Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Tzaridis, Simone; Friedlander, Martin

doi:10.1167/iovs.62.3.6

Cited by 5 publications

(3 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our study used PRR as a quantitative measure of photoreceptor anatomic integrity. PRR turned out to be also a reliable indicator of photoreceptor function, aligning with studies on macular telangiectasia type 2, 32 age-related macular degeneration, 19 , 33 retinal dystrophies, and other vitreoretinal diseases. 34 On the other hand, the presence of the EZ band did not conclusively predict the functional status.…”

Section: Discussionsupporting

confidence: 65%

Photoreceptor Integrity in MEWDS: Longitudinal Structure-Function Correlations

Cicinelli,

Montesano,

Berni

et al. 2024

Invest. Ophthalmol. Vis. Sci.

View full text Add to dashboard Cite

Purpose The purpose of this study was to investigate structure-function correlations in multiple evanescent white dot syndrome (MEWDS) using microperimetry (MP) and spectral-domain optical coherence tomography (SD-OCT). Methods Single-center prospective observational study including 14 eyes from 13 patients with MEWDS monitored over a median of 49.5 days (interquartile range = 29–92 days). Investigations focused on best-corrected visual acuity (BCVA), foveal granularity, and the Photoreceptor Reflectivity Ratio (PRR) as a measure of photoreceptor integrity. MP assessed average retinal threshold sensitivity (RTS) and bivariate contour ellipse area (BCEA) for fixation stability. A linear mixed model was used to test associations and interactions among RTS, time, and clinical variables. A hierarchical linear mixed model was used to analyze structure-function relationships, addressing both individual and location-specific variations. Results Overall, 2340 MP locations were tested. PRR revealed a transient decrease within 30 days post-presentation, indicative of early photoreceptor disruption, followed by a progressive increase, signaling recovery. Significantly lower foveal sensitivity (RTS = 14.8 ± 7.4 vs. 22.5 ± 4.4 decibel [dB], P = 0.04) and increased fixation spread (63% BCEA = 1.26 ± 0.97 vs. 0.48 ± 0.35 deg 2 , P = 0.06) were noted in eyes with foveal granularity compared to those without. A significant increase in RTS was demonstrated over time (0.066 dB/day, P < 0.001), with a central-to-peripheral gradient of improvement. The interaction between follow-up time and baseline BCVA ( P < 0.001) indicated more rapid improvement in eyes with worse initial vision. There was a robust, nonlinear association between PRR and RTS across all tested locations ( P < 0.001), becoming asymptotic for sensitivity losses exceeding 20 dB. Conclusions Photoreceptor reflectivity accurately aligned with visual function in MEWDS on longitudinal examinations. The central-to-peripheral gradient of improvement may suggest specific vulnerabilities underlying the area around the disc.

show abstract

Section: Discussionsupporting

confidence: 65%

Photoreceptor Integrity in MEWDS: Longitudinal Structure-Function Correlations

Cicinelli,

Montesano,

Berni

et al. 2024

Invest. Ophthalmol. Vis. Sci.

View full text Add to dashboard Cite

show abstract

“…Inhibiting EMT in the Vldlr-/-model resulted in increased neovascular growth and exudation, confirming the beneficial effect of perivascular pigment accumulation we had clinically observed in patients with MacTel. Pigment plaques, on the other hand, have been shown to be associated with a focal loss in retinal sensitivity ("absolute scotomas" on fundus-controlled perimetry) [8,39] and the pigment area with a decrease in visual acuity in patients with MacTel [5], thus representing a mixed blessing.…”

Section: Discussionmentioning

confidence: 99%

Retinal pigment epithelial cells reduce vascular leak and proliferation in retinal neovessels

