Disorders of the thoracic cage and diaphragm
Disorders of the thoracic skeleton can lead to a severe restrictive ventilatory defect, the risk of respiratory failure being highest with (1) scoliosis—particularly if the following characteristics are present: early onset, severe angulation, high in the thorax, respiratory muscle weakness, low vital capacity; (2) kyphosis—but only if of very sharp angulation (gibbus), most commonly seen following tuberculous osteomyelitis; and (3) after thoracoplasty—historically performed as treatment for pulmonary tuberculosis. While not a disorder of the skeleton, a similar pathophysiological pattern is seen in extreme obesity, and this is the fastest growing cause of referral to home ventilation centres. Arterial blood gases and quality of life can both be readily improved with non-invasive ventilation, usually using a nasal or face mask. Survival in most skeletal disorders after starting ventilation leads to apical bullae, pleural thickening/effusions, and cricoarytenoid arthritis, but rarely causes respiratory failure in the absence of other comorbidities.