Diagnosis and surgical treatment of tethered cord syndrome accompanied by congenital dermal sinus tract in adults
Received date: 2020-03-21
Online published: 2022-12-19
目的: 总结成人先天性皮窦道(dermal sinus tract, DST)合并脊髓拴系综合征(tethered cord syndrome, TCS)的临床特点、影像学特征及手术治疗经验。方法: 回顾性分析2010年9月至2019年10月经手术治疗的25例成人先天性DST合并TCS患者的临床资料。本组男10例,女15例,年龄18~48岁,平均年龄29.7岁。25例在腰骶部中线附近见皮窦口,周围皮肤变性,临床上以腰腿痛、双下肢麻木无力、大小便功能障碍为主要症状,2例以急性脑脊液漏合并脑膜炎急诊入院。根据术前磁共振(magnetic resonance imaging, MRI)影像了解DST在硬膜下走行、脊髓圆锥位置、硬膜下有无病变、内终丝直径等,显微镜下行DST分离并切除、内终丝切断、TCS松解、脊膜囊重建术,术后患者俯卧位7 d。结果: MRI显示,25例患者脊髓圆锥位置均低于腰2椎体水平,DST远端进入硬膜下构成脊髓牵张因素之一,24例患者见内终丝脂肪浸润增粗,另1例患者终丝增粗直径5 mm牵张脊髓圆锥。手术将DST的异常皮肤、皮下瘘管及硬膜下索条全切除,将增粗终丝离断,松解TCS,2例患者合并硬膜下皮样囊肿,予以分离切除。手术后病理符合皮窦道和终丝结构,患者术后疼痛缓解或消失,双下肢无力及大小便功能障碍逐渐恢复,无手术并发症。随访3个月至9年,平均3.9年,所有患者神经功能完好,复查MRI显示胸腰椎生理曲度完好,随访期间未见TCS复发。结论: 先天性DST合并的成人型TCS均合并内终丝增粗牵张,手术在显微镜下行皮窦道全程分离切除、终丝离断、TCS松解及硬膜重建,手术预后较好。
谢京城 , 陈晓东 , 杨军 . 成人先天性皮窦道脊髓拴系综合征的诊断和治疗[J]. 北京大学学报(医学版), 2022 , 54(6) : 1163 -1166 . DOI: 10.19723/j.issn.1671-167X.2022.06.017
Objective: To summarize the clinical manifestations, imaging characteristics and experience of surgical treatment of tethered cord syndrome (TCS) accompanied by dermal sinus tract (DST) in adulthoods. Methods: The authors retrospectively analyzed a series of 25 adult patients with TCS due to DST that were surgically treated under microscope from September 2010 to October 2019. There were 10 males and 15 females with an average age of 29.7 years (rang, 18-48 years). Characterized cutaneous malformation and dermal sinus were found in the lumbosacral region in all the 25 patients. Clinically, all the patients presented with chronic back and lower-extremity pain, numbness and weakness of lower limbs, and bowel and bladder dysfunction. Two cases were admitted to the emergency room with acute infectious cerebral spinal fluid (CSF) leakage complicated with meningitis. According to magnetic resonance imaging (MRI) images, the subdural course of DST whose traction of the spinal cord, the location of the conus medullaris, the presence of subdural lesions, and the diameter of the internal filum terminale were evaluated. The surgical procedure included separating and excising of the DST, section of the internal filum terminale, detethering of the TCS, and reconstruction of the dural sac under microscopy. The patients remained in prone position in 7 days postoperation. Results: MRI showed that the position of the conus medullaris was lower than the level of lumbar 2 vertebrae, and the distal part of the DST entered the subdural stretched part of the spinal cord, to constitute one of the factors of TCS in all the 25 patients. Twenty patients had fatty infiltration of internal filum terminale and another patient had thickened (approximately 5 mm in diameter) internal filum terminale resulting in tightening the conus medullaris. A total of 25 operations were performed including completely dissection and resection of the DST through the skin down to the subdural space, section of the internal filum terminale, detethering of the TCS, and the subdural dermoid cysts were removed in two patients. There were no postoperative complications. The postoperative pathology was consistent with the structure of the DST and internal filum terminale. The local pain was relieved, and the lower-extremity weakness and bowel and bladder dysfunction were gradually relieved postoperatively. The period of follow-up ranged from 3 months to 9 years (mean, 3.9 years). The neurological function of all the patients was intact, and MRI showed that the physiological curvature of the thoracolumbar spine remained normal. There was no recurrence of TCS observed during the follow-up. Conclusion: The adult TCS accompanied with DST is characterized by typical cutaneous malformation in the lumbosacral region and tethering of the spinal cord. The patients are usually combined with internal filum terminale enlargement tightening of conus medullaris as well. The surgical treatment including totally resection of the DST and section of the internal filum terminale to detethering the TCS at the same time under microscopy. The outcome of surgical treatment is satisfactory.
Key words: Tethered cord syndrome; Dermal sinus tract; Filum terminale; Microsurgery
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