Rigid spine syndrome associated with marked hypoxemia and hypercapnia
Toh, Y.; Yoshizawa, A.; Kubo, M.; Koshino, T.; Horiuchi, T.; Kudo, K.; Kabe, J.
Nihon Kyobu Shikkan Gakkai Zasshi 34(5): 605-609
1996
ISSN/ISBN: 0301-1542 PMID: 8753123 Document Number: 465781
A 48-year-old man was referred to our hospital because of hypoxemia (PaO2 = 43 mmHg), hypercapnia (PaCO2 = 70 mmHg), complete atrio-ventricular block, and heart failure. He also had limitation of spine flexion, scoliosis, deformity of the rib cage, and constriction of the ankle joints, complicated by cor pulmonale. These findings were compatible with rigid spine syndrome. To avoid progressive pulmonary hypertension and hypoxemia, nasal BiPAP and home oxygen therapy (0.5 liters/minute) were begun. Rigid spine syndrome is clinically characterized by limitation of spine flexion, and the limitation of thoracic movement often causes severe constrictive respiratory dysfunction. This syndrome should be considered when evaluating patients who have both thoracic deformity, especially scoliosis, and respiratory failure.