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Case Reports
. 2022 Feb 18;35(2):146-148.
doi: 10.4103/1319-4534.337854. eCollection 2021 Apr-Jun.

Fading Kayser-Fleischer ring revisited

Maansi Sethi  1 , Siddharth Madan  1 , Sarita Beri  1
Affiliations
Case Reports

Fading Kayser-Fleischer ring revisited

Maansi Sethi et al. Saudi J Ophthalmol. .

Abstract

Kayser-Fleischer (KF) ring, caused by deposition of excess copper in the Descemet membrane, is a characteristic ocular manifestation of Wilson disease (WD). Disappearance of KF rings following successful treatment of Wilson disease is typically a slow process that occurs over years. Herein, we describe a 19-year old girl who presented with neuropsychiatric manifestations and was found to have KF rings on slit lamp examination. Subsequent evaluation (brain imaging, liver function tests, serum ceruloplasmin and urinary copper studies) confirmed a diagnosis of Wilson disease with neurological and hepatic involvement. She was treated with d-penicillamine. She had remarkable fading of KF rings within a span of 6 months of copper-chelating therapy, which was also associated with significant improvement in her neurological symptoms. Though KF rings are a harbinger of neurological Wilson's, their disappearance does not always correlate with systemic improvement - an interesting finding in this case.

Keywords: KF ring; Wilson disease.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Slit lamp photograph: Right eye- Direct illumination of Cornea showing 360° Kayser Fleisher ring (white arrow) (b) Slit lamp photograph: Left eye-Direct illumination of Cornea showing 360° Kayser Fleisher ring (white arrow)
Figure 2
Figure 2
(a) Slit lamp photograph: Right eye- Optical section of Cornea showing copper deposition at the level of Descemet's membrane (blue arrow) (b) Slit lamp photograph: Left eye-Optical section of Cornea showing copper deposition at the level of Descemet's membrane (blue arrow)
Figure 3
Figure 3
Magnetic resonance imaging of the patient's brain: Symmetrical T2/ FLAIR hyperintensities involving midbrain especially posterior pontine region (green arrow)
Figure 4
Figure 4
Magnetic resonance imaging of the patient's brain revealed nearly symmetrical T2/ FLAIR hyperintensities involving bilateral basal ganglia and thalami (red arrow)
Figure 5
Figure 5
Slit lamp photograph: Both eyes- Fading of Kayser Fleisher rings pigmentation (yellow arrow)
Figure 6
Figure 6
Slit lamp photograph: Both eyes- Optical section of Corneas showing reduction in copper deposition at the level of Descemet's membrane (yellow arrow)
See this image and copyright information in PMC

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