Macular telangiectasia is a progressive ocular disease with two forms, type 1 (MacTel1) and type 2 (MacTel2). MacTel1 is a rare congenital disorder, accounting for 20% to 25% of all MacTel cases, that presents with unilateral symptoms and mostly affects the male population. MacTel2 is a more common form with an estimated prevalence of 0.1% in patients over 40 years in the United States. MacTel2 differs from MacTel1 in that it mostly affects females and symptoms present bilaterally with central vision loss; however, patients usually do not experience complete blindness. MacTel2 is a neurodegenerative condition that causes the loss of Müller cells, which are responsible for growth factor secretion, angiogenesis/antiangiogenesis, neurotransmitter metabolism, synaptogenesis, neuroprotection, and photoreceptor survival. Over time, MacTel2 progresses to a proliferative phase, affecting the macular vasculature and causing blood vessels surrounding the fovea to proliferate, dilate, and leak, ultimately leading to macular degeneration. In the proliferative phase intravitreal anti-vascular endothelial growth factor (VEGF) inhibitors have shown to retard the progression of the disease.
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