Epilepsy Surgery Often Helps Seizures In Children With Sturge-Weber

What was studied

This study looked at children with Sturge-Weber syndrome (SWS) who had epilepsy surgery at Great Ormond Street Hospital between 1993 and 2022. The researchers reviewed past medical records for 36 children.

They examined seizure outcomes after surgery and also analysed results of standardised developmental assessments for cognition, language, and motor function. The surgeries included hemispherotomy, multilobar surgery, and lobectomy or lesionectomy.

What they found

Overall, 77.2% of the children had an Engel 1 seizure outcome after surgery. Engel 1 outcomes were reported in 88.2% after hemispherotomy, 46.2% after multilobar surgery, and 83.3% after lobectomy/lesionectomy. Children who were older at the time of surgery were more likely to have worse seizure outcomes. The type of surgery was not independently associated with seizure outcome in this analysis. Good seizure control was also reported in children who had small residual pial angiomatoses following surgery.

For development, most individual children showed either no change or improvement in their cognitive and language trajectories over time. At the group level, these changes were not statistically significant. Among children who had hemispherotomy, all already had hemiparesis before surgery. After surgery, 10% of children showed a decline in gross motor functional skills, while 60% had deterioration in fine motor abilities.

Limits of the evidence

This was a retrospective review from a single hospital, so it can show associations and patterns but cannot establish that surgery caused the outcomes. The study included 36 children, which is a relatively small sample, especially when divided into different surgery types. Follow-up times varied widely, and the abstract does not provide full detail on how complete developmental testing was for all children.

Because SWS is rare, this is still an important series, but the results may not apply to every child with SWS. The abstract gives limited detail about social communication findings, and group-level developmental changes were not statistically significant, so those results should be interpreted cautiously.

For families and caregivers

For families, this study suggests that epilepsy surgery for SWS was often followed by good seizure control, and younger age at surgery was associated with better seizure outcomes. Many children also showed stable or improved cognitive and language trajectories after surgery.

At the same time, surgery does not remove all risks. Some children had worsening in motor skills, especially fine motor abilities, and outcomes varied between children. These findings may help families discuss possible benefits and trade-offs of surgery with their epilepsy team.

What to watch next

Larger multicentre studies are needed to better define which factors are linked to improved seizure and developmental outcomes after epilepsy surgery for SWS.

Terms in this summary

Sturge-Weber syndrome

A rare condition present from birth that affects blood vessels and can involve the brain, skin, and eyes.

epilepsy surgery

An operation used to try to reduce or control seizures when medicines are not enough.

hemispherotomy

A surgery that disconnects much of one side of the brain to help control seizures.

lobectomy

Surgery to remove one lobe, or section, of the brain.

lesionectomy

Surgery to remove a specific abnormal area in the brain.

hemiparesis

Weakness on one side of the body.

Engel 1

A seizure outcome category indicating the best seizure outcome in this scale, often meaning seizure-free or near seizure-free.

pial angiomatoses

Abnormal blood vessel changes on the surface of the brain seen in Sturge-Weber syndrome.