Idiopathic Juxtafoveal Retinal Telangiectasis: New Findings by Ultrahigh-Resolution Optical Coherence Tomography

doi:10.1016/j.ophtha.2005.08.016

Ophthalmology

Volume 113, Issue 1, January 2006, Pages 48-57

https://doi.org/10.1016/j.ophtha.2005.08.016 Get rights and content

Objective

To investigate the capabilities of ultrahigh-resolution optical coherence tomography (UHR OCT); to compare with the commercially available OCT standard-resolution system, StratusOCT, for imaging of idiopathic juxtafoveal retinal telangiectasis (IJT); and to demonstrate that UHR OCT provides additional information on disease morphology, pathogenesis, and management.

Design

Retrospective, observational, interventional case series.

Participants

Nineteen eyes of 10 patients diagnosed with IJT in at least one eye.

Method

All patients were imaged with UHR OCT and StratusOCT at the same visit. A subset of patients was also imaged before and after treatment of IJT.

Main Outcome Measures

Ultrahigh- and standard-resolution cross-sectional tomograms of IJT pathology.

Results

Using both standard- and ultrahigh-resolution OCT, we identified the following features of IJT: (1) a lack of correlation between retinal thickening on OCT and leakage on fluorescein angiography, (2) loss and disruption of the photoreceptor layer, (3) cystlike structures in the foveola and within internal retinal layers such as the inner nuclear or ganglion cell layers, (4) a unique internal limiting membrane draping across the foveola related to an underlying loss of tissue, (5) intraretinal neovascularization near the fovea, and (6) central intraretinal deposits and plaques. In 63% of cases, the presence of abnormal vessels and a discontinuity of the photoreceptor layer correlated with visual acuity.

Conclusions

Ultrahigh-resolution OCT improves visualization of the retinal pathology associated with IJT and allows identification of new features associated with it. Some of these features, such as discontinuity of the photoreceptor layer, are revealed only by UHR OCT.

Section snippets

Materials and Methods

A prototype clinical UHR OCT system suitable for performing studies in an ophthalmology clinic was developed. A femtosecond titanium:sapphire laser with a ∼125-nm bandwidth centered at the wavelength of 815 nm was developed and used as the OCT imaging light source.²² The UHR OCT system achieved axial imaging resolutions of ∼3 μm in the eye. The UHR OCT prototype system was integrated on a slit-lamp biomicroscope that was adapted with a charge-coupled device to provide a video image of the

Results

Standard-resolution and UHR OCT images of a normal macula are presented in Figure 1. The intraretinal layer morphology in the OCT images correlates well with the histological morphology of the retina in the macular region.²⁴ The nerve fiber layer and the plexiform layers are more optically backscattering than the nuclear layers and presented as red, yellow, or bright green false color in the OCT images.25, 26, 27, 28 In both OCT images, the first highly backreflecting layer is the nerve fiber

Discussion

A comparative imaging study was performed using the UHR OCT and StratusOCT systems on patients with clinically diagnosed IJT. Both OCT systems can noninvasively acquire retinal images that are capable of differentiating most major intraretinal layers, such as the retinal nerve fiber, inner plexiform, inner nuclear, outer plexiform, and outer nuclear, as previously demonstrated on other pathologies.9, 10, 20, 21, 25, 26 In our study, the StratusOCT, at its best resolution, is capable of

