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Coats' Disease

Coats' disease is a rare, congenital, nonhereditary eye disorder, which may cause partial or full blindness to his patients. It is characterized by abnormal development of blood vessels behind the retina. Specifically, the blood leaks from the abnormal blood vessels in the back of the eye, leaving cholesterol deposits, which destroy the retina.

The progress of the disease is slow and at advanced stages there is a high risk of retinal detachment. Glaucoma and cataracts may also develop secondary to Coats' disease.
It usually occurs in the first decade of life and is unilateral (affects only one eye). Predominantly affects young males according to studies.

 


Φυσιολογικός ΒυθόςNatural Fundus

Fundus with Coats' diseaseFundus with Coats' disease

 

 

Symptoms of Coats' disease

The disease is asymptomatic in early stages. The most common symptom is called leukocoria (abnormal white reflection of the retina). In addition, blurred vision, especially when one eye is closed (due to unilateral nature of the disease). However, the good vision of the contralateral eye delays the onset of symptoms, especially in children who believe their vision quality is natural, acting against the 3D visual perception. The vision loss can be started either from the central or the peripheral visual field. Symptoms can also include flashes of light and floaters.
One early warning sign of Coats’ disease is yellow-eye in flash photography. If you think about the "red eye" phenomenon, which is caused by the reflection of blood vessels in the back of a normal eye, the image of an eye with Coats' disease has a yellow reflection, due to cholesterol deposits. These children should be referred immediately to an ophthalmologist, who will diagnose the disease and assess the whole situation.


Φαινόμενο κίτρινης αντανάκλασης κατά τη λήψη φωτογραφίαςYellow eye phenomenon in a childwith Coats' disease.

Coats' disease is painless. Pain may occur if the fluid flowing in the eye (aqueous humor) cannot properly drained, causing increase of the intraocular eye pressure (glaucoma).

Diagnosis of Coats' disease

The diagnosis of the disease is performed by funduscopic eye examination, where the abnormal dilated retinal vessels are observed and located temporally in early stages. At advanced stages, retinal detachment can be observed as well as hemorrhage from the abnormal blood vessels.
MRI and b-ultrasonography (US) can also give us useful information. On ultrasound, Coats' disease appears as a hyperechoic mass in the posterior vitreous. It also may appear bleeding.

Therapy of Coats' disease

The therapy strategy depends on the severity of the disease.
Mild peripheral vascular abnormalities are usually examined frequently by a vitreo-retinal specialist through fundoscopy.
The destruction of the abnormal blood vessels can be performed by laser surgery or cryotherapy, thus halting the progression of the disease.
At advanced stages, when a retinal detachment is observed, surgery for reattachment is performed, combined with vitrectomy. Intravitreal injection of corticosteroids, as well as anti-VEGF factors can be used to enhance the treatment result.
Removal of the eye (enucleation) is an option if the patient suffers from severe pain or complications arise.

Complications of Coats' disease

If leakage of blood vessels is observed around the area of the optic nerve, the laser treatment is not recommended, as poses a risk of nerve destruction, which may lead to permanent blindness.

Prognosis of Coats' disease

In literature there are cases reported, where either disease progression ceased by itself, or it reversed. However, despite treatment's success, lesions may recur or develop on healthy, new areas. Therefore, frequent follow-up examinations at your eye doctor's office are recommended.

Success Rates

Retinal detachment
Final restoration → 98,7%!

Μacular hole, final
restoration → 100%!

Epiretinal Membrane → Final Restoration 100%!
Lameral Hole → research in progress, results will be presented soon

 

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