Walsh & Hoyt: Management

The prognosis in patients with SWS is much better than in some of the other phacomatoses, particularly VHLD and neurofibromatosis. Patients with SWS develop intracranial and systemic malignancies with no greater frequency than do healthy individuals. The major difficulties encountered by these patients are neurologic and include seizures, progressive intellectual deterioration, and hemiparesis or hemiplegia. If the seizures are difficult to control with antiepileptic medication or adrenocorticotropic hormone for those with infantile spasms, progressive neurologic deterioration may ensue. Hemispherectomy is an effective means of reducing the frequency and severity of seizures. In selected patients, callosotomy or focal cortical resection are other surgical options; however, the timely role of surgery to stem the progression of seizures or development of mental retardation remains controversial, in part because the natural course of the seizures in individual patients is unpredictable. Nevertheless, adequate seizure control is obtainable in up to 65% of patients. Hemispherectomy is recommended for patients with unilateral disease and medically intractable seizures. Patients with bilateral disease are less likely to benefit from surgical intervention. Aspirin therapy to prevent recurrent venous thromboses is advocated as a prophylactic measure to stem neurologic progression.