Coats' Disease

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