Figure 1. .

Color photograph of the right eye of a 10-year-old affected child (IV:3). At this age, a very obvious and unusual perivascular “cuff” of atrophy (arrows) around the superior and inferior vascular arcades is visible. The optic disc appears normal in color, although the retinal arterioles are narrow, and there was no pigmentary degeneration at this stage. The cuff of RPE atrophy was found in three other children in this family as well but was not found in the adults of this pedigree. At a later age, this feature apparently disappears.