Rheumatic fever and long-term use of benzathine penicillin as possible risk factors for extensive macular atrophy with pseudodrusen in a Brazilian cohort.

BACKGROUND: Although there has been a large increase in the number of extensive macular atrophy with pseudodrusen (EMAP) cases, the basic aspects of this disease remain unknown. Brazilian patients have a common past history of rheumatic fever (RF) and/or benzathine penicillin (BP) treatment possibly related to the disease. We analyzed how RF and BP might be correlated with EMAP in Brazilian patients. DESIGN: Observational, retrospective, case-control study. METHODS: The databases of three private eye clinics in Brazil were searched for patients with an EMAP-like appearance. Each patient was asked about a previous history of RF and/or long-term use of BP. Patients underwent best-corrected visual acuity (BCVA) measurement, color fundus imaging, fundus autofluorescence (FAF) imaging, optical coherence tomography (OCT) imaging, and electroretinography (ERG). The following characteristics were analyzed: subretinal drusenoid deposits (SDD), pigment mottling, retinal pigment epithelial/basement membrane (RPE/BM) separation, outer retinal or RPE atrophy, and identification of a paving stone-like appearance. The choroidal thickness was measured using enhanced depth imaging OCT. The central atrophic area was measured manually on ultra-wide-field FAF. RESULTS: A total of 154 eyes of 77 patients (women, 66.2%; mean age, 58.6 years) with EMAP were included; 90.9% of patients were diagnosed with RF; 94.8% had been treated with BP and treatment was started at an average age of 7.3 years (mean duration, 11.8 years). The treatment duration was significant for the area of atrophy (P = 0.027) in which each 1-year increase in treatment duration led to an average reduction of 6.91 mm2 in area. The age at diagnosis of RF was significant (P = 0.026) for SDD. The increase of 1 year in the diagnosis of RF (late disease) led to a reduction of 24% in the chance of central SDD being present. On OCT, 65.5% eyes had SDD and more than 70% had a split RPE/BM and outer retinal or RPE atrophy. The choroidal thickness in patients with EMAP was significantly (P < 0.001) thinner than the control group. The ERG was abnormal in all eyes. CONCLUSION: These findings may suggest a relation between RF and EMAP in Brazilian patients. Patients with EMAP should be questioned about a history of RF.

Photic maculopathy: five case reports and literature review / Maculopatia fótica: cinco relatos de caso e revisão de literatura

ABSTRACT Maculopathy from prolonged exposure to solar light is a rare but well-recognized clinical entity of vision loss and macular damage. Photochemical damage precedes visual decline, and in mild cases, vision usually returns fully or partially. With the advancement of humanity, other forms of macular injuries induced by light radiation have emerged, increasing the group of photic maculopathies. In this report, we describe the cases of five patients where a diagnosis of photic maculopathy was made based on the anamnesis, clinical findings, and complementary exams. We compare the five cases regarding their similarities and differences, as well as review the literature on the subject.

RESUMO A maculopatia causada pela exposição prolongada à luz solar é uma entidade clínica rara, mas bem reconhecida, de perda de visão e dano macular. O dano fotoquímico precede o declínio visual e em casos leves a visão geralmente retorna total ou parcialmente. Com o avanço da humanidade, surgiram outras formas de lesões maculares induzidas pela radiação luminosa, aumentando o grupo das maculopatias fóticas. Neste relato, descrevemos os casos de cinco pacientes onde o diagnóstico de maculopatia fótica foi feito com base na anamnese, achados clínicos e exames complementares. Comparamos os quatro casos quanto às suas semelhanças e diferenças, bem como revisamos a literatura sobre o assunto.

OCT Angiography Use in Pregnancy: Branch Retinal Artery Occlusion Associated with Patent Foramen Ovale - A Case Report and Multimodal Analysis.

This case report is a multimodal analysis of a pregnant patient with branch retinal artery occlusion (BRAO) associated to patent foramen ovale (PFO). A 28-year-old woman presented at the clinic 20 h after an acute, painless black spot appearance in the inferior temporal visual field of the right eye (OD). At that time, she was 18 weeks pregnant and had no report of complications in her previous pregnancy. Best-corrected visual acuity was 1.0 in both eyes. Color fundus photo, perimetry, and OCT angiography were required. The results clearly showed an embolus in the superior nasal retinal arteriole, associated with a pallor in the distal retina. Patient was referred to a cardiologist and a transcranial Doppler with contrast indicated a right-to-left intracardiac shunt, confirmed by the presence of a PFO at the transesophageal echocardiography. Thrombophilic conditions were excluded. Enoxaparin 1 mg/kg was started and kept until the delivery. Now, a PFO surgical closure is on schedule. This case highlights the noteworthiness of considering PFO as a source of embolism for BRAO in young patients, the capability of OCTA as a dye-free method for use in pregnancy and emphasizes the importance of systemic evaluation in patients with BRAO.

