Background: Spinal muscular atrophy (SMA) frequently progresses to severe scoliosis requiring complex surgical intervention. Perioperative management remains challenging. 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 Peking Union Medical College Hospital from January 2018 to December 2024. Demographic, intraoperative, and postoperative data were collected. Subgroup comparisons between SMA type 2 and type 3 were performed.
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 fiberoptic techniques. 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: 723-1293mL). An extraordinarily high incidence of perioperative hypokalemia was observed, affecting 65.1% of patients (n = 28) intraoperatively and 79.1% (n = 34) postoperatively. Surgical correction was effective (mean Cobb correction 49.0 ± 13.5%), with no operative mortality and a low overall complication rate (2.3%, n=1). Perioperative parameters were broadly similar between SMA type 2 and type 3.
Conclusions: This study represents the largest case series to date in Asia on the perioperative management of SMA patients undergoing scoliosis surgery. Advanced airway planning, hemodynamic support, blood management strategies, and proactive correction of electrolyte imbalances are all critical to optimizing outcomes. With appropriate planning, scoliosis surgery can be safely performed in SMA patients, offering structural and functional benefits.