Dominant Exudative Vitreoretinopathy and other Vascular Developmental Disorders of the Peripheral Retina

Dominant exudative vitreoretinopathy (DEVR) is an eye disease which has only recently received wider attention. In 1969 Criswick and Schepens used the designation "familial exudative vitreoretinopathy" to describe a syndrome they observed in six patients belonging to two families. The condition was characterized by several symptoms involving the vitreous and retina, e. g. "posterior vitreous detachment, organized vitreous membranes, heterotopia of the macula, retinal neovascularizations, subretinal and intraretinal exudates, and localized retinal detachment". The clinical features impressed the authors as strongly reminiscent of retrolental fibroplasia, but none of the patients had a record of premature birth or postnatal oxygen administration. In 1971 Cow and Oliver described the same syndrome in several members of one family. They considered their findings to be compatible with auto- somal dominant transmission. Canny and Oliver (I976) were the first to de- monstrate the fluorescein-angiographic changes of DEVR in four members of the abovementioned family. The most striking finding was "abrupt cessation of the capillary network in a scaloped edge near the equator".
Fluorescein was seen to leak from the retinal vessels localized in this marginal zone, and in some eyes from massive fibrovascular lesions as well. Similar fluorescein- angiographic changes have been described in recent years in other reports on families with DEVR (Nijhuis et aI. , 1979; Slusher and Hutton, 1979; Dudgeon, 1979; Ober et a1. , 1980; Laqua, 1980). In 1979 I commenced a clinical study of this still little-known condition at the Nijmegen University Institute of Ophthalmology (The Netherlands).