PubMed · 5919172
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A Hagedoorn. 1966. Dystrophia phakofibroelastica.. https://doi.org/10.1159/000304929
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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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Author observed two patients affected by Pseudoxanthoma elasticum with different extent of alternation on the eye background. The separate states of progression of this eye disease are observed by fluorescein angiography. Beside the finding of angioid streaks fluorescein angiography more precisely demonstrates extent of Bruch membrane abnormalities and alteration of pigmentary epithelium of the retina. This way fluorescein angiography significantly contributes to clarification of other accompanying pathology of this eye disease.