PDF(Pubmed)
Abstract：
UNASSIGNED: To evaluate the safety of growth-friendly instrumentation for early-onset scoliosis (EOS) in patients with spinal muscular atrophy (SMA) type 1 who received disease-modifying treatment (DMT) and analyze short-term efficacy.
UNASSIGNED: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included. SMA types 2 and 3 and patients who do not receive DMTs were excluded. Clinical and radiographic data were collected at preoperative, postoperative, and latest follow-up visits.
UNASSIGNED: Twenty-eight patients (mean follow-up: 16 months (range 2-41)) were included. The mean age at surgery was 60 months (range 29-96). Fifteen were treated with dual magnetically controlled growing rods (MCGR), four with unilateral MCGR and a contralateral guided growth system, three with Vertical Expandable Prosthetic Titanium Rib (VEPTR®) implants, five with self-distracting systems, and one with traditional dual growing rods. The mean amount of correction was 57% (44°± 17) for scoliosis and 83% (13°± 11) for pelvic obliquity. The mean T1-12 height gain during surgery was 31 mm (±16 mm), while the mean T1 S1 height gain was 51 mm (±24 mm), and instrumented growth was observed during follow-up. Five patients (18%) developed six serious adverse events: three surgical site infections, two anchor failures, and one rod fracture, and all required unplanned reoperations. No neurologic complication, difficulty during nusinersen injections, or respiratory decline was recorded.
UNASSIGNED: We report that spinal deformity in this population can be safely treated with growth-friendly instrumentation, with similar complication rates when compared with SMA type 2.
UNASSIGNED: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included. SMA types 2 and 3 and patients who do not receive DMTs were excluded. Clinical and radiographic data were collected at preoperative, postoperative, and latest follow-up visits.
UNASSIGNED: Twenty-eight patients (mean follow-up: 16 months (range 2-41)) were included. The mean age at surgery was 60 months (range 29-96). Fifteen were treated with dual magnetically controlled growing rods (MCGR), four with unilateral MCGR and a contralateral guided growth system, three with Vertical Expandable Prosthetic Titanium Rib (VEPTR®) implants, five with self-distracting systems, and one with traditional dual growing rods. The mean amount of correction was 57% (44°± 17) for scoliosis and 83% (13°± 11) for pelvic obliquity. The mean T1-12 height gain during surgery was 31 mm (±16 mm), while the mean T1 S1 height gain was 51 mm (±24 mm), and instrumented growth was observed during follow-up. Five patients (18%) developed six serious adverse events: three surgical site infections, two anchor failures, and one rod fracture, and all required unplanned reoperations. No neurologic complication, difficulty during nusinersen injections, or respiratory decline was recorded.
UNASSIGNED: We report that spinal deformity in this population can be safely treated with growth-friendly instrumentation, with similar complication rates when compared with SMA type 2.
摘要：
■评估在接受疾病改善治疗（DMT）的1型脊髓性肌萎缩症（SMA）患者中，生长友好型仪器用于早发性脊柱侧凸（EOS）的安全性并分析短期疗效。
■在2017年至2023年之间进行了回顾性搜索。经基因证实的1型SMA患者，因脊柱畸形接受手术治疗并接受DMT（nusinersen，risdiplam,或asemnogeneabeparvovec）被包括在内。2型和3型SMA和未接受DMT的患者被排除。术前收集临床和影像学数据，术后,以及最新的后续访问。
■28例患者（平均随访：16个月（范围2-41））被纳入。手术时的平均年龄为60个月（范围29-96）。用双磁控生长棒(MCGR)处理15个，四个具有单侧MCGR和对侧引导生长系统，三个带有垂直可扩展的人造钛肋骨（VEPTR®）植入物，五个有自我分散注意力的系统，还有一个传统的双重生长棒。脊柱侧凸的平均矫正量为57％（44°±17），骨盆倾斜的平均矫正量为83％（13°±11）。手术期间T1-12的平均身高增加为31毫米（±16毫米），而平均T1S1高度增益为51毫米(±24毫米)，在随访期间观察到仪器生长。5例患者（18%）发生6例严重不良事件：3例手术部位感染，两个锚故障，一根杆断裂，和所有需要计划外的重新操作。没有神经系统并发症,在nusinersen注射期间有困难，或记录呼吸下降。
■我们报告说，该人群的脊柱畸形可以用生长友好的仪器安全治疗，与SMA2型相比，并发症发生率相似。
■在2017年至2023年之间进行了回顾性搜索。经基因证实的1型SMA患者，因脊柱畸形接受手术治疗并接受DMT（nusinersen，risdiplam,或asemnogeneabeparvovec）被包括在内。2型和3型SMA和未接受DMT的患者被排除。术前收集临床和影像学数据，术后,以及最新的后续访问。
■28例患者（平均随访：16个月（范围2-41））被纳入。手术时的平均年龄为60个月（范围29-96）。用双磁控生长棒(MCGR)处理15个，四个具有单侧MCGR和对侧引导生长系统，三个带有垂直可扩展的人造钛肋骨（VEPTR®）植入物，五个有自我分散注意力的系统，还有一个传统的双重生长棒。脊柱侧凸的平均矫正量为57％（44°±17），骨盆倾斜的平均矫正量为83％（13°±11）。手术期间T1-12的平均身高增加为31毫米（±16毫米），而平均T1S1高度增益为51毫米(±24毫米)，在随访期间观察到仪器生长。5例患者（18%）发生6例严重不良事件：3例手术部位感染，两个锚故障，一根杆断裂，和所有需要计划外的重新操作。没有神经系统并发症,在nusinersen注射期间有困难，或记录呼吸下降。
■我们报告说，该人群的脊柱畸形可以用生长友好的仪器安全治疗，与SMA2型相比，并发症发生率相似。
