Surgical correction of spinal deformity in patients with congenital muscular dystrophy.

BACKGROUND: Congenital muscular dystrophy (CMD), among the myopathic disorders is one form of flaccid neuromuscular disorder (NMD). Patients with NMD frequently develop progressive spinal deformity. For NMD patients who have a severe spinal deformity, sitting is often difficult and is accompanied by pain and breakdown of the skin. Spinal deformity surgery in these patients has been highly effective in stabilizing the spine, maintaining upright, comfortable sitting balance, and improving patients' quality of life. However, many studies have reported significant rates of peri/postoperative complications in these patients. To our knowledge, there has been no study on the results of spinal deformity surgery in patients with CMD. The purpose of this study was to review the clinical and radiological results of spinal deformity surgery in this group of patients with CMD.
METHODS: Between 2004 and 2007, a total of 10 CMD patients underwent scoliosis surgery. There were three patients with Fukuyama CMD, three with Ullrich CMD, and 4 with nonsyndromic CMD (merosin-negative). They were nonambulatory. All the patients had standard posterior spinal fusion and pedicle-screw-alone fixation from T3 or T4 to L5 for spinal deformity. Our inclusion criteria required that each patient (1) had considerable difficulty with sitting balance and pain or breakdown of the skin due to scoliosis; (2) was able to ventilate his or her lung autonomously; (3) was not ventilator-dependent; and (4) did not have cardiac failure. Sufficient informed consent was important, and the decision to perform surgery was made by the patient/family with sufficient preoperative informed consent. Patients were trained with inspiratory muscle training (IMT) using an inspiratory muscle trainer (Threshold IMT) for 6 weeks prior to surgery Cardiac function was assessed preoperatively. Pulmonary function tests were performed preoperatively and postoperatively. Radiographic assessments were performed on sitting anteroposterior (AP) and lateral radiographs. These assessments were made periodically. The Cobb angles of the curves and spinal pelvic obliquity (SPO) on the coronal plane, thoracic kyphosis, and lumbar lordosis were measured. The preoperative AP radiograph and side-bending films were examined to determine flexibility. Patients' and parents' satisfaction were surveyed by a self-completed questionnaire at the last follow-up.
RESULTS: Percent forced vital capacity (%FVC) increased from a mean of 30% before IMT to a mean of 34% the day before surgery. The preoperative scoliosis was 75 degrees (range 61 degrees -95 degrees ). The scoliotic curvature on preoperative side-bending films was 19 degrees (range 11 degrees -28 degrees ). All patients were extubated on the day of surgery. No patients developed cardiac or respiratory complications. The scoliotic curvature was 18 degrees (range 10 degrees -25 degrees ) immediately after surgery, and 19 degrees (range 12 degrees -27 degrees ) at the last follow-up. The pelvic obliquity improved from a mean of 17 degrees (range 14 degrees -20 degrees ) preoperatively to a mean of 6 degrees (range 4 degrees -9 degrees ) postoperatively and to 7 degrees (range 4 degrees -10 degrees ) at the last follow-up. Balanced sitting posture was achieved and maintained. On the sagittal plane, good reconstruction of sagittal plane alignment was recreated and maintained. There were no major complications or deaths. All patients/parents completed the outcome satisfaction questionnaire. Eight patients/parents were very satisfied and two were satisfied.
CONCLUSIONS: Pedicle-screw-alone fixation and fusion to L5 was safe and effective in CMD patients with scoliosis of <95 degrees and pelvic obliquity of <20 degrees . 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.