Coats’ Disease



Coats’ Disease

(AKA Coats' retinitis, Coats' syndrome, Morbus Coats, exudative retinitis, and retinal telangiectasis)

description: Coats' disease involves leakage of the blood vessels in the retina. Various components in the blood actually leak into the retina. Most of these components seep back out of the eye, but cholesterol tends to remain and gradually build up until the retina is damaged and eyesight is diminished. Eventually, the retina becomes detached - separated from the other layers of the eye. Coats' disease typically afflicts young males in a single eye. Specifically, the typical age is between 18 months and 18 years. Males are afflicted 69% of the time, and between 80% to 95% of cases involve only a single eye.

cause: Its cause is not currently known. However, it has been described as a manifestation of facioscapulohumeral dystrophy, which is a more precisely characterized condition.

symptoms: Symptoms begin as blurred vision, usually pronounced when one eye is closed. Often the unaffected eye will compensate for the loss of vision in the other eye; however, this results in some loss of depth perception and parallax. Deterioration of sight may begin in either the central or peripheral vision. Deterioration is likely to begin in the upper part of the vision field as this corresponds with the bottom of the eye where blood usually pools. Flashes of light, known as photopsia, and floaters are common symptoms. Persistent color patterns may also be perceived in the affected eye. Initially, these may be mistaken for psychological hallucinations, but are actually the result of both retinal detachment and foreign fluids mechanically interacting with the photoreceptors located on the retina.

Important!!

One early warning sign of Coats’ disease is yellow-eye in flash photography. Just as the red-eye effect is caused by a reflection off blood vessels in the back of a normal eye, an eye affected by Coats’ will glow yellow in photographs as light reflects off cholesterol deposits. Children with yellow-eye in photographs are typically advised to immediately seek evaluation from an ophthalmologist.

treatment: The two most common treatments for Coats' Disease are Laser Photocoagulation and Cryotherapy. The treatment chosen depends on the stage at which the disease was diagnosed. There are a few new treatments in various stages of clinical trials, but are years away from becoming available. Some doctors are experimenting with a drug called Avastin, which is actually a drug used to treat cancer. Avastin is an FDA approved therapy designed to inhibit angiogenesis, the process by which new blood vessels develop. In cancer patients, this anti-angiogenic process starves the tumor of new blood cells. In Coats' disease, it is believed that Avastin would greatly inhibit the development of new blood vessels. This could potentially eliminate the need for repeated sessions of laser therapy and the scar tissue it causes. Other drugs that may show potential to treat Coats' disease are Macugen, Lucentis and Retaane, all currently used to treat AMD (Age related Macular Degeneration). Retaane and Lucentis are currently in clinical trials, but will only accept patients over 18 years of age.  These potential treatments are probably 5 to 7 years away from FDA approval for use in treating Coats'.

Illustration:

sources:

1. 1. Dictionary of Eye Terminology 5th Ed., Barbara Cassin and Melin L. Rubin, MD, Editor, 2006. Gainesville: FL, pg 67.

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developed by:Darren Stilwell

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