[Retinal exudative disease in childhood: Coats' disease and familial exudative vitreoretinopathy (FEVR)].

This article reviews the pathophysiology of retinal vascular disease with emphasis on Coats' disease and familial exudative vitreoretinopathy (FEVR). Both Coats' disease and FEVR demonstrate vascular abnormalities and associated exudation. Coats' disease manifests as teleangiectasia and aneurysms. Exudative subretinal lipid deposits can be extensive. Coats' disease is in 90 % unilateral and affects predominantly otherwise healthy young males. If the retina is attached, laser and cryocoagulation are the method of choice. Vitreoretinal surgery is only rarely indicated in advanced cases after a retinoblastoma has been excluded prior to surgery. FEVR inheritance is 56 % dominant (FZD4 und TSPAN12) and 44 % recessive (LRP5 und NDP). Temporal dragging of the vascular arcades and heterotopia of the macula are characteristic for FEVR. Subretinal exudates are indicators for progression of the disease with visual loss due to subsequent exudative or tractive retinal detachment. Exudative forms require treatment and reduction of peripheral ischaemia with laser photocoagulation and cryopexia. In cases of tractive detachments vitreoretinal surgery is necessary. Coats' disease and FEVR are both progressive diseases requiring lifelong follow-up and therapy.