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Purpose: To describe 6 cases of acute central serous chorioretinopathy (CSCR) and the response to laser treatment, focusing on the underlying pathogenic mechanism., Methods: Multimodal imaging from 6 eyes of 6 patients with acute and recurrent CSCR were reviewed, including fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT) at baseline and after laser therapy., Results: In 3 of the 6 cases with acute CSCR, the hyporeflective lucency sign was identified with cross-sectional and en face OCT and co-localized with an intense active inkblot retinal pigment epithelium (RPE) leak on FA. The development of this sign was suggestive of active leakage into the subretinal space displacing overlying subretinal hyperreflective material (SHRM) and suggestive of a reversal of RPE pump function. All 6 cases were treated with focal laser to directly target the intense leakage points with remarkable resolution of the fluid due to destruction of the RPE cells mediating reversal of pump function., Conclusions: Unlike chronic CSCR in which degenerative changes of the RPE lead to oozing of fluid into the subretinal space, in acute forms of CSCR including bullous CSCR, there are focal leaks of the RPE that actively drive fluid into the subretinal space suggestive of RPE pump reversal. We propose that pachychoroid disease causes increased hydrostatic pressure and increased resistance to the RPE pump, thereby triggering a reversal in pump function. Understanding this concept can have therapeutic implications., (Published by Elsevier Inc.)

Purpose: To describe a potential biomarker termed as inner choroidal fibrosis in cases of chronic central serous chorioretinopathy (CSCR) presenting to a tertiary referral center., Design: Observational case series., Methods: Five eyes of 4 patients with CSCR were noted to have a gray-white subretinal lesion in the macula, which was analyzed with multimodal imaging., Results: The lesions were hypofluorescent on autofluorescence, fundus fluorescein angiography, and indocyanine angiography. In all cases, a characteristic heterogeneous, hyperreflective lesion in the inner choroid was seen on optical coherence tomography (OCT), corresponding to the white subretinal lesions. The lesion was distinct from the adjacent choroid, with greater reflectivity and greater thickness than the adjacent compressed choroidal vasculature. The dilated outer pachyvessels were pushed outward or sideways in all cases. On OCT-angiography, the corresponding lesion showed flow void areas. We have termed this zone of inner choroidal hyperreflectivity "inner choroidal fibrosis." Upon analyzing the clinical course, 3 of the patients had a history of choroidal neovascularization. The contralateral eye in 2 of these 3 patients also developed choroidal neovascularization. Three of the eyes had an overlying focal choroidal excavation., Conclusions: We report a potential biomarker of CSCR termed "inner choroidal fibrosis" in 4 cases of chronic CSCR. It can best be identified on enhanced depth or swept source OCT as a region of heterogeneous hyperreflectivity in the inner choroid. This fibrosis could be regarded as a degenerative process secondary to more severe disease in the past., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To compare the differences among clinical, demographic, and multimodal imaging features of choroidal granulomas associated with tuberculosis and sarcoidosis., Design: Retrospective comparative case series., Methods: Clinical features and fundus imaging, including fluorescein and indocyanine green angiography and optical coherence tomography of patients with tuberculomas and sarcoid choroidal granulomas seen at 3 tertiary care centers, were reviewed. The differences among clinical appearances, including morphology of the lesions (size, shape, extent), vascularity, and multimodal imaging features, were compared. Repeated logistic regression measurements with a multilevel random effects model was used to assess characteristics of individual granulomas that could predict the underlying cause., Results: The study included 47 eyes of 38 patients (22 with tuberculomas and 16 with sarcoid granulomas; total of 138 granulomas). Patients with tuberculomas were significantly younger (33.8 ± 10.1 vs. 48.6 ± 14.3 years, respectively; P = .002), but no sex differences were observed. In comparison with sarcoid granulomas, tuberculomas were solitary (P <.001), intense yellow, lobulated, full thickness, and located in the perivascular region (all P <.001); they were also larger (16.01 ± 9.7 mm 2 vs. 2.7 ± 4.5 mm 2 , respectively; P <.001) and were vascularized (P <.001). Sarcoid granulomas were associated with retinal vasculitis (P = .003) and disc hyperfluorescence (P <.001). Logistic regression showed that multiple granulomas were associated with sarcoidosis (odds ratio [OR]: 3.5; 95% confidence interval: 1.8-6.9; P <.001). Granulomas larger than 6.45 mm 2 had the highest area under the receiver operating curves (0.94) for differentiating tuberculomas from sarcoid granulomas., Conclusions: Tuberculomas and sarcoid choroidal granulomas have various clinical and imaging features that help differentiate between the 2 entities with high predictability and can supplement immunological and radiological tests in a diagnosis., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: Exophytic juxtapapillary retinal capillary hemangioblastoma (JRCH) can be difficult to diagnose. We explore the value of multimodal imaging to aid in the diagnosis., Design: Retrospective case series., Methods: Medical records and multimodal imaging studies were reviewed on all patients diagnosed with RCH at Bascom Palmer Eye Institute, Miami, Florida, between January 2013 and December 2019. Patients with exophytic lesions within 2 mm of the disc were included. One patient from the Baylor College of Medicine, Houston, Texas was included. Patient demographics, referring diagnosis, history of von