Characteristics of Pigmented Lesions in Type 2 Idiopathic Macular Telangiectasia

Leung, Irene; Sallo, Ferenc B; Bonelli, Roberto; Clemons, Traci E; Pauleikhoff, Daniel; Chew, Emily Y.; Bird, Alan C.

doi:10.1097/iae.0000000000001842

Cited by 32 publications

(27 citation statements)

References 33 publications

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“…The latter include pigmentary changes, possibly deriving from migrating RPEcells, neurodegenerative processes, cellular debris, and vascular alterations. [21][22][23] Although a correlation of hyperreflectivity with disease progression has been proposed, its functional relevance has yet not been evaluated. In this study, we used an approach that quantified hyperreflective changes in MacTel.…”

Section: Discussionmentioning

confidence: 99%

Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Tzaridis

Friedlander

2021

Invest. Ophthalmol. Vis. Sci.

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The purpose of this study was to quantify hyper-reflective lesions on en face optical coherence tomography (OCT) and study its functional relevance in macular telangiectasia type 2 (MacTel). DESIGN. This was a retrospective, cross-sectional cohort study. METHODS. Baseline image and functional data from participants of a phase II clinical trial (NCT01949324) that studied the effect of Ciliary Neurotrophic Factor in patients with MacTel were analyzed. The projection of hyper-reflectivity within different layers on OCT was used to generate an en face view and measure the en face size of hyper-reflectivity. Ellipsoid zone (EZ)-loss was additionally evaluated, and en face images were superimposed onto microperimetry sensitivity maps, allowing to estimate mean retinal sensitivity within areas displaying hyper-reflectivity and EZ-loss, respectively. Best-corrected visual acuity (BCVA) and reading speed were also analyzed. RESULTS. Fifty-two eyes from 52 patients were analyzed. Hyper-reflectivity was present in 32 eyes (62%), and EZ-loss in 50 (96%) eyes. Mean lesion size was 0.11 mm 2 (range = 0.01-0.26) for hyper-reflectivity and 0.51 mm 2 (range = 0.02-1.34) for EZ-loss, and lesion sizes correlated strongly (Spearman r = 0.79, P < 0.001). Although both hyperreflectivity and EZ-loss were associated with a significant decrease in retinal sensitivity, mean sensitivity thresholds differed significantly between lesions (0.9 dB vs. 16.3 dB; P < 0.001), indicating an almost complete loss of sensitivity in hyper-reflective areas. No correlations were found between the size of hyper-reflectivity and BCVA (r = 0.09) or reading speed (r = −0.17). CONCLUSIONS. En face OCT can be used to quantify the area of hyper-reflective lesions in MacTel. Hyper-reflectivity in MacTel is associated with severe functional impairment, leading to an almost complete loss of retinal sensitivity as observed on microperimetry.

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Section: Discussionmentioning

confidence: 99%

Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Tzaridis

Friedlander

2021

Invest. Ophthalmol. Vis. Sci.

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“…The prevalence of SRNV was estimated to 12.1% in one study investigating a sample of 1244 affected MacTel probands (Leung et al. ). SRNV may threaten central vision directly and responds to anti‐vascular endothelial growth factor therapy, whereas the basic neurodegenerative disease process does not (Kupitz et al.…”

Section: Discussionmentioning

confidence: 99%

Progression characteristics of ellipsoid zone loss in macular telangiectasia type 2

Pauleikhoff¹,

Bonelli²,

Dubis³

et al. 2019

Acta Ophthalmologica

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Purpose To investigate the progression characteristics of ellipsoid zone (EZ) loss in eyes with macular telangiectasia type 2 (MacTel) as reflected by area and linear measurements, and their relevance for visual acuity. Methods Participants were selected from the MacTel Study cohort. Linear and area measurements of EZ loss were performed in Spectral‐Domain Optical Coherence Tomograph (SD‐OCT) volume scans. Progression characteristics and correlations between linear and area measurements were analysed using linear mixed effects models. Results A total of 134 eyes of 70 patients were included (85 eyes with follow‐up, mean 4.7 years, range: 1.4–8 years). Ellipsoid zone (EZ) loss significantly progressed at a mean annual increment of 0.057 mm2 (p = 0.005). The progression rate was non‐linear and interacted significantly with initial EZ lesion size indicating an exponential growth before reaching a plateau. There was a strong heterogeneity in area sizes between fellow eyes. EZ break length had a significant linear effect on EZ break area (b = 1.06, p < 0.001) and could predict it. The location of the EZ break had a significant impact on visual acuity. Conclusion Ellipsoid zone (EZ) loss in MacTel has a non‐linear progression characteristic, and its rate depends on area size at baseline, which must be taken into account at sample selection in clinical trials. Our results show a good correlation of linear and area measures of EZ loss and a segregation of best‐corrected visual acuity by EZ location, which may help routine clinical practice.

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“…These right-angled venules typically develop temporally. It has been suggested that a retinal Muller glial dysfunction or cell death may contribute to the formation of these venules [ 4 ]. Due to RPE migration into the retina along the course of the right-angled venules, retinal pigmented epithelial hyperplasia or clumps may be seen around the parafoveolar right-angled venules, and these pigments may extend into the inner retina to form irregular plaques enveloping these venules [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Due to RPE migration into the retina along the course of the right-angled venules, retinal pigmented epithelial hyperplasia or clumps may be seen around the parafoveolar right-angled venules, and these pigments may extend into the inner retina to form irregular plaques enveloping these venules [ 5 ]. This pigmentary “sheath” may also provide support for the abnormal blood vessels and increase the propensity of the tissue for fibrosis and tissue contraction [ 4 ]. Pigment clumping is initiated in the areas of outer retinal thinning and disruption, which suggests that atrophic changes in the photoreceptor layer may create a permissive environment for RPE transformation.…”

Section: Discussionmentioning

confidence: 99%

An unusual presentation of macular telangiectasia type 2 with a large pigment deposit

Goel

Nangia

et al. 2021

GMS Ophthalmology Cases; 11:Doc03

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Characteristics of Pigmented Lesions in Type 2 Idiopathic Macular Telangiectasia

Abstract: Our data may be useful for refining the current system for staging disease severity in MacTel.

Cited by 32 publications

References 33 publications

Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Progression characteristics of ellipsoid zone loss in macular telangiectasia type 2

An unusual presentation of macular telangiectasia type 2 with a large pigment deposit

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