Related Experiment Video
Updated: Jul 9, 2026
06:15
Anterior High-Resolution Optical Coherence Tomography in the Diagnosis and Therapeutic Monitoring of Ocular Surface Squamous Neoplasia
Published on: August 9, 2024
Non-vascular vision loss in pseudoxanthoma elasticum
Irena Tsui1, Brian S Fuchs, Chai Lin Chou
1Department of Ophthalmology, Edward Harkness Eye Institute, 635 West 165th Street, New York, NY 10032, USA. irena.tsui@gmail.com
Documenta Ophthalmologica. Advances in Ophthalmology
|November 24, 2007
Summary
Pseudoxanthoma elasticum can cause chronic vision loss from macular atrophy, not just acute bleeding. Autofluorescence and pattern electroretinogram aided diagnosis in a patient with retinal pigment epithelium dysfunction.
Area of Science:
- Ophthalmology
- Medical Genetics
- Retinal Diseases
Background:
- Pseudoxanthoma elasticum (PXE) is a rare genetic disorder affecting elastic tissue.
- Angioid streaks are a common ocular manifestation of PXE, often leading to acute vision loss via choroidal neovascularization and hemorrhage.
- Chronic vision loss due to macular atrophy in PXE is less understood.
Observation:
- A case of sub-acute vision loss in one eye is presented.
- The patient's vision loss was attributed to impaired macular retinal pigment epithelium (RPE) function.
- Diagnostic imaging and functional tests were employed to investigate the cause.
Findings:
- Autofluorescence imaging revealed abnormalities consistent with RPE dysfunction.
- Pattern electroretinogram (PERG) demonstrated reduced macular function.
- These findings helped pinpoint the source of vision loss in the macula.
Implications:
- This case highlights macular atrophy as a significant cause of vision loss in PXE.
- Autofluorescence and PERG are valuable tools for diagnosing RPE dysfunction in PXE patients.
- Further research into chronic macular changes in PXE is warranted to improve patient management.
Related Concept Videos
01:22Photoreceptors and Visual Pathways
At the molecular level, visual signals trigger transformations in photopigment molecules, resulting in changes in the photoreceptor cell's membrane potential. The photon's energy level is denoted by its wavelength, with each specific wavelength of visible light associated with a distinct color. The spectral range of visible light, classified as electromagnetic radiation, spans from 380 to 720 nm. Electromagnetic radiation wavelengths exceeding 720 nm fall under the infrared category, whereas...
01:27Diabetic Retinopathy
DefinitionDiabetic retinopathy is a microvascular complication of diabetes affecting the retinal blood vessels.Risk FactorsDiabetic retinopathy is present in almost all individuals with type 1 diabetes and more than 60% of those with type 2 diabetes after two decades of disease.The risk increases with poor glycemic control, hypertension, dyslipidemia, smoking, pregnancy, and puberty.Although cataracts and glaucoma are also more frequent in people with diabetes, retinopathy remains the leading...
01:25Glaucoma: Overview
Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...