Cases reported "Sturge-Weber Syndrome"

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1/16. sturge-weber syndrome: a rare cause of gastrointestinal hemorrhage.

    A case of sturge-weber syndrome (SWS) with gastrointestinal hemorrhage is presented. SWS is a neurocutaneous disorder characterized by cutaneous facial angioma leptomeningeal angioma with seizures and other neurologic complications. Associated anomalies beyond the encephalofacial territory are very rare. The patient presented repeated bleeds from extensive gastric varices of the fundus secondary to a splenic venous malformation. This is the first report on this association to our knowledge.
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ranking = 1
keywords = fundus
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2/16. indocyanine green angiographic findings in diffuse choroidal hemangioma associated with sturge-weber syndrome.

    PURPOSE: To analyze indocyanine green angiography (ICGA) features in one case of diffuse choroidal hemangioma associated with sturge-weber syndrome. methods: color fundus photography, fluorescein angiography (FA) and ICGA were performed in a patient with diffuse choroidal hemangioma associated with sturge-weber syndrome. RESULTS: The diffuse choroidal hemangioma was not identified by FA. ICGA revealed that the vascular tissue of the diffuse hemangioma filled rapidly with dye during the arterial phase of the choroidal angiogram; copious dye leakage appeared early and persisted into the late phase of angiography. Late "wash-out" phenomenon was not observed 30 minutes after dye injection. Sectors of reduced choroidal perfusion in the upper or lower half of the midperiphery were present. CONCLUSION: ICGA may be an important and sensitive technique in detecting the diffuse choroidal hemangioma associated with sturge-weber syndrome.
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ranking = 71.608185678803
keywords = angiography, fundus
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3/16. intracranial hypertension in Sturge-Weber/Klippel-Trenaunay-Weber overlap syndrome due to impairment of cerebral venous outflow.

    klippel-trenaunay-weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. sturge-weber syndrome (SWS), also a mesodermal phakamatosis, is characterized by meningofacial angiomatosis with cerebral calcification. Overlap between KTWS and SWS is recognized. We describe the case of a young woman with features of both KTWS and SWS who presented with symptoms of acute intracranial hypertension, including headache, vomiting and marked visual impairment. cerebral angiography revealed paucity of the superficial cortical veins overlying one of the cerebral hemispheres and centripetal venous drainage via small deep venous channels. Based upon this pattern of cerebral venous outflow, we postulate a mechanism to explain the acute episode of intracranial hypertension.
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ranking = 23.536061892934
keywords = angiography
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4/16. sturge-weber syndrome associated with early diffuse choroidal hemangiaoma: a case report.

    PURPOSE :To report indocyanine green angiography (ICGA) findings in one patient of diffuse choroidal hemangioma associated with Struge-Weber syndrome. methods: color fundus photography, fluorescein angiography (FA) and ICGA were performed in a patient with diffuse choroidal hemangioma associated with sturge-weber syndrome. RESULTS: Three findings were unveiled by ICGA: rapid filling of diffuse choroidal hemangio's vascular network in the early stages; diffuse hyperfluorescence visual up to the late phase; no "wash-out" phenomenon was observed in the late phase. CONCLUSION: indocyanine green angiography can provide information that is not detected by clinical or fluorescence angiographic examination in the patient with sturge-weber syndrome. ICGA may be important and sensitive in detecting the diffuse choroidal hamangioma associated with sturge-weber syndrome.
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ranking = 71.608185678803
keywords = angiography, fundus
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5/16. Oromaxillofacial osseous abnormality in sturge-weber syndrome: case report and review of the literature.

    We report a case of a 17-month-old child affected by sturge-weber syndrome who had unusually rapid overgrowth of the left frontal, temporal, orbital, and maxillary regions. CT angiography illustrated osteohypertrophy with periostitis and associated soft tissue hypertrophy directly corresponding to the distribution of the cutaneous port-wine stain. Extended maxillectomy was performed because of rapid growth and clinical debilitation, with surgical pathology revealing juvenile ossifying fibroma.
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ranking = 23.536061892934
keywords = angiography
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6/16. iris neovascularization in sturge-weber syndrome.

    An unusual case of sturge-weber syndrome with raised intraocular pressure is presented. The slit lamp examination and anterior segment fluorescein angiography were consistent with iris neovascularization. Cause and effect relationship of iris neovascularization with glaucoma in this syndrome is discussed.
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ranking = 23.536061892934
keywords = angiography
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7/16. Non-filling of the subependymal veins of the lateral ventricles.

    Four cases of non-filling of the subependymal veins of the lateral ventricles and internal cerebral vein at carotid angiography are reported. The patients were all children ranging in age from 4 months to 2 years and had a variety of neurologic conditions. Several hypotheses are suggested as to the possible pathogenesis of these findings.
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ranking = 23.536061892934
keywords = angiography
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8/16. epilepsy surgery in sturge-weber syndrome.

    A patient with sturge-weber syndrome developed seizures at the age of 4 years. At 13 years of age, she had intractable complex partial seizures with marked visual symptomatology. Interictal encephalograms showed bilateral slow activity, more marked over the right hemisphere with epileptogenic activity maximal in the right temporal region. Serial computerized axial tomography scans demonstrated evolution of bilateral occipital lesions with calcification and adjacent low density areas that were more marked on the right. magnetic resonance imaging, angiography and neuropsychological evaluations were performed. An extensive resection of the right occipital lobe was achieved. One year after surgery, the patient has had seven brief seizures. Delineation of an epileptogenic focus and surgical removal of the lesion in patients with intractable seizures can now be considered in selected patients with bilateral central nervous system pathology.
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ranking = 23.536061892934
keywords = angiography
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9/16. Megalencephaly due to impaired cerebral venous return in a Sturge-Weber variant syndrome.

    An infant with a Sturge-Weber variant syndrome developed progressive megalencephaly and eventual hydrocephalus, which required shunting. cerebral angiography revealed absence of the deep cerebral venous system and the development of abnormal drainage channels via the periorbital veins. It is postulated that the abnormal enlargement of the brain was due to the impaired venous return. Resistance of the brain to continued expansion may have caused an increase in hydrostatic pressure and the development of hydrocephalus.
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ranking = 23.536061892934
keywords = angiography
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10/16. Cilio-optic vein associated with phakomatosis.

    This presentation demonstrates the fluorescein angiographic characteristics of the cilio-optic vein. These congenitally enlarged vessels appear at the disc edge and dip into the optic nerve to anastomose with branches of the central retinal vein. fluorescein angiography shows lamellar filling of the vessels in the early choroidal phase. In one patient, these findings are demonstrated bilaterally with the use of bilateral simultaneous angiography. A second type of communication is presented, a retinociliary vein which drains the retina into the choroidal circulation. In both types presented, a branching hypofluorescent pattern extends from the disc vessel into the choroid. Our patients have evidence of a phakomatosis: neurofibromatosis or sturge-weber syndrome. This anomalous disc vessel should not be confused with optociliary shunt, disc neovascularization, cilioretinal artery, or arteriovenous shunt.
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ranking = 47.072123785868
keywords = angiography
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