Coats' Disease

A 21-year-old male presented with gradual decreased vision in the right eye, 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