CHOROIDAL AND RETINA MORPHOMETRIC ANALYSIS IN ANGIOID STREAKS SECONDARY TO PSEUDOXANTHOMA ELASTICUM

Abstract

Purpose: To investigate the choroidal and retinal morphometric characteristics in eyes with angioid streaks secondary to pseudoxanthoma elasticum (PXE).

Methods: Patients with PXE and evidence of angioid streaks were included. A comprehensive ophthalmic evaluation including visual acuity (VA) and axial length (AL) was conducted. In addition, the macular region was scanned with swept source optical coherence tomography. The mean macular choroidal and retinal thickness were calculated. The presence of choroidal neovascularization (CNV) and subretinal fluid (SRF) were also evaluated. All these measurements were compared to an age, gender and AL matched control group.

Results: A total number of 12 eyes of 6 patients (3 men and 3 women, age range: 18-64) were included; 30 eyes of 15 patients (7 men and 8 woman; mean age: 41.47 (p=0.539) were used as control eyes. The mean VA was 0.68 (range: 0,05-1,0). The mean AL was 23.42+/-1.02 mm (control group: 23.36+/-0.93 mm; p=0.999). The mean foveal retinal thickness was 231.42+/-48.44 μm (control group: 238.56+/-15.83 μm; p=0.707) and the mean macular retinal thickness was 268.66+/-17.12 microns (μm) (control group: 281.79+/-12.55 μm; p=0.043). The mean foveal choroidal thickness was 225.75+/-79.94 μm (control group: 330.47+/-78.22 μm; p=0.001) and the mean macular choroidal thickness was 216.63+/-72.07 μm (control group: 309.77+/-69.87 μm; p=0.001). Patients with CNV compared with those without CNV or SRF, showed a significant thinning in both retinal and choroidal thickness when comparing each quadrant of the ETDRS grid (p=0.01), but not affecting the mean macular thickness values (p=0.52). Other intergroup analysis did not show significant differences (CNV versus SRF, and SRF versus without CNV or SRF).

Conclusions: In our study patients with PXE showed a significant thinning in retinal and choroidal mean macular thickness and also in the foveal choroidal thickness, compared with the control group. In addition, a significant decrease in retinal and choroidal thickness was evidenced in patients with CNV compared with patients without SRF or CNV. Choroidal vessels may be affected in patients with PXE, playing an important role in the pathogenesis of CNV and SRF, further studies should be performed to confirm this hypothesis.

Keywords: 452 choroid • 688 retina • 550 imaging/image analysis: clinical