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Citation Excerpt :
Structural OCT shows the presence of macular cystoid spaces in different retinal layers. Inner macular cystoid spaces are located immediately behind the ILM while outer cavitations disrupt the ONL and the EZ (Gaudric et al., 2006; Paunescu et al., 2006; Yannuzzi et al., 2006). FA shows a mild leakage and staining of the retinal tissue around the telangiectasia, but interestingly, the larger cystoid spaces visible on OCT in the foveal center do not fill with dye (Koizumi et al., 2006) (Fig. 24).
Besides cystoid macular edema due to a blood-retinal barrier breakdown, another type of macular cystoid spaces referred to as non-vasogenic cystoid maculopathies (NVCM) may be detected on optical coherence tomography but not on fluorescein angiography. Various causes may disrupt retinal cell cohesion or impair retinal pigment epithelium (RPE) and Müller cell functions in the maintenance of retinal dehydration, resulting in cystoid spaces formation. Tractional causes include vitreomacular traction, epiretinal membranes and myopic foveoschisis. Surgical treatment does not always allow cystoid space resorption. In inherited retinal dystrophies, cystoid spaces may be part of the disease as in X-linked retinoschisis or enhanced S-cone syndrome, or occur occasionally as in bestrophinopathies, retinitis pigmentosa and allied diseases, congenital microphthalmia, choroideremia, gyrate atrophy and Bietti crystalline dystrophy. In macular telangiectasia type 2, cystoid spaces and cavitations do not depend on the fluid leakage from telangiectasia. Various causes affecting RPE function may result in NVCM such as chronic central serous chorioretinopathy and paraneoplastic syndromes. Non-exudative age macular degeneration may also be complicated by intraretinal cystoid spaces in the absence of fluorescein leakage. In these diseases, cystoid spaces occur in a context of retinal cell loss. Various causes of optic atrophy, including open-angle glaucoma, result in microcystoid spaces in the inner nuclear layer due to a retrograde transsynaptic degeneration. Lastly, drug toxicity may also induce cystoid maculopathy. Identifying NVCM on multimodal imaging, including fluorescein angiography if needed, allows guiding the diagnosis of the causative disease and choosing adequate treatment when available.
Idiopathic macular telangiectasia type 2: Prevalence and a morphometric and phenotypic study
2018, Archivos de la Sociedad Espanola de Oftalmologia
Las telangiectasias maculares idiopáticas de tipo 2 (MacTel 2) son una enfermedad retiniana poco frecuente y aún no completamente caracterizada. El objetivo del presente trabajo es describir las características clínicas en imagen multimodal de la retina, presentar los resultados funcionales y estimar la prevalencia de la enfermedad en nuestro medio.
Estudio retrospectivo en el que se analizaron 12 ojos de 6 pacientes con MacTel 2. Se realizaron fotografías en color de fondo, autofluorescencia, angiografía fluoresceínica, tomografía de coherencia óptica (OCT) y angiografía por OCT y se analizaron posteriormente. Se registró prospectivamente la agudeza visual. La prevalencia se estableció en base en los pacientes referidos a una unidad especializada en enfermedades maculares. El período mínimo de seguimiento fue de 18 meses.
La prevalencia de MacTel 2 en nuestro medio fue de 0,12%. Se presentaron las características clínicas en imagen multimodal de la retina. La agudeza visual permaneció estable durante el seguimiento (p = 0,492). Tres pacientes desarrollaron neovascularización coroidea (NVC), requiriendo tratamiento intravítreo con agentes antiangiogénicos.
La prevalencia de la enfermedad en nuestro medio es del 0,12%. El estudio mediante imágenes multimodales permite un diagnóstico y seguimiento de esta patología más preciso. La agudeza visual se mantuvo durante los 18 meses de seguimiento. Los pacientes que desarrollan NVC y son tratados con agentes antiangiogénicos parecen responder adecuadamente a ellos. Se necesitan más estudios para establecer estas conclusiones.
Type 2 idiopathic macular telangiectasia (MacTel 2) is a rare retinal disease that has still not been well-defined. The aim of the present manuscript is to describe the clinical features by multimodal retinal imaging, to present the functional characteristics, and to estimate the prevalence of the disease.
A retrospective study was conducted on the 12 eyes of 6 patients with MacTel 2. Fundus colour photographs, fundus autofluorescence, fluorescein angiography, optical coherence tomography (OCT), and OCT-angiography were performed and subsequently analysed. Visual acuity (VA) was prospectively recorded. The prevalence was established based on the patients referred to a specialised macular diseases unit. Minimum follow-up period was 18 months.
Prevalence of MacTel 2 in our study was 0.12%. Clinical features were presented using multimodal retinal imaging. VA remained stable during follow-up. Three patients developed choroidal neovascularisation (CNV), requiring intravitreal treatment with antiangiogenic agents.
The prevalence of the disease found was 0.12%. The study using multimodal imaging allows a more accurate diagnosis and follow-up of this pathology. The VA is maintained during the 18-month follow-up (P = .492). Patients who develop CNV and are treated with antiangiogenic agents appear to respond adequately to them. More studies are needed to establish these conclusions.
Semi-automated quantification of retinal IS/OS damage in en-face OCT image
2016, Computers in Biology and Medicine
A variety of vision ailments are indicated by structural changes in the retinal substructures of the posterior segment of the eye. In particular, integrity of the inner-segment/outer-segment (IS/OS) junction directly relates to the visual acuity. In the en-face optical coherence tomography (OCT) image, IS/OS damage manifests as a dark spot in the foveal region, and its quantification, usually performed by experts, assumes diagnostic significance. In this context, in view of the general scarcity of experts, it becomes imperative to develop algorithmic methods to reduce expert time and effort. Accordingly, we propose a semi-automated method based on level sets. As the energy function, we adopt mutual information which exploits the difference in statistical properties of the lesion and its surroundings. On a dataset of 27 en-face OCT images, segmentation obtained by the proposed algorithm exhibits close visual agreement with that obtained manually. Importantly, our results also match manual results in various statistical criteria. In particular, we achieve a mean Dice coefficient of 85.69%, desirably close to the corresponding observer repeatability index of 89.45%. Finally, we quantify algorithmic accuracy in terms of two quotient measures, defined relative to observer repeatability, which could be used as bases for comparison with future algorithms, even if the latter are tested on disparate datasets.
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Citation Excerpt :
Furthermore intraretinal hyporeflective spaces, that have been named as internal limiting membrane drape, cyst, cystoid, or pseudocystoid spaces by different authors, are commonly seen but are usually not related to retinal thickening or fluorescein angiographic leakage21,34–41 (Fig. 6). Interestingly, foveal thickness is decreased in most patients with Mac Tel 2.11,33–37,42 Spectral domain OCT has also documented the presence of outer retina abnormalities1 (Fig. 5).
Macular telangiectasia type 2 (Mac Tel 2) also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A is an enigmatic disease of unknown etiology. It manifests both neurodegenerative and vasculopathic characteristics.
It manifests itself during the fifth or sixth decades of life. Clinical characteristics include minimally dilated parafoveal capillaries with loss of the retinal transparency in the area involved, absence of lipid exudation, right-angled retinal venules, superficial retinal refractile deposits, hyperplasia of the retinal pigment epithelium (RPE), foveal atrophy and subretinal neovascularization (SRNV). Optical coherence tomography (OCT) images typically demonstrate outer retinal abnormalities and the presence of intraretinal hyporeflective spaces that are usually not related with retinal thickening or fluorescein leakage. The typical fluorescein angiographic finding is a deep intraretinal hyperfluorescent staining in the temporal parafoveal area. With time this fluorescein hyperfluorescence involves the whole parafoveal area but does not extend to the center of the fovea. Long-term prognosis for central vision is poor, because of the development of SRNV or macular atrophy. Its pathogenesis remains unclear but multimodality imaging with fluorescein angiography, spectral domain OCT, adaptive optics, confocal blue reflectance, short wave fundus autofluorescence, OCT angiography, and clinicopathological correlations implicate Müller cells. Currently there is no known treatment for this condition.
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2024, Seminars in Ophthalmology
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Manuscript no. 2005-357.

