Sorsby's fundus dystrophy.

Background: Sorsby's fundus dystrophy describes the condition in which an autosomal-dominant inherited macular dystrophy with bilateral central visual loss is accompanied by progressive atrophy of the peripheral choroid and, subsequently, the outer retina. The first symptom of this condition is nyctalopia, whereas (typically) the first sign is the appearance of white-yellow deposits in the posterior pole.

Case Report: We report a case of Sorsby's fundus dystrophy, manifested with central visual loss in the fifth decade of life. A battery of tests--including ophthalmoscopy, electroretinography, color vision, Pelli-Robson contrast sensitivity, and fluorescein angiography--were used to evaluate the condition.

Conclusions: While advances in understanding the etiology of Sorsby's fundus dystrophy have been made, the condition remains difficult to treat and the outcome is often devastating. The benefits of various methods of treatment, such as laser photocoagulation and dietary supplementation are unclear Patients affected by this condition should be managed in a manner similar to that for end-stage sufferers of age-related macular degeneration.