摘要：

目的 总结脊髓末端积水合并脊髓栓系综合征的临床表现、影像学特点、诊断与治疗经验。方法与结果 10 例脊髓末端积水合并脊髓栓系综合征患者（1 例为全脊髓积水、8 例为骶管内脊膜囊肿、1 例合并骶部皮毛窦），临床表现为不同程度双下肢无力，尤以肢体远端显著，5 例伴尿道和肛门括约肌功能障碍；5 例存在与脊髓空洞平面相关的感觉障碍平面，尤以浅感觉减退为主。术前MRI 显示脊髓低位、脊髓末端无圆锥结构、脊髓和终丝内异常信号。于手术显微镜下切断终丝、引流髓内积水、切除骶管内脊膜囊肿、松解脊髓栓系。手术成功率达100%，平均手术时间2.15 h、术中出血量220 ml。无一例发生手术相关并发症。术后视觉模拟评分降低，下肢肌力、尿道和肛门括约肌功能改善。术后平均随访6.10 年，McCormick 神经功能分级均达到Ⅰ级。末次随访时复查MRI 显示，脊髓圆锥结构恢复，脊髓末端上升，髓内积水消失，脊柱生理曲度未发生变化。结论 脊髓末端积水合并脊髓栓系综合征临床罕见，主要表现为慢性脊髓功能障碍；MRI 特征性表现为脊髓低位，圆锥结构消失，终丝和髓内呈长T₁、长T₂信号，囊壁无强化。于手术显微镜下切断终丝、引流髓内积水、松解脊髓栓系，效果满意。

关键词: 神经管缺损, 脊髓空洞症, 马尾, 显微外科手术

Abstract:

Objective To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of spinal cord terminal syringomyelia with tethered cord syndrome (TCS).  Methods and Results  Clinical data of 10 patients with spinal cord syringomyelia combined with TCS surgically treated under microscope from January 1999 to March 2014 in our hospital were retrospectively analyzed. There were 3 males and 7 females with average age of 15.06 years old (ranged from 2 to 35 years old). The course of disease ranged from 3 months to 20 years (average 42.17 months). Among those patients, one patient presented hydromyelia, 8 patients suffered from meningeal cyst within the sacral canal, and one patient were concurrent with sacral dermal sinus. The weakness of lower extremities, especially distal limbs, was the main clinical manifestation. Five patients were accompanied with bowel and bladder dysfunction and 5 patients with sensory disturbance below the level of syringomyelia, especially hypesthesia. Preoperative MRI showed conus medullaris disappeared at the end of spinal cord, and there was fluid signal in the lower spinal cord with hypo-intensity signal in T₁WI and hyper-intensity signal in T₂WI without enhancement. All patients underwent surgical procedures. Under microscope, filum terminale was cut off, drainage was performed, meningeal cyst within the sacral canal was removed, and tethered cord was released. The success rate of operations was 100%. The duration of surgery ranged from 1.52 to 3.07 h (average 2.15 h), with average intraoperative blood loss 220 ml (ranged from 100 to 410 ml). The tethering filum had been totally resected and histological examination showed typical filum tissue in all cases. No operative complication was found. Visual Analogue Scale (VAS) score was decreased, and the lower limbs weakness as well as bowel and bladder dysfunction was gradually relieved after operation. The period of follow-up was ranged from 6 months to 14.50 years (average 6.10 years). All patients presented neurological intactness (McCormick gradeⅠ), recovery of conus medullaris, rising of the end of spinal cord, no recurrence of syringomyelia with favorable alignment of spine during follow- up period.  Conclusions  Terminal syringomyelia in the spinal cord with TCS is rarely occurred and characterized by chronic dysfunction of spinal cord and nerve. The manifestation in MRI imaging is characterized by low level of spinal cord, disappearing of conus medullaris, long T₁ and long T₂ signal of filum terminale and spinal cord, and no enhancement of capsule wall. The surgical treatment includes resection of filum terminale, drainage of the cerebrospinal fluid (CSF) in syringomyelia, and de-tethering of the spinal cord under microscope. The outcome is satisfactory.

Key words: Neural tube defects, Syringomyelia, Cauda equina, Microsurgery