Multimodality imaging in macular telangiectasia 2: A clue to its pathogenesis

• Main Author: Lihteh Wu
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2015-01-01
• Series: Indian Journal of Ophthalmology
• Subjects: Community; digital imaging; Karnataka Internet-assisted Diagnosis of Retinopathy of Prematurity; retinopathy of prematurity; telemedicine; universal screening; Age-related macular degeneration; en-face optical coherence tomography; polypoidal choroidal vasculopathy; Choroid; enhanced depth imaging technique; swept source optical coherence tomography; Choroidal imaging; choroidal thickness; retinal dystrophies; Choroidal neovascularization; idiopathic juxtafoveal telangiectasis; juxtafoveal retinal telangiectasia; lutein; macular edema; macular pigment; macular telangiectasia; Müller cells; parafoveal telangiectasis; perifoveal telangiectasis; retinal angiomatous proliferation; retinal telangiectasis; subretinal neovascularization; zeaxanthin
• Online Access: http://www.ijo.in/article.asp?issn=0301-4738;year=2015;volume=63;issue=5;spage=394;epage=398;aulast=Wu

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.