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

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
DOI: 10.1007/s00264-009-0764-7 Publication Date: 2009-04-01T06:20:35Z
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.
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