VANISHING LUNG SYNDROME OR GIANT BULLOUS EMPHYSEMA

Abstract

TYPE: Abstract Publication TOPIC: Obstructive Lung Diseases PURPOSE: We report a case of Giant Bullous Emphysema (Vanishing Lung Syndrome) that presented with pneumothorax in a smoker/COPD case. METHODS: A 40 years old female, current smoker, presented with pleuritic chest pain and dyspnoea. A chest x-ray showed a large right pneumothorax that was managed with a chest drain. However, pneumothorax recurred post-discharge and required another chest drain. Due to recurrence, a CT chest was performed, that showed bilateral giant bullae that occupied more than one-third of the lungs, located predominantly in upper lobes. The remaining lung parenchyma appeared normal except minor/early centrilobular emphysema. Alpha 1-antitrypsin levels were normal. RESULTS: CT Chest findings were consistent with giant bullous emphysema. She successfully managed to quit smoking and had a right-sided thoracoscopic bullectomy with resolution in her symptoms. CONCLUSIONS: A major complication of giant bullous emphysema is pneumothorax, that may pose a diagnostic challenge on a chest -x-ray in the presence of a giant bulla. A CT thorax remains an important tool to define the extent/distribution of bullous disease, pneumothorax, associated emphysema, and to assist immediate (chest drain) and long-term management (pre-operative planning). Smoking cessation and surgical resection of giant bullae (if occupy >1/3rd of lung) remain an effective strategy with improvement in dyspnoea, lung functions and exercise capacity. CLINICAL IMPLICATIONS: Vanishing Lung Syndrome is a rare clinical entity, that tends to be progressive, however, there appear to be no guidelines on its management or follow-up. The role of new interventions such as endoscopic lung volume reduction (ELVRS) remains undefined in the management of Giant Bullous Emphysema. DISCLOSURE: No significant relationships. KEYWORDS: Emphysema, Giant Bullae, Vanishing Lung