Late Stage Best Disease

These images were taken from a 71 year-old female patient with late-stage Best disease. This is a hereditary condition that results in the accumulation of metabolic waste material under the retina, typically in the macula, but sometimes outside the macula. In this case the macula was involved (yellow arrows). The OCT images illustrate the disruption of the ellipsoid zone (red arrows) and accumulation of hyperreflective waste material under the retina (green arrow). Our patient was diagnosed at the age of 20 and both her mother and brother have the disease. We began seeing her in 2005 and her vision has gradually gotten worse over time. She was seeing 20/50 OD in 2005 and now sees 20/200 in 2019. The left eye, however, has remained stable at 20/125.