Congenital retinal telangiectasia with vascular anomalies that nearly always presents unilaterally and can lead to exudation and eventually to exudative retinal detachment.
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.
Telangiectasia and aneurysms lead to exudation and eventu-ally to retinal detachment.
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.
Ophthalmoscopy will reveal telan-giectasia, subretinal whitish exudate with exudative retinal detachment and hemorrhages (Fig. 12.21).
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.
The treatment of choice is laser photocoagulation or cryotherapyto destroy anomalous vasculature.
Left untreated, the disease will eventually cause blindness due tototal retinal detachment. Treatment is effective in preventing blindness in about 50% of all patients.