than 5 years of follow-up, cuticular drusen disappeared from view in 58.3% of eyes, drusen coalescence was seen in 70.8% of eyes, and new RPE pigmentary changes developed in 56.2% of eyes. Retinal pigment epithelium abnormalities, AVLs, neovascularization, and GA occurred at a frequency of 47.5%, 24.2%, 12.5%, and 25%, respectively, and were significantly more common in patients older than 60 years of age (all P < 0.015). Occurrence of GA and neovascularization were important determinants of final visual acuity in eyes with the cuticular drusen phenotype (both P < 0.015). Small cuticular drusen typically demonstrated a homogenous ultrastructural appearance similar to hard drusen, whereas fragmentation of the central and basal contents was seen frequently in larger cuticular drusen., Conclusions: Although the ultrastructural characteristics of cuticular drusen appear more similar to those of hard drusen, their lifecycle and macular complications are more comparable with those of soft drusen. Cuticular drusen phenotype may confer a unique risk for the development of GA and neovascularization., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM)., Design: Retrospective, observational, multicenter case series review., Participants: Consecutive patients diagnosed with idiopathic AEPVM., Methods: Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature., Main Outcome Measures: Clinical features of idiopathic AEPVM and differential diagnosis., Results: Eighteen patients (age range, 21-74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations., Conclusions: Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe atypical cases of multiple evanescent white dot syndrome (MEWDS) associated with foveal exudation, increased choroidal thickness, and secondary Type 2 (subretinal) neovascularization., Methods: Four cases of atypical MEWDS were studied at a retina referral center. Patients underwent evaluation with multimodal retinal imaging, including fluorescein angiography, indocyanine green angiography, spectral-domain and enhanced depth imaging optical coherence tomography (OCT). Two patients were imaged with OCT angiography., Results: Four patients (3 female, 1 male) with a median age of 23.5 years presented with acute onset, painless, decreased central vision. All cases demonstrated fundus findings consistent with MEWDS on color photography, indocyanine green angiography, fluorescein angiography, fundus autofluorescence, and structural OCT imaging. On structural OCT, all 4 patients were noted to have hyperreflective subretinal material and increased subfoveal choroidal thickness ranging from 307 μm to 515 μm. Type 2 neovascularization was diagnosed in all four patients using fluorescein angiography, indocyanine green angiography, and/or OCT angiography. Two patients had poor visual acuity at the last follow-up despite resolution of characteristic clinical findings of MEWDS., Conclusion: A subset of patients with atypical MEWDS may develop persistent poor vision due to subfoveal exudation and secondary Type 2 neovascularization. Patients showing increased choroidal thickness at presentation may be more susceptible to this unusual presentation.

Purpose: To evaluate the spectrum of macular chorioretinal lesions occurring in idiopathic multifocal choroiditis using optical coherence tomography angiography (OCTA) to evaluate those showing neovascular flow., Methods: This was a descriptive, retrospective study of 18 eyes of 14 patients with multifocal choroiditis. Macular lesions were characterized as subretinal pigment epithelium, subretinal, or mixed and evaluated during active and presumed inactive states of multifocal choroiditis. Correlations between structural optical coherence tomography and OCTA were performed. In select cases, correlations between OCTA, fluorescein angiography, and fundus autofluorescence were evaluated. In 5 eyes, quantitative measurements of neovascular lesions were compared at baseline and following intravitreal anti-vascular endothelial growth factor therapy., Results: Mean patient age was 48 years (SD: 13.8; 86% women). Optical coherence tomography angiography flow signatures consistent with neovascularization were identified in 83% of eyes, including in 0% of subretinal pigment epithelium, 91% of subretinal, and 100% of mixed lesions. Lesions that did not demonstrate definitive signs of fluorescein angiography leakage were frequently found to have neovascularization using OCTA. There was no change in quantitative measurements of neovascular lesions after anti-vascular endothelial growth factor therapy (all tested variables P > 0.05)., Conclusion: Optical coherence tomography angiography may be a useful imaging modality for understanding the pathophysiology of multifocal choroiditis and monitoring its clinical course.

