What is the efficacy of epilepsy surgery 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

Three centers have reported surgery outcomes involving groups of more than 10 patients, including Hoffman et al, from Toronto; Arzimanoglou and Aicardi, from Paris; and the authors' series, from Children's Hospital, Boston (see Table 5, below). Of the 32 patients from these groups who had limited resection, 18 became seizure free, 10 experienced improvement, and 4 had no improvement. Of the 26 patients who were treated with hemispherectomy, 24 became seizure free.

The Toronto researchers found that, in terms of developmental outcome, surgical treatment was preferable to medical therapy. They compared the ultimate developmental outcome (determined by intelligence quotient [IQ] score) of medical and surgical therapies in 50 patients, 17 of whom underwent surgery and 33 of whom were given medical therapy. Normal or borderline functioning was more common after surgical treatment (10 of 17 patients [58.8%]) than after medical therapy (11 of 33 patients [33.3%]). [90]

When surgery is considered, the choice of appropriate procedure must be the main consideration. The epileptogenic region is located in the cortex adjacent to the angioma, and electrocorticography (ECOG) may be needed. However, the LA usually covers the entire hemisphere, and even areas without angioma may be epileptogenic and therefore need resection to achieve seizure control.

Table 5. Surgical Results of Hemispherectomy and Limited Resection from 3 Centers (Open Table in a new window)

Center

Hemispherectomy

Seizure Free

Limited resection

Seizure Free

Improved

Toronto

12

11

11

8

2

Paris

5

5

15

7

8

Boston

9

8

6

3

0

Total

26

24

32

18

10

24 of 26 patients with hemispherectomy - Seizure free

28 of 32 patients with limited resection - Seizure free or improved


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