Older patients with kyphoscoliosis are at risk for the development of respiratory failure because the angle of curvature typically worsens with age. Although most cases of idiopathic scoliosis tend to stabilize just after puberty, further spinal deformity may result from the osteoporosis, vertebral body weakening, and loss of muscle tone that accompany older age. Surgical procedures to straighten and stabilize the vertebral column usually fail to restore ventilatory capacity. Such procedures have their primary role early in the course of kyphoscoliosis, when they may prevent progression of the deformity before the development of respiratory compromise. Supportive measures may sustain meaningful life for many years, even in those patients with chronic respiratory failure.
Many patients can adapt very well to a state of chronically disordered gas exchange.
Chronic hypoxemia accompanied by secondary erythrocytosis, worsening of pulmonary hypertension, and cor pulmonale should be treated with supplemental oxygen administration. Such therapy can be augmented by nocturnal ventilatory support. Although kyphoscoliosis is not a primary sleep-related breathing disorder, the degree of oxygen desaturation that occurs during sleep in this disorder is greater than that observed in other types of lung disease, possibly because the baseline hypoxemia and hypercapnia are more severe and lung volumes are smaller. Thus, nocturnal oxygen therapy and mechanical ventilatory support during sleep often improve functional status and symptoms. Devices used for mechanical ventilation in such patients include the iron lung, specially fitted upper-body negative pressure ventilators, and positive pressure ventilators applied via tracheostomy or nasal mask.