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

Abstract

Purpose: 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. Materials and Methods: 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. Results: 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. Conclusion: 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.

Decoding Wilson disease: a machine learning approach to predict neurological symptoms

“…Moreover, it was found that the WD-N group had a higher incidence of the K-F ring than the no-WD-N group. These results are consistent with previous research findings (16,(32)(33)(34). Furthermore, the WD-N group had significantly higher psychiatric symptom scores and liver symptom scores than the no-WD-N group.…”

A grading method for Kayser Fleischer ring images based on ResNet

“…[ 34 ] and Degirmenci, C, etc. [ 11 ], including the scope and Scheimpflug imaging. In this research, K–F ring were graded in five grades, of which is grade 0 to 4.…”

“…The corneal K–F (Kayser Fleischer) ring is the most common ophthalmic manifestation of WD patients. K–F ring usually exists in 90.4%–100% of WD patients with neuropsychiatric symptoms, and 50–60% of WD patients without neurological symptoms, but only in 10%–40% of asymptomatic WD patients [ [10] , [11] , [12] , [13] ]. The K–F ring is named after Kayser (1902) and Fleischer (1903) respectively.…”

“…First, this paper collected and sorted out 1850 images of 399 WD patients in the hospital from January 2018 to June 2022 through slit lamp corneal K–F ring examination. At the same time, the images were quantified with different grades according to Wang Gongqiang et al [ 34 ] and Degirmenci, C et al [ 11 ]. Second, this paper statistically analyzed the relevant attribute information of the patient's K–F ring image and established a useable corneal K–F ring images database (KFID) after analysis.…”

Eye Involvement in Wilson’s Disease: A Review of the Literature

“…Follow-up and multiple examinations of 12 patients of the same cohort suggested that this method may be used to follow-up the patients as a marker of whole-body copper reduction under treatment. In a recent study by Degirmenci and Palamar, Scheimpflug image was used to diagnose and make a grading of KFR in 22 WD patients compared to controls [38]. KFR was observed at the periphery of the cornea as a hyper-reflective area, the extent of which was concordant with KFR severity on SLE.…”