Ophthalmology · Oculoplasty and Orbital Disease (Ptosis, Entropion, Thyroid Eye Disease, Orbital Tumors)

A 60-year-old patient with Wilson's disease is found to have bilateral golden-brown ring-like deposits on the peripheral Descemet's membrane of both corneas, most visible at the 6 and 12 o'clock positions with slit-lamp examination in retroillumination. This sign is:

  • A Kayser-Fleischer ring from copper deposition in Descemet's membrane
  • B Fleischer ring of keratoconus
  • C Hudson-Stahli line from physiological iron deposition
  • D Stocker's line from iron deposition ahead of a pterygium
Correct answer: A. Kayser-Fleischer ring from copper deposition in Descemet's membrane

Explanation

The Kayser-Fleischer (KF) ring is the pathognomonic ophthalmological sign of Wilson's disease (hepatolenticular degeneration); it represents copper deposition within the peripheral Descemet's membrane, appearing as a golden-brown or greenish ring at the corneal periphery. It is most prominent superiorly and inferiorly (where Descemet's is thickest), best seen on slit-lamp examination with gonioscope. KF rings are present in > 95% of patients with neurological Wilson's disease and must be distinguished from other coloured corneal rings. They may reverse with effective copper-chelation therapy (D-penicillamine, trientine).

Reference: Khurana Comprehensive Ophthalmology, 7th ed.

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