Chorioretinal dysplasia in young subjects with Wagner's hereditary vitreoretinal degeneration

Six young patients from three pedigrees with Wagner's disease were submitted to fluorescein angiography of the peripheral fundus. All eyes showed aberrant areas, characterized by: 1. localization principally in equatorial region of temporal half of the fundus. 2. whitish appearance of neuroretina with sharp demarcations. 3. absence of retinal vasculature. 4. marked atrophy of choriocapillaris and retinal pigment epithelium. 5. abnormal deflections of retinal vessels at the posterior margin of these areas. Arguments are presented in support of the hypothesis that these areas were dysplastic and not degenerative. The difference with lattice degeneration, another wellknown finding in Wagner's disease, is that retinal vasculature never developed in the dysplastic peripheral areas. It is probable that hypoplasia of the choroid has led to a too inferior structure of the overlying retina to permit development of retinal vessels. The frequent occurrence of retinal breaks in young patients with Wagner's disease, especially in the temporal periphery, seems to be a consequence of the poor condition of the retina in these dysplastic areas caused by the inferior development of both retinal and choroidal vasculature.