June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
Proliferation of intraretinal vessels and disease progression in Macular Telangiectasia Type 2 (MacTel)
Author Affiliations & Notes
  • Lukas Goerdt
    Department of Ophthalmology, Rheinische Friedrich-Wilhelms-Universitat Bonn, Bonn, Nordrhein-Westfalen, Germany
  • Kristina Hess
    Department of Ophthalmology, Rheinische Friedrich-Wilhelms-Universitat Bonn, Bonn, Nordrhein-Westfalen, Germany
    National Eye Institute, Bethesda, Maryland, United States
  • Philipp Herrmann
    Department of Ophthalmology, Rheinische Friedrich-Wilhelms-Universitat Bonn, Bonn, Nordrhein-Westfalen, Germany
  • Frank G Holz
    Department of Ophthalmology, Rheinische Friedrich-Wilhelms-Universitat Bonn, Bonn, Nordrhein-Westfalen, Germany
  • Simone Tzaridis
    Department of Ophthalmology, Rheinische Friedrich-Wilhelms-Universitat Bonn, Bonn, Nordrhein-Westfalen, Germany
    The Lowy Medicial Research Institute, La Jolla, California, United States
  • Footnotes
    Commercial Relationships   Lukas Goerdt None; Kristina Hess None; Philipp Herrmann None; Frank Holz Acucela, Alcon, Apellis, Avalanche, Bayer, Genentech, Grayburg, Heidelberg Engineering, Lin Bioscience, Novartis, Oxurion, Roche, Code C (Consultant/Contractor), Acucela, Allergan, Bayer, Carl Zeiss Meditec, Centervue, Heidelberg Engineering, NightStarX, Novartis, Optos, Roche, Code F (Financial Support); Simone Tzaridis None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 2668. doi:
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      Lukas Goerdt, Kristina Hess, Philipp Herrmann, Frank G Holz, Simone Tzaridis; Proliferation of intraretinal vessels and disease progression in Macular Telangiectasia Type 2 (MacTel). Invest. Ophthalmol. Vis. Sci. 2022;63(7):2668.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To evaluate the natural progression of vascular and neurodegenerative alterations using multimodal retinal imaging in patients with MacTel.

Methods : In this retrospective, longitudinal analysis, patients underwent annual ophthalmic examinations including color fundus photography (CFP), fluorescein angiography (FLA), optical coherence tomography (OCT) and OCT-angiography (OCTA). Only treatment-naïve eyes lacking any signs of neovascular membranes/ activity were included, and reviewed for ≥20 months. Eyes were analyzed for the progression of vascular and neurodegenerative changes, including vascular proliferation, perivascular leakage, pigment accumulation, and progression of ellipsoid zone (EZ) loss.

Results : 97 eyes from 57 patients (mean age 63.1 years (range: 39-83); 35 females) were included and followed over a mean period of 22.3 months (range: 20.5-38.2). On OCTA, a proliferation of retinal vessels was observed in 36 eyes (37%). Proliferating vessels were primarily found at the level of the deep retinal plexus and within the temporal parafovea. Seven eyes showed a proliferation of vessels at the level of the outer retina/choriocapillaris layer, possibly involving both choriocapillaris and displaced deep plexus vessels. The latter finding was associated with the development of retinal-choroidal anastomoses in all cases.
In all eyes, the proliferation of intraretinal vessels (PIV) was associated with a focal increase in reflectivity on OCT and increased leakage on FLA. A de novo development of subretinal/sub-RPE neovascular membranes was observed in a single eye only. The progression of EZ-loss did not differ between eyes with or without PIV (0.0017 mm2/month versus 0.0015 mm2/month; p=0.09). Eyes with PIV showed a significantly higher frequency of perivascular pigment accumulation compared to eyes without PIV (10/36 (28%) vs 4/61 (7%); chi-square test X2 (1, N=97) = 8.3, p = 0.004).

Conclusions : The proliferation of intraretinal vessels appears to represent a common finding in MacTel, and can be observed in eyes previously classified as “non-proliferative”. Our results thus indicate the need to reevaluate our current understanding and classification of MacTel into “proliferative” and “non-proliferative” disease stages.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.

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