Cases reported "Retinal Diseases"

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1/22. association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus.

    OBJECTIVES: To study the prevalence and characteristics of retinal vascular disease in patients with systemic lupus erythematosus (SLE) and to analyze their relationship with antiphospholipid antibodies (aPL) and other serological markers. patients AND methods: Eighty-two consecutive patients (77 women and 5 men; mean age, 36 years) were studied. All patients fulfilled the 1982 revised criteria of the American College of rheumatology for the classification of SLE. Ophthalmologic examination included assessment of best corrected visual acuity, tonometry, slit-lamp biomicroscopy, and fundus examination. Serologic studies included determination of anticardiolipin antibodies (aCL) (ELISA), lupus anticoagulant (LA) (coagulation tests), antinuclear antibodies (indirect immunofluorescence), anti-dna (Farr's test), and anti-ENA antibodies (counterimmunoelectrophoresis). RESULTS: Retinal vascular disease was detected in 13 (15%) of 82 SLE patients. The retinal lesions consisted of retinal vascular occlusions in six patients (five arterial and one venous), cotton-wool spots in three, optic disc edema in three, retinal hemorrhages in three, and ischemic optic neuropathy in one. Antiphospholipid antibodies were detected in 10 (77%) of these 13 patients: nine had aCL and two had the LA. When compared with patients without retinal vascular disease, patients with retinopathy had a higher prevalence of aPL (77% v. 29%, P = .005). CONCLUSIONS: Retinal vascular disease is frequent in patients with SLE. The presence of aPL is associated with a higher prevalence of retinal abnormalities in SLE patients.
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ranking = 1
keywords = vascular disease
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2/22. Severe visual loss associated with retinal telangiectasis and facioscapulohumeral muscular dystrophy.

    Facioscapulohumeral (FSH) muscular dystrophy is known to be associated with retinal telangiectasis. However, there are only few reports of severe visual loss due to exudative complications, so the risk to vision has not been established. Because of the possible therapeutic implications, we have described two cases of young girls who developed FSH muscular dystrophy and exudative retinal detachment due to telangiectasis. In the first patient, the severity of the disease precluded visual recovery despite extensive photo- and cryotherapy. In the other, visual acuity in both affected eyes was retained after treatment. Fundus examinations in young children at risk of having the gene for FSH muscular dystrophy may be justified so that retinal vascular disease can be detected before it becomes untreatable.
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ranking = 0.125
keywords = vascular disease
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3/22. Group 2A idiopathic juxtafoveolar retinal telangiectasia in monozygotic twins.

    PURPOSE: To report the clinical and fluorescein angiographic characteristics of monozygotic twins affected by group 2A idiopathic juxtafoveolar retinal telangiectasia. DESIGN: Observational case report. methods: Both eyes of identical twins were examined in a clinical practice setting. RESULTS: Two 68-year-old Caucasian, monozygotic, female twins were examined. In one twin, visual acuity was 20/50 in the right eye (OD) and 20/40 in the left (OS). ophthalmoscopy demonstrated right-angle venules without edema in both eyes (OU). fluorescein angiography demonstrated areas of leakage temporal to the fovea OU with some nasal leakage OS. In her identical twin sister, with type II diabetes, visual acuity was 20/60 OD and 20/25 OS. Right-angle venules OU, refractile deposits OS, and no macular edema OU were present. fluorescein angiography demonstrated areas of parafoveal leakage temporal and nasal OD and temporal OS. CONCLUSION: This is the second set of female monozygotic twins with group 2A idiopathic juxtafoveolar retinal telangiectasia reported in the literature. This finding suggests a genetic component in the pathogenesis of this retinal vascular disease.
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ranking = 0.125
keywords = vascular disease
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4/22. Novel ophthalmological features in hereditary endotheliopathy with retinopathy, nephropathy and stroke syndrome.

