Advertisement

Pediatric Radiology

, Volume 49, Issue 1, pp 136–140 | Cite as

Ultrasound-guided cervical puncture for nusinersen administration in adolescents

  • Carlos B. Ortiz
  • Kamlesh U. Kukreja
  • Timothy E. Lotze
  • Alex Chau
Original Article
  • 206 Downloads

Abstract

Background

Spinal muscular atrophy (SMA) is an autosomal-recessive disease affecting motor neurons and is the most common genetic cause of death in infants. Intrathecal nusinersen is the only therapy approved by the U.S. Food and Drug Administration for SMA. Deformities and spinal instrumentation from orthopedic surgeries are common in children with SMA, complicating traditional intrathecal access for nusinersen delivery. Cervical punctures are routinely performed in adults for cervical myelograms and should be considered for children with SMA as a viable form of intrathecal access.

Objective

This retrospective study assessed technical feasibility and complications of ultrasound-guided cervical puncture for nusinersen administration.

Materials and methods

We reviewed 14 consecutive ultrasound-guided cervical punctures for nusinersen administration with local anesthesia. We reviewed technical success and complications.

Results

All procedures were technically successful. There were no major complications. Two minor complications included headaches that resolved by observation within 24 h after the procedure.

Conclusion

Our series describes a successful novel method of ultrasound-guided cervical spine access for intrathecal administration of nusinersen, adding to the armamentarium of procedures for delivering nusinersen to adolescents with challenging lumbar spine access caused by scoliosis and spinal instrumentation. This technique has the advantages of real-time ultrasound guidance and potential avoidance of general anesthesia in children.

Keywords

Adolescents Cervical puncture Nusinersen Spinal muscular atrophy Ultrasound guidance 

Notes

Compliance with ethical standards

Conflicts of interest

None

References

  1. 1.
    Lunn MR, Wang CH (2008) Spinal muscular atrophy. Lancet 371:2120–2133CrossRefGoogle Scholar
  2. 2.
    Brzustowicz LM, Lehner T, Castilla LH et al (1990) Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome 5q1 1.2–13.3. Nature 344:540CrossRefGoogle Scholar
  3. 3.
    Hua Y, Vickers TA, Baker BF et al (2007) Enhancement of SMN2 exon 7 inclusion by antisense oligonucleotides targeting the exon. PLoS Biol 5:e73CrossRefGoogle Scholar
  4. 4.
    Mercuri E, Bertini E, Iannaccone ST (2012) Childhood spinal muscular atrophy: controversies and challenges. Lancet Neurol 11:443–452CrossRefGoogle Scholar
  5. 5.
    Haché M, Swoboda KJ, Sethna N et al (2016) Intrathecal injections in children with spinal muscular atrophy: nusinersen clinical trial experience. J Child Neurol 31:899–906CrossRefGoogle Scholar
  6. 6.
    Yousem DM, Gujar SK (2009) Are C1–2 punctures for routine cervical myelography below the standard of care? AJNR Am J Neuroradiol 30:1360–1363CrossRefGoogle Scholar
  7. 7.
    Grau T, Leipold RW, Conradi R, Martin E (2001) Ultrasound control for presumed difficult epidural puncture. Acta Anaesthesiol Scand 45:766–771CrossRefGoogle Scholar
  8. 8.
    Finkel RS, Chiriboga CA, Vajsar J et al (2016) Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study. Lancet 388:3017–3026CrossRefGoogle Scholar
  9. 9.
    Botwin KP, Gruber RD, Bouchlas CG et al (2000) Complications of fluoroscopically guided transforaminal lumbar epidural injections. Arch Phys Med Rehabil 81:1045–1050CrossRefGoogle Scholar
  10. 10.
    Phillips DP, Roye DP Jr, Farcy JP et al (1990) Surgical treatment of scoliosis in a spinal muscular atrophy population. Spine 15:942–945CrossRefGoogle Scholar
  11. 11.
    Weaver JJ, Natarajan N, Shaw DWW et al (2018) Transforaminal intrathecal delivery of nusinersen using cone-beam computed tomography for children with spinal muscular atrophy and extensive surgical instrumentation: early results of technical success and safety. Pediatr Radiol 48:392–397CrossRefGoogle Scholar
  12. 12.
    Islander G (2013) Anesthesia and spinal muscle atrophy. Paediatr Anaesth 23:804–816CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Carlos B. Ortiz
    • 1
  • Kamlesh U. Kukreja
    • 1
    • 2
  • Timothy E. Lotze
    • 1
    • 3
  • Alex Chau
    • 1
    • 2
  1. 1.Baylor College of MedicineHoustonUSA
  2. 2.Department of Interventional RadiologyTexas Children’s HospitalHoustonUSA
  3. 3.Department of Pediatric NeurologyTexas Children’s HospitalHoustonUSA

Personalised recommendations