Our patient shows classic bilateral findings for macular telangiecasia (MacTel) type 2 with coarse pigment clumps.
The OCTs show central/temporal retinal atrophy with disorganized retinal layers. Absent in our patient, the OCT will also often display hyporeflective cavitations of tissue loss.
The majority of patients will also have subclinical chorioretinal anastomosis in association with right-angle venules.
Learning Points:
MacTel type 2 is a neurodegenerative disorder, most likely originating from Muller cell dysfunction. The term, telangiectasia, is misleading in that the funduscopic findings are mostly non-vascular.
It is thought that photoreceptor loss allows RPE cells to migrate along intraretinal capillaries causing the clinical pigment clumping. There are also numerous refractile inner retinal ‘crystals,’ thought to be Muller cell footplates.
Although there is much to be learned about its pathophysiology and there is no treatment for the underlying disease, secondary macular neovascularization can be successfully treated per AMD protocols.
