Treatment of subcutaneous and mediastinal emphysema with cuirass ventilation in a patient with Pompe disease undergoing non-invasive positive pressure ventilation

Abstract

We report a case of subcutaneous and mediastinal emphysema in a 39-year-old woman with late-onset Pompe disease who was undergoing non-invasive positive pressure ventilation (NPPV). Although the patient had a history of pneumothorax, she did not present with pneumothorax at the time of admission. She had not undergone adequate respiratory rehabilitation, which resulted in decreased respiratory compliance. We speculated that the emphysema had been caused by an increase in the airway pressure due to NPPV. Decrease in inspiratory pressure of NPPV from 14 cm H2O to 9 cm H2O made the patient dyspneic and hypoxic. Cuirass ventilation by itself resulted in dyspnea and hypoxia. By using a combination of cuirass ventilation (control mode) and NPPV (assist/control mode), we were able to decrease the inspiratory pressure to 7 cm H2O. After 26 days of treatment the patient recovered from subcutaneous and mediastinal emphysema. After the treatment her maximum inspiratory capacity was increased from 400 ml to 600 ml, which indicates increased thoracic compliance. Thus, a combination of cuirass ventilation and NPPV is beneficial in managing barotrauma that may occur during NPPV in a respirator-dependent patient.