An Overview of Bietti’s Crystalline Corneo-Retinal Dystrophy
Bietti’s crystalline corneoretinal dystrophy (BCCD) is a rare heritable disease first described in a case-series of three patients in 1937. Since then, BCCD has been reported worldwide in a range of ethnic groups, although it is known to be particularly prevalent in the Japanese and Chinese populations. In its early stages BCCD is often asymptomatic, and may only be picked up as an incidental finding in patients that have undergone slit-lamp fundoscopy. The disease typically becomes symptomatic at 20 to 40 years of age, with affected patients reporting impaired night-vision, constriction of the visual-fields, and a decline in visual acuity, sometimes with colour-vision impairment. Both eyes are typically affected, although often asymmetrically.