Vanishing Lung Syndrome, or Idiopathic Giant Bullous Emphysema, with Pneumothorax, and Subcutaneous Emphysema in a 58-Year-Old Female Smoker with Chronic Obstructive Pulmonary Disease

Abstract

Patient: Female, 58-year-old Final Diagnosis: Giant bullae • spontaneous pneumothorax • subcutaneous emphysema Symptoms: Crepitus • dysphagia • dysphonia • facial swelling Medication: — Clinical Procedure: — Specialty: Critical Care Medicine • Pulmonology Objective: Unusual clinical course Background: Vanishing lung syndrome (VLS), also known as idiopathic giant bullous emphysema, is a rare manifestation of chronic obstructive pulmonary disease (COPD) and usually occurs in middle-aged smokers. This report presents a 58-year-old female smoker with COPD and VLS who presented with spontaneous pneumothorax. The pneumothorax was managed with a chest tube and was later complicated by massive subcutaneous emphysema. Case Report: A 58-year-old woman with a past medical history of long-term smoking and COPD presented with worsening shortness of breath. Upon initial evaluation, she had tachypnea and hypoxia (SpO 2 93%). Chest radiography revealed a new right-sided pneumothorax on top of extensive bullous disease, which the patient already had. The drainage of the pneumothorax was successful with a pigtail catheter. However, during the following night, after insertion of the pigtail catheter, the patient developed massive subcutaneous emphysema, which was confirmed with imaging. The patient remained hemodynamically stable, and diffuse subcutaneous crepitus was present on examination. The pigtail catheter was repositioned, resulting in complete resolution of the subcutaneous emphysema in the following 2 weeks. Conclusions: This case highlights the importance of a timely diagnosis and management of the possible presentations and complications of VLS. Complications such as pneumothorax are life-threatening and require urgent management, taking precedence over the curative treatment for VLS, surgical bullectomy.

Vanishing Lung Syndrome in a Young Male With Chronic Marijuana Use: A Case Report

“…Studies have shown that there are higher rates of emphysematous disease among marijuana smokers when compared to non-smokers and tobacco-only smokers [3]. High-resolution computed tomography scan (HRCT) remains the standard modality for diagnosis of VLS and its complications; with imaging demonstrating asymmetric subpleural bullae located unilaterally and predominately affecting the upper lobes, as was seen in this case [2,4].…”

“…VLS or type I bullous emphysema is a condition that most commonly affects people over age 45 with concomitant tobacco smoking or at an earlier age in patients with a history of alpha-1 antitrypsin deficiency and/or marijuana use [2]. Cannabis is the most widely used illicit substance in the world and the second most smoked substance after tobacco products, with emphysematous disease being more prevalent amongst marijuana smokers compared to non-smokers and tobacco-only smokers [1].…”

“…Chronic marijuana smoking can lead to parenchymal and histologic changes because of airway inflammation and irritation secondary to prolonged inhalation of toxic particles and elevated inspiratory pressures from deep inhalation. Histopathologic studies have demonstrated extensive goblet cell hyperplasia, blunting of ciliated epithelial cells, intra-alveolar accumulation of pigmented histiocytes, and extensive inflammation of the intraepithelial and subepithelial cells [1,2]. These changes can be seen as poorly defined centrilobular groundglass opacities on imaging and can be differentiated between tobacco-induced bullae which are typically centrilobular and uniformly distributed while marijuana-induced bullous emphysema is situated in a preseptal distribution along the upper lobes [5].…”

“…This case describes a rare cause of emphysematous lung disease known as vanishing lung syndrome (VLS), a type I bullous emphysema or idiopathic bullous disease characterized by irreversible lung parenchymal damage resulting in giant bullous emphysema involving one or both lungs [1,2]. It is characterized as a progressive lung disease that can present with dyspnea, chest tightness, and respiratory failure due to inadequate participation in gas exchange [2,3]. Progression of the disease can lead to pneumothorax, subcutaneous emphysema, and respiratory failure with hypoxia and hypercapnia.…”

“…Progression of the disease can lead to pneumothorax, subcutaneous emphysema, and respiratory failure with hypoxia and hypercapnia. The most common factors known to cause the development of VLS include alpha-1 antitrypsin deficiency, human immunodeficiency virus (HIV), Ehlers-Danlos syndrome, Marfan syndrome, tobacco smoking, marijuana smoking, and sarcoidosis [2,4]. Studies have shown that there are higher rates of emphysematous disease among marijuana smokers when compared to non-smokers and tobacco-only smokers [3].…”