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Purpose: To evaluate the depth and pattern of retinal hemorrhage in acute central retinal vein occlusion (CRVO) and to correlate these with visual and anatomic outcomes., Methods: Retinal hemorrhages were evaluated with color fundus photography and fluorescein angiography at baseline and follow-up. Snellen visual acuity (VA), central foveal thickness (CFT), extent of retinal ischemia, and development of neovascularization were analyzed., Results: 108 eyes from 108 patients were evaluated. Mean age was 63.6 ± 16.1 years with a predilection for the right eye (73.1%). Average follow-up was 17.2 ± 19.2 months. Mean VA at baseline was 20/126 and 20/80 at final follow-up. Baseline (P = 0.005) and final VA (P = 0.02) in eyes with perivascular nerve fiber layer (NFL) hemorrhages were significantly worse than in eyes with deep hemorrhages alone. Baseline CFT was greater in the group with perivascular hemorrhages (826 ± 394 µm) compared to the group with deep hemorrhages alone (455 ± 273 µm, P < 0.001). The 10 disc areas of retinal ischemia was more common in patients with perivascular (80.0%) and peripapillary (31.3%) versus deep hemorrhages alone (16.1%, P < 0.001). Neovascularization of the iris was more common, although this differrence was not significant, in the groups with peripapillary (14.3%) and perivascular (2.0%) NFL versus deep hemorrhages alone (0.0%)., Conclusions: NFL retinal hemorrhages at baseline correlate with more severe forms of CRVO, with greater macular edema, poorer visual outcomes, and greater risk of ischemia and neovascularization. This may be related to the organization of the retinal capillary plexus. The depth and pattern of distribution of retinal hemorrhages in CRVO may provide an easily identifiable early biomarker of CRVO prognosis.

Purpose: To establish a process to evaluate and standardize a state-of-the-art nomenclature for reporting neovascular age-related macular degeneration (AMD) data., Design: Consensus meeting., Participants: An international panel of retina specialists, imaging and image reading center experts, and ocular pathologists., Methods: During several meetings organized under the auspices of the Macula Society, an international study group discussed and codified a set nomenclature framework for classifying the subtypes of neovascular AMD and associated lesion components., Main Outcome Measures: A consensus classification of neovascular AMD., Results: The study group created a standardized working definition of AMD. The components of neovascular AMD were defined and subclassified. Disease consequences of macular neovascularization were delineated., Conclusions: The framework of a consensus nomenclature system, a definition of AMD, and a delineation of the subtypes of neovascular AMD were developed. Establishing a uniform set of definitions will facilitate comparison of diverse patient groups and different studies. The framework presented is modified and updated readily, processes that are anticipated to occur on a periodic basis. The study group suggests that the consensus standards outlined in this article be used in future reported studies of neovascular AMD and clinical practice., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Retinal vascular tortuosity may occur in a wide range of ocular disorders. When retinal vascular tortuosity involves both arteries and veins, and presents unilaterally and without hemorrhage, a diagnosis of Wyburn Mason syndrome (WMS) should be considered due to the potential morbidity and mortality associated with cerebral involvement. Magnetic resonance imaging (MRI) and MRI angiography (MRA) are important tools for identifying cerebral arteriovenous malformations (AVMs), but these imaging modalities have limited spatial resolution to detect very small vascular lesions. Annular array contact ocular ultrasound is a new imaging modality capable of detecting small intraorbital AVMs. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:239-243.]., (Copyright 2020, SLACK Incorporated.)

Purpose: To describe novel spectral-domain (SD) and swept-source (SS) optical coherence tomography (OCT) linear and planar reflection artifacts produced by hyperreflective crystalline deposits (HCD)., Methods: Imaging from 10 eyes with HCD producing linear and planar artifacts on OCT was retrospectively analyzed. All eyes had SD-OCT (Spectralis HRA + OCT, Heidelberg Engineering, Germany) and SS-OCT angiography (PLEX Elite 9000, Carl Zeiss Meditec, Inc., Dublin, CA) acquired on the same day. The horizontal extent of planar artifacts and the corresponding HCD on B-scans was measured using a digital caliper. Artifact features from HCD in eyes with non-neovascular age-related macular degeneration (AMD) were analyzed and compared to those seen in two eyes with the "onion sign," an OCT signature previously shown to represent cholesterol crystals (CC) in the sub-retinal pigment epithelium-basal laminar space of eyes with neovascular AMD. A third eye with the "onion sign" was imaged with dense B-scan (DB)-OCTA., Results: Ten eyes of ten patients (77.4 ± 8.7 years) with HCD were analyzed. On SS-OCTA, HCD produced linear artifacts of high signal intensity passing through the HCD and spanning the entire scan depth. On SD-OCT, HCD produced planar artifacts located anterior to both the retina and a hyporeflective space representing normal vitreous signal. The horizontal extent of the artifact did not differ significantly from the corresponding HCD on OCT B-scans (P = 0.62). The OCT artifacts produced by the "onion sign" appeared similar to those of HCD. The additional eye with neovascular AMD imaged with DB-OCTA was characterized by a single, vertical, linear false-flow signal crossing retinal layers., Conclusions: To the authors' knowledge, this is the first description of SD- and SS-OCT/OCTA artifacts corresponding to both HCD and the "onion sign." These artifacts are likely due to highly reflective CC previously shown on histology to correspond to both of these OCT signatures.

