Syndrome of Sturge-Weber

The Syndrome of Sturge-Weber (or SSW ) is associated with 10% of the cases of plane Angiome of the frontal and palpebral area higher present than the birth.

It is neither a Tumeur, nor a hereditary Maladie. Its etiology is still unknown. It can sometimes be accompanied by ocular disorders and neurological.

In more than 50% of the cases, the person touched by such a Syndrome will develop a Glaucome same side as the facial plane angioma of the frontal and palpebral area higher, especially if the AP (flat angioma) occupies the Paupière S higher and lower.

One finds sometimes anomalies vascular Fond of eye or a leptoméningée vascular anomaly being able to cause, often before 2 years, a Comitialité (at 75% of the patients having intracranial vascular anomalies) and, a more or less important driving deficit of the opposed hémicorps, with delay of acquisitions.

Evolution

Hémiatrophie cerebral on the same side as the flat angioma,
Appearance of Calcification S in volutes grinding the cerebral convolutions in the child (seldom in the newborn or the infant)

Incidence:

1 case for 50.000

Origin?

An assumption is that it could result from an intra-uterine somatic change affecting the former primordium neural, before the migration of the cephalic peaks neurales .

Treatment

The SSW is a neuropediatric medical emergency. The treatment is symptomatic. The plane angioma perhaps erased by pulsated laser with dye as soon as the epilepsy is treated and controlled (Anti-convulsivant S). Because of the risk of glaucome, regular examinations opthalmologic are prescribed from 0 to 2 years, and are spaced until the adulthood, even if the first examinations are normal. Possible a glaucome can be treated by the surgery. The muscular weakness of the child must be dealt with.

Forecast

The repetition and the importance of the epileptic fits are one of the elements. Those appear generally precociously (as of the first year of life) and can have after-effects (paralyzes of certain parts of the body, backwardness, possibly severe. The neurosurgery remains rare.

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