Patients with Spinal Muscular Atrophy (SMA) may have scoliosis requiring spinal hardware for spinal fixation. The scoliosis in addition to the spinal hardware makes intrathecal access difficult with conventional approaches. With recent FDA approval of nusinersen (SpinrazaÒ) for SMA, intrathecal access is required for administration of this meditation. We describe our experience in these patients using a variety of lumbar puncture approaches (intra-spinous process, trans-pedicle, and trans-foraminal) utilizing conventional fluoroscopy, cone-beam CT (CBCT) with fluoroscopy overlay and/or conventional CT with CT fluoroscopy. Approach to the type of access and image guidance is based on the presence of spinal hardware and degree of scoliosis and spinal fusion. Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Patel Manish, Weiss Judith, Kandil Ali, Johnson Neil

Keywords: Spinal Muscular Atrophy, Lumbar Puncture, Spinraza

Slow-flow vascular malformations, most commonly multifocal or diffuse venous (VM), venous-lymphatic (VLM) and capillary-lymphatic-venous malformations (CLVM), are associated with coagulation abnormalities affecting hemostasis and thrombosis and increase risk of hematological complications with procedural interventions. Although not completely understood, pathogenesis of this coagulopathy, termed localized intravascular coagulopathy (LIC), is presumed secondary to stagnant blood in abnormal vessels and consumption of coagulation factors. LIC is characterized by elevated D-dimer, low fibrinogen, and/or mild thrombocytopenia and may progress to disseminated intravascular coagulopathy following surgical procedures. In patients with high-risk malformations, hematologic complications of sclerotherapy and use of low molecular weight heparin (LMWH) as a preventative measure have not been well studied. Read More

Meeting name: SPR 2018 Annual Meeting & Postgraduate Course , 2018

Authors: Ricci Kiersten, Mobberley-schuman Paula, Hammill Adrienne, Dasgupta Roshni, Patel Manish

Keywords: LIC, Vasculat Malformation, Coagulopathy