Preoperative inspiratory muscle training for patients with severe scoliosis and high-risk pulmonary dysfunction in duchenne muscular dystrophy

Abstract

Severe spinal collapse and pulmonary function deterioration are so common in patients with Duchenne muscular dystrophy (DMD). The limit for scoliosis surgery has been a minimum forced vital capacity (FVC) of about 30% of predicted value. DMD patients with low %FVC who might benefit from scoliosis surgery have not been being offered surgery. Between 2005 and 2007, a total of 17 DMD patients with FVC of <30% at admission underwent scoliosis surgery. %FVC at admission was 22% (range 16–27%). After admission, they were trained with inspiratory muscle training, using a pulmonary trainer (threshold IMT) for 6 weeks prior to surgery and encouraged to continue the training even after surgery and discharge. %FVC increased in all patients and noted 26% (range 22–31%) the day before surgery. The preoperative scoliosis was 97° (range 81°–130°). All patients had posterior spinal fusion and were extubated on the operative day. No patients developed any respiratory complications. No ventilatory support was needed. The mean ICU stay was 0.5 days (range 0–1 day). The postoperative scoliosis was 31° (range 18°–40°). DMD patients with severe scoliosis and FVC considered too low to permit reasonable surgical risk could undergo surgery after inspiratory muscle training, with no major complications.