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

Kaden, Talia R; Gattoussi, Sarra; Dolz-Marco, Rosa; Balaratnasingam, Chandrakumar; Yannuzzi, Lawrence A; Freund, K Bailey
BACKGROUND AND OBJECTIVE/OBJECTIVE:To describe the prevalence and anatomic correlates for hyperautofluorescence related to outer retinal disruption in eyes with multifocal choroiditis (MFC). PATIENTS AND METHODS/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.].
PMID: 31755966
ISSN: 2325-8179
CID: 4209422

Early hydroxychloroquine retinopathy: optical coherence tomography abnormalities preceding Humphrey visual field defects

Garrity, Sean T; Jung, Joo Yeon; Zambrowski, Olivia; Pichi, Francesco; Su, Daniel; Arya, Malvika; Waheed, Nadia K; Duker, Jay S; Chetrit, Yaïr; Miserocchi, Elisabetta; Giuffrè, Chiara; Kaden, Talia R; Querques, Giuseppe; Souied, Eric H; Freund, K Bailey; Sarraf, David
BACKGROUND/AIMS/OBJECTIVE:Hydroxychloroquine (HCQ) retinopathy may result in severe and irreversible vision loss, emphasising the importance of screening and early detection. The purpose of this study is to report the novel finding of early optical coherence tomography (OCT) abnormalities due to HCQ toxicity that may develop in the setting of normal Humphrey visual field (HVF) testing. METHODS:Data from patients with chronic HCQ exposure was obtained from seven tertiary care retina centres. Ten patients with HCQ-associated OCT abnormalities and normal HVF testing were identified. Detailed analysis of the OCT findings and ancillary tests including colour fundus photography, fundus autofluorescence, multifocal electroretinography and microperimetry was performed in these patients. RESULTS:Seventeen eyes from 10 patients illustrated abnormalities with OCT and normal HVF testing. These OCT alterations included (1) attenuation of the parafoveal ellipsoid zone and (2) loss of a clear continuous interdigitation zone. Several eyes progressed to advanced parafoveal outer retinal disruption and/or paracentral visual field defects. CONCLUSION/CONCLUSIONS:Patients with high risk HCQ exposure and normal HVF testing may develop subtle but characteristic OCT abnormalities. This novel finding indicates that, in some cases of early HCQ toxicity, structural alterations may precede functional impairment. It is therefore important to employ a screening approach that includes OCT to assess for these early findings. Ancillary testing should be considered in cases with suspicious OCT changes and normal HVFs.
PMID: 30819690
ISSN: 1468-2079
CID: 3722212

Correlation between Histologic and OCT Angiography Analysis of Macular Circulation

Balaratnasingam, Chandrakumar; An, Dong; Freund, K Bailey; Francke, Ashley; Yu, Dao-Yi
PMID: 31227322
ISSN: 1549-4713
CID: 3939532

Differentiating veins from arteries on optical coherence tomography angiography by identifying deep capillary plexus vortices

Xu, Xiaoyu; Yannuzzi, Nicolas A; Fernández-Avellaneda, Pedro; Echegaray, Jose J; Tran, Kimberly D; Russell, Jonathan F; Patel, Nimesh A; Hussain, Rehan M; Sarraf, David; Freund, K Bailey
PURPOSE/OBJECTIVE:To introduce a simple method for differentiating retinal veins from arteries on optical coherence tomography angiography (OCTA). DESIGN/METHODS:Cross-sectional pilot study. METHODS:Four default en face slabs including color depth encoded, greyscale full-thickness retina, superficial plexus, and deep capillary plexus (DCP) from 9 3x3 mm and 9 6x6 mm OCTA scans were exported and aligned. Nine ophthalmologists with minimum OCTA experience from 2 eye institutions were instructed to classify labeled vessels as arteries or veins in 3 stages. Classification was performed based on graders' own assessment at Stage 1. Graders were taught that a capillary-free zone was an anatomic feature of arteries at Stage 2 and were trained to identify veins originating from vortices within the DCP at Stage 3. Grading accuracy was analyzed and correlated with grading time and graders' years in practice. RESULTS:Overall grading accuracy in Stages 1, 2, and 3 was (50.4 ± 17.0) %, (75.4 ± 6.0) %, and (94.7 ± 2.6) %, respectively. Grading accuracy for 3x3 mm scans in Stages 1, 2, and 3 was (49.9 ± 16.3) %, (79.2 ± 9.6) %, and (96.9 ± 3.1) %, respectively. Accuracy for 6x6 mm scans in Stages 1, 2, and 3 was (51.4 ± 20.8) %, (72.3 ± 7.9) %, and (93.2 ± 3.3) %, respectively. Grading performance improved significantly at each stage (all P < .001). No significant correlation was found between accuracy and time spent grading or between accuracy and years in practice (r = -0.164 - 0.617, all P ≥ 0.077). CONCLUSIONS:We describe a simple method for accurately distinguishing retinal arteries from veins on OCTA which incorporates the use vortices in the DCP to identify venous origin.
PMID: 31226248
ISSN: 1879-1891
CID: 3954702

