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CASE REPORT
Year : 2007  |  Volume : 24  |  Issue : 1  |  Page : 30-32 Table of Contents   

Unilateral bullous emphysema of lung


1 Department of Surgery, Jawaharlal Nehru Medical College, AMU, Aligarh, U.P-202002., India
2 Department of Tuberculosis & Chest Diseases, Jawaharlal Nehru Medical College, AMU, Aligarh, U.P-202002., India

Correspondence Address:
N N Shah
Department a Chest Diseases, Jawaharlal Nehru Medical College, AMU, Aligarh, U.P-202002.
India
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DOI: 10.4103/0970-2113.44202

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   Abstract 

Emphysematous bullae in a lung can coalesce into a large lucency and may cause a shift of mediastinal structures. The present report describes a case of multiple emphysematous bullae in a 35 years old man that occupied the entire left hemi­thorax with mediastinal compression of the right lung. The patient underwent a left sided thoracotomy with a left pneumonectomy. The patient had an uneventful recovery.

Keywords: Bulla; Emphysema; Dyspnea


How to cite this article:
Shah N N, Bhargava R, Ahmed Z, Pandey D K, Shameem M, Bachh A A, Akhtar S, Dar K A, Mohsina M. Unilateral bullous emphysema of lung. Lung India 2007;24:30-2

How to cite this URL:
Shah N N, Bhargava R, Ahmed Z, Pandey D K, Shameem M, Bachh A A, Akhtar S, Dar K A, Mohsina M. Unilateral bullous emphysema of lung. Lung India [serial online] 2007 [cited 2014 Oct 31];24:30-2. Available from: http://www.lungindia.com/text.asp?2007/24/1/30/44202


   Introduction Top


Emphysematous disease of the lungs and its complications account for a significant number of visits to the emergencies every year [1] . Emphysema refers to the abnormal and permanent enlargement of air spaces distal to the terminal bronchioles, characterized by hyperinflation and destruction of the alveolar walls. Rupture of alveolar air into the interstitium with dissection into the visceral pleura results in formation of pulmonary blebs. In advanced stages, bullae may form in the subpleural space, commonly at the apices of the lung. Bullae are air-filled, thin-walled spaces greater than 2 centimeter in diameter in the distended state [2] . Giant bullae are those that encompass more than one-third of the lung volume. They are uncommon but when present can lead to compression of adjacent normal lung tissue. The presence of emphysema associated with large bullae is referred to as bullous emphysema. It is either congenital without general lung disease or a complication of chronic obstructive lung disease with generalized lung disease.

We present a case of a 35 years old male with unilateral bullous emphysema where multiple bullae in the left lung coalesced into a large lucency causing shift of the mediastinum to the opposite side.


   Case Report Top


A 35 years old man presented with increasing shortness of breath for last 3 months with worsening exercise intolerance. The patient had a greater than 20­pack year history of tobacco use. He denied cough, sputum production, hemoptysis, night sweats, fever or chest pain. There was no history of any past medical problems, hospitalization or surgery. He reported no allergies. He denied alcohol or substance use.

Physical examination revealed a young male, alert and oriented without any respiratory distress speaking full sentences. Vital signs were: blood pressure 122/74 mmHg, pulse 92 beats/min, respiratory rate 22 breaths/ min, temperature 35.8 0 C, pulse oximetry 97% on room air. There was no cyanosis, clubbing or peripheral edema. Chest examination results were: non-traumatic, symmetrical with chest accessory muscle of respiration working. Trachea was shifted to right. Tactile fremitus was absent on left side with ipsilateral diminution of breath sounds without dullness to percussion. No added sounds were present. Cardiac examination revealed a rapid rate but no murmurs. The apex beat was shifted retrosternally. Heart sounds were auscultated over the right hemithorax. Rest of the examination was unremarkable. All routine blood investigations were within normal limits. An electrocardiogram showed a normal sinus rhythm of 90 beats/min, with axis shifted to right without any evidence of acute ischaemia. Chest roentgenogram showed hyper translucent left lung with shift of the mediastinum to the right compressing the right lung [Figure 1]. A computed tomography (CT) scan of thorax was done, which revealed large emphysematous bullae in whole of the left lung with shift of mediastinum to the right [Figure 2]. His alpha-1 antitrypsin levels were 186 IU/L (normal 93­224). Preoperative pulmonary function tests (PFT) were consistent with an obstructive pattern (Forced expiratory volume in 1 sec (FEV 1 ) 1.71L; 45% predicted).

