Chantal Dysli, Nicola Sagurski, Lorenzo Ferro Desideri, Martin S Zinkernagel
Macular telangiectasia type 2 (MacTel type 2) is a progressive neurodegenerative macular disease whose diagnosis, prognostic assessment, and longitudinal monitoring rely critically on the recognition of its characteristic clinical and imaging features. This systematic review provides a comprehensive overview of the most important diagnostic findings in MacTel type 2, encompassing both biomicroscopic clinical signs and the full spectrum of multimodal imaging modalities currently employed in clinical practice-including color fundus photography, fluorescein angiography, fundus autofluorescence, multicolor imaging, spectral-domain optical coherence tomography (OCT), and OCT angiography. For each modality, key diagnostic signs, their contribution to disease staging, and their value in monitoring disease progression are discussed. Additionally, a specific focus on emerging imaging techniques is provided with potential relevance to future diagnostic and research applications. In MacTel type 2, a multimodal imaging approach represents the most effective strategy for accurate diagnosis, reliable staging of disease severity, and meaningful prognostic assessment.