When is macular edema not macular edema? An update on macular telangiectasia type 2

Abstract

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 intra-retinal 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 multi-modality 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.

Keywords: Müller cells; juxtafoveal retinal telangiectasia; macular telangiectasia; parafoveal telangiectasis; perifoveal telangiectasis.

Fig. 1

Grayish discoloration of the retina with loss of retinal transparency.

Fig. 2

Multiple, golden, tiny, crystalline, refractile deposits near the inner retinal surface.

Fig. 3

Intraretinal pigmented black plaque composed of hyperplastic retinal pigment epithelium cells.

Fig. 4

Late fluorescein angiogram frame showing oval shaped parafoveal hyperfluorescence.

Fig. 5

Optical coherence tomography demonstrating no correlation between retinal thickening and fluorescein leakage. Notice the disruption of the outer retina as well.

Fig. 6

Optical coherence tomography demonstrating intraretinal hyporeflective spaces not related to retinal thickening.

Fig. 7

Short wavelength fundus autofluorescence (SW-FAF) imaging of the normal macula is characterized by a central dark area. In Mac Tel 2 there is an increased SWFAF signal in the foveal region.

Fig. 8

Confocal blue reflectance imaging showing increased reflectance.