Purpose: To evaluate multimodal imaging including volume-rendered angiographic and structural optical coherence tomography of macular telangiectasia Type 2 (MacTel2) for right-angle vein complexes, macular cavitations, and signs of deeper retinal vascular invasion., Methods: Retrospective review of imaging performed in a community-based retinal referral center. The eyes were scanned using optical coherence tomography using split-spectrum amplitude-decorrelation techniques to derive flow information. These data were extracted and used to create volume-rendered images of the retinal vasculature with integrated structural information derived from the component optical coherence tomographic images., Results: There were 24 eyes of 16 patients who had a mean age of 61.8 years. Right-angle veins seemed in association with vascular proliferation external to the deep vascular plexus. The origin of a right-angle vein was surrounded by a stellate arrangement of radiating retinal vessels apparently caused by contraction of surrounding tissue in the temporal macula. Cavitations were found in the fovea and varied in size and configuration from one examination to the next. Many smaller cavitations, called microcavitations, were seen in the surrounding macula. Vascular invasion occurred into the subretinal space., Conclusion: There are contractile features of the tissue in the temporal macula and the number, size, and temporal variations in the cavitations have not been in not mentioned in previous published descriptions of MacTel2. Vascular invasion of deeper layers occurred in the temporal macula through the outer nuclear layer. Volume-rendered angiographic and structural optical coherence tomography offers unprecedented ability to examine the vascular interrelationships their associations with cavitations in the macula.

Purpose: To study the cross-sectional and en face optical coherence tomography angiography (OCTA) findings in Type 3 neovascularization (NV)., Methods: Optical coherence tomography angiography imaging of 27 eyes of 23 patients with Type 3 NV was analyzed with 9 eyes having consecutive follow-up OCTA studies., Results: Type 3 NV appeared as a linear high-flow structure on cross-sectional OCTA corresponding to a high-flow tuft of vessels seen on en face OCTA. Cross-sectional OCTA seemed to enable the distinction between vascular and nonvascular intraretinal hyperreflective foci. Two patterns of flow were observed; Pattern 1 (11%): a flow signal confined to the neurosensory retina and Pattern 2 (74%): a flow signal extending through the retinal pigment epithelium. No definitive retinal-choroidal anastomosis was observed; however, projection artifacts confounded the interpretation of deeper structures. An increase in the intensity of the high-flow tuft was seen during the progression or recurrence of Type 3 NV. Intravitreal anti-vascular endothelial growth factor therapy caused a reduction in the intensity of the high-flow tuft which was not sustained., Conclusion: Compared with conventional imaging, OCTA may improve detection and delineation of vascular changes occurring in Type 3 NV. Cross-sectional and en face OCTA may prove useful in studying the pathogenesis and guiding the management of these lesions.

