They are usually brown or dark reddish in color. Early on they may need a slit lamp to be visible before they become visible to the naked eye. The ring can also be incomplete initially. Kayser-Fleischer rings are present in 95% of Wilson disease cases, including in nearly all cases with neurological manifestations 1,4. The density of the ring correlates well with the activity of the disease 1.
The rings are not pathognomonic for Wilson disease.
Kayser-Fleischer rings are caused by direct copper deposition in the Descemet membrane of the cornea and are thought to be from epithelial cells absorbing copper from the aqueous humor 2.
Treatment and prognosis
After commencement of copper chelating agents (e.g. penicillamine), the size of the rings usually diminish and may eventually vanish altogether 3.
History and etymology
They were initially described by German ophthalmologist Bernhard Kayser (1869-1954) 7 and German physician Bruno Fleischer (1848-1904) 7 in 1902 and 1903 respectively 5,6.
- 1. Sullivan CA, Chopdar A, Shun-Shin GA. Dense Kayser-Fleischer ring in asymptomatic Wilson's disease (hepatolenticular degeneration). (2002) The British journal of ophthalmology. 86 (1): 114. Pubmed
- 2. Tauber J, Steinert RF. Pseudo-Kayser-Fleischer ring of the cornea associated with non-Wilsonian liver disease. A case report and literature review. (1993) Cornea. 12 (1): 74-7. Pubmed
- 3. Schrag, Anette, Schott, Jonathan M.. Kayser–Fleischer Rings in Wilson's Disease. (2012) The New England journal of medicine. 366 (12): e18. doi:10.1056/NEJMicm1101534 - Pubmed
- 4. Lorincz MT. Neurologic Wilson's disease. (2010) Annals of the New York Academy of Sciences. 1184: 173-87. doi:10.1111/j.1749-6632.2009.05109.x - Pubmed
- 5. Kayser B. Über einen Fall von angeborener grünlicher Verfärbung des Cornea. (1902) Klin Monatsbl Augenheilk. 40 (2): 22–25.
- 6. Fleischer B. Zwei weitere Fälle von grünlicher Verfärbung der Kornea.(1903) Klin Monatsbl Augenheilk. 41(1):489–491.
- 7. William Alexander Newman Dorland. Dorland's Illustrated Medical Dictionary. (2018) ISBN: 9781416023647