Best Disease (Best Vitelliform Macular Dystrophy)
-Unifocal lesion in the central macula.
-Accumulation of lipofuscin in the subsensory retinal space,there may be a pseudohypopyon appearance(“pseudohypopyon” stage).
-There is may be a zone of atrophy of the pigment epithelium (atrophic stage).
-Choroidal neovascularization and hemorrhagic detachment of the macula may occur at each stage.
-Abnormal electrooculographic (EOG) findings are universally present in patients with VMD regardless of the clinical presentation and are therefore helpful in making the diagnosis.