Clinical Image
 
A case of vanishing lung syndrome
Andra Malina Farcas1, Priti Dangayach2, Dharani Kumari Narendra3
1MD, Resident, Department of Emergency Medicine, Northwestern University Feinberg School of Medicine, Chicago, USA
2MD, Assistant Professor, Internal Medicine, Baylor College of Medicine, Houston, USA
3MBBS, Assistant Professor, Medicine, Pulmonary Critical Care and Sleep, Baylor College of Medicine, Houston, USA

Article ID: 100044Z09AF2017
doi: 10.5348/Z09-2017-44-CR-16

Corresponding Author:
Andra Malina Farcas
211 E. Ontario Street
Suite 200, Chicago
IL 60611, USA

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How to cite this article
Farcas AM, Dangayach P, Narendra DK. A case of vanishing lung syndrome. J Case Rep Images Med 2017;3:58–60.


case Report

A 37-year-old African-American male with past medical history of tobacco and marijuana abuse presented with sudden onset shortness of breath on exertion and chest pain. The chest pain was severe, right-sided, intermittent, and pleuritic. He reported diaphoresis and a 50-pound weight loss over the last year. He also noted a rapid decline in effort tolerance over the last year and being symptomatic on activities of daily living. He denied trauma, hemoptysis, and chronic lung diseases. The patient was a chronic cigarette smoker, averaging seven cigarettes per day for 22 years, and had a distant history of chronic marijuana smoking, averaging 1 ounce per day. On examination, the patient was in respiratory distress, tachypneic, tachycardic, with tenderness to palpation of the right chest. Chest X-Ray (Figure 1) showed right apicolateral pneumothorax with underlying severe lung parenchymal bullous disease. Computed tomography scan of chest with contrast (Figure 2) and (Figure 3) confirmed moderate-sized right pneumothorax with bilateral giant emphysematous bulla, left greater than right, with rightward shift of mediastinum. While in the emergency room, the patient suddenly developed acute respiratory distress with distended neck veins, which was concerning for tension pneumothorax. Chest tube was inserted into the right lateral chest, and there was return of air. Chest X-ray showed improvement in the right pneumothorax. A V/Q scan (Figure 4) showed 91% perfusion occurring in the right lung, 9% in the left lung, and no lung ventilation on the left. Alpha-1-antitrypsin testing was negative. The chest tube was removed after >96 hours without air leak, and the patient did not have recurrence of the pneumothorax. Cardiothoracic surgery was consulted and felt that the patient lacked lung reserve to successfully undergo bullectomy and recommended lung transplantation. The patient declined transplantation and was lost to follow-up.



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Figure 1: Chest X-Ray showing right pneumothorax and large bulla on the left pushing the mediastinum to the right.



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Figure 2: Axial chest computed tomography scan with intravenous contrast revealing multiple giant bullae and right pneumothorax.



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Figure 3: Axial chest computed tomography scan with intravenous contrast revealing multiple giant bullae and right pneumothorax.



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Figure 4: Ventilation perfusion scan showing major ventilation on right compared to left lung



Discussion

Idiopathic giant bullous emphysema, also known as vanishing lung syndrome (VLS), is a rare condition. It is often asymptomatic but may present with progressive dyspnea and hypoxia. This condition usually occurs in young, thin, male smokers [1]. Risk factors include smoking, marijuana abuse, and alpha-1-antitrypsin deficiency [2][3][4]. Marijuana smokers have asymmetrical bullous disease with pathological changes happening approximately 20 years earlier than in tobacco smokers [2][3]. The radiographic criteria proposed in 1987 [5] include giant bullae in one or both upper lobes occupying at least one third of the hemithorax and compressing surrounding normal parenchyma. One of the major complications of VLS is spontaneous pneumothorax, which presents as chest pain with acute deterioration in respiratory function [6]. Vanishing lung syndrome bullae can also mimic pneumothorax, and it is difficult to distinguish between the two clinically and with chest radiography. The distinction can usually be made on CT scan of chest [7]. Lung-volume-reduction surgery (or bullectomy) is considered for selected patients with VLS after assessment of exercise capacity, pulmonary function testing, and smoking cessation [1]. Bullectomy leads to improvements in pulmonary function for up to five years [8].


Conclusion

Vanishing lung syndrome (or idiopathic giant bullous emphysema) is a rare condition that may present with progressive dyspnea and hypoxia in young smokers. Imaging reveals giant bullae compressing lung parenchyma. These bullae can mimic pneumothorax, and it can be difficult to make the distinction on chest radiography.

Keywords: Idiopathic giant bullous emphysema, Large bulla, Lung


References
  1. Ladizinski B, Sankey C. Vanishing lung syndrome. N Engl J Med 2014 Feb 27;370(9):e14.   [CrossRef]   [Pubmed]    Back to citation no. 1
  2. Hii SW, Tam JD, Thompson BR, Naughton MT. Bullous lung disease due to marijuana. Respirology 2008 Jan;13(1):122–7.   [CrossRef]   [Pubmed]    Back to citation no. 2
  3. Beshay M, Kaiser H, Niedhart D, Reymond MA, Schmid RA. Emphysema and secondary pneumothorax in young adults smoking cannabis. Eur J Cardiothorac Surg 2007 Dec;32(6):834–8.   [CrossRef]   [Pubmed]    Back to citation no. 3
  4. Hutchison DC, Cooper D; British thoracic society. Alpha-1-antitrypsin deficiency: Smoking, decline in lung function and implications for therapeutic trials. Respir Med 2002 Nov;96(11):872–80.   [CrossRef]   [Pubmed]    Back to citation no. 4
  5. Roberts L, Putman CE, Chen JTT. Vanishing lung syndrome: Upper lobe bullous pneumopathy. Revista Interamericana de Radiologia 1987;12:249–55.    Back to citation no. 5
  6. Sood N, Sood N. A rare case of vanishing lung syndrome. Case Reports in Pulmonology 2011;2011:2.   [CrossRef]    Back to citation no. 6
  7. Lai CC, Huang SH, Wu TT, Lin SH. Vanishing lung syndrome mimicking pneumothorax. Postgrad Med J 2013 Jul;89(1053):427–8.   [CrossRef]   [Pubmed]    Back to citation no. 7
  8. Palla A, Desideri M, Rossi G, et al. Elective surgery for giant bullous emphysema: A 5-year clinical and functional follow-up. Chest 2005 Oct;128(4):2043–50.   [CrossRef]   [Pubmed]    Back to citation no. 8

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Author Contributions
Andra Malina Farcas – Conception and design, Acquisition of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Priti Dangayach – Conception and design, Acquisition of data, Critical revision of the article, Final approval of the version to be published
Dharani Kumari Narendra – Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Guarantor of Submission
The corresponding author is the guarantor of submission.
Source of Support
None
Conflict of Interest
Author declare no conflict of interest.
Copyright
© 2017 Andra Malina Farcas et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.