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Coats' Disease

21-year-old male presented with gradual decreased vision, and diagnosed with abnormal telangiectatic vessels in the superotemporal periphery with submacular and extramacular lipid exudation associated with tractional retinal detachment and foveal ectopia.

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

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