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Everything you need to know about Wilson Disease

kayser fleischer ring2019

Definition

Kayser-Fleischer Ring: Clinical sign. Brownish-yellow ring visible around the corneo-scleral junction (limbus). Consists of copper deposits in Descemet’s membrane, extending into the trabecular meshwork. Sign of Wilson’s disease. (Dictionary of Eye Terminology, 3rd edition; Barbara Cassin, Sheila A.B. Solomon)

Description and Location

Golden to greenish-brown annular deposition of copper located in the periphery (limbus) of the cornea (Descemet’s membrane). First appears as a superior crescent, then develops inferiorly and ultimately becomes circumferential. Usually requires a slit-lamp examination to detect rings in their early stage of formation.

Prevalence

Approximately 95% of WD patients presenting with neurological signs will have a K-F ring. Where as approximately 65% of WD patients presenting with hepatic signs will present with a ring.

Copper chelation therapy may cause fading and even disappearance of the corneal copper over time.

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