Comparing six cases of external macular holes and literature review / Comparação de seis casos de buracos maculares externos e revisão de literatura

ABSTRACT The characteristic optical coherence tomography finding in solar maculopathy is a well-defined outer retinal hyporeflective space primarily involving the photoreceptor inner and outer segment layers. This typical optical coherence tomography image may be present in a few other pathologies, which currently constitute their main differential diagnoses. Our study shows the report of 12 eyes of 6 patients treated at the Hospital de Olhos do Paraná, presenting their clinical history and diagnostic images, with the purpose of comparing the findings of the first 3 patients (diagnosed with solar maculopathy) with the last 3 patients, which are also cases of external macular holes.

RESUMO O achado característico da tomografia de coerência óptica na maculopatia solar é um espaço hiporrefletivo retiniano externo bem definido, envolvendo principalmente as camadas dos segmentos interno e externo dos fotorreceptores. Essa imagem típica da tomografia de coerência óptica pode estar presente em algumas outras patologias, que atualmente constituem seus principais diagnósticos diferenciais. Nosso estudo mostra o relato de 12 olhos de 6 pacientes atendidos no Hospital de Olhos do Paraná, apresentando sua história clínica e imagens diagnósticas, com o objetivo de comparar os achados dos 3 primeiros pacientes (diagnosticados com maculopatia solar) com os 3 últimos pacientes, que também são casos de buracos maculares externos.

Efficacy and safety of angiotensin-converting enzyme inhibitors or angiotensin receptor blockers for IgA nephropathy in children.

BACKGROUND: IgA nephropathy (IgAN) is one of the most prevalent primary glomerulopathies in children. There are various studies investigating the efficacy of angiotensin-converting enzyme inhibitors (ACEI) and angiotensin II receptor blockers (ARB) in adults with IgAN. However, only few studies evaluated the efficacy of these medications in pediatric patients. OBJECTIVE: To evaluate the efficacy and safety of ACEI/ARB in children with IgAN. DATA SOURCES: Databases including PubMed, Web of Science, Cochrane, Scopus, and Google Scholar were searched between the 1st of April and 20th of July of 2021 using the keywords "IgA Nephropathy," "Berger's Disease," "Angiotensin-Converting Enzyme Inhibitors," "Angiotensin Receptor Antagonists," "Angiotensin II Type 1 Receptor Blockers," and similar entry terms collected from the Medical Subject Headings (MeSH). STUDY ELIGIBILITY CRITERIA: Observational studies (case series, case-control, cohort, and cross-sectional) and clinical trials with descriptions of pediatric patients (under 19 years old) with histopathological diagnosis of IgA nephropathy and who received ACEI and/or ARB. PARTICIPANTS AND INTERVENTIONS: Pediatric patients (under 19 years old) with histopathological diagnosis of IgA nephropathy and who received ACEI and/or ARB. STUDY APPRAISAL: For quality assessment, the Risk of Bias 2 tool (RoB 2), the Risk Of Bias In Non-randomized Studies of Interventions tool (ROBINS-I), the National Institutes of Health (NIH) quality assessment tool, and the Newcastle-Ottawa Scale (NOS) were used. RESULTS: After recovering 1,471 studies, only eight, published between 2003 and 2019, met the eligibility criteria and were included in this systematic review. Of the 737 included children in the studies, 202 (25.8%) used ACEI/ARB and were compared with placebo and other therapy regimens. Of the seven studies that evaluated proteinuria, six reported an efficacy of ACEI/ARB in reducing this marker. ACEI/ARB also showed a possible effect in reducing hematuria and oxidative stress. The most common side effect was dizziness. LIMITATIONS: The number of studies about the treatment with ACEI/ARB in children with IgAN is scarce. In addition, the studies are very heterogeneous. There are few studies that compared ACEI/ARB with placebo. CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: The use of ACEI and/or ARB appears to be safe and to reduce proteinuria in pediatric patients with IgAN. Nonetheless, further randomized controlled trials, with greater methodological rigor and longer follow-up time, are required to establish the efficacy and safety of this therapy in this population. SYSTEMATIC REVIEW REGISTRATION NUMBER: The protocol of this systematic literature review was registered in PROSPERO under the number CRD42021245375, and in the OSF registries ( https://osf.io/qft4z/ ) with the registration https://doi.org/10.17605/OSF.IO/VADYR . A higher resolution version of the Graphical abstract is available as Supplementary information.