Purpose: Cuticular drusen (CD) have been associated with manifestations of age-related macular degeneration such as atrophy and neovascularization in the macula. In this study, eyes with CD were followed and investigated for the estimated 5-year risk of progression to sequelae of age-related macular degeneration such as geographic atrophy (GA) and macular neovascularization (MNV)., Methods: A consecutive series of patients with CD were followed for the development of GA and MNV. Whenever possible, they were also studied retrospectively. The patients with CD were categorized into three phenotypic groups. Phenotype 1: eyes had concentrated, densely populated CD in the macular and paramacular area, Phenotype 2: eyes showed scattered CD in the posterior fundus, and Phenotype 3: involved eyes with CD mixed with large drusen (>200 µm). The 5-year incidence of progression was then estimated using a Kaplan-Meier estimator., Results: A total of 63 eyes from 38 patients (35 women with a mean age at presentation of 58.9 ± 14.2 years) were studied and followed for a mean of 40 ± 18 months. Thirteen patients had single eyes with GA (84.5%; 11/13) or MNV (15.5%; 2/13) in one eye at presentation and were subsequently excluded. Geographic atrophy developed in 19.0% (12/63) of eyes and MNV in 4.8% (3/63) of eyes. The cumulative estimated 5-year risk of GA and MNV was 28.4% and 8.7%, respectively. The estimated 5-year incidence of MNV or GA was 12.6%, 50.0%, and 51.6% in Phenotype 1, Phenotype 2, and Phenotype 3, respectively (P = 0.0015, log-rank test). No difference in risk was found in the development of GA or MNV (P = 0.11) between the subgroup of patients presenting with GA or MNV in their fellow eye and those with both eyes included., Conclusion: When patients with CD are followed longitudinally, there was a significant risk of progression to GA or MNV for Phenotype 2 and Phenotype 3. Patients with CD are commonly first diagnosed in the fifth decade of life, and there is a female predominance. Clinicians should use multimodal imaging to detect and be aware of the risk of progression to manifestations of GA and MNV. These risks of GA and MNV suggest that patients with CD may be part of the overall spectrum of age-related macular degeneration.

Purpose: To characterize structural and angiographic findings in macular telangiectasia Type 2 (MacTel 2) and examine associations with visual acuity., Methods: MacTel 2 patients with complete ophthalmologic examination, including fundus photography, autofluorescence, spectral-domain optical coherence tomography, and projection-resolved optical coherence tomography angiography, were retrospectively evaluated., Results: There were 43 eyes of 22 patients with a mean age 63.9 (±10.3) years. Six patients had diabetes. Twenty-one eyes (48.8%) had retinal-choroidal anastomoses (RCAs) without any evidence of neovascularization extending laterally in a plane above or below the retinal pigment epithelium. None of the eyes had hemorrhage, lipid, or signs of subretinal exudation. When present, an average of 55 (±33.7) individual RCAs were clustered primarily in temporal juxtafoveal region of involved eyes. Right-angle veins were seen in all 21 eyes with RCAs, and hyperpigmentation was present in 18 (P < 0.001 for both). A conical collection of hyperreflective material spanning from Bruch membrane past external limiting membrane of ≥200-μm basal diameter was found in 21 eyes and labeled outer retinal hyperreflective lesion. Retinal-choroidal anastomoses occurred in clusters, often within the outer retinal hyperreflective lesion. This lesion colocalized with focal thinning of the outer nuclear layer and was surrounded by a larger defect in the ellipsoid zone. The presence of diabetes (P = 0.015), outer retinal hyperreflective lesion (P = 0.006), RCA (P = 0.005), and ellipsoid zone defect extent (P < 0.001) were associated with decreased visual acuity., Conclusion: Retinal-choroidal anastomoses occur in eyes with MacTel 2 without signs of exudation. Retinal-choroidal anastomoses occur in numerous clusters particularly in the temporal juxtafoveal macula. Diabetes, ellipsoid zone defect extent, RCAs, and the outer retinal hyperreflective lesion predict poorer vision in MacTel 2.

