Objective:To explore the staged surgery for children with supratentorial arteriovenous malformation rupture and hemorrhage and evaluate the prognosis of staged treatment of arteriovenous malformation(AVM)in children.Methods:From January 2015 to December 2018, retrospective analysis was conducted for clinical data of 37 children with supratentorial cerebral arteriovenous malformation hemorrhage undergoing staged surgery.Clinical manifestations, surgical approaches and follow-up outcomes were recorded.Results:Emergency operation in the first stage was performed.There were intracranial hematoma evacuation( n=27)and extraventricular drainage( n=10). There was no mortality.After stabling, computed tomographic angiography(CTA)or digital subtraction angiography(DSA)was examined and the results were graded according to the Spetzler-Martin scheme.The grades were Ⅰ( n=12), Ⅱ( n=16)and Ⅲ( n=9). AVM resection was performed at Week 2 after an initial operation.DSA indicated that AVMs were completely removed at Month 6.Glasgow outcome scale(GOS)score at the end of follow-up was 5( n=32, 86.5%)and 4( n=5, 13.5%). No hemorrhage occurred during a follow-up period of(6-48)months. Conclusion:When preoperative cerebrovascular imaging cannot be completed due to certain conditions, staged treatment of supratentorial cerebral arteriovenous malformation rupture during acute phase is a feasible option for achieving a better prognosis with less neurological dysfunction in children.

目的 结合文献探讨胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNET)的临床特点及治疗策略.方法 回顾性分析苏州大学附属儿童医院神经外科2017年12月至2018年5月收治的5例胚胎发育不良性神经上皮肿瘤患儿临床资料,结合文献复习,总结胚胎发育不良性神经上皮肿瘤的临床表现、影像学特点、分类、治疗方法和预后.结果 5例患儿中4例表现为癫痫发作,1例表现为头痛、嗜睡等脑积水症状.术前CT和磁共振检查诊断为胚胎发育不良性神经上皮肿瘤,按影像学特点分类并经手术及病理检查确诊.根据影像学特点,5例中DNET Ⅰ型1例,Ⅱ型3例,Ⅲ型1例.1例Ⅰ型患儿行单纯额叶病灶切除术;2例Ⅱ型患儿行颞叶病灶+前内侧颞叶切除术;1例Ⅱ型患儿病灶侵犯功能区及基底节区,予病灶大部切除术;1例Ⅲ型患儿病灶位于脑室内,行脑室镜下肿瘤切除术.术后随访8～14个月,5例患儿Engel分级均为Ⅰ级,1例脑积水患儿术后症状改善.结论 DNET患儿常表现为癫痫,也可表现为脑积水等症状,临床表现与影像学分型相关,大部分患儿经单纯肿瘤切除或包含肿瘤的脑叶切除可获得良好治疗效果.

神经管连接部发育缺陷(JNTD)是一种较为罕见的中枢神经系统先天畸形，主要表现为脊髓形态和功能不连续。检索国内外文献仅见个案报道。本文报道了1例JNTD伴皮毛窦的患儿，行皮毛窦切除术+脊髓拴系松解术，术中证实为JNTD，术后随访3个月，术后无手术相关并发症及新发神经系统症状。

问题导向式教学模式作为常用的教学范式,被广泛应用于神经外科临床教学之中.为进一步细化教学实施活动,明确教学路径,文章以问题为导向、以任务为驱动,以学生为中心,多主体共同参与的具有连续性和拓展性的深度教学模式,为临床教学实践提供参考.

目的 探讨神经电生理监测技术在儿童脑干胶质瘤手术中的应用价值.方法 回顾性分析苏州大学附属儿童医院神经外科2016年1月至2021年9月期间行术中神经电生理监测下肿瘤切除术的18例儿童脑干胶质瘤临床资料.患儿年龄1～14岁,术中实施监测项目包括脑干图(map-ping)、体感 诱发电位(somatosensory evoked potential,SSEP)、运动诱发电位(motor evoked potential,MEP)、脑干听觉诱发电位(brainstem auditory evoked potential,BAEP)和肌电图(electromyography,EMG)等.观察围手术期并发症及术后6个月至6年的随访情况,记录肿瘤的影像学变化及患儿神经功能变化.结果 18例患儿在术中神经电生理监测技术下成功实施手术,无一例围手术期死亡病例.术后偏瘫加重2例,经1个月康复治疗后恢复至术前水平.Karnofsky功能状态评分(KPS)由术前的(73.89±21.46)分提升至术后1个月(放化疗前)的(86.11±9.79)分,差异有统计学意义(t=3.96,P＜0.01).结论 儿童脑干胶质瘤手术中辅助神经电生理监测技术具有较好的可行性和稳定性.术中神经电生理监测可有效提高脑干胶质瘤手术的安全性,有利于改善患儿长期预后.

