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Genetic and Rare Diseases Information Center (GARD)

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Coats disease

Other Names for this Disease
  • Retinal telangiectasis
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What are the signs and symptoms of Coats disease?

The signs and symptoms in affected individuals depend on the extent and size of the blood vessels involved.[1] Some individuals may be asymptomatic while others are very severely affected. Signs and symptoms typically begin at an early age (most commonly between ages 6 and 8).[2] The disease is almost always progressive, although alternating periods of acute worsening with periods of no apparent progression are common.[2] Affected individuals may first experience loss of vision; crossed eyes (strabismus); and/or the development of a white mass in the pupil behind the lens of the eye so that the pupil appears white (leukokoria or "cat's eye" reflex).[1] As the disease progresses, individuals may develop glaucoma; cataracts; reddish discoloration in the iris (rubeosis iridis or neovascular glaucoma); shrinking of the affected eyeball (phthisis bulbi); and/or swelling and irritation of the middle layer of the eye (uveitis). The majority of affected individuals eventually experience profound vision loss and retinal detatchment.[1][2]
Last updated: 12/5/2011

  1. Coats disease. NORD. April 21, 2008; Accessed 12/2/2011.
  2. Del Longo A. Coats disease. Orphanet Encyclopedia. September 2004; Accessed 12/2/2011.