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. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Ortiz Carlos, Chau Alex, Desai Sudhen, Kukreja Kamlesh

Keywords: nusinersen, spinal muscular atrophy, intrathecal administration

Stenosis of the inferior vena cava or the hepatic veins is an underlying cause for hepatic compromise in liver transplant patients and in patients with inflammatory diseases of the liver, leading to considerable morbidity. CT-guided or ultrasound-guided minimally invasive vessel recanalization procedures can reverse liver failure in these patients by restoring vessel patency. However, it can be difficult to employ these techniques using traditional guidewire tips against fibrotic obstructions associated with vessels that have undergone repeated stenosis. Sharp recanalization offers an alternative strategy to gain access through these obstacles by utilizing needles or the sharp end of the guidewire tip to penetrate the vessel obstruction. This technique has been well-described and employed successfully in adult patients, but has not yet been reported in the pediatric setting. Read More

Meeting name: SPR 2019 Annual Meeting & Postgraduate Course , 2019

Authors: Shum Thomas, Cleveland Heather, Chau Alex, Ashton Daniel, Hernandez Alberto

Keywords: interventional