Ann Thorac Surg 2011;92:1905. doi:10.1016/j.athoracsur.2011.04.068
© 2011 The Society of Thoracic Surgeons
Images in Cardiothoracic Surgery
Refractory Bilateral Pneumothoraces in End-Stage Cystic Fibrosis
Don Hayes, Jr, MDa,*,
Jeffrey A. Golden, MDb,
Charles W. Hoopes, MDa
a University of Kentucky Medical Center, Lexington, Kentucky
b University of California at San Francisco Medical Center, San Francisco, California
* Address correspondence to Dr Hayes, Advanced Lung Disease and Lung Transplant Programs, C424 University of Kentucky Medical Center, 800 Rose St, Lexington, KY 40536 (Email: don.hayes{at}uky.edu).
A 23-year-old woman with cystic fibrosis and advanced lung disease, requiring tracheostomy, was transferred for treatment of acute chronic respiratory failure. Her arterial blood gas at time of the initial transfer revealed hypercapnic respiratory failure (pH 7.29; PCO2 83 mm Hg; PO2 124 mm Hg. Figure 1
shows the chest roentgenogram at admission, which demonstrated bilateral pneumothoraces. She was found to have significant bronchopleural fistula with continuous air leak. Despite all our efforts of respiratory support with mechanical ventilation and refractory pneumothoraces, her hypercapnic respiratory failure progressed with a follow-up arterial blood gas (pH 7.27; PCO2 105 mm Hg; PO2 42 mm Hg. The decision was made to bridge her to lung transplantation with venovenous extracorporeal membrane oxygenation (VV ECMO). A 27 Fr bi-caval dual lumen catheter (Avalon Laboratories, LLC, Los Angeles, CA) was subsequently placed through the right internal jugular vein, and soon after the VV ECMO was started, mechanical ventilation was discontinued. Although the patient was on the VV ECMO, she was ambulated and thus received rehabilitation with physical therapy, and her nutrition was maximized with a regular diet. With removal of the mechanical ventilation due to treatment with VV ECMO, re-expansion of both lungs occurred during the next 48 hours, as seen in Figure 2
. The bronchopleural fistula actually resolved during the next 10 days. She eventually underwent bilateral sequential lung transplantation after 15 days of ambulatory VV ECMO. The surgical procedure and postoperative course were unremarkable. The patient continues to do well, and she is now home with an excellent quality of life and no limitations 8 months after transplantation. In hindsight, we would have proceeded with VV ECMO, even if transplantation was not the desired therapeutic alternative, because the removal of mechanical ventilation at the time allowed resolution of the pneumothoraces and bronchopleural fistula.