Extensive macular atrophy with pseudodrusen-like appearance

摘要

Extensive macular atrophy with pseudodrusen-like appearance (EMAP) is a recently reported, rare clinical entity1 resembling dry age-related macular degeneration (AMD), but with a distinct clinical appearance. It is characterized by early onset (<50 years), bilateral, symmetric macular atrophy with a larger vertical axis, surrounded by numerous pseudodrusen-like deposits, with a rapid involvement of the fovea and of the entire posterior pole. In addition, in the original description,all EMAP patients have found to present paving stone degeneration, predominantly observed in the inferior part of the peripheral retina. Conversely, in dry AMD, macular atrophy appears after the age of 50 years, as a result of the confluence of multiple small foci after drusen regression, and is characterized by a slow growth rate and a larger horizontal axis. Moreover, peripheral paving stone degeneration does not seem to be a typical characteristic of dry AMD.