EXUDATIVE MACULAR NEOVASCULARIZATION BEFORE VISIBLE PIGMENTATION IN MACULAR TELANGIECTASIA TYPE-2 (MacTel)

To examine multimodal imaging findings in eyes before and after the onset of exudative macular neovascularization (ExMNV) bypassing the stage of pigmentation in Macular Telangiectasia Type-2.

From a total of 798 patients, Macular Telangiectasia Type-2 eyes with ExMNV that exhibited no signs of focal hyperpigmentation on color fundus photograph, fundus autofluorescence, and spectral-domain optical coherence tomography were retrospectively evaluated.

There were 16 eyes of 16 patients (2%) with a mean age of 50.60 ± 9.82 years. Among them, 11 eyes had ExMNV at the time of examination, whereas the remaining five eyes developed ExMNV during the follow-up period, all lacking pigment when it occurred. The average time for ExMNV development in the five eyes was 27.6 months (range: 11-46 months). Preceding signs of ExMNV included right-angled vessels and telangiectatic leaks causing the following optical coherence tomography changes-temporal parafoveal thickening in three eyes, minimal intraretinal fluid pockets in three eyes, convex hyporeflective subretinal space in two eyes, and subretinal fluid in two eyes. With further declension/descent of inner into outer retina, progression of ellipsoid zone loss was seen and outer retinal hyperreflectivity formed in areas with preexisting ellipsoid zone loss with visual acuity decline. Multiple retinal vessels formed an anastomosis with each other and extended into the center of the subretinal neovascular lesion.

Patients with early Macular Telangiectasia Type-2 can proceed directly to ExMNV without pigmentation. Recognizing the signs before ExMNV develop will allow for closer follow-up and earlier treatment of these patients to prevent irreversible visual loss.