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Meredith Remmer Klifto, Jesse T McCann, Lawrence A. Yannuzzi; Multimodal Imaging of Large Microaneurysms in Macular Telangiectasia Type 1 With Correlation to OCT-Angiography. Invest. Ophthalmol. Vis. Sci. 2016;57(12):1638.
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© 2017 Association for Research in Vision and Ophthalmology.
Idiopathic Juxtafoveal Macular Telangiectasia type 1 (MacTel1) is a rare sporadic unilateral disorder of peri-foveal aneurysmal capillary telangiectasia, often considered a variant of Coats’ Disease. MacTel1 is predominantly found in males and is usually asymptomatic until the fifth decade, when ectatic incompetent vessels lead to the accumulation of lipids and fluid. The leaking, dilated capillaries often lead to exudative retinal detachments. The etiology of MacTel1 is unknown but is thought to be related to chronic venous congestion in the setting of right angle venules. Large microaneurysms in MacTel1 can be analyzed with multimodal imaging to understand structural changes, flow dynamics, and disease progression. We performed a retrospective observational study on 6 patients with MacTel1 and correlate key features on traditional imaging modalities with OCT Angiography (OCT-A).
A retrospective observational clinical review on twelve eyes of six patients with MacTel1 was conducted. Demographics, acuity, fundus photography, swept-source OCT, OCT-A, and Intravenous Fluorescein angiography (IVFA) were analyzed.
The median age was 58.7 years and the range was 43-67 years. All six patients were male. Uncorrected distance visual acuity ranged from 20/25- to 20/200. Fundus photos demonstrated telangiectatic vessels and IVFA demonstrated late leakage from the abnormal vessels. Five of the six patients were found to have edema on OCT. Three of six patients had lipid deposition. OCT-A detected a consistent set of retinal vascular changes, predominantly large microaneurysms with abnormal flow. The aneurysm occurred within deep retinal layers in five of the six patients, and all had associated neovascularization.
Large microaneurysms in MacTel1 found predominantly within the deep retinal plexus can be found on OCT-A. Sequential analysis of flow patterns by OCT-A may provide insight into the pathophysiology of disease progression and gauge treatment protocols. Variability in the flow and leakage patterns in this phenoype are consistent with the variability of clinical presentation.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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