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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Additional information Further information on this disease Classification s 8 Gene s 1 Disability Clinical signs and symptoms Publications in PubMed Other website s Loading Stack – 0 images remaining. To improve our services and products, we use “cookies” own or third parties authorized to show advertising related to client preferences through the analyses of navigation customer behavior. If you are a member of the AEDV: Sturge-Weber syndrome was first described by Sturge infollowed by Weber and Volland in who described the intracranial calcification.
According to a recent report, prenatal diagnosis may be suggested by ultrasound or MRI revealing unilateral hemispheric gyriform calcification, focal hemispheric atrophy and white matter changes. Synonyms or Alternate Spellings: Check for errors and try again.
Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20, persons In rare cases, patients may not present with PWS. The leptomeningeal hemangioma results in a vascular steal affecting the subjacent cortex and white matter producing localised ischemia. Glaucoma may be present at birth or develop later. Case 14 Case Ophthalmological examination is also essential to identify and treat ocular involvement 4.
Soft tissue and bony hypertrophy can be associated with a developing PWS that can lead to vision, hearing, swallowing, and speaking problems. Sometimes, the PWS may also cover studge maxillary and mandibular areas of the face and in some cases may extend to the trunk and limbs. Case 8 Case 8.
Detailed information Article for general public Svenska Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma.
Seizures are the most common neurological manifestation and typically present in the first months of life. June Pages ee38 Pages Treatment revolves primarily around seizure control, with surgical resection only indicated rarely in refractory cases. Genetic counseling The disease is sporadic. Granell bE. Diagnostic methods Diagnosis is suspected upon clinical examination. Frequent ophthalmologic examinations should be carried out in cases with glaucoma.
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Síndrome de Sturge-Weber: revisión | Actas Dermo-Sifiliográficas (English Edition)
This item has received. Case 4 Case 4. With the progression of the disease, and depending on the severity of seizures, patients may develop hemiparesis, hemiplegia, and variable degrees of intellectual disability.
Case 7 Case 7.
Only comments written in English can be processed. For all other comments, please send your remarks via contact us. Check this box if you wish to receive a copy of your message. Clinical description The facial capillary malformation classically referred to as angioma is a port-wine stain PWS that is generally present at birth and located on the forehead or upper eyelid on one or both sides of the face.
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Unable to process the form. Case 10 Case It is part of a wide spectrum of possible phenotypes included in the craniofacial arteriovenous metameric syndrome CAMS. Therapy with eye drops is used to decrease pressure in the eye. Case 6 Case 6. Are you a health professional able to prescribe or dispense drugs?
Approximately a third of patients have choroidal or scleral angiomatous involvement, zturge may be enfegmedad with retinal detachment, buphthalmos or glaucoma 1. Surgery may be recommended. Prognosis The prognosis depends on the severity of the epileptic crises which may lead to variable degrees of psychomotor regression and intellectual disability. It is characterized by a facial Port-wine stain, leptomeningeal angiomatosis, and glaucoma. Case 12 Case Case 11 Case SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal’s impact.