分析42例儿童结节性硬化伴难治性癫痫的手术治疗情况。

选择2008年4月至2019年3月本院临床及病理明确诊断为结节性硬化伴难治性癫痫患儿42例。所有患儿均行磁共振检查，41例实施长程视频脑电图监测，30例实施基因检测。所有患儿均实施详细术前评估并行直接切除手术或颅内电极热凝手术。

42例患儿中Engel Ⅰ级31例，占73.8%；Ⅱ级6例，占14.3%；Ⅲ级3例，占7.1%；Ⅳ级2例，占4.8%。31例无发作患儿中有脑电图异常19例，其中16例一直有放电，3例在术后1年内脑电图异常，之后正常；其余12例患儿术后复查脑电图正常。1例全面性痉挛发作术后6年无发作。

儿童结节性硬化伴难治性癫痫的致痫灶往往是单一病灶或者有主要局限区域，通过详细术前评估可准确识别出致痫结节，获得良好的手术疗效。

To summarize the surgical treatment of tuberous sclerosis with refractory epilepsy in 42 children.

Forty-two children who were clinically and pathologically diagnosed with tuberous sclerosis with refractory epilepsy in Yuquan Hospital from April 2008 to March 2019 were selected. All children underwent magnetic resonance imaging, 41 underwent long time video electroencephalogram (EEG) monitoring, and 30 underwent genetic examination. All children underwent detailed preoperative evaluation, and either direct resection or intracranial electrode thermocoagulation was then selected.

Among the 42 children, 31 (73.8%) had Engel grade Ⅰ, 6 (14.3%) had grade Ⅱ, 3 (7.1%) had grade Ⅲ, and 2 (4.8%) had grade Ⅳ. Among the 31 children without seizures, 19 had EEG abnormality, among whom 16 cases had discharge, 3 cases had EEG abnormality in 1 year, but returned to normal after 1 year. The EEG of the other 12 children was normal after operation. One case of generalized seizure was free of seizure in 6 years after surgery.

The epileptogenic foci of tuberous sclerosis with refractory epilepsy in children are usually single or have major localized areas. Detailed preoperative evaluation can accurately identify the epileptogenic nodules and help obtain a good surgical effect.