Evaluation and grading of Kayser-Fleischer ring in Wilson disease by Scheimpflug camera

Evaluation the anterior segment parameters of Wilson disease patients with Kayser-Fleischer ring, the diagnostic power of Scheimpflug imaging for Kayser-Fleischer ring and suggest a scoring system.

A total of 44 eyes of 22 Wilson disease patients with Kayser-Fleischer ring and 40 right eyes of 40 healthy age matched subjects were enrolled to the study. Serum ceruloplasmin and urine copper/24 hours levels were recorded. Anterior segment parameters including steep and flat keratometry, corneal thickness at central, 2, 4, 6, 8 and 10 mm, anterior chamber angle width, volume and depth, corneal volume, pupillary diameter were evaluated by Scheimpflug imaging. Images of cornea were scored according to Kayser-Fleischer ring size.

Serum ceruloplasmin level was below 10 mg/dL in 17 patients and was 12, 18.5, 20, 22, 37 mg/dl in the remaining five patients. Urinary copper/24 hours was 249.55 ± 304.14 (23-1050) µg/day. Central corneal thickness and corneal thickness at 2 mm were statistically different (p values 0.02, 0.04, respectively). Scheimpflug images apparently showed Kayser-Fleischer ring as a hyper-reflective band at the corneal endothelial surface. Kayser-Fleischer ring in 24 eyes was grade 1, 16 eyes were grade 2 and 4 eyes were grade 3.

Scheimpflug imaging seems to be a helpful diagnostic tool for detecting and grading the Kayser-Fleischer ring. Corneal thickness in Wilson disease patients with Kayser-Fleischer ring tends to be higher, so that the possible affection in corneal thickness should be kept in mind for clinical evaluation of these patients.