Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 88
Filter
1.
Int J Retina Vitreous ; 9(1): 62, 2023 Oct 11.
Article in English | MEDLINE | ID: mdl-37822004

ABSTRACT

BACKGROUND: This study aimed to develop a deep learning (DL) algorithm that enhances the quality of a single-frame enface OCTA scan to make it comparable to 4-frame averaged scan without the need for the repeated acquisitions required for averaging. METHODS: Each of the healthy eyes and eyes from diabetic subjects that were prospectively enrolled in this cross-sectional study underwent four repeated 6 × 6 mm macular scans (PLEX Elite 9000 SS-OCT), and the repeated scans of each eye were co-registered to produce 4-frame averages. This prospective dataset of original (single-frame) enface scans and their corresponding averaged scans was divided into a training dataset and a validation dataset. In the training dataset, a DL algorithm (named pseudoaveraging) was trained using original scans as input and 4-frame averages as target. In the validation dataset, the pseudoaveraging algorithm was applied to single-frame scans to produce pseudoaveraged scans, and the single-frame and its corresponding averaged and pseudoaveraged scans were all qualitatively compared. In a separate retrospectively collected dataset of single-frame scans from eyes of diabetic subjects, the DL algorithm was applied, and the produced pseudoaveraged scan was qualitatively compared against its corresponding original. RESULTS: This study included 39 eyes that comprised the prospective dataset (split into 5 eyes for training and 34 eyes for validating the DL algorithm), and 105 eyes that comprised the retrospective test dataset. Of the total 144 study eyes, 58% had any level of diabetic retinopathy (with and without diabetic macular edema), and the rest were from healthy eyes or eyes of diabetic subjects but without diabetic retinopathy and without macular edema. Grading results in the validation dataset showed that the pseudoaveraged enface scan ranked best in overall scan quality, background noise reduction, and visibility of microaneurysms (p < 0.05). Averaged scan ranked best for motion artifact reduction (p < 0.05). Grading results in the test dataset showed that pseudoaveraging resulted in enhanced small vessels, reduction of background noise, and motion artifact in 100%, 82%, and 98% of scans, respectively. Rates of false-positive/-negative perfusion were zero. CONCLUSION: Pseudoaveraging is a feasible DL approach to more efficiently improve enface OCTA scan quality without introducing notable image artifacts.

2.
Ophthalmic Genet ; 43(3): 385-388, 2022 06.
Article in English | MEDLINE | ID: mdl-34889688

ABSTRACT

BACKGROUND: Papillorenal syndrome is an autosomal dominant disorder associated with mutations in the gene PAX2 and often presents with characteristic and specific optic disc findings, frequently with renal dysplasia. In at least half of cases, an identifiable mutation in the PAX2 gene can be detected. We report the ocular findings in a second case of papillorenal syndrome with the c.350 G > C (p.Arg117Pro) mutation detected within the PAX2 gene. METHODS: A case report of papillorenal syndrome due to PAX2 mutation. Complete ophthalmologic examination was performed as well as color fundus photography, fundus autofluorescence, and optical coherence tomography (OCT). Genetic testing was performed using a next-generation sequencing with CNV calling (NGS-CNV) panel test containing 55 genes associated with nephrotic syndrome or focal segmental glomerulosclerosis. RESULTS: An 11-year-old boy who presented with hypertension and proteinuria was found to have stage IV chronic kidney disease. Presenting visual acuity was 20/25 in the right eye and 20/20 in the left eye. The fundus exam showed bilateral centrally excavated optic discs with absent central retinal vessels and a compensatory multiplicity of cilioretinal vessels, characteristic and specific for papillorenal syndrome. OCT showed outer retinal atrophy and macular schisis. Genetic testing identified the likely pathogenic c.350 G > C (p.Arg117Pro) mutation in PAX2. CONCLUSIONS: We report the first description, to our knowledge, of the clinical presentation, ocular and systemic findings, and ophthalmic imaging in an individual with papillorenal syndrome associated with the PAX2 c.350 G > C (p.Arg117Pro) mutation. Our case adds to the current understanding of papillorenal syndrome and demonstrates that this condition is associated with a pathognomonic optic disc appearance and significant renal disease.


