RT Journal Article
SR Electronic
T1 Gorham Syndrome with Post-operative Respiratory Failure and Requiring Prolonged Mechanical Ventilation
JF Respiratory Care
FD American Association for Respiratory Care
SP respcare.02355
DO 10.4187/respcare.02355
A1 Shiang-Yu Huang
A1 Ying-Min Lee
A1 Shiau-Tzu Tzeng
A1 Chiu-Ping Su
A1 Shiu-Feng Huang
A1 Yao-Kuang Wu
A1 Chou-Chin Lan
YR 2013
UL http://rc.rcjournal.com/content/early/2013/04/02/respcare.02355.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. Here is a report of a 34-year-old male with Gorham syndrome presenting as progressive kyphosis with severe back pain and unstable gait. He also had exertional dyspnea. Pulmonary function test revealed severe restrictive ventilatory impairment. He underwent spinal operation but could not be extubated after surgery. Post-operative pulmonary complications of left lower lung pneumonia and respiratory failure required prolonged mechanical ventilation. After a weaning program of pressure support ventilation and T-piece trials, the patient was successfully weaned from mechanical ventilation.