[Morphological characteristics in macular telangiectasia type 2]. / Morphologische Charakteristika bei makulären Teleangiektasien Typ 2.

Macular telangiectasia type 2 is characterized by atrophic alterations of the central retina which is accompanied by a defined vascular phenotype. The disease manifests within an oval central retinal area the size of approximately two disc diameters, with a topographic predisposition temporal to the foveal center. Funduscopy reveals reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, thickened venules and retinal pigment plaques. Secondary neovascularization and macular holes may occur during the disease course. Fluorescein angiography usually shows a diffuse leakage and often ectatic capillaries. On optical coherence tomography (OCT) examination, hyporeflective cavities and focal atrophy of the photoreceptor layer represent a frequent finding. A characteristic sign is an increased (para) central signal on fundus autofluorescence imaging due to a reduced density of macular pigment.

[Functional characteristics of macular telangiectasia type 2]. / Funktionelle Charakteristika bei makulären Teleangiektasien Typ 2.

The first symptoms of macular telangiectasia type 2 usually occur between 50 and 70 years of age. Functional alterations topographically correspond to the morphological changes. Characteristic paracentral scotomata due to focal photoreceptor atrophy can be detected using microperimetry. The predominant paracentral functional loss may cause reading difficulties despite visual acuity in the range between 20/20 and 20/50. Visual acuity around 20/200 may occur once the paracentral photoreceptor atrophy extends centrally, or due to the development of a macular hole or a secondary neovascular membrane. Progression of functional loss can often only be detected by mapping scotoma size or occurrence using microperimetry, while visual acuity may remain unchanged.

[Therapeutic approaches for macular telangiectasia type 2: status quo and perspectives]. / Therapeutische Ansätze bei makulären Teleangiektasien Typ 2: Status quo und Perspektiven.

Macular telangiectasia type 2 is characterized by neurodegenerative as well as vascular and retinal alterations. Previous therapeutic approaches mainly targeted the vascular changes; however, this did not prove to be beneficial except for secondary neovascularization which may be successfully treated with intravitreal vascular endothelial growth factor inhibitors. As the natural history of the disease is primarily characterized by the neurodegenerative processes, new therapeutic strategies, such as neuroprotective agents are already being explored in clinical trials.