Title : Role of anterior segment optical coherence tomography in detecting subtle kayser fleischer ring
Abstract:
Case Description: A 70-year-old female presented to the neurology clinic with complaints of bilateral symmetrical hand tremor since three years and head tremor since six months. The patient had a family history of Wilson disease. She was referred to the ophthalmology clinic to screen for Kayser-Fleischer (KF) ring. On ocular examination, a golden-brown pigmented bands encircling the periphery of the superior cornea was noted which was suggestive of early KF ring (Figure A,B). It was confirmed on ASOCT as hyper-reflective deposits in the Descemet membrane, corresponding to the location of KF rings (Figure C,D).
Discussion: Wilson disease is a rare autosomal recessive disorder characterized by impaired hepatic copper transport, leading to copper accumulation in various organs.1 The diagnostic investigations for wilson’s disease include 24-hour urine copper measurement, ophthalmological slit-lamp examination for Kayser-Fleischer (KF) rings, blood ceruloplasmin levels, serum copper, and liver biopsy with measurement of quantitative copper[2]. As Kayser-Fleischer (KF) rings is an important diagnostic criteria for wilson’s disease, a vigilant slit lamp biomicroscopic examination is mandatory for its detection. But, detection of subtle KF ring on slit lamp examination can be challenging[3], especially for a less experienced ophthalmologist. ASOCT demonstrates hyper-reflective deposits in the Descemet membrane, corresponding to its location[4]. Here, we present a case of Wilson disease who exhibited ocular symptoms and discuss the diagnostic challenges and significance of KF rings. ASOCT can be used as an adjunctive imaging test to confirm the presence of KF ring especially in subtle KF ring, patient not cooperative for gonioscopy examination or less experienced ophthalmologists.