Age-related macular degeneration and resource utilization in the Brazilian public healthcare system: a real-world retrospective study.

BACKGROUND: Age-related macular degeneration (AMD) is a disease that causes damage in the macular region of the retina, leading to irreversible blindness. This study aims to understand the profile and care of patients with AMD and its cost at the Brazilian public health system to identify AMD-care needs. METHODS: This is a retrospective observational study of AMD with real-world data from the Brazilian public healthcare system, using DATASUS claim databases. Patients with AMD were selected from 01/Jan/2014 to 31/Jan/2020; had at least one claim of ICD10 code H35.3 (Degeneration of macula and posterior pole), and were submitted to one of two procedures exclusively available for AMD patients - optical coherence tomography (OCT) and medical treatment of retinal disease (antiangiogenic); aged ≥18 years at first ICD10 claim, and presenting at least 1 year of follow-up in the database. We described patients' characteristics, healthcare resource utilization and cost, and the antiangiogenic intravitreal treatment received by AMD patients, including the number of doses and interval time between them. RESULTS: Patients searching for AMD treatment since 2014 were mostly females (59%), white (61%), and a mean age of 72 years. They were mainly located in the Southeast (87%), and few patients were found in the North (1%) and Central-West (1.5%) regions, probably reflecting where the Brazilian guideline to treat AMD (Protocolo Clínico e Diretrizes Terapêuticas - PCDT) was incorporated as routine care for AMD. The average antiangiogenic dose of 2.5 antiangiogenic therapies within a year was below the expected. Most injections had an interval time of 20 to 40 days between doses, although some patients were treated more than 100 days. Another setback is that patients traveled longer distances for OCT and antiangiogenic treatment than overall AMD-healthcare, between 10 and 100 km. CONCLUSIONS: AMD patients seem to be undertreated, as they receive a mean of 2.5 doses of antiangiogenic treatment within a year. Inequalities among regions are evident, as the Southeast and South regions comprise almost all patients receiving the treatment from the public health system, probably reflecting the region with more access to AMD care according to PCDT recommendations.

En-face OCT and OCT angiography analysis of macular choroidal macrovessel.

PURPOSE: To analyze en-face optical coherence tomography (OCT) and decorrelation signals on OCT angiography (OCTA) in two cases of macular choroidal macrovessel (MCM). OBSERVATIONS: Case report. Both the 64-year-old and 71-year-old females presented for a routine evaluation, and multimodal imaging analysis, including color fundus photography, indocyanine green angiography (ICG), spectral-domain optical coherence tomography (SD-OCT) and OCTA, was performed to diagnose a MCM. En-face OCT, en-face OCTA and decorrelation signals were analyzed through the MCM. In both reported cases, color fundus photograph revealed a serpiginoid lesion in the temporal macula. Red-free imaging enhanced the appearance of this lesion resembling a dilated choroidal vessel. Cross-sectional OCT showed an enlarged choroidal vessel causing elevation of the retinal pigment epithelium (RPE) within the fovea. En-face OCTA with segmentation below the choriocapillaris enhanced the MCM delineation. En-face OCT with segmentation below the choriocapillaris showed MCM with a greater distinctness than the en-face OCTA imaging. Decorrelation signals were not observed within MCM on cross-sectional OCTA. CONCLUSION AND IMPORTANCE: En-face OCT and decorrelation signals on OCTA may have diagnostic value in distinguishing macular choroidal macrovessel from other choroidal vascular diseases.

Test-retest variability of microperimetry in geographic atrophy.