Hippel-Lindau syndrome, initial and last visual acuity, and treatments were recorded. Fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), OCT angiography, and B-scan images were reviewed., Results: Twelve patients were identified with exophytic JRCH. The mean age was 54 years (range 38-73 years). Five patients had von Hippel-Lindau syndrome. The most common referral diagnoses were choroidal neovascularization and neuroretinitis. Imaging features included nodular outer retinal thickening with shadowing and intra-/subretinal fluid on OCT, hypoautofluorescence on fundus autofluorescence, middle to outer retinal hypervascularity on OCT angiography, early hyperfluorescence with late leakage on fluorescein angiography, and lack of choroidal vascular lesion on indocyanine green angiography. Treatments included photodynamic therapy (6 patients), intravitreal anti-vascular endothelial growth factor therapy (6 patients), argon laser photocoagulation (2 patients), intravitreal or sub-Tenon's triamcinolone (3 patients), and observation (4 patients)., Conclusions: A key to the accurate diagnosis of exophytic JRCH is its intraretinal location, typically involving the outer retinal layers, which results in a clinical appearance that is distinct from the more common and easily recognizable endophytic RCH. Multimodal imaging can aid in ruling out choroidal neovascularization and disc edema by demonstrating an absence of involvement of those structures., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: To assess the relationship between demographics, clinical characteristics, and structural optical coherence tomography (OCT) findings and the development of sight-threatening macular complications (choroidal neovascularization [CNV], large areas of retinal pigment epithelium [RPE] atrophy, and cystoid macular degeneration [CMD]) in a cohort of eyes with "resolved" chronic central serous chorioretinopathy (CSC) at study baseline., Design: Retrospective cohort study., Methods: In this study, a total of 71 eyes with "resolved" (absence of subretinal fluid) chronic CSC at baseline and 36 months of regular follow-up examinations were retrospectively enrolled. Structural OCT scans were reviewed. Baseline OCT qualitative features reflecting distress of the neuroretina, RPE, or choroid were assessed and included ellipsoid zone discontinuity, outer nuclear layer (ONL) thinning; presence of hyper-reflective intraretinal foci; dome-shaped pigment epithelium detachment (PED); hyper-reflective flat, irregular PED; hyporeflective flat, irregular PED; and inner choroidal attenuation. OCT images obtained at follow-up visits were also reviewed for development of macular complications (CNV, large areas of RPE atrophy [at least 250 μm in diameter], and CMD). Main outcome measurements included incidence of macular complications and hazard ratio (HR) for demographics, clinical characteristics, and OCT risk factors., Results: At month 36, 20 eyes (28.2%) developed macular complications. Nine eyes (12.7%) displayed CNV, 9 eyes (12.7%) had large areas of RPE atrophy, and 2 eyes (2.8%) developed cystoid macular degeneration. The following factors were associated with an increased risk of development of CNV: intraretinal hyper-reflective foci had an HR of 11.58 (95% confidence interval [CI]: 1.10-37.24; P = .040); inner choroidal attenuation had an HR of 9.66 (95% CI: 1.07-22.34; P = .043); and the presence of macular complications in the fellow eye had an HR of 20.17 (95% CI: 1.34-39.41; P = .030). Factors associated with the development of RPE atrophy were also identified: ONL thinning had an HR of 13.47 (95% CI: 1.10-39.86; P = .042); dome-shaped PED had an HR of 21.40 (95% CI: 1.50-41.10; P = .031); and inner choroidal attenuation had an HR of 13.20 (95% CI: 1.07-39.32; P = .044)., Conclusions: OCT risk factors were identified for the development of macular complications in eyes with chronic CSC. Findings may help in the identification of high-risk patients., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To describe patients with late-onset pseudoxanthoma elasticum (PXE) associated with a likely hypomorphic ABCC6 variant., Design: Retrospective observational case series., Methods: Clinical evaluation, multimodal retinal imaging, genetic testing, and molecular modeling., Results: Three patients, in whom vision symptoms first arose at 80 years of age or later, showed age-related macular degeneration (AMD)-like fundus changes. However, features characteristic of PXE, including discrete angioid streaks and reduced fluorescence on late-phase indocyanine green angiography, prompted genetic testing which revealed the c.1171A>G variant in combination with a large deletion in the ABCC6 gene in each case. None of the patients had obvious skin changes or cardiovascular disease atypical for their age. Comparative molecular modeling supported the hypothesis that the c.1171A>G ABCC6 variant acted as a hypomorphic variant., Conclusions: Late-onset PXE extends the spectrum of ectopic calcification disorders caused by mutations in ABCC6 and may clinically be limited to the eye, mimicking AMD. Patients may be identified based on specific ocular changes, whereas skin and cardiovascular changes may remain ambiguous. The study provides evidence for a role for hypomorphic ABCC6 variants in the pathogenesis of PXE., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To assess whether chronic central serous chorioretinopathy (cCSC) patients without a complete resolution of subretinal fluid (SRF) after either half-dose photodynamic therapy (PDT) or high-density subthreshold micropulse laser (HSML) treatment may benefit from crossover treatment., Design: Multicenter prospective interventional case series., Methods: cCSC patients with persistent SRF at the final visit of the PLACE trial were included. Patients received crossover treatment with either half-dose PDT or HSML., Results: Thirty-two patients received PDT and 10 patients received HSML. At the first