Kayser-Fleischer Ring Detection and Quantification in Wilson's disease using Optical Coherence Tomography

Delays in the diagnosis of Wilson's Disease (WD) arising from the challenging heterogeneity of presentation, lead to avoidable morbidity and mortality. Clinically a Kayser-Fleischer (KF) ring is useful in diagnosis, reported as being present in nearly all neurological WD, half of hepatic WD and less than half of presymptomatic WD. Most KF rings regress with medical therapy or liver transplantion, however, may return when treatment ceases. Lifelong monitoring of serum and urine is cumbersome for the patient and a reliable non-invasive disease activity score is sought. The aim of the study was to establish the feasibility of quantitative analysis of KF rings in established WD using non-invasive anterior segment optical coherence tomography (AS-OCT).