Orphanet Journal of Rare Diseases (Feb 2023)

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

  • Cuijie Wei,
  • Zhenwei Liang,
  • Ying Wu,
  • Shan Liu,
  • Jianxing Qiu,
  • Lingchao Meng,
  • Chunde Li,
  • Shuang Li,
  • Xinhua Bao,
  • Zhaoxia Wang,
  • Luzeng Chen,
  • Hui Xiong

DOI
https://doi.org/10.1186/s13023-023-02630-8
Journal volume & issue
Vol. 18, no. 1
pp. 1 – 9

Abstract

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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.

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