[Central areolar choroidal dystrophy with retinal crystals]

Central choroidal dystrophy is characterized by lack of large parts of the central choroid. In the dystrophic region outer retinal layers are also involved whereas inner retinal layers may be unaffected. Three patients with central choroidal dystrophy and retinal crystals in the dystrophic region or near its border are described. Predominantly, these crystals are located in the inner or middle retinal layers. The amplitude of the electroretinogram is reduced according to the extent of the defect in the neurosensory epithelium. In the cases described here the inner retinal layers may also be involved in the pathologic process. In two patients with the most marked clinical and electrophysiological disturbances, a multiple lipoprotein-type hyerlipidemia was found.