evaluation visit (6-8 weeks after treatment), 81% of patients in the PDT group had complete resolution of SRF, while none of the HSML-treated patients had complete resolution of SRF. At final visit (1 year after baseline), 78% (P = .030) and 67% (P = .109) of the patients, respectively, had a complete resolution of SRF. The mean retinal sensitivity in the PDT group increased from 21.7 dB (standard error [SE]: 0.9) to 23.4 dB (SE: 0.8) at evaluation visit 1 (P = .003), to 24.7dB (SE: 0.8) at final visit (P < .001), while there were no significant changes in the HSML group (23.7 dB [SE: 1.6] at baseline, 23.8 dB [SE: 1.4] at evaluation 1, and 23.3 dB [SE: 1.4] at final visit). The mean visual acuity and mean visual quality-of-life questionnaire score did not change significantly in both groups., Conclusions: Crossover to half-dose PDT after previous unsuccessful HSML treatment for cCSC may lead to improved anatomic and functional endpoints, while crossover to HSML after half-dose PDT does not seem to significantly affect these endpoints., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To investigate the features of neovascularization (NV) in eyes with neovascular age-related macular degeneration using optical coherence tomography angiography (OCTA) according to the treatment interval of intravitreal aflibercept injection (IVI)., Design: Retrospective, interventional, comparative case series., Methods: Patients with type 1 NV treated with the "pro re nata" regimen after 3 loading IVI were classified into 2 groups based on the numbers of treatments during 12 months, specifically a stable group who required fewer than 2 injections and an unstable group who required more than 3 injections. Quantitative features of OCTA including NV area, NV length, NV density, endpoint density (open-ended vessels per unit length), junction density (vessel junction per unit length), lacunarity, and largest vessel caliber were compared between the 2 groups., Results: Among 71 eyes, 38 and 33 eyes were classified into the stable and unstable groups, respectively. The unstable group had higher endpoint densities (stable vs unstable: 2.72 vs 3.18; P = .03) and higher levels of lacunarity (0.177 vs 0.211; P = .028). The area, density, length of NV, junction density, and largest vessel caliber were not different between the 2 groups (P = .057, P = .184, P = .062, P = .160, and P = .473, respectively). Endpoint density was correlated with the unstable group in both univariate and multivariate analyses (P = .004, P = .002, respectively). A predictive model with an endpoint index demonstrated a sensitivity of 93.75% and a negative predictive value of 89.47% for the unstable group., Conclusions: The characteristics of NV in eyes of exudative age-related macular degeneration with type 1 NV were different according to treatment requirements. Identifying the features of NV on OCTA might be helpful for predicting clinical outcomes and optimal treatment intervals., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To characterize temporal trends and regional variance in retinal imaging utilization in the United States Medicare fee-for-service population from 2012-2016., Design: Cross-sectional, retrospective database analysis., Methods: This study addresses office or operating-room based retinal imaging. Our study population included retina specialists, defined as ophthalmologists performing either intravitreal anti-vascular endothelial growth factor injections or posterior segment laser photocoagulation and no neodymium-doped yttrium aluminum garnet laser capsulotomy. We recorded fundus photography, optical coherence tomography (OCT), intravenous fluorescein angiography (IVFA), indocyanine-green angiography, and ophthalmic ultrasound (B-scan) billed in the Medicare fee-for-service population from 2012-2016. Imaging obtained on any platform or device was eligible for inclusion (eg, posterior pole imaging vs ultrawidefield imaging). The main outcome measure was the relative utilization of retinal imaging modalities., Results: National relative utilization of OCT increased from 61.5% in 2012 to 70.5% in 2016 (P < .001), while IVFA fell from 20.9% to 15.1% over the same interval (P < .001). Fundus photography decreased from 14.6% in 2012 to 11.7% in 2016 (P < .001). By 2016, the Midwest region had the highest relative utilization of OCT (75.2%) and lowest of IVFA (12.0%), while the West region had the lowest OCT (68.4%) and highest IVFA (17.0%)., Conclusions: Among retina specialists, OCT usage increased while the utilization of fundus photography and IVFA has declined. The Midwest region had the highest utilization of OCT and lowest of IVFA., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: The aim of this study is to investigate temporal trends in multifocal ERG (mfERG) parameters and analyze their relationships with anatomic and functional markers in patients with birdshot chorioretinopathy (BSCR)., Design: Prospective observational case series., Methods: Sixteen BSCR patients were include and underwent 2 standardized follow-up (FU) visits within 5 years following a baseline examination, including mfERG, visual acuity (VA), visual field (VF), Lanthony desaturated panel D-15 test for color vision, quality of life (QoL), fluorescein and indocyanine green angiography, and optical coherence tomography (OCT)., Results: A significant trend toward a decrease in absolute N1 amplitude values was observed over the follow-up period (P < .001) while N1 implicit time remained unchanged. In contrast, P1 amplitude decreased (P < .001) and P1 implicit time increased (P < .001) over the same period. No significant temporal change was found for VA, color vision score, foveal threshold, mean deviation of VF, and QoL. After adjusting for time to FU, increasing N1 and P1 IT were both associated with decreasing values of logMAR, foveal threshold, and QoL and with increasing color vision score and mean deviation of VF. A significant relationship was observed