Other Names for this Disease
- Retinal telangiectasis
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The signs and symptoms in affected individuals depend on the extent and size of the blood vessels involved. 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). The disease is almost always progressive, although alternating periods of acute worsening with periods of no apparent progression are common. 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). 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.
Last updated: 12/5/2011
- Coats disease. NORD. April 21, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/837/viewAbstract. Accessed 12/2/2011.
- Del Longo A. Coats disease. Orphanet Encyclopedia. September 2004; http://www.orpha.net/data/patho/Pro/en/Coats-FRenPro1645.pdf. Accessed 12/2/2011.