Pneumothorax With Extensive Subcutaneous Emphysema in Combined Pulmonary Fibrosis Emphysema (CPFE) Syndrome

Document Type

Conference Proceeding

Publication Date

5-21-2023

Publication Title

American Journal of Respiratory and Critical Care Medicine

Abstract

Introduction: There is increasing recognition of CPFE (Combined Pulmonary Fibrosis Emphysema) syndrome which is characterized by upper lobe predominant emphysema and lower lobe fibrosis. Usual interstitial pneumonia/idiopathic pulmonary fibrosis (IPF) appears to be the most commonly seen pattern in CPFE. Emphysema and pulmonary fibrosis predispose these patients to SSP (secondary spontaneous pneumothorax). We report a case of CPFE in which SSP complicated to subcutaneous emphysema. Case presentation: A 90-year-old gentleman with a past medical history of CPFE on 4 L/min (liters/minute) of baseline oxygen presented to the emergency department with acute onset shortness of breath and chest pain. Initially, he was saturating 60% on 4 L/min and had a systolic blood pressure of 70 millimeters of mercury. He was put on bilevel positive airway pressure ventilation. On auscultation, breath sounds were absent on the left side. Bedside chest-x-ray was obtained emergently which showed a large left-sided basilar pneumothorax with the mediastinal shift. Chest tube was placed imminently with improvement in the patient's vital signs and resolution of symptoms. A large air leak was noted from the chest tube and was continued in suction. In the next 3 days, he remained stable but continued to have a large air leak from the chest tube. He underwent robot and video-assisted thoracoscopy on which lung parenchymal injury was noted secondary to initial chest tube insertion. The injury was sutured with interval placement of two 28 French chest tubes. The next day, a rapid response was called as the patient became acutely hypoxic with oxygen requirements of 30 L/min via high-flow nasal cannula. CT (Computed Tomography) chest with intravenous contrast was obtained which showed extensive severe subcutaneous emphysema, pneumomediastinum, with the chest tube in the region of pneumothorax (figure). Due to multiple comorbidities, the patient was made comfort care and passed the next day. Discussion: SSP, commonly seen with COPD (Chronic Obstructive Pulmonary Disease) and interstitial lung disease is associated with higher mortality and recurrence rates. Persistent air leaks are seen in cases with lower DLCO, lower FEV1, and upper lobe predominant emphysema as in this case. It is advised to obtain a thoracic surgery opinion along with pleurodesis and endobronchial valve in select surgical candidates. Management of extensive subcutaneous emphysema requires addressal of the underlying cause or insertion of subcutaneous drains depending on the scenario. (Figure Presented).

Volume

207

Issue

1

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