Ellipsoidopathy in Big Blind Spot Syndrome

Big blind spot syndrome (BBSS) was first described by Fletcher et al. in 1988. They reported seven patients with sudden onset of photopsias and a blind spot enlargement, but an otherwise normal fundus [1]. It is a rare retinopathy, characterized by an acute loss of peripapillary outer retinal function, and occurs predominantly in young myopic women. BBSS might belong to the spectrum of occult outer retinopathies, including multiple evanescent white dot syndrome (MEWDS) [2,3]. The mechanisms leading to BBSS and other occult outer retinopathies are speculative, but infection, vaccination, and autoimmune disorders have been implicated. The diagnosis is often difficult, mostly relying on visual field defects and electroretinographic abnormalities in the absence of clinical ocular findings.