Correlation of Clinical and Structural Progression With Visual Acuity Loss in Macular Telangiectasia Type 2

Pető, Tünde; Heeren, Tjebo; Clemons, Traci E; Sallo, Ferenc B; Leung, Irene; Chew, Emily Y.; Bird, Alan C.

doi:10.1097/iae.0000000000001697

Cited by 56 publications

(51 citation statements)

References 23 publications

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“…In the present study, the eyes with ELM, EZ, and IDZ disruptions, indicating photoreceptor damage, had worse visual acuity regardless of the foveal centre involvement. This is in accordance with the longitudinal study by Peto et al 10 . They reported that the clinically meaningful loss of visual acuity occurred as the EZ disruption progresses to the foveal centre although a statistically significant modest loss of the mean vision was observed in the presence of EZ disruption on OCT 10 .…”

Section: Discussionsupporting

confidence: 94%

Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study—Report No. 1

Kim

Chung

et al. 2020

Sci Rep

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We analysed the imaging findings of macular telangiectasia (MacTel) type 2 in Korea using spectral domain optical coherence tomography (SD-OCT) and investigated their relationship with visual acuity and clinical stages. A retrospective multicentre cross-sectional study was conducted in six tertiary hospitals in Korea and included 129 patients. We analysed all the SD-OCT images encompassing the macular area. Hyporeflective cavities (77.7%) were the most frequently detected abnormalities in SD-OCT. Disruption of the external limiting membrane, ellipsoid zone, and interdigitation zone were found in 67 (40.4%), 87 (52.4%), and 94 eyes (56.6%), respectively. Four eyes (2.4%) had lamellar macular hole, and five eyes (3.0%) full-thickness macular hole. Neovascularisation, either subretinal or intraretinal, was found in 14 eyes (8.4%). Eyes with outer retinal hyperreflective band disruption had lower visual acuity than those without them. The presented characteristic clinical features of OCT in MacTel type 2 can not only aid in differentiating this disease from others but are also helpful for better judgement of the disease stage in daily clinical practice. Inner retinal hyporeflective cavities without outer retinal abnormalities on SD-OCT, although classified as severity scale 3, could be considered a relatively early stage in the disease process in terms of vision.

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Section: Discussionsupporting

confidence: 94%

Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study—Report No. 1

Kim

Chung

et al. 2020

Sci Rep

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“…The latter event directly affects central vision as the fovea is involved (Peto et al. ). Both events are possible to document reliably using OCT imaging – an objective, structural method.…”

Section: Discussionmentioning

confidence: 99%

“…Another possible major event in MacTel is the incidence of subretinal neovascular proliferation (SRNV) (Peto et al. ). This appears to be a secondary complication of the disease and may occur at any point during the evolution of the disease.…”

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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“…9 In general, VA decreases at a very slow rate of approximately 1 letter per year. 17 However, although VA may test well in patients with MacTel, visionrelated quality of life is reduced compared with patients with early age-related macular degeneration (AMD) and similar VA. 18 Funduscopic features present in eyes in the Mac-Tel Project cohort at baseline, as graded on color fundus photographs, included a loss of retinal clarity / grayish perifoveal sheen (74%), telangiectatic macular vessels (51%), retinal pigment epithelial (RPE) hypertrophy (33%), blunted "right-angle" venules (29%), and crystalline deposits (21%) ( Figures 1A and 2A). 9 There are no peripheral retinal abnormalities in MacTel.…”

Section: Clinical Featuresmentioning

confidence: 99%

The Diagnosis and Management of Macular Telangiectasia

Christakis¹,

Fine²,

Wiley³

2019

Ophthalmic Surg Lasers Imaging Retina

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Correlation of Clinical and Structural Progression With Visual Acuity Loss in Macular Telangiectasia Type 2

Abstract: Ellipsoid zone loss is frequently found in macular telangiectasia Type 2 and is an important structural component reflecting visual function. Its presence in the fovea significantly correlates with worse visual prognosis.

Cited by 56 publications

References 23 publications

Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study—Report No. 1

Optical coherence tomographic features of macular telangiectasia type 2: Korean Macular Telangiectasia Type 2 Study—Report No. 1

Progression characteristics of ellipsoid zone loss in macular telangiectasia type 2

The Diagnosis and Management of Macular Telangiectasia

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