Progression of vascular changes in macular telangiectasia type 2: comparison between SD-OCT and OCT angiography

PurposeTo evaluate the role of right-angled vessels (RAVs) during disease progression in macular telangiectasia type 2 (MacTel).MethodsIn this study, 100 eyes of 52 patients and 52 eyes of 26 age-related controls were examined using fundus photography, spectral-domain optical coherence tomography (SD-OCT), OCT angiography (OCT-A) and fundus fluorescein angiography (FFA). Two masked readers graded fundus photographs of patients’ eyes into five disease stages according to Gass and Blodi, and evaluated all eyes for the presence of RAVs. If RAVs were present, their course and origin (arterial vs venous) was evaluated with OCT-A and FFA, respectively. Additionally, we looked for morphological correlates of these vessels on SD-OCT scans. Neovascular eyes were analysed for the presence of RAVs and for morphological changes on formation of neovascularisations (NVs).ResultsIn OCT-A, RAVs were already detectable in eyes with early stages (1 to 2), could be tracked from superficial to outer retinal layers and were shown to form anastomoses in the outer retina with disease progression. These vessels were of both arterial and venous origin as shown by early phase FFA. Dilated capillaries and RAVs in OCT-A corresponded to hyper-reflective alterations of the outer retina on SD-OCT scans. In 19/19 eyes, NVs were associated with the presence of RAVs, and RAVs were shown to directly connect to neovascular complexes and to undergo morphological changes upon NV formation.ConclusionsThe results emphasise the role of RAVs during disease progression from an early stage on and demonstrate their involvement in the development of secondary NVs in MacTel.

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Multimodal characterization of macular telangiectasia type 2 and correlation with multifocal electroretinogram

10.21203/rs.3.rs-178402/v1 ◽

2021 ◽

Author(s):

Apoorva Ayachit ◽

Lakshmipriya Uday Reddy ◽

Shrinivas Joshi ◽

Guruprasad Ayachit

Keyword(s):

Multimodal Imaging ◽

Multifocal Electroretinogram ◽

Imaging Systems ◽

Control Group ◽

Macular Telangiectasia ◽

Macular Telangiectasia Type 2 ◽

Stage 1 ◽

Sd Oct

Abstract 1. Purpose: To study the correlation of multimodal imaging in macular telangiectasia (Mac Tel) with foveal function on multifocal electroretinogram (mfERG)2. Methods: Eyes with non- proliferative Mac Tel diagnosed based on clinical examination, optical coherence tomography (OCT), autofluorescence (AF), fluorescein angiography (FA), OCT angiography (OCTA). Control group with normal eye exam included for multifocal electroretinogram.Staging of OCT, OCTA, AF and FA done in Mac Tel subjects. mfERG done in study subjects and controls. Correlation of imaging modalities and P1 amplitudes at fovea (ring 1) studied in terms of correlation co-efficient.3. Results: Twenty nine eyes of 16 patients of Mac Tel and 25 eyes of 19 controls were included. BCVA was 0.38 ± 0.266 in study eyes and 0 in control eyes. On OCT it was observed that 41.4% Mac Tel eyes (n = 12) belonged to stage 3, 37.9%(n = 11)) eyes belonged to stage 2 and 20.7% (n = 6) eyes belonged to stage 1. AF- Stage 3 comprised of 75.9% eyes (n = 22); 4 eyes belonged to stage 2 and 3 eyes to stage 1. On FA, 18 eyes belonged to stage 3 (62.1%); stage 2 was seen in 1 (3.4%) eye and stage 1 was seen in 10 (34.5%) eyes. There was decrease in P1 amplitudes from R1(p < 0.001), R2( 0.001), R3 (< 0.001) and R4 (0.001) in Mac Tel eyes compared to control eyes but not in R5 (p 0.785). SD- OCT had positive correlation with FAF (CC 0.747,p < 0.001) FFA (CC 0.775, p < 0.001) and R1P1 (CC 0.682, p < 0.001). With OCTA there was no significant correlation (CC 0.318, p 0.093). There was positive and significant correlation of OCT (0.682,<0.001), OCTA (0.379,p 0.042) AF ( 0.635, p < 0.001) and FA (0.495, p < 0.006) with R1P1.4. Conclusions: Existing multimodal imaging systems can be reliable indicators of foveal function as on mfERG.

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Topographic Relationship between Telangiectasia and Cone Mosaic Disruption in Macular Telangiectasia Type 2

Journal of Clinical Medicine ◽

10.3390/jcm9103149 ◽

2020 ◽

Vol 9 (10) ◽

pp. 3149

Author(s):

Roya Zandi ◽

Jessica Song ◽

Paul S. Micevych ◽

Amani A. Fawzi

Keyword(s):

Adaptive Optics ◽

Early Stage ◽

Cross Sectional ◽

Photoreceptor Layer ◽

Cone Mosaic ◽

Spectral Domain Oct ◽

Macular Telangiectasia ◽

Macular Telangiectasia Type 2 ◽

Sd Oct

In this cross-sectional observational study, we investigated the relationship between photoreceptor layer disruption and telangiectasia in patients diagnosed with early stage macular telangiectasia type 2 (MacTel). A total of 31 eyes (17 patients) with MacTel were imaged with adaptive optics scanning laser ophthalmoscopy (AOSLO) and optical coherence tomography angiography (OCTA). Confocal AOSLO was used to visualize dark regions of nonwaveguiding outer segments, which we refer to as “photoreceptor lesions”. En-face OCTA images of the deep capillary plexus (DCP) were used in conjunction with confocal AOSLO to evaluate the topographic relationship between areas of capillary telangiectasias and photoreceptor lesions. Among seven eyes with early stage MacTel (stage 0–2 based on OCT), we identified ten photoreceptor lesions, all of which were located within parafoveal quadrants containing DCP telangiectasia on OCTA. Seven of the lesions corresponded to the intact ellipsoid zone on spectral-domain OCT (SD-OCT), and three of these also corresponded to the intact interdigitation zone. This work demonstrates a topographic relationship between AOSLO photoreceptor lesions and DCP telangiectasias, and it also suggests that these lesions with normal SD-OCT appearance may represent areas of photoreceptors at risk for dysfunction. Thus, confocal AOSLO may have a meaningful role in detecting early photoreceptor abnormalities in eyes with MacTel.

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

Scientific Reports ◽

10.1038/s41598-020-73803-9 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Young Ho Kim ◽

Yoo-Ri Chung ◽

Jaeryung Oh ◽

Seong-Woo Kim ◽

Christopher Seungkyu Lee ◽

...

Keyword(s):

Visual Acuity ◽

Macular Hole ◽

Early Stage ◽

Disease Process ◽

Disease Stage ◽

Optical Coherence ◽

Macular Telangiectasia ◽

Macular Telangiectasia Type 2 ◽

Sd Oct

Abstract 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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