IGCTs所致梗阻性脑积水...期分流装置移除的可行性探讨_王立.pdf

612J Clin Neurosurg，April 2023，Vol 20，No 2书书书DOI:103969/j issn1672-7770 2023 02 019临床研究IGCTs 所致梗阻性脑积水远期分流装置移除的可行性探讨王立，李禄生，梁平，翟瑄，周渝冬，纪文元，周建军，朱涛【摘要】目的探讨可调压抗虹吸分流装置在颅内生殖细胞肿瘤(IGCTs)所致脑积水治疗中的意义及远期移除分流装置的可行性。方法重庆医科大学附属儿童医院神经外科 2013 年 1 月2017 年 12 月间 42例拟诊为 IGCTs 伴梗阻性脑积水患儿，均采用可调压抗虹吸分流装置行脑室腹腔分流术(VPS)，术后予以行诊断性化疗;回顾性分析其中 20 例在肿瘤达到完全缓解后行分流装置移除术的诊疗过程。结果行 VPS 术后，20 例患儿的高颅压症状均获得显著缓解，无症状加重者。术后复查 CT 或 MRI 显示脑室系统正常;眼底检查未见明显损害情况，术前视力模糊者术后视力逐渐恢复正常，现视力与同龄人相仿。治疗过程中未见分流装置堵塞、感染病例，予以诊断性化疗，肿瘤获得完全缓解，6 12 个月后行分流装置夹闭或移除术，随访36 92 个月，20 例患者均未出现颅内高压症状。至随访结束时均已入学，生活质量未见明显影响。结论IGCTs 所致梗阻性脑积水行 VPS 早期可以快速降低颅压，缓解颅内高压症状，降低颅内压持续升高而发生脑疝的风险。术后经诊断性化疗，在肿瘤获得完全缓解后，可行分流装置移除术，降低分流装置堵塞、感染、断裂等风险，避免患儿终生留置体内分流装置，可有效提高患儿生活质量。【关键词】可调压抗虹吸分流装置;颅内生殖细胞瘤;梗阻性脑积水;脑室腹腔分流术;儿童【中图分类号】R739 41【文献标志码】B【文章编号】1672-7770(2023)02-0216-04Feasibility of removal of long-term shunt device for obstructive hydrocephalus caused by IGCTs WANG Li，LI Lu-sheng，LIANG Ping，et al Department of Neurosurgery，Affiliated Childrens Hospital，Chongqing Medical University，Ministry of Education Key Laboratory of Child Developmental Disorders，Chongqing Key Laboratory of Pediatrics，ChinaInternational Science Technology cooperation Base of Child Development Critical Disorders，Chongqing 400014，ChinaCorresponding author:LI Lu-shengAbstract:ObjectiveTo explore the significance of adjustable pressure anti-siphon shunt device in thetreatment of hydrocephalus caused by intracranial germ cell tumor(IGCTs)and the feasibility of long-term removal ofshunt device MethodsFrom January 2013 to December 2017，42 patients suspected of IGCTs with obstructivehydrocephalus admitted to the Department of Neurosurgery，the Affiliated Childrens Hospital of Chongqing MedicalUniversity were treated with ventriculoperitoneal shunt(VPS)and diagnostic chemotherapy Among them，20 caseswere treated with shunt device after the tumor reached complete remission The diagnosis and treatment of the 20cases were analyzed retrospectively ResultsAfter VPS，the symptoms of high intracranial pressure in 20 childrenwere significantly relieved，and the symptoms were not aggravatedPostoperative CT or MRI showed that theventricular system was normal，fundus examination showed no obvious damage，and the postoperative visual acuity ofchildren with blurred vision gradually returned to normal Now their visual acuity was similar to that of their peersNo case of obstruction or infection of shunt device was found during the treatment Diagnostic chemotherapy was givenand the tumor was completely