Evaluation and medical management of giant bullae
- Fernando J Martinez, MD, MS
Fernando J Martinez, MD, MS
- Bruce Webster Professor of Medicine
- Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medical College
A bulla is defined as an air space in the lung measuring more than one centimeter in diameter in the distended state. The term giant bulla is used for bullae that occupy at least 30 percent of a hemithorax [1-4]. A single giant bulla may be present, or a giant bulla may be accompanied by a number of smaller adjacent bullae.
The evaluation and management of giant bullae in patients with emphysema will be reviewed here. The general management of chronic obstructive pulmonary disease and the roles of bullectomy, lung volume reduction surgery, and lung transplantation in the management of advanced emphysema are discussed separately. (See "Management of stable chronic obstructive pulmonary disease" and "Bullectomy for giant bullae" and "Lung volume reduction surgery in COPD" and "Lung transplantation: General guidelines for recipient selection".)
Giant bullae typically develop as a consequence of cigarette smoking, although some are idiopathic and others have less common associations. Marijuana smoking has been associated with giant bullae in a few patients, although this observation may be confounded by concomitant cigarette smoke exposure .
Bullous lung disease has also been described in association with HIV infection [6-8] and in intravenous drug users, particularly following injection of methadone, methylphenidate, or talc-containing drugs, although these relationships are not clearly established. In methylphenidate injectors, the pattern of emphysema is basilar, similar to that seen in patients with alpha-1 antitrypsin deficiency. (See "Overview of pulmonary disease in injection drug users", section on 'Bullous lung disease and emphysema'.)
Patients with Marfan's syndrome occasionally develop apical bullae, but these generally do not meet criteria for giant bullae. Similarly, bullae have been described in Ehlers-Danlos type IV, polyangiitis with granulomatosis, Sjögren's syndrome and sarcoidosis, but usually not giant bullae [9-12]. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)
- Thurlbeck WM. Pathophysiology of chronic obstructive pulmonary disease. Clin Chest Med 1990; 11:389.
- Palla A, Desideri M, Rossi G, et al. Elective surgery for giant bullous emphysema: a 5-year clinical and functional follow-up. Chest 2005; 128:2043.
- Neviere R, Catto M, Bautin N, et al. Longitudinal changes in hyperinflation parameters and exercise capacity after giant bullous emphysema surgery. J Thorac Cardiovasc Surg 2006; 132:1203.
- Celli BR, MacNee W, ATS/ERS Task Force. Standards for the diagnosis and treatment of patients with COPD: a summary of the ATS/ERS position paper. Eur Respir J 2004; 23:932.
- Johnson MK, Smith RP, Morrison D, et al. Large lung bullae in marijuana smokers. Thorax 2000; 55:340.
- Diaz PT, Clanton TL, Pacht ER. Emphysema-like pulmonary disease associated with human immunodeficiency virus infection. Ann Intern Med 1992; 116:124.
- Diaz PT, King MA, Pacht ER, et al. Increased susceptibility to pulmonary emphysema among HIV-seropositive smokers. Ann Intern Med 2000; 132:369.
- Mani D, Guinee DG Jr, Aboulafia DM. Vanishing lung syndrome and HIV infection: an uncommon yet potentially fatal sequela of cigarette smoking. J Int Assoc Physicians AIDS Care (Chic) 2012; 11:230.
- Inokuma S. Pulmonary involvement in Sjögren's syndrome. Intern Med 2002; 41:73.
- Mireles-Cabodevila E, Sahi H, Farver C, et al. A young patient with a minimal smoking history presents with bullous emphysema and recurrent pneumothorax. Chest 2007; 132:338.
- Clark JG, Kuhn C 3rd, Uitto J. Lung collagen in type IV Ehlers-Danlos syndrome: ultrastructural and biochemical studies. Am Rev Respir Dis 1980; 122:971.
- Mouly S, Brillet G, Stern M, et al. Pulmonary giant bulla in Wegener's granulomatosis. Scand J Rheumatol 2000; 29:333.
- Meyers BF, Patterson GA. Chronic obstructive pulmonary disease. 10: Bullectomy, lung volume reduction surgery, and transplantation for patients with chronic obstructive pulmonary disease. Thorax 2003; 58:634.
- Chen CW, Perng WC, Li MH, et al. Hemorrhage from an enlarged emphysematous bulla during commercial air travel. Aviat Space Environ Med 2006; 77:1275.
- Nagashima O, Suzuki Y, Iwase A, Takahashi K. Acute hemorrhage in a giant bulla. Intern Med 2012; 51:2673.
- Boushy SF, Kohen R, Billig DM, Heiman MJ. Bullous emphysema: clinical, roentgenologic and physiologic study of 49 patients. Dis Chest 1968; 54:327.
- Barker SJ, Clarke C, Trivedi N, et al. Anesthesia for thoracoscopic laser ablation of bullous emphysema. Anesthesiology 1993; 78:44.
- Ohta M, Nakahara K, Yasumitsu T, et al. Prediction of postoperative performance status in patients with giant bulla. Chest 1992; 101:668.
- Martinez FJ, Chang A. Surgical therapy for chronic obstructive pulmonary disease. Semin Respir Crit Care Med 2005; 26:167.
