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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)
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