Hwang Steven W, Thomas Jonathan G, Blumberg Todd J, Whitehead William E, Curry Daniel J, Dauser Robert C, Luerssen Thomas G, Jea Andrew
Department of Neurosurgery, Division of Pediatric Neurosurgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas 32610, USA.
J Neurosurg Pediatr. 2011 Jul;8(1):63-70. doi: 10.3171/2011.4.PEDS1130.
Significant lumbar kyphosis is frequently observed in patients with myelomeningocele and has been associated with increasing functional impairment, decreased abdominal volume, respiratory impairment, discomfort, and skin ulcerations overlying the prominent gibbus. Treatment of severe kyphotic deformities can include kyphectomy, with or without ligation of the thecal sac, with posterior spinal fixation. However, most series have reported a high rate of morbidity and complications associated with surgical intervention for correction of kyphosis in patients with myelomeningocele. The authors describe a technique in which pedicle screw (PS)-only constructs are used without transection of the thecal sac to treat severe kyphosis successfully, with minimal morbidity.
The authors retrospectively reviewed medical records and radiographic images in 2 patients with myelomeningoceles in whom kyphectomies had been performed at the authors' institution between January 2007 and July 2010. They also reviewed the existing literature for case reports or published series of patients with myelomeningocele treated with kyphectomies, to evaluate the outcomes.
Both patients were male and had thoracic-level myelomeningoceles that had been repaired at birth, with associated paraplegia. Neither patient had any significant scoliotic deformity associated with the kyphosis, and both had fixation from T-9 to the ilium, which was performed using PS constructs, along with L1-2 kyphectomies. The patient in Case 1 was 20 years old and was treated for progressive kyphosis and an ulcerated nonhealing wound over the gibbus. The patient in Case 2 was 10 years old and was treated for progressive pain and functional impairment. The 2 patients had a mean correction of 63%, with a mean correction of kyphotic deformity from 136° to 51°. Neither patient developed any complication in the short term postoperatively, whereas published series have reported high complication rates, including wound infection, poor wound healing, CSF leakage, pseudarthrosis, and shunt malfunction.
Severe kyphotic deformities in patients with myelomeningocele can be safely treated using PS-only constructs without ligation of the thecal sac. Further evaluation with a larger sample and longer follow-up are needed to detect any associated complications, such as proximal junctional kyphosis. Further evaluation may also validate whether PS-only constructs permit successful outcomes with a shorter construct and fewer instrumented levels.
脊髓脊膜膨出患者常出现明显的腰椎后凸，这与功能障碍加剧、腹腔容积减小、呼吸功能受损、不适以及突出驼背上方的皮肤溃疡有关。严重后凸畸形的治疗方法可包括椎体切除，可在切断或不切断硬膜囊的情况下进行，并辅以脊柱后路固定。然而，大多数系列报道显示，脊髓脊膜膨出患者接受手术矫正后凸畸形的发病率和并发症发生率较高。作者描述了一种技术，即仅使用椎弓根螺钉（PS）结构，不切断硬膜囊，成功治疗严重后凸畸形，且发病率极低。
作者回顾性分析了2007年1月至2010年7月在作者所在机构接受椎体切除术的2例脊髓脊膜膨出患者的病历和影像学资料。他们还查阅了现有文献中关于脊髓脊膜膨出患者接受椎体切除术的病例报告或已发表系列研究，以评估治疗效果。
两名患者均为男性，患有胸段脊髓脊膜膨出，出生时已修复，伴有截瘫。两名患者均无与后凸相关的明显脊柱侧弯畸形，均采用PS结构进行了从T-9至髂骨的固定，并进行了L1-2椎体切除。病例1患者20岁，因进行性后凸及驼背处溃疡不愈合伤口接受治疗。病例2患者10岁，因进行性疼痛和功能障碍接受治疗。两名患者平均矫正率为63%，后凸畸形平均从136°矫正至51°。两名患者术后短期内均未出现任何并发症，而已发表的系列研究报道的并发症发生率较高，包括伤口感染、伤口愈合不良、脑脊液漏、假关节形成和分流器故障。
脊髓脊膜膨出患者的严重后凸畸形可通过仅使用PS结构且不结扎硬膜囊进行安全治疗。需要通过更大样本和更长随访时间进行进一步评估，以发现任何相关并发症，如近端交界性后凸。进一步评估还可能验证仅使用PS结构是否能通过更短的结构和更少的固定节段获得成功治疗效果。