Purpose: To determine if the area of the foveal avascular zone (FAZ) is correlated with visual acuity (VA) in diabetic retinopathy (DR) and retinal vein occlusion (RVO)., Design: Cross-sectional study., Participants: Ninety-five eyes of 66 subjects with DR (65 eyes), branch retinal vein occlusion (19 eyes), and central retinal vein occlusion (11 eyes)., Methods: Structural optical coherence tomography (OCT; Spectralis, Heidelberg Engineering) and OCT angiography (OCTA; Avanti, Optovue RTVue XR) data from a single visit were analyzed. FAZ area, point thickness of central fovea, central 1-mm subfield thickness, the occurrence of intraretinal cysts, ellipsoid zone disruption, and disorganization of retinal inner layers (DRIL) length were measured. VA was also recorded. Correlations between FAZ area and VA were explored using regression models. Main outcome measure was VA., Results: Mean age was 62.9±13.2 years. There was no difference in demographic and OCT-derived anatomic measurements between branch retinal vein occlusion and central retinal vein occlusion groups (all P ≥ 0.058); therefore, data from the 2 groups were pooled together to a single RVO group for further statistical comparisons. Univariate and multiple regression analysis showed that the area of the FAZ was significantly correlated with VA in DR and RVO (all P ≤ 0.003). The relationship between FAZ area and VA varied with age (P = 0.026) such that for a constant FAZ area, an increase in patient age was associated with poorer vision (rise in logarithm of the minimum angle of resolution visual acuity). Disruption of the ellipsoid zone was significantly correlated with VA in univariate and multiple regression analysis (both P < 0.001). Occurrence of intraretinal cysts, DRIL length, and lens status were significantly correlated with VA in the univariate regression analysis (P ≤ 0.018) but not the multiple regression analysis (P ≥ 0.210). Remaining variables evaluated in this study were not predictive of VA (all P ≥ 0.225)., Conclusions: The area of the FAZ is significantly correlated with VA in DR and RVO and this relationship is modulated by patient age. Further study about FAZ area and VA correlations during the natural course of retinal vascular diseases and following treatment is warranted., (Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: The range of subretinal hyperreflective material (SHRM) seen in macular disease includes type 2 macular neovascularization, fibrosis, exudation, vitelliform material, and hemorrhage. The prognostic significance of SHRM has been evaluated retrospectively in clinical trials, but discriminating SHRM subtypes traditionally requires multiple imaging modalities. The purpose of this study is to describe optical coherence tomography angiography (OCTA) flow characteristics and artifacts that might help to distinguish SHRM subtypes., Design: Validity analysis., Methods: Patients with age-related macular degeneration (AMD), myopia, pachychoroid disease, and macular dystrophy, manifesting SHRM on optical coherence tomography (OCT), were recruited. Clinical chart review and multimodal imaging established the SHRM subtype. All patients underwent OCTA. OCT and OCTA images were examined together for (1) intrinsic flow, (2) retinal projection onto the anterior SHRM surface (strong, weak, absent), (3) retinal projection through SHRM onto retinal pigment epithelium (RPE), and (4) masking of choriocapillaris flow., Results: Thirty-three eyes of 25 patients were included (type 2 neovascularization ×3; fibrosis ×4; exudation ×10; hemorrhage ×5; vitelliform ×17). Mean age per eye was 76 years (standard deviation: 12). Intrinsic flow was strongest in type 2 neovascularization. Subretinal fibrosis showed limited flow in residual large-caliber vessels and branches. Flow was not detected within foci of exudation, hemorrhage, or vitelliform lesions. Retina-SHRM surface projection was strongest onto smooth-surfaced SHRM and weaker onto exudation. Retinal projection was weakest on the surface of vitelliform lesions. Retina-RPE projection was masked by dense hemorrhage and vitelliform material. In compound SHRM, OCTA distinguished between vascular and avascular components., Conclusion: Optical coherence tomography angiography can distinguish vascular from avascular SHRM components. OCTA artifacts may distinguish certain avascular SHRM components., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the vascular structure of eyes with macular telangiectasia type 2 (MacTel2) using volume-rendered optical coherence tomography angiography (OCTA)., Design: Retrospective cross-sectional study., Participants: A total of 14 consecutive patients (20 eyes) with MacTel2 who had a signal strength score ≥55 and could maintain fixation during the scan process., Methods: The eyes were scanned using optical coherence tomography with split-spectrum amplitude decorrelation techniques to derive flow information. Data were extracted and used to create volume-rendered images of the retinal vasculature that could be rotated about 3 different axes for evaluation., Main Outcome Measures: Descriptive appraisal of the vascular abnormalities associated with MacTel2., Results: Vessels posterior to the outer boundary of the deep retinal plexus were secondary to retinal thinning, vascular invasion, or a combination of both. These vessels had the same shape and distribution as the late staining seen during conventional fluorescein angiography. Lateral contraction in the temporal macula in 5 eyes created an appearance of vessels radiating from a central locus, which was the site of a right angle vein. Loss of macular tissue as part of the disease process led to a central amalgamation of the inner vascular plexus and the deep vascular plexus, which appeared to be in a state of decline. Subretinal neovascularization originated from the retinal circulation but involved not only the subretinal space but also could infiltrate the remaining, thinned, retina., Conclusions: Volume rendering of OCTA information preserves the 3-dimensional relationships among retinal vascular layers and provides opportunities to visualize retinal vascular abnormalities in unprecedented detail. The retinal vascular leakage and invasion in MacTel2 may arise as a consequence of loss of control with depletion of Müller cells and exposure of the remaining retinal vessels to the more hypoxic environment near the inner segments of the photoreceptors., (Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To use volume-rendered optical coherence tomography angiography to investigate vascular proliferation in macular telangiectasia type 2 (MacTel2), extending beyond the retinal pigment epithelium (RPE)., Methods: Six eyes of four patients with MacTel2 with neovascularization proliferating external to the RPE confines were studied. Eyes were scanned using optical coherence tomography using split-spectrum amplitude-decorrelation techniques to derive flow information (RTVue XR; Optovue). These data were extracted and used to create volume rendered images of the area of vascular proliferation., Results: Mean age was 66.2 years. There was demonstrable vascular proliferation in the sub-RPE space observable by both optical coherence tomography and optical coherence tomography angiography. Fibrovascular RPE detachments were identified in all eyes. The topographic distribution of abnormal vessels located below the plane of the deep retinal vascular plexus and above the RPE closely matched the pattern of hyperfluorescence and leakage on fluorescein angiography. Vessels under the RPE demonstrated different branching patterns and larger diameter lumens than those above the RPE, but anastomosis with the choroidal circulation was difficult to demonstrate., Conclusion: This study provides evidence that sub-RPE vascular proliferation may be a complication of MacTel2. Retinal pigment epithelium abnormalities are known to occur in MacTel2 and may provide a conduit for abnormal vessels in the subretinal space to proliferate into the sub-RPE compartment. The authors have no reason to exclude the possibility that the choroid contributes to the deep proliferation.

