Noninvasive Ventilation in Chest Wall Deformities: When and Why

Abstract

Diseases affecting the chest wall reduce the functional capacity of the diaphragm and lung, invariably leading to ventilatory failure. Chest wall or thoracic cage deformities can be primary, like idiopathic kyphoscoliosis, ankylosing spondylitis, pectus excavatum, and pectus carinatum, or secondary to pleural fibrosis, thoracoplasty (after lobectomy or pneumonectomy), or poliomyelitis. These restrictive diseases can affect the components of the inspiratory pump, including the bony rib cage, respiratory musculature, and the spine and its articulations, as well as the soft tissue comprising the abdomen. Respiratory muscle weakness is the most common factor leading to chest infections, hospital admissions, and early mortality in these patients. Most of these patients gradually develop nocturnal hypoventilation, initially during rapid-eye-movement (REM) sleep before progressing to non-REM (NREM) sleep, eventually leading to daytime chronic ventilatory failure. Management of chronic ventilatory failure with noninvasive ventilation (NIV) is standard recommended care. Nocturnal nasal intermittent positive pressure ventilation (NIPPV) has been shown to increase survival and improve blood gases, respiratory function, sleep architecture, and quality of life in patients with chest wall diseases.