Choriocapillaris Flow Signal Impairment in Patients with Pseudoxanthoma Elasticum.

PURPOSE. To quantify choriocapillaris flow alterations in patients with pseudoxanthoma elasticum (PXE) in pre-atrophic stages and its association with structural changes of the choroid and outer retina. METHODS. Thirty-two eyes of 21 patients with PXE and 35 healthy eyes of 35 controls were included. The density of choriocapillaris flow signal deficits (FDs) was quantified on 6 x 6-mm optical coherence tomography angiography (OCTA) images. Spectral-domain optical coherence tomography (SD-OCT) images were analyzed for thicknesses of the choroid and outer retinal microstructure and correlated with choriocapillaris FDs in the respective Early Treatment Diabetic Retinopathy Study subfield.RESULTS. The multivariable mixed model analysis for choriocapillaris FDs revealed signifi-cantly higher FDs associated with the group (PXE patients vs. controls +13.6; 95% confi-dence interval [CI] 9.87-17.3; P < 0.001), with increasing age (+0.22% per year; 95% CI 0.12-0.33; P < 0.001), and with retinal location (significantly higher FDs in nasal compared to temporal subfields). Choroidal thickness (CT) did not differ significantly between both groups (P = 0.078). The CT and choriocapillaris FDs were inversely corre-lated (-1.92 & mu;m per %FDs; interquartile range -2.81 to -1.03; P < 0.001). Larger values of the choriocapillaris FDs were associated with significant thinning of the overlying photoreceptor layers (outer segments: -0.21 & mu;m per %FDs, P < 0.001; inner segments: -0.12 & mu;m per %FDs, P = 0.001; outer nuclear layer: -0.72 & mu;m per %FDs; P < 0.001).CONCLUSIONS. Patients with PXE display significant alterations of the choriocapillaris on OCTA even in pre-atrophic stages and in the absence of significant choroidal thinning. The analysis favors choriocapillaris FDs over choroidal thickness as a potential early outcome measure for future interventional trials in PXE. Further, increased FDs in nasal compared to temporal locations mirror the centrifugal spread of Bruch's membrane calci-fication in PXE.