Manuscript Accepted Early View Article
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Early View Article: Online published version of an accepted article before
publication in the final form.
Journal Name: Journal of Case Reports and Images in Medicine
doi: To be assigned
Early view version published: November 29, 2017
How to cite the article: Farcas AM, Dangayach P, Narendra DK. A Case of
Vanishing Lung Syndrome. Journal of Case Reports and Images in Medicine.
Forthcoming 2017.
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Manuscript Accepted Early View Article
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TYPE OF ARTICLE: Clinical Images 1
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TITLE: A Case of Vanishing Lung Syndrome 3
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AUTHORS: 5
Andra Malina Farcas1, M.D, 6
Priti Dangayach2, M.D, 7
Dharani Kumari Narendra3, M.B.B.S. 8
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AFFILIATIONS: 10
1Resident, Department of Emergency Medicine, Northwestern University Feinberg 11
School of Medicine, Chicago, IL, [email protected] 12
2Assistant Professor, Internal Medicine, Baylor College of Medicine, Houston, TX, 13
3Assistant Professor, Medicine, Pulmonary Critical Care and Sleep, Baylor College 15
of Medicine, Houston, TX, [email protected] 16
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CORRESPONDING AUTHOR DETAILS 18
Andra Malina Farcas 19
211 E. Ontario Street, Suite 200, Chicago, IL 60611 20
Email: [email protected] 21
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Short Running Title: A Case of Vanishing Lung Syndrome 23
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Guarantor of Submission : The corresponding author is the guarantor of 25
submission. 26
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CASE REPORT 33
A 37-year-old African American man with past medical history of tobacco and 34
marijuana abuse presented with sudden onset shortness of breath on exertion and 35
chest pain. The chest pain was severe, right-sided, intermittent, and pleuritic. He 36
reported diaphoresis and a 50-pound weight loss over the past year. He also noted a 37
rapid decline in effort tolerance over the last year and being symptomatic on 38
activities of daily living. He denied trauma, hemoptysis, and chronic lung diseases. 39
He was a chronic cigarette smoker, averaging 7 cigarettes per day for 22 years, and 40
had a distant history of chronic marijuana smoking, averaging 1 ounce per day. On 41
exam, the patient was in respiratory distress, tachypneic, tachycardic, with 42
tenderness to palpation of the right chest. Chest X-Ray (Figure 1) showed right 43
apicolateral pneumothorax with underlying severe lung parenchymal bullous 44
disease. Computed tomography (CT) Chest scan with contrast (Figures 2 and 3) 45
confirmed moderate-sized right pneumothorax with bilateral giant emphysematous 46
bulla, left greater than right, with rightward shift of mediastinum. While in the 47
emergency room, the patient suddenly developed acute respiratory distress with 48
distended neck veins, which was concerning for tension pneumothorax. Chest tube 49
was inserted into the right lateral chest, and there was return of air. Chest X-ray 50
showed improvement in the right pneumothorax. A V/Q scan (Figure 4) showed 91% 51
perfusion occurring in the right lung, 9% in the left lung, and no lung ventilation on 52
the left. Alpha-1-antitrypsin testing was negative. The chest tube was removed after 53
>96 hours without air leak, and the patient did not have recurrence of the 54
pneumothorax. Cardiothoracic surgery was consulted and felt that the patient lacked 55
lung reserve to successfully undergo bullectomy and recommended lung 56
transplantation. The patient declined transplantation and was lost to follow-up. 57
58
DISCUSSION 59
Idiopathic giant bullous emphysema, also known as Vanishing lung syndrome (VLS), 60
is a rare condition. It is often asymptomatic but may present with progressive 61
dyspnea and hypoxia. This condition usually occurs in young, thin, male smokers [1]. 62
Risk factors include smoking, marijuana abuse, and alpha-1-antitrypsin deficiency 63
[2,3,4]. Marijuana smokers have asymmetrical bullous disease with pathological 64
Manuscript Accepted Early View Article
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changes happening approximately 20 years earlier than in tobacco smokers [2,3]. 65
The radiographic criteria proposed in 1987 [5] include giant bullae in one or both 66
upper lobes occupying at least one third of the hemithorax and compressing 67
surrounding normal parenchyma. One of the major complications of VLS is 68
