Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy

摘要

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° (range, 81°–130°). 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° (range, 20°–40°) (65%). FVC remained stable at six weeks after operation. FVC decreased to 19.8% (range, 16–25%) and the mean scoliosis was 35° (range, 23°–40°)(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.