Study of Retinal Vascular Parameters in Patients With Wilson's Disease

Study of Retinal Vascular Parameters in Patients With Wilson's Disease

The rare disease reference center " Wilson disease and other rare copper-related diseases" of the Rothschild Foundation follows a large number of patients with Wilson's with varying degrees of impairment and located at different times of their care.

Many people with Wilson's disease have a characteristic greenish-brown ring, known as Kayser-Fleischer, appearing at the periphery of the cornea due to a deposit of copper at the Descemet membrane. As a general rule, if the patient is compliant with his treatment, the ring usually disappears within a few years, although it may persist in some patients. However, apart from the stage of diagnosis, and the evolution of the ring, ophthalmological examinations are little used for the follow-up of these patients.

The objective of this study is to describe the retinal parameters, in particular vascular with two new retinal imaging technologies (OCT-A :Optical Coherence Tomography-Angiography , Adaptive optics) in patients with Wilson's disease and to correlate them with the parameters of the usual follow-up of these patients (hepatic assessment, exchangeable plasma copper, neurological scores, compliance, etc.).

Inclusion Criteria:

• Wilson's disease with Ferenci score ≥4

Exclusion Criteria:

• Neurological or behavioral handicap that does not allow for ophthalmological examinations
• Hepatic decompensation that does not allow for ophthalmological examinations
• Pregnant or breastfeeding woman