    The ophthalmic features are reported of a member of an Australian pedigree with three affected individuals spanning two generations with a hereditary endotheliopathy syndrome resulting in retinopathy, leukoencephalopathy and nephropathy. The index case initially presented with asymptomatic retinopathy, cerebral microvascular disease, nephropathy and raised inflammatory markers. The clinical, neuro-radiological, biochemical and histopathological findings in this patient are consistent with a diagnosis of hereditary endotheliopathy, retinopathy, nephropathy and stroke (HERNS). Linkage analysis has identified a locus for HERNS on chromosome 3p21.1-p21.3. This locus is shared with two other diseases: hereditary vascular retinopathy (HVR) and cerebroretinal vasculopathy (CRV). Although peripheral retinal involvement is a feature of HVR, it has not previously been described in HERNS. This case represents a novel phenotype of HERNS, and serves to blur the distinction between the vasculopathies mapping to chromosome 3p21. Although previously thought to be distinct clinical entities, it is possible that HERNS, HVR and CRV simply represent different phenotypes of the same disease. This will only be clarified with the identification of the gene or genes. This case also raises the question of how best to manage rare forms of vascular retinopathy.
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ranking = 0.125
keywords = vascular disease
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5/22. Crohn's disease and retinal vascular disease.

    patients with Crohn's disease may manifest extraintestinal findings including ocular involvement. We treated two patients with Crohn's disease who manifested retinal vascular disease that may have been related to the immune nature of the underlying disease or possibly to changes in the vasculature or coagulation system. One patient had a central retinal vein occlusion and the other had retinal vasculitis involving retinal arteries and veins with an apparent branch retinal artery occlusion. Both patients were in excellent health except for Crohn's disease, which was confirmed by a biopsy specimen. In one patient, the diagnosis of retinal vasculitis preceded the clinical diagnosis of Crohn's disease, whereas for the second patient the symptomatology of the intestinal disease preceded the ocular manifestations. We suggest that inflammatory bowel disease should be considered in the differential diagnosis of retinal vascular occlusive disease, especially in a young patient.
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ranking = 0.625
keywords = vascular disease
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6/22. Panretinal cryotherapy in neovascular disease.

    Panretinal cryotherapy (PRC) was used to treat 15 eyes with rubeosis, nine of which had established neovascular glaucoma, and seven eyes with proliferative diabetic retinopathy. The rubeosis regressed, with preservation of vision and return to normal of intraocular pressure, in all but one eye. With one exception all eyes with proliferative retinopathy also showed new vessel regression after treatment. PRC may be considered an effective alternative to retinal photocoagulation in the treatment of advanced rubeosis, early rubeotic glaucoma, and in certain circumstances proliferative diabetic retinopathy.
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ranking = 0.5
keywords = vascular disease
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7/22. Embolic anterior ischemic optic neuropathy.

    The presumed cause of anterior ischemic optic neuropathy is atherosclerotic vascular changes. Small-vessel occlusive vascular disease most likely causes occlusion of one or more posterior ciliary arteries, compromising blood flow to the optic nerve head and choroid. We present the case of a 59-year-old black man with cholesterol emboli within the retinal vasculature combined with a clinical picture of anterior ischemic optic neuropathy and choroidal nonperfusion demonstrated by i.v. fluorescein angiography. We believe this is evidence that, in a rare case, embolic phenomena may be the cause of anterior ischemic optic neuropathy.
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ranking = 0.125
keywords = vascular disease
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8/22. Cerebral thromboembolic disease in pediatric and adult inflammatory bowel disease: case report and review of the literature.

    Cerebral and retinal vascular disease are rare complications of inflammatory bowel disease. Most reports are of adult patients, with only seven instances described in children. The eighth case, a 14-year-old boy with ulcerative colitis and cerebral venous thrombosis is reported in whom the diagnosis was confirmed by magnetic resonance imaging. The adult and pediatric literature is also reviewed.
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ranking = 0.125
keywords = vascular disease
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9/22. Retinal arterial macroaneurysms.

    Retinal arterial macroaneurysm is a distinct, infrequently occurring fundus sign usually found in older patients with systemic vascular disease. The lesion arises on a main branch of the retinal artery within the first three orders of bifurcation. Visual disturbance is uncommon, although it may occur as a result of vitreous or retinal hemorrhage or macular edema. This paper presents two cases of retinal arterial macroaneurysm and discusses the diagnosis, etiology, and management of this condition.
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ranking = 0.125
keywords = vascular disease
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10/22. Antiphospholipid antibodies associated with retinal vascular disease.

    Two patients with retinal vascular disease also had antiphospholipid antibodies, defined by the presence of the circulating lupus anticoagulant (LAC), anticardiolipin antibodies, or both. One had retinal arterial occlusions and the other had a nonischemic central retinal vein occlusion. The association of thrombotic vascular disease, including both retinal and cerebral vessels, with the presence of these antibodies should be investigated in otherwise normal individuals or in patients presenting with a lupuslike syndrome and retinal vascular occlusive disease. Low-dose aspirin administration could be of therapeutic value in managing thrombotic events in this group of patients.
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ranking = 0.75
keywords = vascular disease
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