Macular Telangiectasia Type 2
LMacular Telangiectasia Type 2 (MacTel Type 2) is a rare, progressive retinal disorder characterized by neurodegeneration of Müller cells, leading to central vision loss and macular atrophy. We present a case of a patient with a 4-year history of gradual, bilateral vision decline unresponsive to optical correction. Examination revealed classic findings of bilateral juxtafoveal telangiectasia, temporal macular graying, and right-angle venules. Multimodal imaging played a pivotal role in diagnosis and staging. Spectral-domain optical coherence tomography (SD-OCT) revealed ellipsoid zone disruption, temporal widening of the foveal pit, and inner retinal cavitations, consistent with early non-proliferative MacTel Type 2. Fluorescein angiography (FA) demonstrated parafoveal leakage and deep retinal vascular proliferation, while OCT angiography (OCTA) identified microvascular dropout and structural abnormalities in the outer retina.
The differential diagnosis included tamoxifen maculopathy and age-related macular degeneration, but with the absence of outer retinal thickening, crystalline deposits, and distinct vascular changes supported a diagnosis of MacTel Type 2. Emerging therapies, including encapsulated cell therapy (ECT) delivering Ciliary Neurotrophic Factor (CNTF), offer promising neuroprotective strategies aimed at preserving photoreceptor integrity and slowing disease progression. This case underscores the critical role of advanced imaging techniques in diagnosis and monitoring, while highlighting the potential of novel therapeutics to transform the management paradigm for MacTel Type 2.
Presentation Date: 01/30/2025
Issue Date: 04/18/2025
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