Central serous chorioretinopathy (CSC) is a common cause of central vision loss, primarily affecting men 20-60 years of age. To date, no consensus has been reached regarding the classification of CSC, and a wide variety of interventions have been proposed, reflecting the controversy associated with treating this disease. The recent publication of appropriately powered randomised controlled trials such as the PLACE trial, as well as large retrospective, non-randomised treatment studies regarding the treatment of CSC suggest the feasibility of a more evidence-based approach when considering treatment options. The aim of this review is to provide a comprehensive overview of the current rationale and evidence with respect to the variety of interventions available for treating CSC, including pharmacology, laser treatment, and photodynamic therapy. In addition, we describe the complexity of CSC, the challenges associated with treating CSC, and currently ongoing studies. Many treatment strategies such as photodynamic therapy using verteporfin, oral mineralocorticoid antagonists, and micropulse laser treatment have been reported as being effective. Currently, however, the available evidence suggests that half-dose (or half-fluence) photodynamic therapy should be the treatment of choice in chronic CSC, whereas observation may be the preferred approach in acute CSC. Nevertheless, exceptions can be considered based upon patient-specific characteristics., (Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background and Objective: To describe the prevalence and anatomic correlates for hyperautofluorescence related to outer retinal disruption in eyes with multifocal choroiditis (MFC)., Patients and Methods: Retrospective review of MFC patients., Results: Fifty-nine eyes from 37 patients were analyzed. Multimodal imaging was utilized to identify nine eyes (15.2%) of six patients with either transient (Group 1) or persistent (Group 2) regions of hyperautofluorescence associated with ellipsoid zone (EZ) disruption over intact retinal pigment epithelium (RPE). Group 1 included four eyes (6.8%) of three patients in which the hyperautofluorescence and EZ loss resolved within a few months (range: 28 days to 125 days) and had intact overlying outer nuclear (ONL) and outer plexiform layers (OPL) (mean follow-up: 1.3 years). Group 2 included five eyes (8.5%) of three patients with regions of permanent EZ disruption associated with absent or reduced ONL and OPL (mean follow-up: 4.6 years)., Conclusions: Hyperautofluorescence correlating with EZ disruption over intact RPE is a rare occurrence in MFC. Evaluating outer retinal integrity by optical coherence tomography may help identify eyes with potential for EZ restoration, which may have implications regarding treatment strategies. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:675-683.]., (Copyright 2019, SLACK Incorporated.)

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 : Leber congenital amaurosis (LCA) due to RDH12 mutations typically manifests with severe vision loss and panretinal dystrophy. We sought to describe a case of LCA with choroidal neovascularization (CNV) in a 17-year-old patient. Materials and Methods : Case report of a 17-year old with LCA who presented with acute central vision loss of the right eye in the context of a chronic retinal dystrophy. Multimodal retinal imaging including spectral-domain optical coherence tomography and indocyanine green angiography revealed CNV. Results : A 17-year-old boy with previously diagnosed LCA/early-onset retinal dystrophy (EOSRD), with subsequently identified biallelic mutations in RDH12 was found to have type 2 CNV. Patient was treated with intravitreal ranibizumab and exhibited improvement on follow-up exam. Conclusions : Choroidal neovascularization may be a unique occurrence in RDH12- associated retinal dystrophy. Successful treatment of the neovascularization could be accomplished with intravitreal antivasogenic therapy.

Purpose: To explore patterns of disease progression in nonneovascular age-related macular degeneration (AMD) associated with hyperreflective crystalline deposits (HCDs) in the sub-retinal pigment epithelium-basal laminar space., Methods: Retrospective review of medical records, multimodal imaging, and longitudinal eye-tracked near-infrared reflectance (NIR) and optical coherence tomography (OCT) spanning ≥2 years. NIR/OCT images were analyzed with ImageJ software to identify HCD morphology and location. Associated macular complications were reviewed from the time of HCD detection to the most recent follow-up, using NIR/OCT., Results: Thirty-three eyes with HCDs from 33 patients (mean age: 72 ± 7.5 years) had 46.7 months (95% confidence limits: 33.7, 59.6) of serial eye-tracked NIR/OCT follow-up. Baseline best-corrected visual acuity (BCVA) was 0.44 logMAR (Snellen equivalent 20/55). At a mean of 11.3 months (3.1, 19.6) after HCD detection, 31/33 (93.9%) eyes had developed macular complications including de novo areas of complete retinal pigment epithelium and outer retinal atrophy (cRORA) in 21/33 (64%) eyes, enlargement of preexisting cRORA in 4/33 (12%) eyes, and incident macular neovascularization in 3/33 (9%) eyes. Movement and clearance of HCDs in 9/33 (27%) eyes was associated with enlargement of preexisting cRORA (r = 0.44, P = 0.02). BCVA at the last follow-up visit had decreased to 0.72 logMAR (20/105)., Conclusions: Eyes with nonneovascular AMD demonstrating HCDs are at risk for vision loss due to macular complications, particularly when movement and clearance of these structures appear on multimodal imaging. HCD reflectivity and dynamism may be amenable to automated recognition and analysis to assess cellular activity related to drusen end-stages.

Objective: To explore the structural differences between X-linked retinoschisis (XLR) and stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) using swept-source optical coherence tomography angiography (SS-OCTA)., Methods: A case series of two patients, a 9-year-old male with XLR and a 58-year-old woman with SNIFR were imaged with swept-source optical coherence tomography angiography (SS-OCTA; PLEX Elite 900, Carl Zeiss Meditec, Inc, Dublin, CA). Automated segmentation was manually adjusted to include the areas of retinoschisis within en face flow and structural slabs. The flow data were binarized using ImageJ 1.51s (Wayne Rasband, National Institutes of Health, USA, http://imagej.nih.gov.ij ) and superimposed onto the structural slab., Results: In the eye with XLR, OCTA flow data superimposed on the structural slab demonstrated flow signal within numerous bridging structures connecting the inner and outer plexiform layers containing the intermediate (ICP) and deep (DCP) capillary plexuses. In contrast, the same technique applied to the eye with SNIFR demonstrated an absence of flow signal in the cystic retinal spaces within Henle's fiber layer., Conclusions: The vascular pattern of bridging vessels between the ICP and DCP is closely related to the structural "retinoschisis" pattern of XLR and appears to be structurally different from that seen in SNIFR. Moreover, the connecting vessels appear to be highly represented and regularly distributed, thereby supporting a serial arrangement of the retinal capillary plexuses within the perifoveal macula.

