Management of Idiopathic Macular Telangiectasia Type 2

Abstract

Macular telangiectasia type 2 (MacTel) is a relatively rare disease without established treatments. Although MacTel was previously considered a primarily vascular condition, the thinking on its pathogenesis has shifted to it now being considered principally a neurodegenerative disease. This has resulted in a subsequent change in the approach to treatment toward neuro-protection for the non-proliferative phase of this disease. Carotenoid supplementation has had mixed results. Ciliary neurotrophic factor (CNTF) has demonstrated some promising early results, but further study is necessary to determine its actual effect. Some structural improvements have been seen in the non-proliferative phase with oral acetazolamide but without accompanying functional improvement. Anti-vascular endothelial drugs have been studied and not found to have benefit in the non-proliferative phase of disease but have demonstrated significant structural and functional value in the treatment of secondary neovascularization. There is no level I evidence for the various proposed MacTel treatments, and efforts need to be directed toward conducting multicenter randomized trials to better understand possible treatments for this condition.

Keywords: Anti-vascular endothelial growth; Macular telangiectasia; Management; Retina.

Fig. 1

Non-proliferative and proliferative phases of MacTel. 1: A 34-year-old male with bilateral progressive visual loss. Fundus photo of the right eye showed temporal parafoveal foci of retinal pigment epithelium (RPE) hyperplasia with foveal pigmentary change (a). Corresponding OCT demonstrated outer retinal layer atrophy and outward configuration of the inner retinal layers while the RPE layer appeared to be intact (b). 2: A 41-year-old male, a known case of MacTel with acute onset of vision loss in the left eye. Fundus photo and OCT imaging of the left eye showed not only RPE hyperplasia and pigmentary changes but also retinal and sub-retinal hemorrhages due to RPE disruption and choroidal neovascularization (c, d). Images courtesy of Dr. Hamid Riazi

Fig. 2

MacTel proliferative phase treated with Anti-VEGF. The same patient mentioned in Fig. 1c, d with active choroidal neovascularization and visual acuity of 20/200 underwent 3 monthly doses of intravitreal bevacizumab (a). After 3 months, most of the fluid was resolved with visual acuity improvement to 60/200 (b). Images courtesy of Dr. Hamid Riazi