Ⅰ型脊髓纵裂畸形的显微手术治疗  

Microsurgical treatment of typeⅠsplit cord malformation

作　　者：尚爱加[1] 陶本章 罗天宝[2] 高干 孙梦纯 李士强 SHANG Ai-jia;TAO Ben-zhang;LUO Tian-bao(Department of Neurosurgery,The First Medical Center of Chinese PLA General Hospital,Beijing 100853,China;不详)

机构地区：[1]解放军总医院第一医学中心神经外科,北京100853 [2]清华大学玉泉医院神经外科

出　　处：《临床神经外科杂志》2021年第3期269-274,共6页Journal of Clinical Neurosurgery

基　　金：国家重点研发计划(2018YFC1002500);首都临床特色应用研究与推广(Z171100001017140);解放军总医院科技创新苗圃基金项目(17KMM11);解放军总医院临床科研扶持项目(2017FC-TSYS-2026)。

摘　　要：目的探讨Ⅰ型脊髓纵裂(SCM)的临床特点和手术方法及疗效。方法回顾性分析96例Ⅰ型脊髓纵裂(Pang分型)患者的临床资料。其中男27例,女69例;纵裂位于胸段26例、胸腰段35例、腰段28例、腰骶段5例、骶段2例,3例患者的纵裂为多发。患者均采用显微手术治疗;根据患者的年龄、主要症状以及术中脊髓神经粘连松解程度和终丝张力,采用两种不同术式;其中82例患者行骨嵴切除、粘连松解和硬脊膜成型,同时行终丝切断术;14例患者行骨嵴切除、粘连松解和硬脊膜成型术。采用脊髓拴系Karachi评分量表评价患者手术前后下肢肌力、步态、感觉和大小便功能的改变;并分析影响手术效果和预后的因素。结果本组患者术后随访3个月~10年,平均30个月。结果显示,术后症状明显改善者31例、部分改善者34例、无变化27例、加重4例;术后总有效率为95.8%,改善率为67.7%。结论腰背部多毛是Ⅰ型脊髓纵裂最常见和典型的外观特征,下肢发育不对称和脊柱侧弯是其常见临床表现,疼痛是成人患者较为常见的症状。手术治疗除切除骨性纵隔外,还应去除硬膜囊的卡压和所有引起拴系的因素,达到脊髓和神经的完全松解。儿童Ⅰ型脊髓纵裂患者应及早手术治疗,可以阻止或减轻神经损伤导致的一系列临床症状;对于已出现的症状,儿童患者的疗效优于成人患者。在Ⅰ型脊髓纵裂的各种症状中,手术对疼痛症状的疗效最为理想,对于大小便异常和感觉障碍有一定疗效,而对于下肢的不对称发育的效果欠佳。Objective To discuss the clinical characteristics,surgical methods and therapeutic effect of type I split cord malformation(SCM).Methods The clinical data of 96 patients(27 males,69 females)with type I SCM(according to Pang’s classification)were analyzed retrospectively.There were 26 with thoracic split cord,35 with thoracolumbar one,28 with lumbar one,5 with lumbosacral one,and 2 with sacral one.3 patients had SCM at multiple levels.All patients underwent microsurgery.The surgical methods were classified into two groups against the age,main symptoms,the extent of detethering,and the tension of the filum terminale.Eighty-two patients underwent the microsurgery of resecting the bony septum,detethering spinal cord,meningoplasty,and cutting off the filum terminale,while 14 underwent the similar microsurgery except cutting off the filum terminale.We analyzed the muscle strength of the lower limbs,gait,sensory functions,and bladder and bowel functions preoperatively and postoperatively by Karachi Scale,and valuated the influence factors of surgical effects and prognosis.Results The patients were followed up from 3 months to 10 years postoperatively,with 30 months on average.Thirty-one patients had remarkably released symptoms;thirty-four patients had partially released symptoms;twenty-seven patients had unchanged symptoms;and four patients had deteriorated symptoms.The effective rate of the microsurgery was 95.8%,and the improvement rate was 67.7%.Conclusions Lumbar hairiness is the most common and typical appearance characteristic of type I SCM,and asymmetric lower limbs and scoliosis are also common clinical manifestations.Pain is common in adult patients.Besides resecting the bony septum,neurosurgeons should decompress the dura sac and eliminate all tethering factors in order to detether the spinal cord and nerves radically.Children with type I SCM should be treated as soon as possible,which could prevent or release the clinical symptoms induced by nerve injuries.For the existing symptoms,the therapeutic effect i

关 键 词：脊髓纵裂畸形 脊髓拴系综合征 分型 显微手术 治疗 预后 

分 类 号：R651.2[医药卫生—外科学]