Optical coherence tomography angiography (OCTA) can reveal microvascular changes with diagnostic utility in the early detection of ocular changes in patients with Wilson disease (WD), according to a study published in Photodiagnosis and Photodynamic Therapy.

The study found that OCTA could detect retinal microvascular changes in patients with WD without clinical evidence of retinal or optic disc involvement. The patients with WD had eyes with significantly lower levels of vascular density (VD) in the inferior perifoveal deep capillary plexus (DCP; P =.041) and inferior radial peripapillary capillary (RPC; P =.043) compared to healthy controls. The inferior peripapillary retinal nerve fiber layer (PPRNFL) was also found to be significantly thinner (P =.045) in patients with WD compared to healthy controls.

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Subdividing the patients with WD into those with Kayser-Fleischer (KF) rings (n=18), and those without (n=17) revealed that the PPRNFL thickness and RPC VD were both significantly lower in patients with KF rings (P =.041 and P =.013, respectively).

“We have shown that there is decreased CMT and inferior PPRNFL thickness in WD patients without significant retinal involvement, which is consistent with the literature, and that vascular density, especially inferior perifoveal DCP, and inferior RPC, is remarkably reduced,” the authors wrote.

While the inferior perifoveal DCP had significantly lower VD, no changes were observed in the VD of the superficial capillary plexus (SCP). The authors hypothesized that the capillaries in the DCP may be more vulnerable to ischemic injury than the SCP due to the DCP having more indirect connection to arterioles that could result in earlier blood flow delays. They also conjectured that decreased VD would be observed in more regions of the eye as disease duration and more severe ocular development occur.

“OCTA parameters may play an important role in the diagnosis and follow-up of retinal findings in WD patients and may provide a stimulus for further investigations into the detection and follow-up of precursors of neurodegeneration,” the authors wrote.

A total of 35 patients with WD and 36 healthy controls were recruited for the study. A single eye in each participant was observed using OCTA. The mean time since diagnosis for the patients with WD was 6.4 ± 3.7 years.


Seyyar SA, Kanat E, Özcan ZÖ, et al. Vascular changes in macula, optic disc, and choroid in Wilson’s disease: a cross-sectional optical coherence tomography angiography study. Photodiagnosis Photodyn Ther. Published online April 5, 2023:103549. doi:10.1016/j.pdpdt.2023.103549