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Published in: Pediatric Radiology 1/2019

01-01-2019 | Original Article

Ultrasound-guided cervical puncture for nusinersen administration in adolescents

Authors: Carlos B. Ortiz, Kamlesh U. Kukreja, Timothy E. Lotze, Alex Chau

Published in: Pediatric Radiology | Issue 1/2019

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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.
Literature
2.
go back to reference 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 344:540CrossRefPubMed 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 344:540CrossRefPubMed
3.
4.
go back to reference Mercuri E, Bertini E, Iannaccone ST (2012) Childhood spinal muscular atrophy: controversies and challenges. Lancet Neurol 11:443–452CrossRefPubMed Mercuri E, Bertini E, Iannaccone ST (2012) Childhood spinal muscular atrophy: controversies and challenges. Lancet Neurol 11:443–452CrossRefPubMed
5.
go back to reference Haché M, Swoboda KJ, Sethna N et al (2016) Intrathecal injections in children with spinal muscular atrophy: nusinersen clinical trial experience. J Child Neurol 31:899–906CrossRefPubMedPubMedCentral Haché M, Swoboda KJ, Sethna N et al (2016) Intrathecal injections in children with spinal muscular atrophy: nusinersen clinical trial experience. J Child Neurol 31:899–906CrossRefPubMedPubMedCentral
6.
go back to reference Yousem DM, Gujar SK (2009) Are C1–2 punctures for routine cervical myelography below the standard of care? AJNR Am J Neuroradiol 30:1360–1363CrossRefPubMed Yousem DM, Gujar SK (2009) Are C1–2 punctures for routine cervical myelography below the standard of care? AJNR Am J Neuroradiol 30:1360–1363CrossRefPubMed
7.
go back to reference Grau T, Leipold RW, Conradi R, Martin E (2001) Ultrasound control for presumed difficult epidural puncture. Acta Anaesthesiol Scand 45:766–771CrossRefPubMed Grau T, Leipold RW, Conradi R, Martin E (2001) Ultrasound control for presumed difficult epidural puncture. Acta Anaesthesiol Scand 45:766–771CrossRefPubMed
8.
go back to reference 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 388:3017–3026CrossRefPubMed 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 388:3017–3026CrossRefPubMed
9.
go back to reference Botwin KP, Gruber RD, Bouchlas CG et al (2000) Complications of fluoroscopically guided transforaminal lumbar epidural injections. Arch Phys Med Rehabil 81:1045–1050CrossRefPubMed Botwin KP, Gruber RD, Bouchlas CG et al (2000) Complications of fluoroscopically guided transforaminal lumbar epidural injections. Arch Phys Med Rehabil 81:1045–1050CrossRefPubMed
10.
go back to reference Phillips DP, Roye DP Jr, Farcy JP et al (1990) Surgical treatment of scoliosis in a spinal muscular atrophy population. Spine 15:942–945CrossRefPubMed Phillips DP, Roye DP Jr, Farcy JP et al (1990) Surgical treatment of scoliosis in a spinal muscular atrophy population. Spine 15:942–945CrossRefPubMed
11.
go back to reference 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 48:392–397CrossRefPubMed 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 48:392–397CrossRefPubMed
Metadata
Title
Ultrasound-guided cervical puncture for nusinersen administration in adolescents
Authors
Carlos B. Ortiz
Kamlesh U. Kukreja
Timothy E. Lotze
Alex Chau
Publication date
01-01-2019
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 1/2019
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-018-4240-7

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