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.

Morphological characteristics preceding exudative neovascularisation secondary to macular telangiectasia type 2

2021 ◽  
pp. bjophthalmol-2020-318470
Author(s):  
Kristina Hess ◽  
Peter Charbel Issa ◽  
Frank G Holz ◽  
Simone Tzaridis
Keyword(s):  
De Novo ◽  
Morphological Changes ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Morphological Characteristics ◽  
Macular Pigment ◽  
Fundus Photography ◽  
Macular Telangiectasia ◽  
Macular Telangiectasia Type 2

AimsTo identify morphological characteristics preceding the development of exudative neovascularisation secondary to Macular Telangiectasia type 2 (MacTel) using multimodal retinal imaging.MethodsIn this retrospective study, eyes with a minimum observation period of 6 months prior to the de novo diagnosis of an exudative neovascularisation secondary to MacTel were analysed. Morphological changes preceding the formation of neovascularisation were evaluated using colour fundus photography, infrared imaging, fluorescein angiography, macular pigment measurement and optical coherence tomography (OCT). OCT-angiography (OCT-A) images were additionally available in a subset of patients.ResultsTwenty eyes from 20 patients were examined over a median period of 17 months (range: 6–100 months). Eyes were characterised by an accelerated progression of ellipsoid zone loss (median of 0.013 mm2/month), increased thickness of the temporal parafovea and hyper-reflective lesions on OCT. The latter underwent morphological changes preceding the development of exudative neovascularisation, including an increase in size and density, and expansion to outer retinal layers and the retinal pigment epithelium. All eyes showed a foveal depletion of macular pigment. On OCT-A, a focal increase in blood flow was observed at the level of the outer retina/choriocapillaris, and retinal–retinal and retinal–choroidal anastomoses preceded the formation of exudative neovascularisation.ConclusionsMultimodal imaging allows the identification of prognostic morphological features preceding the formation of exudative neovascularisation in MacTel. Eyes exhibiting these characteristics should be monitored closely and patients should be alert for emergent symptoms in order to detect and treat neovascularisation early and, thereby, prevent irreversible visual loss.

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 Improvements in visual acuity and macular morphology following cessation of anti-estrogen drugs in a patient with anti-estrogen maculopathy resembling macular telangiectasia type 2: a pathogenic hypothesis

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Akihiro Shinkai ◽  
Wataru Saito ◽  
Yuki Hashimoto ◽  
Susumu Ishida
Keyword(s):  
Visual Acuity ◽  
Vision Loss ◽  
Pigment Epithelium ◽  
Fundus Autofluorescence ◽  
Retinal Pigment ◽  
Macular Telangiectasia ◽  
History Of ◽  
Macular Telangiectasia Type 2 ◽  
The Relationship

Abstract Background The relationship between anti-estrogen drugs and macular telangiectasia type 2 (MacTel-2) remains unknown. Here we report a case with anti-estrogen maculopathy resembling MacTel-2 with improved visual function and macular morphology following cessation of anti-estrogen drugs. Case presentation A 53-year-old woman presented with a 5-month history of central vision loss and anorthopia in both eyes. She had received oral tamoxifen followed by toremifene for 69 months. Funduscopy, fluorescein angiography, and optical coherence tomography (OCT) revealed MacTel-2-like findings OU. Fundus autofluorescence (FAF) showed hyper-autofluorescence at the fovea OU. Visual acuity, macular morphology on OCT, and FAF findings gradually improved after cessation of anti-estrogen drugs. Conclusions In the present case, visual acuity, macular morphology, and impairment of the retinal pigment epithelium (RPE) improved following cessation of anti-estrogen drugs, suggesting the relationship between retinal toxicity of anti-estrogen drugs and the development of MacTel-2-like findings. From these results and the previous observations, toxicity of both photoreceptor and RPE cells caused by anti-estrogen drugs may contribute to the development of anti-estrogen maculopathy similar to MacTel-2.

Macular thickness profile and diabetic retinopathy: the Singapore Epidemiology of Eye Diseases Study

2017 ◽  
Vol 102 (8) ◽  
pp. 1072-1076 ◽  
Author(s):  
Wei Dai ◽  
Yih Chung Tham ◽  
Ning Cheung ◽  
Masayuki Yasuda ◽  
Nicholas Y Q Tan ◽  
...  
Keyword(s):  
Diabetic Retinopathy ◽  
Macular Oedema ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Plexiform Layer ◽  
Glycated Haemoglobin ◽  
Eye Diseases ◽  
Inner Plexiform Layer ◽  
Outer Retina ◽  
Sd Oct

