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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Frequent ophthalmologic examinations should be carried out in cases with glaucoma. 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. Si continua navegando, consideramos que acepta su uso. Genetic counseling The disease is sporadic. SRJ is a prestige metric based on the idea that not all citations are the same.
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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.
The diagnosis is usually obvious on account of a congenital facial cutaneous hemangioma also known as port wine stain or facial naevus flammeus.
Standard treatment for Sturge-Weber syndrome includes laser treatment for the Enffrmedad stain, anticonvulsants, and medical or surgical treatment for the glaucoma. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
Case 4 Case 4. Radiographic identification of cerebral calcification was first described by Dimitri in You can also scroll through stacks with your mouse wheel or the keyboard arrow keys.
Sturge-Weber syndrome | Radiology Reference Article |
Edit article Share article View revision history. Focal resection or hemispherectomy should be investigated when medical management fails to control the seizures.
Articles Cases Courses Quiz. Diagnostic methods Diagnosis is suspected upon clinical examination.
Laser treatment, usually started in infancy, reduces the progression of the PWS and allows partial, or in rare cases, complete, clearance. Thank you for updating your details.
enfermedad de sturge-weber
Epidemiology Clinical presentation Pathology Radiographic features Treatment and prognosis History and etymology Differential studge Related articles References Images: The documents contained in this web site are presented for information purposes only. Infants typically present in the first year of life with leptomeningeal angiomatosis, responsible for the occurrence of focal or complex partial seizures, early-handedness, and visual gaze preference.
In rare cases, patients may not present with PWS. Migraines and stroke-like episodes are also very common. The finding usually becomes evident between 2 and 7 years of age 2. Management and treatment Laser treatment, usually started in infancy, reduces the ssturge of the PWS and allows partial, or in rare cases, complete, clearance.
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. Ophthalmological examination is also essential to identify and treat ocular involvement 4. If you are a member of the AEDV: It is part of a wide spectrum sturgr possible phenotypes included in the craniofacial arteriovenous metameric syndrome CAMS.
Treatment revolves primarily around seizure control, with surgical resection only indicated rarely in refractory cases.
Orphanet: Síndrome de Sturge Weber
Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma 1. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma. Antenatal diagnosis 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.
Pemphigus Vegetans engermedad the Inguinal Folds.
Are you a health professional able to prescribe or dispense enfermedac Continuing navigation will be considered as acceptance of this use. Unlike most phakomatoses, Sturge-Weber syndrome is sporadic with no definite identifiable hereditary component 5, With the progression of the disease, and depending on the severity of seizures, patients may develop hemiparesis, hemiplegia, and variable degrees of intellectual disability.