Sturge-Weber syndrome - Indications and results of surgery in 20 patients

Objective: To discuss the indications and timing for resective surgery in p atients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy. Background: SWS that causes epilepsy severe enough to merit surgery is rar e. Because of the variable natural history of the disease, it is difficult to establish clear-cut indications for surgery and prospective studies are not feasible. Attitudes of clinicians and surgeons remain variable. Methods : The authors assessed the presurgical epilepsy profile, criteria for surge ry, monitoring techniques, and the postoperative outcome of epilepsy in all patients with SWS consecutively admitted between 1972 and 1990 to two refe rral centers (Paris and Montreal) and underwent surgery for intractable sei zures. Results: All 20 patients had a minimal postoperative follow-up of 4 years and all but one are still followed by one of the authors. One patient had a callosotomy, five underwent hemispherectomy, and 14 had cortical res ection. Despite variability in the age at onset of seizures (range: 2 month s to 12 years), age at operation (range: 8 months to 34 years) and surgical methods, almost all patients benefited from surgery. Visually guided compl ete resection of the pial angioma and underlying cortex, whenever possible, seemed sufficient; results were no better with intraoperative corticograph y. In children with previous hemiparesis, hemispherectomy proved particular ly effective: all five became seizure free. None of the patients showed any aggravation of cognitive impairment following surgery; none of those who w ere operated on early presented with severe mental retardation, and 13 of 2 0 became seizure free. Conclusion: Although the natural history of SWS is i mperfectly known, increasing duration of seizures and of postictal deficits , increase in atrophy or of calcified lesions or both, are indicative of it s progressive nature. Despite the expected heterogeneity that renders forma l comparison of the various approaches difficult, the current study provide s new evidence to support early surgery in patients with SWS and drug-resis tant epilepsy. The authors' results suggest that lesionectomy is a good app roach, provided that the pial angioma is unilateral and the resection can b e complete.