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中华临床医师杂志(电子版)

中华临床医师杂志(电子版) ›› 2021, Vol. 15 ›› Issue (08) : 578 -583. doi: 10.3877/cma.j.issn.1674-0785.2021.08.004

儿童癫痫·临床研究

1岁及以下Sturge-Weber综合征患儿的半球手术治疗与预后
王澍1, 赵萌1, 潘军红1, 王雄飞1, 张春生1, 马皓伟1, 王梦阳2, 李天富3, 王静2, 周健1, 刘长青1, 栾国明4,†(), 关宇光4,†()   
  1. 1. 100093　北京，首都医科大学三博脑科医院神经外科
    2. 100093　北京，首都医科大学三博脑科医院神经内科
    3. 100093　北京，首都医科大学三博脑科医院神经内科；100093　北京，北京市临床医学研究癫痫病重点实验室；100093　北京，北京脑重大疾病研究院癫痫研究所
    4. 100093　北京，首都医科大学三博脑科医院神经外科；100093　北京，北京市临床医学研究癫痫病重点实验室；100093　北京，北京脑重大疾病研究院癫痫研究所
  • 收稿日期:2021-06-11 出版日期:2021-08-15
  • 通信作者: 栾国明, 关宇光
  • 基金资助:
    国家重点研发计划(2021YFC2401203，2018YFC2000700); 北京市自然科学基金面上项目(7222098)

Hemispheric surgery and outcomes of Sturge-Weber syndrome patients under 1 year old

Shu Wang1, Meng Zhao1, Junhong Pan1, Xiongfei Wang1, Chunsheng Zhang1, Haowei Ma1, Mengyang Wang2, Tianfu Li3, Jing Wang2, Jian Zhou1, Changqing Liu1, Guoming Luan4,†(), Yuguang Guan4,†()   

  1. 1. Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China
    2. Department of Neurology, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China
    3. Department of Neurology, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China; Key Laboratory of Epilepsy in Beijing Clinical Medicine Research, Beijing 100093, China; Epilepsy Institute of Beijing Brain Major Disease Research Institute, Beijing 100093, China
    4. Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China; Key Laboratory of Epilepsy in Beijing Clinical Medicine Research, Beijing 100093, China; Epilepsy Institute of Beijing Brain Major Disease Research Institute, Beijing 100093, China
  • Received:2021-06-11 Published:2021-08-15
  • Corresponding author: Guoming Luan, Yuguang Guan
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引用本文：

王澍, 赵萌, 潘军红, 王雄飞, 张春生, 马皓伟, 王梦阳, 李天富, 王静, 周健, 刘长青, 栾国明, 关宇光. 1岁及以下Sturge-Weber综合征患儿的半球手术治疗与预后[J]. 中华临床医师杂志(电子版), 2021, 15(08): 578-583.

Shu Wang, Meng Zhao, Junhong Pan, Xiongfei Wang, Chunsheng Zhang, Haowei Ma, Mengyang Wang, Tianfu Li, Jing Wang, Jian Zhou, Changqing Liu, Guoming Luan, Yuguang Guan. Hemispheric surgery and outcomes of Sturge-Weber syndrome patients under 1 year old[J]. Chinese Journal of Clinicians(Electronic Edition), 2021, 15(08): 578-583.

目的

总结1岁及以下Sturge-Weber综合征（SWS）患儿的临床特点及半球手术治疗与预后情况，以期为临床治疗提供参考和依据。

方法

连续回顾性收集2009年1月至2018年2月三博脑科医院接受半球手术的SWS患儿共10例，其中4例接受解剖半球切除术，6例接受改良环岛半球离断术，术后2年随访癫痫预后及认知发育预后作为本研究手术预后纳入分析。癫痫预后采取国际抗癫痫联盟（ILAE）癫痫预后标准，认知发育评估采取丹佛发育筛查量表（DDST）。

结果

本研究纳入患儿年龄为3~12个月，平均（8.10±3.44）个月，6例患儿符合药物难治性癫痫合并认知发育异常和/或偏侧运动功能障碍的手术指征，4例患儿符合≥2次癫痫持续状态发作的手术指征，术后2年随访均实现ILAE 1级无癫痫发作，70%的患儿实现了认知发育改善。改良环岛半球离断术与解剖半球切除术手术预后差异无统计学意义（认知发育改善率：83.3% vs 50.0%，P=0.500），但改良环岛半球离断术安全性更优，创伤更小。

结论

半球手术是治疗1岁及以下SWS伴发难治性癫痫或癫痫持续状态患儿的安全有效措施，并对病灶引起的认知发育异常有改善作用。改良环岛半球离断术是传统解剖半球切除术的改良术式，预后相似但更安全、微创。

关键词: Sturge-Weber综合征, 癫痫, 儿童, 手术治疗, 预后
Objective

To analyze the clinical characteristics, hemispheric surgical treatment, and outcomes of Sturge-Weber syndrome (SWS) patients under 1 year old, in order to provide guidance for treatment.

