Department of Anesthesiology, Critical Care & Pain Medicine, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Pediatr Neurol. 2022 Jul;132:33-40. doi: 10.1016/j.pediatrneurol.2022.04.008. Epub 2022 Apr 30.
Spinal deformity and prior spinal fusion pose technical challenges to lumbar puncture (LP) for nusinersen administration for patients with spinal muscular atrophy (SMA). In this retrospective study over two study phases, we evaluated (1) factors associated with difficult LP or unscheduled requirement for image guidance and (2) effectiveness of a triage pathway for selective use of image guidance and nonstandard techniques, particularly for patients with spinal instrumentation/fusion to the sacrum.
With institutional review board approval, electronic health records, imaging, and administrative databases were analyzed for patients receiving nusinersen from January 2012 through September 2021. Descriptive statistics and univariate analyses were used.
From January 2012 to March 2018 (phase 1), among 82 patients with SMA, 461 of 464 (99.4%) LP attempts were successful. Univariate analyses associated difficulty with prior spinal instrumentation, higher body mass index, and severity of the spinal deformity. Based on this experience, starting in April 2018 (phase 2), 125 patients were triaged selectively for ultrasound, fluoroscopy, or Dyna computed tomography. Patients with spinal instrumentation/fusion to the sacrum were treated primarily via intrathecal ports (137 doses) or transforaminal LP (55 doses). From April 2018 through September 2021, 704 of 709 (99.3%) LPs were successful. In total from January 2012 to September 2021, 1415 doses were administered. Over 50% of LPs were performed by neurology nurse practitioners without image guidance. Safety outcomes were excellent.
A stratified approach resulted in successful intrathecal nusinersen delivery and efficient resource allocation for patients with SMA, with or without complex spinal anatomy.
脊柱畸形和既往脊柱融合给脊髓性肌萎缩症（SMA）患者进行诺西那生钠腰椎穿刺（LP）带来了技术挑战。在这项回顾性研究的两个研究阶段中，我们评估了（1）与 LP 困难或需要非计划性影像引导相关的因素，以及（2）用于选择性使用影像引导和非标准技术的分诊途径的有效性，特别是对于存在脊柱内固定/融合至骶骨的患者。
在获得机构审查委员会批准后，我们分析了 2012 年 1 月至 2021 年 9 月期间接受诺西那生钠治疗的患者的电子健康记录、影像学和行政数据库。使用描述性统计和单变量分析。
从 2012 年 1 月至 2018 年 3 月（第 1 阶段），在 82 名 SMA 患者中，464 次 LP 尝试中的 461 次（99.4%）成功。单变量分析将困难与既往脊柱内固定、较高的体重指数和脊柱畸形的严重程度相关联。基于这一经验，从 2018 年 4 月（第 2 阶段）开始，125 名患者被选择性地进行超声、透视或 Dyna CT 检查。对于存在脊柱内固定/融合至骶骨的患者，主要通过鞘内端口（137 剂）或经椎间孔 LP（55 剂）进行治疗。从 2018 年 4 月至 2021 年 9 月，709 次 LP 中的 704 次（99.3%）成功。自 2012 年 1 月至 2021 年 9 月，共进行了 1415 次给药。超过 50%的 LP 由神经科执业护士在没有影像引导的情况下进行。安全性结果极佳。
对于存在 SMA 且具有或不具有复杂脊柱解剖结构的患者，分层方法成功地进行了鞘内诺西那生钠给药，并实现了高效的资源分配。
