First Author: I.Garcia-Basterra SPAIN
Co Author(s): N. Rachwani M. Morillo A. Garcia Ben
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To describe the follow-up of three cases of bilateral idiopathic macular telangiectasia type 2 (MacTel 2) treated with intravitreal ranibizumab in comparison with two cases managed with conservative treatment.
University Hospital Virgen de la Victoria, Málaga, Spain.
5 subjects (2 men and 3 women) with bilateral macular lesions and decreased vision loss were evaluated in the Retina Department. Funduscopy revealed circumscribed and heavily pigmented small lesions of the retina located at fovea, loss of transparency with grey discolouration surrounding the foveola, crystalline-like deposits and angled veins. Fluorescein angiography (AFG) revealed hypofluorescence lesions with a prominent halo of hyperfluorescence during late phase and dilated vessels with mild extravasation of dye. The main findings on OCT were segmental retinal elevation, increased optical reflectivity on inner surface with optical shadowing of deeper structures, hyporreflective cavitations and disruption of perifoveal photoreceptor outer-inner segments.
Three cases were treated with 3 doses of intravitreal ranibizumab and two cases were observed without treatment. After 6 months of follow-up, there were neither anatomical nor visual changes in the treated group compared with the non-treated group. The final visual acuity remained unchanged during the follow-up.
MacTel-2 is a rare and acquired retinal disorder characterized by incompetence, ectasia, and irregular dilations of the juxtafoveolar capillary network. The diagnosis is based on funduscopy, OCT and AFG findings. The main complications include photoreceptors damage, choroidal neovascularization and proliferation of pigmented plaques. The aetiology and physiopathology is unknown. Vascular endothelial growth factors may play a role in this disorder due to the excessive vascular permeability from telangiectatic vessels. Although intravitreal ranibizumab has demonstrated that decreases vascular leakage in the absence of neovascularization in Mac-Tel 2, it does not improve outcomes compared with observation, as has been reported in our cases.