What is the role 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

A hemispherectomy is performed when an extensive, unilateral epileptogenic region exists. When the epileptogenic region is smaller, a focal cortical resection (ie, a more limited resection) is preferable, since it is less likely to cause a neurological deficit. Hoffman reported that focal disease responds well to resection, ECOG identifies adjacent epileptogenic cortex, and hemispherectomy produces a significant improvement in outcome, leading to normal intelligence and a greater than 90% chance of becoming seizure free.

Residual seizures, however, are more likely to occur after a more limited resection than they are following hemispherectomy. Gilly et al reported a 30% failure rate after limited resection, [95] and in the combined data from 3 surgical centers, 12.5% of those patients who underwent a limited resection had no improvement. (See Table 5, below.)

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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