Successful Combination of Neuraxial and Regional Anesthesia in a Child With Advanced Spinal Muscular Atrophy Type 1 Receiving Maintenance Nusinersen Therapy: A Case Report.
Spinal muscular atrophy (SMA) is an autosomal recessive condition characterized by degeneration of the anterior horn cells of the spinal cord, which causes progressive muscle atrophy and weakness. SMA type 1 is the most common type and is associated with severe disability and early mortality. Concomitant restrictive respiratory physiology often manifests with significant implications for anesthetic management. Here, we describe a successful spinal anesthetic for orthopedic surgery in an SMA type 1 patient receiving intrathecal nusinersen maintenance therapy, an antisense oligonucleotide designed to increase expression of the survival motor neuron protein, and the first US Food and Drug Administration-approved drug to treat SMA.