Coats' disease

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Coats' disease is a rare, nonhereditary eye disorder that usually begins in childhood. It affects the blood vessels behind the retina, causing them to leak fluid and cholesterol-rich material. This buildup damages the retina and can lead to partial or total vision loss. The disease almost always affects one eye and is more common in boys.

What it is
- Abnormal, leaky retinal vessels with cholesterol deposits.
- Usually progresses slowly; in advanced cases, the retina can detach.
- Pain is not common, but eye pain can occur if fluid buildup raises eye pressure (glaucoma).

Signs and symptoms
- Blurred vision or decreased vision in one eye.
- A white or yellowish reflection in photos of the eye (leukocoria or “yellow-eye” in flash photography).
- Flashes of light (photopsia) and floaters.
- Depth perception may be reduced because the other eye compensates.
- Vision loss can start in central or peripheral parts of the field of view.

Diagnosis
- Eye exam shows abnormal, dilated, twisted blood vessels mainly at the edge of the retina.
- Imaging tests (ultrasound, CT, MRI) help confirm the diagnosis and show any detachment.

Treatment
- Early treatment aims to stop leakage: laser therapy or freezing (cryo-therapy) to destroy abnormal vessels.
- If vessels are near the optic nerve, treatment may be avoided or carefully planned to prevent blindness.
- The disease may stabilize or improve on its own in some cases.
- If the eye becomes painful or the retina detaches, more invasive options like removing the eye (enucleation) may be considered.
- Vitrectomy (removal of the vitreous gel) can be used after detachment in some infants.

Cause
- Thought to result from a breakdown of the blood-retina barrier, causing leakage of lipid-rich fluid and cholesterol into the retina and space beneath it. This exudate thickens the retina and can lead to detachment.

Prognosis
- Vision often declines, especially with retinal detachment, but some cases stabilize or even improve. The outcome varies widely.

Additional notes
- Coats' disease is typically a solitary, unilateral condition in young people. It is not usually inherited, but its exact cause is not fully understood.