Intravenous high-dose methotrexate based systemic therapy in the treatment of isolated primary vitreoretinal lymphoma: An LOC network study.

The treatment of primary vitreoretinal lymphoma (PVRL) remains controversial regarding the use of local, systemic, or combined treatments. The aim of this study was to analyze the efficacy and toxicity of intravenous high-dose methotrexate (IV HD-MTX) based systemic therapy in a uniformly treated population of PVRL patients. From a nationwide French database, we retrospectively selected 59 patients (median age: 70 years, median Karnofsky Performance Status: 90%) with isolated PVRL at diagnosis who received first-line treatment with HD-MTX between 2011 and 2018. 8/59 patients also received a local treatment. No deaths or premature discontinuations of MTX due to toxicity were reported. A complete response was obtained in 40/57 patients after chemotherapy. Before treatment, IL-10 was elevated in the aqueous humor (AH) or in the vitreous in 89% of patients. After treatment, AH IL-10 was undetectable in 87% of patients with a CR/uCR/PR and detectable in 92% of patients with PD/SD. After a median follow-up of 61 months, 42/59 (71%) patients had relapsed, including 29 isolated ocular relapses as the first relapse and a total of 22 brain relapses. The median overall survival, progression-free survival, ocular-free survival and brain-free survival were 75, 18, 29 and 73 months, respectively. IV HD-MTX based systemic therapy as a first-line treatment for isolated PVRL is feasible, with acceptable toxicity, even in an elderly population. This strategy seems efficient to prevent brain relapse with prolonged overall survival. However, the ocular relapse rate remains high. New approaches are needed to improve local control of this disease, and ocular assessment could be completed by monitoring AH IL-10.

INCIDENCE, PROGRESSION, AND RISK FACTORS OF EPIRETINAL MEMBRANES IN THE ELDERLY.

PURPOSE: To determine the incidence, progression rate, and risk factors for epiretinal membranes (ERMs) in a population of French elderly subjects. METHODS: Seven hundred and thirty-five eyes of 413 participants of the population-based ALIENOR study were included between 2009 and 2010. Participants were re-evaluated every 2 years between 2011 and 2017 (i.e., three follow-up visits). The mean duration of follow-up was 5.09 years (SD, 1.8; range, 0.99-7.85). Epiretinal membranes were graded from spectral-domain optical coherence tomography images according to a standardized classification. RESULTS: The incidence rate of ERMs was 9.42 per 100 eye-years (95% confidence interval, 7.36-12.05), corresponding to a 5-year cumulative incidence of 37.6%. In the final multivariable model, ERM incidence was significantly associated with vitreomacular or vitreopapillary adhesion at baseline (hazard ratio, 2.15; P = 0.02), choroidal thinning (hazard ratio, 1.04 per 10 µm decrease; P = 0.02), ERM in the contralateral eye (P = 0.02), and smoking after 85 years (hazard ratio, 6.01; P = 0.003). The 5-year cumulative progression rate was 6.9%. CONCLUSION: Incidence of ERMs was higher in our population than that previously reported, most probably because of the use of spectral-domain optical coherence tomography images. Incident ERMs were found to be associated with vitreous adhesion at baseline, choroidal thinning, ERM in the contralateral eye, and smoking after 85 years.

CHOROIDAL THICKNESS, VASCULAR FACTORS, AND AGE-RELATED MACULAR DEGENERATION: The ALIENOR Study.

