scholarly journals Early-stage macular telangiectasia type 2 vascular abnormalities are associated with interdigitation zone disruption

PLoS ONE ◽  
2021 ◽  
Vol 16 (11) ◽  
pp. e0259811
Author(s):  
Janice X. Ong ◽  
Roya Zandi ◽  
Amani A. Fawzi
Keyword(s):  
Early Stage ◽  
Cross Sectional ◽  
Photoreceptor Layer ◽  
Spectral Domain Oct ◽  
Macular Telangiectasia ◽  
Macular Telangiectasia Type 2 ◽  
Interdigitation Zone ◽  
Longitudinal Imaging ◽  
Sd Oct

Purpose To investigate the relationship between disruption in different photoreceptor layers and deep capillary plexus (DCP) telangiectasias in eyes with macular telangiectasia type 2 (MacTel). Methods 35 eyes (21 patients) with MacTel imaged with optical coherence tomography angiography (OCTA) were included. Circumscribed areas of DCP telangiectasia were traced from OCTA slabs and the corresponding spectral-domain OCT (SD-OCT) slabs were used to visualize the photoreceptor layer interdigitation zone (IZ) and ellipsoid zone (EZ). IZ attenuation, IZ loss, and EZ loss were graded by reviewing en face SD-OCT slabs for hypo-reflective areas and confirming their status on cross-sectional views. Total area of photoreceptor disruption and overlap with DCP telangiectasia were evaluated with respect to OCT-based MacTel stage. Longitudinal changes were evaluated in a subset of patients with follow-up imaging. Results Overlap of DCP telangiectasia with IZ attenuation significantly decreased with MacTel severity, while overlap with IZ and EZ loss significantly increased. Overlap with IZ loss peaked in moderate MacTel (Stages 3–5). Longitudinal imaging showed that new EZ loss at 6 months was largely predicted by baseline IZ loss. Conclusions Worsening MacTel severity is characterized by greater overlap between DCP telangiectasia and zones of increasing severity of photoreceptor disruption, with EZ loss enlarging over time within areas of preexisting IZ disruption. We suggest that IZ disruption may indicate early photoreceptor dysfunction that eventually progresses to EZ loss, with IZ loss being a more reliable metric than IZ attenuation. Additional studies will be necessary to further explore long-term photoreceptor changes and evaluate their relationship with visual function in MacTel.

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

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.

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

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.

scholarly journals Contrast sensitivity and visual acuity under low light conditions in macular telangiectasia type 2

2018 ◽  
Vol 103 (3) ◽  
pp. 398-403 ◽  
Author(s):  
Simone Müller ◽  
Tjebo F C Heeren ◽  
Roberto Bonelli ◽  
Marcus Fruttiger ◽  
Peter Charbel Issa ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Contrast Sensitivity ◽  
Neutral Density Filter ◽  
Light Conditions ◽  
Low Light ◽  
Cross Sectional ◽  
Macular Telangiectasia ◽  
Low Luminance ◽  
Macular Telangiectasia Type 2

Background/AimMacular pigment optical density (MPOD) is centrally depleted early on in macular telangiectasia type 2 (MacTel). Contrast sensitivity (CS) might be related to MPOD, and thus impaired in early MacTel. The effect of low luminance was assessed on both CS and best corrected visual acuity (BCVA).MethodsThis is a cross-sectional study. Pelli-Robson charts were used for CS testing at 1 m in photopic (110 lux) and mesopic (1 lux) conditions. BCVA was tested with ETDRS charts and low luminance visual acuity (LLVA) with a 2.0 log unit neutral density filter. MPOD was obtained with dual-wavelength autofluorescence.ResultsOne hundred and three eyes of 52 patients with MacTel (mean±SD age 62.9±10.2, range 35–77) were compared with 34 healthy eyes of 17 controls (mean±SD age 65.2±7.4, range 53–78). CS was significantly lower in the eyes with MacTel. This impairment was higher in low light conditions (low light contrast sensitivity (LL-CS)). Eyes at the early stages of MacTel had significantly lower LL-CS than controls, but normal (photopic) CS. The results were similar but less pronounced for BCVA/LLVA. Decrease in CS was correlated with loss of MPOD.ConclusionsLow light conditions have a detrimental effect on visual performance in MacTel. Impaired CS might correlate with MPOD depletion as a pathognomonic finding in MacTel. Functional impairment might precede structural disintegration, indicating dysfunction at the cellular level. The applied tests might be useful as additional functional assessments in clinical routine and as outcome measures in future interventional clinical trials.

scholarly journals Characterizing right-angled vessel in macular telangiectasia type 2 with structural optical coherence tomography

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yoo-Ri Chung ◽  
Young Ho Kim ◽  
Jaeryung Oh ◽  
Seong-Woo Kim ◽  
Christopher Seungkyu Lee ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Cross Sectional Study ◽  
Fundus Photography ◽  
Optical Coherence ◽  
Cross Sectional ◽  
Macular Telangiectasia ◽  
Advanced Phase ◽  
Tertiary Hospitals ◽  
Sd Oct

AbstractWe investigated the structural findings on spectral-domain optical coherence tomography (SD-OCT) related to the presence of right-angled vessels (RAV) in patients with macular telangiectasia (MacTel) type 2 with severity 3 in Korea. A retrospective multicenter cross-sectional study was conducted in six tertiary hospitals in Korea; the study included 116 MacTel type 2 eyes with severity 3. The SD-OCT findings were compared between eyes with RAV on fundus photography or fluorescein angiography and those without RAV. Logistic regression was performed to determine factors associated with the presence of RAV. Fifty eyes presented with RAV and 61 eyes without RAV. More eyes presented with only inner retinal (IR) cavities on SD-OCT among eyes without RAV than among those with RAV (P < 0.001). However, eyes with RAV presented with IR disorganization, outer retinal (OR) cavity, and ellipsoid zone (EZ) disruption more frequently than eyes without RAV did (all P < 0.001). These SD-OCT findings were significantly associated with the presence of RAV. The presence of RAV was closely related to IR disorganization, OR cavities, and EZ disruption on SD-OCT. These findings suggest an advanced phase of MacTel type 2.

