RT Journal Article
SR Electronic
T1 Gorham Syndrome With Postoperative Respiratory Failure and Requiring Prolonged Mechanical Ventilation
JF Respiratory Care
FD American Association for Respiratory Care
SP e144
OP e148
DO 10.4187/respcare.02355
VO 58
IS 11
A1 Huang, Shiang-Yu
A1 Lee, Ying-Min
A1 Tzeng, Shiau-Tzu
A1 Su, Chiu-Ping
A1 Huang, Shiu-Feng
A1 Wu, Yao-Kuang
A1 Lan, Chou-Chin
YR 2013
UL http://rc.rcjournal.com/content/58/11/e144.abstract
AB Gorham syndrome is a rare disease that presents as progressive osteolysis, and may affect any part of the skeleton. The pathologic process involves the replacement of normal bone by aggressively expanding but non-neoplastic vascular tissue, resulting in massive osteolysis of the adjacent bone. If the spine and ribs are affected, the subsequent kyphosis and chest wall deformity may cause severe restrictive ventilatory impairment. We report a 34-year-old male with Gorham syndrome presenting as progressive kyphosis, severe back pain, unstable gait, and exertional dyspnea. Pulmonary function testing revealed severe restrictive ventilatory impairment. He underwent spinal surgery but could not be extubated after surgery. Postoperative left lower lung pneumonia and respiratory failure required prolonged mechanical ventilation. After a weaning program of pressure support ventilation and T-piece spontaneous breathing trials, he was successfully weaned from mechanical ventilation.