Barley Jessica Lauren, Mooney James F, Glazier Steven S, Johnson Tamara, Kornegay Adam L, Turner Robert P, Edwards Jonathan C
Division of Clinical Neurophysiology, Department of Neurosciences, Medical University of South Carolina, Charleston, SC, USA.
J Pediatr Orthop. 2010 Sep;30(6):624-8. doi: 10.1097/BPO.0b013e3181e79041.
: Tethered cord syndrome occurs when the distal spinal cord or filum adheres to adjacent structures resulting in progressive sensorimotor deficits in the lower extremities, fecal and/or urinary incontinence, and musculoskeletal deformities. Tethering of the distal cord may be idiopathic, may be associated with an intraspinal abnormality such as a lipoma, but most commonly the distal spinal cord remnant is adherent to the area of the original dysraphism repair in patients with myelodysplasia. Surgery to untether the cord is indicated in patients with worsening pain symptoms, progressive limb deformity or spasticity, or before any acute correction of an associated spinal deformity. Neurophysiologic intraoperative monitoring is used to minimize the risk of inadvertent nerve root or spinal cord injury during the untethering procedure and to assess any changes in cord function at the time of an associated spinal deformity correction. We present a patient with a lumbar level myelodysplasia, Chiari II malformation, severe scoliosis, and tethered cord that underwent concurrent scoliosis correction and tethered cord syndrome surgery, who demonstrated immediate intraoperative improvement in neurophysiologic responses in a previously flaccid upper extremity after untethering. These monitoring changes correlated with clinical improvements noted by physicians and family postoperatively.
Level IV.
脊髓拴系综合征是指脊髓远端或终丝与相邻结构粘连，导致下肢进行性感觉运动功能障碍、大小便失禁以及肌肉骨骼畸形。脊髓远端的拴系可能是特发性的，可能与脊髓内异常如脂肪瘤有关，但在脊髓发育异常患者中，最常见的是脊髓远端残余部分与原脊柱裂修补区域粘连。对于疼痛症状加重、肢体畸形或痉挛进行性加重的患者，或在对相关脊柱畸形进行任何急性矫正之前，均需进行松解脊髓的手术。术中神经生理监测用于在松解手术过程中尽量降低意外神经根或脊髓损伤的风险，并在矫正相关脊柱畸形时评估脊髓功能的任何变化。我们报告一例患有腰骶部脊髓发育异常、Chiari II型畸形、严重脊柱侧弯和脊髓拴系的患者，该患者同时接受了脊柱侧弯矫正和脊髓拴系综合征手术，在松解后，其先前弛缓的上肢神经生理反应在术中立即得到改善。这些监测变化与医生和家属术后观察到的临床改善情况相关。
四级。