Purpose: To describe multimodal imaging findings in a patient with foveal red spot syndrome., Methods: We report a case of a 57-year-old man with foveal red spot syndrome. Multimodal imaging techniques, including fundus color and red-free photographs, fluorescence angiography (Topcon 50DX; Topcon, Tokyo, Japan), MultiColor scanning laser imaging, spectral domain optical coherence tomography (Spectralis; Heidelberg Engineering, Heidelberg, Germany), swept source optical coherence tomography (DRI OCT-1 Atlantis; Topcon, Tokyo, Japan), adaptive optics (RTX-1; Imagine Eyes, Orsay, France), and microperimetry (MP1 Microperimeter; Nidek Technologies, Padua, Italy), were performed to confirm the diagnosis and determine the anatomical abnormalities related to the disease., Results: Conventional clinical examination and imaging studies failed to explain the patient's visual dysfunction and this specific macular abnormality. Using advanced ophthalmic technologies, including MultiColor imaging, spectral domain optical coherence tomography with high-density acquisition, swept source optical coherence tomography, adaptive optics, and microperimetry, we identified the nature of the macular manifestation accounting for pathology of the foveal red spot syndrome and his visual dysfunction., Conclusion: When conventional clinical examination and imaging techniques fail to identify the presence of and visual symptoms in foveal red spot syndrome, advanced technologies may be used to confirm the diagnosis and explain the etiology of the abnormality.

The authors describe fundus autofluorescence (AF) and spectral-domain optical coherence tomography (SD-OCT) findings in three patients with enhanced S-cone syndrome and their correlation around the hyperautofluorescent ring border. Patients had AF imaging in combination with SD-OCT line-scans through the fovea, at the posterior pole, and at a temporal locus centered on the ring border. All eyes demonstrated a macular ring of high-intensity AF. The inner segment ellipsoid band showed thinning and disorganization toward the ring border, where it was lost., (Copyright 2014, SLACK Incorporated.)