Subject(s)
Coloboma , Optic Disk , Coloboma/complications , Coloboma/diagnosis , Coloboma/genetics , Humans , Mutation , Optic Disk/pathology , PAX2 Transcription Factor/genetics , Phenotype , Renal Insufficiency , Vesico-Ureteral Reflux
3.
Ophthalmic Surg Lasers Imaging Retina ; 52(8): 450-453, 2021 08.
Article in English | MEDLINE | ID: mdl-34410188

ABSTRACT

The authors describe identical twin sisters with similar symptoms, fundus findings, and ophthalmic testing consistent with retinitis punctata albescens (RPA). The pathognomonic white retinal flecks, extensive midperipheral retinal pigment epithelium atrophy, and pigmentary changes demonstrate overlapping phenotypic properties of RPA and other inherited retinal degenerative diseases. Genetic testing confirmed two mutations of the RLBP1 gene: one pathogenic for RPA, and the other a variant of uncertain significance previously reported as a novel RPA mutation in the literature. This report highlights identical phenotypic findings and genetic mutations in twin sisters aiding in further classification of pathogenic mutations associated with RPA. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:450-453.].


Subject(s)
Carrier Proteins/genetics , Retinal Degeneration , Adult , Electroretinography , Female , Humans , Mutation , Retinal Diseases
4.
Curr Opin Ophthalmol ; 32(3): 247-252, 2021 May 01.
Article in English | MEDLINE | ID: mdl-33741759

ABSTRACT

PURPOSE OF REVIEW: Age-related macular degeneration (AMD) affects a significant percentage of the elderly population and end-stage disease classified by either geographic atrophy (GA) or neovascular AMD (nvAMD) is one of the leading causes of vision loss worldwide. Despite the fact that there are currently treatments for nvAMD, there are no treatments in practice to prevent disease onset or progression of GA. This topic is at the forefront of ophthalmic research demonstrated by the recent advances in disease characterization, genetic and environmental risk factor classification, biomarker discovery and mechanism of pathogenesis categorization. There are also numerous clinical treatment trials underway, targeting proposed pathways and biomarkers associated with GA that are promising. RECENT FINDINGS: With several clinical trials of potential treatments underway and numerous recent publications on disease diagnosis and classification, the understanding of GA pathogenesis has increased substantially. Although the exact mechanism of pathology is still elusive, recent literature has highlighted the utilization of current and new ophthalmic imaging modalities and discovery of objective and functional markers that can lead to earlier diagnosis and treatment. SUMMARY: Herein, we will provide an overview and discussion of the current status of GA including advances in mechanism of pathogenesis, diagnosis, classification and current treatment modalities.


Subject(s)
Geographic Atrophy , Geographic Atrophy/classification , Geographic Atrophy/diagnosis , Geographic Atrophy/etiology , Geographic Atrophy/therapy , Humans
5.
Ther Adv Respir Dis ; 14: 1753466620951053, 2020.
Article in English | MEDLINE | ID: mdl-32873175

ABSTRACT

Infection with novel SARS-CoV-2 carries significant morbidity and mortality in patients with pulmonary compromise, such as lung cancer, autoimmune disease, and pneumonia. For early stages of mild to moderate disease, care is entirely supportive.Antiviral drugs such as remdesivir may be of some benefit but are reserved for severe cases given limited availability and potential toxicity. Repurposing of safer, established medications that may have antiviral activity is a possible approach for treatment of earlier-stage disease. Tetracycline and its derivatives (e.g. doxycycline and minocycline) are nontraditional antibiotics with a well-established safety profile, potential efficacy against viral pathogens such as dengue fever and chikungunya, and may regulate pathways important in initial infection, replication, and systemic response to SARS-CoV-2. We present a series of four high-risk, symptomatic, COVID-19+ patients, with known pulmonary disease, treated with doxycycline with subsequent rapid clinical improvement. No safety issues were noted with use of doxycycline.Doxycycline is an attractive candidate as a repurposed drug in the treatment of COVID-19 infection, with an established safety profile, strong preclinical rationale, and compelling initial clinical experience described here.The reviews of this paper are available via the supplemental material section.


