Background and

Objective: To report the association of angioid streaks in patients with Pseudoxanthoma elasticum (PXE) with reticular pseudodrusen (RPD), thin choroid, and retinal pigment epithelium (RPE) atrophy using swept-source optical coherence tomography (SS-OCT) and short-wave autofluorescence (SWAF). Patients and

Methods: Retrospective cross-sectional study. Records of consecutive patients with angioid streaks due to PXE, who presented with a decrease of vision due to choroidal neovascularization (CNV), were reviewed for best-corrected visual acuity, color fundus photographs, SS-OCT, SWAF, and red-free images with special emphasis on presence or absence of RPD, subfoveal choroidal thickness (SFCT), and RPE atrophy.

Results: Sixteen eyes of eight patients with a mean age of 45.5 years ± 9.4 years were enrolled in the study. RPD were seen in 10 of the 16 eyes and were seen commonly along the superotemporal quadrant. Mean subfoveal thickness in study eyes (175.7 μm ± 37.2 μm) was significantly reduced when compared to controls (286.4 μm ± 40.8 μm). The mean SFCT was similar between the eyes with and without CNV. Four eyes had RPE atrophy in the macular area, whereas four eyes had peripapillary RPE atrophy.

Conclusions: Angioid streaks in PXE are associated with RPD, thin choroid, and RPE atrophy. These features occur at a younger age as compared to age-related macular degeneration and appear to be interrelated because of single pathophysiological mechanism. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:402-408.].