PURPOSE: To study the associations of subfoveal choroidal thickness with vascular risk factors and age-related macular degeneration. METHODS: Two hundred sixty-one participants of the Alienor study had gradable enhanced-depth imaging optical coherence tomography scans of the macula and available data on vascular and genetic risk factors (assessed through face-to-face interview and fasting blood samples) and age-related macular degeneration status (assessed from retinal photographs and optical coherence tomography). Subfoveal choroidal thickness was measured manually on one horizontal scan passing through the fovea. RESULTS: In a multivariate mixed linear model, subfoveal choroidal thickness was independently associated with age greater than 80 years (-21.77 µm, P = 0.02), axial length (-21.77 µm, P < 0.0001), heavy smoking (≥20 pack-years: -24.89 µm, P = 0.05), fasting blood glucose higher than 7 mmol/L (-53.17 µm, P = 0.02), and lipid-lowering treatment (+18.23, P = 0.047). After multivariate adjustment for age, sex, axial length, and vascular and genetic risk factors, subfoveal choroidal thickness was thinner in eyes with central hyperpigmentation (-45.39 µm, P = 0.006), central hypopigmentation (-44.99 µm, P = 0.001), and central pigmentary abnormalities (-44.50 µm, P = 0.001), but not in eyes with late age-related macular degeneration (-18.05 µm, P = 0.33) or soft drusen. CONCLUSION: These findings indicate a relationship between vascular risk factors and choroidal thinning and suggest an early involvement of the choroid in the pathogenesis of age-related macular degeneration.

Clinicopathologic Correlation of Anti-Vascular Endothelial Growth Factor-Treated Type 3 Neovascularization in Age-Related Macular Degeneration.

PURPOSE: To correlate histologic results with previously recorded multimodal imaging results from a patient with type 3 neovascularization secondary to age-related macular degeneration (AMD). DESIGN: Case study, clinical imaging, laboratory imaging, and eye-tracked clinicopathologic correlation. PARTICIPANT: An 86-year-old white woman with type 3 neovascularization secondary to AMD treated with 6 intravitreal injections of bevacizumab. METHODS: Multimodal retinal imaging at each clinic visit was correlated with ex vivo and high-resolution histologic images of the preserved donor eye. Clinical imaging included serial near-infrared reflectance and eye-tracked spectral-domain OCT. Eye tracking, applied to the donor eye, enabled identification of histologic features corresponding to clinical OCT signatures. MAIN OUTCOME MEASURES: Histologic correlates for clinical OCT signatures were sought, including reflectivity of the vascular complex, intraretinal hyperreflective foci and intraretinal cellularity, analysis of the topography of pathologic features, and evaluation of the sub-retinal pigment epithelium (RPE) plus basal lamina (BL) space. RESULTS: Clinical imaging showed a deep neovascular lesion in close relationship with a mixed serous and drusenoid pigment epithelium detachment (PED), characteristic of type 3 neovascularization. Antiangiogenic therapy achieved a complete resolution of exudation. The PED progressively flattened with each treatment, leaving a persistent triangular hyperreflectivity in the outer retina. This persistent deep lesion histologically correlated with a vascular complex implanted into sub-RPE basal laminar deposit. No connection between the choriocapillaris and the sub-RPE plus BL space was observed. Both RPE-derived and lipid-filled cells were correlated with clinical intraretinal hyperreflective foci. The sub-RPE plus BL space contained macrophages, lymphocytes, Müller cell processes, and subducted RPE. CONCLUSIONS: Clinicopathologic correlation of type 3 neovascularization showed vascular elements of retinal origin accompanied by collagenous material and Müller cell processes implanting into thick sub-RPE basal laminar deposit, which may simulate the appearance of chorioretinal anastomosis. Surrounding RPE-derived and lipid-filled cells thought to be microglia correlated with clinical intraretinal hyperreflective foci.

Associations of drusen location with risk factors and incidence of late age-related macular degeneration in the Alienor study.

