Sorsby Pseudoinflammatory Fundus Dystrophy

This dominantly inherited disease begins with fine, pale, drusen-like deposits or confluent faint yellow material or sheets beneath the retinal pigment epithelium (RPE), but it eventually progresses to either geographic atrophy with pigmentary clumps or scar due to choroidal neovascular membrane (at about 40 years of age) (Figs. 20.1, 20.2 and 20.3). The patient usually becomes symptomatic, with loss of central vision (about 20/200 or less), in the fourth to sixth decade of life. When neovascular membrane develops, it mimics age-related macular degeneration (AMD), but the age of onset is much earlier.