Gorham syndrome with postoperative respiratory failure and requiring prolonged mechanical ventilation
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.