Revista Cubana de Pediatría
On-line version ISSN 1561-3119
The encephalotrigeminal angiomatosis (Sterge-Weber syndrome) is the commonest of the neurocutaneous syndromes with predominance of vascular anomalies. Its complete form consists in the association of cerebral, cutaneous and ocular angiomas, which are characterized by a wine-clored stain, epilepsy, mental retardation, neurological deficits (hemiparesis and hemianopsia) and glaucoma. CAT and cranial magnetic resonance imaging are very useful for diagnosing the disease and for studying the extension of the leptomeningeal angioma or underlying atrophy. These studies are also important for the prognosis and medical conduct to be followed. The control of the epileptic seizures is an essential element for the favorable evolution of the patients. The surgical treatment must be reserved for young patients with exclusive unilateral affection, with refractary epilepsy and without deep mental retardation. Resection of the affected area, lobectomy or hemispherectomy will be performed whenever the other hemisphere is intact. The clinical, diagnostic, pathogenic and therapeutic aspects of this disease are thoroughly analyzed.
Keywords : STURGE-WERBER SYNDROME [terap]; STURGE-WERBER SYNDROME [diag]; EPILEPSY [terap].