BackgroundTo evaluate retinal thickness profiles in eyes with and without diabetic retinopathy (DR) using spectral-domain optical coherence tomography (SD-OCT) among individuals with diabetes.MethodsParticipants were recruited from the Singapore Epidemiology of Eye Diseases Study, a population-based study among Chinese, Malays and Indians aged 40 years or older residing in Singapore. All participants underwent standardised systemic and ophthalmic examinations. Average thickness of the macula, ganglion cell-inner plexiform layer and outer retina layer (from the outer plexiform layer to the retinal pigment epithelium layer) were measured using SD-OCT. DR status and severity were graded based on fundus photographs using the modified Airlie House classification system. Participants with macular oedema were excluded.Results2240 eyes from 1280 participants were included. Of these, 1764 (78.7%) eyes had no DR, 351 (15.7%) eyes had minimal or mild DR and 125 (5.6%) eyes had moderate or worse DR. After adjusting for age, gender, ethnicity, axial length, hypertension, glycated haemoglobin, body mass index, total cholesterol and diabetes duration, eyes with DR had thicker macula (245.44 µm vs 243.04 µm, P=0.03) and outer retina (124.26 µm vs 123.08 µm, P=0.01) than eyes without DR. When stratified by DR severity, thicker macula (250.24 µm vs 242.88 µm, P=0.011) and outer retina (126.4 µm vs 123.0 μm, P=0.006) were observed in eyes with moderate or worse, but not minimal or mild DR, compared with eyes without DR.ConclusionsEven in the absence of macular oedema, eyes with DR, particularly those with more severe DR, had thicker macular and outer retinal layers than eyes without DR.

scholarly journals Optical Coherence Tomography Findings (SD-OCT and OCTA) in Early-Stage Type 3 Neovascularization

2020 ◽  
Vol 11 (2) ◽  
pp. 493-499
Author(s):  
Nisa Silva ◽  
Ana Marta ◽  
Pedro Baptista ◽  
Maria João Furtado ◽  
Miguel Lume
Keyword(s):  
Optical Coherence Tomography ◽  
Early Stage ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Optical Coherence ◽  
Outer Retina ◽  
Type 3 Neovascularization ◽  
Intraretinal Fluid ◽  
Type 3 ◽  
Sd Oct

A 76-year-old male presented with a small hyperreflective density in the outer nuclear layer with subtle retinal pigment epithelium (RPE) elevation and few intraretinal cysts on spectral-domain optical coherence tomography (SD-OCT). Optical coherence tomography angiography (OCTA) confirmed the presence of a tuft-shaped intraretinal neovascular lesion. SD-OCT performed 2 months before showed a smaller RPE elevation at the same location without intraretinal fluid. A 79-year-old male presented with a small hyperreflective density in the outer retina surrounded by scant intraretinal fluid on SD-OCT and a bright vessel on OCTA, suggesting early-stage type 3 neovascularization. SD-OCT performed 2 months before showed a smaller hyperreflectivity at the same location, without intraretinal fluid. An 84-year-old female presented with hyperreflective foci in the outer retina overlying a serous pigment epithelium detachment (PED) with focal RPE disruption on SD-OCT. SD-OCT performed 2 months before showed the same hyperreflective lesion associated with a shallower PED. No neovascular lesions were found on OCTA after six injections of bevacizumab. To conclude, careful evaluation of SD-OCT allows for early detection of type 3 neovascularization at a pre-exudative stage. OCTA may be useful in confirming the presence of intraretinal neovascular lesion and monitoring response to anti-vascular endothelial growth factor agents.

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 Alterations of Retinal Pigment Epithelium–Photoreceptor Complex in Patients with Type 2 Diabetes Mellitus without Diabetic Retinopathy: A Cross-Sectional Study

2020 ◽  
Vol 2020 ◽  
pp. 1-6 ◽  
Author(s):  
Zheren Xia ◽  
Hao Chen ◽  
Suilian Zheng
Keyword(s):  
Diabetes Mellitus ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Cross Sectional Study ◽  
Healthy Controls ◽  
Sectional Study ◽  
Cross Sectional ◽  
Type 2 Dm ◽  
Sd Oct

Aim. A cross-sectional study was performed to examine the alterations of the retinal pigment epithelium– (RPE–) photoreceptor complex layer in type 2 diabetes mellitus (DM) without diabetic retinopathy (DR), using spectral-domain optical coherence tomography (SD-OCT). Methods. Patients with type 2 DM without DR and healthy controls without DM were recruited. All participants underwent examinations including SD-OCT. The thickness measurements of the retinal neural layers were calculated after automatic segmentation. An independent-sample t-test was used to compare the means of the thickness of retinal neural layers in patients with DM and healthy controls. Results. Sixty-seven eyes from 67 patients with DM and 30 eyes from 30 healthy controls were included in this study. No significant differences were found in age (P = 0.601), gender (P = 0.560), axial length (P = 0.414), best-corrected visual acuity (P = 0.963), or intraocular pressure (P = 0.112) between the two groups. There were significant increases in the hemoglobin A1c value (P < 0.001) and mean thicknesses of the RPE–photoreceptor complex layer in the foveal area (P = 0.027) and paracentral area (P = 0.001) in the DM group compared to the control group, whereas the thickness of the retinal nerve fiber and ganglion cell layers in the foveal and paracentral areas between the two groups showed no significant differences. Conclusion. Lesions in the RPE–photoreceptor complex are present without vascular abnormalities, which may precede the alterations of ganglion cells in patients with type 2 DM.

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.

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