Purpose: To evaluate the long-term visual outcomes and causes of vision loss in chronic central serous chorioretinopathy (CSC)., Design: Retrospective, longitudinal study., Participants: A total of 133 participants (217 eyes) with chronic CSC., Methods: A retrospective review of clinical and multimodal imaging data of patients with chronic CSC managed by 3 of the authors between May 1977 and March 2018. Multimodal imaging comprised color photography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence (FAF), and OCT., Main Outcome Measures: Best-corrected visual acuity (BCVA) at the final visit; change in BCVA between first visit and 1-, 5-, and 10-year follow-up visits; and causes of vision loss at final visit., Results: Data from 6228 individual clinic visits were analyzed. Mean age of patients at the first visit was 60.7 years, and mean period of follow-up from first to last visit was 11.3 years. The cohort included 101 male patients (75.9%). At the final visit, 106 patients (79.7%) maintained driving-standard vision with BCVA of 20/40 or better in at least 1 eye, and 17 patients (12.8%) were legally blind with BCVA of 20/200 or worse in both eyes. Mean BCVA at first visit was not significantly different from mean BCVA at 1- or 5-year follow-up visits (both P ≥ 0.65) but was significantly better than the mean BCVA at the 10-year follow-up visit (P = 0.04). Seventy-nine percent of eyes with 20/40 or better vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Ninety-two percent of eyes with 20/200 or worse vision at the first visit maintained the same level of vision at the 10-year follow-up visit. Cystoid macular degeneration, choroidal neovascularization (CNV), outer retinal disruption on OCT, and FAF changes were associated with poorer vision at the final visit (all P ≤ 0.001). Multivariable analysis revealed that greater age at first visit was associated with greater BCVA change at the 10-year follow-up visit (P = 0.001)., Conclusions: Chronic CSC can be a sight-threatening disease leading to legal blindness. Age at presentation and outer retinal changes on multimodal imaging were associated with long-term BCVA changes and may be predictors of long-term visual outcomes., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe a patient with a presumed retinal pigment epithelium (RPE) tumor originating from unilateral RPE dysgenesis., Methods: Case report., Results: A 30-year-old woman with an unremarkable medical and ocular history was referred for an evaluation of progressive central metamorphopsia in her left eye. Visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination of the left eye revealed an elevated mass within an area of unilateral RPE dysgenesis showing hyperpigmentation and hypopigmentation with scalloped margins. Fundus autofluorescence of the lesion showed a marginal pattern of hyperautofluorescence and hypoautofluorescence that was the inverse of the fluorescein angiography pattern. A well-circumscribed subretinal mass appeared to originate from the unilateral RPE dysgenesis lesion with surrounding subretinal fluid extending beneath the fovea. Ultrasonography showed medium-to-high reflectivity of the mass with no evidence of choroidal involvement. Optical coherence tomography showed a subretinal hyporeflective mass consistent with a tumor of RPE origin. The tumor appeared to invade the overlying retina where fluorescein angiography showed hyperfluorescent leakage and OCT angiography showed retinal vascular deformation. Indocyanine green angiography showed no evidence of choroidal neovascularization. Findings in the right fundus were normal. Over a 5-month follow-up, intravitreal anti-vascular endothelial growth factor therapy induced a resolution of subretinal exudation and modest reduction in tumor thickness., Conclusion: To the authors' knowledge, this is the first report of a presumed RPE tumor described as originating from unilateral RPE dysgenesis. Multimodal imaging was crucial for establishing the diagnosis and showing that the patient's visual symptoms were a product of the exudation produced by the tumor's invasion of the retina.

Purpose: To describe the preclinical and very early stages of type 3 neovascularization using multimodal retinal imaging to expand our understanding of the pathogenesis of this disorder and potentially to prevent late treatment., Design: Retrospective cohort study., Participants: Patients diagnosed with treatment-naïve type 3 neovascularization in the setting of age-related macular degeneration were identified at 4 retina referral centers. Inclusion criteria were: patients older than 55 years with at least 1 OCT and OCT angiography (OCTA) examination before the onset of clinically active type 3 neovascularization (i.e., preclinical stage)., Methods: Patients underwent a complete ophthalmologic examination including at least OCT and OCTA at the baseline and preclinical stage examinations, and dye angiographies when available. Demographics and clinical findings were analyzed., Main Outcome Measures: Description of multimodal imaging features of nascent type 3 neovascularization., Results: Fifteen eyes (15 patients; mean age, 83 ± 9 years) were included. At the baseline, mean BCVA was 0.32 ± 0.17 logarithm of the minimum angle of resolution and central macular thickness was 313 ± 50 μm. Preclinical (i.e., prebaseline) structural OCT illustrated the presence of intraretinal hyperreflective foci (HRF) at the site of type 3 neovascularization development in all patients. These foci were characterized by hyperfluorescence on dye angiography and by detectable flow on OCTA, identified with either the avascular slab (20%) or with both the deep retinal capillary plexus (DCP) and avascular slabs (80%). Typically, HRF with detectable flow on OCTA were characterized by the absence of intraretinal exudation (or very mild microcystic changes) until the lesion progressed from the DCP into the retinal pigment epithelium (RPE) and sub-RPE space. Of note, in 1 patient we observed the complete resolution of HRF despite the presence of OCTA flow and dye angiography hyperfluorescence detected at the preclinical stage examination., Conclusions: Hyperreflective foci on structural OCT may represent early intraretinal neovascularization originating from the DCP, namely nascent type 3 neovascularization; these lesions can progress to active type 3 neovascularization or more rarely may regress without functional impairment. An advanced multimodal imaging approach is useful in detecting nascent type 3 lesions, which should be followed up carefully and treated as soon as possible if flow progresses to the RPE and sub-RPE space to prevent progression to late stages., (Copyright © 2018 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

