Vanishing Lung Syndrome in a Patient with HIV Infection
and Heavy Marijuana Use
Basheer Tashtoush,1Fernando Gonzalez-Ibarra,2Roya Memarpour,1
Anas Hadeh,1and Laurence Smolley1
1Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard,
Weston, FL 33331, USA
2Department of Internal Medicine, Jersey City Medical Center, Mount Sinai School of Medicine, 355 Grand Street, Jersy City,
NJ 07302, USA
Correspondence should be addressed to Basheer Tashtoush; firstname.lastname@example.org
Received 11 November 2013; Accepted 9 December 2013; Published 8 January 2014
Academic Editors: J. Murchison and H. Wilkens
Copyright © 2014 Basheer Tashtoush et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
Vanishing lung syndrome (VLS) is a rare and distinct clinical syndrome that usually affects young men. VLS leads to severe
progressive dyspnea and is characterized by extensive, asymmetric, peripheral, and predominantly upper lobe giant lung bullae.
Case reports have suggested an additive role of marijuana use in the development of this disease in young male tobacco smokers.
We herein report a case of a 65-year-old Hispanic male previously diagnosed with severe emphysema and acquired immune
department with severe progressive shortnessofbreath he was found tohave multiple large subpleural bullae occupying more than
one-third of the hemithorax on chest computerized tomography (CT), characteristic of vanishing lung syndrome. The patient was
mechanically ventilated and later developed a pneumothorax requiring chest tube placement and referral for surgical bullectomy.
Surgical bullectomy has shown high success rates in alleviating the debilitating symptoms and preventing the life threatening
complications of this rare syndrome. This case further emphasizes the importance of recognizing VLS in patients with severe
emphysema and heavy marijuana smoking.
Vanishing lung syndrome, also termed giant bullous emphy-
sema (GBE), is a rare syndrome first described by Burke
in 1937 . It is an idiopathic and distinct clinical syn-
drome that affects young men, usually smokers. It causes
severe progressive dyspnea and is characterized by extensive,
predominantly asymmetric upper lobe bullous emphysema,
which may eventually lead to respiratory failure . Case
in the development of this disease in young male smokers
where tobacco consumption was less than what is commonly
20 pack years) [3, 4].
We report a case of a 65-year-old male diagnosed with
end stage emphysema for 3 years, AIDS, and a history of
heavy marijuana smoking. He presents to the hospital in
acute respiratory distress with multiple large peripheral lung
bullae on chest CT consistent with vanishing lung syndrome.
2. Case Presentation
A 65-year-old Hispanic male was brought to the emergency
department due to severe shortness of breath at home. The
shortness of breath started approximately one week prior
to presentation and had progressively worsened over time.
Upon arrival to the emergency department the patient was
hypoxemic and in severe respiratory distress, with minimal
bilateral breath sounds on auscultation and an oxygen satu-
ration of 88%.
He had a past medical history of severe chronic obstruc-
tive pulmonary disease (COPD), treated with home oxygen
Hindawi Publishing Corporation
Case Reports in Pulmonology
Volume 2014, Article ID 285208, 4 pages
2Case Reports in Pulmonology
Figure 1: Anteroposterior chest X-ray following endotracheal tube
placement showing hyperinflated lungs with multiple areas of
hyperlucency on the left lung base and left apex, surrounded by a
thin linear demarcation (arrows).
than one-third of the left hemithorax.
over the last year, history of AIDS for 17 years, receiving
antiretroviral medications, (last documented HIV RNA load
of 14,070copies/mm3and a CD4count of 285/mm3). He had
active heavy marijuana smoking for the last 20 years on a
daily-basis, and no history of tobacco use.
On physical examination the patient was intubated on
mechanical ventilation, with reduced bilateral breath sounds
and minimal chest wall expansion. Arterial blood gas anal-
ysis showed respiratory acidosis with no other laboratory
abnormalities. serum antineutrophil antibodies (ANA) were
negative; serum angiotensin converting enzyme (ACE) and
alpha 1 antitrypsine were at normal levels. Chest X-ray
showed hyperinflation with increased lucency in bilateral
upper lobes (Figure 1). Chest CT revealed multiple large
subpleural bullae occupying more than one-third of the
hemithorax, characteristic of VLS (Figures 2 and 3).
ventilation, the patient developed a tension pneumothorax
secondary to ruptured bullae, requiring a chest tube place-
ment. He was later referred for surgical bullectomy.
with a wall thickness less than 1mm. Giant bullae form when
usually subpleural in distribution .
