farcas am, dangayach p, narendra dk. a case of · 87 andra malina farcas, m.d. 88 group 1 -...

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Manuscript Accepted Early View Article Page 1 of 10 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. Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the Early View Article. The Early View Article is an online published version of an accepted article before publication in the final form. The proof of this manuscript will be sent to the authors for corrections after which this manuscript will undergo content check, copyediting/proofreading and content formatting to conform to journal’s requirements. Please note that during the above publication processes errors in content or presentation may be discovered which will be rectified during manuscript processing. These errors may affect the contents of this manuscript and final published version of this manuscript may be extensively different in content and layout than this Early View Article.

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Page 1: Farcas AM, Dangayach P, Narendra DK. A Case of · 87 Andra Malina Farcas, M.D. 88 Group 1 - Conception and design, Acquisition of data 89 Group 2 - Drafting the article, Critical

Manuscript Accepted Early View Article

Page 1 of 10

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.

Disclaimer: This manuscript has been accepted for publication. This is a pdf

file of the Early View Article. The Early View Article is an online published

version of an accepted article before publication in the final form. The proof of

this manuscript will be sent to the authors for corrections after which this

manuscript will undergo content check, copyediting/proofreading and content

formatting to conform to journal’s requirements. Please note that during the

above publication processes errors in content or presentation may be

discovered which will be rectified during manuscript processing. These errors

may affect the contents of this manuscript and final published version of this

manuscript may be extensively different in content and layout than this Early

View Article.

Page 2: Farcas AM, Dangayach P, Narendra DK. A Case of · 87 Andra Malina Farcas, M.D. 88 Group 1 - Conception and design, Acquisition of data 89 Group 2 - Drafting the article, Critical

Manuscript Accepted Early View Article

Page 2 of 10

TYPE OF ARTICLE: Clinical Images 1

2

TITLE: A Case of Vanishing Lung Syndrome 3

4

AUTHORS: 5

Andra Malina Farcas1, M.D, 6

Priti Dangayach2, M.D, 7

Dharani Kumari Narendra3, M.B.B.S. 8

9

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

[email protected] 14

3Assistant Professor, Medicine, Pulmonary Critical Care and Sleep, Baylor College 15

of Medicine, Houston, TX, [email protected] 16

17

CORRESPONDING AUTHOR DETAILS 18

Andra Malina Farcas 19

211 E. Ontario Street, Suite 200, Chicago, IL 60611 20

Email: [email protected] 21

22

Short Running Title: A Case of Vanishing Lung Syndrome 23

24

Guarantor of Submission : The corresponding author is the guarantor of 25

submission. 26

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Page 3: Farcas AM, Dangayach P, Narendra DK. A Case of · 87 Andra Malina Farcas, M.D. 88 Group 1 - Conception and design, Acquisition of data 89 Group 2 - Drafting the article, Critical

Manuscript Accepted Early View Article

Page 3 of 10

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

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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

76

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

82

CONFLICT OF INTEREST 83

None 84

85

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

96

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Manuscript Accepted Early View Article

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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

101

ACKNOWLEDGEMENTS 102

Jason Pelton, M.D 103

104

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

121

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

right pneumothorax. 195

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Figure 4: Ventilation perfusion scan showing major ventilation on right compared to 211

left lung. 212