Wilson Disease Association

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Kayser-Fleischer Rings


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.