Background: Idiopathic juxtafoveolar telangiectasis (IJT) is a rare retinal vasculopathy of adulthood that may cause central loss of visual acuity. The purpose of this paper is to evaluate the type and distribution of fundus changes in patients with this disorder. Methods: Medical records of 19 patients with IJT using the Gass & Blodi classification were studied. Eight patients in group 1A (42%) and 11 in group 2A (58%) were analyzed. By projecting color photographs we divided the macula into four quadrants and studied the fundus changes in each one. Results: All patients in group 1A presented unilateral disease with easily seen telangiectasis, lipid deposits and cystic macular edema. The mean age of this group was 55.8 years. Five (63%) patients were male and 3 (37%) were female. All patients in group 2A, 2 males (18%) and 9 females (82%), had bilateral disease evidenced by fluorescein angiography. The mean age of this group was 54.5 years. Fundoscopic findings included: intraretinal pigment plaques, grayish coloring of the macular retina, superficial crystalline deposits, right-angle venules, subretinal and intraretinal neovascularization. Conclusions: All patients in this survey were adults. In group 1A, men were affected 2 times more often than women, and the main affected macular quadrant was the inferior temporal, followed by the inferior nasal. In group 2A, women were affected 4 times more often than men and all eyes had inferior and superior temporal macular quadrant involvement.

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