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Final ID: Poster #: EDU-048

An Overview of Techniques for Intrathecal Administration of Nusinersen in Children with Spinal Muscular Atrophy

Purpose or Case Report: 1) Reviewing the pathology and outcomes of children with spinal muscular atrophy (SMA)
2) Learning the procedures available for intrathecal access
3) Understanding the indications for choosing more advanced techniques to deliver nusinersen

Spinal muscular atrophy is an autosomal recessive disease affecting motor neurons and is the most common genetic cause of death in infants. Nuinersen (Spinraza) was recently approved by FDA for intrathecal administration in SMA patients. Commonly administered by lumbar puncture in the clinic or with imaging guidance, this is the initial method of administering nusinersen. Deformities and spinal instrumentation from orthopedic surgeries are common in SMA patients, preventing traditional intrathecal access by lumbar puncture for nusinersen delivery. Transforaminal lumbar sac access, ultrasound or fluoroscopy guided cervical spine access, or subcutaneous catheter placement can be the alternative approaches with failed/difficult lumbar access. Given the potential benefit of nusinersen, understanding all methods to obtain intrathecal access is essential for a pediatric interventional radiology (IR) practice.
Methods & Materials: We intend to review the technique, indications, complications, tips and tricks for each of these techniques and their relevance to an IR practice.

1) Ultrasound guided lumbar puncture
2) Fluoroscopy guided lumbar puncture
3) Ultrasound guided cervical puncture
4) Fluoroscopy guided cervical puncture (C1-C2)
5) Transforaminal delivery using cone-beam computed tomography
6) Transosseous access via drill
7) Subcutaneous Intrathecal Catheter System
Results:
Conclusions: Nusinersen administration for SMA is providing a novel treatment for a previously untreatable condition and the number of patients requiring these procedures is expected to increase. Pediatric interventional radiologists can provide the safest approach for delivering nusinersen by being aware of the options.
  • Ortiz, Carlos  ( Baylor College of Medicine , Houston , Texas , United States )
  • Chau, Alex  ( Texas Children's Hospital , Houston , Texas , United States )
  • Desai, Sudhen  ( Texas Children's Hospital , Houston , Texas , United States )
  • Kukreja, Kamlesh  ( Texas Children's Hospital , Houston , Texas , United States )
Session Info:

Posters - Educational

Interventional

SPR Posters - Educational

More abstracts from these authors:
BioPlug Utilized for Closure of Esophagocutaneous Fistula

Johnson Brittany, Desai Sudhen, Minifee Paul

Image-guided Percutaneous Bone Biopsy for Pediatric Osteomyelitis: Correlating MRI Findings, Tissue Pathology and Culture, and Effect on Clinical Management

Yen Christopher, Hernandez Alberto, Gardner Gregory, Nazario Maricarmen, Kukreja Kamlesh, Desai Sudhen

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