Objective:To investigate the therapeutic outcomes of surgical treatment of dural terminal cystoid meningocele (DTCM) in children.Methods:From January 2015 to January 2021, 11 children with DTCM underwent surgical treatment in the Department of Neurosurgery of the Children′s Hospital of Soochow University, of which 8 cases underwent cutting of the terminal filum + resection of intraspinal cyst + repair of terminal dural defect, 2 cases only underwent cutting of the terminal filum, 1 case underwent cutting of the terminal filum + partial resection of cyst wall + fat packing of cyst cavity. We retrospectively observed the clinical and imaging improvement of the children′s conditions.Results:None of the children had complications such as infection and cerebrospinal fluid leakage after surgery. The postoperative follow-up period was 3 months to 5 years and 4 months (median: 3 years and 6 months), and none of the patients developed new neurological symptoms. The original symptoms of 4 children with preoperative symptoms were significantly improved in 3 cases. Re-examination of MRI 3 to 6 months after surgery showed that all the children had cut terminal filum. In 8 patients who underwent repair of the terminal dural defect, postoperative MRI showed that the sacral cysts disappeared. In the patient who underwent cutting of the terminal filum + partial resection of cyst wall + fat packing of cystic cavity, postoperative MRI showed that the cyst was replaced by packed fat tissue. In 3 children with preoperative extreme dilation of the central canal of distal spinal cord and terminal filum, postoperative MRI showed that the dilation was significantly improved.Conclusion:Surgical treatment of DTCM in children is safe, which can improve the clinical symptoms and imaging changes of the children to a certain extent.

目的 总结软骨发育不全(ACH)合并脑积水患儿的诊治经验.方法 回顾性分析经基因检测确诊的5例ACH合并脑积水的临床资料,并结合相关文献进行分析.结果 1例外院接受脑室-腹腔分流术治疗脑积水,术后随访3年,脑积水未进展,未出现分流相关并发症.另外4例均采用随访观察,随访时间2～12年,其中2例单纯合并脑积水病人的脑室大小稳定,无脑积水相关临床表现;1例脑积水合并枕骨大孔狭窄病人的脑室大小稳定,枕骨大孔狭窄及颈髓受压自发改善;1例脑积水合并脊髓信号异常及脊柱畸形,未出现新发临床症状.结论 ACH患儿往往合并脑积水,但脑积水有自发趋于稳定甚至改善的可能,一段时间的随访观察可能会获得较好的临床预后.

Objective To investigate the expression of NF-kappaB1 ( NF-κB1 ) in human glioma and glioma cell lines SHG44 and U87 and its correlation with apoptosis , which lay a theoretical foundation for the mechanism of NF-κB1 in human glioma .Methods qRT-PCR was used to determine the expression of NF-κB1 in noncancerous brain tissue and human glioma tissue samples respectively .Chemical synthesis of NF-κB1 silencing expression plasmids was transfected into the cell lines by the Lipofectamine 2000 liposome .The effects of NF-κB1 on the apoptosis of glioma cell lines SHG44 and U87 were detected by flow cytometry .The expression of NF-κB1 and BCL2 proteins were detected by western blot .Results qRT-PCR results showed that the expression of NF-κB1 in human glioma and glioma cell lines SHG 44 and U87 were significantly higher than those in non-brain tissue and increased with the increase of tumor malignancy .The expression of BCL2 was the same in the same human glioma specimen .The results of flow cytometry showed that inhibition of NF-κB1 expression significantly promoted the apoptosis of SHG 44 and U87 in human glioma cells(P<0.05).Western blot analysis further confirmed that the expression of BCL 2 protein was also decreased after inhibition of NF-κB1 protein expression .Conclusion NF-κB1 inhibits early apoptosis of glioma cells by promoting the expression of BCL 2.

Objective By exploring the expression of microRNA (miR)-140-5p in human glioma tissues,to investigate the effects of miR-140-5p on proliferation apoptosis and invasion of glioma cell lines.Methods By real-time fluorescence quantitative PCR (Real-time PCR) detects the expression ofmiR-140-5p in human glioma tissues and U251,U87MG cell lines.miR-140-5p mimics were transfected into U251 and U87MG cells by Lipofectamine 2000.At the same time,the blank control group and negative control group were established.The expression level of miR-140-5p was identified by Real-time PCR.After transfection,transfection efficiency was observed by fluorescent microscope.The ability of cell proliferation was detected by cell counting kit-8 (CCK-8);cell cycle and apoptosis were detected by flow cytometry;the cell invasive ability was evaluated by Transwell assay.Results Real-time PCR showed that miR-140-5p expression in non-cancerous brain tissues was lower than in human brain glioma tissues and glioma cell lines,while it decreased with the increasing degree of malignancy in glioma (the relative expression of non-cancerous brain tissues,low grade and high grade glioma were 1.013,0.375 and 1.130,P ＜0.01).After over-expression of miR-140-5p,the U87MG and U251 cells proliferation and invasion ability were obviously inhibited (P ＜ 0.01).Among them,the relative proliferation rate were 61.22％ and 58.44％,the cell nember of who cross cell membrane fell by almost half.By flow cytometry on cell cycle analysis,results showed that cell cycle was arrested in G0/G1 phase and S phase was decreased (P ＜ 0.01).The cell apoptosis was significantly increased larger than 12％ after over-expression of miR-140-5 p (P ＜ 0.01).Conclusion miR-140-5 p in glioma tissues is low expression,over-expression of miR-140-5p can inhibit glioma cell proliferation,arrest cell cycle,promote its apoptosis,and can effectively inhibit the invasiveness.