The correlation between spinal and chest wall deformities and pulmonary function in Marfan syndrome.

Purpose Scoliosis, chest wall deformities and pulmonary involvement are common features of Marfan syndrome (MFS). We aimed to assess the impact of spinal and chest wall deformities on pulmonary function in paediatric MFS patients with a surgically managed spinal deformity. Methods In this multicentre retrospective study, spirometry, lung volumes and radiographic imaging were performed on 26 MFS patients between the ages of seven and 18 years who were undergoing planned spinal fusion surgery for spinal deformity. A correlation analysis assessed the relationship between radiographic measurements of spinal and chest wall deformities and predicted total lung capacity (TLC), forced vital capacity (FVC) and the ratio between forced expiratory volume in one second and FVC (FEV1/FVC). Results In total, 18 patients (70%) had impaired pulmonary function. Thoracic kyphosis (mean 19.3 degrees; -32 degrees to 54 degrees) had a strong positive correlation with FEV1/FVC (r = 0.65; p < 0.001). Significant decrease in FEV1/FVC below 80% occurred at kyphosis under 15 degrees (p = 0.004). Kyphosis had a moderate negative correlation with FVC (r = -0.43; p = 0.03). Chest wall deformity had a strong negative correlation with FEV1/FVC (r = -0.61; p = 0.001). The magnitude of the thoracic curve (mean 55.2 degrees; 28 degrees to 92 degrees) had a significant moderate negative correlation with TLC (r = -0.45; p = 0.04). Conclusion In MFS, three factors correlate with decreased pulmonary function measures: hypokyphosis, increasing chest wall deformity and increasing corona! curve magnitude. Hypokyphosis and increased chest wall deformity correlated with diminished FEV1/FVC; increasing thoracic spinal curvature with diminished TLC. Further analysis with a larger cohort will help better define the relationship between these deformities and pulmonary function in this unique population.