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

• DOI: 10.1007/s00264-009-0764-7
• Publication Date: 2009-04-01T06:20:35Z
• Authors: Masashi Takaso; Toshiyuki Nakazawa; Takayuki Imura; Naonobu Takahira; Moritoshi Itoman; Kazuhisa Takahashi; Masashi Yamazaki; Seiji Otori; Tsutomu Akazawa; Shohei Minami; Toshiaki Kotani
• Keywords: Lung Diseases; Male; Adolescent; Bone Screws; Vital Capacity; Breathing Exercises; 3. Good health; Muscular Dystrophy, Duchenne; 03 medical and health sciences; Postoperative Complications; Spinal Fusion; Treatment Outcome; 0302 clinical medicine; Scoliosis; Humans; Child

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.