This 80YO male was referred for bilateral macular findings. He was without new visual complaints. Vision was 20/70 OD and 20/200 OS.
Optos color RG imaging shows bilateral foveal pigment loss with coarse intraretinal pigment migration. The changes are mostly hypo-autofluorescent.
Triton swept-source OCT shows atrophy and disorganization of all retinal layers in the central and temporal foveas.
Learning Points:
Originally described by Gass (Arch Ophthalmology 1982;100:769-780), macular telangiectasia type 2 (MacTel2) is a neurodegenerative disorder, most likely originating from Muller cell dysfunction.
The term telangiectasia is misleading, as the funduscopic findings are mostly non-vascular. The refractile inner retinal ‘crystals,’ not seen in our patient, are thought to be Muller cell footplates. The foveal retina shows a whitish discoloration, most likely due to retinal opacification caused by dysfunctional Muller cells. Photoreceptor loss allows RPE cells to migrate along intraretinal capillaries, causing the coarse clinical pigment clumping.
The majority of patients will also have subclinical chorioretinal anastomosis associated with right-angle venules (Spaide et al, Retina 2018;38:1920-1929). Secondary macular neovascularization can be successfully treated per age-related macular degeneration protocols.
