Purpose. To describe the complete sequence of the progressive vasculopathy in macular
telangiectasia type 2.
Methods. This is a report of a case demonstrating the complete vasogenic sequence in macular
telangiectasia type 2 over the course of 15 years, and representative images from a collective of 150 patients with macular
telangiectasia type 2 employing fundus photography,
fluorescein angiography, and optical coherence tomography.
Results. Macular
telangiectasia may progress along a predictable vasogenic sequence which consists of nonproliferative stages, characterized by temporal loss of macular luteopigment and inner
retinal volume loss in the absence of vascular changes, followed by a progressive proliferative vasculopathy, first involving the deep capillary plexus with eventual extension of the vascular changes circumferentially in the inner
retinal capillary plexus. Late proliferative stages may become indistinguishable from advanced neovascular
age-related macular degeneration.
Conclusions. While it is rare to observe the complete vasogenic sequence of macular
telangiectasia type 2, a classification into nonproliferative and proliferative stages can be established, and may prove helpful as the mechanisms driving the pathogenic process through those stages are identified.