Perioperative Management for Patients With Spinal Muscular Atrophy Undergoing Scoliosis Surgery: A Single-Center Retrospective Study.

OBJECTIVE

Spinal muscular atrophy (SMA) is a rare neuromuscular disorder frequently associated with progressive scoliosis, often requiring complex surgical intervention. Perioperative management for SMA patients remains challenging due to severe respiratory compromise, difficult airway anatomy, and a high risk of perioperative complications. This study aimed to analyze perioperative strategies and outcomes in SMA patients undergoing posterior spinal fusion for scoliosis correction.

METHODS

We retrospectively reviewed SMA patients who underwent posterior spinal fusion at our institution from January 2018 to December 2024. Primary outcomes included intraoperative blood loss, postoperative complications, and perioperative hypokalemia; secondary outcomes included postoperative mechanical ventilation duration, length of stay in the ICU and hospital, and radiographic outcomes. Subgroup comparisons between SMA type 2 and type 3 were performed using the independent-samples t-test or Mann-Whitney U test for continuous variables and the chi-square test or Fisher's exact test for categorical variables.

RESULTS

Forty-three SMA patients (72.1% type 2, 27.9% type 3) with a mean age of 15.9 ± 4.9 years were included. Spinal deformity was severe, with a mean preoperative Cobb angle of 113.2° ± 22.8°. Pulmonary function was markedly impaired, with a median forced vital capacity (FVC) of 34.8% (IQR: 24.6%-46.4%) of the predicted value. Endotracheal intubation was challenging, with 93.0% (n = 40) requiring video laryngoscopy or awake tracheal intubation using flexible bronchoscopy. Blood loss was substantial, with a median intraoperative blood loss of 1200 mL (IQR: 1000-1550 mL) and a postoperative drainage of 1040 mL (IQR: 723-1293 mL). An extraordinarily high incidence of perioperative hypokalemia was observed, affecting 65.1% of patients (n = 28) intraoperatively and 79.1% (n = 34) postoperatively. All patients successfully underwent surgery with a mean Cobb angle correction rate of 49.0% ± 13.5%. There were no cases of operative mortality, and the overall complication rate was low (2.3%, n = 1). SMA type 2 and type 3 shared similar perioperative parameters.

CONCLUSIONS

This study represents the largest case series to date in Asia on the perioperative management of SMA patients undergoing scoliosis surgery. These patients present complex perioperative risks requiring meticulous multidisciplinary care. Advanced airway planning, hemodynamic support, blood management strategies, and proactive correction of electrolyte imbalances may help support safer perioperative care. With appropriate planning, scoliosis surgery can be safely performed in SMA patients, offering structural and functional benefits.