A 56‐year‐old African American man presented to the emergency room complaining of shortness of breath, wheezing, and productive cough with yellowish sputum. His symptoms began acutely 1 day prior to presentation and were not associated with any fevers or chills. At baseline, the patient was ambulatory and independent in ADLs, but with limited exercise tolerance due to chronic dyspnea and fatigue. Medical history was notable for emphysema, with an extensive smoking history of more than 50 pack‐years. Surgical history was notable for right chest tube insertion 5 years prior due to the development of a pneumothorax. Review of symptoms was negative for chest pain, palpitations, dizziness, or hemoptysis and positive for weight loss. He was found to have elevated WBCs and lactate and was hypercarbic with respiratory acidosis. Chest x‐ray showed extensive bilateral bullous disease with inferior left lung and right midlobe compression, volume loss, and mediastinal shift to the right. Chest CT confirmed severe bullous emphysema replacing the majority of both lungs and collapse of the right middle lobe. He was started on nebulizers, steroids, and antibiotics, placed on BiPAP with 80% FIO2, and sent to ICU for acute respiratory failure. He improved slowly over the hospital course and on discharge remained on O2 therapy and daily nebulizers, and was referred to CT surgery for lung transplant evaluation.
Bulla refers to a thin‐walled, sharply demarcated airspace in the lung that is greater than 1 cm in diameter, and the term giant bulla is used when the airspace is greater than 30% of the hemithorax. The disease known as giant bullous emphysema was originally described in 1937 as “vanishing lung syndrome.” It affects approximately 1:1100 people and usually young males who are smokers. The majority of giant bullae are typically associated with emphysema as a consequence of cigarette smoking, but have been described in patients with Marfan's syndrome, Sjögren's, sarcoidosis, and HIV patients who are injection drug users. Pathophysiology and symptoms are similar to those of other causes of emphysema. In giant bullous emphysema, the bullae compress the normal lung parenchyma and often displace it centrally. On exam, there may be hypoxia, tachypnea, decreased breath sounds, and/or barrel chest and clubbing. Definitive diagnosis is made by HRCT.
Complications most often include spontaneous pneumothorax (which our patient had in the past), and more commonly infected bullae from adjacent lung infections. Medical treatment is O2 therapy, inhaled anticholinergics, short‐acting b‐agonists, and smoking cessation. Surgical options for bullectomy, via VATS or open, can be done to reduce bullae in order to release compressed lung tissue, thereby improving lung mechanics and air exchange. However, though this may slightly improve life expectancy, long‐term prognosis remains poor.
Chest CT showing large bullae, worse on left, and subsequent compressed lung tissue and mediastinal shift to the right.
Chest X‐ray from ED showing loss of lung markings, worse on left, bibasilar infiltrates consistent with atelectasis and a mediastinal shift to the right.
To cite this abstract:Mayer K, Sison L, Gurunathan R. Holy Bullae, Batman! the Case of the Vanishing Lung. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 355. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/holy-bullae-batman-the-case-of-the-vanishing-lung/. Accessed January 22, 2020.