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

Q4 Medicine

中华小儿外科杂志 Pub Date : 2020-02-15 DOI:10.3760/CMA.J.ISSN.0253-3006.2020.02.009

Xin-ke Xu, Yanping Xie, Jun-liang Li, Cheng Chen, Lin Jinrong, Yu Hongyao, Fangcheng Li

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Abstract

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

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儿童斯特奇-韦伯综合征所致癫痫的外科治疗

目的总结儿童斯特奇-韦伯综合征所致癫痫的外科治疗方法。方法2016年1月至2018年6月对8例斯特奇-韦伯综合征患儿进行手术治疗。男2例，女6例，平均年龄26.25(5 ~ 48)个月。年龄组为3岁以下(n=7)和3岁以上(n=1)。平均病程8.98个月(2天~ 2年)。详细分析病程、发作类型、影像学表现、脑电图(EEG)、手术时机、手术入路、术后癫痫控制情况及并发症等，并对患者的症状、影像学表现、脑电图和认知功能进行术前评估。结果分别行肺叶切除术(3例)和功能半球切除术(5例)。术后结果为颅内感染(n=2)，皮下积液(n=2)，对侧肢体肌力下降(n=5)，无明显变化(n=3)。随访6 ~ 30个月，无癫痫发作。根据恩格尔等级，所有的孩子都是一级，没有死亡。术后3个月对侧肢体肌力均恢复至术前水平。认知功能评估显示8名儿童有不同程度的改善。结论斯特奇-韦伯综合征是儿童常见的神经皮肤综合征，常以耐药癫痫为特征。早期手术干预是改善癫痫患儿认知功能的有效治疗方法。关键词:斯特奇-韦伯综合征;癫痫;外科治疗;孩子

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来源期刊

中华小儿外科杂志 Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.40

自引率

0.00%

发文量

8707

期刊介绍： Chinese Journal of Pediatric Surgery is an academic journal sponsored by the Chinese Medical Association. It mainly publishes original research papers, reviews and comments in this field. The journal was founded in 1980 and is included in well-known databases such as Peking University Journal (Chinese Journal of Humanities and Social Sciences) and CSCD Chinese Science Citation Database Source Journal (including extended version). It is one of the national key academic journals under the supervision of the China Association for Science and Technology. Chinese Journal of Pediatric Surgery enjoys a high reputation and influence in the academic community. The articles published in this journal have a high academic level and practical value, providing readers with a large number of practical cases and industry information, and have received widespread attention and citations from readers.