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Mark P Breazzano, Lawrence A. Yannuzzi, Richard F Spaide; Characterizing Retinal-Choroidal Anastomosis in Macular Telangiectasia Type 2. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4999. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To characterize structural and angiographic findings in macular telangiectasis type 2 (MacTel2) and examine associations with visual acuity (VA).
MacTel2 patients with examination, fundus photography including autofluorescence, spectral-domain optical coherence tomography (SD-OCT), and projection-resolved OCT angiography were retrospectively evaluated.
There were 43 eyes of 22 patients with mean age 63.9 (±10.3) years. Six had diabetes. Twenty-one (48.8%) eyes had retinal-choroidal anastomoses (RCAs) without any evidence of subretinal or choroidal neovascularization. When found, an average 55 (±33.7) individual RCAs were clustered primarily in temporal perifoveal in each of these eyes. Right-angle veins were seen in all 21 eyes with RCAs. Hyperpigmentation was present in 18 eyes, all with RCAs (p<0.001). Conical hyperreflective material spanning Bruch’s past external limiting membrane ≥200um basal diameter was found in 21 eyes, and labeled outer retinal hyperreflective lesion. RCAs colocalized with outer retinal hyperreflective lesion (p<0.001), ≥2/3 outer nuclear layer absence with posterior vascular descent (grade 2 retinal subsidence, p<0.001), and ellipsoid zone defect ≥500um (p=0.002) as shown in Figure. Diabetes (p=0.015), RCA quantity (p=0.04), and ellipsoid zone defect extent (p=0.01) each correlated independently with decreased VA.
The outer retinal hyperreflective lesion is a novel SD-OCT finding consistent with altered architecture in MacTel2, offering an intriguing insight regarding pathophysiologic changes. Diabetes, ellipsoid zone defect extent, and RCAs predict poorer vision with MacTel2. Additional cases may be needed to tease out individual contributions from these highly inter-correlated findings for decreased vision in MacTel2.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
FIGURE. Optical coherence tomography angiography B-scan with projection-resolved flow overlay (red) of a representative eye with macular telangiectasia type 2 affecting temporal perifovea. Absence of outer nuclear layer (*) with posterior vascular descent (grade 2 retinal subsidence) colocalizes with a retinal-choroidal anastomosis between deep vascular plexus and choriocapillaris (single arrow), and an ellipsoid zone defect (double arrow). Conical hyperreflective material spanning Bruch’s membrane with its apex (arrowhead) extending beyond external limiting membrane (outer retinal hyperreflective lesion) also colocalizes with these features.
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