Tran Diem Kieu, Shah Vaibhavi, Muhonen Michael G
Neurosurgery, University of California Irvine, Orange, USA.
Neurosurgery, Childrens Hospital Orange County, Orange, USA.
Cureus. 2020 Aug 2;12(8):e9525. doi: 10.7759/cureus.9525.
Introduction Many patients with the spinal muscular atrophy (SMA) have complex spinal anatomy, secondary to thoraco-lumbar spinal fusions. Their fragile musculoskeletal anatomy potentiates limb and joint injury if conventional spinal fluid access modalities are utilized. This creates a challenge when attempting to deliver intrathecal medications such as nusinersen (Spinraza®). Catheter placement in the cervical subarachnoid space with a caudally directed tip is potentially beneficial. This article describes our experience with Spinraza injections into the thecal space through a suboccipital port. This allowed for simple, chronic, and reliable cerebrospinal fluid (CSF) aspiration and intrathecal injections. Methods A total of 15 patients with SMA and complex spinal anatomy were implanted with a cervical subarachnoid catheter, connected to a suboccipital access port. We retrospectively reviewed the charts of these patients for clinical outcomes and complications. All patients then underwent serial port cannulation, aspiration of CSF, and injection of Spinraza following standard manufacturer dosage guidelines. Results The age range was 3 to 49. Two had type-1 SMA, 10 had type-2 SMA, and three had type-3 SMA. We were able to successfully cannulate the port, aspirate CSF, and inject Spinraza during all access attempts. Two incidents of subcutaneous CSF leaks were resolved through reoperation and one incident of transient CSF leak was resolved without surgical repair. Conclusion Patients with SMA requiring intrathecal injections of Spinraza can be treated safely and efficiently with this novel implantation technique. The complication rates are low and the injection time is dramatically lower than with conventional injection techniques.
引言 许多脊髓性肌萎缩症（SMA）患者由于胸腰椎脊柱融合而具有复杂的脊柱解剖结构。如果采用传统的脊髓液获取方式，其脆弱的肌肉骨骼解剖结构会增加肢体和关节损伤的风险。这在尝试给予鞘内注射药物如诺西那生钠（Spinraza®）时带来了挑战。将导管置于颈段蛛网膜下腔且尖端指向尾端可能有益。本文描述了我们通过枕下端口向鞘内注射Spinraza的经验。这使得脑脊液（CSF）的抽取简单、长期且可靠，以及鞘内注射得以实现。
方法 共有15例具有复杂脊柱解剖结构的SMA患者植入了颈段蛛网膜下腔导管，并连接至枕下接入端口。我们回顾性查阅了这些患者的病历以了解临床结果和并发症情况。随后，所有患者均按照标准的制造商剂量指南进行了连续的端口插管、脑脊液抽取以及Spinraza注射。
结果 年龄范围为3至49岁。其中2例为1型SMA，1０例为2型SMA，3例为3型SMA。在所有的接入尝试中，我们均成功进行了端口插管、抽取脑脊液以及注射Spinraza。通过再次手术解决了2例皮下脑脊液漏事件，1例短暂性脑脊液漏事件未经手术修复即得到解决。
结论 对于需要鞘内注射Spinraza的SMA患者，采用这种新型植入技术可以安全有效地进行治疗。并发症发生率低，且注射时间比传统注射技术显著缩短。