Subject(s)
Adenocarcinoma of Lung/complications , Coronavirus Infections/drug therapy , Doxycycline/administration & dosage , Pneumonia, Viral/drug therapy , Pulmonary Disease, Chronic Obstructive/complications , Sarcoidosis, Pulmonary/complications , Adenocarcinoma of Lung/diagnosis , Adenocarcinoma of Lung/therapy , Adult , Aged , Aged, 80 and over , COVID-19 , Comorbidity , Coronavirus Infections/complications , Coronavirus Infections/diagnosis , Female , Follow-Up Studies , Humans , Male , Middle Aged , Multimorbidity , Pandemics , Pneumonia, Viral/complications , Pneumonia, Viral/diagnosis , Pulmonary Disease, Chronic Obstructive/diagnosis , Pulmonary Disease, Chronic Obstructive/therapy , Risk Assessment , Sampling Studies , Sarcoidosis, Pulmonary/diagnosis , Sarcoidosis, Pulmonary/therapy , Treatment Outcome
6.
Article in English | MEDLINE | ID: mdl-32377380

ABSTRACT

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.

7.
Article in English | MEDLINE | ID: mdl-32206342

ABSTRACT

BACKGROUND: The purpose of this study was to investigate the association between diabetic retinopathy (DR) severity and macular choriocapillaris (CC) flow deficit percentage (FD %) in different macular regions using swept-source optical coherence tomography angiography (SS-OCTA). METHODS: Diabetic patients with SS-OCTA images were graded by severity and retrospectively assessed. CC FD % was calculated in four different regions of the OCTA image: inner, middle, outer, and full-field region. The generalized estimating equations (GEE) approach for clustered eye data was used to determine effect size and significance of age and disease severity on FD % for each region. RESULTS: 160 eyes from 90 total diabetic patients met inclusion criteria. Out of 90 patients, 33 had no DR, 17 had mild nonproliferative DR (NPDR), 8 had moderate NPDR, 10 had severe NPDR and 22 had proliferative DR. Age and DR severity had a significant positive association with FD % for each region studied with a greater effect in the two centermost regions. The increase in flow deficit percentage per year of age by region was: inner 0.12 (p < 0.001), middle 0.09 (p < 0.001), outer 0.05 (p < 0.001, full-field 0.06 (p < 0.001). The increase in flow deficit percentage per increase in diabetic retinopathy severity stage by region was: inner 0.65 (p < 0.0087), middle 0.56 (p < 0.0012), outer 0.33 (p < 0.045), full-field 0.36 (p < 0.018). CONCLUSIONS: Topographic analysis of the CC FD % in diabetic eyes suggests that CC flow impairment corresponds to DR severity, with all studied regions of the CC significantly affected. There was greater regional impairment due to age and disease severity in the inner and middle regions.

