Bowman Robin M, Mohan Avinash, Ito Joy, Seibly Jason M, McLone David G
Division of Pediatric Neurosurgery, Children's Memorial Hospital, Chicago, USA.
J Neurosurg Pediatr. 2009 Mar;3(3):181-7. doi: 10.3171/2008.12.PEDS0874.
All children born with a myelomeningocele at the authors' institution undergo aggressive treatment to maintain or improve functional outcome. Consequently, when any neurological, orthopedic, and/or urological changes are noted, a search for the cause is initiated. The most common cause of decline in a child born with a myelomeningocele is shunt malfunction. The second most common cause is tethering of the distal spinal cord at the site of the original back closure. In this report, the authors review the indicators of symptomatic spinal cord tethering and discuss the surgical interventions and outcomes in the children with myelomeningocele who underwent treatment at Children's Memorial Hospital from 1975 to 2008.
Among the 502 children who underwent original closure at Children's Memorial Hospital, a symptomatic tethered spinal cord developed in 114 (23%). Eighty-one patients (71%) have undergone 1 untethering procedure, and 33 patients (29%) have undergone multiple untetherings, for a total of 163 total surgeries. The indicators of symptomatic spinal cord tethering include scoliosis, decline in lower-extremity (LE) motor strength, LE contractures, LE spasticity, gait change, urinary changes, and pain.
Pain has shown the best response to surgical untethering, with 100% of children experiencing postoperative improvement. The results of long-term follow-up (average 12 years, range 1 month-23.3 years) in this cohort demonstrated scoliosis progression after surgical untethering in 52% of patients, with 28% requiring spinal fusion. On the 3-month postoperative manual muscle test, 70% of patients showed improved LE muscle strength compared to preoperatively. Gait was also similarly improved after untethering as evaluated by an orthopedic surgeon. Spasticity improved in two-thirds of the cohort, and as expected, LE contractures were stable (78%) postoperatively, as assessed by orthopedic and rehabilitation medicine specialists. Urologically, 64% of patients showed improvements on postoperative bladder evaluation.
Although this is a clinical outcome study with no control group, the authors' experience has been that tethered cord release is beneficial in maintaining neurological, urological, and orthopedic functioning in children born with a myelomeningocele.
在作者所在机构出生的所有患有脊髓脊膜膨出的儿童都接受积极治疗以维持或改善功能预后。因此，当发现任何神经、骨科和/或泌尿系统变化时，都会着手寻找病因。脊髓脊膜膨出患儿病情恶化的最常见原因是分流器故障。第二常见原因是脊髓远端在原背部闭合部位发生拴系。在本报告中，作者回顾了有症状的脊髓拴系的指标，并讨论了1975年至2008年在儿童纪念医院接受治疗的脊髓脊膜膨出患儿的手术干预措施及预后情况。
在儿童纪念医院接受初次闭合手术的502名儿童中，114名（23%）出现了有症状的脊髓拴系。81名患者（71%）接受了1次松解手术，33名患者（29%）接受了多次松解手术，总共进行了163次手术。有症状的脊髓拴系的指标包括脊柱侧弯、下肢运动力量下降、下肢挛缩、下肢痉挛、步态改变、泌尿系统变化和疼痛。
疼痛对手术松解的反应最佳，100%的患儿术后病情得到改善。该队列的长期随访结果（平均12年，范围1个月至23.3年）显示，52%的患者在手术松解后脊柱侧弯仍有进展，其中28%需要进行脊柱融合术。术后3个月的徒手肌力测试显示，70%的患者与术前相比下肢肌肉力量有所改善。经骨科医生评估，松解术后步态也有类似改善。三分之二的队列痉挛情况有所改善，正如预期的那样，经骨科和康复医学专家评估，术后下肢挛缩情况稳定（78%）。在泌尿系统方面，64%的患者术后膀胱评估结果有所改善。
虽然这是一项没有对照组的临床结局研究，但作者的经验是，脊髓拴系松解术有利于维持脊髓脊膜膨出患儿的神经、泌尿和骨科功能。
