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EVALUATION OF SEGMENTAL RETINAL ARTERITIS WITH OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY

Tsui, Edmund; Leong, Belinda C S; Mehta, Nitish; Gupta, Akash; Goduni, Lediana; Cunningham, Emmett T; Freund, K Bailey; Lee, Gregory D; Dedania, Vaidehi S; Yannuzzi, Lawrence A; Modi, Yasha S
PURPOSE/OBJECTIVE:To describe the vascular anatomy and intraluminal flow characteristics of segmental retinal arteritis (SRA) using structural and angiographic optical coherence tomography (OCT). METHODS:Retrospective case series of consecutive patients presenting with SRA. All patients were evaluated at presentation with fundus photography, spectral domain OCT, and OCT angiography. One patient was imaged with dense B-scan OCT angiography. RESULTS:Three eyes of three male patients were evaluated. All examinations were consistent with reactivation of ocular toxoplasmosis with an area of active retinochoroiditis adjacent to a focal chorioretinal scar. Spectral domain OCT through areas of SRA noted on clinical examination demonstrated areas of hyperreflectivity circumscribing the affected vessel with a normoreflective lumen. Optical coherence tomography angiography and dense B-scan OCT angiography demonstrated narrowing of the intraluminal flow signal that correlated with areas of segmental hyperreflectivity on spectral domain OCT. Vascular sections proximal and distal to areas of SRA showed normal flow signal. CONCLUSION/CONCLUSIONS:Vessels with SRA demonstrated hyperreflectivity highlighting the vessel wall on spectral domain OCT. Optical coherence tomography angiography showed narrowing of the flow signal within these segments suggesting reduced lumen diameter. Coupling these finding with previous indocyanine green imaging findings in SRA, the collective data suggest the plaques are localized within the vessel wall to either the endothelium or the muscular tunica media without occlusion of the vessel lumen.
PMID: 31313702
ISSN: 1937-1578
CID: 3977882

Presumed Natural History of Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHRRPE)

Ledesma-Gil, Gerardo; Essilfie, Juliet; Gupta, Rajan; Fung, Adrian T; Lupidi, Marco; Pappuru, Rajeev R; Nayak, Sameera; Sahoo, Niroj Kumar; Kaliki, Swathi; Yannuzzi, Lawrence A; Reid, Kate; Lim, Lianne; Sacconi, Riccardo; Dave, Vivek; Singh, Sumit R; Ayachit, Apoorva; Gabrielle, Pierre-Henry; Cai, Sophie; Lima, Luiz; Querques, Giuseppe; Arevalo, Fernando; Freund, K Bailey; Shields, Carol L; Chhablani, Jay
PURPOSE/OBJECTIVE:To correlate structural changes of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with patient age. DESIGN/METHODS:Retrospective study. SUBJECTS/METHODS:There were 50 eyes of 49 patients (age range 1-74 years) with CHRRPE studied at nine tertiary vitreoretinal institutions. METHODS:We analyzed the clinical findings with respect to lesion topography and pigmentation as well as investigated the optical coherence tomography (OCT) findings regarding the thickness, vitreoretinal interface, outer plexiform layer distortion, ellipsoid zone disruption and retinal pigment epithelium/Bruch's membrane complex involvement of CHRRPE. MAIN OUTCOMES/RESULTS:Clinical and imaging findings of CHRRPE at different ages. RESULTS:Analysis of 50 CHRRPE revealed younger patients were more likely to have partial thickness involvement of the retina (p = 0.009) with predominantly inner retinal layer involvement (p = 0.04). The inverse was true for older patients with CHRRPE. In addition, older patients more commonly showed pigmentary changes. Eyes with CHRRPE were more likely to have an increase in central macular thickness independently of tumor location. CONCLUSION/CONCLUSIONS:Based on these findings, we believe that CHRRPE typically begins in the inner retina and continues towards the outer retina over time, with increase in central macular thickness, despite the location of the tumor.
PMID: 33516918
ISSN: 2468-6530
CID: 4775712

Salivary alpha amylase levels may correlate with central serous chorioretinopathy activity

Abdelhakim, Aliaa H; Ledesma-Gil, Gerardo; Yannuzzi, Lawrence A
PMID: 34292223
ISSN: 1539-2864
CID: 4948462

Bacillary detachment in an idiopathic chorioretinitic disorder

Hilgert, Carolina R; Japiassú, Ricardo M; Hilgert, Álvaro H; Lima, Luiz H; Balaratnasingam, Chandrakumar; Yannuzzi, Lawrence A; Vianna, Raul N G
PMID: 34171906
ISSN: 1937-1578
CID: 4925882

