Coats' Disease

A 29-year-old young patient suffers from a condition called Coats' disease. This disease has caused problems with his vision, in particular he has noticed "flying flies" (small dark spots that seem to float in the field of vision) due to bleeding within the eye. 

After consulting a specialist, he was diagnosed with Coats' disease. To confirm the diagnosis, the doctor performed a test called fluorangiography, which allows visualization of the blood vessels in the retina. Subsequently, the patient received Argon laser treatment to try to improve his condition.

Now the patient presented to our observation for recheck with fluorangiography. This examination showed the signs of the previous laser treatment, but also showed that the treatment needs to be completed. In particular, the following were observed:

• Areas of the retina with poor blood supply
• Dilated and irregular blood vessels
• Accumulation of fluid in the retina
• Abnormal connections between blood vessels in the peripheral part of the eye

These results indicate that further treatment may be needed to better manage the disease and preserve the patient's vision.

Figures 1, 2, 3, 4: Retinographies

Figure 5: Central Fag

Figures 6, 7: Early Fag

Figure 8: Late Fag

Coats' Disease (Exudative Retinitis)

Coats' disease is an idiopathic ocular condition characterized by retinal telangiectasia, aneurysms, and exudation, usually unilateral and predominantly in males.

The pathogenesis is associated with somatic mutations and abnormalities in the Wnt signaling pathway during retinal angiogenesis.

The main symptoms of Coats' disease include decreased visual acuity (43%), strabismus (23%), leukocoria or xanthocoria (20%), pain (3%), heterochromia (1%), and nystagmus (1%).

Visual acuity can range from 20/20 to 20/50 in 12% of cases, 20/60 to 20/100 in 11%, 20/200 to counting fingers in 18%, and from hand movements to no light perception in 58%. The main causes of poor vision include subfoveal fluid or lipids, subfoveal fibrosis, macular edema, epiretinal membrane, and optic atrophy.

Diagnosis is based on clinical examination, fluorangiography, retinography, optical coherence tomography, and ultrasonography.

Treatment varies depending on the stage of the disease and may include laser photocoagulation, cryotherapy, surgery, and intravitreal therapy with corticosteroids and anti-VEGF.

In advanced stages, the visual prognosis is worse, with potential progression to neovascular glaucoma and bulbar phthisis.

Interprofessional management is key to improving outcomes in patients with Coats disease.

Important Points and Description

Description: Coats' disease is an idiopathic ocular condition characterized by retinal telangiectasia, aneurysms, and exudation, usually unilateral and predominantly in males.

Pathogenesis: Associated with somatic mutations and abnormalities in the Wnt signaling pathway during retinal angiogenesis.

Main Symptoms:

• Decreased visual acuity (43%)
• Strabismus (23%)
• Leukocoria or xanthocoria (20%)
• Pain (3%)
• Heterochromia (1%)
• Nystagmus (1%)

Visual Acuity:

• 20/20 to 20/50 in 12% of cases
• 20/60 to 20/100 in 11%
• 20/200 counting fingers in 18%
• Hand movements to no light perception in 58%

Causes of Poor Vision:

• Subfoveal liquid or lipids
• Subfoveal fibrosis
• Macular edema
• Epiretinal membrane
• Optic atrophy

Diagnosis:

• Clinical examinations
• Fluorangiography
• Retinography
• Optical coherence tomography
• Ultrasound

Treatment: Varies depending on the stage of the disease and may include laser photocoagulation, cryotherapy, surgery, and intravitreal therapy with corticosteroids and anti-VEGF.

Prognosis: In advanced stages, the visual prognosis is worse, with potential progression to neovascular glaucoma and bulbar phthisis.

Management: Interprofessional management is essential to improve outcomes in patients with Coats disease.

Source: https://albertobellone.it/malattia-di-coats/
Thanks to Dr. Alberto Bellone