PT  - JOURNAL ARTICLE
AU  - Shiang-Yu Huang
AU  - Ying-Min Lee
AU  - Shiau-Tzu Tzeng
AU  - Chiu-Ping Su
AU  - Shiu-Feng Huang
AU  - Yao-Kuang Wu
AU  - Chou-Chin Lan
TI  - Gorham Syndrome with Post-operative Respiratory Failure and Requiring Prolonged Mechanical Ventilation
AID  - 10.4187/respcare.02355
DP  - 2013 Apr 02
TA  - Respiratory Care
PG  - respcare.02355
4099  - http://rc.rcjournal.com/content/early/2013/04/02/respcare.02355.short
4100  - http://rc.rcjournal.com/content/early/2013/04/02/respcare.02355.full
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.