Idiopathic macular telangiectasia type 1: Clinical, multimodal imaging features and response to treatment

Purpose: To describe the clinical characteristics, multimodal imaging findings, and treatment response in patients with idiopathic macular telangiectasia type 1 (MactTel Type 1). Methods: This retrospective cohort study involved patients affected by idiopathic MactTel Type 1. Multimodal imaging with fundus photography, optical coherence tomography (OCT), and optical coherence tomography angiography (OCT-A) and fluorescein angiography were reviewed. Treatment response to intravitreal anti-vascular endothelial growth factor (anti-VEGF) and dexamethasone (DEX) implants was evaluated. Results: A retrospective, observational study including 10 eyes from 10 patients diagnosed with MactTel Type 1. All eyes exhibited telangiectasias and microaneurysms in the foveal region. OCT showed intraretinal cysts and macular thickening. OCT-A demonstrated a mild reduction in superficial capillary density and numerous telangiectasias in the deep capillary plexus, correlating with macular edema. Fluorescein angiography revealed prominent parafoveal capillaries with late-phase leakage. Moreover, the presence of equatorial leakage was identified in 20% of cases and vascular hyperfluorescence in 60% of cases. Complete response to treatment was observed in 40% patients. Conclusion: MactTel type 1 predominantly affects the temporal retinal quadrants. While anti-VEGF therapy is minimally effective, intravitreal DEX appears to be a promising alternative.