Outer Retina Capillary Invasion and Ellipsoid Zone Loss in Macular Telangiectasia Type 2 Imaged by Optical Coherence Tomography Angiography

Gaudric, Alain; Krivosic, Valérie; Tadayoni, Ramin

doi:10.1097/iae.0000000000000799

Cited by 55 publications

(36 citation statements)

References 17 publications

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“…In addition, we found that there was a strong correlation between the area of outer retina capillary proliferation and the area of EZ loss. This builds on previous findings described qualitatively by Gaudric et al, 18 which noted that “deep retinal capillaries invaded the outer retina specifically in the area of EZ loss.”…”

Section: Discussionsupporting

confidence: 88%

“…14–17,23 Other studies have measured the en face total area of the EZ disruption, or have reported on the co-localization of area of EZ breakdown and proliferation of abnormal capillary vessels. 10,11,13,18 Our study adds to the current knowledge of EZ disruption in MacTel type 2 by investigating EZ mapping technology for quantifying EZ breakdown. 20 First, we found that EZ-RPE thickness, area, and volume measurements in eyes with MacTel type 2 were significantly decreased when compared to age- and sex-matched normative controls, and percentage of EZ atrophy and attenuation were significantly increased when compared to normative controls (Table 3).…”

Section: Discussionmentioning

confidence: 93%

“…10,11,13,14,16–18,26 While the pathophysiology of MacTel type 2 remains uncertain, it is hypothesized to be linked to Müller cell death or dysfunction, which leads to the resulting photoreceptor loss (and thus EZ disruption) and juxtafoveal capillary proliferation. 1,18 …”

Section: Discussionmentioning

confidence: 99%

“…3–18 Spectral-domain (SD)-OCT demonstrates characteristic features of MacTel type 2, including hyporeflective cavities and atrophy of the neurosensory retina, ellipsoid zone (EZ) breakdown, increased reflectivity of inner retinal layers, and retinal thinning. 1 Of particular interest is the EZ, which has been shown to be disrupted in MacTel type 2 and may serve as a marker to measure disease progression and treatment response.…”

mentioning

confidence: 99%

“…Studies investigating the features of MacTel type 2 using OCT-A have demonstrated alterations in the superficial and deep retinal capillary plexuses, including capillary dilation, abnormal anastomoses, telangiectasias, and decreased capillary density. 3–7,12,18 …”

mentioning

confidence: 99%

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OCT Angiography and Ellipsoid Zone Mapping of Macular Telangiectasia Type 2 From the AVATAR Study

Runkle

Srivastava

Schachat

et al. 2017

Invest. Ophthalmol. Vis. Sci.

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PurposeTo evaluate alterations on optical coherence tomography angiography (OCT-A) and quantitatively assess alterations in the ellipsoid zone (EZ) in eyes with macular telangiectasia type 2 (MacTel type 2).MethodsThe Observational Assessment of Visualizing and Analyzing Vessels With Optical Coherence Tomography Angiography in Retinal Diseases study is an institutional review board-approved prospective, observational study investigating OCT-A in macular disease. Patients underwent spectral-domain (SD)-OCT and OCT-A imaging at a single visit. SD-OCT data were analyzed using a novel OCT EZ-mapping software to obtain linear, area, and volumetric measurements of the EZ-retinal pigment epithelium (RPE) complex across the macular cube. OCT-A retinal capillary density was measured using the Optovue Avanti split-spectrum amplitude-decorrelation angiography algorithm. EZ-RPE parameters were compared to age-matched, sex-matched controls.ResultsFourteen eyes of seven patients (mean age, 59 ± 6.5 years) were analyzed. Mean visual acuity was 20/45 (range, 20/20–20/150). EZ-RPE central foveal mean thickness was 27.8 ± 6.7 μm, EZ-RPE central foveal thickness was 22.1 ± 21.6 μm, EZ-RPE central foveal area was 0.17 ± 0.04 mm2, and EZ-RPE central subfield volume was 0.017 ± 0.012 mm3. Each of these measurements was significantly inversely correlated with visual acuity (P < 0.02). In addition, all of these measurements were significantly reduced compared to controls (all P ≤ 0.005). OCT-A showed a reduced parafoveal vessel density of 50.8% temporally compared to 53.8% nasally (P = 0.01) in the superficial vascular plexus. In the deep vascular plexus, similar findings were noted with a parafoveal vessel density of 56.7% temporally and 58.8% nasally (P = 0.01).ConclusionsAbnormalities in EZ-RPE thickness, area, and volume are correlated with visual acuity in MacTel type 2, and may provide quantitative markers to measure disease progression and treatment response. OCT-A was a useful adjunct for determining disease severity.

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Section: Discussionsupporting

confidence: 88%