Anterior segment optical coherence tomography (AS-OCT) as a new method of detecting copper deposits forming the Kayser-Fleischer ring in patients with Wilson disease

Abstract

Purpose: Kayser-Fleischer ring pathognomonic for Wilson disease (WD) is formed of corneal copper deposits present predominantly within the anterior chamber angle at the Schwalbe's line. The slit-lamp assessment commonly used as a standard of care cannot detect them early enough, as the angle view is obscured by the corneal limbus. The aim of the research was to evaluate the anterior segment optical coherence tomography (AS-OCT), as objective diagnostic assessment of copper deposits forming KF ring in patients with WD.

Methods: Twenty-nine subjects (17 women) and twenty-nine controls (14 women) were enrolled and underwent slit lamp and AS-OCT assessment.

Results: Kayser-Fleischer ring was not detected - either with a slit lamp or with AS-OCT - in any of the controls. Fifteen subjects presenting without KF ring at a slit-lamp examination were found to have a KF ring when examined with AS-OCT (p < 0.001), which confirms improved accuracy of AS-OCT, as compared to the slit-lamp examination.

Conclusion: Our results support AS-OCT as a diagnostic procedure to offer better objectivity and accuracy as compared to a slit-lamp examination, both at the diagnostic stage and when monitoring changes in KF ring during medical therapy to assess its efficacy and patient compliance.

Keywords: Kayser-Fleischer ring; Wilson disease; anterior segment optical coherence tomography; copper deposit.