between decreasing P1 amplitude values and increasing mean deviation of VF. Lower absolute values of N1 amplitude were associated with venous vasculitis, whereas lower P1 amplitude values correlated with alteration of the outer retina in OCT., Conclusions: Progressive deterioration in mfERG during a 5-year period is detected in BSCR, whereas classical functional test results were unchanged. This study suggests a better sensitivity of mfERG in monitoring the retinal function of BSCR patients., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To analyze optical coherence tomography angiography (OCTA) quantitative features in patients affected by new-onset choroidal neovascularization (CNV) in 1 eye and early/intermediate age-related macular degeneration (AMD) in the fellow eye., Design: Case-control study., Methods: Setting: Clinical practice., Study Population: Thirty patients and 30 age-matched controls., Observation Procedures: Both cohorts underwent complete ophthalmologic examination, including best-corrected visual acuity (BCVA) measurement, biomicroscopy, fluorescein angiography (FA) and indocyanine green angiography (ICGA) examination, optical coherence tomography, and OCTA scans. The 1-way a test with Bonferroni correction was used to assess statistical significance and Tau Kendall's correlation analysis was performed., Main Outcome Measures: BCVA, choroidal thickness, vessel density, vessel tortuosity, vessel dispersion. and vessel rarefaction., Results: Mean BCVA was 20/32 for CNV eyes and 20/20 for both fellow and control eyes. Choroidal thickness was 190.33 ± 63.98 μm for CNV eyes, 216.83 ± 50.31 μm for fellow eyes, and 310.52 ± 27.13 μm for controls. The quantitative analysis of retinal vessels revealed significant alterations, especially in the deep capillary plexus and radial peripapillary capillaries, both in CNV and in fellow eyes, compared with controls. In particular, decreased vessel density and tortuosity and increased dispersion and rarefaction were found. Several significant correlations were also found among the quantitative parameters adopted., Conclusions: New postprocessing OCTA parameters are able to detect deep retinal vascular alterations quantitatively, in both CNV-affected and fellow eyes of patients with new-onset CNV. Further investigations are warranted in order to explore the validity of these new approaches on follow-up., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To quantitatively compare near-infrared autofluorescence (NIR-AF) and short-wave autofluorescence (SW-AF) as imaging modalities used to monitor retinitis pigmentosa (RP) disease progression, measured as a function of hyperautofluorescent ring constriction over time., Design: Retrospective cohort study., Methods: NIR-AF and SW-AF images were acquired from 22 participants (44 eyes) at 2 clinic visits separated by an average of 2 years. On the images from each modality, the horizontal and vertical diameters and area of the hyperautofluorescent rings were measured twice, 2 weeks apart. A progression rate for each parameter was obtained. Descriptive and comparative statistics were calculated to analyze these parameters and their respective progression rates., Results: At both visits, the hyperautofluorescent ring exhibited a larger horizontal diameter (both visits: P < .001), vertical diameter (visit 1: P < .001, visit 2: P = .040), and ring area (visit 1: P = .001, visit 2: P = .011) in SW-AF vs NIR-AF images. In SW-AF, the horizontal diameter, vertical diameter, and ring area decreased yearly by 168 ± 204 μm, 131 ± 159 μm, and 0.7 ± 1.1 mm 2 , respectively, while in NIR-AF, they decreased by 151 ± 156 μm, 135 ± 190 μm, and 0.7 ± 1.0 mm 2 . No difference was observed in these rates between SW-AF and NIR-AF. Similar results were observed in the left eye., Conclusions: In SW-AF and NIR-AF images, similar rates of RP disease progression are observed. As such, NIR-AF may confer more advantages as the primary tool for tracking disease progression over the commonly used SW-AF, given the increased patient comfort and cooperation during imaging., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the type 1 choroidal neovascularization (CNV) incidence and associated factors in eyes with central serous chorioretinopathy (CSC)., Design: Retrospective case series., Methods: Records of 363 eyes (324 patients) with CSC were reviewed. Age, sex, CSC type, choroidal vascular hyperpermeability (CVH), best-corrected visual acuity (BCVA), subfoveal choroidal thickness (SCT), and systemic hypertension (HT) were assessed and compared between subjects with and without neovascular CSC., Results: We identified 219 and 144 eyes with chronic and acute CSC, respectively. The mean participant age was 55.2 ± 12.0 years, and 58 (15.6%) eyes had neovascular CSC. Age (no CNV: 54.8 ± 12.1 years, CNV: 57.3 ± 10.9 years; P = .118) and SCT (no CNV: 388.0 ± 104.5 μm, CNV: 377.4 ± 108.9 μm; P = .487) were comparable between eyes with and without CNV. However, BCVA (logarithm of the minimum angle of resolution) was significantly worse in subjects with CNV (0.28 ± 0.33 [20/38] vs 0.15 ± 0.29 [20/28]; P = .014). Neovascular CSC occurred more often in women (72 [23.6%] vs 20 [34.5%], P = .099) and in cases of chronic CSC (171 [56.1%] vs 48 [82.8%], P < .001), CVH (205 [67.2%] vs 58 [100%], P < .001), and HT (91 [29.8%] vs 24 [41.4%], P = .092). Chronic CSC (P = .001), female sex (P = .075), and poor BCVA (P = .091) were associated with neovascular CSC (multiple regression)., Conclusions: Chronic CSC, female sex, CVH, and poor BCVA are risk factors for CNV in eyes with CSC., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To describe abnormalities in choroidal and retinal vasculature associated with Val30Met familial transthyretin amyloidosis (V30M-FTA) using fluorescein and indocyanine green (ICG) angiography., Design: Prospective, cross-sectional study., Methods: This study was conducted at the French National Reference Center for FTA. We