Pneumothorax In Chronically Ventilated Neuromuscular And Chest Wall Restricted Patients: A Case Series

RATIONALE: Pneumothorax can occur in chronically ventilated neuromuscular and chest wall-restricted patients using noninvasive home mechanical ventilation (HMV) and airway clearance techniques. Limited descriptions of these cases and their management exist in the literature.OBJECTIVES: To understand the unique considerations in diagnosis and management of pneumothorax in the neuromuscular and chest wall-restricted patient population.METHODS: A retrospective chart review was performed at the Peter Lougheed Neuromuscular Respiratory Clinic between 2006 and 2017. A case series of six patients is presented.RESULTS: Median duration of HMV at time of the pneumothorax was 3years (range 0.25-9years). Five patients used airway clearance techniques. The mean forced vital capacity (FVC) was 1.0L (27% predicted). Patients had a low mean body mass index (BMI) of 163kg/m(2). Five of 6 patients required chest tube and had prolonged air leak (>48hours). Four of 6 had recurrent pneumothoraces. One patient received chemical pleurodesis.CONCLUSIONS: Clinical suspicion and rapid diagnosis are necessary for pneumothorax in neuromuscular and chest wall-restricted patients dependent on HMV. CT imaging of the chest may be necessary. Management can include: reducing ventilator pressure settings, suspending airway clearance techniques and chest tube insertion. Chemical or surgical pleurodesis are felt to be high risk; however, recurrent pneumothorax was frequent in this cohort. Low BMI raises the possible association between malnutrition and risk of pneumothorax in patients receiving HMV.