Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration

Guymer, Robyn H.; Rosenfeld, Philip J.; Curcio, Christine A.; Holz, Frank G.; Staurenghi, Giovanni; Freund, K. Bailey; Schmitz-Valckenberg, Steffen; Sparrow, Janet R.; Spaide, Richard F.; Tufail, Adnan; Chakravarthy, Usha; Jaffe, Glenn J.; Csaky, Karl G.; Sarraf, David; Monés, Jordi; Tadayoni, Ramin; Grunwald, Juan E.; Bottoni, Ferdinando; Liakopoulos, Sandra; Pauleikhoff, Daniel; Pagliarini, Sergio; Chew, Emily Y.; Viola, Francesco; Fleckenstein, Monika; Blodi, Bárbara A.; Lim, Tock Han; Chong, Victor; Lutty, Jerry; Bird, Alan C.; Sadda, Srinivas R

doi:10.1016/j.ophtha.2019.09.035

Cited by 186 publications

(201 citation statements)

References 42 publications

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“…Atrophy was defined as the presence of signal hypertransmission on the B-scan and accompanying RPE attenuation. Because the extent of these two features were not measured, incomplete RPE and outer retinal atrophy (iRORA) and complete RPE and outer retinal atrophy (cRORA) were not distinguished [29]. Atrophy was measured on a custom slab 320 μm below Bruch's membrane.…”

Section: Image Analysis and Outcome Measurementsmentioning

confidence: 99%

The long-term effects of anti-vascular endothelial growth factor therapy on the optical coherence tomography angiographic appearance of neovascularization in age-related macular degeneration

et al. 2020

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Background: The short-term effects of anti-vascular endothelial growth factor (anti-VEGF) treatment on macular neovascularization (MNV) morphology is well described, but long-term studies on morphologic changes and correlation of such changes to the type of MNV have not been conducted. This study aims to determine if different types of MNVs in neovascular AMD (nAMD) behave differently with anti-VEGF treatment as visualized on optical coherence tomography angiography (OCTA). Methods: Treatment-naïve nAMD patients were retrospectively screened for baseline and follow-up OCTA imaging 10 or more months after initial treatment. Images were graded for MNV type, area, activity, mature versus immature vessels, vessel density, presence of atrophy, atrophy location and area. Growth rate was calculated as the percent change in lesion area from baseline over the years of follow-up. In addition, the occurrence of complete regression and the percent of lesions that grew, remained stable, and shrunk per type was also evaluated. Results: Forty-three eyes from 43 patients with a mean follow-up of 2 years were evaluated. On structural OCT, 26 lesions were classified as pure type 1 MNVs, 12 MNVs had a type 2 component, and 5 MNVs had a type 3 component. Of these cases, 2 mixed-type MNVs were considered to have completely regressed. There was no significant differences in MNV area and growth rate between type 1 and type 2 lesions, but all cases of type 3 lesions shrunk in the follow-up period. There was no correlation between the number of injections per year and growth rate, endpoint MNV area or endpoint activity status for any MNV type. There was no significant association between the development of atrophy and the number of injections, baseline MNV area, baseline vessel density, or lesion growth rate.

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Section: Image Analysis and Outcome Measurementsmentioning

confidence: 99%

The long-term effects of anti-vascular endothelial growth factor therapy on the optical coherence tomography angiographic appearance of neovascularization in age-related macular degeneration

et al. 2020

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“…Knowing which of AMD's many layers is affected first can focus attention to treating or preventing problems and advising patients earlier than later in the course of AMD. A timeline can be generated for drusen-driven disease whereas SDD-driven disease is poorly understood [112,113]. Existing large prospective studies focus on mechanisms of end-stage disease such as expansion of geographic atrophy, but little research addresses structural-functional mechanisms involved in disease onset.…”

