Lumbar laminotomy for the intrathecal administration of nusinersen for spinal muscular atrophy: technical note and outcomes

Restricted access

Nusinersen (Spinraza) is a US Food and Drug Administration–approved intrathecal medication for the treatment of spinal muscular atrophy (SMA). Adult patients with SMA often undergo thoracolumbar fusion to treat neurogenic scoliosis, preventing thecal access. The authors report a laminotomy technique and the ease of intrathecal access in three SMA patients with prior thoracolumbar fusions.

Patients were positioned in the lateral decubitus position or prone. Lumbar laminotomy was performed below the conus, between the lateral longitudinal rods, to preserve mechanical stability. Fluoroscopy provided real-time identification of instruments. Hardware was contoured with a carbide drill bit to develop the surgical window. Fiducial screws were placed along the perimeter for demarcation. Sublaminar wire removal caused dural defects that were repaired with a layer of dural substitute onlay and sealant. All patients successfully received nusinersen thecal injections via lumbar puncture by an interventional radiologist. Fluoroscopy time ranged from 6 to 36 seconds. No postoperative pseudomeningoceles, cerebrospinal fluid leaks, or wound complications occurred.

For patients with SMA and posterior fusion from prior scoliosis treatment, lumbar laminotomy is an effective method for creating thecal access for the administration of nusinersen.

ABBREVIATIONS CSF = cerebrospinal fluid; SMA = spinal muscular atrophy; SMN = survival motor neuron.

Article Information

Correspondence Ahmed M. Raslan: Oregon Health & Science University, Portland, OR. raslana@ohsu.edu.

INCLUDE WHEN CITING Published online April 19, 2019; DOI: 10.3171/2019.2.SPINE181366.

Disclosures Dr. Karam is a consultant for Akcea, Alnylam, Alexion, Biogen, and Genzyme. Dr. Raslan is a consultant for Abbott.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Left: Anteroposterior lumbosacral radiograph demonstrating the termination of the fusion construct, fiducial screws at the perimeter of the high-sacral laminotomy defect, and lumbar puncture needle placed within the window. Right: The same radiograph as in panel A, edited in relief to highlight the fiducial screws and lumbar puncture needle.

  • View in gallery

    A: Intraoperative lumbosacral lateral fluoroscopy image demonstrating an instrument in the L3 level laminotomy. B: Anteroposterior lumbosacral radiograph demonstrating the surgical window demarcated by the absence of crossing sublaminar wires. C: Lateral radiograph demonstrating interval placement of a lumbar puncture needle into the surgical window.

References

  • 1

    Arnold WDKassar DKissel JT: Spinal muscular atrophy: diagnosis and management in a new therapeutic era. Muscle Nerve 51:1571672015

  • 2

    Butchbach ME: Copy number variations in the survival motor neuron genes: implications for spinal muscular atrophy and other neurodegenerative diseases. Front Mol Biosci 3:72016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Darras BT: Spinal muscular atrophies. Pediatr Clin North Am 62:7437662015

  • 4

    Finkel RSChiriboga CAVajsar JDay JWMontes JDe Vivo DC: Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study. Lancet 388:301730262016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Finkel RSMercuri EDarras BTConnolly AMKuntz NLKirschner J: Nusinersen versus sham control in infantile-onset spinal muscular atrophy. N Engl J Med 377:172317322017

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Holt JBDolan LAWeinstein SL: Outcomes of primary posterior spinal fusion for scoliosis in spinal muscular atrophy: clinical, radiographic, and pulmonary outcomes and complications. J Pediatr Orthop 37:e505e5112017

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 7

    Islander G: Anesthesia and spinal muscle atrophy. Paediatr Anaesth 23:8048162013

  • 8

    Kolb SJKissel JT: Spinal muscular atrophy. Neurol Clin 33:8318462015

  • 9

    Mercuri EDarras BTChiriboga CADay JWCampbell CConnolly AM: Nusinersen versus sham control in later-onset spinal muscular atrophy. N Engl J Med 378:6256352018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Mesfin ASponseller PDLeet AI: Spinal muscular atrophy: manifestations and management. J Am Acad Orthop Surg 20:3934012012

  • 11

    Mousa MAAria DJSchaefer CMKaye RDAbruzzo TABernes SM: A comprehensive institutional overview of intrathecal nusinersen injections for spinal muscular atrophy. Pediatr Radiol 48:179718052018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Ohmura TSaeki SOgiwara KTobita KLing YTorii S: [Pharmacological and clinical profile of spinal muscular atrophy (SMA) therapeutic drug nusinersen (Spinraza®).] Nippon Yakurigaku Zasshi 152:1471592018 (Jpn)

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Ortiz CBKukreja KULotze TEChau A: Ultrasound-guided cervical puncture for nusinersen administration in adolescents. Pediatr Radiol 49:1361402019

  • 14

    Prior TWFinanger E: Spinal muscular atrophy in Adam MPArdinger HHPagon RAWallace SEBean LJHStephens K (eds): GeneReviews®. Seattle: University of Washington1993

    • Search Google Scholar
    • Export Citation
  • 15

    Robinson DGalasko CSDelaney CWilliamson JBBarrie JL: Scoliosis and lung function in spinal muscular atrophy. Eur Spine J 4:2682731995

  • 16

    Rodillo EMarini MLHeckmatt JZDubowitz V: Scoliosis in spinal muscular atrophy: review of 63 cases. J Child Neurol 4:1181231989

  • 17

    Schmalbruch HHaase G: Spinal muscular atrophy: present state. Brain Pathol 11:2312472001

  • 18

    Schroth MK: Special considerations in the respiratory management of spinal muscular atrophy. Pediatrics 123 (Suppl 4):S245S2492009

  • 19

    Sporer SMSmith BG: Hip dislocation in patients with spinal muscular atrophy. J Pediatr Orthop 23:10142003

  • 20

    Stolte BTotzeck AKizina KBolz SPietruck LMönninghoff C: Feasibility and safety of intrathecal treatment with nusinersen in adult patients with spinal muscular atrophy. Ther Adv Neurol Disorder 11:1756286418803246 2018

    • Search Google Scholar
    • Export Citation
  • 21

    Touznik AMaruyama RYokota T: In vitro evaluation of antisense-mediated exon inclusion for spinal muscular atrophy. Methods Mol Biol 1828:4394542018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 22

    Veerapandiyan APal RD’Ambrosio SYoung IEichinger KCollins E: Cervical puncture to deliver nusinersen in patients with spinal muscular atrophy. Neurology 91:e620e6242018

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 23

    Wang JSchultz PGJohnson KA: Mechanistic studies of a small-molecule modulator of SMN2 splicing. Proc Natl Acad Sci U S A 115:E4604E46122018

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 24

    Weaver JJNatarajan NShaw DWWApkon SDKoo KSHShivaram GM: 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 48:3923972018

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 52 52 52
Full Text Views 14 14 14
PDF Downloads 34 34 34
EPUB Downloads 0 0 0

PubMed

Google Scholar