Analysis of Hyperreflective Dots Within the Central Fovea in Healthy Eyes Using En Face Optical Coherence Tomography

Corradetti, Giulia; Au, Adrian; Borrelli, Enrico; Xu, Xiaoyu; Freund, K Bailey; Sarraf, David
Purpose/UNASSIGNED:The purpose of this retrospective study was to describe and quantify superficial hyperreflective dots within the central fovea and correlate them with age, using en face and cross-sectional B-scan optical coherence tomography (OCT). Methods/UNASSIGNED:Healthy eyes, evaluated with a spectral domain instrument (primary cohort) at the Stein Eye Institute (UCLA) and with a swept source instrument (secondary cohort) at the Vitreous Retina Macula Consultants of New York, were included in this study. En face OCT images (3 × 3 mm) segmented at the level of the superior vascular plexus were acquired, and hyperreflective dots in the foveal avascular zone were quantified by two different methodologies. The threshold reflectivity methodology quantified these dots on a cropped en face OCT image using ellipsoid zone mean reflectivity as the threshold cutoff. The OCT B-scan methodology consisted of a manual count of elevated hyperreflective signals on B-scans that colocalized with the dots by en face OCT. Primary outcome was to quantify these dots and correlate them with age. Results/UNASSIGNED:A total of 44 healthy eyes were evaluated in the primary cohort, and 16 healthy eyes were evaluated in the secondary cohort. The hyperreflective dots steadily increased in number, especially in patients older than 50 years of age, with a strongly positive statistical significant correlation, using both quantitative strategies. Conclusions/UNASSIGNED:Remarkable superficial hyperreflective dots in the central fovea of healthy subjects are novel anatomical findings that may be readily identified with both en face and cross-sectional OCT and steadily increase in number with age. We propose that these dots may represent a normal anatomical landmark, such as Müller cell end feet or inner limiting membrane basal lamina.
PMID: 31652329
ISSN: 1552-5783
CID: 4161892

Filigree Vascular Pattern in Combined Hamartoma of Retina and Retinal Pigment Epithelium on OCT Angiography

Gupta, Rajan; Pappuru, Rajeev R; Fung, K Adrian T; Lupidi, Marco; Kaliki, Swathi; Yannuzzi, Lawrence; Freund, K Bailey; Reid, Kate; Zur, Dinah; Iglicki, Matias; Chhablani, Jay
PURPOSE/OBJECTIVE:To evaluate the vascular structure within combined hamartoma of retina and retinal pigment epithelium (CHRRPE) lesions using OCT angiography (OCTA). DESIGN/METHODS:Multicenter, retrospective, observational analysis, PARTICIPANTS: Twenty eyes of patients diagnosed with CHRRPE. METHODS:Retrospective analysis of color fundus photographs, OCT, and OCTA of 20 eyes with CHRRPE. Morphologic characteristics of CHRRPE and the OCT features were correlated with the density of the filigree vascular pattern and with the published histopathologic findings of CHRRPE lesions. MAIN OUTCOME MEASURE/METHODS:Density of flow signals, that is, the filigree vascular pattern seen on OCTA in the deep capillary plexus, graded as high (>20), intermediate (10-20), or low (<10). RESULTS:Of 20 lesions, 11 were peripapillary, 8 were macular, and 1 was equatorial in location. A high density of filigree vascular pattern was observed in most peripapillary CHRRPE lesions, which also showed full-thickness retinal involvement (8/10). A low density of filigree pattern was seen in macular lesions, which showed partial-thickness retinal involvement and preretinal fibrosis (5/6). CONCLUSIONS:A filigree vascular pattern on OCTA is seen in CHRRPE lesions. High density of this pattern is noted in CHRRPE lesions with a peripapillary location, full-thickness retinal disorganization, and minimal preretinal fibrosis. These findings correlate well with published histopathologic findings of CHRRPE lesions both in terms of topographic and morphologic features. OCT angiography provides a promising method for further study of these lesions.
PMID: 31229419
ISSN: 2468-7219
CID: 3954842

SPECKLED HYPOAUTOFLUORESCENCE AS A SIGN OF RESOLVED SUBRETINAL HEMORRHAGE IN NEOVASCULAR AGE-RELATED MACULAR DEGENERATION

