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Macular telangiectasia type 2-also known as idiopathic perifoveal telangiectasia and juxtafoveolar retinal telangiectasis type 2A or Mac Tel 2-is an acquired bilateral neurodegenerative macular disease that usually manifests itself during the fourth to sixth decades of life and is characterized by minimal dilatation of the parafoveal capillaries with graying of the retinal area involved, a lack of lipid exudation, right-angled retinal venules, refractile deposits in the superficial retina, hyperplasia of the retinal pigment epithelium, foveal atrophy, and subretinal neovascularization (SRNV). Optical coherence tomography images typically demonstrate intraretinal hyporeflective spaces that are usually not related to retinal thickening or fluorescein leakage. The typical fluorescein angiographic finding is a deep intraretinal hyperfluorescent leakage in the temporal parafoveal area. With time the leakage may involve the whole parafovea, but does not extend to the center of the fovea. Long-term prognosis for central vision is variable and depends on the development of SRNV or macular atrophy. Pathogenesis remains unclear, but Müller cells and macular pigment appear to play a central role. Currently there is no known treatment for the underlying cause of this condition, but treatment of the SRNV may be beneficial., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: To compare pathologic changes in the photoreceptors of eyes with idiopathic macular telangiectasia types 1 and 2 using adaptive optics scanning laser ophthalmoscopy (AO-SLO)., Design: Prospective cross-sectional study., Methods: Eleven eyes with idiopathic macular telangiectasia type 1, 14 eyes with type 2, and 10 normal eyes underwent a full ophthalmologic examination, spectral-domain optical coherence tomography (SD-OCT), and imaging with an original prototype AO-SLO system. All eyes with idiopathic macular telangiectasia were examined with fluorescein angiography (FA), confocal blue reflectance, and microperimetry., Results: Compared with normal eyes (29 017 ± 5507 cones/mm(2)), those with idiopathic macular telangiectasia type 1 had significantly lower cone density on the temporal side (18 427 ± 4908 cones/mm(2), P = .010). The FA leakage area (5.90 ± 3.23 mm(2)) was larger than the dark regions (2.45 ± 2.40 mm(2)) on AO-SLO (P = .003)-which were observed only in the FA leakage area. Compared with normal eyes, those with idiopathic macular telangiectasia type 2 had significantly lower cone density in all areas (P < .05 for all). Patchy dark regions were also seen in areas without FA abnormalities in 12 eyes. Higher mean retinal sensitivity correlated with greater mean cone density 0.5 mm from the center of the fovea (type 1: P = .010; type 2: P < .001)., Conclusions: Idiopathic macular telangiectasia type-specific differences in the distribution of photoreceptor abnormalities were shown in the AO-SLO images. For both idiopathic macular telangiectasia types 1 and 2, visual impairment was associated with cone damage., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: To investigate the efficacy and safety of intravitreal ranibizumab for the treatment of nonproliferative macular telangiectasia (MacTel) type 2., Design: Prospective, open-label, uncontrolled, nonrandomized interventional clinical trial., Methods: One eye (disease stage 2 or 3) of each patient (n = 10) with nonproliferative MacTel type 2 was injected with 0.5 mg ranibizumab at monthly intervals for one year. Visual acuity, angiographic findings, and retinal thickness were assessed at each visit. The primary endpoint was the change in best-corrected distance visual acuity after one year compared to baseline., Results: Mean visual acuity showed a transient increase in the study eye. However, after 12 months of treatment there was no significant change of visual acuity compared to baseline or compared to the fellow eye. Fluorescein angiography revealed a decrease of telangiectatic-appearing capillaries and of late-phase leakage, which was accompanied by a topographically related significant reduction in macular thickness. Three to 5 months after the last treatment, angiographic appearance and retinal thickness were similar to baseline. In one patient, the last intravitreal injection was not performed because of safety concerns after a transitory ischemic attack. Otherwise, no serious adverse events were observed., Conclusions: The angiographic and tomographic effects after intravitreal inhibition of vascular endothelial growth factor (VEGF) using ranibizumab implicate a pathophysiological role of the VEGF pathway in nonproliferative MacTel type 2. As the morphologic response was not associated with a clear functional benefit, and because of the transient nature of the treatment effect, monthly intravitreal ranibizumab is not recommended for the nonproliferative disease stage of MacTel type 2., (Copyright © 2011 Elsevier Inc. All rights reserved.)