PURPOSE: Microperimetry (MP) allows for measurement of retinal sensitivity at precise locations and is now commonly employed as a clinical trial endpoint. Test-retest reliability is important when evaluating treatment effects in patients with geographic atrophy (GA). This study aimed to determine the test-retest variability of MP in patients with moderate to severe GA using the MAIA MP device. METHODS: In this prospective study, patients with a confirmed diagnosis of foveal-involving GA were enrolled. Participants performed three MP assessments of a selected eye over two visits with the Macular Integrity Assessment (MAIA) 2 instrument (Centervue, Padova, Italy) utilizing a wide 30° grid, consisting of 93 stimuli (Goldmann III) using a 4-2 representation strategy, encompassing the entire area of GA and beyond. Mean retinal sensitivity (MS) was expressed as an average threshold value (dB) for the entire field tested. Coefficients of Repeatability at a 95% level (CoR95) were calculated for Point Wise Sensitivity (PWS). Fixation stability (FS) was assessed by evaluating the area of an elliptical representation encompassing 95% of the cloud of fixation points (CFP) dataset generated by the MAIA MP, known as the bivariate contour ellipse area (BCEA). RESULTS: A total of 8 subjects were enrolled (21 tests), with six subjects completing 3 MP assessments. BCVA in these patients ranged from 20/100 to 20/800. The mean area of GA was 18.7 ± 12.3 mm2. The average time to complete one MP assessment was 13 min 9 s and mean BCEA@95% was 38.5 ± 19.3°2. The MS was 14.3 ± 4.5 dB. No significant increase in MS was noted between testing pairs 1&2 and 2&3. The preferred retinal locus was maintained in the same quadrant on successive tests. The mean CoR95 for PWS were similar for testing pairs 1&2 (± 3.50 dB) and 2&3 (± 3.40). CONCLUSION: Microperimetry using a wide grid can be reliably performed in a reasonable amount of time in patients with moderate and severe vision loss secondary to GA. There was no learning effect seen between sequential assessments when analyzing MS or PWS. A change of approximately 4 dB in PWS provides a threshold for considering a true change in this patient cohort.

A low glycemic diet protects disease-prone Nrf2-deficient mice against age-related macular degeneration.

Age-related macular degeneration (AMD) is a major blinding disease, affecting over 14% of the elderly. Risk for AMD is related to age, diet, environment, and genetics. Dietary modulation of AMD risk is a promising treatment modality, but requires appropriate animal models to demonstrate advantages of diet. Mice lacking the antioxidant transcription factor Nrf2 (Nfe2l2) develop age-related retinopathy relevant to human AMD. Here we evaluated the effect of consuming high glycemic (HG) or low glycemic (LG) diets until 18-months of age on development of features relevant to AMD in Nrf2-null mice. Nrf2-null mice that consumed HG diets developed atrophic AMD, characterized by photoreceptor degeneration, retinal pigment epithelium (RPE) atrophy and pigmentary abnormalities, basal laminar deposits, and loss of the choriocapillaris. In contrast, Nrf2-null-mice that consumed LG diets did not develop retinal disease phenotypes. Consumption of HG diets was associated with accumulation of advanced glycation end-products in the RPE and systemically, whereas consumption of the LG diet was associated with increased levels of anti-glycative and anti-oxidative detoxification machinery. Together our data indicate that the Nrf2-null HG mouse is a good model for atrophic AMD studies and that the LG diet can activate protective pathways to prevent AMD, even in a genetically predisposed animal.

MACULAR ATROPHY IN NEOVASCULAR AGE-RELATED MACULAR DEGENERATION: A Pilot Post Hoc Analysis of Patients With Pigment Epithelial Detachments.

PURPOSE: To determine optical coherence tomography signs associated with macular atrophy (MA) in eyes with neovascular age-related macular degeneration and pigment epithelial detachments treated with vascular endothelial growth factor inhibitors. METHODS: Optical coherence tomography scans from a subgroup of the pigment epithelial detachment cohort of the HARBOR study were analyzed for MA. Two groups were formed based on MA presence/absence at Month 24. Then, optical coherence tomography scans from each baseline visit were graded with standard reading center grading parameters including ellipsoid zone disruption, intraretinal cysts, subretinal fluid, and MA or nascent MA in the study and fellow eyes. RESULTS: Twenty-eight eyes from 28 patients were included in the analysis. Fourteen eyes had optical coherence tomography-based MA at Month 24 and 14 did not. Macular atrophy at Month 24 was significantly associated with 1) MA/nascent MA at baseline (P = 0.0136), 2) intraretinal cysts at baseline (P = 0.0048), and 3) collapse of pigment epithelial detachments in the study eye (P = 0.0025). Macular atrophy was not associated with ellipsoid zone disruption or subretinal fluid in the study eye at baseline. CONCLUSION: This study suggests that some optical coherence tomography findings in eyes of patients with neovascular age-related macular degeneration were present before the start of anti-vascular endothelial growth factor therapy and may predict the development of MA.