PURPOSE: To test the hypothesis that central drusen location is strongly linked with known Age-related Macular Degeneration (AMD) risk factors and risk of incident late AMD. METHODS: The Alienor study is a prospective population-based cohort study of residents of Bordeaux, France, followed from 2009 to 2017. On retinal photographs, we defined central drusen as at least one soft drusen (>63 µm) within 500 µm from fovea and pericentral drusen as at least one drusen 500-3000 µm from fovea, in the absence of any central drusen. Late AMD (atrophic and/or neovascular) was diagnosed using multimodal imaging. In total, 481 eyes were included in the analysis: 160 central and 321 pericentral. We investigated associations with systemic (age, sex, smoking, medical prescriptions, plasma concentrations of lipids and nutrients, UV exposure, blood pressure), ocular (retinal thickness, cataract extraction) and genetic risk scores (GRS). RESULTS: In multivariate logistic regression central drusen were associated with smoking (OR, 2.95 for smoking more than 20 pack-years, p = 0.02), HDL-cholesterol (OR, 1.57 for 1 standard deviation (SD) increase, p = 0.0048), pulse pressure (OR, 0.77 for 1 SD increase, p = 0.04), Age-Related Maculopathy Susceptibility 2 (ARMS2) GRS (OR, 1.42; 95% CI, 1.11-1.83) and complement GRS (OR, 1.55; 95% CI, 1.15-2.10). In Cox modelling, the central location of drusen (at baseline or during the follow-up) was associated with a 4.41-fold increased risk (95% CI,1.98-9.81) for an incident late AMD. CONCLUSION: Central drusen were strongly associated with AMD risk factors and incident late AMD, suggesting that it represents a key marker for AMD progression.

Ophthalmologic Phenotype-Genotype Correlations in Patients With Oculocutaneous Albinism Followed in a Reference Center.

Purpose: Albinism is a group of genetic disorders that includes several conditions related to a defect in melanin production. There is a broad phenotypic and genotypic variability between the different forms. The aim of this study was to assess the ophthalmologic characteristics according to patients' genotypes in a cohort followed in the Reference Center for oculocutaneous albinism (OCA) of Bordeaux University Hospital, France. Methods: A retrospective observational study was conducted in a cohort of patients with OCA seen in consultation in the ophthalmology department between 2017 and 2021 in whom a genetic analysis was performed. Results: In total, 127 patients with OCA were included in this study and matched with the results of the genetic analysis. In the population aged over 6 years, there was no statistical difference in binocular visual acuity between the OCA1, OCA2, and OCA4 forms (P = 0.27). There was difference in ametropia between the three forms (P = 0.003). A two-by-two comparison using the Bonferroni correction showed a significant difference in ametropia between the OCA2 and OCA4 forms (P = 0.007) and between the OCA1 and OCA2 forms (P = 0.0075). Regardless of the form, most patients (75.4%) had grade 4 foveal hypoplasia. There was no association between the grade of foveal hypoplasia and the gene involved (P = 0.87). Conclusions: We described a genotype-phenotype correlation for the three most represented forms of albinism in our cohort. This study allowed assessing the degree of visual deficiency in young children with OCA.

Choroidal granulomas due to Bartonella henselae infection: A case series.

PURPOSE: To report a case series of 3 patients with choroidal granulomas due to Bartonella henselae infection in order to raise awareness about this etiology in the differential diagnosis of choroidal granulomas. METHODS, PATIENTS: A retrospective case series of patients with choroidal granulomas due to Bartonella henselae infection who consulted between 2018 and 2020. Data were collected from the medical records (demographics, visual acuity (VA), laboratory tests, treatment, imaging). RESULTS: Patients were a 48-year old man, a 14-year old girl and a 31-year old man. They all had a choroidal granuloma seen on optical coherence tomography (OCT) and angiography. The laboratory work-up revealed a positive serology for Bartonella henselae in all patients. CONCLUSION: On multimodal imaging choroidal granulomas in B Henselae appeared as single or multiple, uni or bilateral round yellowish lesions. Fluorescein and indocyanine green angiography of the granuloma showed respectively a late staining and a hypofluorescence. On EDI-OCT choroidal granuloma appeared as a round hyporeflective lesion in the choroid with a retinal elevation. The exclusion of other diagnosis, the natural course and the serology must lead the ophthalmologist to evoke the diagnosis.

LATE RECURRENCE OF CHOROIDAL NEOVASCULARIZATION IN PATIENTS WITH MULTIFOCAL CHOROIDITIS: CLINICAL SURVEILLANCE IN PERPETUITY.

