Pathophysiology of ocular manifestations in Wilson’s disease and its management

Figure 1 Kayser-Fleischer ring progression in Wilson’s disease. A: Early Kayser-Fleischer (KF) ring appearing as a superior peripheral corneal arc on slit-lamp examination; B: Inferior corneal involvement due to progressive copper deposition; C: Complete circumferential KF ring at the corneal periphery.

Figure 2 Imaging modalities used for detection of Kayser-Fleischer ring in Wilson’s disease. A: Slit-lamp photograph demonstrating complete circumferential Kayser-Fleischer (KF) ring pigmentation at the limbus; B: Scheimpflug imaging showing increased peripheral corneal density corresponding to copper deposition; C: Anterior segment optical coherence tomography (AS-OCT) demonstrating a peripheral hyper-reflective band at the level of Descemet’s membrane consistent with KF ring in right eye; D: AS-OCT image illustrating circumferential hyper-reflective deposition along Descemet’s membrane, corresponding to corneal copper accumulation in left eye. OCT: Optical coherence tomography; ART: Ambrosio’s relational thickness; OS: Oculus sinister; OD: Oculus dexter; IR: Infrared; HS: High speed.

Figure 3 Diagnostic algorithm for detection and monitoring of Kayser-Fleischer ring. ring in suspected Wilson’s disease. AS-OCT: Anterior segment optical coherence tomography; KF: Kayser-Fleischer; IVCM: In vivo confocal microscopy; WD: Wilson’s disease.