Bietti crystalline dystrophy

Bietti crystal dystrophy is a congenital hereditary disease of the retina with tapetoretinal degeneration beginning between the ages of 20 and 30 years. In the posterior parts of the retina, as well as in the area of the cornea limbus and the leukocytes, crystalline deposit rings are deposited whose composition is still unclear. Furthermore, sclerotherapy of the choroidal vessels occurs.

In the course of the disease, it comes to night blindness, progressive deterioration in vision and pericentral visual field defects and ultimately leads to blindness.

The disease is also seen as a special form of retinitis pigmentosa.

The cause is a gene defect in the CYP4V2 gene, which encodes the cytochrome P450 4V2 protein. The inheritance is autosomal recessive and it is assumed an increased prevalence in people of Asian origin.