Man presents with slow, progressive vision loss

A 61-year-old man visiting from the Ivory Coast presented to the New England Eye Center for slow, progressive vision loss in both eyes starting 2 to 3 years before presentation. His medical history was significant only for hypertension and gout. His family ocular history included his father, who was blind in one eye possibly due to a retinal detachment. He was evaluated by his local ophthalmologist on several occasions, but an etiology was never determined. Before his evaluation at the New England Eye Center, our patient was seen at another eye clinic in the United States and was told he may have a maculopathy.On exam, best corrected visual acuities were count fingers at 8 feet eccentrically in the right eye and 20/200 in the left eye. Pupils were round, equal and reactive without relative afferent pupillary defect. The patient was unable to identify the Ishihara control plate in either eye. Extraocular movements were full bilaterally. Anterior segment exam was remarkable only for 1+ nuclear sclerosis bilaterally. Dilated fundus exam was remarkable for a white linear scar extending from the inferior arcade to the temporal aspect of the macula in the right eye (Figure 1). The cup-to-disc ratio was 0.65 bilaterally, and there was moderate temporal pallor of both optic nerves (Figure 2).