Supported in part by the National Institutes of Health, Bethesda, Maryland (contract nos.: RO1-EY11289-16, R01-EY13178, P30-EY13078); National Science Foundation, Arlington, Virginia (contract no.: ECS-0119452); Air Force Office of Scientific Research, Arlington, Virginia (contract no.: F49620-98-1-0139); Medical Free Electron Laser Program, Arlington, Virginia (contract nos.: F49620-01-1-0186, FWF P14218-PSY, FWF Y159-PAT, CRAF-1999-70549); Massachusetts Lions Eye Research Fund Inc., New Bedford, Massachusetts; Research to Prevent Blindness, New York, New York; and Carl Zeiss Meditec, Dublin, California.

Drs Fujimoto and Schuman receive royalties from intellectual property licensed to Carl Zeiss Meditec.

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Original ArticleIdiopathic Juxtafoveal Retinal Telangiectasis: New Findings by Ultrahigh-Resolution Optical Coherence Tomography

Objective

Design

Participants

Method

Main Outcome Measures

Results

Conclusions

Section snippets

Materials and Methods

Results

Discussion

Ophthalmology

Am J Ophthalmol

Ophthalmology

Ophthalmology

Ophthalmology

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Idiopathic juxtafoveolar retinal telangiectasis

Arch Ophthalmol

Bilateral juxtafoveolar telangiectasis in monozygotic twins

Am J Ophthalmol

Photodynamic therapy of a subretinal neovascular membrane in type 2A idiopathic juxtafoveolar retinal telangiectasis

Am J Ophthalmol

Classification of the spectrum of Coats’ disease as subtypes of idiopathic retinal telangiectasis with exudation

Acta Ophthalmol Scand

Idiopathic juxtafoveolar retinal telangiectasis and pigment epithelial hyperplasiaan optical coherence tomographic study

Arch Ophthalmol

Original Article
Idiopathic Juxtafoveal Retinal Telangiectasis: New Findings by Ultrahigh-Resolution Optical Coherence Tomography