Acute respiratory failure in scoliosis or kyphosis: prolonged survival and treatment
Libby, D.M.; Briscoe, W.A.; Boyce, B.; Smith, J.P.
American Journal of Medicine 73(4): 532-538
1982
ISSN/ISBN: 0002-9343 PMID: 6812420 Document Number: 185868
Acute respiratory failure (ARF) in adults with severe thoracic spinal deformity is said to be a preterminal event with a median survival of 1 yr. Patients (20) with ARF (mean .+-. SD arterial O2 tension [PaO2] 35 .+-. 7 mm Hg, PaCO2 63 .+-. 9 mm Hg, pH 7.34 .+-. 0.08) due to severe scoliosis or kyphosis (spinal curve 113 .+-. 28.degree.) were seen between 1965-1980. All 20 survived the initial episode of ARF and during the follow-up period (median, 6 yr) experienced 2.4 additional episodes of ARF. The mean age at presentation was 52 yr (range 13-78) and the cause of spinal deformity was idiopathic (7 patients), poliomyelitis (7), tuberculosis (5) and arthrogryposis multiplex congenita (1). ARF was treated with controlled low-dose O2 by Venturi mask and intensive general measures in 13 patients and by mechanical ventilation in 7. Of the latter 7 patients, ventilatory failure was treated in 2 with a tank respiratory and a cuirass, avoiding endotracheal intubation. Outpatient management was similar to conventional therapy for chronic obstructive pulmonary disease (COPD). Severe restrictive ventilatory impairment characterized the group after recovery from the 1st episode of ARF: vital capacity (VC) 906 .+-. 362 ml (31% predicted) and FEV1 [1-s forced expiratory volume] 589 .+-. 197 ml (23%). During follow-up, the VC decreased by 1.5 ml/yr and the FEV1 by 12.9 ml/yr. The PaO2 increased by 2.6 mm Hg/yr and the PaCO2 increased by 1.7 mm Hg/yr. Successful management of ARF due to severe scoliosis or kyphosis is possible in the great majority of patients, and long-term survival may be expected. Unlike COPD, pulmonary function following ARF in kyphosis or scoliosis deteriorates at a slower than expected rate and may improve with treatment over many years.