Central Areolar Choroidal Dystrophy

Central areolar choroidal dystrophy is a rare, hereditary disorder of the macular area. In the present communication we report the fourth case in which pathological examination has been done, and the observations are similar to those noted in the three previous patients. The retinal pigment epithelium and choriocapillaris are absent in the area of the lesion, exposing the underlying atrophic choroid and the sclera. In this sharply circumscribed area photoreceptors are absent and there is a profound reduction in number of photoreceptor nuclei in the outer nuclear layer. The disease is transmitted in autosomal dominant fashion. Appropriate genetic and vocational counseling should be offered to afflicted individuals and their children.