Impact Of Oct Diagnostics In Wilson-Konovalov Disease. Correlation Between Ocular Changes And Severity Of The Disease

Abstract

Wilson disease is a genetically transmitted disease with severe hepatic, neurological and psychiatric findings. Purpose: The aim of our survey is to show a new method for objective evaluation of the Keiser-Fleisher Ring in the cornea in patients with Wilson-Konovalov disease. Material and method: In our prospective study 7 patients with Wilson disease and decreased levels of ceruloplasmine under 5.2 g/l were enrolled. They were divided into two groups – with initial finding of the disease – 3 patients and with developed clinical picture – 4 patients. Two patients were after successful liver transplantation. The Keiser-Fleisher ring has been evaluated biomicroscopically, with color photography and with anterior segment ОСТ (Rtvue, Optovue, 3D OCT 2000, Topcone). Results: In the first group of patients with initial stage of the disease on biomicroscopy a slight yellow orange ring in the corneal periphery has been spotted in some of the cases. The AS OCT showed a well outlined hyper-reflective band on the level of the Descemet membrane corresponding to the cooper accumulation in the cornea. In the second group of patients with developed disease, this hyper reflective band was larger with more than 60 mc in comparison to the previous group. In patients after liver transplantation disappearance of the ring and cooper accumulation was observed. The ring vanished form the periphery to the center. Conclusion: Wilson disease is always suspected in cases of undiagnosed liver dysfunction. Its confirmation however, is in many cases subjective and connected with the detection of Keiser-Fleisher Ring. The proposed method with anterior segment OCT is noninvasive, repeatable and can objectively show the presence or absence of cooper depositions in the cornea. OCT diagnostics might be useful also in the follow up of these patients after liver transplantation.