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 deep retinal vascular changes potentially present in macular telangiectasis Type 2 (MacTel 2) with projection resolved optical coherence tomography angiography including volume rendering., Methods: Retrospective consecutive evaluation of patients with MacTel 2 in a community-based retinal referral practice with a comprehensive ophthalmologic examination to include optical coherence tomography and projection-resolved optical coherence tomography angiography with volume rendering. Main outcome measures were the characterization of vessel presence and anatomical arrangement in the outer retina., Results: There were 26 eyes of 13 patients with a mean age of 64.9 (±11.3) years, and 6 were men. The mean visual acuity was logMAR 0.4 (Snellen equivalent 20/50). No eye had signs of choroidal neovascularization or exudation. Focal hyperpigmentation was seen in 13 (50%) and right-angle veins in 17 (65%) eyes. Retinal-choroidal anastomoses were seen in 17 (65%) eyes. These anastomoses typically occurred in multiple clusters of small vessels. The presence of anastomoses was associated with pigment (P < 0.001), although the anastomoses did not necessarily colocalize with the pigment, and right-angle veins (P < 0.001), which were found in every eye with a retinal-choroidal anastomosis., Conclusion: Retinal-choroidal anastomoses were commonly observed in eyes with MacTel 2 using projection-resolved optical coherence tomography angiography. One animal model for MacTel 2 uses very low-density lipoprotein receptor mutant mice and shows multiple retinal-choroidal anastomoses in the disease pathogenesis as well. These findings suggest MacTel 2 is more than just a neurodegenerative disease with secondary vascular abnormalities, as the choroid may be involved in the disease process.

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 report the posterior segment and retinal vascular manifestations of calcific uremic arteriolopathy (calciphylaxis). Clinical findings are correlated with multimodal imaging results., Methods: Observational case report., Results: A 65-year-old white woman on hemodialysis was referred for assessment of poor vision bilaterally. Clinical examination demonstrated a crystalline retinopathy with stigma of previous retinal arterial occlusion. Fluorescein angiography revealed delayed retinal arterial filling bilaterally, sheathing of vessels, and peripheral nonperfusion. The crystals were hyperautofluorescent. Spectral domain and enhanced depth imaging optical coherence tomography localized the crystals within the retina with a predilection for the retinal arterial vasculature. The choriocapillaris was not involved. Two years prior, the patient developed necrotic skin lesions which were biopsied and confirmed the diagnosis of calciphylaxis., Conclusion: Calcific uremic arteriolopathy is an extremely rare cause of thrombogenic microangiopathy in end-stage renal disease patients. Retinal arterial occlusion appears to be a rare but significant cause of visual loss in this disease and is likely to be consequent to crystalline deposition in the retinal vasculature.

Purpose: To report the clinical course and multimodal imaging features of acute zonal occult outer retinopathy (AZOOR) complicated by choroidal neovascularization (CNV) treated with anti-vascular endothelial growth factor (VEGF) treatment or photodynamic therapy (PDT)., Methods: Observational case series. Retrospective analysis of patients presenting to different institutions with evidence of AZOOR and neovascular lesions. Diagnosis of AZOOR was made on the basis of clinical presentation and multimodal imaging. All patients underwent a comprehensive ophthalmic evaluation and multimodal retinal imaging, including color fundus photos, fundus autofluorescence, fundus fluorescein angiography and spectral-domain optical coherence tomography., Results: Four patients (three males, mean age 53.5 years) were included in the study. Mean follow-up was 5.1 years. Presentation of AZOOR was unilateral in two patients and bilateral in the remainder two patients. One of the patients presenting with unilateral AZOOR developed zonal lesions in the fellow eye during follow-up. All patients presented with unilateral type 2 (subretinal) CNV. Three patients underwent intravitreal anti-VEGF injections and one patient underwent a single PDT. Multimodal retinal imaging showed zonal or multizonal progression during treatment. After treatment, visual acuity and CNV stabilization was observed in all patients., Conclusions: The presence of CNV expands the clinical spectrum of AZOOR. CNV complicating AZOOR may be effectively treated with intravitreal injections of anti-VEGF, despite progression of the zonal lesions. Further studies are required to define the role of treatment in the progression of the zonal lesions.

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.)

