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1/7. The diagnostic challenge of occult large vessel ischemia of the retina and choroid.

    Vascular occlusions of the retina and choroid can cause severe visual loss. These occlusions can occur as a result of systemic disease or after surgery. In most cases, the retinal appearance provides evidence of ischemia as the cause of visual loss. On occasion, however, clinical examination shows no objective signs of vascular occlusion, and this can lead the clinician to suspect optic nerve pathology as the cause of visual loss. This paper outlines some of the diagnostic criteria, clinical findings, and ancillary studies that can be used to differentiate between occult occlusion of the retina or choroid and optic nerve disease.
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2/7. Benefits and complications of photodynamic therapy of papillary capillary hemangiomas.

    OBJECTIVE: To evaluate the potential benefit and risks of photodynamic therapy (PDT) in the treatment of papillary capillary hemangioma. DESIGN: Prospective, noncomparative, interventional case series. PARTICIPANTS: Five patients with solitary capillary hemangioma on the temporal portion of the optic nerve presenting with exudative decompensation and decrease in visual acuity (VA). methods: All eyes received a standardized PDT treatment with 6 mg/kg body surface area verteporfin and application of 100 J/cm(2) light at 692 nm. One to three PDT courses were performed until resolution of exudation was achieved. A continuous follow-up was provided with documentation 1 week before and at 4 to 6 weeks, 3 months, and 12 months after the last treatment application. MAIN OUTCOME MEASURES: Functional parameters included best-refracted VA (Early Treatment diabetic retinopathy Study), and central scanning laser ophthalmoscope (SLO) scotometry and peripheral (automated perimetry) visual fields; anatomic parameters were presence of retinal edema or serous detachment (ophthalmoscopy) and tumor size (ultrasonography). RESULTS: Pretreatment VA levels ranged from 20/40 to 20/800; posttreatment levels ranged from 20/64 to 20/2000. Tumor regression with resolution of macular exudate and serous retinal detachment was obtained in all eyes. A decline in VA of 1, 3, and 10 lines, respectively, was documented in three patients. Complications included transient decompensation of vascular permeability, occlusion of retinal vessels, and ischemia of the optic nerve. CONCLUSIONS: PDT is successful in reducing tumor size and exudative activity. Vaso-occlusive effects at the level of the retina and optic nerve compromise the functional benefit. Parameters proven safe in choroidal neovascularization may be inappropriate in retinal capillary lesions of the optic nerve.
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3/7. Multifocal choroiditis in patients with familial juvenile systemic granulomatosis.

    PURPOSE: To document clinical features of uveitis in patients with familial juvenile systemic granulomatosis. DESIGN: Retrospective chart review. methods: Ophthalmologic examination, medical history, and clinical course in 16 patients from eight families examined at six academic medical centers. RESULTS: Of the 16 patients, 15 had evidence of panuveitis with multifocal choroiditis. One patient had only an anterior uveitis. Ischemic optic neuropathy, presumably due to a small vessel vasculopathy, and retinal vasculopathy each occurred in one patient. Ocular complications were common, including cataracts in 11, glaucoma in six, band keratopathy in six, cystoid macular edema in six, and optic disk edema in six. All 16 patients had polyarthritis, and at least nine had skin rash. Often patients were misdiagnosed initially as having either juvenile rheumatoid arthritis or sarcoidosis. CONCLUSIONS: Familial juvenile systemic granulomatosis is an uncommon genetic disease characterized by polyarthritis and uveitis. panuveitis and multifocal choroiditis often may be present. patients with a diagnosis of juvenile rheumatoid arthritis but having a family history of the disease and multifocal choroiditis should be suspected of having familial juvenile systemic granulomatosis.
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4/7. Posterior ischemic optic neuropathy after hemodialysis.

    PURPOSE: To report a case of visual loss from posterior ischemic optic neuropathy (PION) after hemodialysis. DESIGN: Observational case report. methods: Neuro-ophthalmic examination, neuro-imaging including computed tomography (CT) scan, magnetic resonance imaging (MRI) of the head and orbits, and magnetic resonance angiography (MRA) of the neck and cerebral vasculature, as well as electrophysiologic testing including electroretinogram (ERG) and visually evoked response (VER) were performed. RESULTS: Acute onset of painless bilateral no light perception vision with absent pupillary response to light and normal funduscopic examination occurred shortly after completion of hemodialysis. Computed tomography scan and MRA results were normal. magnetic resonance imaging scan showed small vessel ischemic white matter changes. Electroretinogram results were normal and the VER was unrecordable. CONCLUSIONS: Visual loss after hemodialysis is a rare complication and is associated with anemia and hypotensive events. The visual loss is usually a result of anterior ischemic optic neuropathy. We were unable to find another instance in the literature of visual loss after hemodialysis resulting from PION.
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5/7. Ocular ichemia syndrome - a malignant course of giant cell arteritis.

    PURPOSE: To call attention to a malignant course of ocular ischemic syndrome in patients with giant cell arteritis (GCA). methods/PATIENT: A 84-year-old woman developed severe headache for about 3 (1/2) months prior to myocardial infarction and visual disturbances. RESULTS: An anterior ischemic optic neuropathy (AION) in the right eye with a distinct reduction in visual acuity was found. The retina revealed several cotton-wool spots in both eyes. Serologic examinations showed inflammatory signs. Despite treatment with prednisolone, eye pressure decreased to 2 mm Hg in the right eye and 4 mm Hg in the left eye in a few days. An ischemic iritis developed in the right eye. visual acuity worsened to detection of hand motions in the right eye and to 0.1 in the left eye. Approximately 8 (1/2) months after her initial headache, a biopsy was carried out. The patient was treated continuously with corticosteroids. histology of the superficial temporal artery indicated inflammatory cells in the vessel wall. - The patients daughter developed symptoms of GCA at the age of 54 years. CONCLUSION: An ocular ischemic syndrome points to a malignant course of the disease. A cardiac infarction can develop in GCA. A biopsy of the temporal artery can reveal inflammatory changes even after 8 (1/2) months.
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6/7. Anterior ischemic optic neuropathy associated with pulseless disease.

    A 41-year-old man with pulseless disease presented with a sudden onset of visual loss in the right eye. The right fundus showed moderate optic disk swelling with mild pallor. aortography demonstrated bilateral obstruction of the common carotid arteries and left subclavian artery, and only collateral vessels supplied the upper portion. Temporal artery biopsy revealed no inflammation. In addition, the patient originally had relatively small disks. This anatomical factor and marked hypoperfusion of the posterior ciliary artery presumably caused nonarteritic anterior ischemic neuropathy in this patient.
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7/7. Uveal effusion syndrome complicated by anterior ischemic optic neuropathy.

    We report on a case of idiopathic uveal effusion syndrome complicated by AION. To our knowledge such an association hasn't been previously described. We suggest that scleral thickening caused obstruction of vortex veins followed by uveal effusion and compression of posterior ciliary arteries within their intrascleral tract, leading to AION. Nevertheless is can't be excluded that AION was the result of mechanical compression on ciliary vessels of optic disc by choroidal detachment.
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