included 18 consecutive genetically confirmed V30M-FTA patients (36 eyes) who underwent complete neurologic examination, including staging with polyneuropathy disability (PND) score, and complete ophthalmic evaluation, including staging of intraocular amyloid deposits and fluorescein and ICG angiograms (ICG-A). The grading of choroidal and retinal angiopathy, and their association with neurologic functional impairment, were the main outcome measures., Results: Eleven men and 7 women, mean age 61.6 ± 12.1 years, were included. Retinal amyloid angiopathy (RAA) was detected in 24 eyes (92%) of 13 patients, with microaneurysms, retinal hemorrhages, and retinal ischemia of variable extent. Three patients (5 eyes) had neovascular glaucoma and 2 (2 eyes) had preretinal neovascularization. ICG-A indicated choroidal amyloid angiopathy (CAA) in all patients, with 3 distinct patterns-diffuse (9/18 patients), focal (5/18 patients), or punctiform (4/18 patients)-based on the extent of late hypercyanescence along the choroidal arteries. PND scores were significantly higher in patients with diffuse CAA (firework pattern) compared to those with limited CAA (focal and punctiform patterns) (2.89 vs 1.78, P = .045)., Conclusion: RAA is a frequent and severe complication of V30M-FTA that may lead to anterior and posterior segment neovascularization. CAA was detected in all patients, with a late hypercyanescent delineation of the choroidal arterial vasculature, which was more extensive with increased disease severity., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To describe the clinical characteristics, therapies, visual outcomes, and prognoses of patients with retinal vasculitis associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV)., Design: Retrospective case series., Methods: Patients diagnosed with retinal vasculitis associated with AAV and at least 6 months of follow-up were included. Demographic data, systemic and ocular features, best-corrected visual acuity at the initial visit and latest visit, fluorescein angiography (FA) and indocyanine green angiography (ICGA) findings, therapy regimen, and outcome were collected from the Massachusetts Eye Research and Surgery Institution (MERSI) database from 2006 to 2017., Results: Fourteen patients (22 eyes) were identified. Twelve had granulomatosis with polyangiitis (GPA) and 1 each had microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). FA showed that AAV affected small-to-medium-size retinal vessels. Seven cases (50%) had both vein/venule and artery/arteriole involvement. Four cases co-presented with choroidal vasculitis. All of them failed various immunomodulatory therapies prior to referral to MERSI. Six patients received rituximab plus prednisone as their final therapy and 5 of them achieved remission. Four patients who failed cyclophosphamide previously were induced into remission by rituximab. Patients were followed for 33.4 ± 25.5 (range 6-84) months. Nine of 14 patients (64.3%) achieved remission at their latest visit. Seventeen of 22 eyes (77.3%) met the criteria for a good (≥20/40) visual outcome., Conclusion: The majority of patients enjoyed a good visual outcome and achieved remission after aggressive treatment. Rituximab should be considered as an initial treatment for patients with refractory retinal vasculitis associated with AAV., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To describe the characteristics and natural history of quiescent choroidal neovascularization (CNV) in geographic atrophy (GA) secondary to nonexudative age-related macular degeneration (AMD) through multimodal imaging., Design: Retrospective observational case series., Methods: Patients diagnosed with quiescent CNV were analyzed in 2 high-volume referral centers. Imaging features obtained using fluorescein angiography (FA), indocyanine green angiography (ICGA), structural optical coherence tomography (OCT), and OCT angiography (OCT-A) were noted at first presentation and during the study period., Results: Nineteen eyes of 19 patients were included. Mean (+SD) follow-up was 45.7 ± 14.7 months. Quiescent CNV appeared as an ill-defined hyperfluorescent lesion without leakage or pooling of dye in the late phase of FA. On ICGA, quiescent CNV appeared as a distinct area of hyperfluorescence (vascular network) in early to intermediate frames and as a hyperfluorescent plaque in the late frame (late plaque). OCT-A revealed a flow signal beneath the small irregular elevation of the retinal pigment epithelium at the site of the quiescent CNV visualized by structural OCT. During the study period, 5 of the 19 CNV patients developed exudation. The remainder showed specific alterations in both structural OCT and OCT-A imaging. At last follow-up, 92% of the quiescent CNV seemed to cover the area spared from atrophy., Conclusions: The characteristics of the quiescent CNVs were very similar to those already described for intermediate AMD, although they had several specific features in the context of GA., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To investigate long-term efficacy and prognostic factors of half-dose photodynamic therapy (PDT) in chronic central serous chorioretinopathy (CSCR)., Design: Retrospective multicenter interventional case series., Methods: Patients with chronic CSCR undergoing half-dose PDT between 2005 and 2011 were reviewed. Main outcome measures included resolution of serous retinal detachment (SRD) with single PDT, change in best-corrected visual acuities (BCVAs), and recurrence rate of CSCR at 36 months after PDT. Prognostic factors of visual outcome and recurrence of CSCR after PDT were identified with multivariate regression analysis., Results: A total of 136 eyes of 123 patients were followed up for 57.7 ± 16.2 months. At 36 months after PDT, 132 eyes (97.1%) achieved complete resolution of SRD with single PDT and 4 eyes (2.9%) had CSCR recurrence. The mean logMAR BCVA improved from 0.36 ± 0.29 (Snellen equivalent 20/46; range: 