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INTRODUCTION: Pneumothorax occurs in chronically ventilated neuromuscular and chest wall-restricted patients. Non-invasive home mechanical ventilation (HMV) and airway clearance techniques are standard care, but are perceived to put patients at increased risk of pneumothorax. Malnutrition as reflected by low body mass index was identified as an association with pneumothorax in this case series. METHODS: Retrospective chart review of six cases of pneumothorax in patients with neuromuscular and chest wall diseases receiving chronic HMV. RESULTS: Six cases were reviewed. Median duration of HMV at time of the pneumothorax was 3 years (range 0.25 to 9 years). 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 16 ± 3 kg/m2. Five of 6 patients required chest drain and 4 of 6 had recurrent pneumothoraxes. One patient received chemical pleurodesis. CONCLUSION: Pneumothorax occurs in neuromuscular patients receiving HMV and using airway clearance techniques. Management can include reducing ventilator pressure settings, holding airway clearance techniques and use of a chest drain. Chemical or surgical pleurodesis were felt to be high risk. Recurrence of pneumothorax was common. Low BMI in this case series raises the possible association between malnutrition and risk of pneumothorax in patients receiving HMV.
