A 7-year-old male patient presented with visual loss related to marked submacular lipid exudation and fibrosis. He had superotemporal peripheral retinal telangiectasis.

Severe form of retinal telangiectasia Typically presents in the first decade; average age 5 Clinical features: Signs: Telangiectatic vessels that appear as small, red "light bulbs" in the retinal periphery Most often located in the inferior and temporal quadrants between the equator and ora serrata Intraretinal and subretinal yellow exudation often affecting areas distant from the vascular abnormalities Hard exudates commonly present in the posterior pole Leukocoria in patients who present with extensive subretinal exudation Ultrasonography is useful to exclude retinoblastoma Differential diagnosis: Retinal capillary angiomatosis or hemangioma Late onset retinoblastoma Toxocara endophthalmitis Retinopathy of prematurity Familial Exudative Retinopathy Persistent primary hyperplastic vitreous Management: Cryotheraphy Laser photocoagulation to the telangiectatic vessels In advanced cases with total serous retinal detachment, external drainage of subretinal fluid and scleral buckling may be an option Poor visual prognosis in cases of massive exudation

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