Coats' Disease

Coats' disease is also known as Coats' retinitis, Coats' syndrome, Morbus Coats, exudative retinitis, and retinal telangiectasis. The proper spelling is with the apostrophe at the end of Coats'.

Authoritative descriptions of Coats' disease are found at Whonamedit.com and Review of Optometry. Those sites also mention muscular dystrophy and mental retardation, but a diagnosis of Coats' Disease does NOT indicate the presence of the other conditions.

In our own words, 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. The cause of origin is unknown, although currently evidence seems to point to genetic mutations (Del Longo and Sims).

Coats' Disease is believed to be congenital, meaning a patient is born with the disease. It may not show up, however, until later in life.

Symptoms in our child

These are the symptoms we compiled from our own son's case.

• Vision in one eye is noticeably impaired. Once the child is old enough to read, this problem becomes obvious. Unfortunately, parents of younger children have little indication of this impairment, and the child will silently accept the impairment as normal.
• The child keeps rubbing the affected eye with his hand. This was the first symptom of our son.
• The child has intermittent exotropia (one eye points outward, but only when looking at a distant object). This was the symptom that got our son into the eye doctor.
• In flash pictures, one eye glows yellow rather than red. This was actually an earlier symptom that we missed, until we went back and looked at old pictures. The yellow is light reflecting off the cholesterol-laden retina. Red is normal, because light reflects off of normal red blood vessels. The term patients will hear during an opthalmologist exam is "red reflex". A finding of "negative red reflex" means there is a problem.

  Yellow eye in picture

  A yellow eye in a flash picture may also indicate retinoblastoma - a malignant eye tumor that is fatal if left untreated! We strongly recommend that you immediately contact a medical professional to identify the problem and start the appropriate treatment.
• In a doctor's eye exam, with the eye dilated, eye damage is seen. This indication can only be caught by a doctor, but is mentioned here to bring up two important points. The first is that only a doctor can perform the examination. The second is that the doctor has to dilate the eye to see the damage. Normal examinations of children will miss Coats' Disease, since the eye is not dilated.