Purpose: Mutations in the eyes shut homolog (EYS) gene are a frequent cause of autosomal recessive retinitis pigmentosa (arRP). This study used multimodal retinal imaging to elucidate genotype-phenotype correlations in EYS-related RP (EYS-RP)., Design: Cross-sectional study., Methods: Multimodal retinal imaging and electrophysiologic testing were assessed for 16 patients with genetic confirmation of EYS-RP., Results: A total of 27 unique EYS variants were identified in 16 patients. Seven patients presented with an unusual crescent-shaped hyperautofluorescent (hyperAF) ring on fundus autofluorescence (FAF) imaging encompassing a large nasal-superior area of the posterior pole. Three patients had a typical circular or oval perifoveal hyperAF ring and 6 patients had no hyperAF ring. Spectral-domain (SD) and en face optical coherence tomography (OCT) showed preserved ellipsoid zone and retinal thickness spatially corresponding to areas within the hyperAF rings. Eleven patients presented with a rod-cone dystrophy on full-field electroretinogram (ffERG), 1 patient presented with cone-rod dystrophy, and 4 patients did not undergo ffERG testing. A significant spatial association was found between EYS variant position and FAF phenotype, with variants occurring at a nucleotide position greater than GRCh37 6:65300137 (c.5617C) being more associated with patients exhibiting hyperAF rings at presentation., Conclusions: EYS-RP is a heterogeneous manifestation. Variants occurring in positions closer to the C-terminus of EYS are more common in patients presenting with hyperAF rings on FAF imaging., (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 expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A)., Design: Retrospective cohort study., Methods: Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups., Results: Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti-vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC., Conclusions: PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Importance: Acute zonal occult outer retinopathy (AZOOR) remains a challenging diagnosis. Early recognition of the disease depends on advances in imaging modalities that can improve phenotyping and contribute to the understanding of the underlying pathogenesis., Objectives: To expand the range of approaches available to assist in the identification of AZOOR by multimodal imaging and to analyze the fundus lesions by quantifying short-wavelength fundus autofluorescence (quantitative fundus autofluorescence [qAF]) and spectral-domain optical coherence tomography., Design, Setting, and Participants: In this observational study, patients underwent imaging at Columbia University Medical Center between November 2010 and March 2016 and were analyzed between September 2015 and August 2016. Six patients diagnosed as having AZOOR were studied by qAF and spectral-domain optical coherence tomography and were compared with 30 age and race/ethnicity–matched controls from a database of 277 healthy control eyes., Main Outcomes and Measures: In unaffected regions of the macula, qAF was calculated within predetermined circularly arranged segments (qAF8). In addition, qAF was measured within specified regions of interest positioned at the autofluorescent lesion border (AZOOR line). Electroretinograms and electro-oculograms were recorded in 5 of 6 patients., Results: Among 6 patients (age range, 26-61 years; 4 female; 4 of white race/ethnicity, 1 Asian, and 1 Hispanic), 5 exhibited an autofluorescent AZOOR line in short-wavelength fundus autofluorescence images, delineating the peripapillary lesion. The mean (SD) region-of-interest qAF measured on the AZOOR line was 60 (26) times higher than in healthy control eyes (P = .03) at equivalent fundus locations. The qAF8 within nondiseased macular regions were within the normal range. At the lesion border, spectral-domain optical coherence tomography revealed a loss of outer retinal integrity in all patients. Single-flash cone b-wave latency and 30-Hz flicker latency responses were significantly delayed bilaterally. Lesions with smooth, homogeneous borders exhibited only minimal expansion in size over time, while the lesion in a patient with a heterogeneous border progressed more rapidly., Conclusions and Relevance: The finding that qAF is elevated at the border between diseased and nondiseased retina in patients with AZOOR contributes to the understanding of the natural history of the disease.

Purpose: To investigate the clinical and morphologic characteristics of serous retinal disturbances in patients taking mitogen-activated protein kinase kinase (MEK) inhibitors., Participants: A total of 313 fluid foci in 50 eyes of 25 patients receiving MEK inhibitors for treatment of their metastatic cancer, who had evidence of serous retinal detachments confirmed by optical coherence tomography (OCT)., Design: Single-center, retrospective cohort study., Methods: Clinical examination and OCT were used to evaluate MEK inhibitor-associated subretinal fluid. The morphology, distribution, and location of fluid foci were serially evaluated for each eye. Choroidal thickness was measured at each time point (baseline, fluid accumulation, and fluid resolution). Two independent observers performed all measurements. Statistical analysis was used to correlate interobserver findings and compare choroidal thickness and visual acuity at each time point., Main Outcome Measures: Comparison of OCT characteristics of retinal abnormalities at baseline to fluid accumulation., Results: The majority of patients had fluid foci that were bilateral (92%) and multifocal (77%) and at least 1 focus involving the fovea (83.3%). All fluid foci occurred between the interdigitation zone and an intact retinal pigment epithelium. The 313 fluid foci were classified into 4 morphologies, as follows: 231 (73.8%) dome, 36 (11.5%) caterpillar, 31 (9.9%) wavy, and 15 (4.8%) splitting. Best-corrected visual acuity at fluid resolution was not statistically different from baseline; and no eye lost more than 2 Snellen lines from baseline at the time of fluid accumulation. There was no statistical difference in the choroidal thickness between the different time points (baseline, fluid accumulation, and fluid resolution). A strong positive interobserver correlation was obtained for choroidal thickness measurements (r = 0.97, P < 0.0001) and grading of foci morphology (r = 0.97, P < 0.0001)., Conclusion: The subretinal fluid foci associated with MEK inhibitors have unique clinical and morphologic characteristics, which can be distinguished from the findings of central serous chorioretinopathy. In this series, MEK inhibitors did not cause irreversible loss of vision or serious eye damage., (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: The authors describe a woman diagnosed with hemophagocytic lymphohistiocytosis and found to have retinal examination findings consistent with Purtscher retinopathy., Methods: A 52-year-old woman underwent multimodal imaging, including color fundus photography and spectral-domain optical coherence tomography, to confirm the diagnosis., Results: The ophthalmic examination and imaging confirmed the findings of Purtscher retinopathy with significant inner retinal thickening on spectral-domain optical coherence tomography. Throughout a hospital course complicated by multi-organ failure, she continued to have profoundly limited visual acuity, likely resulting from inner retinal ischemia affecting the posterior pole of both eyes., Conclusion: The authors describe a patient with hemophagocytic lymphohistiocytosis, a disease characterized by disruption of normal natural killer cell activity with subsequent uncontrolled cytokine release, who presented with Purtscher retinopathy confirmed with spectral-domain optical coherence tomography.