PURPOSE: To report a very late recurrence of choroidal neovascularization (CNV) in elderly patients with noninfectious multifocal choroiditis (MFC). METHODS: Retrospective case series of patients with MFC with confirmed recurrence of CNV. Choroidal neovascularization was diagnosed with multimodal imaging, including optical coherence tomography angiography. Multifocal choroiditis-associated CNV eyes were treated with intravitreal injections of anti-vascular endothelial growth factor medication. RESULTS: Four eyes of three patients were included in our study, with a mean (range) age of 73 years (67-78). The period between the original CNV and the recurrence was 53 years, with a range of 48-60 years. The mean number (range) of injections given after the late recurrence per eye was 7 (5-11). The mean duration (range) of follow-up post-treatment initiation was 93 (40-122) weeks. All eyes improved to 20/30 visual acuity or better at 6 months after initial treatment. CONCLUSION: Patients with MFC are never exempt from recurrent CNV, warranting follow-up in perpetuity. Age-related factors are important to consider which may increase the susceptibility for activating MFC-associated CNV in elderly people. Macular neovascularization could respond to a standard approach to management, in these patients with MFC, by a judicious use of intravitreal injections of anti-vascular endothelial growth factor therapy.

Preoperative imaging optimized for epiretinal membrane surgery.

BACKGROUND: To compare imaging modalities for visualizing primary epiretinal membrane (ERM) with each other and with intraoperative digital images (IDI) after blue staining. METHODS: The records of consecutive patients operated for primary ERM over a 12-month period were retrospectively reviewed. Preoperative imaging included color fundus photography (CFP), En Face spectral-domain optical coherence tomography (OCT), 45° infrared- (IR) and blue-reflectance (BR) scanning laser ophthalmoscopy. All images were qualitatively analyzed and scored from 0-4 according to the ability to visualize ERM details (0 = no visible ERM or vessel contraction, 1 = vessel contraction, 2 = retinal folds, 3 = ERM limits, 4 = elevated ERM edge). The preoperative ERM morphology was then compared to that seen on the IDI acquired after 1-min blue dye staining when available. RESULTS: Seventy eyes were included. The highest score for ERM visualization was obtained on BR and En Face OCT. A score of 3 or 4 was obtained in 68.5%, 62.1%, 17.9% and 13.6% of cases on En Face OCT, BR, CFP and IR images, respectively. IDI were available for 20 eyes, and showed a similar ERM morphology compared to preoperative images in most cases: a negative staining pattern corresponded to a plaque on En face OCT in 91% of eyes. However, IDI failed to show the ERM edges in 37.5% of cases. CONCLUSION: ERM morphology was better visualized preoperatively by BR and En Face OCT, in a similar way to the IDI after staining. Future intraoperative visualization systems could integrate both imaging modalities overlaid with the IDI for guiding ERM removal instead of staining.

OCT angiography analysis in acute non-arteritic anterior ischemic optic neuropathy: The importance of segmentation.

BACKGROUND: In non-arteritic anterior ischemic optic neuropathy (NAION), optical coherence tomography angiography (OCTA) shows changes in peripapillary vascularization. However, the presence of an optic disc edema may induce artifacts that prevent visualizing the peripapillary network. The aim of this study was to evaluate the peripapillary vascularization in acute NAION using swept-source OCTA algorithms allowing segmenting only the retinal nerve fiber layer (RNFL). METHODS: Retrospective analysis of 15 eyes with acute NAION of 15 patients. The optic nerve head was imaged using swept-source OCTA. Morphological and quantitative analyzes were performed. The capillary flux index (CFI), defined as the total weighted area of perfused vasculature per unit area, and the capillary perfusion density (CPD), defined as the total area of perfused microvasculature per unit area, were quantified. Each NAION eye was compared to the unaffected fellow eye using a Wilcoxon test for matched samples. RESULTS: After segmentation at the RNFL, the morphological analysis showed less vascular dropout and more vascular tortuosity than the analysis of a larger segmentation. The quantitative analysis showed that the mean CFI and the CFI in the four quadrants were significantly higher in NAION eyes compared to healthy eyes (p = 0.0002 and p < 0.01). The mean CPD and the CFD in the inferior quadrant were lower in NAION eyes (p = 0.03 and p = 0.0054, respectively). DISCUSSION: The RNFL segmentation allowed better visualizing the peripapillary network because the edema related darkening was reduced. The increased CFI suggests an autoregulatory phenomenon to compensate the ischemic process at the ciliary vasculature.