Macular edema in a peculiar case of pigmentary maculopathy

Pimentel, Sergio L G; Conci, Lívia S; Lima, Luiz H; Balaratnasingam, Chandrakumar; Yannuzzi, Lawrence A; Shields, Carol L
PMID: 34171897
ISSN: 1937-1578
CID: 4925872

Hyperreflective Foci, Optical Coherence Tomography Progression Indicators in Age-Related Macular Degeneration, Include Transdifferentiated Retinal Pigment Epithelium

Cao, Dongfeng; Leong, Belinda; Messinger, Jeffrey D; Kar, Deepayan; Ach, Thomas; Yannuzzi, Lawrence A; Freund, K Bailey; Curcio, Christine A
Purpose:By optical coherence tomography (OCT) imaging, hyperreflective foci (HRF) indicate progression risk for advanced age-related macular degeneration (AMD) and are in part attributable to ectopic retinal pigment epithelium (RPE). We hypothesized that ectopic RPE are molecularly distinct from in-layer cells and that their cross-retinal course follows Müller glia. Methods:In clinical OCT (61 eyes, 44 patients with AMD, 79.4 ± 7.7 years; 29 female; follow-up = 4.7 ± 0.9 years), one HRF type, RPE plume (n = 129 in 4 morphologies), was reviewed. Twenty eyes of 20 donors characterized by ex vivo OCT were analyzed by histology (normal, 4; early/intermediate AMD, 7; geographic atrophy, 6; neovascular AMD, 3). Cryosections were stained with antibodies to retinoid (RPE65, CRALPB) and immune (CD68, CD163) markers. In published RPE cellular phenotypes, red immunoreactivity was assessed semiquantitatively by one observer (none, some cells, all cells). Results:Plume morphology evolved over time and many resolved (40%). Trajectories of RPE plume and cellular debris paralleled Müller glia, including near atrophy borders. RPE corresponding to HRF lost immunoreactivity for retinoid markers and gained immunoreactivity for immune markers. Aberrant immunoreactivity appeared in individual in-layer RPE cells and extended to all abnormal phenotypes. Müller glia remained CRALBP positive. Plume cells approached and contacted retinal capillaries. Conclusions:HRF are indicators not predictors of overall disease activity. Gain and loss of function starts with individual in-layer RPE cells and extends to all abnormal phenotypes. Evidence for RPE transdifferentiation, possibly due to ischemia, supports a proposed process of epithelial-mesenchyme transition. Data can propel new biomarkers and therapeutic strategies for AMD.
PMCID:8399556
PMID: 34448806
ISSN: 1552-5783
CID: 5011192

3-D assessment of gaze-induced eye shape deformations and downgaze-induced vitreous chamber volume increase in highly myopic eyes with staphyloma

Hoang, Quan V; Chang, Stanley; Yu, Daryle Jason Go; Yannuzzi, Lawrence A; Freund, K Bailey; Grinband, Jack
PURPOSE/OBJECTIVE:To determine if the stress of normal eye movements results in gaze-induced globe deformations, vitreous chamber axial length and vitreous chamber axial volume (VCAV) change in highly myopic eyes. METHODS:A prospective imaging study was performed on 82 eyes of 43 patients with high myopia (>27 mm of axial length) with a clinical diagnosis of staphyloma. Three-dimensional MRI scans were acquired while subjects gazed in five directions (primary, nasal, temporal, superior and inferior). Surface renderings were generated, and a processing pipeline was created to automate alignment of the eye and to measure VCAV within 5.5 mm of the visual axis for each eye in every gaze. The degree of gaze-induced globe deformation was determined by calculating the Dice coefficient to assess the degree of overlap of the sclera at each eccentric gaze with that found in primary gaze. Each eccentric gaze VCAV was compared to VCAV in primary gaze using a fixed-effects regression allowing for subject-specific and eye-specific effects. RESULTS:(p=0.002, 95% CI 1.71 to 7.86). CONCLUSION/CONCLUSIONS:Significant gaze-induced globe deformation was noted in all gazes, but a reversible, instantaneous VCAV increase occurred only in downgaze, which is consistent with studies supporting the association of environmental factors such as near work with myopia development and progression.
PMID: 32830122
ISSN: 1468-2079
CID: 4575042

Optic nerve tortuosity and displacements during horizontal eye movements in healthy and highly myopic subjects

