Refractory Bilateral Pneumothoraces in End-Stage Cystic Fibrosis

Abstract

A23-year-old woman with cystic fibrosis and advanced lung disease, requiring tracheostomy, was ransferred for treatment of acute chronic respiratory ailure. Her arterial blood gas at time of the initial ransfer revealed hypercapnic respiratory failure (pH .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 ung transplantation with venovenous extracorporeal embrane oxygenation (VV ECMO). A 27 Fr bi-caval ual lumen catheter (Avalon Laboratories, LLC, Los ngeles, CA) was subsequently placed through the right