Anesthesia Care, Complications, and Airway Management for Patients With Spinal Muscular Atrophy: A Retrospective Chart Review From a Quaternary Children’s Hospital

BACKGROUND: Spinal muscular atrophy (SMA) is a genetic disorder resulting in progressive muscle atrophy due to the degradation of motor neurons. There are limited data on anesthesia care for these patients, the incidence of anesthesia-related adverse events, and difficult intubations. The investigators aim to characterize patients with SMA who required anesthetics at a large quaternary pediatric hospital, describe the procedures being performed, report the incidence of severe anesthesia-related adverse events, and determine the incidence of difficult intubations. The investigators hypothesized that lumbar puncture for nusinersen administration would represent the most common procedure for which patients with SMA required anesthesia care. METHODS: A retrospective chart review of anesthetics provided to SMA patients from June 1, 2012, to December 30, 2023. Data obtained included procedures performed, patient characteristics, perioperative care, anesthesia technique, and outcomes. RESULTS: In total, 1804 procedures were performed for 175 patients with SMA. The majority of procedures (1423/1804, 78.9%) were for lumbar puncture for nusinersen administration; 234 of 1804 (13.0%) received general anesthesia with endotracheal tube placement; 22 of 1804 total cases (1.2%) or 22 of 234 (9.4%) of those with endotracheal tube placement met the definition of difficult intubation. There were no statistically significant associations between difficult intubation and SMA type, age, and presence of halo headframe (all P > .05). There were six severe anesthesia-related adverse events (0.33%). Of 1423 total procedures for lumbar punctures for nusinersen administration, 1254 of 1423 (88.1%) were performed with a natural airway (nasal canula, facemask, or home continuous positive airway pressure [CPAP] or biphasic positive airway pressure [BiPAP]) or pre-existing tracheostomy. CONCLUSIONS: Lumbar puncture for nusinersen administration made up the vast majority of procedures for which patients with SMA presented for anesthesia care. The incidence of difficult intubation was 9.4%, and the incidence of anesthesia-related severe adverse events was 0.33%. These results indicate the need to focus research on the perioperative and airway-related risks for this evolving and medically complex population.