目的:对患有Sturge-Weber综合征(SWS)的难治性癫痫患者广泛进行手术,但是关于其有效性的报道有限。本研究旨在分析癫痫发作,电机,以及这些患者的手术认知结果,并确定与结果相关的因素。
方法:这是一项多中心回顾性观察性研究,使用2000年至2020年在中国16个中心接受癫痫手术的SWS和难治性癫痫患者的数据。术后纵向癫痫按Engel类分类,恩格尔一课被认为是无癫痫发作的结果。使用SWS神经评分评估功能(运动和认知)结果,基线和随访之间改善或不变的评分被认为具有稳定的结局.结果使用Kaplan-Meier分析进行分析。多因素Cox回归用于确定与结果相关的因素。
结果:共有214名中位年龄为2.0(四分位距1.2-4.6)岁的患者接受了手术(局灶性切除术,FR[n=87];半球手术,HS[n=127])并完成了中位3.5(1.7-5.0)年的随访。术后1年、2年和5年无癫痫发作的总体估计概率为86.9%(95%CI82.5-91.6),81.4%(95%CI76.1-87.1),和70.7%(95%CI63.3-79.0),分别。术后同时运动稳定的总体估计概率为65.4%(95%CI58.4-71.2),80.2%(95%CI73.8-85.0),和85.7%(95%CI79.5-90.1),分别。在1年、2年和5年认知稳定的总体概率为80.8%(95%CI74.8-85.5),85.1%(95%CI79.3-89.2),和89.5%(95%CI83.8-93.2),分别。FR和HS在确保癫痫发作控制方面均有效。对于不同的HS技术,改良大脑半球切开术与解剖性大脑半球切开术相比,结局相当,但安全性提高.关于FR,部分切除(校正风险比[aHR]11.50,95%CI4.44-29.76),急性术后癫痫(APOS,手术后30天内;aHR10.33,95%CI3.94-27.12),和全身发作(aHR3.09,95%CI1.37-6.94)与持续发作相关.对于HS,癫痫持续发作与APOS相关(aHR27.61,9.92-76.89),全身性癫痫发作(AHR7.95,2.74-23.05),发作频率≥30次/月(aHR4.76,1.27-17.87),和手术年龄≥2岁(aHR3.78,1.51-9.47);运动稳定性与严重运动缺陷(aHR5.23,2.27-12.05)和术后无癫痫发作状态(aHR3.09,1.49-6.45)相关;认知稳定性与术后无癫痫发作状态(aHR2.84,1.39-5.78)和手术年龄<2岁(aHR1.76,1.13-2.75)相关。
结论:FR是治疗SWS患者难治性癫痫的有效选择,与HS的结果相似,与难治性癫痫相关的发病率较低。早期手术治疗(2岁以下)导致更好的结果HS后,但手术年龄影响FR结局的证据不足.这些发现保证了未来的前瞻性多中心队列与国际合作和长期随访,以更好地探索更精确的结果和开发预后预测模型。
方法:这项研究提供了IV类证据,表明在SWS和难治性癫痫患儿中,局部手术切除,大脑半球切除术,或改良半球切开术可改善预后。
OBJECTIVE: Surgery is widely performed for refractory epilepsy in patients with Sturge-Weber syndrome (SWS), but reports on its effectiveness are limited. This study aimed to analyze seizure, motor, and cognitive outcomes of surgery in these patients and to identify factors associated with the outcomes.
METHODS: This was a multicenter retrospective observational study using data from patients with SWS and refractory epilepsy who underwent epilepsy surgery between 2000 and 2020 at 16 centers throughout China. Longitudinal postoperative seizures were classified by Engel class, and Engel class I was regarded as seizure-free outcome. Functional (motor and cognitive) outcomes were evaluated using the SWS neurologic score, and improved or unchanged scores between baseline and follow-up were considered to have stable outcomes. Outcomes were analyzed using Kaplan-Meier analyses. Multivariate Cox regression was used to identify factors associated with outcomes.
RESULTS: A total of 214 patients with a median age of 2.0 (interquartile range 1.2-4.6) years underwent surgery (focal resection, FR [n = 87]; hemisphere surgery, HS [n = 127]) and completed a median of 3.5 (1.7-5.0) years of follow-up. The overall estimated probability for being seizure-free postoperatively at 1, 2, and 5 years was 86.9% (95% CI 82.5-91.6), 81.4% (95% CI 76.1-87.1), and 70.7% (95% CI 63.3-79.0), respectively. The overall estimated probability of being motor stable at the same time post operatively was 65.4% (95% CI 58.4-71.2), 80.2% (95% CI 73.8-85.0), and 85.7% (95% CI 79.5-90.1), respectively. The overall probability for being cognition stable at 1, 2, and 5 years was 80.8% (95% CI 74.8-85.5), 85.1% (95% CI 79.3-89.2), and 89.5% (95% CI 83.8-93.2), respectively. Both FR and HS were effective at ensuring seizure control. For different HS techniques, modified hemispherotomy had comparable outcomes but improved safety compared with anatomical hemispherectomy. Regarding FR, partial resection (adjusted hazard ratio [aHR] 11.50, 95% CI 4.44-29.76), acute postoperative seizure (APOS, within 30 days of surgery; aHR 10.33, 95% CI 3.94-27.12), and generalized seizure (aHR 3.09, 95% CI 1.37-6.94) were associated with seizure persistence. For HS, seizure persistence was associated with APOS (aHR 27.61, 9.92-76.89), generalized seizure (aHR 7.95, 2.74-23.05), seizure frequency ≥30 times/month (aHR 4.76, 1.27-17.87), and surgical age ≥2 years (aHR 3.78, 1.51-9.47); motor stability was associated with severe motor defects (aHR 5.23, 2.27-12.05) and postoperative seizure-free status (aHR 3.09, 1.49-6.45); and cognition stability was associated with postoperative seizure-free status (aHR 2.84, 1.39-5.78) and surgical age <2 years (aHR 1.76, 1.13-2.75).
CONCLUSIONS: FR is a valid option for refractory epilepsy in patients with SWS and has similar outcomes to those of HS, with less morbidity associated with refractory epilepsy. Early surgical treatment (under the age of 2 years) leads to better outcomes after HS, but there is insufficient evidence that surgical age affects FR outcomes. These findings warrant future prospective multicenter cohorts with international cooperation and prolonged follow-up in better exploring more precise outcomes and developing prognostic predictive models.
METHODS: This study provides Class IV evidence that in children with SWS and refractory seizures, surgical resection-focal, hemispherectomy, or modified hemispherotomy-leads to improved outcomes.