Macular Telangiectasia Type 2: Long-Term Disease Progression and Management of Complications.

Abstract

Objectives: To evaluate the long-term progression of macular telangiectasia type 2 (MacTel) using a standardized classification system and to assess the incidence, progression, and management strategies of complications such as macular neovascularization (MNV) and macular hole (MH).

Materials and methods: This retrospective study analyzed the medical records of patients diagnosed with MacTel at a tertiary referral center in Türkiye from January 2004 to February 2025. Patients with a minimum follow-up of 3 years and no confounding macular pathologies were included. Data collection included best corrected visual acuity (BCVA), multimodal imaging (optical coherence tomography [OCT], fundus autofluorescence, fluorescein angiography), and demographic variables. Disease severity was classified using the MacTel Classification System developed by Chew et al. Longitudinal changes in BCVA and OCT parameters were statistically analyzed.

Results: A total of 184 eyes from 94 patients (mean age: 63.89±9.98 years; mean follow-up: 79.27±50.69 months) were included. A significant decline in BCVA was observed (p<0.001). MNV was present in 29 eyes (15.8%), with 18 receiving intravitreal anti-vascular endothelial growth factor (anti-VEGF) therapy (mean injections: 5.89±3.72). While posttreatment BCVA showed improvement (p<0.001), long-term visual outcomes were not significantly different from baseline (p=0.213). MH formation occurred in 8 eyes (4.3%), with 6 undergoing successful surgical closure. Structural retinal changes, including ellipsoid zone disruption and pigmentation, significantly progressed over time (p<0.001).

Conclusion: MacTel demonstrates a progressive decline in visual and structural integrity over extended follow-up. While anti-VEGF therapy offers short-term benefits for MNV, its long-term efficacy remains limited. MH development, though rare, poses a significant challenge, with variable surgical outcomes.