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Journal of the association of physicians of india • vol 62 • december, 2014

51

Case Reports

A Rare Case of Vanishing Lung Syndrome


Amarjit Singh Vij*, Robert James**, Akashdeep Singh***, Amrinder Singh Dhaliwal****,
Ajay Chhabra †, Kamaljeet Kaur Vij‡

Abstract
Vanishing lung syndrome (VLS) is a rare radiological syndrome in which the lungs appear to be disappearing
on X-ray. It is a chronic, progressive condition usually affecting young male smokers and is characterised
by giant emphysematous bullae, which commonly develop in the upper lobes. We describe here a rare
case of 60-year-old male patient who had a history of chronic smoking for 30 years. He had been admitted
in the hospital multiple times due to spontaneous pneumothorax, type 2 respiratory failure and infective
exacerbations. He was earlier diagnosed having chronic obstructive pulmonary disease (COPD) with
predominant emphysema on the basis of his history and chest X-ray findings. Eventually, his CT chest
revealed the diagnosis of giant bullous disease/vanishing lung syndrome. He had been surviving with
his little lung tissue for about 10 years. No such case has been reported in the literature so far. He was
attended last on 12th October, 2009 in medical outdoor of Christian Medical College and Hospital, Ludhiana
by the first three authors. Thereafter, the patient was not traceable.

Introduction
In 1937, Burke described a case of “vanishing lungs” in a 35 year old
man who had progressive dyspnoea, respiratory failure, radiographic
and pathologic findings of giant bullae that occupied two thirds of both
hemithoraces. 1 The radiographic criteria for this syndrome defined by
Roberts et al 2 include the presence of giant bullae in one or both upper
lobes, occupying at least one third of the hemithorax and compressing
surrounding normal parenchyma. Vanishing lung syndrome (VLS) is
also known as type I bullous disease or primary bullous disease of the
lung in which the lungs appear to be disappearing on X-ray. 3
Most patients are young male, 4,5 the risk factors are smoking, alpha-
1-antitrypsin deficiency, and marijuana abuse. 6-8 Extensive paraseptal
emphysema coalesce to form giant bullae, compressing the normal
lung parenchyma and often displacing it centrally. The bullae range in
size from a few centimetres to giant bullae nearly filling hemithorax,
mimicking a pneumothorax. The giant bullae may remain asymptomatic
for a long time, their progression may cause worsening dyspnoea. A
major complication of VLS is pneumothorax. Infection of the bulla is
also common. Computed tomography (CT) is an important tool for the
*
Professor, Department of Medicine, Punjab Institute
of Medical Sciences, Jalandhar;**Associate Professor,
diagnosis of this bullous disease. 9,10
Department of Medicine, Maharishi Markandeshwar Surgical resection of giant bullae is the treatment of choice, the
Medical College and Hospital, Kumarhatti, Solan; indications include (1) spontaneous pneumothorax, 11 (2) infection, 12 or
***
Associate Professor, Department of Medicine, Dayanand
(3) dyspnoea,13 the best results are seen following limited bullectomy.14,15
Medical College and Hospital, Ludhiana; ****Associate
Professor, Department of Radiology, †Assistant Professor, Thoracoscopic treatment of giant bullae is an effective alternative to
Department of Medicine, Govt. Medical College, Amritsar; conventional thoracotomy with minimal morbidity. 16

Assistant Professor, Department of Anatomy, Punjab
Institute of Medical Sciences, Jalandhar
Received: 18.07.2013; Revised; 10.09.2013;
Accepted: 15.10.2013
52 Journal of the association of physicians of india • vol 62 • december, 2014

(1) (2) (3)

(4) (5) (6)


Figs. 1 to 6 : Extensive bilateral multiple thin walled emphysematous bullae, mostly subpleural, 1 cm to 14 cm in size. They
involve predominantly upper lobes and lying asymmetrically, more so on left side and severely compressing
the lung parenchyma. Rest of the lung fields show geographic areas of decreased attenuation and paucity of
pulmonary vessels suggestive of emphysema. Trachea is central. Mediastinal structures are normal except
calcification in the aortic arch. No intrabronchial pathology seen.

Case Report type II respiratory failure and put on ventilator but


unfortunately, he developed left sided pneumothorax.
Mr. X, 50 years old, chronic smoker for 30 years He recovered with thoracotomy. Then, he had acute
and known case of chronic obstructive pulmonary infective exacerbation in December, 2007 and managed
disease was admitted with right sided pneumothorax with I/V antibiotics. Contrast enhaced CT chest was
and type 2 respiratory failure, first time in March, done which revealed findings of vanishing lung
2000 in CMCH, Ludhiana. He was put on ventilator syndrome (Figures 1, 2, 3, 4, 5, 6). Echocardiography
and thoracotomy done. He recovered after one week. revealed moderate tricuspid regurgitation (TR) with
He was discharged on inhaled glucocorticoid and right ventricular systolic pressure (RVSP) of 45 mmHg.
bronchodilator combination and empirical anti- He was discharged after recovery. Then, he got
tubercular treatment for one year. He was counselled admitted in March, 2009 with left sided pneumothorax
to stop smoking. In December, 2001, he developed and recovered with thoracotomy. He developed
right sided lower lobe pneumonia which resolved infective exacerbation again in October, 2009 and got
with intravenous (I/V) antibiotics. In January, 2002, treated. He was doing well when last seen in medical
he developed diabetes mellitus type-2. In July, 2002, outpatient department on 12 th October, 2009.
he had left sided pneumothorax and recovered with
conservative management. He did well for about Discussion
three years except occasional episodes of mild to
This patient got admitted first time with spontaneous
moderate airflow obstruction. In December, 2005,
pneumothorax which is a common complication
he developed systemic hypertension. He developed
o f C O P D . T h e c h e s t X - r a y s h o we d r i g h t s i d e d
left sided pneumothorax and required thoracotomy
pneumothorax and emphysematous lung on left side,
again in May, 2007. In November, 2007, he came with
Journal of the association of physicians of india • vol 62 • december, 2014 53

VLS was not suspected that time. In his subsequent VLS, the patient can be referred to a pulmonologist/
admissions also, the diagnosis of VLS was not made cardiothoracic surgeon for appropriate preventive/
till his CT chest done in December, 2007 made it clear. curative bullectomy which may help the patient to
CT chest revealed multiple emphysematous bullae lead a better life with less morbidity.
bilaterally ranging in size from 1 cm to as large as
14 cm. This is in close agreement with the findings References
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