Change Language German

Pathogenesis

• Deficiency of ceruloplasmin -> extensive copper deposits in tissues

Findings

• Kayser Fleischer ring ¹ = Zone of copper granules in the peripheral part of the Descemet membrane
  • Initially visible only superiorly (gonioscopy)
• Always perform gonioscopy; discrete manifestation initially visible in the anterior chamber angle ²
• Sunflower-Cataract ³ : Anterior and posterior subcapsular copper deposits.

Work-up

• Determine serum copper, ceruloplasmin levels, and urine copper levels
• Refer to internist/neurologist for therapy
• Follow-up
  • Successful therapy results in regression of Kayser-Fleischer Ring.
  • Progression over time is an indication of non-compliance

Sources

• EyeWiki Wilson’s Disease / Kayser Fleischer ring
• The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
• Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)
• ^{1 , 2, 3} von Eyerounds.org, © The University of Iowa; Licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License
  • ^{1 , 2, 3} Contributor: Thomas J.E. Clark, MD; Photographer: Brice Critser