relieved Half a year to 1 year later，the shunt device was clipped or removed Duringthe follow-up of 36 to 92 months，20 children had no symptoms of intracranial hypertension They were enrolled at theend of follow-up，and there was no significant effect on the quality of life ConclusionsVPS in the early stage ofIGCTs-induced obstructive hydrocephalus can quickly reduce intracranial pressure，relieve the symptoms ofintracranial hypertension and reduce the risk of cerebral hernia caused by continuous increase of intracranial pressureAfter diagnostic chemotherapy and complete remission of the tumor，it is feasible to remove the shunt device，reducethe risk of blockage，infection and rupture of the shunt device，and avoid indwelling the shunt device lifelong，whichcan effectively improve the quality of life of the childrenKey words:adjustable pressureanti-siphonshuntdevice;intracranialgermcelltumor;obstructivehydrocephalus;ventricular-abdominal shunt;child基金项目:重庆市技术创新与应用发展专项面上项目(cstc2019jscx-msxmX0143);重庆市卫健委科卫联合科研项目重点项目(2023ZDXM023)作者单位:400014 重庆，重庆医科大学附属儿童医院神经外科，国家儿童健康与疾病临床医学研究中心，儿童发育疾病研究教育部重点实验室，儿科学重庆市重点实验室(王立，李禄生，梁平，翟瑄，周渝冬，纪文元，周建军，朱涛);西北妇女儿童医院神经外科(王立);酉阳县人民医院普通外科(朱涛)通信作者:李禄生颅 内 生 殖 细 胞 肿 瘤(intracranial germ cell tumors，IGCTs)约占所有颅内肿瘤的 1%3%1，是东亚儿童青少年发病率较高的恶性肿瘤。IGCTs 好发于中线部位，就诊时常伴梗阻性脑积水，故需在综合治疗前治疗脑积水。目前公认的 方 法 是 内 镜 下 第 三 脑 室 造 瘘 术(endoscopic thirdventriculostomy，ETV)和脑室腹腔分流术(ventriculoperitonealshunt，VPS);VPS 常用于不适合 ETV 或 ETV 失败的病例。既往认为，VPS 术后往往需终生带管，影响患儿远期生存质量。重庆医科大学附属儿童医院神经外科 2013 年 1 月2017 年 12 月间42 例拟诊为 IGCTs 伴梗阻性脑积水的患儿，应用可调压抗虹吸分流装置行 VPS，其中 20 例在肿瘤达到完全缓解后行分流装置移除术，取得了较好的效果。现对20 例患儿的临床资料进行回顾性分析，以探讨 VPS 对于IGCTs 所致梗阻性脑积水的治疗价值及术后分流装置移除方案的应用前景。1资料与方法1 1一般资料共纳入42 例拟诊为 IGCTs 伴梗阻性脑积水的患儿，应用可调压抗虹吸分流装置行 VPS，其中 19 例行ETV，7 例行脑室外引流术(external ventricular drainage，EVD)，其余未行手术治疗。42 例行 VPS 的患儿中，20 例在肿瘤获得完全缓解后行分流装置移除术，其中男 11 例，女 9 例;年龄75 15 岁，平均年龄117 岁;病程7 d 4 个月;位于鞍区或松果体区 IGCTs 主要临床表现为头痛、呕吐 12 例，视力模糊4 例，双眼斜视伴平衡障碍 4 例。本研究已获得医院医学伦理委员会批准，患儿法定监护人均签署知情同意书。1 2影像学表现松果体区及鞍区生殖细胞瘤在 CT 上表现为圆形、椭圆形或不规则形的等密度或稍高密度影;MRI 显像肿瘤实质部分为 T1WI 呈等或稍低信号，T2WI 等或稍高信号，少数呈混杂信号，均伴双侧侧脑室及第三脑室扩大(图1)。图 1术前影像学检查结果1 3手术治疗应用可调压抗虹吸分流装置行常规 VPS手术治疗，术中测压30 cmH2O 12 例，30 cmH2O 8 例。术中调压阀设定压力为 13 20 cmH2O(调节至患儿可耐受最高颅内压)。分流装置移除手术采用静脉吸入复合麻醉方案;麻醉显效后，取平卧位，于手术原切口切开皮肤全层，显露脑室段硬脑膜孔，分离并显露阀门及储液囊，夹闭分流装置脑室段，于脑室段、储液囊间剪断;调整体位为头高位，顺分流装置方向缓慢拔出分流管，修剪腱膜覆盖于硬脑膜孔，严密缝合，未见脑脊液流出;同样方式处理腹腔段分流装置，严格止血，缝合皮肤，结束手术。14化/放疗术后行以铂类药物为基础，辅以依托泊苷的化疗方案，间隔 3 周 1 个疗程，连续 2 个疗程后间隔 3 周行颅脑 CT 和 MRI 评估化疗效果。考虑为单纯性生殖细胞瘤患儿继续化疗 4 个疗程。而非生殖细胞瘤性生殖细胞肿瘤(non-germinoma of germ cell tumors，NGGCTs)则