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Spaide, Richard F., MD*; Yannuzzi, Lawrence A., MD*; Maloca, Peter M., MD

doi: 10.1097/IAE.0000000000002289
Original Study

Purpose: To investigate the deep retinal vascular changes potentially present in macular telangiectasis Type 2 (MacTel 2) with projection resolved optical coherence tomography angiography including volume rendering.

Methods: Retrospective consecutive evaluation of patients with MacTel 2 in a community-based retinal referral practice with a comprehensive ophthalmologic examination to include optical coherence tomography and projection-resolved optical coherence tomography angiography with volume rendering. Main outcome measures were the characterization of vessel presence and anatomical arrangement in the outer retina.

Results: There were 26 eyes of 13 patients with a mean age of 64.9 (±11.3) years, and 6 were men. The mean visual acuity was logMAR 0.4 (Snellen equivalent 20/50). No eye had signs of choroidal neovascularization or exudation. Focal hyperpigmentation was seen in 13 (50%) and right-angle veins in 17 (65%) eyes. Retinal–choroidal anastomoses were seen in 17 (65%) eyes. These anastomoses typically occurred in multiple clusters of small vessels. The presence of anastomoses was associated with pigment (P < 0.001), although the anastomoses did not necessarily colocalize with the pigment, and right-angle veins (P < 0.001), which were found in every eye with a retinal–choroidal anastomosis.

Conclusion: Retinal–choroidal anastomoses were commonly observed in eyes with MacTel 2 using projection-resolved optical coherence tomography angiography. One animal model for MacTel 2 uses very low-density lipoprotein receptor mutant mice and shows multiple retinal–choroidal anastomoses in the disease pathogenesis as well. These findings suggest MacTel 2 is more than just a neurodegenerative disease with secondary vascular abnormalities, as the choroid may be involved in the disease process.

Volume rendering and 2D representations of optical coherence tomography angiography with projection artifact removal show multiple retinal–choroidal anastomoses in the disease, before the development of subretinal neovascularization. These anastomoses that seemed to be clinically silent and imply MacTel 2 are not solely a retinal disease, but have choroidal involvement.

*Vitreous, Retina, and Macula Consultants of New York; and

Department of Ophthalmology, University Hospital Basel, Basel, Switzerland.

Reprint requests: Richard F. Spaide, MD, Vitreous, Retina, Macula Consultants of New York, 460 Park Avenue, New York, NY 10022; e-mail:

Supported by the Macula Foundation, New York, NY, which had no input as to the content of the work.

R. F. Spaide declares royalties and consulting funds from Topcon Medical Systems, Consulting fees from Boehringer Ingelheim and Quark Pharmaceuticals, and royalty fees from DORC, and imaging patents. P. M. Maloca receives consulting fees from Topcon Medical Systems and imaging patents. The remaining author has no financial/conflicting interests to disclose.

© 2018 by Ophthalmic Communications Society, Inc.