Wang, Xiaofei; Chang, Stanley; Grinband, Jack; Yannuzzi, Lawrence A; Freund, K Bailey; Hoang, Quan V; Girard, Michael Ja
AIMS/OBJECTIVE:(1) To assess the morphology and 3-dimensional (3D) displacements of the eye globe and optic nerve (ON) in adduction/abduction using MRI. (2) To assess differences between healthy emmetropic and highly myopic (HM) subjects. METHODS:MRI volumes of both eyes from 18 controls and 20 HM subjects in primary gaze, abduction and adduction (15°) were postprocessed. All ONs were manually segmented and fitted to a 3D curve to assess ON tortuosity. ON displacements were evaluated in four quasicoronal planes which were perpendicular to the ON in primary gaze and were 3 mm apart. RESULTS:Axial length was higher in the HM group (28.62±2.60 vs 22.84±0.89 mm; p<0.0001). Adjusted ON tortuosities (ie, ON tortuosities estimated before myopia onset) were lower in HM eyes (0.9063±0.0591) versus controls (1.0152±0.02981) in primary gaze, adduction (0.9023±0.05538 vs 1.0137±0.0299) and abduction (0.9100±0.0594 vs 1.0182±0.0316); p<0.0001 for all cases. In all eyes, ON displacements in adduction were significantly different from those in abduction in the naso-temporal direction (p<0.0001 in all planes) but not in the supero-inferior direction. ON displacements in the posterior segments of the ON were smaller in the HM group in both gaze directions and were larger in the anterior-most ON segment in adduction only. CONCLUSION/CONCLUSIONS:The adjusted tortuosity of the ON was significantly lower in HM eyes, suggesting that eyes destined towards HM exhibited higher ON traction forces during eye movements before the onset of myopia. Our ON metrics may be valuable to explore a potential link between eye movements and axial elongation.
PMID: 34039559
ISSN: 1468-2079
CID: 4888022

Retinal racemose hemangioma (retinal arteriovenous communication) diagnosed and managed with multimodal imaging

Breazzano, Mark P; Ledesma-Gil, Gerardo; Essilfie, Juliet O; Bacci, Tommaso; Pandey, Avi; Yannuzzi, Lawrence A
PURPOSE/OBJECTIVE:Retinal racemose hemangioma (RRH) is a rare congenital abnormality of the retinal vasculature with a variety of secondary manifestations that can cause vision loss, including macular edema. This report aims to demonstrate the utility of swept-source optical coherence tomography angiography (SS-OCTA) in further characterizing this abnormality. METHODS:Case report with multimodal imaging including SS-OCTA. RESULTS:A 56-year-old woman with blurred vision was diagnosed macular edema secondary to RRH. Localization of the arterial-venous connection was identified with SS-OCTA at the deep capillary plexus (DCP). Conservative management of the associated foveal exudation ultimately led to a favorable outcome. DISCUSSION/CONCLUSIONS:Observation or topical therapy may be useful and warranted in select cases of RRH. The anomalous vascular connection in RRH appears to originate at the level of the DCP in this case, however larger studies are necessary for corroboration. Evolving angiographic modalities like SS-OCTA may continue to provide insights for this rare disease.
PMID: 34033599
ISSN: 1937-1578
CID: 4887762

Differential Response to Glucocorticoid Immunosuppression of Two Distinct Inflammatory Signs Associated with Punctate Inner Choroidopathy

Abdelhakim, Aliaa H; Yannuzzi, Lawrence A; Freund, K Bailey; Jung, Jesse J
PURPOSE/OBJECTIVE:To describe the differential response of two distinct inflammatory signs occurring in eyes with punctate inner choroidopathy (PIC). METHODS:Retrospective, observational case series utilizing multimodal imaging (MMI). RESULTS:Four eyes of 4 myopic female patients (mean age 35 years, range 31-42) presenting with retinal manifestations of PIC. All study eyes had 2 distinct signs of active disease: 1) acute focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch's membrane (RPE/BrM) complex on optical coherence tomography (OCT) which appeared hypoautofluorescent on fundus autofluorescence (FAF), and 2) more diffuse areas of outer retinal disruption (ORD) limited to the ellipsoid zone and interdigitation zone on OCT and corresponding to hyperautofluorescence on FAF. All patients were treated with oral prednisone and demonstrated prompt regression of the RPE/BrM complex lesions with a concurrent, paradoxical centrifugal expansion of ORD. The ORD eventually resolved in all eyes (mean time 6 weeks, range 4-10 weeks). CONCLUSIONS:In patients with PIC, two distinct inflammatory signs observed with MMI display a differential response to systemic corticosteroids. Whereas focal inflammatory lesions splitting the RPE/BrM complex appear to respond rapidly, the more diffuse, transient ORD shows little response. This difference in treatment response may reflect different immunological phenomena with independent natural history.
PMID: 32804829
ISSN: 1539-2864
CID: 4566602