早期脑损伤伴局灶性皮质发育不良Ⅲd型患儿的临床、病理学特点及疗效分析  

Clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia Ⅲd in children

作　　者：李岩[1] 李云林[2] 刘永玲[1] 付静[1] Li Yan;Li Yunlin;Liu Yongling;Fu Jing(Department of Pathology,Haidian Hospital of Beijing,Haidian Branch of Third Hospital of Peking University,Beijing 100080,China;Department of Neurosurgery,Children's Hospital,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]北京市海淀医院(北京大学第三医院海淀院区)病理科,北京100080 [2]首都儿科研究所附属儿童医院神经外科,北京100020

出　　处：《中华神经医学杂志》2022年第9期891-896,共6页Chinese Journal of Neuromedicine

摘　　要：目的探讨早期脑损伤伴局灶性皮质发育不良(FCD)Ⅲd型患儿的临床、病理学特点及疗效。方法选择北京市海淀医院(北京大学第三医院海淀院区)及首都儿科研究所附属儿童医院神经外科自2008年1月至2017年7月采用手术治疗的49例癫痫患儿,均经病理确诊为FCDⅢd型。根据早期脑损伤的类型将患儿分为7组:脑出血组(13例)、中枢神经系统感染性疾病组(19例)、脑梗死组(1例)、产时缺氧组(5例)、2种脑损伤类型组(2例)、高热惊厥组(6例)、脑外伤组(3例)。术后2年采用Engel分级评价患儿的手术疗效,回顾性分析患儿的临床和病理学特点。结果患儿脑损伤年龄为0 d~3.8岁,癫痫发作年龄为4.0~5.3岁,手术年龄为7.0~10.2岁。脑出血组患儿多脑叶损伤的发生率(13/13)大于中枢神经系统感染性疾病组(7/19)、高热惊厥组(3/6),差异均有统计学意义(P<0.05)。术后2年时,脑出血组患儿Engel分级Ⅰ级者占比(11/13)高于中枢神经系统感染性疾病组(8/19),差异有统计学意义(P<0.05)。产时缺氧组患儿术后Engel分级Ⅰ级、Ⅱ级4例。所有患儿均具有早期脑损伤后的病理改变及FCD的病理特点。结论早期脑损伤类型为脑出血、产时缺氧的FCDⅢd型患儿手术疗效好,可能与早期脑损伤发生早、病理改变更为明确清晰、病灶与周围脑组织分界清相关。Objective To investigate the clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia(FCD)Ⅲd in children.Methods Forty-nine children with epilepsy accepted surgical treatment in our hospital from January 2008 to July 2017 were chosen in our study;these patients were pathologically diagnosed as having FCD Ⅲd.These children were divided into 7 groups according to different brain injuries:cerebral hemorrhage(n=13),central nervous system infectious disease(n=19),cerebral infarction(n=1),intrapartum hypoxia(n=5),two kinds of early brain injury(n=2),febrile seizure(n=6),traumatic brain injury(n=3).Two years after surgery,Engel grading was used to evaluate the surgical efficacy of the children.The clinical and pathological characteristics of these children were retrospectively analyzed.Results The average age of brain injury was 0 d-3.8 years,the average age of epilepsy was 4-5.3 years,and the average age of surgery was 7-10.2 years.The incidence of multiple lobe brain injury in the cerebral hemorrhage group(100%,13/13)was significantly higher than that in the central nervous system infectious diseases group(36.8%,7/19,)and febrile seizure group(50%,3/6,P<0.05).There was a significant difference in Engel grading Ⅰ ratio between cerebral hemorrhage group(84.6%,11/13)and central nervous system infectious disease group(42.1%,8/19,P<0.05);Engel grading Ⅰ and Ⅱ was noted in 80.0%children(4/5)of the intrapartum hypoxia group.All children had pathological changes and pathological characteristics of FCD after early brain injury.Conclusion FCD Ⅲd children with cerebral hemorrhage or intrapartum hypoxia have good surgical effect,which may be related to the early occurrence of early brain damage,clear pathological changes,and clear boundary between the focus and the surrounding brain tissues.

关 键 词：局灶性皮质发育不良 儿童 临床特点 病理特点 

分 类 号：R742[医药卫生—神经病学与精神病学]