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Review
. 2015 May;63(5):394-8.
doi: 10.4103/0301-4738.159864.

Multimodality Imaging in Macular Telangiectasia 2: A Clue to Its Pathogenesis

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Free PMC article
Review

Multimodality Imaging in Macular Telangiectasia 2: A Clue to Its Pathogenesis

Lihteh Wu. Indian J Ophthalmol. .
Free PMC article

Abstract

Macular telangiectasia type 2 also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A is an acquired bilateral neurodegenerative macular disease that manifests itself during the fifth or sixth decades of life. It is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right-angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV). Our understanding of the disease has paralleled advances in multimodality imaging of the fundus. Optical coherence tomography (OCT) images typically demonstrate the presence of intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic (FA) finding is a deep intraretinal hyperfluorescent staining in the temporal parafoveal area. With time, the staining may involve 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 FA, spectral domain OCT, adaptive optics, confocal blue reflectance and short wave fundus autofluorescence implicate Müller cells and macular pigment. Currently, there is no known treatment for this condition.

Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Late frame from a fluorescein angiogram of an eye with macular telangiectasia type 2 showing the typical late leakage that does not involve the foveal center
Figure 2
Figure 2
Spectral domain optical coherence tomography showing disruption of the ellipsoid line
Figure 3
Figure 3
Increased confocal blue reflectance
Figure 4
Figure 4
Increased short wave fundus autofluorescence

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