dystrophie choroïdienne aréolaire centrale

CONT: Central areolar choroidal dystrophy (CACD) is a hereditary retinal disorder that primarily affects the macula. In the early stages, there is subtle, mottled depigmentation in the posterior pole. Ultimately, the depigmentation enlarges into in a well-circumscribed round or oval area of atrophy of the retinal pigment epithelium (RPE) and choriocapillaris in the center of the macula. Patients usually become symptomatic in the [third] to [fourth] decade when a fine, hardly detectable, mottling of the RPE leads to the development of absolute central visual scotomas. Later, between the [fourth] and [seventh] decade of life, progressive macular atrophy leads to a dramatic decline in central visual acuity and severe visual disability. CACD may be autosomal dominant or recessive; however, autosomal-recessive cases are rare. Although CACD has a genetic basis, sporadic cases have been reported.