Surgical treatment of epilepsy caused by Sturge-Weber syndrome in children

Objective To summarize the surgical treatments of epilepsy caused by Sturge-Weber syndrome in children. Methods A total of 8 children with Sturge-Weber syndrome were operated from January 2016 to June 2018.There were 2 boys and 6 girls with an average age of 26.25(5-48) months.The age group was under 3 years (n=7) and above 3 years (n=1). The average disease course was 8.98 months (2 days to 2 years). Detailed analysis was performed for disease course, seizure types, imaging findings, electroencephalography (EEG) profiles, surgical timing, surgical approaches, postoperative status of epileptic control and complications, etc.And preoperative evaluations of symptoms, imaging findings, EEG profiles and cognitive functions were also conducted. Results Lobectomy (n=3) and functional hemispherectomy (n=5) were performed.Postoperative outcomes were intracranial infection (n=2), subcutaneous effusion (n=2), lowered muscle strength of contralateral extremity (n=5) and no obvious change (n=3). During a follow-up period of 6 to 30 months, there was no instance of seizure.Based upon the Engel's grade, all children were Grade I and there was no mortality.Muscle strength of contralateral extremity all recovered to preoperative levels at Month 3 postoperatively.Cognitive function evaluations indicated that 8 children had varying degrees of improvement. Conclusions As a common neurocutaneous syndrome in children, Sturge-Weber syndrome is usually characterized as drug-resistant epilepsy.Early surgical intervention is an effective treatment of improving the cognitive function of epileptic children. Key words: Sturge-Weber syndrome; Epilepsy; Surgical treatment; Child