Hussnain, S Amal; Dolz-Marco, Rosa; Dunaief, Joshua L; Curcio, Christine A; Freund, K Bailey
PURPOSE/OBJECTIVE:To describe patterns of hypoautofluorescence in eyes with neovascular age-related macular degeneration occurring after subretinal hemorrhage. METHODS:This was a retrospective descriptive analysis of neovascular age-related macular degeneration eyes presenting with subretinal hemorrhage over the last 5 years that underwent serial multimodal imaging. A review of color fundus photographs, fundus autofluorescence, near-infrared reflectance, and optical coherence tomography was performed at baseline and all available follow-up visits to document the course and evolution of subretinal hemorrhage in these eyes. RESULTS:Eleven eyes of 10 patients (9 female, 1 male; mean age: 84.1 years, range: 72-99 years) with a mean follow-up of 19.8 months (range: 3-68 months) were included. Color fundus photographs showed subretinal hemorrhage that resolved over a mean of 5.5 months. During and after hemorrhage resolution, all eyes showed hypoautofluorescence, which appeared distinct from that due to retinal pigment epithelium loss. Discrete multifocal punctate hyperpigmented lesions were observed in 90% of eyes and were markedly hypoautofluorescent, producing a speckled pattern on fundus autofluorescence. CONCLUSION/CONCLUSIONS:Areas of hypoautofluorescence in the absence of retinal pigment epithelium atrophy, often with a speckled pattern, delineate areas of prior subretinal hemorrhage long after its resolution in patients with neovascular age-related macular degeneration. Potential mechanisms for the development of this pattern are proposed.
PMID: 30355956
ISSN: 1539-2864
CID: 3384942

Classification and Guidelines for Widefield Imaging: Recommendations from the International Widefield Imaging Study Group

Choudhry, Netan; Duker, Jay S; Freund, K Bailey; Kiss, Szilard; Querques, Giuseppe; Rosen, Richard; Sarraf, David; Souied, Eric H; Stanga, Paulo E; Staurenghi, Giovanni; Sadda, SriniVas R
PURPOSE/OBJECTIVE:To summarize the results of a consensus meeting aimed at defining terminology for widefield imaging across all retinal imaging methods and to provide recommendations for the nomenclature used to describe related images. DESIGN/METHODS:An international panel with expertise in retinal imaging was assembled to define consensus terminology for widefield imaging and associated terminology. PARTICIPANTS/METHODS:A panel of retina specialists with expertise in retinal imaging. METHODS:Before the consensus meeting, a set of 7 images acquired with a range of imaging methods and representing both healthy and diseased eyes was circulated to the expert panel for independent assignment of nomenclature for each example. The outputs were assembled and used as the starting point for discussions occurring at a subsequent roundtable meeting. The anatomic location, field of view, and perspective provided by each image example was reviewed. A process of open discussion and negotiation was undertaken until unanimous terminology for widefield imaging was achieved. MAIN OUTCOME MEASURES/METHODS:Definitions of widefield imaging applicable to multiple imaging methods. RESULTS:Across a range of different imaging methods, the expert panel identified a lack of uniform terminology being used in recent literature to describe widefield images. The panel recommended the term widefield be limited to images depicting retinal anatomic features beyond the posterior pole, but posterior to the vortex vein ampulla, in all 4 quadrants. The term ultra widefield was recommended to describe images showing retinal anatomic features anterior to the vortex vein ampullae in all 4 quadrants. The definitions were recommended over other device-specific terminology. CONCLUSIONS:A consistent nomenclature for widefield imaging based on normal anatomic landmarks that is applicable to multiple retinal imaging methods has been proposed by the International Widefield Imaging Study Group. The panel recommends this standardized nomenclature for use in future publications.
PMID: 31302104
ISSN: 2468-7219
CID: 4040872

Photopigment Bleaching Phenomenon With Scanning Laser Ophthalmoscopy and Fluorescein Angiography

Breazzano, Mark P; Fernandez-Avellaneda, Pedro; Kurup, Shree K; Freund, K Bailey
Photopigment bleaching occurs with saturation of photoreceptor pigment by short-wavelength fundus autofluorescence imaging. This phenomenon is seen as characteristic hyperautofluorescence with subsequent imaging acquisition. Herein, a patient with multiple sclerosis was found to exhibit increased choroidal hyperfluorescence during fluorescein angiography (FA) that corresponded with a circumscribed area of intense blue light exposure during initial scanning laser ophthalmoscopy. To the authors' knowledge, this case is the first description of photobleaching phenomenon during FA and should be recognized as nonpathologic by the clinician. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:590-592.].
PMID: 31589758
ISSN: 2325-8179
CID: 4129322

Choroidal Effusion after Laser Peripheral Iridotomy

Kaden, Talia R; Freund, K Bailey; Engelbert, Michael
PMID: 31443786
ISSN: 1549-4713
CID: 4063942