This 60YO female presented with 20/70 vision OD and 20/50 vision OS, along with classic findings of macular telangiectasia type 2 (MacTel2).
The perifoveal retina is somewhat opaque with significant pigmentary changes, particularly in the right eye.
OCT scanning of the right eye shows significant central outer retinal atrophy with hyper-reflective pigment migration. The left OCT shows milder atrophy, central inner cavitations, but no pigment migration.
En face imaging helps highlight some of the findings described above. The right en face image shows scattered patchy hyperreflective areas corresponding to the pigment migration, while the left en face image highlights the hyporeflective cavitations.
Learning Points:
Originally described by Gass (Arch Ophthalmology 1982;100:769-780), MacTel2 is a neurodegenerative disorder, most likely originating from Müller cell dysfunction. The term telangiectasia is misleading because the funduscopic findings are mostly nonvascular.
The refractile inner retinal ‘crystals’ are thought to be Müller cell footplates. The foveal retina shows a whitish discoloration, most likely due to retinal opacification resulting from Müller cell dysfunction.
Photoreceptor loss allows RPE cells to migrate along intraretinal capillaries, causing the coarse clinical pigment clumping (not seen in our patient).
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 according to age-related macular degeneration protocols.
