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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Coats disease


Other Names for this Disease

  • Retinal telangiectasis
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Symptoms

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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

The Human Phenotype Ontology provides the following list of signs and symptoms for Coats disease. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of the retinal vasculature 90%
Strabismus 90%
Abnormality of the macula 50%
Glaucoma 50%
Retinal detachment 50%
Abnormality of the anterior chamber 7.5%
Aplasia/Hypoplasia of the iris 7.5%
Cataract 7.5%
Visual impairment 7.5%

Last updated: 9/2/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Coats disease. NORD. April 21, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/837/viewAbstract. Accessed 12/2/2011.
  2. Del Longo A. Coats disease. Orphanet Encyclopedia. September 2004; http://www.orpha.net/data/patho/Pro/en/Coats-FRenPro1645.pdf. Accessed 12/2/2011.


Other Names for this Disease
  • Retinal telangiectasis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.