gms | German Medical Science

Familial exsudative vitreo-retinopathy (FEVR) or autosomal dominat exsudative vitreo-retinal degeneration (Criswick-Schepens, 1969) is an often overlooked disease, that commonly effects children but more seldom effects also adults. It is the most common reason of juvenile retinal detachment. Its most detailed description is provided by Nouhuys (1982). Unlike other hereditary diseases FEVR is rather asymmetrically expressed. While juvenile retinal detachments can be managed fairly well, the problem arises when the disease is not diagnosed or misdiagnosed as uveitis and presents with exsudative retinal detachment, vitreous hemorrhage and finally phtisis. The expression of FEVR varies extensively from avascular areas in the temporal periphery to intra- and subretinal exsudates from abnormal retinal vessels: Macroaneurysms, dilated vessels, narrowed angle kappa. The prime therapeutic goal of exudative stages is the destruction of the abnormal retinal vessels. Therapeutic options range from observation (peripheral avascular area), via buckling surgery (juvenile retinal detachment) to vitrectomy and vitreous tamponade and photocoagulation.