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学龄儿童、青少年及青年成人脊髓栓系综合征的外科治疗及预后

Surgical management and outcome of tethered cord syndrome in school-aged children, adolescents, and young adults.

作者信息

Kang Joon-Ki, Yoon Kang-Jun, Ha Sang-Su, Lee Il-Woo, Jeun Sin-Soo, Kang Seok-Gu

机构信息

Department of Neurosurgery, Kangnam St. Peter's Hospital, Seoul, Korea.

出版信息

J Korean Neurosurg Soc. 2009 Nov;46(5):468-71. doi: 10.3340/jkns.2009.46.5.468. Epub 2009 Nov 30.

Abstract

OBJECTIVE

The adolescent presentation of tethered cord syndrome (TCS) is well-recognized, but continues to pose significant diagnostic and management controversies. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS.

METHODS

All 83 patients with a lipomyelomeningocele (LMMC) underwent untethering surgery for caudal cord tethering between 1987 and 2007. The clinical charts and follow-up data were reviewed. Of these patients, 24 school-aged children, adolescents, and young adults with TCS were studied with respect to the clinical, radiologic, pathologic features, and surgical outcomes.

RESULTS

Untethering procedures were performed in 24 patients (age range, 7-25 years) for TCS of various origins (lipoma, lipomyelomeningocele, and tight filum terminale). Specific circumstances involving additional tugging of the already tight conus, and direct trauma to the back precipitated the onset of symptom in 50% of the patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities, as well as bladder and bowel dysfunction, were also common findings, but progressive foot and spinal deformities were noted less frequently. The most common tethered lesions were intradural lipomas, thickened filum and fibrous band adhesions into the placode sac. The surgical outcome was gratifying in relation to pain and motor weakness, but disappointing with respect to resolution of bowel and bladder dysfunction. Of the 24 patients with TCS, pre-operative deficits improved after surgery in 14 (58.3%), remained stable in 8 (33.4%), and worsened in 2 (8.3%).

CONCLUSION

The pathologic lesions of tethered cord syndrome in school-aged children, adolescents, and young adults, are mostly intradural lipomas and tight filum. It is suggested that the degree of cord traction results in neurologic dysfunction in late life due to abnormal tension, aggravated by trauma or repeated tugging of the conus during exercise. Early diagnosis and adequate surgical release might be the keys to the successful outcome in school-aged children, adolescents, and young adults with TCS.

摘要

目的

脊髓栓系综合征(TCS)在青少年中的表现已得到充分认识,但在诊断和治疗方面仍存在重大争议。作者对24例患有TCS的学龄儿童、青少年和青年成人进行手术干预后的临床结果进行了回顾性研究。

方法

1987年至2007年间,所有83例脂肪瘤型脊髓脊膜膨出(LMMC)患者均接受了尾侧脊髓栓系松解手术。回顾了临床病历和随访数据。其中,对24例患有TCS的学龄儿童、青少年和青年成人的临床、放射学、病理学特征及手术结果进行了研究。

结果

24例患者(年龄范围7 - 25岁)因各种原因(脂肪瘤、脂肪瘤型脊髓脊膜膨出和终丝紧张)导致的TCS接受了松解手术。50%的患者症状发作是由涉及对已紧张的圆锥体额外牵拉的特殊情况以及背部直接创伤引起的。弥漫性且非皮节性腿痛,常放射至肛门直肠区域,是最常见的首发症状。下肢进行性感觉运动功能障碍以及膀胱和肠道功能障碍也是常见表现,但足部和脊柱的进行性畸形较少见。最常见的栓系病变是硬膜内脂肪瘤、增厚的终丝和进入神经板囊的纤维带粘连。手术在缓解疼痛和运动无力方面效果良好,但在解决膀胱和肠道功能障碍方面令人失望。24例TCS患者中,术后14例(58.3%)术前缺陷得到改善,8例(33.4%)保持稳定,2例(8.3%)恶化。

结论

学龄儿童、青少年和青年成人脊髓栓系综合征的病理病变主要是硬膜内脂肪瘤和紧张的终丝。提示脊髓牵拉程度因异常张力导致晚年神经功能障碍,运动时圆锥体受到创伤或反复牵拉会加重这种情况。早期诊断和充分的手术松解可能是患有TCS的学龄儿童、青少年和青年成人取得成功治疗结果的关键。

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本文引用的文献

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Pediatr Neurosurg. 2007;43(3):236-48. doi: 10.1159/000098836.
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