Kayser-Fleischer Ring in Wilson Disease: Utility of Anterior Segment Optical Coherence Tomography

Wilson's disease (WD) is a hepatolenticular degeneration caused by mutations in the ATP7B gene.It is an autosomal recessive disorder resulting in excessive copper deposition, particularly in liver, eyes, and brain.This occurs due to a deficiency of ceruloplasmin, a copper binding protein, often leading to liver cirrhosis.A characteristic ocular sign of Wilson disease is the Kayser-Fleischer (KF) ring, represent copper deposition in Descemet's membrane of the cornea.KF ring is most common in neuro WD, detected in 78-85% of cases.Its prevalence in hepatic WD ranges from 36-62%, while it may be detected in 10% of asymptomatic patients. 1Traditionally KF rings are detected using slit-lamp biomicroscopy.However anterior segment optical coherence tomography (AS-OCT) has emerged as a promising tool, offering a non-invasive, objective, high-resolution cross-sectional imaging tool that can detect subtle KF ring especially in early stage or uncooperative patients challenges with slit lamp examination Copper deposits primarily in the anterior chamber angle at Schwalbe's line within the Descemet's membrane.Early detection with a standard slit-lamp is challenging since the angle view is obscured by the corneal limbus.Copper deposits only become visible after crossing the limbus. role of as-octAS-OCT is a high-resolution non-contact optical imaging technique using infrared light (wavelength 1310 nm) permitting greater penetration, allowing better visualisation of the structures at the angle.The KF ring appears as a distinct hyperreflective band at the level of the Descemet's membrane on AS-OCT.It displays greenish-yellow to brownish coloration on the imaging scale, signifying copper accumulation at the corneal periphery.AS-OCT captures high-resolution, reproducible images without requiring corneal anaesthesia, with each scan taking less than 20 seconds

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Journal
Pediatric Oncall
Published
2025-07-25
DOI
https://doi.org/10.7199/ped.oncall.2026.42
Primary Topic
Trace Elements in Health
Type
article
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article

Kayser-Fleischer Ring in Wilson Disease: Utility of Anterior Segment Optical Coherence Tomography

Ankit Agrawal, Shivani Gupta
Pediatric Oncall
Trace Elements in Health
article

Kayser-Fleischer Ring in Wilson Disease: Utility of Anterior Segment Optical Coherence Tomography

Ankit Agrawal, Shivani Gupta
article en

Abstract

Wilson's disease (WD) is a hepatolenticular degeneration caused by mutations in the ATP7B gene.It is an autosomal recessive disorder resulting in excessive copper deposition, particularly in liver, eyes, and brain.This occurs due to a deficiency of ceruloplasmin, a copper binding protein, often leading to liver cirrhosis.A characteristic ocular sign of Wilson disease is the Kayser-Fleischer (KF) ring, represent copper deposition in Descemet's membrane of the cornea.KF ring is most common in neuro WD, detected in 78-85% of cases.Its prevalence in hepatic WD ranges from 36-62%, while it may be detected in 10% of asymptomatic patients. 1Traditionally KF rings are detected using slit-lamp biomicroscopy.However anterior segment optical coherence tomography (AS-OCT) has emerged as a promising tool, offering a non-invasive, objective, high-resolution cross-sectional imaging tool that can detect subtle KF ring especially in early stage or uncooperative patients challenges with slit lamp examination Copper deposits primarily in the anterior chamber angle at Schwalbe's line within the Descemet's membrane.Early detection with a standard slit-lamp is challenging since the angle view is obscured by the corneal limbus.Copper deposits only become visible after crossing the limbus. role of as-octAS-OCT is a high-resolution non-contact optical imaging technique using infrared light (wavelength 1310 nm) permitting greater penetration, allowing better visualisation of the structures at the angle.The KF ring appears as a distinct hyperreflective band at the level of the Descemet's membrane on AS-OCT.It displays greenish-yellow to brownish coloration on the imaging scale, signifying copper accumulation at the corneal periphery.AS-OCT captures high-resolution, reproducible images without requiring corneal anaesthesia, with each scan taking less than 20 seconds

Pediatric OncallVol. 23(2)
Institute of Child Health (IN)
Openalex Percentile: Top 76%
Trace Elements in Health
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