Abstract
Sturge–Weber syndrome is a rare congenital disease, also called encephalotrigeminal
angiomatosis, caused by persistence of transitory primordial arteriovenous connections
of the fetal intracranial vasculature. It is characterized by vascular malformations
with capillary venous angiomas that involve the face, choroid of the eye, and leptomeninges.
The main clinical features of this syndrome are port-wine stains, glaucoma, convulsions,
and angiomas of the airway. Anesthesia management is directed toward anticipating
a difficult airway, avoiding trauma to the hemangioma during airway manipulation,
preventing the rise in the intracranial and intraocular pressures, anticipating and
managing massive blood loss and the complications associated with massive blood transfusion,
and avoiding factors that might trigger a seizure such as hypoxia, hypercarbia, hypotension,
hypoglycemia, and hyperthermia.
Keywords
Sturge–Weber syndrome - epilepsy surgery - anesthetic implications
