Cornea in Wilson Disease: Kayser Fleischer Ring and Beyond
Keywords:
Kayser-Fleischer ring, Wilson disease, slit lamp biomicroscopy, optical coherence tomography, copper, cornea
Abstract
The identification of Kayser-Fleischer (KF) ring remains the most important clinical sign for the diagnosis of Wilson disease (WD). Slit lamp biomicroscope (SL) examination by a skilled observer is still the preferred method of evaluating KF ring. Anterior segment optical coherence tomography (AS-OCT) is an alternative method of evaluating KF ring in WD. Hyper-reflective layer in the corneal periphery at the level of Descemet membrane (DM) is the characteristic appearance of KF ring on AS-OCT. In a suspected case of WD, features of KF ring on AS-OCT may alert the clinician to do a careful SL examination to look for early KF ring. AS-OCT has been found useful in measuring the length of KF ring with ease even in patients with severe rigidity and children. AS-OCT can measure the length of KF ring better compared to SL in patients with limbal pathology. Early detection of increased corneal copper even before KF rings appear is a potential area for future research.
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2017-08-28
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