CHOROIDODERMIA

It is characterized by progressive vision loss, with the first symptom usually being incapacity for night vision, which can occur in early childhood. It is a recessive degenerative disease of the retina, linked to the X chromosome of the gene that encodes the Rab guard protein 1, and affects only males. It is characterized by the degeneration or atrophy of the capillary lamina of the choroid, the retinal pigment epithelium, and the photoreceptor cells of the retina. There is a progressive narrowing of the field of vision and a loss of fine vision of details, vision is progressively lost until reaching total blindness in adulthood. The capillaries of the choroid irrigate, supplying oxygen and nutrients, to the retina. When these atrophies, the blood flow to some of the layers of the retina ceases and the cells of the retina begin to die, which in turn causes the beginning of blindness.

Treatment: There are no treatments or cure for choroideremia, help is provided with low vision lenses.