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Rahul Komati, Sidney Schechet, Pamela Hulvey, Dimitra Skondra; Use of Ocular Coherence Tomography Angiography in Diagnosis of Atypical Macular Telangectasia Type 2. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4555.
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Macular telangiectasia Type 2 (mac-tel) is associated with characteristic retinal vascular ectasia and neurosensory degeneration which can be subtle or asymmetric in early stages. Often, these findings are missed or misdiagnosed on examination and ocular coherence tomography (OCT) alone. The purpose of this study is to evaluate OCT angiography (OCTA) as a non-invasive modality to distinguish early and atypical mac-tel from masquerader conditions and guide appropriate management.
This is a retrospective, observational case series of patients referred to our academic retina clinic for macular diagnoses other than mac-tel. We evaluated patients using OCTA in addition to standard funduscopic exam and OCT and made the proper diagnosis of mac-tel, guiding appropriate management.
Seven eyes from four patients were included in this study. The referring diagnoses were full-thickness macular hole, lamellar hole, vitreomacular traction, postoperative macular edema, and macular degeneration. OCTA revealed abnormally dilated and telangiectatic capillaries in the temporal perifoveal area in all 4 patients consistent with mac-tel. OCTA also uncovered occult choroidal neovascularization in 3 of 7 eyes. OCT showed areas of ellipsoid zone disruption and cystoid spaces within the inner and outer retina in all 7 eyes. Cystic changes were best visualized with en face OCTA images at the level of deep capillary plexus and outer retina. Fluorescein angiography (FA) was performed in 3 of 4 patients, reaffirming mac-tel diagnosis.
OCTA can be a useful, fast, and non-invasive modality to distinguish mac-tel from other macular disorders that can masquerade as mac-tel. This is critical, especially for surgical conditions like macular holes where the treatment plan and patient expectations could vary significantly, or macular edema that could require lengthy treatment with multiple drops and steroid injections. Early choroidal neovascularization, which can be challenging to identify on FA due to temporal macula leakage from telangiectatic vessels, can be revealed distinctly with OCTA allowing for timely intervention with anti-VEGF therapy. Early and appropriate diagnosis of mac-tel with OCTA could also identify candidates for ongoing and future clinical trials and new emerging treatments prior to advanced stages and irreversible vision loss.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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