PubMed · 5848398
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A Hagedoorn. 1965-09-11. [Phacofibroelastic dystrophy].. https://pubmed.ncbi.nlm.nih.gov/5848398/
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BACKGROUND: Optic disc drusen accompanied by angioid streaks is rarely seen in Japan. Laser photocoagulation for choroidal neovascularization (CNV) in patients with angioid streaks is controversial. CASE: A 45-year-old woman presented with bilateral papilledema. Clinical examination revealed optic disc drusen and angioid streaks. During the follow-up period, juxtapapillary CNV developed in both eyes. CNV in the right eye developed progressively towards the fovea and was eventually treated by laser, but the CNV in the left eye regressed spontaneously. CONCLUSION: These are the first precise clinical records on optic disc drusen with angioid streaks to be reported. The outcome of laser treatment for CNV in this case was favorable, because alteration of Bruch's membrane was mild, in the form of angioid streaks.
In a 48-year-old man who suffered from progressive loss of vision in his left eye and metamorphopsia, pseudoxanthoma elasticum was diagnosed. Angioid streaks were observed in both eyes and the skin showed small yellow papules in a linear pattern. The right retina was twice treated by laser coagulation which resulted in temporary regression of neovascularization. Pseudoxanthoma elasticum is a genetic disorder with variable penetrance. There is no treatment. Patients should be told to refrain from contact sports and to avoid using aspirin.
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.