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PubMed · 9170798

Understanding angioid streaks.

Abstract

BACKGROUND: Angioid streaks are defined as a series of linear, cracked-line dehiscences of Bruch's membrane, with secondary changes in the retinal pigment epithelium and choriocapillaris. They may be progressive or degenerative, with varied presentation, color, distribution, and retinal involvement. METHODS: The epidemiology, pathophysiology, diagnosis, and management of angioid streaks are described. In addition, the systemic diseases most commonly associated with the disease are reviewed. RESULTS: Optometrists need to be able to differentially diagnose angioid streaks and to refer for evaluation of underlying systemic disease. CONCLUSIONS: Angioid streaks are considered a rare disorder, associated with pseudoxanthoma elasticum, Paget's disease of the bone, sickle hemoglobinopathies. Marfan syndrome, and Ehlers-Danlos syndrome. Ocular complications include subretinal choroidal neovascular membrane formation. Clinicians should be aware of the disease's subtle ocular appearance, its association with systemic diseases, its potential for producing subretinal ocular complications, and correct management protocols and treatments.

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BibTeXRIS

A S Gurwood, D L Mastrangelo. 1997. Understanding angioid streaks.. https://pubmed.ncbi.nlm.nih.gov/9170798/

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Intravascular ultrasound findings of coronary wall morphology in a patient with pseudoxanthoma elasticum.

Pseudoxanthoma elasticum (PXE) is an inherited disorder characterised by progressive calcification of the elastic fibres in the skin, eye, and cardiovascular system. Recently, mutations in the ATP binding cassette transporter gene (ABCC6) were identified as cause of this disease. Although patients with PXE often have coronary artery disease, little is known about the process and the mechanism of coronary artery disease in PXE. In this report, intravascular ultrasound (IVUS) imaging was performed in a female patient with PXE seven years after the onset of skin lesion to assess the coronary wall morphology in detail. IVUS showed a unique five layer appearance without acoustic shadowing along the vessel wall observed in the angiographically normal portion. These findings may reflect the earlier stage of coronary artery disease caused by PXE before calcification of the internal elastic laminae.

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