Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study

Idiopathic juxtafoveolar retinal telangiectasis may cause visual loss. The treatment of this disease is controversial.

The authors reviewed the records of 140 patients with idiopathic juxtafoveolar telangiectasis. A classification scheme based on biomicroscopic and fluorescein angiographic findings is presented. In addition, the effect of photocoagulation on the natural history of the disorder is evaluated.

Patients are categorized into three groups. Group 1 comprises 39 male patients with nonfamilial, easily visible telangiectasis and intraretinal exudation. The telangiectasis is unilateral in 94% of patients. The telangiectasia in this group is probably of developmental origin (Coats syndrome). Group 2 comprises 94 patients with occult juxtafoveolar telangiectasis, minimal exudation, superficial retinal crystalline deposits, and right-angle venules. Late in the course of the disease, foveolar atrophy, intraretinal pigment plaques, and subretinal neovascularization develop. The telangiectasis is acquired during middle age and is bilateral in 98% of patients. Group 3 comprises seven patients with bilateral easily visible telangiectasis, minimal exudation, and capillary occlusion. All of these patients had systemic disease, which was probably related to their eye disease.

Slow visual loss beginning in adulthood characterizes most of these patients. The telangiectasis appears to be caused primarily by retinal capillary leakage in group 1, capillary diffusion abnormalities in group 2, and capillary occlusion in group 3. Photocoagulation is probably beneficial for patients in group 1 and not for patients in group 2, at least before their development of subretinal neovascularization.