Coats’ Disease

Coats’ Disease

Definition

Congenital retinal telangiectasia with vascular anomalies that nearly always presents unilaterally and can lead to exudation and eventually to exudative retinal detachment.

Epidemiology: 

This rare disorder manifests itself in young children andteenagers. Boys are usually affected (in about 90% of all cases).

Coats’ disease usually occurs in young and teenage boys. It is nearly always unilateral.

Pathogenesis: 

Telangiectasia and aneurysms lead to exudation and eventu-ally to retinal detachment.

Symptoms: 

The early stages are characterized by loss of visual acuity, thelater stages by leukocoria (white pupil; see Fig. 12.36) or unilateral stra-bismus, although the combination of leukocoria and strabismus is also possible.

Diagnostic considerations and findings: 

Ophthalmoscopy will reveal telan-giectasia, subretinal whitish exudate with exudative retinal detachment and hemorrhages (Fig. 12.21).

Differential diagnosis: 

In the advanced stages of the disorder, retinoblas-toma should be excluded by ophthalmoscopy and retinopathy of prematurity on the basis of the patient’s history. Both disorders may also cause leukocoria.

Treatment: 

The treatment of choice is laser photocoagulation or cryotherapyto destroy anomalous vasculature.

Prognosis: 

Left untreated, the disease will eventually cause blindness due tototal retinal detachment. Treatment is effective in preventing blindness in about 50% of all patients.