Author: Lukas Goerdt (Germany)
Co-authors: Kristina Hess, Philipp Herrmann, Frank G. Holz, Simone Tzaridis
Purpose
To investigate the progression of vascular alterations using optical coherence tomography-angiography (OCT-A) in macular telangiectasia type 2 (MacTel).
Setting/Venue
Longitudinal, retrospective single center observational study.
Methods
Patients with a confirmed diagnosis of MacTel underwent annual ophthalmic examinations including best-corrected visual acuity (BCVA), color fundus photography (CFP), fluorescein angiography (FLA, if available), optical coherence tomography (OCT) and OCT angiography (OCT-A). Only eyes without subretinal/ sub-RPE neovascular membranes and no signs of neovascular exudation at baseline were included and reviewed for ≥20 months. Grading was performed by two independent readers. Main outcome measures included vessel proliferation, vascular leakage, perivascular pigment accumulation, formation of subretinal/sub-RPE neovascular membranes, and neovascular exudation.
Results
A total of 124 eyes from 64 patients (mean age 62.4 years (range: 39-83 years); 37 females) were included and reviewed over a mean period of 23.3 months (range 20.5-40.5). A proliferation of vessels was observed in 68 eyes (55%), while 24 patients (38%) showed occurrence. Vascular proliferation was primarily observed at the level of the deep retinal plexus (n=48 (71%)). In eyes showing a formation of retinal-choroidal anastomoses (n=20 (29%)), a proliferation of vessels could also be observed within the outer retina-choriocapillaris (ORCC)-layer, possibly involving both choriocapillaris and displaced deep plexus vessels. Vascular proliferation was predominantly found in the temporal parafovea. In all cases vascular proliferation was associated with an increase in reflectivity on OCT and increased fluorescein leakage on FLA. Pigment plaques accumulated along proliferating vessels and vessels forming retinal-choroidal anastomoses. Fourty eyes (35%) showed a de novo development of perivascular pigment. A de novo formation of exudative subretinal/sub-RPE neovascular membranes was observed in 3 eyes (2%).
Conlusions
The results indicate intraretinal vessel proliferation as part of the natural course of MacTel in eyes that have been previously considered “non-proliferative”. Our findings indicate the need to reevaluate our current understanding and classification system of MacTel into “proliferative” and “non-proliferative” disease stages.
Financial Disclosure
LG: Heidelberg Engineering: non-financial support, Carl Zeiss MedicTec AG: non-financial support, Optos: non-financial support KH: Heidelberg Engineering: non-financial support, Carl Zeiss MedicTec AG: non-financial support, Optos: non-financial support PH: Heidelberg Engineering: non-financial support, Carl Zeiss MedicTec AG: non-financial support, Optos: non-financial support FGH: Heidelberg Engineering: Grant, Personal fees, non-financial support, Novartis: Grant, personal fees, Bayer: Grant, personal fees, Genetech: Grant, personal fees, Acucela: Grant, personal fees, Boehringer Ingelheim: Personal fees, Alcon: Grant, personal fees, Allergan: Grant, personal fees, Optos: Grant, personal fees, non-financial support, Carl Zeiss MediTec AG: non-financial support ST: Heidelberg Engineering: non-financial support, Carl Zeiss MedicTec AG: non-financial support, Optos: non-financial support
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