6533b822fe1ef96bd127cac7

RESEARCH PRODUCT

Kyphoscoliotic Ventilatory Insufficiency

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Objectives: To determine the effects of long-term nocturnal intermittent positive-pressure ventilation (NIPPV) on symptoms, pulmonary function test results, sleep, and respiratory muscle performance in patients with ventilatory insufficiency due to severe kyphoscoliosis. Design: A prospective study in which 16 severe kyphoscoliotic patients were treated with NIPPV delivered by volume-cycled and pressure-cycled ventilators, over a period of 36 months. Interventions and measurements: At baseline, pulmonary function tests, blood gas measurements, polysomnography, and respiratory muscle strength (measured by noninvasive indexes) were obtained. Symptoms and the number of hospitalizations in the previous 6 months also were recorded. Patients then began using a ventilator for > 1 to 2 days, in order to select the type of ventilator and the appropriate interface. Patients returned for evaluation (in outpatient setting) every 6 months for a follow-up period of 3 years. At 6 months, polysomnography was repeated, and by the third year clinical and functional parameters had been reassessed. Results: All symptoms improved significantly with NIPPV therapy, when compared with the baseline values. The mean (± SD) Pa o 2 and FVC values increased at 36 months compared with baseline values (62.6 ± 7.1 vs 67.8 ± 8.8 mm Hg, respectively; and 37.9 ± 7.2% vs 47.5 ± 11.9%, respectively; p 2 O), maximal expiratory pressure (53.8 ± 17.7 to 72.3 ± 11.0 cm H 2 O), mouth pressure (0.28 ± 0.08 to 0.22 ± 0.02 cmH 2 O), and pressure-time index (0.18 ± 0.05 to 0.11 ± 0.02; p Conclusions: Long-term NIPPV therapy improves daytime blood gas levels, respiratory muscle performance, and hypoventilation-based symptoms in patients with severe kyphoscoliosis.