Methods

A total of ten SWS children who underwent hemispheric surgery in Sanbo Brain Hospital from January 2009 to February 2018 were collected. Four patients received anatomical hemispherectomy (AH) and six received modified peri-insular hemispherotomy (MH). The seizure outcome was assessed following the International League against Epilepsy standard (ILAE) and the cognitive outcome was analyzed according to the Denver Developmental Screening Scale-Ⅱ(DDST).

Results

The patients included ranged in age from 3 to 12 months, with an average of (8.10±3.44) months. Six patients met the surgical indications for drug resistant epilepsy with cognitive developmental abnormalities and/or motor dysfunction, and four met the surgical indications for ≥2 times of status epilepticus. After a 2-year follow-up, all of the children achieved ILAE 1 (seizure-free), and 70% of patients achieved cognitive improvement. There was no significant difference in outcomes between AH and MH (cognitive improvement: 83.3% vs 50.0%, P=0.500). MH was safer and less invasive than AH.

Conclusion

Hemispheric surgery is safe and effective for SWS children under 1 year old in terms of both seizures and cognitive function. MH is a modified operation of the traditional AH, and it has similar outcomes with AH but is safer and minimally invasive.

Key words: Sturge-Weber syndrome, Epilepsy, Children, Surgery, Outcome
表1 10例Sturge-Weber综合征患儿术前一般资料特征
编号 年龄（月） 性别 癫痫病程（月） 发作类型 面部血管痣 青光眼 认知发育 MRI
1 12 女 9 CPS 左额面 有 异常 左半球萎缩、异常信号、脑回样强化
2 9 女 7.5 CPS 右眼周 无 异常 右半球萎缩、异常信号、脑回样强化
3 12 男 8.5 CPS、GTCS 右额面 无 异常 右半球萎缩、异常信号、脑回样强化
4 6 男 5 CPS、SE 右额面 有 异常 右半球萎缩、异常信号、脑回样强化
5 6.5 男 6 CPS、GTCS 右额眼周 有 异常 右半球萎缩、异常信号、脑回样强化
6 3 男 2 CPS、SE 左面 无 异常 左半球萎缩、异常信号、脑回样强化
7 4 男 2 CPS、SE 双面 无 异常 右半球萎缩、异常信号、脑回样强化
8 12 女 10 SPS 左面 有 异常 左半球萎缩、异常信号、脑回样强化
9 6 男 5 CPS、SE 右额 无 可疑 右半球萎缩、异常信号、脑回样强化
10 10.5 女 7 CPS 左面 有 异常 左半球萎缩、异常信号、脑回样强化
图1 Sturge-Weber综合征患儿行改良环岛半球离断术术前及术后CT及MRI影像（10号患儿）。图a~c为术前MRI T1增强；图d为术前CT影像；图e为术后CT影像；图f为术后MRI T1增强
表2 10例Sturge-Weber综合征患儿手术及预后情况
编号 手术类别 侧别 出血量（ml） 术后住院时长（d） 癫痫预后（ILAE分级） 认知发育预后（DDST评价）
1 解剖半球切除术 左侧 1000 22 1 改善
2 解剖半球切除术 右侧 750 15 1 未改善
3 改良环岛半球离断术 右侧 650 14 1 改善
4 解剖半球切除术 右侧 800 12 1 改善
5 解剖半球切除术 右侧 600 13 1 未改善
6 改良环岛半球离断术 左侧 600 12 1 改善
7 改良环岛半球离断术 右侧 500 10 1 未改善
8 改良环岛半球离断术 左侧 450 9 1 改善
9 改良环岛半球离断术 右侧 750 10 1 改善
10 改良环岛半球离断术 左侧 550 9 1 改善
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