8.
Retina ; 40(3): 412-420, 2020 Mar.
Article in English | MEDLINE | ID: mdl-30576300

ABSTRACT

PURPOSE: To combine advances in high-speed, wide-field optical coherence tomography angiography (OCTA) with image processing methods for semiautomatic quantitative analysis of capillary nonperfusion in patients with diabetic retinopathy (DR). METHODS: Sixty-eight diabetic patients (73 eyes), either without retinopathy or with different degrees of retinopathy, were prospectively recruited for volumetric swept-source OCTA imaging using 12 mm × 12 mm fields centered at the fovea. A custom, semiautomatic software algorithm was used to quantify areas of capillary nonperfusion. RESULTS: The mean percentage of nonperfused area was 0.1% (95% confidence interval: 0.0-0.4) in the eyes without DR; 2.1% (95% confidence interval: 1.2-3.7) in the nonproliferative DR eyes (mild, moderate, and severe), and 8.5% (95% confidence interval: 5.0-14.3) in the proliferative DR eyes. The percentage of nonperfused area increased in a statistically significant manner from eyes without DR, to eyes with nonproliferative DR, to eyes with proliferative DR. CONCLUSION: Capillary nonperfusion area in the posterior retina increases with increasing DR severity as measured by swept-source OCTA. Quantitative analysis of retinal nonperfusion on wide-field OCTA may be useful for early detection and monitoring of disease in patients with diabetes and DR.


Subject(s)
Diabetes Mellitus, Type 2/complications , Diabetic Retinopathy/diagnosis , Fluorescein Angiography/methods , Regional Blood Flow/physiology , Retinal Vessels/pathology , Tomography, Optical Coherence/methods , Capillaries/pathology , Diabetic Retinopathy/etiology , Diabetic Retinopathy/physiopathology , Female , Follow-Up Studies , Fundus Oculi , Humans , Male , Middle Aged , Retinal Vessels/physiopathology , Retrospective Studies
9.
Sci Rep ; 9(1): 9096, 2019 06 24.
Article in English | MEDLINE | ID: mdl-31235795

ABSTRACT

The recent clinical adoption of optical coherence tomography (OCT) angiography (OCTA) has enabled non-invasive, volumetric visualization of ocular vasculature at micron-scale resolutions. Initially limited to 3 mm × 3 mm and 6 mm × 6 mm fields-of-view (FOV), commercial OCTA systems now offer 12 mm × 12 mm, or larger, imaging fields. While larger FOVs promise a more complete visualization of retinal disease, they also introduce new challenges to the accurate and reliable interpretation of OCTA data. In particular, because of vignetting, wide-field imaging increases occurrence of low-OCT-signal artifacts, which leads to thresholding and/or segmentation artifacts, complicating OCTA analysis. This study presents theoretical and case-based descriptions of the causes and effects of low-OCT-signal artifacts. Through these descriptions, we demonstrate that OCTA data interpretation can be ambiguous if performed without consulting corresponding OCT data. Furthermore, using wide-field non-perfusion analysis in diabetic retinopathy as a model widefield OCTA usage-case, we show how qualitative and quantitative analysis can be confounded by low-OCT-signal artifacts. Based on these results, we suggest methods and best-practices for preventing and managing low-OCT-signal artifacts, thereby reducing errors in OCTA quantitative analysis of non-perfusion and improving reproducibility. These methods promise to be especially important for longitudinal studies detecting progression and response to therapy.


Subject(s)
Angiography , Artifacts , Tomography, Optical Coherence , Humans , Image Processing, Computer-Assisted , Signal-To-Noise Ratio
10.
Ophthalmic Surg Lasers Imaging Retina ; 49(10): S5-S17, 2018 10 15.
Article in English | MEDLINE | ID: mdl-30339262

ABSTRACT

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.].


Subject(s)
Fluorescein Angiography/methods , Fovea Centralis/blood supply , Retinal Vessels/diagnostic imaging , Tomography, Optical Coherence/methods , Adult , Female , Follow-Up Studies , Fundus Oculi , Healthy Volunteers , Humans , Male , Prospective Studies , Reproducibility of Results
11.
Ophthalmol Retina ; 2(10): 1028-1040, 2018 10.
Article in English | MEDLINE | ID: mdl-31047490

