Imaging Kayser-Fleischer Ring in Wilson Disease Using In Vivo Confocal Microscopy

Cornea. 2019 Mar;38(3):332-337. doi: 10.1097/ICO.0000000000001844.

Abstract

Purpose: This study analyzes images of Kayser-Fleischer (K-F) rings in patients with Wilson disease (WD) using in vivo confocal microscopy (IVCM) and explores whether IVCM can be a useful clinical tool in facilitating the diagnosis and characterization of K-F rings.

Methods: One hundred four eyes of 52 patients with WD and K-F rings (K-F group) and 52 normal eyes of 52 age- and gender-matched control subjects (control group) were enrolled in the study. Both K-F and control groups consisted of 29 male patients and 23 female patients. IVCM imaging was performed, and images of the peripheral Descemet membrane were analyzed.

Results: All patients in K-F group showed abnormal patterns in the peripheral Descemet membrane from IVCM images. These abnormalities can be generally divided into 3 types: patchy, stripy, and spotty patterns. Each patient may have a combination of these patterns, with patchy pattern being most prevalent (100%), whereas stripy and spotty patterns are present in 30% to 40% of the K-F rings. Notably, these patterns are not correlated with other systematic symptoms of WD.

Conclusions: IVCM images can be used as an objective clinical diagnostic tool to facilitate the identification of K-F rings and the diagnosis of WD.

MeSH terms

  • Adolescent
  • Adult
  • Case-Control Studies
  • Child
  • Corneal Diseases / diagnostic imaging*
  • Descemet Membrane / diagnostic imaging*
  • Hepatolenticular Degeneration / pathology*
  • Humans
  • Microscopy, Confocal
  • Young Adult