What is the efficacy of hemispherectomy in the treatment of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

In a study of hemispherectomy outcomes in cases of SWS, 81% of patients were found to be seizure free, with 53% off antiepileptic drugs; the type of surgery (anatomic hemispherectomy vs functional hemispherectomy vs hemidecortication) did not influence outcome. In the report, Kossoff et al evaluated the results of hemispherectomy in 32 patients with SWS, using a questionnaire; these patients were identified through the Sturge-Weber Foundation. [110]

Although this study was limited because of the volunteer basis of the returned questionnaires, it nonetheless included a larger number of patients were involved in previous studies. Patients had hemispherectomy between 1979 and 2001; the mean age of seizure onset was 4 months, and the median age of surgery was 1.2 years.

Sixteen patients in the study had anatomic hemispherectomy, 14 had functional hemispherectomy, and 2 had hemidecortications, with the surgeries performed in 18 different centers throughout the world. Fifteen patients had complications in the immediate postoperative period, including hemorrhage, infection, and severe headaches, and they underwent reoperation due to persistent seizures, shunting, or hypertension. No deaths occurred.

Age of seizure onset did not predict seizure freedom. Older age, however, had a positive correlation with surgical outcome. Postoperative hemiparesis was not worse than it was before the surgery. Cognitive outcome was not related to age at surgery, side of surgery, or seizure freedom.

The Toronto group suggested that hemispherectomy is more successful if done during infancy, since earlier seizure control helps to preserve the function of the normal hemisphere. [90, 96]

Alternatively, if the patient is not a candidate for a limited resection or hemispherectomy, such as when disease is bilateral, corpus callosotomy can be performed or VNS can be administered. VNS has been shown to be effective for focal seizures; its mechanism of action is a putative increase in CNS inhibitory activity.


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