Purpose: To describe two patients who showed full-thickness macular holes (FTMH) combined with pigment epithelial detachments (PED) and had contrasting outcomes to treatment., Methods: A retrospective report of two cases., Results: Case 1 was treated with intravitreal antivascular endothelial growth factor and photodynamic therapy, and the PED flattened. Subsequently, a vitrectomy was performed and the FTMH closed. Her visual acuity improved from 20/200 to 20/25. In Case 2, the same medical therapy, with less frequency compared with Case 1, did not affect the PED, and the FTMH failed to close with surgery. Later, a spontaneous collapse of the PED occurred but the FTMH was persistent with an associated poor visual acuity of 20/200., Conclusion: Full-thickness macular hole is rare, but can occur in association with large PEDs. Although the pathologic mechanism was uncertain, visual outcomes were dependent on response to treatments of the PED, as well as anatomical closure of the FTMH.

Purpose: To demonstrate unusual retinal findings in a patient with progressive renal failure due to idiopathic monoclonal immunoglobulin light chain deposition disease, using multimodal imaging., Methods: Observational case report of a 43-year-old white man with renal failure due to light chain deposition disease. His course over 6 years was documented with multimodal imaging including fundus photography, fundus autofluorescence, fluorescein angiography, and spectral domain optical coherence tomography. Additional evaluations included ocular ultrasound, electroretinography, positron emission tomography, serum protein electrophoreses, skeletal surveys to detect osteolytic lesions, and renal, liver, and rectal biopsies in search of amyloid., Results: The patient's ocular course mirrored the severity of his renal dysfunction for which he required a renal transplant. Changes observed in the native kidney recurred in the transplant 2 years later, as evidenced by immunohistochemistry, revealing thick linear deposits of kappa chains, with no complement, overlying the glomerular basement membrane. The systemic workup was negative for amyloid but showed an overwhelming ratio of kappa to lambda light chains on serum protein electrophoreses and no clinical signs of plasma cell dyscrasias, all consistent with idiopathic light chain deposition disease. The patient presented with a generalized, bilateral "leopard-spot" fundus appearance on fundus autofluorescence, striking globular subretinal deposits on spectral domain optical coherence tomography, and subfoveal subretinal fluid without retinal pigment epithelium detachment or choroidal effusions. The subfoveal fluid did not respond to intravitreal injections of antiangiogenic agents or steroids but resolved after renal transplantation. A temporary posttransplant visual improvement was associated with lessening of the subretinal drusenoid deposits demonstrated by multimodal imaging. The terminal vision deterioration was associated with amorphous, vitelliform-like material deposition and atrophic changes., Conclusion: This case may illustrate a resemblance in the renal glomerulus basement membrane and retinal pigment epithelium-Bruch membrane complex, because the authors observed deposits of excess monoclonal kappa chains manifesting as extracellular, proteinaceous aggregates on the basement membrane of the glomerulus, and striking, globular subretinal deposits that overlay a thickened retinal pigment epithelium-Bruch membrane complex. The ocular lesions' refractoriness to intravitreal treatments could be attributed to the fact that they represent proteinaceous aggregates similar to those documented in the glomeruli. This is the first report of generalized, large, subretinal drusenoid deposits and their course, as documented through multimodal imaging, paralleling the chronology of systemic changes in a patient with light chain deposition disease.

