Pulmonary Function Testing in Children with Restrictive Chest Wall Disorders

Disorders of the chest wall, spine, and respiratory muscles lead to lung function abnormalities consistent with restrictive lung disease. Common disorders of the thorax include diseases with respiratory muscle weakness, growth abnormalities of the thorax and spine, such as scoliosis. Spirometry alone is not sufficient to diagnose these abnormalities but may be useful to serially monitor changes over time or in response to treatments. In contrast, obesity does not produce restrictive respiratory disease in most children and produces obstructive lung disease more often. Test of respiratory muscle function, breathing during sleep, and during exercise have led to additional insights about the pathophysiology of these conditions that may provide new avenues for future therapies.