Ultrasound-guided interlaminar approach for nusinersen administration in patients with spinal muscular atrophy with spinal fusion or severe scoliosis

Orphanet J Rare Dis. 2023 Feb 17;18(1):30. doi: 10.1186/s13023-023-02630-8.

Abstract

Background: Intrathecal injection of medications can be challenging in spinal muscular atrophy (SMA) patients with severe scoliosis or after spine surgery. Here we report our experience with real-time ultrasound (US)-guided intrathecal administration of nusinersen in patients with SMA.

Methods: Seven patients (six children and one adult) with either spinal fusion or severe scoliosis were enrolled. We performed intrathecal injections of nusinersen under US guidance. The efficacy and safety of US-guided injection were explored.

Results: Five patients had undergone spinal fusion, while the other two presented severe scoliosis. Success was achieved in 19/20 lumbar punctures (95%), 15 of which were performed through the near-spinous process approach. The intervertebral space with a designated channel was selected for the five postoperative patients, while the interspaces with the smallest rotation angle were chosen for the other two patients with severe scoliosis. In 89.5% (17/19) of the punctures, the number of insertions was no more than two. No major adverse events were observed.

Conclusion: Given its safety and efficacy, real-time US guidance is recommended for SMA patients with spine surgery or severe scoliosis, and the near-spinous process view can be used as a interlaminar puncture approach for US guidance.

Keywords: Nusinersen; Scoliosis; Spinal fusion; Spinal muscular atrophy; Ultrasound guidance.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Child
  • Humans
  • Muscular Atrophy, Spinal* / drug therapy
  • Muscular Atrophy, Spinal* / surgery
  • Scoliosis* / drug therapy
  • Scoliosis* / surgery
  • Spinal Fusion*
  • Ultrasonography, Interventional

Substances

  • nusinersen