Purpose: To demonstrate the utility and characteristics of fundus autofluorescence in late-onset retinitis pigmentosa., Methods: Observational case series. Patients diagnosed with late-onset retinitis pigmentosa were identified retrospectively in an institutional setting. Twelve eyes of six patients were identified and medical records were reviewed., Results: All patients presented with slowly progressive peripheral field loss and initial clinical examination revealed only subtle retinal changes. There was a notable lack of intraretinal pigment migration in all patients. Five out of six patients underwent magnetic resonance imaging of the brain to rule out intracranial processes and all were referred from another ophthalmologist for further evaluation. Fundus autofluorescence was ultimately employed in all patients and revealed more extensive retinal pathology than initially appreciated on clinical examination. Fundus autofluorescence directed the workup toward a retinal etiology in all cases and led to the eventual diagnosis of late-onset retinitis pigmentosa through electroretinogram testing., Conclusion: Fundus autofluorescence may be a more sensitive marker for retinal pathology than stereo fundus biomicroscopy alone in late-onset retinitis pigmentosa. Early use of fundus autofluorescence imaging in the evaluation of patients with subtle retinal lesions and complaints of peripheral field loss may be an effective strategy for timely and cost-efficient diagnosis.

Background: Most patients with central serous chorioretinopathy (CSC) have spontaneous resolution of exudative macular detachments and a good visual prognosis. Patients with CSC have a primary choroidal hyperpermeability problem evident as multifocal areas of hyperpermeability during indocyanine green (ICG) angiography. A small percentage of patients develop chronic or progressive disease with widespread decompensation of the retinal pigment epithelium and severe vision loss. There is no known treatment for this variant of the disorder., Purpose: To study ICG-guided photodynamic therapy (PDT) with verteporfin as a potential treatment for patients with chronic CSC., Methods: Twenty eyes of 15 patients were studied with fluorescein angiography, optical coherence tomography, and ICG angiography to diagnose the maculopathy, monitor the detachments, and localize the choroidal hyperpermeability of the disorder. PDT with ICG guidance was applied to areas of choroidal hyperpermeability, and the patients were observed to determine the anatomic and functional outcomes., Results: Photodynamic therapy guided by ICG was associated with complete resolution of exudative macular detachments in 12 patients and incomplete resolution in the remaining eight eyes. The vision improved in six eyes and remained unchanged in 14 eyes during a mean follow-up of 6.8 months. Six weeks after treatment, the mean visual acuity improved by 0.55 lines, an amount that was marginally significant. There was a significant inverse correlation between the baseline visual acuity and the amount of improvement in acuity at 6 weeks. No patient had any treatment-related side effects., Conclusions: Indocyanine green angiography-guided PDT with verteporfin seems to aid in the resolution of exudative detachments in patients with chronic CSC. This treatment was associated with a rapid reduction in subretinal fluid and improvement in visual acuity. Although the follow-up time and number of patients in this pilot study were limited, the encouraging results and lack of complications suggest that further study is indicated.

This report describes a new system for digital indocyanine green videoangiography (ICGV) that provides enhanced imaging of the choroidal circulation. This newly assembled system was used to study a consecutive series of 129 patients with exudative age-related macular degeneration (AMD), and ill-defined or occult choroidal neovascularization (CNV). Overall, 39% of the patients in this study with occult CNV could be reclassified as having well-delineated or so-called classic CNV by virtue of the additional findings provided by ICGV. In this series, ICGV was particularly useful in identifying occult CNV in eyes with a large, serous pigment epithelial detachment (PED) and in eyes with recurrent CNV after previous laser photocoagulation treatment. Some of these patients were selected for laser photocoagulation of the abnormal choroidal vessels in order to evaluate the feasibility of this form of treatment on the basis of combined clinical, fluorescein angiographic, and ICGV findings. The results of this study suggest that ICGV is an important adjunct in the evaluation, classification, and laser treatment of patients with occult CNV secondary to AMD.

