Macular Telangiectasia Type 2
Macular telangiectasia type 2 (MacTel 2, also called juxtafoveal telangiectasis), which is the most common form of macular telangiectasis, is a rare progressive bilateral idiopathic neurodegenerative disease of the macular, perifoveal, retina. Characteristic findings begin to appear in affected individuals in the fifth to seventh decades of life; they include a reduced foveolar reflex, greying appearance and loss of retinal transparency, superficial retinal crystalline deposits, mildly ectatic capillaries, slightly dilated blunted venules, progression to pigment hyperplasia, and foveal atrophy. Subretinal neovascularization occurs frequently during the natural disease course. Many of the clinical features of MacTel 2 are characterized by dysfunction of both neural and vascular retinal elements, suggesting that a macular Müller cell defect plays an essential role in the pathogenesis of this disease.
The telangiectatic vessels are readily apparent on fluorescein angiography and usually leak. OCT imaging typically demonstrates a thinned central macular retina, including the fovea, with inner lamellar oblong foveal cavitations in which the long axis is parallel to the retinal surface (Fig 7-10; also see Fig 7-9). OCTA visualizes the deep capillary plexus changes of MacTel 2 (Fig 7-11). To date, there is no evidence for an effective treatment of MacTel 2. Intravitreal anti-VEGF therapy is used for the management of subretinal neovascularization.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.