Abstract

Purpose: To describe the progression from outer retinal neovascularization (ORNV) to exudative subretinal new vessels (SRNV) in idiopathic macular telangiectasia type 2 (MacTel2) Patients and Methods: A total of 135 patients (270 eyes) imaged with optical coherence tomography angiography (OCTA) were included. Main Outcome Measures: Ellipsoid zone (EZ) loss, outer retinal hyperreflectivity (ORHR), ORNV, and SRNV. ORNV was defined as a flow signal passing through the outer plexiform layer, with or without vertical linear ORHR on the OCT B-scan. SRNV were defined as an abnormal capillary network with a peripheral anastomotic arcade seen on en face OCTA, and a convex hyperreflectivity at the retinal pigment epithelium. Results: SRNV were observed in 38/270 eyes (14%). SRNV were at a fibrotic stage in 24/38 eyes, at an exudative stage in 6/38 eyes, and a progression from ORNV to SRNV was documented in 8/38 eyes. All cases showed an EZ loss. In 7 eyes (2.5%), SRNV were also associated with subepithelial neovascularization. No retinochoroidal anastomosis was detected. The visual acuity dropped when SRNV were present. Conclusion: In this case series, SRNV were found in 14% of eyes. In all cases, they were associated with an EZ loss and with ORHR. A progression from ORNV to SRNV was observed.

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