0.1-1.2) at baseline to 0.15 ± 0.23 at 36 months (Snellen equivalent 20/28; range: 0.1-1.5; P < .001) and 0.16 ± 0.24 (Snellen equivalent: 20/29; range: 0.1-1.5; P < .001) at final follow-up. Forty-four eyes (32.4%) had ≥3 lines of BCVA gain while 5 eyes (3.7%) had ≥3 lines of BCVA loss at 36 months after PDT. Nine eyes (6.6%) developed CSCR recurrence at final follow-up. Baseline BCVA was significantly associated with the BCVA (P = .009) and the improvement in BCVA (P < .001) at final follow-up. History of bilateral CSCR was significantly associated with CSCR recurrence at final follow-up (P = .036; odds ratio = 15.84, 95% confidence interval = 1.20-208.32). Eight eyes (5.9%) had complications related to PDT., Conclusions: Chronic CSCR patients treated with half-dose PDT can achieve long-term stable visual acuity and resolution of SRD. Patients with chronic CSCR are recommended to undergo half-dose PDT before they have significant visual deterioration. Patients with bilateral CSCR are more likely to develop CSCR recurrence after half-dose PDT., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To investigate the incidence rate of massive submacular hemorrhage (SMH) in patients with polypoidal choroidal vasculopathy (PCV) and analyze the associated risk factors., Design: Retrospective cohort study., Methods: Patients diagnosed with PCV from May 2003 to May 2014 were included. Two hundred forty-five eyes of 245 patients were enrolled. The time between the initial visit to the clinic with subjective visual symptoms and the date of massive SMH was recorded. SMH larger than 4 disc diameters was defined as massive SMH. Age; hypertension; visual acuity (VA); indocyanine green angiography findings, including the greatest linear dimension, largest polyp size, and PCV type (cluster vs non-cluster); and treatment methods were reviewed for risk factor analysis using Kaplan-Meier survival and Cox regression analyses., Results: The incidence rate of massive SMH within 1 year after the initial visit was 2.45%. Massive SMH occurred within 3, 5, and 10 years after the first visit in 6.17%, 11.09%, and 29.85% of patients, respectively. Cox regression analysis revealed that the cluster type of PCV was significantly associated with massive SMH (hazard ratio [HR], 3.418; P = .003). Photodynamic therapy followed by anti-vascular endothelial growth factor injection lowered the risk of massive SMH (HR = .242; P = .047]. Final VA in eyes with massive SMH was significantly lower than that in patients without massive SMH (1.34 ± 0.66 vs 0.63 ± 0.53 logMAR; P < .001)., Conclusions: Patients with PCV who develop massive SMH experience severe vision loss. The incidence rate of massive SMH in PCV increases with time. The cluster type of polyp in PCV is a significant risk factor for massive SMH., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To describe the optical coherence tomography angiography (OCT-A) features of treatment-naïve quiescent choroidal neovascularization (CNV) secondary to age-related macular degeneration, and to estimate the detection rate for neovascularization by means of OCT-A., Design: Diagnostic tool validity assessment., Methods: Treatment-naïve quiescent CNV were identified from a pool of patients at 2 retina referral centers. Patients underwent a complete ophthalmologic examination including fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and OCT-A. Detection rates of CNV by means of OCT-A were estimated with a second cohort of patients without CNV (negative controls)., Results: Twenty-two eyes of 20 consecutive patients with quiescent CNV were included. In 4 out of 22 eyes it was not possible to classify the CNV "shape," "core," "margin," and "location," either because the vascular network was not clearly shown (3 cases) or because it was not visible at all (1 case). CNV shape on OCT-A was rated as circular in 8 eyes and irregular in 10 eyes. CNV core was visible in 2 eyes. CNV margin was considered as well defined in 15 eyes and poorly defined in 3 eyes. CNV margin showed small loops in 9 eyes and large loops in the other 6 eyes. CNV location was foveal-sparing in 12 eyes. Sensitivity and specificity of quiescent CNV detection by OCT-A turned out to be 81.8% and 100%, respectively., Conclusions: OCT-A allows the clinician to noninvasively identify treatment-naïve quiescent CNV and may be considered as a useful tool to guide the frequency of return visits and, possibly, make treatment decisions., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To develop a new method to quantify the choroidal vessel density by binarizing ultra-wide-field indocyanine green angiography (ICGA) images and determine whether values are altered in diseased eyes., Design: Reliability and validity analysis., Methods: Ultra-wide-field fluorescein angiography (FA) and ICGA images were obtained using an ultra-wide-field imaging device (Optos California ultra-wide-field imaging device; Dunfermline, Scotland, UK) in 11 eyes of 11 patients without chorioretinal diseases. The angiographic signals of the choroidal vessels were determined by subtracting those of the retinal vasculature and optic disc on the FA images from the ICGA images, binarized by Niblack's method, and the choroidal vessel density calculated. Reproducibility of the method was assessed by calculating the coefficient of variance, coefficient of repeatability, and intraclass correlation coefficient. The relationships between age, spherical equivalent refractive error (SERE), and intraocular pressure and the vasculature density were assessed. To investigate possible impacts of chorioretinal diseases on the vasculature density, 10 eyes of 7 patients with central serous chorioretinopathy (CSC) were compared with the 11 control eyes., Results: Choroidal vessels were contrasted by binarizing ICGA images. The method to quantify the choroidal vessel density showed high reproducibility. The SERE was correlated significantly (r = 0.573, P < .05) with the vasculature density. In