ABSTRACT

PURPOSE: To investigate the efficacy of intravenous GSK933776, a humanized monoclonal antibody directed against the N-terminal amino acids of amyloid ß, for the treatment of geographic atrophy (GA) in age-related macular degeneration (AMD). DESIGN: Prospective, randomized, placebo-controlled, double-masked, multicenter phase 2 clinical trial. PARTICIPANTS: Patients with GA secondary to AMD, a visual acuity score of at least 35 letters, and GA with a total area of 1.9 to 17 mm2 were enrolled. METHODS: Participants were monitored monthly for 4 months during an observation period to determine the rate of GA enlargement in the study eye. After the observation period, randomization was performed into 1 of 4 treatment arms (GSK933776 at 3, 6, and 15 mg/kg/month and placebo). At each monthly visit over 18 months, participants underwent visual acuity testing under normal luminance and low-luminance conditions. Ocular imaging included color fundus photography, fundus autofluorescence, fluorescein angiography, and spectral-domain OCT. MAIN OUTCOME MEASURE: Enlargement in the area of GA measured from color fundus photographs with reference to fundus autofluorescence images. RESULTS: A total of 191 participants were randomized into the study, with 139 (73%) fulfilling the efficacy population criteria. Over 18 months, GSK933776 did not reduce the rate of GA enlargement relative to placebo. Overall, there were no consistent meaningful differences relative to placebo in any of the visual function measures. There was a correlation between the low-luminance visual acuity (LLVA) deficit at baseline and the rate of GA enlargement. Genetic variations in complement factor I (CFI) gene did not correlate with GA progression. No ocular serious adverse events considered related to the GSK933776 treatment were identified, and a similar number of nonocular serious adverse events were reported across all treatment groups. CONCLUSIONS: Intravenous amyloid ß inhibition with GSK933776 did not slow the rate of GA enlargement compared with placebo, and no clinically meaningful differences relative to placebo were observed in visual function testing over 18 months. The LLVA deficit was associated with faster GA enlargement; however, no correlation was shown between genetic variations in the CFI gene and the rate of GA enlargement.

13.
Ophthalmol Retina ; 2(5): 418-427, 2018 May.
Article in English | MEDLINE | ID: mdl-30820483

ABSTRACT

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.

14.
Article in English | MEDLINE | ID: mdl-28373914

ABSTRACT

BACKGROUND: Despite anti-VEGF therapy, some patients develop chronic diabetic macular edema. The objective of this study was to evaluate anatomic and visual outcomes of switching patients with chronic DME from intravitreal bevacizumab or ranibizumab to intravitreal aflibercept injection. METHODS: In this retrospective observational case series, 11 eyes with recalcitrant diabetic macular edema (DME) were evaluated 6 months prior to and 6 months following initial intravitreal aflibercept injection (IAI). Recalcitrant DME was defined as having a thickened retina (≥350 µm) on spectral domain optical coherence tomography (SD-OCT) with persistent cystic changes (less than a 15% reduction in central retinal thickness) over 6 months prior to intravitreal aflibercept switch despite aggressive treatment for DME during this time. RESULTS: One hundred and forty-seven patients in total were treated with IAI during this time, and of these, 31 patients were treated with IAI for DME. 18 eyes had less than 4 treatments within the 6 months prior to switch to IAI, 6 patients had a central retinal thickness (CRT) on SD-OCT of less than 350 µm at time of switch to IAI, and 2 patients had a greater than 15% decline in CRT on SD-OCT over the 6 months prior to switch to IAI. A total of 11 patients were included in the study. Over the 6 months prior to switch, the mean change in central retinal thickness was +18.6% and over the 6 months following switch to aflibercept the mean change in central retinal thickness was -27.1%. Switching to a regimen of at least 3 intravitreal aflibercept injections over 6 months resulted in some anatomic improvement and improvement or stabilization of Snellen visual acuity in all eligible patients. CONCLUSIONS: In patients with recalcitrant diabetic macular edema, switching to intravitreal aflibercept resulted in improved a 25% or more decrease in central retinal thickness in 81% (9/11) patients at 6-month follow-up. Sixty-three percent (7/11) had improvement in Snellen visual acuity after switching to intravitreal aflibercept injection, suggesting some reversibility of functional damage.

