AZOOR with Uninvolved Fovea

This multimedia montage of images come from a 55-year old female patient with acute zonal occult outer retinopathy (AZOOR). She presented to the clinic complaining of having an enlarged blind spot in the left eye since the age of 18 and having photopsias when she coughed, sneezed, or stood up. The red arrows in the fundus autofluorecence image highlight areas retinal pigment epithelium (RPE) with excess lipofuscin. The turquoise arrows indicate a peripapillary zone of atrophied RPE and dead overlying photoreceptors. The green arrow on the Humphrey Visual Field highlights the enlarged blind spot caused by the zone of dead peripapillary photoreceptors. The yellow arrow on the OCT image highlights the loss of the photoreceptors (no ellipsoid zone) and RPE. Her visual acuity remained 20/20 because the fovea was uninvolved. In some cases the zone of photoreceptor and RPE atrophy enlarges over time.