Because of the severe unilateral nature of the bullae, with essentially no functioning left lung and mediastinal compression of the right lung, a left pneumonectomy was done through a left thoracotomy. The patient's breathing improved significantly postoperatively and follow up pulmonary function test results after the operation were slightly improved (FEV 1 2.21 L; 52% predicted).


   Discussion Top


Bullous emphysema was originally described by Burke in 1937 as an idiopathic, distinct clinical syndrome of severe progressive dyspnea caused by extensive, predominantly asymmetric upper lobe emphysema that may eventually lead to respiratory failure [3] . It is mainly seen in young men and is characterized by the presence of large progressive bullae that occupy a significant volume of a hemithorax and are often asymmetrical. It is often referred to as giant bullous emphysema, vanishing lung syndrome, or primary bullous disease of the lung.

Patients with bullous emphysema may be asymptomatic, the diagnosis being made in course of routine chest radiography, or may complain of progressive dyspnea or chest pain over several months due to the gradual increase in the size of the bullae [4] , though they may occasionally regress spontaneously [5] . They may develop sudden severe breathlessness due to the development of a spontaneous pneumothorax or sudden increase in size of the bulla due to air trapping. Increase in cough with sputum production usually indicates infection in a bulla [6] . Most of the patients of bullous emphysema are cigarette smokers. In elderly non-smokers, bullous emphysema is often associated with alpha-1 antitrypsin deficiency and there is usually a family history of emphysema at a young age [7] . Young patients with large bullae are predisposed to lung cancers [8],[9],[10] . The high incidence of lung cancer may be due to the fact that lung cancer occurs more frequently in lung scars that predispose to the development of bullae or the dystrophic changes caused by the bullae in the lung parenchyma. Poor ventilation of the bullae may lead to accumulation of carcinogens in them [11] .

The physical findings in a patient reflect the state of the lungs overall, or in immediate vicinity of the bulla. Giant bullae cause a localised decrease or absence of breath sounds and an associated increase in resonance to percussion.

Chest radiography is the most practical method for identifying the presence of bullae and their progression. However, occasionally it is difficult to differentiate the hairline shadows produced by a bulla from irregular walls of a cavity or cysts in the lung parenchyma. A forced expiratory film is sometimes helpful in demonstrating the presence of bullae on a chest radiograph: air trapping during the expiratory maneuver accentuates their outline by preventing a decrease in size as the surrounding lung empties. Large bulla sometimes displace the mediastinum contralaterally and may even compress the opposite lung [11] , as was in our case. Several studies now describe the utility of ultrasound in detection of bullae and differentiating then from a pneumothorax [12] . A typical 'comet tailing' phenomenon of the movement of the lung tissue against the pleura during respiration can be seen in bullous disease, but is absent when the lung is collapsed as in pneumothorax [13] .

CT scans are the most accurate means of detecting emphysema, determining its type and extent [14] and distinguishing giant bullae from pneumothorax [15] . A bulla is identified as area of transradiancy that usually do not contain blood vessels and is confined by visible walls. Patients with giant bullous emphysema developing a secondary spontaneous pneumothorax can also be detected. The double wall sign is a valuable sign to help distinguish a pneumothorax from adjacent giant bulla [16] . This sign occurs due to the air outlining both sides of the bulla wall parallel to the chest wall.

Pre-operative evaluation can be done by nuclear medicine techniques. A perfusion scan provides a qualitative assessment of the pulmonary vasculature. Findings of ventilation scans depends upon the technique: a single breath 133Xe Scan fails to demonstrate ventilation of a bulla, while as a continuous ventilation scan shows slow filling and emptying of a bulla or absence of filling during all phases in non communicating bullae [11] .

Asymptomatic bullae are treated conservatively by reassurance, advise to stop smoking, avoid strenuous activities like scuba diving that can promote the rupture of the bullae. Patients are advised an annual chest radiograph and an alert for a prompt visit to a physician should symptoms develop. Infection of a bulla is treated by antibiotics and chest physiotherapy. Patients with giant fluid filled bullae have been successfully treated by closed chest thoracostomy tube drainage; symptomatic patients with progressive dyspnea should undergo bullectomy. Indications for surgery with giant bullae are (1) increasing bulla size (2) pneumothorax (3) pulmonary insufficiency, and (4) infection within the bulla [17],[18] .

