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Coats' Disease

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What is Coats' disease?

In 1912, Dr. George Coats described one sharply outlined form of exudative retinitis, clinically characterized as follows:

  1. Occurrence in infantile or juvenile male patients
  2. Unilaterality
  3. Absence of systemic diseases
  4. Exudates below the retinal vessels
  5. Retinal hemorrhages
  6. Slow progression to retinal detachment, cataract, atrophy, or glaucoma

Today little has been added. This rare disease is not inherited and may be successfully treated if caught early. However, since is not usually diagnosed early, it usually progresses to cause a marked loss of vision or blindness in one eye.

What are causes and risk factors for Coats' disease?

No causes or risk factors are known for the condition.

At this point, although there is no known hereditary component or any other cause, there may be some evidence to suggest that Coats' disease is caused by a somatic mutation of the Norrie disease protein (NDP) gene. There are no known risk factors, although the condition occurs in males three times as often in females.

What are symptoms and signs of Coats' disease?

Vision loss is gradual and may not be recognized at first due to the young age at which the disease begins. Since it often starts in one eye, the child may compensate well and not notice that the vision is bad in the other eye. It may begin in infants, is most commonly seen between 6-9 years of age, and it rarely is seen in geriatric patients. It is a painless disease.

Usually only one eye is affected and it is more common in males. When only one eye is involved, the individual may carry out almost all visual tasks but may have some limitations if binocular vision is required (relatively rarely). This is usually not noticed by the individual or parents. Depth perception is still present, but it may be decreased significantly.

In England, one study found 0.09 cases in every 100,000 individuals. Therefore, it is quite rare.


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