the 11 control eyes, the vasculature density was 34.26% ± 0.77% in the entire area, 31.37% ± 0.97% in the superior portion, 36.98% ± 0.88% in the inferior portion, 37.01% ± 1.44% in the posterior portion, and 34.17% ± 0.77% in the peripheral portion. In eyes with CSC, the density was significantly (P < .05) higher: 36.46% ± 0.49%, 34.02% ± 0.97%, 38.65% ± 0.27%, 41.04% ± 0.82%, and 36.36% ± 0.51%, respectively., Conclusions: Binarization of ultra-wide-field ICGA images enabled quantification of the choroidal vessel density, which was altered in eyes with CSC. This method of measuring the choroidal vessel density may provide new insights into diagnosing and treating chorioretinal diseases., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To compare the effectiveness of intravitreal injection of aflibercept and ranibizumab for patients with polypoidal choroidal vasculopathy (PCV)., Design: Retrospective, interventional case series., Methods: Ninety-eight treatment-naïve PCV eyes were included. The eyes had received intravitreal aflibercept or ranibizumab injections. All patients were treated using an initial series of 3 monthly loading injections, followed by further injections as required. The visual and anatomic outcomes of treatment were evaluated after 12 months., Results: The mean best-corrected visual acuity (BCVA) in the aflibercept-treated group (38 eyes), expressed as the logarithm of the minimal angle of resolution (logMAR), had decreased from 0.63 ± 0.49 to 0.44 ± 0.37 after 12 months of treatment (P = .012). Similarly, in the ranibizumab-treated group (60 eyes), the BCVA had decreased from 0.66 ± 0.43 to 0.49 ± 0.36 (P = .018). The central foveal thickness had decreased in the aflibercept-treated group from 396 ± 167 μm to 212 ± 144 μm, and in the ranibizumab-treated group from 402 ± 198 μm to 240 ± 183 μm (P < .001 in both cases). However, there was no significant difference between the groups with regard to either BCVA improvement or decrease in central foveal thickness. Polyp regression was significantly more frequent in the aflibercept-treated group, occurring in 39.5% of eyes, than in the ranibizumab-treated group (21.6% of eyes; P = .007)., Conclusions: In PCV patients, the visual acuity improvement achieved after 12 months of intravitreal aflibercept did not differ significantly from that achieved using intravitreal ranibizumab. However, aflibercept treatment more often led to polyp regression than did treatment using ranibizumab., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To identify changes in short-wavelength automated perimetry patterns and parameters between the active and inactive states., Design: Retrospective cohort study with age-matched, normal controls., Methods: setting: Private tertiary referral center., Study Population: Seventy-five eyes of 38 patients with active birdshot retinochoroidopathy and 37 eyes of 37 historical normal controls., Intervention: Thirty-seven patients received immunomodulatory therapy. A fluocinolone acetonide intravitreal implant (Retisert) was implanted in both eyes of 1 patient as an initial treatment., Main Outcome Measures: Changes in short-wavelength automated perimetry total deviation scores, pattern deviation scores, mean deviation, and pattern standard deviation in the active phase and the remission state., Results: Mean deviation (P = .006), pattern standard deviation (P = .001), total deviation score (P = .002), and pattern deviation score (P = .007) were significantly different from the active phase to the remission state. The length of time required to achieve remission did not significantly affect the changes in mean deviation (regression coefficient = 0.01; P = .92), pattern standard deviation (regression coefficient = 0.01; P = .87), total deviation score (regression coefficient = -0.1; P = .32), or pattern deviation score (regression coefficient = 0.1; P = .36) from the active phase to the remission state., Conclusion: There was significant improvement in total deviation score, pattern deviation score, mean deviation, and pattern standard deviation on short-wavelength automated perimetry as patients achieved remission. Short-wavelength automated perimetry appears to be a useful and complementary modality in monitoring disease activity in birdshot retinochoroidopathy., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the efficacy of pars plana vitrectomy with infracyanine green (IFCG)-assisted internal limiting membrane peeling for the treatment of idiopathic macular hole., Design: Prospective, noncomparative interventional case series., Methods: Thirty-eight consecutive eyes of 35 patients with idiopathic macular hole were included in the study. Patients underwent early treatment diabetic retinopathy (ETDRS) visual acuity examination, dilated ophthalmoscopy, and optical coherence tomography before treatment and during follow-up. Fluorescein angiography was done in selected cases. Patients underwent a three-port pars plana vitrectomy with complete posterior hyaloid and epiretinal membrane removal. The internal limiting membrane (ILM) was stained with 0.5 cc of IFCG (0.5 mg/ml, 308 mOsm) and peeled up to the vascular arcades. Perfluoropropane gas (C(3)F(8)) 10% was used as tamponade., Results: Mean follow-up duration was 10 +/- 5 months (range, 3 to 24 months). Six eyes had stage 2 macular hole, 15 eyes stage 3, and 16 eyes stage 4. Overall, 37 of 38 macular holes closed after a single surgery. Median visual acuity was 20/100 (range, 20/400 to 20/50) before surgery and 20/50 (range, 20/640 to 20/25) after surgery. Visual acuity after surgery was 20/50 or better in 24 of 38 (63.1%) eyes. Twenty-five (65.8%) eyes improved by 2 or more lines, nine (23.7%) eyes were stable, and four (10.5%) eyes worsened by 2 or more lines., Conclusions: This study suggests that IFCG (0.05%) effectively stains the ILM with apparent safety, and that IFCG-assisted peeling of the ILM may be useful in the treatment of idiopathic macular hole.

