儿童硬膜囊末端脂肪脊髓脊膜膨出型脊髓拴系综合征的显微手术治疗  

Microsurgical Treatment of Lipomyelomeningocele and Tethered Cord Syndrome at the End of Dural Sac in Children

作　　者：林国中[1] 马长城[1] 王振宇[1] 谢京城[1] 刘彬[1] 陈晓东[1] Lin Guozhong;Ma Changcheng;Wang Zhenyu(Department of Neurosurgery,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院神经外科,北京100191

出　　处：《中国微创外科杂志》2019年第5期398-402,共5页Chinese Journal of Minimally Invasive Surgery

基　　金：首都临床特色应用研究项目(Z171100001017120)

摘　　要：目的总结儿童硬膜囊末端脂肪脊髓脊膜膨出型脊髓拴系综合征的特点,探讨其手术疗效。方法回顾性分析2010年7月～2017年10月35例儿童硬膜囊末端脂肪脊髓脊膜膨出型脊髓拴系综合征的临床资料。年龄6～17岁,平均10. 6岁。均有腰骶部疼痛,双下肢无力32例,下肢畸形17例,二便功能障碍29例。CT均证实腰骶部脊柱裂。MRI表现为脊髓圆锥低位,腰骶部椎管内外脂肪信号。手术在显微镜及电生理监测辅助下进行,切除脂肪瘤,切断终丝,彻底解除拴系。采用视觉模拟评分法(Visual Analogue Scale,VAS)评估患者疼痛情况,Hoffman功能评分标准评估手术疗效。结果35例椎管内髓外脂肪瘤均全切,髓内脂肪瘤次全切除(残余比例<10%)。均将终丝切断,并将脊髓从硬脊膜分离,松解粘连,彻底松解拴系。20例使用自体硬膜行终池重建,15例使用自体硬膜和人工硬膜行终池重建。术后除5例有轻度肛周麻木感外,无其他新发神经功能障碍。病理检查证实病变为脂肪瘤。术后随访3个月～7年,中位数3年,腰骶部疼痛减轻,VAS评分从(5. 83±1. 07)分降到(2. 17±0. 62)分,Hoffman分级均恢复到0级(14例)或Ⅰ级(21例)。无再拴系发生。结论儿童硬膜囊末端脂肪脊髓脊膜膨出型脊髓拴系综合征的治疗包括脂肪瘤切除和拴系松解。通过精细的显微微创治疗,大多疗效较好。Objective To summarize the characteristics of lipomyelomeningocele and tethered cord syndrome at the end of dural sac in children and to explore the effect of operation.Methods The clinical data of 35 children with lipomyelomeningocele and tethered cord syndrome at the end of dural sac from July 2010 to October 2017 were reviewed and analyzed.The age of patients ranged from 6 to 17 years old,with an average of 10.6 years old.The neurological symptoms included lumbosacral pain in 35 cases,lower limbs weakness in 32 cases,lower limb deformity in 17 cases and urination or defecation dysfunction in 29 cases.Lumbosacral spina bifida was confirmed on CT in all the cases.The MRI showed a low position of conus medulla,short T1 and long T2 signals in and out the spinal canal which represented lipoma.Under assistance of microscopy and electrophysiological monitoring,resection of lipoma were performed and the fila terminale was cut off to achieve complete release of the tethered cord.The pain was evaluated by Visual Analogue Scale (VAS).The efficacy of surgery was evaluated by the Hoffman's functional grading scheme for lipomyelomeningocele.Results Intraspinal extramedullary lipomas were totally resected,and intramedullary lipomas were subtotally resected in all the 35 cases.The fila terminale was cut off and the spinal cord was separated from the dural mater in all the 35 cases.Consequently complete release of the tethered cord was achieved.The terminal cistern was reconstructed with autogenous dura in 20 cases while with both artificial and autogeous dura in 15 cases.Postoperatively there was no new-onset neurologic dysfunction except mild numbness around anus in 5 cases.The lesions in all the cases were confirmed as lipomas by pathological examination.The period of follow-up ranged from 3 months to 7 years (median,3 years).The lumbosacral pain was alleviated.The VAS score decreased from (5.83±1.07) points to (2.17±0.62) points.The Hoffman grade recovered to grade 0 (14 cases) or grade Ⅰ(21 cases).There was no retethe

关 键 词：脊髓脊膜膨出 脂肪瘤 脊髓拴系综合征 显微手术 电生理监测 

分 类 号：R726.5[医药卫生—儿科]