15.
Retin Cases Brief Rep ; 11(1): 83-85, 2017.
Article in English | MEDLINE | ID: mdl-26934302

ABSTRACT

PURPOSE: To report a previously unreported presentation of advanced geographic atrophy of the macula mimicking nonneovascular (dry) age-related macular degeneration in a patient with light chain deposition disease. METHODS: Ocular examination included dilated fundus examination, fundus autofluorescence, full-field electroretinography, and spectral domain optical coherence tomography. PATIENTS: Single-patient case report. RESULTS: Dilated fundus examination demonstrated diffuse loss of the retinal pigment epithelium in a geographic atrophy pattern in the macula and drusenlike deposits localized to the outer retina and retinal pigment epithelium. There were no signs of choroidal neovascularization or retinal pigment epithelium detachments. Fundus autofluorescence demonstrated wide areas of retinal pigment epithelium loss. Full-field electroretinography was normal. Spectral domain optical coherence tomography displayed atrophy of the outer retinal layers. DISCUSSION: This is the first documented case of drusenlike deposits and maculopathy in a patient with light chain deposition disease that mimics advanced geographic atrophy that is typically observed in nonneovascular age-related macular degeneration. Physicians should be aware of the macular changes that can be associated with light chain deposition disease, and patients with light chain deposition disease should be regularly evaluated for associated macular disease.


Subject(s)
Geographic Atrophy/pathology , Immunoglobulin kappa-Chains/metabolism , Macular Degeneration/pathology , Nephrotic Syndrome/complications , Female , Humans , Middle Aged , Vision Disorders/etiology
16.
Article in English | MEDLINE | ID: mdl-27847636

ABSTRACT

PURPOSE: To report expanded SD-OCT findings of HCQ retinopathy that may assist the clinician in earlier diagnosis. To characterize structural changes of HCQ retinopathy with SD-OCT after drug cessation. METHODS: Setting: Private practice and academic institution. Patient Population: Patients at New England Eye Center and Ophthalmic Consultants of Boston in Boston, MA diagnosed with HCQ retinopathy and followed after drug cessation. Retrospective clinical data review by the Boston Image Reading Center. Main Outcome Measures: SD-OCT findings suggestive of HCQ retinopathy before parafoveal ellipsoid disruption. Change in SD-OCT morphological appearance and retinal thickness of each of the nine subfields corresponding to the Early Treatment of Diabetic Retinopathy Study areas. RESULTS: Thirty eyes with HCQ retinopathy were followed with SD-OCT after drug cessation. Findings before disruption of the parafoveal EZ included parafoveal outer nuclear layer (ONL) thinning, disruption of the parafoveal interdigitation zone, and reduced reflectivity of the parafoveal EZ. In early toxicity, 75 % developed progression after drug cessation, including disruption of the parafoveal EZ and retinal pigment epithelium and thinning of the ONL. Eyes with obvious toxicity had greater inferior outer ring thinning 12 months after drug cessation compared to early toxicity (p = 0.002, 95 % CI -2 to -8 µm). In obvious toxicity, the nasal inner subfield showed more thinning than the temporal inner subfield at 12 months after drug cessation (p = 0.018, 95 % CI -1 to -8 µm). CONCLUSIONS: Once HCQ retinopathy is diagnosed and the medication is discontinued, structural retinal changes commonly occur.