It was originally hypothesized that bullae formation
was due to a “ball-valve mechanism” in which gas entered
the lesion but could not escape. New evidence based on
the measurement of intraluminal bulla pressure, however,
pleural pressure, and is thus preferentially ventilated during
inspiration, and with the considerable loss of elastic recoil air
trapping occurs. This gradient between a bulla and the alve-
olar space provides an explanation for the radiographically
and intraoperatively observed compression of lung tissue
immediately adjacent to a bulla .
Several risk factors for the development of VLS have
been described in literature, including tobacco smoking,
marijuana smoking [3, 4, 7], alpha 1 antitrypsin deficiency
, autoimmune and connective tissue diseases such as SLE,
and sarcoidosis . In subjects who smoke marijuana, the
pathological changes of emphysema occur at a younger age
(approximately 20 years earlier) than in tobacco smokers
[3, 4]. These changes can also be distinguished from tobacco
induced emphysema by the location of the large pulmonary
bullae, found mostly in a paraseptal distribution with a
marked predisposition for the upper lobes, as compared
to the more uniformly distributed bullae of centrilobular
emphysema which are the typical changes associated with a
lifetime of tobacco smoking [7, 10, 11].
How marijuana might cause such severe bullous disease
is not clear. It has been postulated that the methods of
inhalation of marijuana smoke may cause significant baro-
and employ breath-holding techniques for up to four times
longer than cigarette smokers, with a nearly 70% increase in
in carboxyhaemoglobin per cigarette smoked [12–14].
HIV infection also confers an increased risk for emphy-
sema when compared to control subjects (15% versus 1%,
resp.), with studies showing an accelerated progression
towards emphysema in HIV-positive individuals who smoke
. Spirometry based studies revealed that HIV infection is
lung disease , which is thought to be secondary to
the decreased antioxidant defenses, especially superoxide
dismutase and glutathione in HIV-positive patients .
defined by the presence of giant bullae in one or both upper
lobes, occupying at least one-third of the hemithorax and
compressing surrounding normal lung parenchyma. Stern et
al. described the CT findings of giant bullous emphysema,
in diameter without a single dominant giant bulla .
A major complication of VLS is pneumothorax, which
classically presents as a history of acute deterioration in
respiratory function associated with chest pain. When the
diagnosis on chest radiography is uncertain, chest CT is
Case Reports in Pulmonology3
Figure 3: Chest CT axial view showing bilateral upper lobe (a) and lower lobe (b) bullae compressing surrounding relatively normal lung
Treatment of VLS is usually surgical resection of the
giant bullae, especially when patients are symptomatic or
develop a pneumothorax. Surgical options include standard
thoracotomy and video-assisted thoracoscopic surgery, with
no apparent differences in outcomes between one procedure
tion for giant bullae in selected patients reduce the dynamic
the patients lung function and exercise performance .
This case emphasizes the importance of recognizing VLS
in patients with history of heavy marijuana smoking, who
present with findings of severe and atypical emphysema on
imaging studies, as surgical bullectomy becomes a critical
therapeutic option to prevent the life threatening complica-
tions of this rare syndrome.
Conflict of Interests
The authors specified that the research was conducted in the
absence of any related conflict of interests.
 R. Burke, “Vanishing lungs: a case report of bullous emphy-
sema,” Radiology, vol. 28, no. 3, pp. 367–371, 1937.
double-wall sign in detecting pneumothorax in patients with
giant bullous emphysema,” American Journal of Roentgenology,
vol. 174, no. 6, pp. 1765–1768, 2000.
 M. K. Johnson, R. P. Smith, D. Morrison, G. Laszlo, and R. J.
White, “Large lung bullae in marijuana smokers,” Thorax, vol.
55, no. 4, pp. 340–342, 2000.
 S. W. Hii, J. D. Tam, B. R. Thompson, and M. T. Naughton,
1, pp. 122–127, 2008.
