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Shturge-Weber's syndrome


Hereditary disease (a mode of inheritance – preferential autosomal and recessive) – an inborn malformation of mesodermal and ectodermal elements of the head.

Symptoms of the Syndrome of Shturge-Weber:

The triad is characteristic:

1) "a flame nevus" (on a face and the head, almost always unilateral, in a trifacial innervation zone, the I branch is more often);

2) epilepsy (partial attacks - on the contralateral party),

3) inborn glaucoma with an odnostor.gidroftalmiya (not always).

Weak-mindedness is also characteristic.

At X-ray inspection – calcification of intracranial vessels in the form of a double contour.

The main display of a disease is characterized by an inborn vascular nevus (capillary hemangiomas) on a face (table 1). A nevus, them often calls "portveyny", usually unilateral, but can be bilateral, involves an upper half of the face variable from frontal to verkhechelyustny area, being often limited to zones of an innervation of branches of a trifacial.

Important property of capillary hemangiomas is their potential association with intracranial and intraocular vascular anomalies. Glaucoma can develop in an ipsilateralny eye at to 50% of patients, it can be inborn or develop by 2 years of life.  A leptomeningeal angiomatosis almost always ипсилатерален to a front nevus at what, the extent of an intracranial angiomatosis correlates with a size of front spots. The struck hemisphere is exposed to a gradual atrophy with development of calcification of a subcortical plate. At the same time clinical manifestations Shturge-Weber's Syndrome can be characterized by epileptic attacks  (at 70-90% of patients), mental retardation, a hemiparesis, disturbance of sensitivity on a gemitipa, a hemianopsia. The Pharmakorezistentny course of epilepsy can demand a partial or total gemisferektomiya. Also perhaps laser removal of front spots at certain patients with the cosmetic purposes.

Table 1.  Shturge-Weber's syndrome. Diagnostic criteria
Front capillary hemangiomas or glaucoma and clinical or radiological signs of a leptomenigealny angioma
•    Hemiparesis
•    Hemiatrophy (brain or body)
•    Spasms
•    Deficit of fields of vision
•    Delay of mental development
•    Signs of a leptomeningeal angioma at KT or MPT

Внешний вид больного ребенка с синдроом Штурге-Вебера

Outward of the sick child with a syndrome Shturge-Weber

Reasons of the Syndrome of Shturge-Weber:

Is a hereditary syndrome, it is transferred on an autosomal retsesivnomu to type.

Treatment of the Syndrome of Shturge-Weber:

Treatment only symptomatic.

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