Acute zonal occult outer retinopathy: a literature review

Investigators used English language medical literature to review the clinical features, visual field testing, differential diagnosis, results of attempted treatments, and speculated pathogenic mechanisms of 131 cases of acute zonal occult outer retinopathy (AZOOR), a rare disease of unknown etiology characterized by focal degeneration of photoreceptors.

The majority of patients are white, female with an average age of 37 years. Most present with scotoma, photopsia, a visual acuity of 20/40 or better and unremarkable fundus. Electroretinographic abnormalities are present in 99 percent of patients. Good visual acuity is typically retained, although retinal pigment epithelial disturbances usually develop over time. It is unusual for visual field loss to continue beyond six months and no treatment has been proven effective.