Hereditary vitelline macular degeneration presents a widely varied ophthalmoscopic picture. The visual loss and age of onset differ within and between pedigrees. Typically it consists of a macular degeneration with early onset in which the central retinal changes are evident with the ophthalmoscope long before the symptoms appear. There is a loss of central vision, with the onset from long before puberty to the early 30's and slight if any progression, so that most of the affected individuals are still able to read large print in their 50's and 60's. Inheritance is usually autosomal dominant. Visual loss is relatively mild, and the ophthalmoscopic findings run a gamut from mild pigmentary degeneration to dense scarring with pigmentary hyperplasia. The affliction is bilateral, but the onset, visual loss, and appearance often vary between the two eyes.
Since Best,1 in 1905, described the initial pedigree of this condition, many other pedigrees have
BRALEY AE, SPIVEY BE. Hereditary Vitelline Macular DegenerationA Clinical and Functional Evaluation of a New Pedigree With Variable Expressivity and Dominant Inheritance. Arch Ophthalmol. 1964;72(6):743-762. doi:10.1001/archopht.1964.00970020743003