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.

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.

IDIOPATHIC MACULAR TELANGIECTASIA TYPE 2: DISTRIBUTION OF MACULAR PIGMENT AND FUNCTIONAL INVESTIGATIONS

Retina ◽  
2010 ◽  
Vol 30 (4) ◽  
pp. 586-595 ◽  
Author(s):  
MEIKE B. ZEIMER ◽  
BJÖRN PADGE ◽  
BRITTA HEIMES ◽  
DANIEL PAULEIKHOFF
Keyword(s):  
Macular Pigment ◽  
Macular Telangiectasia ◽  
Macular Telangiectasia Type 2 ◽  
Idiopathic Macular Telangiectasia

scholarly journals Quantifying the abnormal macular pigment distribution in macular telangiectasia type 2

2008 ◽  
Vol 86 ◽  
pp. 0-0
Author(s):  
LP VAN DER VEEN ◽  
P CHARBEL-ISSA ◽  
A STIJFS ◽  
FG HOLZ ◽  
HPN SCHOLL ◽  
...  
Keyword(s):  
Macular Pigment ◽  
Macular Telangiectasia ◽  
Pigment Distribution ◽  
Macular Telangiectasia Type 2

Choroidal thickness and vascular density in macular telangiectasia type 2 using enface swept-source optical coherence tomography

2019 ◽  
Vol 103 (11) ◽  
pp. 1584-1589 ◽  
Author(s):  
Jay C Wang ◽  
Inês Laíns ◽  
Patrick Oellers ◽  
Ivana K Kim ◽  
Joan W Miller ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Choroidal Thickness ◽  
Fundus Photography ◽  
Imaging Features ◽  
Optical Coherence ◽  
Swept Source ◽  
Macular Telangiectasia ◽  
Macular Telangiectasia Type 2 ◽  
And Control

PurposeTo investigate the choroidal thickness (CT) and choroidal vascular densities (CVD) of patients with macular telangiectasia type 2 (MacTel2) and their association with other multimodal imaging features, using swept-source optical coherence tomography (SS-OCT).MethodsProspective, cross-sectional study. Consecutive patients with MacTel2 along with controls without any macular disease were included. Fundus photography, confocal blue reflectance, near-infrared reflectance, autofluorescence, fluorescein angiography, spectral domain OCT and SS-OCT were performed. Images were independently analysed by two graders, and CVD was calculated from binarised en face SS-OCT images. CT was obtained from the SS-OCT platform via built-in automated segmentation. Multilevel mixed-effects models were used for statistical analysis.ResultsThirty-nine eyes of 20 patients with MacTel2 and 29 eyes of 15 control patients were included. Average CT and perifoveal temporal CT did not differ significantly between eyes with MacTel2 and control eyes (p≥0.350), when accounting for confounding factors. Overall and temporal CVD also did not significantly differ between the two groups (p≥0.490).ConclusionCT and CVD did not significantly differ between MacTel2 and control eyes in this study using SS-OCT. Even though MacTel2 may include abnormalities involving the choroid, these are likely minor in comparison to the predominant retinal changes.

Quantification of reduced macular pigment optical density in the central retina in macular telangiectasia type 2

2009 ◽  
Vol 89 (1) ◽  
pp. 25-31 ◽  
Author(s):  
Peter Charbel Issa ◽  
Rob L.P. van der Veen ◽  
Astrid Stijfs ◽  
Frank G. Holz ◽  
Hendrik P.N. Scholl ◽  
...  
Keyword(s):  
Optical Density ◽  
Macular Pigment ◽  
Macular Pigment Optical Density ◽  
Macular Telangiectasia ◽  
Central Retina ◽  
Macular Telangiectasia 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.

Sign in / Sign up

Export Citation Format

Share Document