Purpose: To review the history of indocyanine green (ICG) angiography and to present a personal perspective on its use in the clinical setting today., Design: Perspective with literature review and opinions based on personal experience., Methods: To acquire views from international retinal physicians experienced with the technique on uses in their facilities and to compare them to the author's personal standards., Results: The author and contributing retinal physicians had surprisingly similar views for most, but not all, applications for ICG angiography use in the clinical setting., Conclusions: ICG angiography is recommended for a few highly selective chorioretinal disorders, including certain forms of neovascularization in age-related macular degeneration, other neovascular maculopathies, chronic central serous chorioretinopathy, choroidal hemangiomas, and posterior uveitis., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objectives: To describe fundus autofluorescence (FAF) in a series of patients with acute central serous chorioretinopathy (CSC)., Methods: Nine eyes of six patients with acute CSC were evaluated with fluorescein angiography (FA) and FAF imaging to evaluate the nature of the focal retinal pigment epithelial (RPE) leak evident with FA., Results: All nine eyes in this series demonstrated hypo-autofluorescence corresponding precisely to the site of the focal RPE leak seen on FA., Conclusions: In this group of patients, the acute focal RPE leaks seen with FA corresponded precisely to an area of hypo-autofluorescence imaged with FAF. This observation supports the concept that a mechanical defect or absence of the RPE accounts for the leakage from the inner choroid to the sub-neurosensory space in CSC. FAF is also a useful noninvasive diagnostic adjunct to identify the focal RPE leak in patients with acute CSC.

Purpose: To examine the fluorescence patterns of macular drusen using indocyanine green (ICG) angiography in human subjects of different ages., Methods: A total of 180 eyes in 100 consecutive patients aged 34-90 years with macula drusen were evaluated with ICG angiography using the Topcon 1024 ImageNet system. Eyes were divided into two groups depending on age. The older group (subjects over the age of 55 years) comprised 170 eyes. The younger group (subjects 55 years or younger) comprised 10 eyes. The fluorescence patterns of the drusen were graded as hyper-fluorescent (brighter than the background fluorescence), hypofluorescent (darker than the background fluorescence) or isofluorescent (unable to be distinguished from the background fluorescence). The ICG angiogram appearances were correlated with the clinical types of drusen in differing age groups., Results: An age-dependent difference in the fluorescence pattern of drusen was observed. Drusen in the older group were hypofluorescent or isofluorescent (169/170 eyes). In contrast, drusen in the younger group (55 years or younger) were hyperfluorescent (10/10 eyes)., Conclusions: This study defines the distinct age-dependent differences in the ICG fluorescence pattern of drusen. This heterogeneity might be due to differential binding of extravasated ICG to the constituents and histological site of the drusen deposits. Furthermore, ICG angiography is able to differentiate drusen types not possible by clinical and fluorescein angiographic evaluation.

Background: Most patients with central serous chorioretinopathy (CSC) have spontaneous resolution of exudative macular detachments and a good visual prognosis. Patients with CSC have a primary choroidal hyperpermeability problem evident as multifocal areas of hyperpermeability during indocyanine green (ICG) angiography. A small percentage of patients develop chronic or progressive disease with widespread decompensation of the retinal pigment epithelium and severe vision loss. There is no known treatment for this variant of the disorder., Purpose: To study ICG-guided photodynamic therapy (PDT) with verteporfin as a potential treatment for patients with chronic CSC., Methods: Twenty eyes of 15 patients were studied with fluorescein angiography, optical coherence tomography, and ICG angiography to diagnose the maculopathy, monitor the detachments, and localize the choroidal hyperpermeability of the disorder. PDT with ICG guidance was applied to areas of choroidal hyperpermeability, and the patients were observed to determine the anatomic and functional outcomes., Results: Photodynamic therapy guided by ICG was associated with complete resolution of exudative macular detachments in 12 patients and incomplete resolution in the remaining eight eyes. The vision improved in six eyes and remained unchanged in 14 eyes during a mean follow-up of 6.8 months. Six weeks after treatment, the mean visual acuity improved by 0.55 lines, an amount that was marginally significant. There was a significant inverse correlation between the baseline visual acuity and the amount of improvement in acuity at 6 weeks. No patient had any treatment-related side effects., Conclusions: Indocyanine green angiography-guided PDT with verteporfin seems to aid in the resolution of exudative detachments in patients with chronic CSC. This treatment was associated with a rapid reduction in subretinal fluid and improvement in visual acuity. Although the follow-up time and number of patients in this pilot study were limited, the encouraging results and lack of complications suggest that further study is indicated.