spontaneous pneumothorax, which presents as chest pain with acute deterioration in 69
respiratory function [6]. VLS bullae can also mimic pneumothorax, and it is difficult to 70
distinguish between the two clinically and with chest radiography. The distinction can 71
usually be made on CT chest [7]. Lung-volume-reduction surgery (or bullectomy) is 72
considered for selected patients with VLS after assessment of exercise capacity, 73
pulmonary function testing, and smoking cessation [1]. Bullectomy leads to 74
improvements in pulmonary function for up to 5 years [8]. 75
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CONCLUSION 77
Vanishing Lung Syndrome (or idiopathic giant bullous emphysema) is a rare 78
condition that may present with progressive dyspnea and hypoxia in young smokers. 79
Imaging reveals giant bullae compressing lung parenchyma. These bullae can mimic 80
pneumothorax, and it can be difficult to make the distinction on chest radiography. 81
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CONFLICT OF INTEREST 83
None 84
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AUTHOR’S CONTRIBUTIONS 86
Andra Malina Farcas, M.D. 87
Group 1 - Conception and design, Acquisition of data 88
Group 2 - Drafting the article, Critical revision of the article 89
Group 3 - Final approval of the version to be published 90
91
Priti Dangayach, M.D. 92
Group 1 - Conception and design, Acquisition of data 93
Group 2 - Critical revision of the article 94
Group 3 - Final approval of the version to be published 95
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Dharani Kumari Narendra, M.B.B.S. 97
Group 1 - Analysis and interpretation of data 98
Group 2 - Critical revision of the article 99
Group 3 - Final approval of the version to be published 100
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ACKNOWLEDGEMENTS 102
Jason Pelton, M.D 103
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REFERENCES 105
1. Ladizinski, B., Sankey, C. Vanishing Lung Syndrome. New England Journal 106
of Medicine 2014;370(9):e14 107
2. Hii, S.W., Tam, J.D.C., Thompson, B.R., Naughton, M.T. Bullous lung disease 108
due to marijuana. Journal Compilation Asian Pacific Society of Respirology 109
2008;13:122-127. 110
3. Beshay, M., Kaiser, H., Niedhart, D., Reymond, M.A., Schmid, R.A. 111
Emphysema and secondary pneumothorax in young adults smoking 112
cannabis. European Journal of Cardi-thoracic Surgery 2007;834-838. 113
4. Hutchinson, D.C.S., Cooper, D. Alpha-1-antitrypsinn deficiency: smoking, 114
decline in lung function and implication for therapeutic trials. Respiratory 115
Medicine 2002;96:872-880. 116
5. Roberts, L., Putman, C.E. Vanishing lung syndrome: upper lobe bullous 117
pneumopathy. Revista Interamericana de Radiologia 1987;12:249-255. 118
6. Sood, N., Sood, N. A Rare Case of Vanishing Lung Syndrome. Case Reports 119
in Pulmonology 2011;2011, 2pag. 120
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7. Lai, C.C., Huang, S.H., Wu, T.T., Lin, S.H. Vanishing lung syndrome 122
mimicking pneumothorax. Postgrad Medicinal Journal 2013;89(1053):427-123
428. 124
8. Palla, A., et. al. Elective Surgery for Giant Bullous Emphysema: A 5-Year 125
Clinical and Functional Follow-up. Chest Journal 2005;128(4):2043-2050. 126
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FIGURE LEGENDS 129
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Figure 1: Chest X-Ray showing right pneumothorax and large bulla on the left 131
pushing the mediastinum to the right. 132
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Figure 2: Axial chest CT scan with IV contrast revealing multiple giant bullae and 134
right pneumothorax. 135
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Figure 3: Coronal chest CT scan with IV contrast revealing multiple giant bullae and 137
right pneumothorax. 138
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Figure 4: Ventilation perfusion scan showing major ventilation on right compared to 140
left lung. 141
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FIGURES 161
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Figure 1: Chest X-Ray showing right pneumothorax and large bulla on the left 165
pushing the mediastinum to the right. 166
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Figure 2: Axial chest CT scan with IV contrast revealing multiple giant bullae and 179
right pneumothorax. 180
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Figure 3: Coronal chest CT scan with IV contrast revealing multiple giant bullae and 194
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Figure 4: Ventilation perfusion scan showing major ventilation on right compared to 211
left lung. 212