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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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Your Question

My 2 year old son was recently diagnosed with Coats disease stage 3a. I would love to know more about this, the various stages and what happens in them, and the possible treatments and long term prognosis.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Coats disease?

Coats disease is an eye disorder characterized by abnormal development of the blood vessels in the retina (retinal telangiectasia). Most affected people begin showing symptoms of the condition in childhood. Early signs and symptoms vary but may include vision loss, crossed eyes (strabismus), and a white mass in the pupil behind the lens of the eye (leukocoria). Overtime, coats disease may also lead to retinal detachment, glaucoma, and clouding of the lens of the eye (cataracts) as the disease progresses. In most cases, only one eye is affected (unilateral). The exact underlying cause is not known but some cases may be due to somatic mutations in the NDP gene. Treatment depends on the symptoms present and may include cryotherapy, laser therapy, and/or surgery.[1][2]
Last updated: 12/15/2014

What are the stages of Coats disease?

The most recently proposed staging classification for Coats disease was developed on based in a large series of affected people. This classification may help in choosing the most appropriate course of treatment as well as predicting the long-term outcome (prognosis) for people with Coats disease:[2][3]
  • Stage 1 - retinal telangiectasia only (dilation of capillaries in the retina)
  • Stage 2 - telangiectasia and exudation (escape of fluids and material from blood vessels into surrounding tissues)
    • A - extrafoveal exudation (exudation outside of the fovea, which is a small area in the retina responsible for the acute vision)
    • B - foveal exudation (exudation in the fovea)
  • Stage 3 - exudative retinal detachment
    • A - subtotal (partial) detachment
      • 1 - extrafoveal
      • 2 - foveal
    • B - total retinal detachment
  • Stage 4 - total retinal detachment and glaucoma
  • Stage 5 - advanced end-stage disease (defined as a blind, non-painful eye with a total retinal detachment, often with cataract and phthisis bulbi)
Last updated: 12/16/2014

How might Coats disease be treated?

The treatment of Coats disease depends on the signs and symptoms present in each person.[1] Treatment is usually directed towards destroying affected blood vessels in the retina and salvaging as much vision as possible.[4] A procedure that uses extreme cold to destroy abnormal blood vessels (cryotherapy), and/or a procedure that uses laser energy to heat and destroy abnormal tissue (photocoagulation) are often used singly or in combination.[1] These procedures are typically used during the early stages of the disease along with steroids and other medications to control inflammation and leaking from blood vessels.[4][1]

More advanced cases may require surgical treatment.[4] For example, surgery to reattach the retina may be necessary in cases of retinal detachment.[1] Draining or surgically removing the fluids that fill the eyeball between the lens and the retina (vitrectomy) may also be used to treat Coats disease when retinal detachment is present.[4]
Last updated: 12/15/2014

What is the long-term outlook for people with Coats disease?

Factors that effect the long-term outlook (prognosis) for people with Coats disease include the stage at which the person is diagnosed, the rate of disease progression, and the effectiveness of treatment. An ophthalmologist (doctor who specializes in eye problems) with experience in the condition may be able to make a general prediction about the chances of retaining the eye and preserving vision. It has been reported that most people respond well to treatment, but approximately 25% will become worse or require removal of the eye. The visual outcome in affected people varies considerably.[3]

When Coats disease is first diagnosed in older children and young adults, it tends to be less aggressive. In some older children and young adults, spontaneous regression (improvement of symptoms) has been reported. If macular exudation (escape of fluid, cells, and cellular debris from blood vessels) and extensive retinal detachment (such as in stages 1 and 2A) are not present at diagnosis, a good visual outcome can be expected. However, the presence of thick foveal (a small area in the retina responsible for the acute vision) exudation (stage 2B disease and above) is usually associated with a worse prognosis. Most experts agree that visual prognosis is poor in people with total retinal detachment (stage 3B and above), which is usually characterized by little to no useful vision in the affected eye despite treatment. Younger children typically have more aggressive disease and often have a total retinal detachment by the time they are diagnosed; these children usually have a poor visual outcome.[3]
Last updated: 12/15/2014

References
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.