Sneha Das Gupta1, Ankit Agrawal2, Ankita Aishwarya Agrawal3.
1Department of Pediatric, Institute of Child Health, Kolkata, India,
2Department of Pediatric Gastroenterology, Post Graduate Institute of Child Health, Noida, Uttar Pradesh, India,
3Department of Ophthalmology, Post Graduate Institute of Child Health, Noida, Uttar Pradesh, India.
ADDRESS FOR CORRESPONDENCE
Ankit Agrawal, Post Graduate Institute of Child Health, Sector-30, Noida, Uttar Pradesh, India
Email: ankitagrawal456798@gmail.com | | Keywords | | Wilson, Kf ring, AS-OCT | | | 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, which represent copper deposition in Descemet’s membrane of the cornea.
KF rings are most commonly seen in neurological WD, detected in 78–85% of cases. Their prevalence in hepatic WD ranges from 36-62%, while they detected in 10% of asymptomatic patients.1 Traditionally, KF rings are detected using slit-lamp biomicroscopy. However anterior segment optical coherence tomography (ASOCT) has emerged as a promising tool, offering noninvasive, objective, high-resolution, cross-sectional imaging that can detect subtle KF rings, especially in early stages or in uncooperative patients.
Challenges with Slit-Lamp Examination
Copper deposits occur primarily in the anterior chamber angle at Schwalbe’s line within the Descemet’s membrane. Early detection using a standard slit-lamp is challenging, as the angle is obscured by the corneal limbus. copper deposits only become visible after crossing the limbus.
Role of AS-OCT
AS-OCT is a high-resolution non-contact optical imaging technique using infrared light (wavelength 1310 nm) permitting greater penetration and better visualization 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. On imaging, 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 per eye. This makes it particularly advantageous for patients with neurological symptoms such as tremors or dystonia or in early stage, enabling early diagnosis and treatment before significant copper deposition occurs.2
Clinical Comparison of AS-OCT and Slit-Lamp Examination
• Several studies support the higher sensitivity of AS-OCT:
• Sridhar et al. (2017) reported 100% detection by both methods in a small cohort, but AS-OCT provided clearer demarcation and quantification.3
• Broniek-Kowalik et al. (2019) demonstrated AS-OCT detected KF rings in 86% of WD cases versus 58% by slit-lamp.4
• Ormeci et al. (2022) showed that AS-OCT identified rings in a significantly higher proportion of patients across all clinical subtypes, especially in hepatic forms where slit-lamp detection was <10%.5
These findings underscore AS-OCT role in identifying subtle rings and its utility in difficult-to-examine populations.
| | | | Conclusion | AS-OCT is a promising diagnostic and monitoring tool for Kayser-Fleischer rings in Wilson disease. Its advantages non-invasiveness, rapid imaging, high sensitivity and objective quantification—make it particularly useful in pediatric patients and those with limited cooperation. It complements slit-lamp examination, especially when initial findings are inconclusive or minimal. Incorporating AS-OCT into routine diagnostic protocols may enable earlier detection and treatment of Wilson disease.
Key message
• AS-OCT is a highly sensitive, non-invasive tool for detecting KF rings.
• Particularly useful in children and patients with
neurological impairment.
• AS-OCT provides objective documentation and follow-up, unlike the subjective slit-lamp exam. | | | | Compliance with Ethical Standards | | Funding None | | | | Conflict of Interest None | | |
- Medici V, Trevisan CP, D'Incà R, Barollo M, Zancan L, Fagiuoli S, et al. Diagnosis and management of Wilson's disease: results of a single center experience. J Clin Gastroenterol 2006;40(10):936-41. [CrossRef] [PubMed]
- Izatt JA, Hee MR, Swanson EA, Lin CP, Huang D, Schuman JS, et al. Micrometer-scale resolution imaging of the anterior eye in vivo with optical coherence tomography. Arch Ophthalmol 1994;112(12):1584-9. [CrossRef] [PubMed]
- Sridhar MS. Advantages of Anterior Segment Optical Coherence Tomography Evaluation of the Kayser-Fleischer Ring in Wilson Disease. Cornea 2017;36(3):343-6. [CrossRef] [PubMed]
- Broniek-Kowalik K et al. AS-OCT as a method for detecting KF ring. Acta Ophthalmol. 2019;97:e757-60. [CrossRef] [PubMed]
- Örmeci AÇ, Bayraktar Ş, Işık AO, Çavuş B, Akas R, İstemihan Z, et al. Is anterior segment OCT superior to slit-lamp biomicroscopic examination for Kayser Flesicher ring in Wilson's disease? Eur Rev Med Pharmacol Sci 2022;26(14):5178-85.
DOI: https://doi.org/10.7199/ped.oncall.2026.42
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| Cite this article as: | | Gupta S D, Agrawal A, Agrawal A A. Kayser-Fleischer Ring in Wilson Disease: Utility of Anterior Segment Optical Coherence Tomography. Pediatr Oncall J. 2025 Jul 11. doi: 10.7199/ped.oncall.2026.42 |
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