Bietti’s crystalline dystrophy


Bietti's crystalline dystrophy (BCD) is rare inherited eye disease characterized by crystals in the clear covering of the eye. Bietti's crystalline dystrophy is also called Bietti's crystalline corneoretinal dystrophy. It is named after Dr. G. B. Bietti, an Italian ophthalmologist, who described three patients with similar symptoms in 1937.


Some of the most common symptoms of Bietti's crystalline dystrophy are: * crystals in the cornea (the clear covering of the eye) * yellow, shiny deposits on the retina * progressive atrophy of the retina, choriocapillaries and the back layers of the eye * progressive night blindness * visual field constriction


From family studies, we know that BCD is inherited primarily in an autosomal recessive fashion. This means that an affected person receives one nonworking gene from each of his or her parents. A person who inherits a nonworking gene from only one parent will be a carrier, but will not develop the disease. A person with BCD syndrome will pass on one gene to each of his or her children. However, unless the person has children with another carrier of BCD genes, the individual's children are not at risk for developing the disease.


Unfortunately, there is no treatment for Bietti's crystalline dystrophy. However, researchers hope that findings from gene research will be a helpful guide in finding treatments.