Improvement of Pulmonary Function in Arthrogryposis Multiplex Congenita Patients Undergoing Posterior Spinal Fusion Surgery for Concomitant Scoliosis: A Minimum of 3-Year Follow-up

-OBJECTIVE: We sought to investigate the long-term outcome of pulmonary function for arthrogryposis multi-plex congenita (AMC) patients undergoing posterior spinal fusion (PSF) and to further determine influential factors. -METHODS: Eighteen AMC patients with a minimum of 3-year follow-up after PSF were prospectively collected. All the patients underwent a pulmonary function test before surgery and at the final follow-up. The percentage pre-dicted values of vital capacity (VC%) and forced vital ca-pacity (FVC%) were recorded. The following radiographic parameters were collected including Cobb angle and thoracic kyphosis. The total lung volumes (TLV) were measured on the image of 3-dimensional computed to-mography scan by the reconstruction software. -RESULTS: There were 10 males and 8 females with a mean age of 13.8 +/- 6.1 years. The mean preoperative VC% and FVC% were 40.5% +/- 7.6% and 39.5% +/- 4.7%, which were significantly increased to 52.0% +/- 7.5% and 51.2% +/- 6.8% at the final follow-up (P < 0.001). Besides, there was remarkable improvement in terms of TLV (1.57 +/- 0.2 L vs. 2.39 +/- 0.6 L, P < 0.001). Remarkable correlations were observed between TLV and pulmonary function tests (r = 0.79, P < 0.001 for VC%; r = 0.78, P < 0.001 for FVC%). Multiple regression analysis showed that 2 variables including D thoracic kyphosis and D Cobb angle were independently associated with the improvement of pulmo-nary function. -CONCLUSIONS: The pulmonary function of AMC patients can be well improved through PSF surgery. It was remarkably associated with the correction of curve magnitude and restoration of thoracic kyphosis.