Wilson disease (hepatolenticular degeneration) is an inherited autosomal recessive disorder of copper metabolism. The characteristic ocular manifestation of Wilson disease is the Kayser-Fleischer ring, a golden-brown discoloration of Descemet membrane around the periphery of the cornea (Fig 5-21A). In addition, a characteristic sunflower cataract often develops. Reddish-brown pigment (cuprous oxide) is deposited in the anterior lens capsule and subcapsular cortex in a stellate shape that resembles the petals of a sunflower (Fig 5-21B). In most cases, the sunflower cataract does not cause serious visual impairment. See also BCSC Section 8, External Disease and Cornea.
Figure 5-21 Ocular manifestations of Wilson disease. A, Slit-lamp image of Kayser-Fleischer ring, a golden-brown staining of Descemet membrane in corneal periphery. B, Clinical photo of reddish cuprous oxide deposited in the anterior lens capsule in a “sunflower” cataract.
(Courtesy of James Gilman, CRA, FOPS.)
Excerpted from BCSC 2020-2021 series: Section 11 - Lens and Cataract. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.