Purpose: To clarify the frequency and nature of ICG angiographic "hot spots" seen in patients with neovascular age-related macular degeneration (ARMD)., Methods: A consecutive series of newly diagnosed patients with neovascular ARMD and fluorescein angiographic evidence of occult choroidal neovascularization (occult CNV) was imaged with ICG angiography. Eyes with ICG angiographic "hot spots" were identified and further classified. A hot spot was defined as any area of abnormal hyperfluorescence, in the mid to late stages of ICG angiography, measuring less than 1 disk area in size., Results: From a total of 190 patients (220 eyes) with neovascular ARMD, 30 patients and 34 eyes (16%) with hot spots were identified. Hot spots were noted to be of three distinct patterns: polypoidal choroidal neovascularization (polypoidal CNV) in 21 of 34 eyes, or 62%; retinal angiomatous proliferation (RAP) in 11 of 34 eyes, or 30%; and focal occult CNV in 2 of 34 eyes, or 8%., Conclusions: A focal area of intense hyperfluorescence or so-called hot spot seen on ICG angiography in neovascular ARMD is due to one of three possible forms of neovascularization: most frequently polypoidal CNV, less commonly RAP, and infrequently nonspecific, focal occult CNV. Since neovascular ARMD may be caused by different types of neovascularization, each with distinct clinical manifestations, natural course, visual prognosis, and response to treatment, it is important to identify the precise nature of hot spots to establish an accurate diagnosis and, when appropriate, a specific form of management.

The article describes the case of two patients with Coats disease treated with diffuse hyperpermeability from angiographically normal capillaries. Vascular telangiectasia in the retina, aneurysms, and leakage from abnormal blood vessels are the disease's characteristics. Intravitreal bevacizumab and limited laser photocoagulation were used on the first patient while the second patient had only limited photocoagulation. Normal capillaries are preserved by treating only the primary lesions.

The article describes the fundus autofluorescence photographic pattern in a 91-year-old woman with deferoxamine retinopathy. The patient was receiving intravenous deferoxamine treatment. Autofluorescence photography showed well circumscribed areas of hyperautofluorescence that were found to be correlated with areas of pigmentation seen in color photography.

Abstract: More than a quarter century has passed since the original description of polypoidal choroidal vasculopathy (PCV) in 1982 as a peculiar hemorrhagic disorder involving the macula characterized by recurrent subretinal pigment epithelial bleeding. In the ensuing years, numerous reports have described the expanded clinical spectrum of this entity. PCV is the principal vascular composition of patients of pigmented races experiencing neovascular maculopathies, particularly African Americans and Asians. This form of neovascularization is now known to occur in white patients with or without concomitant drusen, and the site of involvement has extended from the peripapillary area to the peripheral fundus. Indocyanine green angiography has made detection of these abnormal vascular changes more reliable and definitive. More precise diagnosis has also led to a better understanding of specific clinical features that distinguish PCV from more typical proliferations of abnormal choroidal vessels. We review the nature of PCV, including its genetic basis, demographic features, histopathology, clinical manifestations, natural course, response to treatments, and the histopathological and genetic bases. We emphasize multimodal ophthalmic imaging of these vessels, in particular fluorescein and indocyanine green angiography and optical coherence tomography. [ABSTRACT FROM AUTHOR]