Health & Environmental Research Online (HERO)


Print Feedback Export to File
6858654 
Journal Article 
The extent of angioid streaks correlates with macular degeneration in pseudoxanthoma elasticum 
Risseeuw, S; Ossewaarde-Van Norel, J; van Buchem, C; Spiering, W; Imhof, SM; van Leeuwen, R 
2020 
Yes 
American Journal of Ophthalmology
ISSN: 0002-9394
EISSN: 1879-1891 
Elsevier Inc. 
220 
82-90 
English 
32702361 
WOS:000599610200021 
PURPOSE: To investigate whether the extent of Bruch's membrane (BM) calcification is associated with choroidal neovascularization (CNV) and macular atrophy in patients with pseudoxanthoma elasticum (PXE) by using the extent of angioid streaks as a surrogate marker for the degree of BM calcification. Design; Retrospective cross-sectional study.

METHODS: We investigated 301 PXE patients (median age 52 years, range 9-79) in a tertiary referral center. For both eyes, we graded the extent of angioid streaks, i.e. their distance from the optic disk, into five groups. Imaging was systematically assessed for signs of CNV and macular atrophy. Associations between extent of angioid streaks and CNV or macular atrophy were investigated using regression analysis.

RESULTS: CNV was present in 148 patients (49%) and retinal atrophy in 71 patients (24%). The extent of angioid streaks was associated with older age (P for trend = 1.92×10-15) and a higher prevalence of CNV and/or macular atrophy (P for trend = 4.22×10-10 and P for trend = 5.17×10-6, respectively). In addition, the extent of angioid streaks was associated with the presence of CNV when adjusted for age and sex (odds ratio (OR) 1.9, 95% CI 1.3 - 2.9) and with more severe macular atrophy (proportional OR 2.3, 95% CI 1.5 - 3.6).

CONCLUSIONS: In PXE patients, longer angioid streaks are associated with an increased risk of CNV and macular atrophy, even after adjustment for age. These findings are relevant when counseling PXE patients on their visual prognosis.