Fried egg appearance of Best disease

Best disease, or Best vitelliform macular dystrophy, is an inherited eye condition affecting the macula, the central region vital for clear vision. Typically appearing in childhood or adolescence and progressing over time, it results in central vision loss. Named after German ophthalmologist Dr. Friedrich Best, who observed a family with various stages of the disease in 1905. Best disease can reveal a distinctive fried egg appearance in the macula. This look, resembling a yellowish egg yolk, is caused by the buildup of lipofuscin, a fatty substance, in the retinal pigment epithelium cells. These cells support the retina, the light-sensitive layer of tissue in the eye. Imaging techniques, like fundoscopy, allow us to see this fried egg appearance.

Reference

Tripathy K, Salini B. Best Disease. [Updated 2023 Aug 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Go to reference

Fried egg appearance, Fried egg

Image from Nentwich MM, Rudolph G. Hereditary retinal eye diseases in childhood and youth affecting the central retina. Oman J Ophthalmol. 2013.