El síndrome de Sturge-Weber es un trastorno vascular congénito . el 77% de los pacientes, siendo la enfermedad más frecuente el glaucoma (46%), seguido . ORPHA Synonym(s). Encephalofacial angiomatosis; Encephalotrigeminal angiomatosis; SWS; Sturge-Weber-Dimitri syndrome; Sturge-Weber-Krabbe. Meanings of “enfermedad de sturge-weber” in English Spanish Dictionary: 1 result(s) Meanings of “enfermedad de sturge-weber” with other terms in English.
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It is part of a wide spectrum of possible phenotypes included in the craniofacial wfber metameric syndrome CAMS. Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20, persons The diagnosis is usually obvious on account of a congenital facial cutaneous hemangioma also known as port wine stain or facial naevus flammeus.
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Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma 1. Unlike most phakomatoses, Sturge-Weber syndrome is sporadic with no definite identifiable enfermedax component 5, The leptomeningeal hemangioma results in a vascular steal affecting the subjacent cortex and white matter producing localised ischemia.
Skull x-rays were historically useful and capable of identifying the gyriform calcification of the subcortical white matter although they no longer play a significant role in the diagnosis or management of this condition.
The finding usually becomes evident between 2 and 7 years of age 2. Treatment revolves primarily around seizure control, with surgical resection only indicated rarely in refractory cases. Ophthalmological examination is also essential to identify and treat ocular involvement 4.
Orphanet: Síndrome de Sturge Weber
Sturge-Weber syndrome was first described by Sturge infollowed by Weber and Volland in who described the intracranial calcification. Radiographic identification of cerebral calcification was first described by Dimitri in The differential is a combination of that for multiple intracranial calcificationscerebral hemiatrophy and leptomeningeal enhancementand sturgr includes:.
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Sturge-Weber syndrome | Radiology Reference Article |
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enfermedad de sturge-weber
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