17.
Article in English | MEDLINE | ID: mdl-27847640

ABSTRACT

BACKGROUND: To investigate the choroidal thickness in older patients with central serous chorioretinopathy (CSCR) compared to age-matched normal subjects. METHODS: Fifteen patients (30 eyes) with CSCR, all aged ≥60 years, and 21 age-matched normal subjects (21 eyes) underwent high-definition raster scanning using SD-OCT. Both eyes from CSCR patients were included in the analysis. The eyes in patients with CSCR were divided into two groups: active CSCR (17 eyes) if there was foveal-involving subretinal fluid and inactive contralateral eye group (13 eyes). Choroidal thickness was measured from the posterior edge of the retinal pigment epithelium to the choroidal-scleral junction at 500 µm intervals up to 2500 µm temporal and nasal to the fovea (11 locations). RESULTS: The mean age of the patients with CSCR was 68.87 ± 6.83 years (mean ± standard deviation). Reliable measurements of choroidal thickness were obtainable in 70.6 % of eyes examined. The choroid was statistically significantly thicker in eyes with both active CSCR (P < 0.001) and inactive contralateral eyes (P < 0.01) when compared to normal age-matched eyes. The subfoveal choroid was 95 µm (P < 0.01) thicker in eyes with active CSCR (338.05 ± 31.42 µm) compared with normal eyes (243.05 ± 13.39 µm). The subfoveal choroid thickness in the inactive contralateral eyes was numerically greater than normal, and it was not statistically significantly thicker compared to the normal eyes (difference-55.68 µm, P > 0.05). CONCLUSION: Choroid in older patients with active CSCR was thicker than the choroid in age-matched normal eyes. It is important to consider CSCR as a differential diagnosis of serous retinal detachment in elderly patients with thickened choroid and to consider SD-OCT as an imaging modality by which to evaluate the choroidal thickness.

18.
Invest Ophthalmol Vis Sci ; 57(9): OCT486-94, 2016 07 01.
Article in English | MEDLINE | ID: mdl-27442342

ABSTRACT

PURPOSE: To evaluate the perifoveolar retinal capillary network at different depths and to quantify the foveal avascular zone (FAZ) in eyes with retinal vein occlusion (RVO) compared with their fellow eyes and healthy controls using spectral-domain optical coherence tomography angiography (SD-OCTA). METHODS: We prospectively recruited 23 patients with RVO including 15 eyes with central RVO (CRVO) and 8 eyes with branch RVO (BRVO), their fellow eyes, and 8 age-matched healthy controls (8 eyes) for imaging on prototype OCTA software within RTVue-XR Avanti. The 3 × 3 mm and 6 × 6 mm en face angiograms of superficial and deep retinal capillary plexuses were segmented. Perifoveolar retinal capillary network was analyzed and FAZ was quantified. RESULTS: Decrease in vascular perfusion at the deep plexus was observed in all eyes with CRVO (8/8, 100%) and BRVO (6/6, 100%) without cystoid macular edema, and in 8 of 15 (53%) and 2 of 8 (25%) of the fellow eyes, respectively. Vascular tortuosity was observed in 13 of 15 (87%) CRVO and 5 of 8 (63%) BRVO eyes. Collaterals were seen in 10 of 15 (67%) CRVO and 5 of 8 (63%) BRVO eyes. Mean FAZ area was larger in eyes with RVO than their fellow eyes (1.13 ± 0.25 mm2 versus 0.58 ± 0.28 mm2; P = 0.007) and controls (1.13 ± 0.25 mm2 versus 0.30 ± 0.09 mm2; P < 0.0001), and in fellow eyes of RVO patients when compared to controls (0.58 ± 0.28 mm2 versus 0.30 ± 0.09 mm2; P = 0.01). CONCLUSIONS: Spectral-domain OCTA reveals abnormalities at different levels of perifoveolar retinal capillary network and is able to quantify the FAZ in RVO. Longitudinal studies may be considered to evaluate the clinical utility of OCTA in RVO and other retinal vascular diseases.


