Comparison between Operated Muscular Dystrophy and Spinal Muscular Atrophy Patients in terms of Radiological, Pulmonary and Functional Outcomes.

Abstract

STUDY DESIGN: Retrospective comparative study.

PURPOSE: To study and compare the surgical outcomes of muscular dystrophy (MD) and spinal muscle atrophy (SMA).

OVERVIEW OF LITERATURE: There are few reports that have evaluated and compared the surgical outcomes of MD and SMA patients.

METHODS: The patients (n = 35) were divided into two groups: a MD group with 24 patients and a SMA group with 11 patients. The average follow-up period was 21 months. All patients were operated for scoliosis correction using posterior instrumentation and fusion. In the immediate postoperative period, all efforts were made to reduce the pulmonary complications using non-invasive positive pressure ventilation and a coughing assist devices. The patients were evaluated by radiograph in terms of the Cobb's angle, pelvic obliquity, T1 translation, thoracic kyphosis and lumbar lordosis. The pulmonary function and self-image satisfaction were also assessed.

RESULTS: There was a lower correction rate in the MD group (41.5%) than in the SMA group (48.3%), even though the curves were smaller in the MD group. The correction in the pelvic obliquity was significantly better in the SMA group (p = 0.03). The predicted vital capacity showed a 4% reduction in the MD group 1 year after surgery, while the SMA group showed a 10% reduction. The peak cough flow and end tidal PCO(2) did not deteriorate and were well maintained. The average score for the improvement in self-image satisfaction postoperatively was 3.96 and 4.64 for the MD and SMA groups, respectively. The total complication rate was 45.7%; 14.3% of which were respiratory-related.

CONCLUSIONS: Surgical intervention for MD and SMA may be performed safely in patients with a very low forced vital capacity (< 30%) through aggressive preoperative and postoperative rehabilitation efforts.