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. Electronic supplementary material The online version of this article (10.1007/s40123-019-0170-1) contains supplementary material, which is available to authorized users.

Serine and 1-carbon metabolism are required for HIF-mediated protection against retinopathy of prematurity

“…36). Yet, the average age of onset of MacTel is 50 to 60 years (37), suggesting that if 1CM is relevant to disease pathogenesis, it is associated with senescence or perhaps compensated by systemic sources of serine/glycine. Our hepatic HIF-1-KO model was not completely devoid of retinal serine, but retinal glycine levels were near 0.…”

Neuroprotective Factors of the Retina and Their Role in Promoting Survival of Retinal Ganglion Cells: A Review

“…Although many studies on phase 2 were conducted on this implant, no further phase 3 trials have been registered at https:// clinicaltrials.gov/ [109][110][111][112][113]. Currently, an extension study is conducted among patients with Macular Telangiectasia Type 2, an idiopathic disease considered as primarily neurodegenerative [114]. This extension study was justified by the positive outcome of previous investigations (NCT03071965) [113].…”

Topographic Relationship between Telangiectasia and Cone Mosaic Disruption in Macular Telangiectasia Type 2

“…Studies using AOSLO can visualize the earliest subclinical disruption to photoreceptors and therefore bring us closer to understanding the progression of these degenerative changes. Recognizing the neural basis in the reactive vascular changes in this disease has profound implications for the treatment strategies [ 18 , 19 ]. In this work, we hypothesized that areas of telangiectatic vessels in the DCP in early stages of MacTel are closely related to zones of cone mosaic disruption, which cannot be visualized on standard SD-OCT imaging.…”