Effect of long term intermittent nocturnal non-invasive positive pressure ventilation on patient with severe kyphoscoliosis and hypoxaemia.

Kyphoscoliosis is one of the causes of restrictive lung disease. It can lead to chronic hypercapnic respiratory failure and hypoxaemia. The benefits of long term intermittent nocturnal non-invasive positive pressure ventilation (NIPPV) to these patients have been shown in regard to improved vital capacity, total lung capacity, muscle strength and daytime oxygenation. In our case, we found long term intermittent nocturnal NIPPV also had a beneficial effect on exercise capacity and pulmonary hypertension. This is good for patients with kyphoscoliosis not only in terms of their survival but also for their quality of life. We recommend long term intermittent nocturnal NIPPV as a routine intervention for severe kyphoscoliosis with hypoxaemia, and transthoracic echocardiography as a routine evaluation tool for determining the response of pulmonary hypertension to management.