Nodular posterior scleritis associated with presumed ocular tuberculosis: A multimodal imaging case report.

PURPOSE: To describe a patient with nodular posterior scleritis associated with presumed ocular tuberculosis (TB). OBSERVATIONS: A 25-year-old Caucasian man reported metamorphopsia in the right eye (OD). He had lost the vision in his left eye when he was 15 years old. The visual acuity (VA) levels were 20/20 and light perception in the right and left eyes, respectively. Wide-field color fundus photography, fluorescein and indocyanine green angiography, optical coherence tomography, and ultrasound suggested an inflammatory condition associated with an elevated lesion in the choroid/sclera. The purified protein derivative (PPD) skin test and all other screening tests were negative. A diagnosis of presumed nodular posterior scleritis was made and after oral corticosteroid therapy, the VA decreased to 20/100 OD associated with a red and painful eye. Subsequently a QuantiFERON-TB test was positive, tuberculosis (TB) treatment was started and the corticosteroid dose was increased. Three months after treatment, the pain resolved and the vision OD recovered to 20/20. CONCLUSIONS AND IMPORTANCE: Nodular posterior scleritis can be associated with ocular TB that did not have a pulmonary manifestation. Rigorous testing is required in order to prevent visual loss in this disease that is not easily diagnosed.

Short-term eplerenone for treatment of chronic central serous chorioretinopathy; a prospective study.

BACKGROUND: Increased mineralocorticoid activity is one of the plausible causes of chronic central serous chorioretinopathy (CSCR) and mineralocorticoid inhibitors such as eplerenone have been investigated as its potential therapy. This study investigates the short-term safety and efficacy of oral eplerenone in patients with chronic CSCR. PATIENTS AND METHODS: Prospective study of 13 eyes of 13 patients with the diagnosis of chronic CSCR. All patients received eplerenone 50 mg/day for 4 weeks. Enhanced depth imaging optical coherence tomography (OCT) was obtained. Best corrected visual acuity (BCVA), and OCT parameters including sub retinal fluid (SRF), choroidal thickness (CT) and central macular thickness (CMT), were measured manually. RESULTS: The mean SRF height decreased slightly at 1-month follow-up as compared to baseline, but the change was not statistically significant (94.18 ± 17.53 vs. 113.15 ± 18.69; p = 0.08). Subfoveal CT and CMT was significantly reduced as compared to baseline (6.6% [p = 0.002] and 7.05% [p = 0.04], respectively). The BCVA did not change significantly (20/28 vs. 20/30 [p = 0.16]). CONCLUSION: This study suggests that oral eplerenone may be used as a safe and potentially effective treatment in chronic CSCR, however there are minimal short-term effects on subretinal fluid or visual acuity therefore therapeutic trials longer than one month are necessary to test its benefits.Trial registration Clinicaltrials.gov identification number: NCT01822561. Registered 3/25/13, https://clinicaltrials.gov/ct2/show/study/NCT01822561.

Optimizing optical coherence tomography and histopathology correlation in retinal imaging.

OBJECTIVE: To develop a methodology to correlate optical coherence tomography (OCT) images and histopathological sections from the same eye. Part 1: To determine the best fixative for optimal OCT and histopathological analysis in post-mortem eyes. Part 2: A protocol is proposed to correlate histopathological features and OCT scans from the same post-mortem eyes. DESIGN: Experimental study. PARTICIPANTS: Part 1: Twenty-three rabbit eyes and 14 post-mortem human eyes. Part 2: Nineteen post-mortem human eyes. METHODS: Part 1: Six different fixatives were tested, and specimens were evaluated on 4 criteria: globe shape, structure opacification, retinal detachment, and nuclear details. Part 2: Based on the findings from Part 1, fixed human eyes were imaged using OCT. Orientation-controlled histopathological processing was performed to obtain serial tissue sections from paraffin embedded tissue, which were matched to corresponding OCT images. RESULTS: Part 1: Of the 6 fixatives, 2% glutaraldehyde and Davidson's solution met the proposed criteria in rabbit eyes. Of these, glutaraldehyde showed similar results in human eyes and was selected for Part 2. Part 2: Using anatomical landmarks, cross-sectional histopathological sections in the same orientation as the OCT images were correlated to their corresponding OCT images. Retinal lesions such as a macular hole, an epiretinal membrane, and the presence of drusen were easily correlated, proving the reliability of our methodology. Moreover, the photoreceptor's inner/outer junction was correlated to a hyperreflective band on OCT. CONCLUSIONS: A standardized protocol was developed to correlate OCT images and histopathological findings by generating serial cross-sections of the retina, which can be used to better understand otherwise ambiguous OCT findings.

