Surgical treatment of epilepsy in Sturge-Weber syndrome in children

Object. The authors sought to analyze the success rate of surgery in the management of medically intractable epilepsy in children with Sturge-Weber syndrome and to determine whether the extent and timing of surgery affected seizure and developmental outcomes. Methods. The authors performed a retrospective review of 27 children who underwent surgery at their institution for medically resistant epilepsy, and they examined the outcomes with regard to epilepsy control and neuropsychological development. Seventeen children (63%) experienced onset of their epilepsy when they were younger than 1 year of age. These patients were significantly more likely to have hemiparesis (p <= 0.001) and status epilepticus (p <= 0.001) and be developmentally delayed (p <= 0.025) than children whose epilepsy started later in life. Eight patients underwent a hemispherectomy (either anatomical or functional), and complete resolution of epilepsy was noted in all. Of the 19 patients in whom a focal resection was performed, 11 (58%) became seizure free. The 10 children in whom there was residual disease were more likely to have continuing epilepsy than the nine whose lesions were completely excised (p <= 0.05). Seventeen children exhibited improvement in their developmental status following surgery. This improvement was significantly affected by completeness of resection (p <= 0.05) and age at surgery (p <= 0.009). Seizure freedom per se was not affected by the timing of surgery. Conclusions. Medically intractable epilepsy in children can be treated effectively by surgery. The degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control. Early surgery is more likely to improve developmental outcome.