Peripapillary (pericentral) pigmentary retinal degeneration

Three patients with peripapillary (pericentral) pigmentary retinal degeneration described in 1978 were examined over a follow-up period of nine to 13 years. There was no extension of the geographic pigmentary abnormalities. The affected areas worsened as evidenced by an increase in pigment abnormalities, focal choroidal atrophy as seen on fluorescein angiography, and a diminution of the electroretinographic responses. One patient had increasing disabling symptoms of nyctalopia and poor peripheral and central vision, whereas the other two patients remained asymptomatic with excellent central vision.