VISUAL ACUITY IN PSEUDOXANTHOMA ELASTICUM
2018
Purpose: To assess the age-specific proportion of visual impairment in patients with
pseudoxanthoma elasticum (PXE) and to compare this with foveal abnormality and similar
data of late age-related macular degeneration patients.
Methods: Cross-sectional data of 195 patients with PXE were reviewed, including bestcorrected visual acuity and imaging. The World Health Organisation criteria were used to
categorize bilateral visual impairment. These results were compared with similar data of
131 patients with late age-related macular degeneration from the Rotterdam study.
Results: Overall, 50 PXE patients (26.0%) were visually impaired, including 21 (11%) with
legal blindness. Visual functioning declined with increasing age. In patients older than 50
years, 37% was visually impaired and 15% legally blind. Foveal choroidal neovascularization was found in 84% of eyes with a best-corrected visual acuity lower than 20/70 (0.30)
and macular atrophy in the fovea in 16%. In late age-related macular degeneration patients,
40% were visually impaired and 13% legally blind. Visual impairment started approximately
20 years later as compared with PXE patients.
Conclusion: Visual impairment and blindness are frequent in PXE, particularly in patients
older than 50 years. Although choroidal neovascularization is associated with the majority
of vision loss, macular atrophy is also common. The proportion of visual impairment in PXE
is comparable with late age-related macular degeneration but manifests earlier in life.
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