Funduscopic and Angiographic Characteristics in Early and Late Macular Telangiectasia Type 2

2017 ◽  
Vol 1 (6) ◽  
pp. 372-378
Author(s):  
Durgasri Jaisankar ◽  
Harshal Gondhale ◽  
Jay Chhablani ◽  
Manisha Agarwal ◽  
Rajiv Raman
Keyword(s):  
Retinal Pigment ◽  
Female Gender ◽  
Early Group ◽  
Cross Sectional ◽  
Macular Telangiectasia ◽  
Stage Disease ◽  
Macular Telangiectasia Type 2 ◽  
Late Stages ◽  
Late Group

Purpose: To report the morphological and vascular characteristics using funduscopic and angiographic features in different cohort as stratified by stages of macular telangiectasia type 2 (MacTel 2). Methods: A cross-sectional, multicentered study of 149 eyes (75 patients) with MacTel 2 was done. The eyes were divided into 2 groups: early group which includes eyes with stages 1 (40 eyes), 2 (41 eyes), and 3 (32 eyes) of MacTel 2 and late group which includes eyes with stages 4 (13 eyes) and 5 (23 eyes) of MacTel 2. The morphological and vascular characteristics on fundus examination and fluorescein angiography (FA) were studied in both groups and their association was found. Results: The gender (female: odds ratio [OR] = 4.98, P = .040), presence of dilated parafoveal capillaries (OR = 2.668, P = .026), retinal pigment plaques (OR = 27.067, P = .0001), patchy choroidal filling in FA (OR = 3.536, P = .004), and presence of prominent arteriole (OR = 3.434, P = .002) were associated with late stages of MacTel 2. Conclusion: Presence of retinal pigment plaques, dilated parafoveal capillaries, patchy choroidal filling, and female gender are more likely to be associated with late-stage disease.

scholarly journals Right-angled vessels in macular telangiectasia type 2

2019 ◽  
pp. bjophthalmol-2018-313364 ◽  
Author(s):  
Simone Tzaridis ◽  
Tjebo Heeren ◽  
Clarissa Mai ◽  
Sarah Thiele ◽  
Frank G Holz ◽  
...  
Keyword(s):  
Disease Progression ◽  
Morphological Changes ◽  
Fundus Photography ◽  
Outer Retina ◽  
Macular Telangiectasia ◽  
Age Related ◽  
Macular Telangiectasia Type 2 ◽  
Sd Oct

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.

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

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.

Incidence and phenotypical variation of outer retina-associated hyperreflectivity in macular telangiectasia type 2

2021 ◽  
pp. bjophthalmol-2020-317997
Author(s):  
Stefanie Mueller ◽  
Frederic Gunnemann ◽  
Kai Rothaus ◽  
Marius Book ◽  
Henrik Faatz ◽  
...  
Keyword(s):  
Disease Severity ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Outer Retina ◽  
Macular Telangiectasia ◽  
Macular Telangiectasia Type 2 ◽  
Disease Severity Scale ◽  
Variable Presentation ◽  
Sd Oct

BackgroundMacular telangiectasia type 2 (MacTel) is a neurodegenerative disease resulting in photoreceptor loss. Optical coherence tomography (OCT) reveals outer retina-associated hyperreflectivity (ORaH) as part of this process. The purpose of this study was to describe the incidence and phenotypical variation of ORaH.MethodsDifferent parameters of ORaH were analysed: OCT characteristics (Spectralis SD-OCT), correlation with vascular changes (OCT angiography; OCTA 3×3 mm Optovue) and correlation with hyperpigmentation (autofluorescence/fundus images). ORaH was also evaluated regarding the grade of severity of photoreceptor loss (Disease Severity Scale).ResultsOf 220 eyes with MacTel type 2, 106 demonstrated ORaH. On OCT, the size, the extension into the inner retina and the contact with retinal pigment epithelium (RPE) of the ORaH were variable. On OCTA neovascularisation (NV) in the outer retina (OR) was present at the location of the ORaH in 97.6%. Increasing size of NV correlated with progressive photoreceptor loss. In 86.6% with NV, the flow signals were visible between the OR and the choriocapillaris. In 85.7%, the ORaH was associated with hyperpigmentation on autofluorescence and fundus colour images.ConclusionsThe presence of ORaH is associated with increasing photoreceptor loss and disease severity. In these more advanced cases of the present study, a variable presentation of ORaH in respect to size and form was seen, but in most cases, ORaH was in contact to the RPE. Additionally, ORaH was associated with hyperpigmentation and OR NV on OCTA. These results are consistent with the concept of ORaH representing fibrovascular OR-NV with RPE proliferation after contact with the RPE.

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