Unilateral Kayser-Fleischer ring.

Abstract

SUMMARY A patient is presented who had unrecognised Wilson's disease. He had developed a clinically obvious Kayser-Fleischer ring in only one eye. The eye without the corneal ring had been injured in childhood and had a low intraocular pressure. Possible mechanisms for formation of a Kayser-Fleischer ring are reviewed and the lack of Kayser-Fleischer ring in this case is discussed. Case historyThe patient first noticed a slight tremor of his hand and slight unsteadiness of gait at the age of 35. These symptoms progressed very slowly, and he was first seen by a neurologist at the age of 52. Signs present at that time were unsteadiness, cerebellar dysarthria, and ataxia of the hands, the right more than the left. The right eye was blind and divergent, the result of a penetrating injury by a dart at the age of 7.His past medical history revealed an episode of jaundice as a teenager and a urinary infection at the age of 40. Relevant family history was that his identical twin brother had died of hepatitis at the age of 47. He had also suffered from ataxia of a similar severity.Heredofamilial ataxia was diagnosed.

Wilson's Disease

“…68,69 Kayser-Fleischer rings are almost always bilateral, but unilateral formation has been reported. 70 The color of the rings can range from gold to brown to green; consequently, they can be difficult to see in individuals with brown irises. Ring formation first becomes visible in the superior aspect of the cornea, followed by the inferior aspect, with subsequent filling in of the medial and lateral aspects.…”

Study of Corneal Copper Deposits in Wilson's Disease by in vivo Confocal Microscopy

“…It has previously been observed that the CBG has a pattern of distribution in the four quadrants that follows the pathophysiological process of copper deposition on Descemet's membrane [6,8,9]. Therefore, in case of doubt or borderline corneal appearance on traditional biomicroscopy, the technique proposed in this study may be helpful to establish the presence of corneal copper deposition, in particular if it is applied to the study of the upper peripheral cornea, where the process of deposition starts.…”

“…In cases of initial deposition, in which ring formation is not clearly visible, the determination of the presence of copper deposits can be doubtful and operator-dependent [6], despite the use of Goldmann's three-mirror contact lens, which facilitates the visualization of Descemet's membrane in the peripheral cornea. This issue is relevant because early identification of KF rings in the presymptomatic phase allows early treatment of WD [6,8,9]. …”

“…Progression of corneal copper deposits is from superior to inferior and finally laterally [6,8,9]. In cases of initial deposition, in which ring formation is not clearly visible, the determination of the presence of copper deposits can be doubtful and operator-dependent [6], despite the use of Goldmann's three-mirror contact lens, which facilitates the visualization of Descemet's membrane in the peripheral cornea.…”

Anéis de Kayser-Fleischer

“…Os anéis são, caracteristicamente, mais pronunciados na periferia, ou seja, na região perilímbica e tendem a se tornar menos evidentes à medida que se aproximam da região central da córnea. São geralmente bilaterais e simétricos, mas existem relatos de anéis unilaterais (2)(3) . Estudos utilizando microscopia eletrônica e histoquímica (4) mostraram que os anéis de K-F são compostos por densas e não uniformes camadas contendo granulações de cobre com tamanhos variados, separadas por (5) .…”