Fluss Rose, Lo Bu Riana, Kobets Andrew J, Gomez Jaime A
1Department of Neurological Surgery, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York.
2Dominik Purpura Department of Neuroscience, Albert Einstein College of Medicine, Bronx, New York; and.
J Neurosurg Case Lessons. 2024 Jan 29;7(5). doi: 10.3171/CASE23574.
Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections.
A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3-4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved.
Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.
与脊髓拴系综合征相关的脊柱侧弯是最难处理的脊柱畸形之一。已经开发了多种手术方法，包括传统的分期和同期手术、脊柱缩短截骨术以及单个椎体切除术。
一名10岁女性患者，患有先天性脊柱后凸侧弯，侧弯进展加重，伴有脊髓拴系和遗尿史。脊柱侧弯已发展为26°的冠状面弯曲和55°的胸腰段后凸。术前脊柱磁共振成像显示L3-4水平之间存在脊髓拴系，L1处有一个大的后凸畸形。患者接受了椎板切除术，术中出现运动信号丢失。在L4椎板切除术、终丝松解术以及T11至L2的后路脊柱融合术之前，先进行了计划中的L1半椎体切除术。术后，患者出现短暂的下肢无力，在接下来的2个月里神经功能从基线水平有所改善。最终，该手术的目标是阻止运动功能的逐渐下降，这一目标已成功实现。
对于这种复杂疾病的治疗，尤其是在合并脊柱侧弯的情况下，仍有许多需要学习的地方。这个病例体现了在试图处理这种临床综合征时存在的复杂治疗模式，还有更多有待了解的地方。
