Sturge-Weber disease: operative indications and surgical results

Patients with Sturge-Weber disease with epilepsy refractory to medical therapy have been reported to develop slowly progressive neurological deficits and ultimately become moderately or severely disabled. We studied six patients with Sturge-Weber syndrome including its incomplete form. Three out of six patients with Sturge-Weber syndrome revealed evolution of calcified angioma on computed tomography. All of the three cases developed medically intractable seizures. Total and/or subtotal hemispherectomy was performed for these three cases. The surgery was effective for controlling seizures in all three cases except one with infantile spasm with hypsarrythmia on electroencephalogram who is still on anticonvulsant. Although the unremitting deterioration in mental retardation and hemiparesis was not effectively prevented by the surgery possibly because the timing of surgery was delayed in one case, the surgery not only stopped the frequent medically-intractable seizures, but also dramatically prevented the psychomotor deterioration in the other case. Although the role of surgical treatment for the patients with Sturge-Weber syndrome remains poorly defined, one can expect excellent results if the indications for surgery are carefully analyzed and hemispherectomy is performed on an individual basis.