1Department of Neurosurgery, University Hospital of Basel.
2Division of Pediatric Neurology, University Children's Hospital of Basel.
Neurosurg Focus. 2022 May;52(5):E4. doi: 10.3171/2022.2.FOCUS21788.
Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder presenting mostly with a facial port-wine stain and leptomeningeal angiomatosis. More than 85% of the patients are affected by epilepsy by the age of 2 years. Seizure and symptom control is the focus of SWS treatment, since no causal therapy exists yet. For pharmacologically intractable epilepsy, surgery is a treatment option. The aim of this systematic review and meta-analysis was to provide an overview of the literature regarding lesionectomy in SWS with a focus on seizure outcome, complications, and motor and cognitive development.
The PubMed and Embase databases were searched using a systematic search strategy to identify studies on SWS from their inception until 2021. Two independent researchers assessed the studies for inclusion and quality. Outcome measures were seizure outcome, postoperative complications, and motor and cognitive development. Thereafter, a systematic review was conducted, and a meta-analysis was performed for all included cohort studies. Risk of bias was assessed using the Newcastle-Ottawa Scale. Forest plots have been generated for all outcomes; risk ratio was used for pooled outcomes. A p value < 0.05 was considered as statistically significant.
After removal of duplicates, the authors screened 439 articles, of which 9 articles with 150 patients were included. Our case and 5 case reports and 4 retrospective cohort studies were included for systematic review. The latter 4 studies qualified for the meta-analysis. In these 4 articles, 144 patients received surgical treatment: 81 (56%) underwent focal lesionectomy and 63 (44%) hemispherectomy. Pooled outcome analysis for postoperative favorable seizure outcome showed a nonsignificant difference between lesionectomy and hemispherectomy (69.2% vs 87.3%; RR 0.73, 95% CI 0.50-1.08; t = -2.56, p = 0.08). Lesionectomy showed a significantly lower rate for developmental delay and postoperative hemiparesis in comparison with hemispherectomy (29.8% vs 76.3%; RR 0.41, 95% CI 0.28-0.59; z = -4.77, p < 0.0001 and 18.1% vs 100%; RR 0.11, 95% CI 0.06-0.21; z = -6.58, p < 0.0001, respectively).
Based on the limited literature available, lesionectomy leads to a nonsignificant lower seizure control rate, while postoperative developmental or motor deficits are significantly lower compared with hemispherectomy. Therefore, focal lesionectomy remains a valid alternative to hemispherectomy in SWS with a clearly localized epileptogenic area; however, individual case-based decisions in a specialized multidisciplinary team are of paramount importance.
Sturge-Weber 综合征（SWS）是一种罕见的神经皮肤疾病，主要表现为面部葡萄酒色斑和软脑膜血管畸形。超过 85%的患者在 2 岁时就会受到癫痫的影响。由于目前还没有针对病因的治疗方法，因此控制癫痫发作和症状是 SWS 治疗的重点。对于药物难治性癫痫，手术是一种治疗选择。本系统评价和荟萃分析的目的是提供 SWS 中病变切除术的文献综述，重点关注癫痫发作结局、并发症以及运动和认知发育。
使用系统搜索策略在 PubMed 和 Embase 数据库中搜索，以确定从成立到 2021 年的 SWS 研究。两名独立的研究人员评估了纳入研究的质量。结局指标为癫痫发作结局、术后并发症以及运动和认知发育。然后，对所有纳入的队列研究进行了系统评价，并进行了荟萃分析。使用纽卡斯尔-渥太华量表评估偏倚风险。为所有结局生成了森林图；使用汇总比值比（RR）进行汇总结局分析。p 值 < 0.05 被认为具有统计学意义。
去除重复项后，作者筛选出 439 篇文章，其中 9 篇文章中有 150 名患者被纳入。我们的病例和 5 例病例报告和 4 项回顾性队列研究被纳入系统评价。后 4 项研究符合荟萃分析的条件。在这 4 篇文章中，144 名患者接受了手术治疗：81 名（56%）行病灶切除术，63 名（44%）行半脑切除术。术后良好癫痫发作结局的汇总结局分析显示，病灶切除术与半脑切除术之间无显著差异（69.2% vs 87.3%；RR 0.73，95%CI 0.50-1.08；t = -2.56，p = 0.08）。与半脑切除术相比，病灶切除术的发育迟缓发生率和术后偏瘫发生率明显较低（29.8% vs 76.3%；RR 0.41，95%CI 0.28-0.59；z = -4.77，p < 0.0001 和 18.1% vs 100%；RR 0.11，95%CI 0.06-0.21；z = -6.58，p < 0.0001，分别）。
基于现有有限的文献，病灶切除术导致癫痫控制率无显著降低，但与半脑切除术相比，术后发育或运动缺陷明显降低。因此，在具有明确局限性致痫区的 SWS 中，病灶切除术仍然是半脑切除术的有效替代方法；然而，在专门的多学科团队中，基于个体病例的决策至关重要。
