Kyphoscoliosis ventilatory insufficiency: noninvasive management outcomes.

Objective: To determine the effects on symptoms, pulmonary function, sleep, and other clinical variables of treating kyphoscoliosis-associated chronic alveolar hypoventilation with nocturnal nasal ventilation.

Design: Sixteen patients with kyphoscoliosis were treated with nocturnal nasal ventilation delivered by volume-cycled (seven patients) and pressure-cycled (nine patients) ventilators. Dyspnea, morning headaches, fatigue, hypersomnolence, and perceived sleep quality were assessed.

Results: All pretreatment symptoms improved significantly with nasal ventilation. Likewise, PaO2 (mm Hg), PaO2/FlO2, PaCO2 (mm Hg), pH, and forced vital capacity (in milliliters and as a percentage of predicted normal) significantly improved with treatment. Maximum inspiratory pressures and maximum expiratory pressures also significantly increased. Tidal volumes increased significantly and breathing frequency decreased (not significant). Although perceived sleep quality improved, as well as sleep oxyhemoglobin saturation, there was no significant change in sleep architecture. Hospitalization days for respiratory difficulties also decreased from 10.9 +/- 13.3 days in the 6 mo before intermittent positive-pressure ventilation to 0 days during the first 6 mo of treatment.

Conclusions: Although not apparently affecting sleep architecture, nocturnal nasal ventilation can significantly improve nocturnal and daytime blood gases, pulmonary function, and symptoms of hypoventilation for patients with severe kyphoscoliosis.