What are Kayser-Fleischer rings in Wilson disease?

Updated: Feb 14, 2019
  • Author: Richard K Gilroy, MBBS, FRACP; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Kayser-Fleischer rings

  • Formed by the deposition of copper in the Descemet membrane in the limbus of the cornea

  • The color may range from greenish gold to brown

  • Well-developed rings may be readily visible to the naked eye or with an ophthalmoscope set at +40

  • When not visible to the unaided eye, the rings may be identified using slit-lamp examination or gonioscopy

  • Observed in up to 90% of individuals with symptomatic Wilson disease and almost invariably present in those with neurologic manifestations

  • No longer considered pathognomonic of Wilson disease unless accompanied by neurologic manifestations, as they may also be observed in patients with chronic cholestatic disorders


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