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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) : 591 -596. doi: 10.3877/cma.j.issn.1674-0785.2021.08.006

儿童癫痫·临床研究

儿童局灶性皮层发育不良的手术疗效及影响因素分析
诸壬卿1, 张钦2, 沈瑾3, 张毅1, 王敏1, 薛萍1, 周渊峰4, 施伟1, 李昊1, 顾硕2, 赵瑞1,†()   
  1. 1. 201102　上海，复旦大学附属儿科医院神经外科
    2. 201102　上海，复旦大学附属儿科医院放射科
    3. 201102　上海，复旦大学附属儿科医院神经内科
    4. 570206　海口，复旦大学附属儿科医院海南分院神经医学部
  • 收稿日期:2021-05-01 出版日期:2021-08-15
  • 通信作者: 赵瑞
  • 基金资助:
    复旦大学附属儿科医院产学研高新技术转化孵育项目(FDEKCXY01)

Pediatric focal cortical dysplasia: surgical outcome and affecting factors

Renqing Zhu1, Qin Zhang2, Jin Shen3, Yi Zhang1, Min Wang1, Ping Xue1, Yuanfeng Zhou4, Wei Shi1, Hao Li1, Shuo Gu2, Rui Zhao1,†()   

  1. 1. Department of Neurosurgery, Children's Hospital of Fudan University, Shanghai 201102, China
    2. Department of Radiology, Children's Hospital of Fudan University, Shanghai 201102, China
    3. Department of Neurology, Children's Hospital of Fudan University, Shanghai 201102, China
    4. Department of Neurosurgery, Children's Hospital of Fudan University Hainan Hospital, Haikou 570206, China
  • Received:2021-05-01 Published:2021-08-15
  • Corresponding author: Rui Zhao
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引用本文：

诸壬卿, 张钦, 沈瑾, 张毅, 王敏, 薛萍, 周渊峰, 施伟, 李昊, 顾硕, 赵瑞. 儿童局灶性皮层发育不良的手术疗效及影响因素分析[J]. 中华临床医师杂志(电子版), 2021, 15(08): 591-596.

Renqing Zhu, Qin Zhang, Jin Shen, Yi Zhang, Min Wang, Ping Xue, Yuanfeng Zhou, Wei Shi, Hao Li, Shuo Gu, Rui Zhao. Pediatric focal cortical dysplasia: surgical outcome and affecting factors[J]. Chinese Journal of Clinicians(Electronic Edition), 2021, 15(08): 591-596.

目的

总结儿童局灶性皮层发育不良（FCD）导致难治性癫痫病例的临床特点，并分析影响手术疗效的因素。

方法

回顾性分析2015年1月至2020年10月复旦大学附属儿科医院神经外科收治并进行手术的儿童FCD病例资料，对其年龄、性别、病理结果、头颅磁共振结果及术后癫痫发作情况进行分析。

结果

共入组FCD患儿99例，其中男66例，女33例；年龄最小5月龄，最大17岁，平均年龄（6.4±3.8）岁；FCDⅠb型6例，FCDⅡa型26例，FCDⅡb型23例，FCDⅢ型44例；磁共振阳性91例（91.9%），磁共振阴性8例（8.1%）。总体来看，Engel 1级86例（86.9%），2级3例（3.0%），3级6例（6.1%），4级4例（4.0%）；而磁共振阴性的8例中，Engel 1或2级5例（62.5%），Engel 3或4级3例（37.5%），磁共振阴性的患者预后更差（P=0.032）。

结论

儿童FCD导致难治性癫痫治疗以外科手术为主，且效果良好，而磁共振阴性是预后不良的重要因素。

关键词: 儿童癫痫, 局灶性皮层发育不良, 手术疗效, 预后分析
Objective

To summarize the clinical characteristics of pediatric focal cortical dysplasia and discuss the variables affecting surgical outcome.

Methods

A retrospective review was performed on the clinical data of children with a postoperative histological diagnosis of FCD at Department of Neurosurgery, Children's Hospital of Fudan University from January 2015 to October 2020. Age, gender, pathology subtypes, magnetic resonance imaging (MRI) results, and Engel classification after surgery were analyzed.

Results

A total of 99 cases were enrolled, including 66 boys and 33 girls. The mean age of the patients was (6.4±3.8) years. The pathology subtypes could be divided into FCDⅠb (6 cases), FCD Ⅱa (26 cases), FCDⅡb (23 cases) and FCDⅢ(44 cases). Ninety-one (91.9%) cases were MRI positive and eight (8.1%) were negative. Eighty-six (86.9%) cases achieved Engel grade 1, three (3.0%) had grade 2, six (6.1%) cases had grade 3 and four (4.0%) had grade 4. Among the eight MRI-negative cases, only five (62.5%) achieved Engel grade 1 or 2, while three (37.5%) ended up with Engel grade 3 or 4. Chi-square test showed that negative findings on MRI was associated with a poor surgical outcome (P=0.032).

Conclusion

Surgery is the main component in treating FCD, and is highly effective. Negative findings on MRI are the most important predictive factor for worse seizure outcome.

Key words: Pediatric epilepsy, Focal cortical dysplasia, Surgical outcome, Prognosis analysis
表1 儿童FCD导致难治性癫痫不同病理类型与磁共振结果的关系（例）
磁共振 FCDⅠ型 FCDⅡa型 FCDⅡb型 FCDⅢ型 合计
阳性 5 21 21 44 91
阴性 1 5 2 0 8
合计 6 26 23 44 99
表2 儿童FCD导致难治性癫痫不同病理类型与预后的关系（例）
临床预后 FCDⅠ型 FCDⅡa型 FCDⅡb型 FCDⅢ型 合计
Engel 1~2级 5 21 21 44 91
Engel 3~4级 1 5 2 0 8
合计 6 26 23 44 99
表3 儿童局灶性皮层发育不良导致难治性癫痫磁共振结果与预后的关系（例）
磁共振 Engel 1~2级 Engel 3~4级 合计
阳性 84 7 91
阴性 5 3 8
合计 89 10 99
表4 儿童局灶性皮层发育不良导致难治性癫痫手术方式与预后的关系（例）
手术方式 Engel 1~2级 Engel 3~4级 合计
SEEG植入 11 2 13
未行SEEG植入 78 8 86
合计 89 10 99
表5 儿童局灶性皮层发育不良（FCD）导致难治性癫痫二次手术病例临床资料
病理类型 癫痫复发时间（月） 二次手术时间（月） 首次手术方式 二次手术方式 二次术后Engel分级
1 FCDⅢ型 3 16 半球离断术 二次离断术 3级
2 FCDⅠb型 5 10 SEEG+病灶切除 扩大切除术 2级
3 FCDⅡa型 5 9 半球离断术 二次离断术 1级
4 FCDⅢ型 11 30 病灶切除术 扩大切除术 1级
5 FCDⅡa型 15 20 半球离断术 二次离断术 1级
6 FCDⅢ型 6 16 病灶切除术 扩大切除术 1级
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