From the Department of Neurosurgery (S.W., X.W., J. Zhou, G.L., Y.G.), SanBo Brain Hospital, Capital Medical University; Department of Neurosurgery (S.W., K.Z., J. Zhang), Beijing Tiantan Hospital, Capital Medical University; Pediatric Epilepsy Center (Q.-Z.L., L.C.), Peking University First Hospital, Beijing; Department of Neurosurgery (R.Z.), Children's Hospital of Fudan University, Shanghai; Department of Neurosurgery (X.Z.), Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders; Epilepsy Center (S. Li), Guangdong Sanjiu Brain Hospital, Guangzhou; Department of Neurosurgery (Z.Y.), Xiangya Hospital, Central South University, Changsha, Hunan; Department of Neurosurgery (Y.S.), Xuanwu Hospital, Capital Medical University; Department of Neurosurgery (K.M., Y.L.), Capital Institute of Pediatrics, Beijing; Department of Neurosurgery (J.H.), Huashan Hospital, Fudan University, Shanghai; Department of Epilepsy Center (L.S.), The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangdong; Department of Neurosurgery (H.C.), The First Affiliated Hospital of Anhui Medical University, Hefei; Department of Neurology (X.L.), Affiliated ZhongDa Hospital, Southeast University, Nanjing, Jiangsu; Department of Neurology (J.S.), Affiliated Children's Hospital of Xi'an Jiaotong University, Shaanxi; Department of Neurosurgery (M.Z.), Henan Sanbo Brain Hospital, Zhengzhou; Department of Neurology (M.W., T.L.), SanBo Brain Hospital; and Department of Functional Neurosurgery (J. Zhang), Beijing Neurosurgical Institute, Capital Medical University; Beijing Key Laboratory of Neurostimulation (J. Zhang); Functional Neurosurgery Department (S. Liang), Beijing Children's Hospital, Capital Medical University, National Center for Children's Health; Beijing Key Laboratory of Epilepsy (G.L., Y.G.); and Center of Epilepsy (G.L., Y.G.), Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, China.
Neurology. 2024 Jul 9;103(1):e209525. doi: 10.1212/WNL.0000000000209525. Epub 2024 Jun 14.
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.
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.
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).
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.
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.
对于患有 Sturge-Weber 综合征（SWS）的难治性癫痫患者，手术广泛用于治疗，但关于其疗效的报道有限。本研究旨在分析这些患者手术的癫痫发作、运动和认知结局，并确定与结局相关的因素。
这是一项多中心回顾性观察研究，使用了 2000 年至 2020 年期间在中国 16 个中心接受癫痫手术的 SWS 伴难治性癫痫患者的数据。术后纵向癫痫发作采用 Engel 分级进行分类，Engel 分级 I 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）结局，基线和随访之间评分改善或不变被认为具有稳定的结局。使用 Kaplan-Meier 分析评估结局。采用多变量 Cox 回归分析确定与结局相关的因素。
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（局灶切除术，FR [n=87]；半球切除术，HS [n=127]），并完成了中位 3.5（1.7-5.0）年的随访。术后 1、2 和 5 年无癫痫发作的总体估计概率分别为 86.9%（95%CI 82.5%-91.6%）、81.4%（95%CI 76.1%-87.1%）和 70.7%（95%CI 63.3%-79.0%）。术后运动功能稳定的总体估计概率分别为 65.4%（95%CI 58.4%-71.2%）、80.2%（95%CI 73.8%-85.0%）和 85.7%（95%CI 79.5%-90.1%）。术后 1、2 和 5 年认知功能稳定的总体概率分别为 80.8%（95%CI 74.8%-85.5%）、85.1%（95%CI 79.3%-89.2%）和 89.5%（95%CI 83.8%-93.2%）。FR 和 HS 均能有效控制癫痫发作。对于不同的 HS 技术，改良半球切开术具有相似的疗效，但安全性优于解剖性半球切除术。对于 FR，部分切除（调整后的危险比[aHR]11.50，95%CI 4.44-29.76）、术后急性癫痫发作（APOS，术后 30 天内；aHR 10.33，95%CI 3.94-27.12）和全面性癫痫发作（aHR 3.09，95%CI 1.37-6.94）与癫痫持续存在相关。对于 HS，APOS（aHR 27.61，9.92-76.89）、全面性癫痫发作（aHR 7.95，2.74-23.05）、癫痫发作频率≥30 次/月（aHR 4.76，1.27-17.87）和手术年龄≥2 岁（aHR 3.78，1.51-9.47）与癫痫持续存在相关；运动功能稳定与严重运动缺陷（aHR 5.23，2.27-12.05）和术后无癫痫发作状态（aHR 3.09，1.49-6.45）相关；认知功能稳定与术后无癫痫发作状态（aHR 2.84，1.39-5.78）和手术年龄<2 岁（aHR 1.76，1.13-2.75）相关。
FR 是 SWS 伴难治性癫痫患者的有效治疗选择，其结局与 HS 相似，但与难治性癫痫相关的发病率较低。早期手术治疗（<2 岁）可使 HS 后结局更好，但尚缺乏证据表明手术年龄会影响 FR 结局。这些发现需要未来有国际合作的多中心前瞻性队列研究和更长时间的随访，以更好地探索更精确的结局并开发预后预测模型。
本研究提供了 IV 级证据，表明在患有 SWS 和难治性癫痫的儿童中，手术切除（局灶切除术、半球切除术或改良半球切开术）可改善结局。
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