Vanishing Lung Syndrome

What Is Vanishing Lung Syndrome?
Vanishing Lung Syndrome is a rare disorder used to describe lungs that seem to disappear on x-rays. It is commonly called Bullous Emphysema or Severe Bullous Disease.

What Causes Vanishing Lung Syndrome?
Giant bullae are responsible for Vanishing Lung Syndrome and are simply pockets of air that are taking up valuable space in the lungs. The lungs do not actually disappear, but when emphysematous bullae cause the lung to enlarge, which causes the healthy lung to shrink. The healthy lung shrinks to such a degree that it appears that the healthy lung is disappearing on an x-ray.

They typically take up space in the upper part of the lung and the spaces between the bullae are less than 1 millimeter apart. On a high resolution CT scan, the bullae can be as large as 20 centimeters in diameter. The alveolar tissue is quickly destroyed. When an individual has Vanishing Lung Syndrome, the bullae take up more than one-third of the lung, depriving the infected of necessary oxygen.

Causes of Vanishing Lung Syndrome include smoking and emphysema. Paraseptal and Centrilobular Emphysema are the two most common causes of Vanishing Lung Syndrome. The disorder is usually seen in older smokers with the male population being affected the most, although there has been an increase in young men being diagnosed with Vanishing Lung Syndrome.

What Are The Symptoms of Vanishing Lung Syndrome?
The symptoms of Vanishing Lung Syndrome are chest pressure, difficulty inhaling, feeling sore in the chest area, feeling bloated, and fatigue due to not receiving enough oxygen.

How Is Vanishing Lung Syndrome Diagnosed?
Chest x-rays and CT scans are the main ways to diagnose Vanishing Lung Syndrome. After initial diagnosis, a doctor may obtain a sample of the bulla or the fluid within the bulla. A doctor will retrieve a sample by inserting a long needle in the chest or by doing a surgical biopsy.

What Is The Treatment For Vanishing Lung Syndrome?
A bullectomy is the only known treatment for Vanishing Lung Syndrome. Success of the treatment depends on what class of the disease a patient is in. Stages of the disease are Group I (single bulla with underlying normal lung), Group II (multiple bullae with underlying normal lung), Group III (multiple bullae with underlying broad emphysema), and Group IV (multiple bullae with underlying respiratory diseases). Group I and Group II have the best chance of survival. Those in Group IV usually are recommended to undergo a lung transplant.

Complications of the treatment for Vanishing Lung Syndrome include air leaks and infection in the area of the incision.

How Can I Prevent Vanishing Lung Syndrome?
Only smokers and those with Paraseptal and Centrilobular Emphysema get Vanishing Lung Syndrome. Most people who get Emphysema are current or former smokers, so the best way to prevent this disease is to stop smoking or to never start.

Sources:

Learning Radiology: Vanishing Lung Syndrome, http://www.learningradiology.com/archives06/COW%20222-Vanishing%20lung/vanishinglungccorrect.html

University of Rochester Medical Center: Giant Bullae, http://www.urmc.rochester.edu/encyclopedia/content.cfm?pageid=P93019