Purpose: To determine the effective minimal concentration of indocyanine green (ICG) for staining the internal limiting membrane (ILM) under air in macular surgeries and to report the clinical outcome of these patients., Design: Prospective, randomized clinical trial., Methods: Consecutive cases of macular hole (17 cases) and epiretinal membrane (ERM) (11 cases) undergoing primary surgery with ICG-stained ILM peeling were randomized to receive one of the three concentrations (mg/ml) of ICG (1): 0.25, (2) 0.5, and (3) 1.25. The number of ICG injections, visual quality of the stained ILM, and time used for ILM peeling were recorded. Internal limiting membrane specimens were subsequently examined under electron microscopy. Preoperative and postoperative clinical data with fluorescein angiography were recorded., Results: There was a significantly smaller number of eyes with poor ILM staining in the 1.25-mg/ml group compared with the 0.25- to 0.5-mg/ml group (Fisher exact test, P =.04). The mean time required for ILM peeling was 4.2 minutes (range, 2.0-8.1 minutes). There was no significant difference in the time required for ILM peeling among the three concentration groups (one-way analysis of variance, P =.18) or between the macular hole and ERM group (two-tailed t test, P =.34). No ICG toxicity was found clinically or angiographically, except in one suspected case with ERM formation at the edge of ILM peeling. Electron microscopy of ILM specimens did not reveal any retinal elements., Conclusions: 1.25-mg/ml ICG under air stains the macular ILM consistently well for its removal in macular surgeries. The safety of ICG-stained ILM peeling needs further evaluation.

Purpose: To demonstrate possible retinal damage caused by indocyanine green dye for staining of the internal limiting membrane in surgery for idiopathic macular hole., Methods: Consecutive interventional case series. We report on the ultrastructural findings of the internal limiting membrane in 10 eyes of 10 patients., Results: All specimens revealed not only the internal limiting membrane, but also some small amounts of retinal elements, such as the plasma membrane of Müller cells and other undetermined structures. This indicates a cleavage plane not exactly at the inner undulating aspect of the internal limiting membrane but within the innermost retinal layers., Conclusion: Dilutions of indocyanine green as recommended in the literature may alter the structure of the retina to some degree. Possible factors responsible for this inadvertent action may include (1) concentration, (2) osmolarity pH, (3) time of tissue contact, and (4) mechanical factors from more forceful traction during peeling. Although functional consequences of these findings remain unclear as yet, factors that may induce damage to the innermost retina should be elucidated.

Purpose: We evaluated the safety of indocyanine green for use in fundus angiography., Methods: We sent a questionnaire concerning complications of indocyanine green to 32 institutions in Japan, which were selected on the basis of the client list from the Topcon Company, which manufactures the indocyanine green fundus camera., Results: Ophthalmologists at 15 institutions responded, reporting a total of 3,774 indocyanine green angiograms performed on 2,820 patients between June 1984 and September 1992. Before angiography, intradermal or intravenous indocyanine green testing, or both was performed at 13 of 15 institutions. For three patients, the decision was made not to proceed with angiography after positive preangiographic testing. The dosage of indocyanine green used for angiography varied from 25 to 75 mg, depending upon the institution. There were 13 cases of adverse reactions (0.34%), ten of which were mild reactions such as nausea, exanthema, urtication, itchiness, and urgency to defecate, and did not require treatment. Also recorded were one case of pain of the vein, which required treatment, and two cases of hypotension. The two hypotensive patients required treatment for shock., Conclusions: A comparison of frequency of adverse reactions to indocyanine green with the previously reported frequency of such reactions to fluorescein sodium indicated that indocyanine green is a safe as fluorescein for use in angiography.