Figure 2. Fundus photographs of affected subjects from the study family

A: Individual IV:1, age 36, with visual acuities of 20/40 in the right eye and 20/50 in the left eye. Color fundus photograph of the right eye. Subtle pigmentary changes are evident in the macula. B: Color fundus photograph of the left eye show similar changes. C: Fundus fluorescein angiogram of the right eye. Taken at 5:42 min after injection of sodium fluorescein, this photographic method reveals more marked changes in the retina that are not immediately evident in the color photographs. Areas of brightness within the macula (arrows) are referred to as "window defects" and represent areas of retinal and retinal pigment epithelium atrophy. D: Fundus fluorescein angiogram of the left eye taken at 6:22 min after injection shows similar changes. E,F: Color fundus photographs from the more severely affected mother of the subject depicted in Figure 2. Individual III:2, age 64, has visual acuities of 20/400 in the right eye and 20/400 in the left eye. E: Color fundus photograph of the right eye reveals atrophy of the macula. F: Color fundus photographs of the left eye reveal similar changes. The peripheral retina looks normal.