Bietti Crystalline Retinopathy

Synonyms

Bietti crystalline corneoretinal dystrophy; Bietti crystalline dystrophy; Bietti crystalline retinopathy; BCD

Definition

Bietti crystalline dystrophy is an autosomal recessive disease characterized by glistening crystalline-like changes in the posterior pole of the retina and atrophy of the retinal pigment epithelium (RPE) and choroid. Furthermore one-third of patients may present with crystal deposits in the superficial stromal layer of the paralimbal region of the cornea. This disease is named after Dr. G. B. Bietti, an Italian ophthalmologist, who described three patients with similar symptoms in 1937 (Okialda et al. 1993–2014; Ryan et al. 2012).

Epidemiology

Bietti crystalline dystrophy is rare and has been estimated to occur in 1 in 67,000 people (Okialda et al. 1993–2014). It is more common in people of East Asian descent, especially those of Chinese and Japanese background. Researchers suggest that Bietti crystalline dystrophy may be under diagnosed because its...