What are the Sturge-Weber Foundation recommendations for the surgical 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

The Sturge-Weber Foundation recruited a task force to evaluate epilepsy surgery in SWS. The following is a summary of recommendations for such surgery, modified to include VNS:

  • Hemispherectomy should not be done in every patient with SWS solely because of the emphasis on increasingly early surgery; surgery is appropriate only for medically refractory seizures

  • Patients with intractable seizures and very localized lesions should have a limited resection that preserves as much normal tissue as possible

  • Video EEG and structural and functional neuroimaging should be used to define the extent of the lesion and the site of seizure origin

  • Corpus callosotomy is reserved for patients with intractable atonic or tonic seizures leading to secondary injury, if the patients are not candidates for more definitive surgery

  • Surgery should be performed only in a center with an ongoing pediatric epilepsy surgery program

  • Although the benefit of surgery for refractory seizures is accepted generally, additional work is needed to evaluate the natural history of the syndrome and the potential benefits and risks of surgery

  • VNS can be performed in patients who are not candidates for other surgical procedures

Data on the natural history of SWS are not yet sufficient to advocate hemispherectomy unless refractory seizures occur.


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