Study Design: A prospective observational study of scoliosis patients who were on non-invasive night ventilation for respiratory failure.
Objective: To report the results of spinal deformity correction in a group of patients with progressive scoliosis and rare forms of muscular dystrophy/myopathy with respiratory failure who were on nocturnal ventilatory support at the time of surgery.
Subjects: 9 patients (6 males, 3 females) with scoliosis and respiratory failure. The mean age at surgery was 12.4years (range 8–16yrs). There were 4 patients with multicore myopathy, 2 with merocin negative congenital muscular dystrophy, 1 with Ullrichs muscular dystrophy, 1 patient with congenital AcylCOA dehyrogenase deficiency and 1 with congenital scoliosis and dextrocardia. All the patients had overnight pulse oximetry, which showed episodes of desaturation at night. This was reversed with the onset of nocturnal ventilation. All the patients underwent posterior fusion performed by the same surgeon. Mean follow-up was 40 months (range 10 to 75)
Outcome Measures: Lung function, Cobb angle, Length of ICU stay, complications
Results: Mean vital capacity at time of surgery was 20% (range 13–28%). All patients recovered well following surgery with no cardiac or pulmonary complications. The mean stay in the ICU was 2.7 days (range 2–5). The mean hospital stay was 14.2 days (range 10–21). The mean preoperative Cobb angle was 70.2 degrees (range 55–85). The average change in the Cobb angle post-operatively was 32 degrees (range 16–65 degrees). The mean vital capacity of patients at latest follow up was 18% (range 10–32%). There was no loss of correction at latest follow-up. None of these patients lost their ambulatory capacity following surgery.
Conclusion: This is the first study reporting results of deformity correction in patients on ventilatory support. Spinal deformity correction in patients on non-invasive nocturnal ventilation presented no increased risk of complications.