Coats' disease

"Yellow eye" redirects here. For the military science fiction novel, see Yellow Eyes. For the plant, see Lupinus flavoculatus.

Coats' disease
Synonyms	Exudative retinitis, Retinal telangiectasis, Coates' disease
Pronounce	N/A
Field	Ophthalmology
Symptoms	Vision loss, leukocoria, photopsia, floaters
Complications	Retinal detachment, glaucoma, cataracts, blindness
Onset	Typically in childhood (1st decade of life)
Duration	Progressive or self-limiting
Types	Unilateral (most common), bilateral (rare)
Causes	Abnormal retinal blood vessel development
Risks	Male gender, childhood onset
Diagnosis	Fundoscopic exam, fluorescein angiography, imaging (US, CT, MRI)
Differential diagnosis	Retinoblastoma, Persistent fetal vasculature, Retinal detachment
Prevention	None known
Treatment	Laser photocoagulation, cryotherapy, vitrectomy
Medication	Anti-VEGF therapy (in select cases)
Prognosis	Variable; may stabilize, progress, or cause blindness
Frequency	1 in 100,000
Deaths	Rare, if misdiagnosed as retinoblastoma

Coats' disease is a rare, congenital, and non-hereditary eye disorder that primarily affects young males. It is characterized by abnormal development of retinal blood vessels, leading to fluid leakage, cholesterol deposits, and potential vision loss. In severe cases, it can result in total blindness due to retinal detachment and secondary complications.

Although Coats' disease primarily presents unilaterally (affecting one eye), bilateral cases have been reported. Due to its similarities in presentation to retinoblastoma, careful diagnosis is essential to avoid unnecessary enucleation (eye removal).

Signs and Symptoms

The symptoms of Coats' disease vary depending on the stage of progression. The earliest symptom is often leukocoria (an abnormal white reflection from the retina), which is frequently first noticed in flash photography.

Common symptoms include:

• Leukocoria – White or yellow pupil reflection in photographs.
• Blurred vision – Often noticed when covering the unaffected eye.
• Loss of depth perception – Due to one-eye compensation.
• Photopsia – Flashing lights in the affected eye.
• Floaters – Small moving spots in vision.
• Peripheral vision loss – Often starts in the upper field due to fluid pooling in the lower retina.
• Painless progression – Until complications such as glaucoma cause discomfort.

Coats' disease is often detected incidentally when parents notice a child with yellow-eye reflex in photos rather than the usual red-eye effect. Early evaluation by an ophthalmologist is critical for diagnosis.

A young child with the yellow-eye sign of Coats' disease, visible only with flash photography.

Advanced Symptoms

If left untreated, the disease may progress to:

• Exudative retinal detachment – Separation of the retina from its underlying support.
• Glaucoma – Increased eye pressure due to fluid buildup.
• Cataracts – Clouding of the lens.
• Painful eye swelling – If fluid drainage is obstructed.
• Blindness – If retinal detachment or optic nerve damage occurs.

Causes and Risk Factors

The exact cause of Coats' disease is unknown, but it is believed to result from defective blood vessel formation in the retina. The disease occurs sporadically and is not inherited.

Risk Factors

• Male sex – About 90% of cases occur in males.
• Childhood onset – Most common between ages 6 and 8.
• Unilateral occurrence – Affects one eye in most cases.

Pathogenesis

Coats' disease arises due to leakage from defective capillaries in the retina. This leads to:

• Cholesterol and lipid deposits within retinal layers.
• Thickening of the retina due to protein buildup.
• Gradual detachment of the retina as fluid accumulates.

As the disease progresses, these changes disrupt normal vision and may eventually lead to permanent vision loss if untreated.

Diagnosis

== Clinical Examination Diagnosis is confirmed through fundoscopic examination by an ophthalmologist. Key findings include:

• Tortuous and dilated blood vessels in the retina.
• Retinal exudates (lipid deposits).
• Varying degrees of retinal detachment.

Imaging Studies

CT scan showing total exudative retinal detachment in Coats' disease.

Additional tests help distinguish Coats' disease from retinoblastoma and other retinal disorders:

• Fluorescein Angiography – Evaluates leaking retinal blood vessels.
• Ultrasound (US) – Detects fluid accumulation and detachment.
• Computed Tomography (CT) – Shows a hyperdense vitreous.
• Magnetic Resonance Imaging (MRI) – Identifies fluid leakage and retinal thickening.

Early-Stage Treatment

When diagnosed early, treatment can halt disease progression and preserve vision. Common approaches include:

• Laser photocoagulation – Seals leaking blood vessels.
• Cryotherapy – Freezes and destroys abnormal vessels.
• Anti-VEGF injections – May help reduce fluid accumulation.

Advanced Disease Management

For severe cases with retinal detachment, more invasive treatments are required:

• Vitrectomy – Surgical removal of vitreous fluid to reattach the retina.
• Scleral Buckle – A silicone band placed around the eye to support the retina.
• Enucleation (Eye Removal) – Performed only if the eye is non-functional and painful.

Prognosis

The prognosis of Coats' disease varies based on stage at diagnosis and treatment response.

• Mild Cases – If caught early, vision can be preserved or stabilized.
• Moderate Cases – May result in permanent vision impairment.
• Severe Cases – Often lead to blindness or eye loss.

Some cases stabilize on their own, while others progress rapidly, requiring aggressive intervention.

Historical Background

Coats' disease is named after George Coats, who first described the condition in 1908.

See Also

• Retinoblastoma
• Glaucoma
• Retinal detachment
• Macular edema

External Links

• GeneReviews on NDP-Related Retinopathies
• Orphanet Overview of Coats' Disease

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