MAINTENANCE OF GOOD VISUAL ACUITY IN BEST DISEASE ASSOCIATED WITH CHRONIC BILATERAL SEROUS MACULAR DETACHMENT.

PURPOSE: We describe the long-term follow-up of a patient with multifocal Best disease with chronic bilateral serous macular detachment and unusual peripheral findings associated with a novel mutation in the BEST1 gene. METHODS: Case report. RESULTS: A 59-year-old white woman was referred for an evaluation of her macular findings in 1992. There was a family history of Best disease in the patient's mother and a male sibling. Her medical history was unremarkable. Best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. The anterior segment examination was normal in both eyes. Funduscopic examination showed multifocal hyperautofluorescent vitelliform deposits with areas of subretinal fibrosis in both eyes. An electrooculogram showed Arden ratios of 1.32 in the right eye and 1.97 in the left eye. Ultra-widefield color and fundus autofluorescence imaging showed degenerative retinal changes in areas throughout the entire fundus in both eyes. Optical coherence tomography, including annual eye-tracked scans from 2005 to 2016, showed persistent bilateral serous macular detachments. Despite chronic foveal detachment, visual acuity was 20/25 in her right eye and 20/40 in her left eye, 24 years after initial presentation. Genetic testing showed a novel c.238T>A (p.Phe80Ile) missense mutation in the BEST1 gene. CONCLUSION: Some patients with Best disease associated with chronic serous macular detachment can maintain good visual acuity over an extended follow-up. To our knowledge, this is the first report of Best disease associated with this mutation in the BEST1 gene.

Incidence and Risk Factors of Reticular Pseudodrusen Using Multimodal Imaging.

Importance: Although retinal multimodal imaging is needed for diagnosing reticular pseudodrusen (RPD), the incidence of RPD in the general population typically has been assessed only using fundus photographs, which may underestimate their incidence. Objectives: To describe the incidence of RPD using retinal color photographs, spectral-domain optical coherence tomography scans, fundus autofluorescence, and near-infrared reflectance images among individuals 77 years of age or older and to analyze the associated risk factors of RPD. Design, Setting, and Participants: The ALIENOR (Antioxydants, Lipides Essentiels, Nutrition et Maladies Oculaires) Study is a cohort of French individuals 77 years of age or older. Data for this study were collected between February 22, 2011, and February 15, 2017, with a mean (SD) follow-up of 3.7 (1.0) years (range, 1.2-5.6 years). At baseline, 501 individuals were eligible to participate. Of 1002 eyes, 197 had prevalent RPD, advanced age-related macular degeneration, or ungradable images. Of the remaining 805 eyes, 333 were missing follow-up data; therefore, the statistical analyses included data from 472 eyes. Data management and statistical analyses were performed between March 15, 2017, and April 5, 2019. Main Outcomes and Measures: Reticular pseudodrusen were considered as present if detected by at least 2 of the following imaging methods: color fundus photographs, fundus autofluorescence, near-infrared reflectance, and spectral-domain optical coherence tomography images. Results: Of the 472 eyes analyzed, 263 (55.7%) were from female participants, and the mean (SD) age was 81.9 (3.2) years. Forty-three eyes developed RPD, corresponding to an annual incidence rate of 2.9% (95% CI, 1.9%-4.4%) per participant and an estimated 5-year risk of 13.5%. In multivariable analysis, 4 risk factors of incident RPD were identified: subfoveal choroidal thinning (hazard ratio [HR], 0.99; 95% CI, 0.99-1.00 per 10-µm decrease in thickness; P = .02) and the presence of the minor allelic variants rs10490924 for ARMS2 (HR, 3.57; 95% CI, 1.80-7.10; P < .001), rs1061170 for CFH (HR, 2.12; 95% CI, 1.02-4.41; P = .04), and rs10468017 for LIPC (HR, 2.57; 95% CI, 1.37-4.82; P = .003). Lipophilic statin therapy was associated with a lower incidence of RPD (HR, 0.13; 95% CI, 0.02-0.74; P = .02). Conclusions and Relevance: With the use of multimodal imaging, the RPD incidence rate was higher than previously reported in other population-based studies using fundus color images. Individuals with subfoveal choroidal thinning or carrying minor allelic variants for ARMS2, CFH, or LIPC had an increased risk for RPD, whereas lipophilic statin therapy was associated with a lower incidence.