Tzaridis,

Aguilar,

Dorrell

et al. 2024

Preprint

View full text Add to dashboard Cite

Retinal pigment epithelial (RPE)-cells possess numerous functions and may respond to stress and damage of the neuroretina. In different neurodegenerative diseases, including age-related macular degeneration (AMD), retinitis pigmentosa, and macular telangiectasia type 2 (MacTel), RPE-cells have been shown to proliferate and migrate into the neuroretina, forming intraretinal pigment plaques. Though pigmentary changes are associated with disease progression, it is not known if their presence is protective or detrimental. In this study, we evaluated the impact of pigment plaques on vascular changes and disease progression in patients with macular telangiectasia type 2 (MacTel), an example of a progressive neurodegenerative retinal disease. We then studied underlying pathomechanisms using a mouse model mirroring these changes, the very-low-density lipoprotein receptor mutant (Vldlr–/–) mouse.In a retrospective, longitudinal study, we analyzed multimodal retinal images of patients with MacTel and showed that pigment plaques were associated with a decrease in vascular leakage and stabilized neovascular growth. Using genetic approaches, we analyzed changes in expression levels of relevant genes in the RPE and retinas of Vldlr-/- mice during RPE-proliferation and migration. Our data indicated that RPE-cells transitioned from an epithelial to a mesenchymal state (“epithelial-mesenchymal transition”, EMT), proliferated and accumulated along neovessels. Using dextran angiography and immunofluorescence, we demonstrated that the perivascular accumulation of RPE-cells reduced vascular leakage. Pharmacologic inhibition of EMT led to a decrease in pigment coverage and exacerbation of neovascular growth and exudation.Our findings indicate that the proliferation, migration and perivascular accumulation of RPE-cells may stabilize vascular proliferation and exudation, thereby exerting a protective effect on the diseased retina. We conclude that interfering with this “natural repair mechanism” may have detrimental effects on the course of the disease and should thus be avoided.

show abstract

“…HRD in outer retina was defined as discrete, clustered, and well-circumscribed hyperreflective materials, extending between retinal layers and retinal pigment epithelium/Bruch membrane (RPE/BM), showing equal or higher reflectivity than the retinal pigment epithelium layer. 18…”

Section: Definitions Of Sd-oct Characteristicsmentioning

confidence: 99%

Outer Foveal Defects in Type-2 Macular Telangiectasia

Chandran

Anantharaman

Gayathri

et al. 2023

Retina

View full text Add to dashboard Cite

Purpose: To report characteristics of outer foveal defects (OFDs) in type-2 macular telangiectasia (MacTel) on spectral domain optical coherence tomography.Methods: This was a single-center observational study. From a registry of 745 patients with MacTel, patients with OFDs were characterized. All patients underwent multimodal imaging including color fundus photography, confocal blue reflectance, fundus autofluorescence, and spectral domain optical coherence tomography. Staging of eyes was done using the Gass and Blodi classification. Spectral domain optical coherence tomography characteristics in the central 1 mm of the macula in eyes with OFD are reported.Results: Outer foveal defect was observed in 21 eyes of 15/745 (2%) patients with MacTel. These defects were bilateral in 6/15 (40%) patients and seen in stage 2 MacTel eyes. In order of prevalence, foveal parameters seen in OFD included hyper-reflective dots in outer retina in 19/21 (90%), ellipsoid zone loss in 18/21 (86%) eyes, interdigitation zone loss in 17/21 (81%) eyes, outer retinal hyporeflective cavitation in 14 (67%) eyes, hyporeflective cavitation at foveal pit in 8 (38%) eyes, and loss of external limiting membrane in 1 (5%) eye. The mean baseline length of the foveal ellipsoid zone loss was 240.17 6 117.249 mm. The mean baseline central subfield thickness was 155.43 6 17.215 mm. A total of 8/11 eyes (73%) showed an increase in size of OFD on follow-up.Conclusion: Outer foveal defect in MacTel eyes is characterized predominantly by foveal loss of ellipsoid zone and interdigitation zone with relative preservation of external limiting membrane.

show abstract

Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Cited by 5 publications

References 26 publications

Photoreceptor Integrity in MEWDS: Longitudinal Structure-Function Correlations

Photoreceptor Integrity in MEWDS: Longitudinal Structure-Function Correlations

Retinal pigment epithelial cells reduce vascular leak and proliferation in retinal neovessels

Outer Foveal Defects in Type-2 Macular Telangiectasia

Contact Info

Product

Resources

About