Surgical correction of spinal deformity in patients with congenital muscular dystrophy

Pedicle-screw-alone fixation and fusion to L5 was safe and effective in CMD patients with scoliosis of <95° and pelvic obliquity of <20°. Scoliosis curves were flexible (75% correction) on side-bending films preoperatively. Curve correction and maintenance of correction in the coronal and sagittal plane was excellent. The pelvic obliquity significantly improved. Balanced sitting posture was achieved and maintained in all patients. Our patients with CMD spinal deformity and a moderately and severely decreased FVC could be operated on safely and successfully with general anesthesia. All patients were extubated in the operating room. There were no major complications or deaths. We believe a FVC of <30% alone is not a predisposition to pulmonary complications. However, cardiomyopathy might be a determining risk of mortality, and we believe surgery for these patients should be avoided. Patients' and parents' satisfaction was high.