MULTIPLE EVANESCENT WHITE DOT SYNDROME WITH SUBRETINAL DEPOSITS.

PURPOSE: To describe the multimodal imaging findings of transient subretinal deposits occurring in multiple evanescent white dot syndrome (MEWDS). METHODS: The multimodal imaging characteristics of transient subretinal deposits occurring in MEWDS were investigated with ultra-widefield color and fundus autofluorescence, cross-sectional and en-face optical coherence tomography (OCT), en face OCT-angiography, and quantitative autofluorescence. RESULTS: A 28-year-old woman presented with photopsia and temporal visual field loss in her right eye. Her best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination showed characteristic peripapillary hyperautofluorescent white dots of MEWDS corresponding to ellipsoid zone disruption on OCT. These lesions became confluent throughout the posterior fundus over the next 4 weeks. As the patient's symptoms were resolving, a second type of transient hyperautofluorescent lesion was noted which corresponded to hyperreflective subretinal deposits on cross-sectional and en face structural OCT. These subretinal deposits were most evident at 10-week follow-up and had nearly resolved at 14-week follow-up. Quantitative autofluorescence showed that, unlike the acute MEWDS lesions, the hyperautoflurescence of the subretinal deposits persisted after photobleaching. At multiple time points over 14 weeks of follow-up, OCT angiography showed no evidence of retinal or choroidal flow abnormalities. CONCLUSION: Transient subretinal deposits may develop during MEWDS in areas of previous diffuse outer retinal disruption. As these deposits remain hyperautoflurescent on quantitative autofluorescence after photobleaching, they may represent accumulations of debris originating from damaged photoreceptor outer segments.

The Nature and Frequency of Outer Retinal Disruption in Idiopathic Multifocal Choroiditis Associated With Persistent Fundus Hyperautofluorescence.

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.].

The European Eye Epidemiology spectral-domain optical coherence tomography classification of macular diseases for epidemiological studies.

PURPOSE: The aim of the European Eye Epidemiology (E3) consortium was to develop a spectral-domain optical coherence tomography (SD-OCT)-based classification for macular diseases to standardize epidemiological studies. METHODS: A European panel of vitreoretinal disease experts and epidemiologists belonging to the E3 consortium was assembled to define a classification for SD-OCT imaging of the macula. A series of meeting was organized, to develop, test and finalize the classification. First, grading methods used by the different research groups were presented and discussed, and a first version of classification was proposed. This first version was then tested on a set of 50 SD-OCT images in the Bordeaux and Rotterdam centres. Agreements were analysed and discussed with the panel of experts and a final version of the classification was produced. RESULTS: Definitions and classifications are proposed for the structure assessment of the vitreomacular interface (visibility of vitreous interface, vitreomacular adhesion, vitreomacular traction, epiretinal membrane, full-thickness macular hole, lamellar macular hole, macular pseudo-hole) and of the retina (retinoschisis, drusen, pigment epithelium detachment, hyper-reflective clumps, retinal pigment epithelium atrophy, intraretinal cystoid spaces, intraretinal tubular changes, subretinal fluid, subretinal material). Classifications according to size and location are defined. Illustrations of each item are provided, as well as the grading form. CONCLUSION: The E3 SD-OCT classification has been developed to harmonize epidemiological studies. This homogenization will allow comparing and sharing data collection between European and international studies.