Parafoveal Retinal Vessel Density Assessment by Optical Coherence Tomography Angiography in Healthy Eyes.

BACKGROUND AND OBJECTIVE: To assess variability in vessel density (VD) measurements across three optical coherence tomography angiography (OCTA) devices to identify a methodology that offers the least amount of variation in VD, and to assess the effect of averaging of multiple scans on VD variability. PATIENTS AND METHODS: Fifteen eyes of eight healthy individuals were imaged consecutively on three OCTA devices. Segmentations at the superficial, deep, and full retinal layers were generated. Repeat scans for each retinal layer were registered and averaged to generate one OCTA image. Two different automated thresholding techniques were used to calculate vessel area density (VAD) from binarized images and vessel skeleton density (VSD) from skeletonized images. Vessel length, a linear measure of the combined lengths of vessels, was calculated. Foveal avascular zone (FAZ) area was measured. RESULTS: All three OCTA devices were significantly different (P < .0001). This finding remained after averaging images (P < .0001). VSD was more repeatable within a device but less reproducible across devices. Conversely, VAD demonstrated less repeatability but greater reproducibility. Differences in VSD between devices were systematic and attributable to differences in resolution. Vessel length, unaffected by resolution, demonstrated no significant differences between the devices (P > .107). There was no significant difference in FAZ area across devices (P = .51). After averaging images, VD was significantly different from the single images for each device and plexus (P < .05) but remained within 1% of the value of a single scan. CONCLUSIONS: OCTA devices show variability in VD for healthy individuals. With greater repeatability, VSD appeared useful for following a patient on one device. VAD and vessel length seemed ideal for comparing vessel parameters between OCTA devices. After averaging multiple scans, VSD remained within 1% of a single scan, for which clinical significance remains to be determined. Caution is advised when comparing quantitative analyses across OCTA devices. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:S5-S17.].

Choriocapillaris Loss in Advanced Age-Related Macular Degeneration.

The purpose of this review is to summarize the current knowledge on choriocapillaris loss in advanced age macular degeneration (AMD). Several histopathological studies in animal models and human eyes had showed that the choriocapillaris density decreases with age. However, the role of choriocapillaris loss is still unclear in AMD and its advanced forms, either choroidal neovascularization (CNV) or geographic atrophy (GA). Some authors have hypothesized that choriocapillaris loss might precede overt retinal pigment epithelium atrophy. Others have hypothesized that deposition of complement complexes on and around the choriocapillaris could be related to the tissue loss observed in early AMD. The development of imaging modalities, such as optical coherence tomography angiography (OCTA), have led to a better understanding of underlying physiopathological mechanisms in AMD. OCTA showed atrophy of choriocapillaris underneath and beyond the region of photoreceptors and RPE loss, in agreement with previous histopathologic studies. The evolution of OCTA technology suggests that CNV seems to originate from regions of severe choriocapillaris alteration. Significant progress has been made in the understanding of development and progression of GA and CNV. In vivo investigation of the choriocapillaris using OCTA may lead to new insights related to underlying disease mechanisms in AMD.

Quantifying Microvascular Changes Using OCT Angiography in Diabetic Eyes without Clinical Evidence of Retinopathy.

