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This retrospective cross-sectional study investigated 301 patients with pseudoxanthoma elasticum (PXE) for prevalence of choroidal neovascularisation (CNV) and macular atrophy. Some patients were excluded due to missing or poor-quality imaging or the fact that they didn’t have angioid streaks, leaving 288 patients in which at least one eye could be assessed. They found that CNV was present in 148 patients (49%) and retinal atrophy in 71 patients (24%). They concluded that greater extent of the angioid streaks was associated with a higher rate of CNV and / or macular atrophy in addition to increasing age. They also reported that males with PXF were at a higher risk of CNV than women with the condition. However, it isn’t clear whether other unknown factors such as cardiovascular risk factors and smoking are confounding factors to the higher incidence in males. This is a large study for a rare disease and can be useful when counselling patients with PXE on their visual prognosis.

The extent of angioid streaks correlates with macular degeneration in pseudoxanthoma elasticum.
Risseeuw S, Norel J, Buchem C, et al.
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Tasmin Berman

University of Liverpool, Liverpool, UK.

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