Abstracts

Rationale: The Sturge-Weber syndrome is a congenital neurocutaneous disorder characterized by facial angioma in the distribution of the first division of the trigeminal nerve, and widespread leptomeningeal angiomatosis which can lead to medically refractory epilepsy. Recent research suggests that this disorder is caused by somatic mutations in GNAQ, which encodes an alpha subunit in the q class of G-proteins. The degree of observed intracerebral vascular disease can be highly variable. We describe 3 cases of children with a Sturge-Weber diagnosis who underwent epilepsy surgery and demonstrated a wide spectrum in extent of gross cerebrovascular disease.Methods: Surgery for seizure control proceeded after extensive multidisciplinary preoperative evaluation including 3T MR imaging and noninvasive monitoring. All patients showed a facial angioma ipsilateral to the involved cerebral hemisphere. Patients 1 and 3 underwent transsylvian functional hemispherotomy, while patient 2 underwent a Rasmussen style functional hemispherectomy.Results: Surgical exposure of the cerebral cortex revealed significant differences in the extent of gross angiomatosis among the 3 cases (figure 1). Age at surgery, operative time, and estimated blood loss are summarized in table 1. All patients achieved an Engel class 1 seizure outcome at least 5 months postoperatively.Conclusions: The degree of leptomeningeal angiomatosis in Sturge-Weber syndrome, as reflected in imaging and pathology, can be highly variable between patients but may not be a prognostic factor regarding seizure outcome. Disconnective surgery may offer advantages regarding operative time and blood loss in this population.