Section: Discussionmentioning

confidence: 99%

Functionally validated imaging endpoints in the Alabama study on early age-related macular degeneration 2 (ALSTAR2): design and methods

et al. 2020

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Background: Age-related macular degeneration (AMD), a leading cause of irreversible vision impairment in the United States and globally, is a disease of the photoreceptor support system involving the retinal pigment epithelium (RPE), Bruch's membrane, and the choriocapillaris in the setting of characteristic extracellular deposits between outer retinal cells and their blood supply. Research has clearly documented the selective vulnerability of rod photoreceptors and rod-mediated (scotopic) vision in early AMD, including delayed rod-mediated dark adaptation (RMDA) and impaired rod-mediated light and pattern sensitivity. The unifying hypothesis of the Alabama Study on Early Macular Degeneration (ALSTAR2) is that early AMD is a disease of micronutrient deficiency and vascular insufficiency, due to detectable structural changes in the retinoid re-supply route from the choriocapillaris to the photoreceptors. Functionally this is manifest as delayed rod-mediated dark adaptation and eventually as rod-mediated visual dysfunction in general. Methods: A cohort of 480 older adults either in normal macular health or with early AMD will be enrolled and followed for 3 years to examine cross-sectional and longitudinal associations between structural and functional characteristics of AMD. Using spectral domain optical coherence tomography, the association between (1) subretinal drusenoid deposits and drusen, (2) RPE cell bodies, and (3) the choriocapillaris' vascular density and rodand cone-mediated vision will be examined. An accurate map and timeline of structure-function relationships in aging and early AMD gained from ALSTAR2, especially the critical transition from aging to disease, will identify major characteristics relevant to future treatments and preventative measures.

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“…This represents a crucial point, as OCT technology allows to disclose atrophic changes at an early stage, when a complete loss of RPE and outer retina has not yet occurred, and to monitor their gradual enlargement. As a consequence, a new rigorous classification of macular atrophy has been proposed [5,9], based on microscopic OCT findings.…”

Section: Discussionmentioning

confidence: 99%

“…This is particularly true for geographic atrophy (GA) [6][7][8]. According to Guymer et al [9], incomplete retinal pigment epithelium (RPE) and outer retina atrophy (iRORA) is defined on OCT as a region of signal hyper-transmission into the choroid, resulting from the interruption of the RPE. This finding corresponds to the zone of attenuation or disruption of the RPE, with evidence of the overlying photoreceptor degeneration, not exceeding 250 µm of width.…”

Section: Introductionmentioning

confidence: 99%

Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration: Correlation with Macular Function

Savastano

Falsini

Cozzupoli

et al. 2020

JCM

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The purpose of this study was to investigate the relationship between the retinal pigment epithelium (RPE) and outer retina changes, expressed in terms of sub-RPE illumination (SRI) on optical-coherence tomography (OCT), and central retinal function, measured by visual acuity and focal electroretinogram (fERG), in patients with non-exudative age-related macular degeneration (neAMD). In this retrospective study, 29 eyes of 29 patients affected by early (24.14%), intermediate (41.38%), and advanced (34.48%) neAMD were evaluated. All enrolled eyes were studied with OCT to measure the total area of SRI, by using an automated standardized algorithm. Visual acuity and fERG were assessed. The area of SRI was negatively correlated with fERG amplitude (r ≤ −0.4, p ≤ 0.02) and best-corrected visual acuity (BCVA) (r ≤ 0.4, p ≤ 0.04). Our results indicate that the severity of retinal pigment epithelium and outer retina atrophy (RORA), indirectly quantified through the detection of SRI areas by commercial OCT algorithms, is correlated with central retinal dysfunction, as determined by visual acuity and fERG, supporting the combined use of structural exams and functional tests as valid tools to detect the extent of RPE and photoreceptors’ disruption.

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Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration

Cited by 186 publications

References 42 publications

The long-term effects of anti-vascular endothelial growth factor therapy on the optical coherence tomography angiographic appearance of neovascularization in age-related macular degeneration

The long-term effects of anti-vascular endothelial growth factor therapy on the optical coherence tomography angiographic appearance of neovascularization in age-related macular degeneration

Functionally validated imaging endpoints in the Alabama study on early age-related macular degeneration 2 (ALSTAR2): design and methods

Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration: Correlation with Macular Function

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