Cases reported "Angioid Streaks"

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1/12. Macular translocation for subfoveal choroidal neovascularization in angioid streaks.

    PURPOSE: To report a case of visual improvement after macular translocation performed for a subfoveal choroidal neovascular membrane in a patient with pseudoxanthoma elasticum and angioid streaks. methods: The fovea was translocated inferiorly by scleral imbrication, intentional retinal detachment with a small posterior retinotomy, and partial fluid-air exchange. The choroidal neovascular membrane was photocoagulated 1 week later. RESULTS: The visual acuity of the patient improved from 20/125 to 20/40. The center of the foveal avascular zone was moved inferiorly 844 microm. The choroidal neovascular membrane was extrafoveal after translocation and was treated with laser photocoagulation. CONCLUSION: Macular translocation may be considered in the management of subfoveal choroidal neovascular membrane in patients with pseudoxanthoma elasticum and angioid streaks.
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2/12. Bruch's membrane in pseudoxanthoma elasticum. Histochemical, ultrastructural, and x-ray microanalytical study of the membrane and angioid streak areas.

    In a case of pseudoxanthoma elasticum (PXE), Bruch's membrane was studied histochemically, ultrastructurally and by element analysis. Two kinds of calcifications were observed, one composed of hydroxyapatite and the other of CaHPO4. They were seen in the scar tissue of angioid streak areas and in the membrane outside these areas, respectively. Furthermore, a thready material was found in the membrane as well as an increased amount of acid mucopolysaccharides. The genesis of the calcifications and the pathogenesis of angioid streaks are discussed. The author is in favor of a malformed collagen as the basic defect in PXE.
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3/12. Juxtapapillary choroidal neovascular membrane in a patient with Paget's disease and lattice corneal dystrophy.

    Paget's disease (osteitis deformans) is a relatively common metabolic bone disease characterized by the gradual development of bony deformities. These deformities lead to cranial and peripheral neuropathies from nerve and blood vessel impingement. angioid streaks are associated with Paget's disease and are the result of linear breaks in Bruch's membrane caused by the calcification and degeneration of this elastic tissue layer. A case report of a patient with Paget's disease with angioid streaks and a secondary choroidal neovascular membrane is presented. The membrane was treated with argon laser photocoagulation. The patient also had an incidental finding of lattice corneal dystrophy which required penetrating keratoplasty.
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4/12. diagnosis and management of angioid streaks.

    angioid streaks are asymptomatic breaks in Bruch's membrane developing later in life. Secondary macular degeneration and other fundus abnormalities often accompany their development. angioid streaks are frequently associated with systemic diseases such as pseudoxanthoma elasticum, Paget's disease of bone, and the sickle cell hemoglobinopathies. The clinical manifestations of angioid streaks, related fundus changes, and these three systemic disorders are discussed as well as principles of treatment and management. Two case reports are presented which illustrate some of the important characteristics of angioid streaks.
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5/12. angioid streaks in beta thalassaemia minor.

    We report what we believe to be the first recorded case of angioid streaks in a patient with beta thalassaemia minor. The occurrence of angioid streaks in a patient with a relatively normal iron balance and only very mild haemolysis may be explained by the combination of pregnancy with associated multiple transfusions in a myopic patient where an inherent defect in Bruch's membrane may exist.
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6/12. Laser treatment in maculopathy of pseudoxanthoma elasticum.

    We report a case of pseudoxanthoma elasticum with exudative retinopathy which was treated with the argon laser and in which the visual acuity was preserved and the subretinal neovascular membrane obliterated for ten months. Laser treatment seems to have helped this case although the same treatment has stimulated the development of subretinal neovascular membranes. These cases must be treated cautiously.
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7/12. argon laser treatment in complications of angioid streaks.

    Five patients with subretinal neovascular complications of angioid streaks were treated with the argon laser. One patient, additionally had a central retinal vein occlusion with preretinal neovascularization. In four out of five cases we obliterated the subretinal neovascular membranes with ensuing improvement of visual acuity. In a follow-up period of 14 months on the average, the treatment appeared to be successful.
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8/12. Laser treatment of choroidal neovascular membranes in angioid streaks.

    Eight patients with active extrafoveal choroidal neovascular membranes related to angioid streaks were studied. One eye of each of four patients was treated with argon laser. Two patients received bilateral treatment. Four patients received multiple treatments at different times for recurrence of neovascular membranes. Two patients were not treated. The visual acuity of the treated eyes remained the same or improved posttreatment, with one exception. The majority of untreated eyes lost vision. The vast majority of the treated eyes showed a marked improvement in central visual field posttreatment, whereas the untreated eyes remained the same or lost considerably more central visual field. In one to four years after treatment, recurrences of the neovascular membranes occurred in four of the treated eyes demonstrating that these cases must be followed indefinitely. Carefully selected cases appear to benefit from complete obliteration of choroidal neovascularization by argon laser photocoagulation.
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9/12. Ocular findings in four siblings with pseudoxanthoma elasticum.

    In a family consisting of 8 surviving siblings, pseudoxanthoma elasticum was diagnosed in 4 sisters. One of them had a severe visual handicap, and another a slight decrease of the visual acuity because of the ocular lesions. The other 2 sisters also had fundal lesions, but they were visually symptom-free. Great variation was also found in the appearance of the ocular lesions in the affected siblings. In addition to the angioid streaks, the fundal findings in the probands affected included changes of the retinal pigment epithelium, exudative maculopathy, optic disc drusen and 'fire-work'-pattern of drusen of the Bruch's membrane. A similar variety of alterations was also found in the histopathology of the skin.
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10/12. angioid streaks associated with abetalipoproteinemia.

    angioid streaks were observed in two patients with abetalipoproteinemia. The progression of the angioid streaks was minimal over the years that these patients received vitamin a and E supplementation, though in one patient the development of subretinal neovascular membranes within the angioid streaks was the cause of rapid central visual loss. The simultaneous appearance of two rare entities in unrelated individuals strengthens the relationship between these two disorders that has been suggested by previous case studies. The authors propose a common metabolic pathway involving trace element deficiencies that may account for this relationship as well as the association of angioid streaks with other rare disorders such as Paget's disease, hypoparathyroidism, lead poisoning, hyperphosphatemia, and a number of hemoglobinopathies. Their study of these two patients underscores the need for further investigations as to the role of copper, zinc and omega-3 fatty acids in the pathogenesis of retinopathy in abetalipoproteinemia.
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