Dense B-Scan Optical Coherence Tomography Angiography.

PURPOSE: To describe a novel imaging technique, which we call "dense B-scan optical coherence tomography angiography" (DB OCTA), in which thin dense raster scans are used to produce highly resolved structural B-scans with superimposed flow signal that provide precise correlation between retinal microstructure and blood flow. DESIGN: Observational case series. METHODS: Normal eyes and eyes with macular findings of interest were imaged with DB OCTA in which 150-400 OCT B-scans were acquired within a narrow area (from a single line to 1 degree) with a width of 10-30 degrees. B-scans containing 5-7 consecutive frames were processed for OCTA signal and then combined and visualized post-acquisition by application of a Gaussian filter across neighboring scans. The result was a single, smoothed, high-resolution image that contained both structural and flow information. Tracked follow-up DB OCTA was used to detect subtle changes in pathology over time. RESULTS: Two hundred and thirty-seven eyes from 205 subjects aged 18-100 years (mean 72.88 ± 14.74 years) with a diverse range of macular findings were imaged with DB OCTA. Highly resolved scans showing precise localization of flow signal were readily obtained, even in patients with poor visual acuity and/or poor fixation. We present clinical examples that demonstrate the utility of DB OCTA for visualizing the associations between retinal microstructure and blood flow. CONCLUSIONS: DB OCTA enables precise correlation between retinal microstructure and blood flow. The ability to obtain accurately aligned follow-up DB OCTA studies has the potential to refine the understanding and clinical management of a wide range of macular diseases.

IDIOPATHIC MULTIFOCAL CHOROIDITIS PRESENTING WITH A TRANSIENT PERIPAPILLARY WHITE RING.

PURPOSE: We describe with multimodal imaging the presentation and follow-up for a patient with idiopathic multifocal choroiditis and a transient peripapillary white ring. METHODS: Case report. RESULTS: A 39-year-old Asian woman was initially seen for an evaluation of lattice degeneration in 2015. Her medical history included Graves disease and psoriasis. Best-corrected visual acuity was 20/25 in her right eye and 20/25 in her left eye. Ultra-widefield fundus autofluorescence imaging showed a curvilinear hyperautofluorescent line in her right eye. One year later, the patient returned complaining of floaters in her right eye for 1 month. Her visual acuity was unchanged. Funduscopic examination showed new inflammatory yellowish lesions in the right eye corresponding to hyperreflective sub-retinal pigment epithelium lesions on structural spectral domain optical coherence tomography. Fluorescein angiography showed corresponding late staining of these active lesions. Late-phase indocyanine green angiography showed multiple nummular hypocyanescent dots. Ultra-widefield fundus autofluorescence showed large areas of hyperautofluorescence. The patient was started on a 60-mg oral prednisone taper and demonstrated subsequent regression of the inflammatory lesions. Ten months later, the patient returned emergently with complaints of floaters in both eyes for 2 days and a new temporal scotoma in her left eye. Funduscopic examination demonstrated a white ring around the optic nerve of the left eye corresponding to a hyperautofluorescent lesion. Ultra-widefield fundus autofluorescence showed new areas of hyperautofluorescence in both eyes. Structural spectral domain optical coherence tomography showed new sub-retinal pigment epithelium inflammatory lesions and a disruption of the ellipsoid zone in both eyes. The patient was again treated with a 60-mg oral prednisone taper and demonstrated subsequent restoration of the ellipsoid zone. CONCLUSION: To our knowledge, this is the first report of a transient annular white ring occurring in a case of multifocal choroiditis. There was marked restoration of the disrupted ellipsoid zone after treatment with oral corticosteroids.