- Schipper PH, Meyers BF, Battafarano RJ, et al. Outcomes after resection of giant emphysematous bullae. Ann Thorac Surg 2004; 78:976.
- Nickoladze GD. Functional results of surgery for bullous emphysema. Chest 1992; 101:119.
- Sharma N, Justaniah AM, Kanne JP, et al. Vanishing lung syndrome (giant bullous emphysema): CT findings in 7 patients and a literature review. J Thorac Imaging 2009; 24:227.
- Stern EJ, Webb WR, Weinacker A, Müller NL. Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients. AJR Am J Roentgenol 1994; 162:279.
- Chen CK, Cheng HK, Chang WH, et al. Giant emphysematous bulla mimicking tension pneumothorax. Am J Med Sci 2009; 337:205.
- Chandra D, Rose SR, Carter RB, et al. Fluid-containing emphysematous bullae: a spectrum of illness. Eur Respir J 2008; 32:303.
- Asai N, Ohkuni Y, Matsunuma R, et al. Infectious giant bulla associated with lung cancer. J Bras Pneumol 2011; 37:404.
- Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX. Diffuse Cystic Lung Disease. Part I. Am J Respir Crit Care Med 2015; 191:1354.
- Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX. Diffuse Cystic Lung Disease. Part II. Am J Respir Crit Care Med 2015; 192:17.
- Snider GL. Reduction pneumoplasty for giant bullous emphysema. Implications for surgical treatment of nonbullous emphysema. Chest 1996; 109:540.
- Boasquevisque CH, Yildirim E, Waddel TK, Keshavjee S. Surgical techniques: lung transplant and lung volume reduction. Proc Am Thorac Soc 2009; 6:66.
- Wakabayashi A, Brenner M, Kayaleh RA, et al. Thoracoscopic carbon dioxide laser treatment of bullous emphysema. Lancet 1991; 337:881.
- Brenner M, Kayaleh RA, Milne EN, et al. Thoracoscopic laser ablation of pulmonary bullae. Radiographic selection and treatment response. J Thorac Cardiovasc Surg 1994; 107:883.
- Wakabayashi A. Thoracoscopic technique for management of giant bullous lung disease. Ann Thorac Surg 1993; 56:708.
- Gaensler EA, Jederlinic PJ, FitzGerald MX. Patient work-up for bullectomy. J Thorac Imaging 1986; 1:75.
- Hatakeyama S, Tatibana A, Suzuki K, Kobayashi R. [Five cases of lung cancer with emphysematous bullae]. Nihon Kokyuki Gakkai Zasshi 2001; 39:415.
- Maki D, Takahashi M, Murata K, et al. Computed tomography appearances of bronchogenic carcinoma associated with bullous lung disease. J Comput Assist Tomogr 2006; 30:447.
- Hirai S, Hamanaka Y, Mitsui N, et al. Primary lung cancer arising from the wall of a giant bulla. Ann Thorac Cardiovasc Surg 2005; 11:109.
- Arab WA, Echavé V, Sirois M, Gomes MM. Incidental carcinoma in bullous emphysema. Can J Surg 2009; 52:E56.
- Richardson MS, Reddy VD, Read CA. New air-fluid levels in bullous lung disease: a reevaluation. J Natl Med Assoc 1996; 88:185.
- Nakamura S, Kawaguchi K, Fukui T, et al. The development of large-cell carcinoma in the wall of a giant bulla complicated by hemorrhage. Surg Case Rep 2016; 2:22.
- Leatherman JW, McDonald FM, Niewohner DE. Fluid-containing bullae in the lung. South Med J 1985; 78:708.
- Chandra D, Soubra SH, Musher DM. A 57-year-old man with a fluid-containing lung cavity: infection of an emphysematous bulla with methicillin-resistant Staphylococcus aureus. Chest 2006; 130:1942.
- Peters JI, Kubitschek KR, Gotlieb MS, Awe RJ. Lung bullae with air-fluid levels. Am J Med 1987; 82:759.
- Takanami I. Endoscopic drainage of an infected giant bulla. Interact Cardiovasc Thorac Surg 2006; 5:794.
- Bull TM, Keith RL, Kraft M, et al. Mycobacterium tuberculosis presenting as an infected pulmonary bulla. Int J Tuberc Lung Dis 2003; 7:504.
- Kirschner LS, Stauffer W, Krenzel C, Duane PG. Management of a giant fluid-filled bulla by closed-chest thoracostomy tube drainage. Chest 1997; 111:1772.
- Chon SH, Shinn SH, Lee CB. Giant fluid-filled bulla treated by instillation of antibiotics after percutaneous drainage. Thorac Cardiovasc Surg 2010; 58:122.
- Hata Y, Takagi K, Sasamoto S, et al. Infected giant bulla treated by percutaneous drainage followed later by resection: report of a case. Surg Today 2007; 37:656.
- CLINICAL MANIFESTATIONS
- Laboratory testing
- Pulmonary function tests
- DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
- Asymptomatic patients
- Dyspneic patients
- Refractory dyspnea due to giant bulla
- Bronchogenic carcinoma
- Fluid-containing bullae
- - Imaging characteristics
- - Benign, sterile bullous fluid
- - Infected bullae
- - Malignancy
- SUMMARY AND RECOMMENDATIONS