Macular Telangiectasia Type 2: A Comprehensive Review

Kiran Chandra Kedarisetti,1 Raja Narayanan,1,2 Michael W Stewart,3 Nikitha Reddy Gurram,1 Arshad M Khanani4,5 1Anant Bajaj Retina Institute, LV Prasad Eye Institute, Hyderabad, Telangana, India; 2Indian Health Outcomes, Public Health and Economics Research Centre (IHOPE), Hyderabad, Telangana, India; 3Department of Ophthalmology, Mayo Clinic, Jacksonville, FL, USA; 4Department of Clinical research, Sierra Eye Associates, Reno, NV, USA; 5Department of Ophthalmology, The University of Nevada, Reno, NV, USACorrespondence: Raja Narayanan, Anant Bajaj Retina Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India, Tel +91-9177111975, Email narayanan@lvpei.orgAbstract: Macular telangiectasia Type 2 (MacTel) is a gradually progressive disease that affects the quality of life by impairing both distant and near vision. It had previously been considered a vascular condition, but recent evidence suggests a neurodegenerative etiology, with primary involvement of Muller cells. Retinal pigment epithelium (RPE) hyperplasia and subretinal neovascularization (SNV) are responsible for most of the vision loss in advanced cases. Neurotrophic factors in the non-proliferative phase and intravitreal anti-Vascular Endothelial growth factor (VEGF) in the proliferative phase have shown to retard the progression of the disease. This review will discuss the pathophysiology, clinical features, important diagnostic imaging studies and available treatment options for MacTel.Keywords: macular telangiectasia type 2, MacTel, CNTF, ciliary neurotrophic factor