Adult form of Best’s Vitelliform Macular Dystrophy – Case Reports

Overview

The purpose of this study is to refer three case reports of adult Best’s maculardystrophy. The group consists of three patients, one woman and two men, aged 49– 67 years. In all patients of the group only one lesion localized in the macula ofthe affected eye was noticed. In two patients, both eyes were affected; in the lastone the finding was unilateral. All lesions had typical clinical finding, whichcorresponded to the vitelliform stage of the Best’s disease.All patients underwent complex ophthalmologic examination including medicalhistory taking, visual acuity for far and for near, intraocular pressure measurement,examination of the visual fields, retinal examination with dilated pupils,optical coherent tomography, and electroretinogram, and electro-oculogram aswell. In all three patients, also the fundus fluorescein angiography was performed.The decrease of the visual acuity closely correlated to the seriousness and theduration of the retinal finding. The vitelliform lesion was located always in themacula, in sub- or juxtafoveolar localization. In none of those patients the authorscould find the genetic linkage or the transmission of the disease to the relatives.

Key words:
Best’s vitelliform macular dystrophy, adult form, autosomal dominantinheritance, pathologic electro-oculogram