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 report the clinical features of bilateral Coats reaction in a patient with Bannayan-Zonana syndrome., Methods: The clinical course and retinal manifestations of a 21-year-old patient with Bannayan-Zonana syndrome and bilateral Coats reaction were studied. Retinal manifestations were documented with clinical examination, ultrawide-field color imaging, and ultrawide-field fluorescein angiography., Results: Best-corrected visual acuities were 20/25 OD and 20/25 OS. Anterior segment examinations were normal bilaterally. The inferotemporal quadrant of the left retina demonstrated retinal telangiectasias, exudation, and a neurosensory retinal detachment extending to the mid-periphery. Fluorescein angiography demonstrated bilateral peripheral nonperfusion and vascular staining. An epiretinal membrane also involved the left macula., Conclusion: Bannayan-Zonana syndrome is an autosomal-dominant, hamartomatous disease characterized by a mutation spectrum that involves genes responsible for vascular development. Retinal vascular abnormalities and exudation can be a manifestation of this syndrome.

Purpose: Drusenoid pigment epithelium detachment (DPED) is a known precursor to geographic atrophy in age-related macular degeneration (AMD). We sought histologic correlates for spectral-domain (SD) optical coherence tomography (OCT) signatures in DPED and determined the frequency and origin of these OCT signatures in a clinical cohort of DPED eyes., Design: Laboratory imaging and histologic comparison, and retrospective, observational cohort study., Participants: Four donor eyes with histopathologic diagnosis of AMD (2 with nonneovascular DPED and 2 with neovascular pigment epithelium detachment [PED]) and 49 eyes of 33 clinic patients with nonneovascular DPED more than 2 mm in diameter., Methods: Donor eyes underwent multimodal ex vivo imaging, including SD OCT, then processing for high-resolution histologic analysis. All clinic patients underwent SD OCT, near-infrared reflectance, and color photography., Main Outcome Measures: Histologic correlates for SD OCT signatures in DPED, estimate of coverage by different retinal pigment epithelium (RPE) phenotypes in the DPED surface; frequency and origin of histologically verified SD OCT signatures in a clinical cohort of DPED eyes, and comparisons of histologic features between neovascular PED and DPED resulting from AMD., Results: Intraretinal and subretinal hyperreflective foci as seen on SD OCT correlated to RPE cells on histologic examination. Hypertransmission of light below the RPE-basal lamina band correlated with dissociated RPE. Subretinal hyperreflective material resulting from acquired vitelliform lesions corresponded to regions of apically expelled RPE organelles. In the clinical cohort, all histologically verified reflectivity signatures were visible and quantifiable. The appearance of intraretinal hyperreflective foci was preceded by thickening of the RPE-basal lamina band. Compared with PEDs associated with neovascular AMD, DPEDs had different crystallization patterns, no lipid-filled cells, and thinner basal laminar deposits., Conclusions: Multiple RPE fates in AMD, including intraretinal cells that are highly prognostic for progression, can be followed and quantified reliably using eye-tracked serial SD OCT. This information may be particularly useful for obtaining an accurate timeline of incipient geographic atrophy in clinic populations and for quantifying anatomic end points and response to therapy in AMD clinical trials., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Idiopathic juxtafoveal retinal telangiectasis type 2 (macular telangiectasia type 2; MacTel) is a rare neurovascular degenerative retinal disease. To identify genetic susceptibility loci for MacTel, we performed a genome-wide association study (GWAS) with 476 cases and 1,733 controls of European ancestry. Genome-wide significant associations (P < 5 × 10 -8 ) were identified at three independent loci (rs73171800 at 5q14.3, P = 7.74 × 10 -17 ; rs715 at 2q34, P = 9.97 × 10 -14 ; rs477992 at 1p12, P = 2.60 × 10 -12 ) and then replicated (P < 0.01) in an independent cohort of 172 cases and 1,134 controls. The 5q14.3 locus is known to associate with variation in retinal vascular diameter, and the 2q34 and 1p12 loci have been implicated in the glycine/serine metabolic pathway. We subsequently found significant differences in blood serum levels of glycine (P = 4.04 × 10 -6 ) and serine (P = 2.48 × 10 -4 ) between MacTel cases and controls.

Purpose: Histologic details of progression routes to geographic atrophy (GA) in AMD are becoming available through optical coherence tomography (OCT). We studied the origins and evolution of an OCT signature called plateau in eyes with GA and suggested a histologic correlate., Methods: Serial eye-tracked OCT scans and multimodal imaging were acquired from eight eyes of seven patients with GA and plateau signatures over a mean follow-up of 7.7 years (range, 3.7-11.6). The histology of unrelated donor eyes with AMD was reviewed., Results: Drusenoid pigment epithelial detachment (PED) on OCT imaging progressed into wide-based mound-like signatures with flattened apices characterized by a hyporeflective yet heterogeneous interior and an overlying hyperreflective exterior, similar to outer retinal corrugations previously ascribed to persistent basal laminar deposit (BLamD) but larger. These new signatures are described as "plateaus." An initial increase of the PED volume and hyporeflectivity of its contents was followed by a decrease in PED volume and thinning of an overlying hyperreflective band attributable to the loss of the overlying RPE leaving persistent BLamD. Both imaging and histology revealed persistent BLamD with defects through which gliotic Müller cell processes pass., Conclusions: Plateaus can be traced back to drusenoid PEDs on OCT imaging. We hypothesize that during progressive RPE atrophy, Müller cell extension through focal defects in the residual persistent BLamD may contribute to the heterogeneous internal reflectivity of these entities. The role of Müller cell activation and extension in the pathogenesis of AMD should be explored in future studies.