OBJECTIVE: To compare quantitative OCT angiography (OCTA) parameters of macular ischemia in diabetic eyes without retinopathy with those in healthy nondiabetic controls. DESIGN: Cross-sectional study from August 2014 through June 2017. SUBJECTS: Thirty-nine eyes of 39 diabetic patients without clinical evidence of diabetic retinopathy and 40 eyes of 40 healthy nondiabetic subjects. METHODS: Subjects underwent OCTA imaging using prototype AngioVue software (RTVue XR Avanti). Analyses of the foveal avascular zone (FAZ) and vasculature surrounding the FAZ were performed on the automatically generated en face OCTA images of the superficial and deep retinal vasculatures using vessel-based and FAZ-based metrics. MAIN OUTCOME MEASURES: Comparison of measurements made in the superficial and deep retinal capillary plexuses of diabetic eyes and normal eyes. RESULTS: FAZ-based analysis revealed statistically significant differences between diabetic and normal eyes in FAZ area (superficial and deep layers), perimeter (superficial layer), major axis length (superficial layer), and minor axis layer (superficial and deep layers). Vessel-based analysis revealed statistically significant differences in the binarized flow index (superficial and deep layers), both including and excluding the FAZ area. CONCLUSIONS: Quantitative OCTA parameters reveal subclinical macular ischemia at both the superficial and deep retinal capillary plexuses in diabetic eyes that do not manifest clinical retinopathy. Vessel-based and FAZ-based metrics applied to OCTA images may serve as effective tools for screening and disease monitoring in patients with diabetes without clinical evidence of retinopathy.

Polypoidal Choroidal Vasculopathy on Swept-Source Optical Coherence Tomography Angiography with Variable Interscan Time Analysis.

PURPOSE: To use a novel optical coherence tomography angiography (OCTA) algorithm termed variable interscan time analysis (VISTA) to evaluate relative blood flow speeds in polypoidal choroidal vasculopathy (PCV). METHODS: Prospective cross-sectional study enrolling patients with confirmed diagnosis of PCV. OCTA of the retina and choroid was obtained with a prototype swept-source OCT system. The acquired OCT volumes were centered on the branching vascular network (BVN) and polyps as determined by indocyanine-green angiography (ICGA). The relative blood flow speeds were characterized on VISTA-OCTA. RESULTS: Seven eyes from seven patients were evaluated. Swept-source OCTA enabled detailed enface visualization of the BVN and polyps in six eyes. VISTA-OCTA revealed variable blood flow speeds in different PCV lesion components of the same eye, with faster flow in the periphery of polyps and slower flow in the center of each polyp, as well as relatively slow flow in BVN when compared with retinal vessels. BVNs demonstrated relatively faster blood flow speeds in the larger trunk vessels and relatively slower speeds in the smaller vessels. CONCLUSIONS: Swept-source OCTA identifies polyps in most, but not all, PCV lesions. This limitation that may be related to relatively slow blood flow within the polyp, which may be below the OCTA's sensitivity. VISTA-OCTA showed heterogeneous blood flow speeds within the polyps, which may indicate turbulent flow in the polyps. TRANSLATIONAL RELEVANCE: These results bring relevant insights into disease mechanisms that can account for the variable course of PCV, and can be relevant for diagnosis and management of patients with PCV.

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Presenting With Branch Retinal Artery Occlusion.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare syndrome affecting the retinal and optic disc vasculature. Diffuse retinal ischemia, macular edema, and neovascularization may lead to bilateral vision loss. The authors report a case of a 36-year-old woman presenting with branch retinal artery occlusion (BRAO) in her right eye who was subsequently diagnosed with IRVAN syndrome. She was treated with panretinal photocoagulation for peripheral retinal ischemia and pars plana vitrectomy for vitreous hemorrhage. She later developed a BRAO in her left eye. This case demonstrates that BRAO may be a presenting feature of IRVAN syndrome. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:948-951.].

Ten-Year Follow-Up after Bilateral Submacular Neovascular Membrane Removal in a Case of Autosomal Recessive Bestrophinopathy.

Herein, we report the case of an 8-year-old girl who presented in December 2000 with a submacular neovascular membrane in the right eye, with a clinical diagnosis of Best disease. At that time, she underwent pars plana vitrectomy (PPV) with removal of the subretinal choroidal neovascularization (CNV). Her vision improved from 20/200 to 20/25. Four years later, a new CNV developed in the other eye. Initially, she underwent unsuccessful photodynamic therapy. As her vision worsened, she underwent a second, this time successful, PPV with membrane removal in the left eye, with vision improving to 20/30. Ten years later, she returned complaining of vision loss over the last year. Her vision was 20/200 OU, and optical coherence tomography demonstrated very large intraretinal cystoid spaces resembling bilateral macular schisis. Four ranibizumab injections as well as dorzolamide eye drops were tried, both without success. Finally, she underwent PPV with internal limiting membrane peeling and gas-fluid exchange in the left eye. One month later, the macula appeared flat and vision had improved to 20/60. The same procedure was performed 1 year later for the right eye, with vision improving to 20/80. One year later, mild cystic spaces developed again in both eyes, although much smaller than previously observed. Her vision remained stable.