Atlas of Ophthalmology

Idiopathic Macular Telangiectasia Type 2 (Colour Photo, FFA, OCT)

Retina -> Enfermedades Maculares Adquiridas
Patient: 60 years of age, female, BCVA 0.4 at OD, 0.3 at OS. General Medical History: empty. Ocular Medical History: since 4 weeks deterioration of vision at both eyes. Main Complaint: deterioration of vision. Purpose: to demonstrate retinal findings in Idiopathic Macular Teleangiectasy Type 2. Methods: Colour Fundus Photography, Fluorescence Angiography, OCT (SD-OCT, HE). Findings: Colour Fundus Photography: showing dilatation of the parafoveal capillaries at both eyes, graying of the retinal area involved, central hyperplasia of the retinal pigment epithelium. Fluorescence Angiography. Showing dilated capillaries that leak dye (arrow), intraretinal hyperfluorescent leakage in the temporal parafoveal area. Optical coherence tomography (SD-OCT, HE): showing intraretinal hyporeflective space representing a detachment of the retinal pigmentepithelium with occult subretinal neovascularization (*), no cystoid changes in the macula. Notice the disruption of the junction of the photoreceptor inner segment and outer segment line. Discussion: Idiopathic macular telangiectasia type 2 (Mac Tel 2) is an acquired bilateral neurodegenerative macular disease. Based on current imaging modalities, Mac Tel 2 appears to be a primary neuroretinal degenerative condition with a secondary vascular involvement. Gass and Oyakawa (1) classified idiopathic juxtafoveolar retinal telangiectasis into four groups. Group 1 is characterized by unilateral parafoveal retinal telangiectasis associated with lipid and serous retinal exudation. Group 2 patients were mostly men with bilateral symmetric juxtafoveolar telangiectasis affecting the temporal half of the juxtafoveolar areas with minimal intraretinal exudation. Group 3 included eyes in both males and females that had bilateral parafoveolar telangiectasis with minimal intraretinal exudation, bilateral easily visible telangiectasis, and capillary occlusion. Group 4 referred to cases with familial optic disc pallor and perifoveolar retinal capillary occlusion. Wu et al. published an overview (2). He stated, that pathogenesis remains unclear. Müller cells and macular pigment appear to play a central role. The disease usually manifests itself during the fourth to sixth decades of life. Long-term prognosis for central vision is variable and depends on the development of SRNV or macular atrophy. Currently there is no known treatment for the underlying cause of this condition, but treatment of the SRNV may be beneficial. Literature: (1) Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology. 1993 Oct;100(10):1536-46 (2) Wu L, Evans T, Arevalo JF. Idiopathic macular telangiectasia type 2 (idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2). Surv Ophthalmol. 2013 Nov-Dec;58(6):536-59.

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