Safety and efficacy of treatment for scolios is secondary to spinal muscular atrophy fused to lumbar 5 level

Abstract

Introduction: Patients with spinal muscular atrophy (SMA) usually have progressive scoliosis. Although fusion of the sacrum or pelvis has been recommended for correcting pelvic obliquity (PO), the procedure is invasive. This study determined as to whether performing instrumentation to the fifth lumbar vertebra (L5) is safe and effective for scoliosis in patients with SMA. Methods: Twelve patients with SMA underwent posterior spinal fusion and stopping instrumentation at the L5 level. We evaluated age at surgery, the duration of surgery, blood loss, complications, preoperative and postoperative Cobb angles, and PO. Results: The mean age at surgery was 11.4 years; the mean duration of surgery was 319 minutes, and the mean blood loss was 1170 mL. The Cobb angle improved from 97.3° to 39.1° at 1 month postoperatively (correction rate, 60.9%) and to 42.3° at the final follow-up. PO was corrected from 27.8° to 13.1° at 1 month postoperatively (correction rate, 51.7%) and to 19.8° at the final follow-up. No complications were reported. All patients showed improvement in low back pain, with reduced difficulty while sitting. However, >10% correction loss of PO was observed in 6 patients with high preoperative PO. Conclusions: The correction rate of scoliosis in SMA patients with posterior spinal fusion and instrumentation to the L5 level was acceptable, and no complications occurred. Scoliosis associated with SMA was more rigid and severer than scoliosis associated with Duchenne muscular dystrophy. Correction rates of the Cobb angle and PO in SMA patients with instrumentation to L5 were similar to those in SMA patients with instrumentation to the sacrum or pelvis. Correction loss of PO was greater in patients with high preoperative PO than in those with low preoperative PO. Instrumentation and fusion to L5 for scoliosis in patients with SMA seems safe and effective, except in cases of high preoperative PO.