Total pneumonectomy is indicated in those with severe unilateral bullae with essentially no functioning lung [19] .

 
   References Top

1.Mandavia DR, Dailey RH. Chronic obstructive pulmonary disease. In: Rosen P. ed. Textbook of emergency medicine: concepts and clinical practice; 5th edn. 2002.  Back to cited text no. 1    
2.CIBA Guest Symposium. Terminology, definitions, and classification of chronic pulmonary emphysema and related conditions. Thorax 1959; 14: 286-99.  Back to cited text no. 2    
3.Burke R. Vanishing lungs: a case report of bullous emphysema. Radiology 1937;28: 367-71.  Back to cited text no. 3    
4.Boushy SF, Kohen R, Billing DM, Heiman MJ. Bullous emphysema: clinical, roentgenologic and physiologic study of 49 patients. Dis Chest 1968; 54: 327-34.  Back to cited text no. 4    
5.Stanescu D, Veriter CL. Spontaneous regression of a giant pulmonary bulla. Thorax 1996; 51: 1283.  Back to cited text no. 5    
6.Peters JI, Kubitschek KR, Gotleib MS, Awe RJ: Lung bullae with air-fluid levels. Am J Med 1987; 82: 759-63.  Back to cited text no. 6    
7.Jack CI, Evans CC. Three cases of alpha-I antitrypsin deficiency in the elderly. Postgrad Med J 1991; 67: 840-2.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Arongerg DI, Sagel SS, LeFrak S, Kuhn C, Susman N. Lung carcinoma associated with bullous lung disease in young men. AJR Am J Roentgenol 1980;134:249-52.  Back to cited text no. 8    
9.Goldstein MJ, Snider GL, Liberson M et al. Bronchogenic carcinoma and giant bullous disease. Am Rev Respir Dis 1968;97:1062-70.  Back to cited text no. 9    
10.Nakamura H, Takamori S, Miwa K, Fukunaga M, Maeshiro K, Matsuo T, et al. Rapid growth lung cancer associated with a pulmonary giant bulla: a case report. Kurume Med J 2003;50:147-50.  Back to cited text no. 10  [PUBMED]  
11.Murphy DM, Fishman AP. Bullous disease of the lung. In: Fishman AP, Elias JA, Fishman JA, Grippi MA, Kaiser LR, Robert M Sr, editors. Fishman's Pulmonary Diseases and Disorders; 3rd edn; Vol 1. New York: McGraw-Hill;1997.p 849-63.  Back to cited text no. 11    
12.Chan SS. Emergency bedside ultrasound to detect pneumothorax. Acad Emerg Med 2003;10: 91-4.  Back to cited text no. 12  [PUBMED]  
13.Simon BC, Paolinetti L. Two cases where bedside ultrasound was able to distinguish pulmonary bleb fzom pneumothorax. J Emerg Med. 2005.29:201-5.  Back to cited text no. 13    
14.Thurlbeck WM. Chronic airflow obstruction. Correlation of structure and function. In: Petty TL, editor. Chronic obstruction pulmonary disease.2nd edn. New York: Dekker, 1985.p 129.  Back to cited text no. 14    
15.Waseem M, Jones j, Brutus S, Munyak j, Kapoor R, Gernsheimer J. Giant bulla mimicking pneumothorax. J EmergMed 2005;29:155­-8.  Back to cited text no. 15    
16.Phillips GD, Trotman-Dickensen B, Hodson ME, Geddes DM. Role of CT in the management of pneumothorax in patients with complex cystic lung disease. Chest 1997;112: 275-8.  Back to cited text no. 16    
17.Laros CD, Gelissen H1, Bergstein PGM, Van den Bosch JM, Vanderschueren RG, Westermann CJ et al. Bullectomy for giant bullae in emphysema. J Thorac Cardiovasc Surg 1986; 91: 63-70.  Back to cited text no. 17    
18.Mehran R1, Deslauriers J. Indications for surgery and patient work-up for bullectomy. Chest Surg Clin N Am 1995; 5: 717-34.  Back to cited text no. 18    
19.Horsley WS, Gal AA, Mansour KA. Unilateral giant bullous emphysema with placental transmogrification of the lung. Ann Thorac Surg 1997; 64:226-8.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]


    Figures

  [Figure 1], [Figure 2]


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