Abstract
IntroductionPseudoxanthoma elasticum (PXE) typically involves elastic fibers in blood vessels and Bruch membrane. Our purpose was to analyze retinal and choroidal macular thickness in patients with angioid streaks due PXE compared with a control group.MethodsBest-corrected visual acuity (BCVA), axial length (AL), and macular swept-source optical coherence tomography were obtained. Automated segmentations of the retina and the choroid were used to obtain the corresponding thickness values. An age, gender and AL matched control group was used to compare the thickness values.ResultsTwelve eyes of 6 patients were included. The mean BCVA was 0.68 ± 0.29 versus 1.0 in controls (p < 0.001). The mean macular retinal thickness was thinner in eyes with PXE (p = 0.038). Only patients with choroidal neovascularization (NV) showed statistically significant differences in the mean macular choroidal thickness (p = 0.008).ConclusionsThe present study shows that choroidal thickness may be thinner in eyes with NV due to angioid streaks in PXE compared with healthy eyes analyzed by an automated segmentation of the choroid. Further studies are warranted in order to assess the importance of this choroidal changes in the pathogenesis of retinal disturbances related to PXE and its influence in long-term follow-up.
Highlights
Pseudoxanthoma elasticum (PXE) typically involves elastic fibers in blood vessels and Bruch mem‐ brane
Pseudoxanthoma elasticum (PXE) is a systemic disease characterized by progressive fragmentation and calcification of the elastic fibers in connective tissues related to a mutation in the ABCC6 gene, resulting in a spectrum of pathologic changes mainly involving the dermis, the blood vessels, and the Bruch membrane [1, 2]
Twelve eyes of 6 patients diagnosed with angioid streaks related to PXE (3 men and 3 women) with a mean age of 40.66 ± 16.25 years were included in the present study
Summary
Pseudoxanthoma elasticum (PXE) typically involves elastic fibers in blood vessels and Bruch mem‐ brane. Our purpose was to analyze retinal and choroidal macular thickness in patients with angioid streaks due PXE compared with a control group. Pseudoxanthoma elasticum (PXE) is a systemic disease characterized by progressive fragmentation and calcification of the elastic fibers in connective tissues related to a mutation in the ABCC6 gene, resulting in a spectrum of pathologic changes mainly involving the dermis, the blood vessels, and the Bruch membrane [1, 2]. In the present study the retinal and choroidal thickness of the macular region in eyes with angioid streaks secondary to PXE were analyzed and compared with control eyes. The use of an automated segmentation of the choroid provides an easier and accurate analysis of the choroidal thickness compared with previous reports
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
