This is sometimes called as "copper ring" since it appears as a brownish-green copper deposit on the inside surface of the peripheral cornea. It is associated with Wilson disease, a failure to metabolize copper properly.The upper pole is affected more frequently than the lower.
Kayser-Fleischer rings are noted in 90% of patients with Wilson disease and occasionally, in patients with prolonged cholestasis or cryptogenic cirrhosis. The ring is noted most easily in patients with blue eyes. In other patients, slit-lamp examination may be required.
A corresponding gene defect has been mapped to chromosome 13. This is an example of an ocular manifestation of a genetic disease.