Use of OCT Angiography to Diagnose and Manage Atypical Presentations of Macular Telangiectasia Type 2

Abstract

Macular telangiectasia Type 2 (MacTel) is a bilateral acquired retinal disease characterized by both vascular changes and atrophy of the retina. The purpose of this case series is to highlight the use of optical coherence tomography angiography (OCTA) as a non-invasive imaging modality to distinguish atypical MacTel from other macular conditions with similar presentations. We performed a retrospective review of patients referred to our academic retinal practice with unconfirmed or misdiagnosed MacTel between July 2017 and July 2021. Patients’ OCTA imaging findings were reviewed to guide the appropriate diagnosis and management of atypical MacTel. Fifteen eyes from eight patients were included in this study. Six patients were referred with previous diagnoses of either full-thickness macular hole, lamellar hole, vitreomacular traction (VMT), postoperative cystoid macular edema (CME), or diabetic macular edema (DME). Two patients were referred to us to confirm the diagnosis of MacTel. OCTA revealed telangiectatic vessels in the temporal parafovea of all 15 eyes. OCTA also highlighted previously undiagnosed subretinal neovascularization (SRNV) in seven eyes. OCTA imaging is a valuable imaging modality to distinguish MacTel from other macular conditions, whose treatment courses vary substantially. Due to its ease of use, it holds immense potential in the future as treatments for non-proliferative MacTel emerge.

Nationwide Screening Practices for Tamoxifen Retinal Toxicity in South Korea: A Population-Based Cohort Study

“…Regarding pathogenesis, tamoxifen retinopathy shares common features with Macular Telangiectasia type 2 (MacTel) [9,10], including telangiectasia of macular blood vessels and crystalline deposits in the macula, as highlighted by various studies [11,12]. These similarities suggest potential overlapping mechanisms in the development of tamoxifen retinopathy and MacTel type 2, warranting further investigation into their shared pathophysiology.…”

“…These similarities suggest potential overlapping mechanisms in the development of tamoxifen retinopathy and MacTel type 2, warranting further investigation into their shared pathophysiology. For example, both conditions may involve vascular endothelial growth factor (VEGF) pathways and Muller cell defects in their pathogenesis, as suggested by recent research [9,10,13]. In addition, emerging evidence suggests a potential link between inflammation and tamoxifen-induced retinal changes [14], although further validation is needed to confirm this association.…”