Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy.
ABSTRACT Between 2005 and 2007, 14 patients who had severe scoliosis in Duchenne muscular dystrophy (DMD) and a poor forced vital capacity (FVC) of <30% at admission underwent scoliosis surgery. FVC on admission was 21.6% (range, 16-27%). The patients were given respiratory muscle training using a pulmonary trainer (Threshold IMT, Philips Respironics, Inc.) for six weeks before operation. FVC increased to 26.2% (range, 22-31%) the day before operation. The mean preoperative scoliosis was 98 degrees (range, 81 degrees-130 degrees). All patients underwent posterior fusion and all-screw construction and were extubated on the operative day. No patients developed any respiratory complications. The postoperative scoliosis was 34 degrees (range, 20 degrees-40 degrees) (65%). FVC remained stable at six weeks after operation. FVC decreased to 19.8% (range, 16-25%) and the mean scoliosis was 35 degrees (range, 23 degrees-40 degrees) (64%) at two years after operation. DMD patients with severe scoliosis and FVC considered too low to permit reasonable surgical risk could undergo surgery and could benefit from surgery.
Article: Dose-dependent effect of individualized respiratory muscle training in children with Duchenne muscular dystrophy.[show abstract] [hide abstract]
ABSTRACT: The aim of this study was to evaluate the effects of low intensity, home inspiratory muscle training on respiratory muscle endurance in children with Duchenne muscular dystrophy, using a double-blind protocol. The originality aspect of this study is the use of a reproducible method of endurance and of the same method for evaluation and training. We studied eight trained children (mean age 14.7+/-4.5 years) and eight control children (mean age, 12.6+/-1.8 years). For 6 weeks, children breathed twice a day for 10 min through a valve with either 30% (training group) or less than 5% (control group) of their maximum inspiratory pressure (P(imax)). The results showed (1) a 46% improvement in the time limit after training in the training group and no change in the control group and (2) a significant correlation between the total time of respiratory muscle training and the percentage of endurance improvement in the training group. We conclude that specific training improves respiratory muscle endurance in Duchenne muscular dystrophy and the effectiveness of training appears to be dependent on the quantity of training.Neuromuscular Disorders 09/2002; 12(6):576-83. · 2.80 Impact Factor