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Br J Ophthalmol 86:114 doi:10.1136/bjo.86.1.114
  • Letter

Dense Kayser-Fleischer ring in asymptomatic Wilson's disease (hepatolenticular degeneration)

  1. Charlotte Anne Sullivan1,
  2. A Chopdar2,
  3. G A Shun-Shin3
  1. 1Department of Ophthalmology, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK
  2. 2Department of Ophthalmology, East Surrey Hospital, Surrey, UK
  3. 3Department of Ophthalmology, Wolverhampton Eye Infirmary, Wolverhampton, West Midlands, UK
  1. Correspondence to: Miss C A Sullivan, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK
  • Accepted 6 June 2001

The Kayser-Fleischer ring is the single most important diagnostic sign in Wilson's disease; it is found in 95% of patients. Virtually all patients with Kayser-Fleischer rings have neurological manifestations.1 Pseudo rings have been described in other conditions.1 The density of a Kayser-Fleischer ring correlates with the severity of Wilson's disease.2 We describe a rare case of a dense Kayser-Fleischer ring in an asymptomatic patient with an extremely high liver copper content. The recent significant genetic advances, and the clinical implications are discussed.

Case report

At the age of 14 this 23 year old white woman had an evisceration of her left eye for painful rubeotic glaucoma following chronic retinal detachment, resulting from ANA positive iridocyclitis. Her optician referred her because over the past few …