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°(range, 81°-130°). All patients underwent posterior fusion and allscrew 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.

Surgical management of severe scoliosis with high risk pulmonary dysfunction in Duchenne muscular dystrophy: patient function, quality of life and satisfaction

“…They were trained with preoperative inspiratory muscle training by a pulmonary trainer (Threshold IMT) (Figs.1 and 2 ) for six weeks prior to surgery [23]. All patients recovered well following the surgery with no major complications [23]. They were encouraged to continue inspiratory muscle training after surgery and even at home after discharge.…”

“…All patients were able to ventilate autonomously and were not respirator dependent. They were trained with preoperative inspiratory muscle training by a pulmonary trainer (Threshold IMT) (Figs.1 and 2 ) for six weeks prior to surgery [23]. All patients recovered well following the surgery with no major complications [23].…”

“…Pulmonary function tests [23] Pulmonary function tests were performed with a computerised pulmonary function system (FUDAC-50, Fukuda Denshi, Tokyo). Pulmonary function values reported in this study are forced vital capacity (FVC) expressed as percent of predicted value (%FVC) on the published normative data.…”

“…In a previous study, the authors reported the clinical and radiological results of DMD scoliosis surgery in patients with a low forced vital capacity (FVC) of <30%, conventionally considered too low to permit reasonable surgical risk [23]. Scoliosis surgery for such patients must provide meaningful benefit in their function, QOL and satisfaction in proportion to risks involved for patients and parents.…”

“…The majority of studies have reported only data on changes in scoliotic curvature and complications. Irreversible changes in cardiac and pulmonary function pose serious risks to anaesthesia and have been relative contraindications to surgery [7,17,18,20,22,23].…”