Subject(s)
Fluorescein Angiography/methods , Fovea Centralis/pathology , Microcirculation/physiology , Regional Blood Flow/physiology , Retinal Vein Occlusion/diagnosis , Retinal Vessels/physiopathology , Tomography, Optical Coherence/methods , Aged , Capillaries/pathology , Female , Follow-Up Studies , Fundus Oculi , Humans , Male , Middle Aged , Prospective Studies , Retinal Vein Occlusion/physiopathology , Retinal Vessels/pathology , Visual Acuity
19.
Retina ; 36(12): 2265-2273, 2016 Dec.
Article in English | MEDLINE | ID: mdl-27285456

ABSTRACT

PURPOSE: To characterize the features of choroidal neovascularization (CNV) in neovascular age-related macular degeneration with spectral domain optical coherence tomography angiography (OCTA) and to determine whether OCTA can be used to determine clinical activity of CNV. METHODS: Observational, retrospective, consecutive case series. RESULTS: Optical coherence tomography angiography revealed CNV in 28 eyes (62.2%) while 17 eyes (37.8%) did not demonstrate CNV vessels. Choroidal neovascularization was classified as well circumscribed in 12 eyes (42.8%) and poorly circumscribed in 16 eyes (57.2%). Twenty-two eyes with a CNV on OCTA were clinically active, whereas six eyes with visible CNV on OCTA were clinically inactive. Of the 17 eyes that did not have evidence of CNV on OCTA imaging, 14 were clinically inactive and 3 were clinically active. Presence of CNV on OCTA correlated with clinical activity and absence of CNV correlated with inactivity (P < 0.0001). CONCLUSION: Optical coherence tomography angiography is a noninvasive imaging technique that can be used to visualize blood flow comprising CNV. Optical coherence tomography angiography detects CNV vessels in some albeit not all eyes with neovascular age-related macular degeneration. Although the presence or absence of CNV vessels on OCTA highly correlated with clinical activity of CNV, the morphologic appearance of CNV on OCTA did not have significant correlation with clinical activity.


Subject(s)
Choroidal Neovascularization/diagnostic imaging , Fluorescein Angiography , Tomography, Optical Coherence/methods , Wet Macular Degeneration/pathology , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Retrospective Studies , Wet Macular Degeneration/diagnostic imaging
20.
Ophthalmic Surg Lasers Imaging Retina ; 47(6 Suppl): S4-S14, 2016 06.
Article in English | MEDLINE | ID: mdl-27348433

ABSTRACT

Diabetic retinopathy (DR) is the leading cause of blindness among working-age adults. DR often leads to diabetic macular edema (DME), which often goes unnoticed until a patient presents with vision loss. However, treatment options and data for DME are continually improving. We know that vascular endothelial growth factor (VEGF) plays a key role in DME progression; therapies that act by inhibiting VEGF production seem to improve visual acuity in patients with DME. Of the anti-VEGF therapies available, two have been approved by the U.S. Food and Drug Administration to treat DME: ranibizumab (Lucentis; Genentech, South San Francisco, CA) and aflibercept (Eylea; Regeneron, Tarrytown, NY). Bevacizumab (Avastin; Genentech, South San Francisco, CA), which is approved for the treatment of certain types of cancer, is occasionally used off-label to treat DME. Anti-VEGF therapy can stop vision loss and even improve visual acuity. Other treatments remain effective, and these various treatment options fuel a need for new data and discussion. This roundtable discussion, which took place during the 2015 annual meeting of the American Academy of Ophthalmology, outlines the current protocols used to treat DME and provides clinical opinions about selecting and treating with an appropriate anti-VEGF therapy. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:S5-14.].


Subject(s)
Angiogenesis Inhibitors/therapeutic use , Diabetic Retinopathy/drug therapy , Macular Edema/drug therapy , Vascular Endothelial Growth Factor A/antagonists & inhibitors , Bevacizumab , Humans , Intravitreal Injections , Ranibizumab , Receptors, Vascular Endothelial Growth Factor , Recombinant Fusion Proteins , Visual Acuity/drug effects
SELECTION OF CITATIONS
SEARCH DETAIL
...