Rozkurcze i wyniki rozwojowe dzieci po operacji epilepsji w przebiegu zespołu Sturge'a-Webera

OBJECTIVE: Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder. Up to 90% of children have epilepsy. Epilepsy surgery is a treatment option. However, the seizure and neurodevelopmental outcomes following this are not well defined. This study aimed to determine the outcomes for children undergoing epilepsy surgery for SWS in relation to seizure control, cognition, language, motor function and social communication. METHODS: A retrospective case-note review was completed for children with SWS undergoing epilepsy surgery at Great Ormond Street Hospital from 1993 to 2022. Results of standardised developmental assessments for cognition, language and motor function were analysed. RESULTS: 36 children were operated on (17 hemispherotomies, 13 multilobar surgeries, 6 lobectomy/lesionectomies) with a median follow-up time of 66 months (range 8-198). 77.2% had Engel 1 outcomes (88.2% hemispherotomy, 46.2% multilobar surgery, 83.3% lobectomy/lesionectomy). Older age at surgery (OR for 1-year increase = 1.46, 95% CI 1.08-1.97, p = 0.013), but not type of surgical procedure, was independently associated with worse seizure outcomes. Good seizure control was still achieved in patients who had small residual pial angiomatoses following surgery. Most individual children showed no change or an improvement in their cognitive (67.8%) and language (72%) trajectories, although at group level this was not significant. All children undergoing hemispherotomies had a pre-existing hemiparesis. Following surgery, only 10% of children showed a decline in gross motor functional skills, but 60% had a deterioration in fine motor abilities. SIGNIFICANCE: This is the second largest SWS epilepsy surgery case series. Epilepsy surgery for SWS resulted in good seizure control, with evidence that younger age is associated with improved outcomes. Most children had stabilisation of their developmental trajectory following surgery. Multicentre studies are needed to better define the factors associated with improved seizure and developmental outcomes.