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儿童和青年中与脊髓脊膜膨出、腰骶部脂肪瘤及脂肪脊髓脊膜膨出相关的脊髓拴系综合征的脊柱缩短术

Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults.

作者信息

Aldave Guillermo, Hansen Daniel, Hwang Steven W, Moreno Amee, Briceño Valentina, Jea Andrew

机构信息

Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston, Texas.

Shriners Hospitals for Children, Philadelphia, Pennsylvania; and.

出版信息

J Neurosurg Pediatr. 2017 Jun;19(6):703-710. doi: 10.3171/2017.1.PEDS16533. Epub 2017 Mar 31.

DOI:10.3171/2017.1.PEDS16533
PMID:28362188
Abstract

OBJECTIVE Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique. METHODS The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome. RESULTS Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8-30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26-37 months). Computed tomography-based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and -13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and -12.8%, respectively. CONCLUSIONS Spinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

摘要

目的 脊髓拴系综合征是脊髓异常受牵拉的临床表现,推测会导致机械性损伤、血供受损及脊髓代谢改变。脊髓拴系松解术是脊髓拴系综合征的标准治疗方法。然而,直接松解脊髓存在潜在风险,如脊髓损伤导致新的神经功能缺损、打开硬脊膜引起脑脊液漏以及术后正常瘢痕形成导致脊髓再次拴系。为避免这些风险,作者对原发性和继发性脊髓拴系综合征的儿童及青少年成人应用了脊柱缩短术,并报告治疗结果。作者进行本研究的目的是通过分析他们应用该手术技术的经验,确定脊柱缩短术治疗脊髓拴系综合征的安全性和有效性。

方法 作者回顾性分析了因原发性或继发性脊髓拴系综合征接受脊柱缩短术的儿童及年轻成人的人口统计学和手术数据。

结果 7例因脊髓脊膜膨出、脂肪瘤型脊髓脊膜膨出和过渡性脊柱脂肪瘤导致脊髓拴系综合征的患者接受了脊柱缩短术。1例随访时间不足24个月的患者被排除在进一步分析之外。其中男性3例,女性4例;手术时的平均年龄为16岁(范围8 - 30岁)。患者的临床表现包括疼痛(5例)、无力(4例)和肠道/膀胱功能障碍(4例)。脊柱截骨术最常于L - 1水平进行,使用椎弓根螺钉 - 棒结构在T - 12和L - 2之间进行融合。6例患者进行了椎弓根截骨术,1例患者进行了脊柱切除术。平均随访期为31个月(范围26 - 37个月)。基于计算机断层扫描的影像学结果显示,在最近一次随访时所有病例均实现了牢固融合且无内固定失败。7例患者中有5例(71%)在随访期间报告术前症状有所改善。脊柱侧凸研究学会结局问卷和奥斯维斯特里残疾指数评分的初始值与最近值的平均差异分别为0.26和 - 13%;SRS - 22和ODI的最小临床重要差异分别假定为0.4%和 - 12.8%。

结论 对于脊髓拴系综合征,脊柱缩短术似乎是传统脊髓松解术的一种安全有效的替代方法。

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