Idiopathic Juxtafoveolar Retinal Telangiectasis: Clinical Pattern in 19 Cases

Abujamra, Suel; Bonanomi, Maria Teresa Bc; Cresta, Fernando Betty; Machado, Cleide Guimarães; Pimentel, Sérgio Luís Gianotti; Caramelli, Christiane Baddini

doi:10.1159/000027534

Cited by 33 publications

(34 citation statements)

References 6 publications

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“…[1][2][3][4][5][6] They commonly have superficial parafoveolar retinal crystalline deposits, 7 gray discoloration of the temporal parafoveolar retina, and right-angle vessels with no exudation. These patients also can develop parafoveal intraretinal pigment migration similar to that seen in patients with retinitis pigmentosa.…”

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confidence: 99%

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Optical Coherence Tomography Findings in Nonproliferative Group 2a Idiopathic Juxtafoveal Retinal Telangiectasis

Cohen

Cohen²,

El-Jabali³

et al. 2007

Retina

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Purpose:To determine the optical coherence tomography (OCT) findings in eyes with group 2a idiopathic juxtafoveal retinal telangiectasis (IJRT).Methods: Forty-one eyes of 22 patients with nonproliferative group 2a IJRT were examined. OCT testing including retinal topographic mapping and analysis, and horizontal and vertical line scans, was obtained on each eye.Results: None of the 41 eyes had a thickened foveal center. The average center foveal thickness was 166 m (31-264 m). Stage 1 eyes (n ϭ 2) were normal fellow eyes in patients with contralateral group 2a IJRT. Stage 2 eyes (n ϭ 11) all had parafoveolar temporal graying and intraretinal temporal fluorescein leakage, but rarely had photoreceptor disruption (18%) on OCT testing. Stage 3 eyes (n ϭ 14) all had clinical and fluorescein findings similar to or more pronounced than stage 2 eyes. All stage 3 eyes also had one or more foveal cysts at various retinal depths on OCT. Most of these eyes (86%) had photoreceptor disruption and outer retinal atrophy on OCT. Stage 4 eyes (n ϭ 14) all had a black foveal or parafoveolar pigment plaque and intraretinal temporal fluorescein leakage. All stage 4 eyes had a hyper-reflective plaque with shadowing on OCT corresponding to the pigment plaque. Most of these eyes had one or more foveal cysts (64%) and all of these eyes had photoreceptor disruption and outer retinal atrophy.Conclusion: OCT helps in the staging of group 2a IJRT and reveals multiple retinal structural abnormalities.

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“…1 These eyes can develop subretinal neovascularization originating from the juxtafoveal telangiectatic vessels. [1][2][3][4][5][6]8 Gass classified group 2a IJRT into five stages based on slit lamp biomicroscopic and fluorescein angiographic findings.…”

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confidence: 99%

Optical Coherence Tomography Findings in Nonproliferative Group 2a Idiopathic Juxtafoveal Retinal Telangiectasis

Cohen

Cohen²,

El-Jabali³

et al. 2007

Retina

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“…The asymmetry between the nasal and temporal sides could be explained by the preferential location of MacTel 2 anomalies in the temporal side of the macula (inner retinal whitening, cystic formations, and telangiectasia). 15 However, we could not rule out that from 4°to 2°the visibility of the cone mosaic could be partially disturbed by the shadow of macular telangiectasia. However, this cone density loss corresponded also to an impairment of the interdigitation zone on OCT while no or minimal changes in ellipsoid zone reflectivity were observed.…”

Section: Discussionmentioning

confidence: 86%

Cone Density Loss on Adaptive Optics in Early Macular Telangiectasia Type 2

Jacob

Krivosic

Pâques

et al. 2016

Retina

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Adaptive optics showed that the macular cone density was lower than normal even outside the telangiectasia in MacTel 2 lacking intraretinal cavitation, although the ellipsoid zone remained intact on optical coherence tomography. These findings do not indicate that the cone density loss is causative of the disease as it might be secondary to Müller cell or rod loss in this area. However, cone density assessment could become a useful parameter to monitor disease progression.

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“…Many other ocular (Bakke and Drolsum, 2006;Gass and Oyakawa, 1982;Querques et al, 2010) and systemic (Abujamra et al, 2000;Fitzsimons et al, 1987;Gass and Blodi, 1993;Gurwin et al, 1985;Lee et al, 2004;Leys et al, 2000;Parodi et al, 2006;Yannuzzi et al, 2006) associated diseases have been reported. However, these often were only single observations and certainly could represent the normal prevalence in the population and thus coincidence.…”

Section: Additional Associated Ocular Findings and Systemic Diseasesmentioning

confidence: 99%

Macular Telangiectasia Type 2

Heeren

Holz

Issa

2013

Microperimetry and Multimodal Retinal Imaging

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Macular telangiectasia type 2 is a bilateral disease of unknown cause with characteristic alterations of the macular capillary network and neurosensory atrophy. Its prevalence may be underestimated and has recently been shown to be as high as 0.1% in persons 40 years and older. Biomicroscopy may show reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy, and neovascular complexes. Fluorescein angiography shows telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. High-resolution optical coherence tomography (OCT) may reveal disruption of the photoreceptor inner segment-outer segment border, hyporeflective cavities at the level of the inner or outer retina, and atrophy of the retina in later stages. Macular telangiectasia type 2 shows a unique depletion of the macular pigment in the central retina and recent therapeutic trials showed that such depleted areas cannot re-accumulate lutein and zeaxanthin after oral supplementation. There have been various therapeutic approaches with limited or no efficacy. Recent clinical trials with compounds that block vascular endothelial growth factor (VEGF) have established the role of VEGF in the pathophysiology of the disease, but have not shown significant efficacy, at least for the nonneovascular disease stages. Recent progress in structure-function correlation may help to develop surrogate outcome measures for future clinical trials.In this review article, we summarize the current knowledge on macular telangiectasia type 2, including the epidemiology, the genetics, the clinical findings, the staging and the differential diagnosis of the disease. Findings using retinal imaging are discussed, including fluorescein angiography, OCT, adaptive optics imaging, confocal scanning laser ophthalmoscopy, and fundus autofluorescence, as are the findings using visual function testing including visual acuity and fundus-controlled microperimetry. We provide an overview of the therapeutic approaches for both non-neovascular and neovascular disease stages and provide a perspective of future directions including animal models and potential therapeutic approaches.

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Idiopathic Juxtafoveolar Retinal Telangiectasis: Clinical Pattern in 19 Cases

Cited by 33 publications

References 6 publications

Optical Coherence Tomography Findings in Nonproliferative Group 2a Idiopathic Juxtafoveal Retinal Telangiectasis

Optical Coherence Tomography Findings in Nonproliferative Group 2a Idiopathic Juxtafoveal Retinal Telangiectasis

Cone Density Loss on Adaptive Optics in Early Macular Telangiectasia Type 2

Macular Telangiectasia Type 2

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