 N. Sharma, A. M. Justaniah, J. P. Kanne, J. W. Gurney, and
T.-L. Mohammed, “Vanishing lung syndrome (giant bullous
emphysema): CT findings in 7 patients and a literature review,”
Journal of Thoracic Imaging, vol. 24, no. 3, pp. 227–230, 2009.
 G. L. Snider, “Reduction pneumoplasty for giant bullous
emphysema. Implications for surgical treatment of nonbullous
emphysema,” Chest, vol. 109, no. 2, pp. 540–548, 1996.
 M. Beshay, H. Kaiser, D. Niedhart, M. A. Reymond, and R. A.
Schmid, “Emphysema and secondary pneumothorax in young
adults smoking cannabis,” European Journal of Cardio-Thoracic
Surgery, vol. 32, no. 6, pp. 834–838, 2007.
 D. C. Hutchison, D. Cooper, and British Thoracic Soci-
ety, “Alpha-1-antitrypsin deficiency: smoking, decline in lung
function and implications for therapeutic trials,” Respiratory
Medicine, vol. 96, no. 11, pp. 872–880, 2002.
 A. Miller, “The vanishing lung syndrome associated with
pulmonary sarcoidosis,” British Journal of Diseases of the Chest,
vol. 75, no. 2, pp. 209–214, 1981.
 M. L. Howden and M. T. Naughton, “Pulmonary effects of
marijuana inhalation,” Expert Review of Respiratory Medicine,
vol. 5, no. 1, pp. 87–92, 2011.
 R. G. Fraser, J. A. P. Pare, P. D. Pare, R. S. Fraser, and G. P.
Genereux, Diagnosis of Diseases of the Chest, W.B. Saunders,
Philadelphia, Pa, USA, 3rd edition, 1990.
 T.-C. Wu, D. P. Tashkin, B. Dhahed, and J. E. Rose, “Pulmonary
hazards of smoking marijuana as compared with tobacco,” The
New England Journal of Medicine, vol. 318, no. 6, pp. 347–351,
 J. L. Azorlosa, M. K. Greenwald, and M. L. Stitzer, “Marijuana
smoking: effects of varying puff volume and breathhold dura-
tion,” Journal of Pharmacology and Experimental Therapeutics,
vol. 272, no. 2, pp. 560–569, 1995.
 J. L. Azorlosa, S. J. Heishman, M. L. Stitzer, and J. M.
Mahaffey, “Marijuana smoking: effect of varying 훿 9-
pp. 114–122, 1992.
to pulmonary emphysema among HIV-seropositive smokers,”
Annals of Internal Medicine, vol. 132, no. 5, pp. 369–372, 2000.
lung disease in HIV population: a cross sectional study,”
Respiratory Medicine, vol. 105, no. 11, pp. 1655–1661, 2011.
defence in individuals infected by the human immunodefi-
ciency virus,” European Journal of Clinical Investigation, vol. 30,
no. 5, pp. 454–459, 2000.
 E. J. Stern, W. R. Webb, A. Weinacker, and N. L. Muller,
“Idiopathic giant bullous emphysema (vanishing lung syn-
drome): imaging findings in nine patients,” American Journal
of Roentgenology, vol. 162, no. 2, pp. 279–282, 1994.
 G. D. Phillips, B. Trotman-Dickenson, M. E. Hodson, and D.
M. Geddes, “Role of CT in the management of pneumothorax
tetrahydrocannabinol content and number of puffs,” Journal of
Pharmacology and Experimental Therapeutics, vol. 261, no. 1,
4 Case Reports in Pulmonology Download full-text
in patients with complex cystic lung disease,” Chest, vol. 112, no.
1, pp. 275–278, 1997.
 A. Palla, M. Desideri, G. Rossi et al., “Elective surgery for giant
Chest, vol. 128, no. 4, pp. 2043–2050, 2005.
 R. Neviere, M. Catto, N. Bautin et al., “Longitudinal changes
in hyperinflation parameters and exercise capacity after giant
bullous emphysema surgery,” The Journal of Thoracic and
Cardiovascular Surgery, vol. 132, no. 5, pp. 1203–1207, 2006.