Konigsberg Matthew W, Matsumoto Hiroko, Ball Jacob R, Roye Benjamin D, Vitale Michael G
Department of Pediatric Orthopaedic Surgery, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center, ATTN: Michael Vitale, 3959 Broadway, CHONY 8-N, New York, NY, 10032-3784, USA.
Spine Deform. 2020 Oct;8(5):1093-1097. doi: 10.1007/s43390-020-00107-3. Epub 2020 Apr 6.
Many children with spinal muscular atrophy (SMA) develop progressive spinal deformity, worsening already compromised pulmonary function and global spinal balance. Early results demonstrate that intrathecal administration of nusinersen, a recent FDA-approved drug, improves motor function and ventilator-free survival, necessitating preservation of intrathecal access when considering PSIF. The purpose of this study is to assess medium-term outcomes of a specialized approach for posterior spinal instrumentation and fusion (PSIF) to preserve intrathecal access in patients with SMA.
A retrospective review of patients with SMA undergoing PSIF at a single tertiary academic medical center during a 3-year period was completed. To facilitate intrathecal drug administration, the traditional approach to PSIF was modified to "skip" one or more intervertebral levels at the thoracolumbar junction. Clinical notes and radiographs were reviewed for postoperative outcomes including major coronal curve correction and complications, including loss of correction, hardware failure and surgical revision.
Eight patients were identified, with a mean age of 12.7 ± 1.6 years and follow-up of 4 years. These patients had a mean preoperative major coronal curve of 56.4°, with mean curve correction of 35.2°. At follow-up, no patients experienced rod breakage, loss of correction, or postoperative chronic pain. Only one patient required revision surgery due to bony overgrowth at the skipped level after three and a half years.
Implementing the skip construct approach for PSIF in patients with SMA allows for scoliosis correction without compromising intrathecal drug delivery. Follow-up at 4 years reveals no adverse clinical events, hardware failure or loss of correction.
IV.
许多脊髓性肌萎缩症（SMA）患儿会出现进行性脊柱畸形，这会使本已受损的肺功能和整体脊柱平衡进一步恶化。早期结果表明，鞘内注射诺西那生钠（一种最近获得美国食品药品监督管理局批准的药物）可改善运动功能并提高无呼吸机生存率，因此在考虑后路脊柱内固定融合术（PSIF）时需要保留鞘内给药途径。本研究的目的是评估一种专门的后路脊柱内固定融合术方法在SMA患者中保留鞘内给药途径的中期疗效。
对一家三级学术医疗中心在3年期间接受PSIF的SMA患者进行回顾性研究。为便于鞘内给药，对传统的PSIF方法进行了改良，在胸腰段交界处“跳过”一个或多个椎间隙。回顾临床记录和X线片以评估术后结果，包括主要冠状面弯曲矫正情况和并发症，如矫正丢失、内固定失败和手术翻修。
共纳入8例患者，平均年龄12.7±1.6岁，随访4年。这些患者术前主要冠状面弯曲平均为56.4°，平均弯曲矫正35.2°。随访时，没有患者出现棒体断裂、矫正丢失或术后慢性疼痛。只有1例患者在3年半后因跳过节段的骨质过度生长而需要进行翻修手术。
在SMA患者中采用跳过结构的PSIF方法可在不影响鞘内给药的情况下矫正脊柱侧弯。4年的随访显示没有不良临床事件、内固定失败或矫正丢失。
IV级。
