Pneumothorax (Lung Collapse)

A pneumothorax, or lung collapse, is a leakage of air (pneumo) from a lung into the chest cavity (thorax).  This air outside the lung abruptly diminishes the negative pressure (vacuum) between the chest wall and the lung, causing the lung to deflate or collapse.

The cause for pneumothorax in LAM is thought to be the rupture of a bleb or cyst just below the surface of the lung.  Often the collapse is only partial, but you still might experience some pain and shortness of breath.  If a large pneumothorax occurs and collapses a sizable portion, or all, of your lung, you’ll likely have chest, abdominal, or shoulder pain and significant difficulty breathing.

There are two types of spontaneous pneumothorax: primary and secondary.  A primary spontaneous pneumothorax occurs for no apparent reason.

A secondary spontaneous pneumothorax is usually associated with an underlying lung disease.  Diseases affecting the lungs (like LAM and TSC, and other problems such as emphysema and cystic fibrosis) can weaken the structure of the lungs and airways, permitting spontaneous pneumothoraces to occur.  An unexplained pneumothorax—or a series of them—often triggers the search for an underlying lung disease.

There are two goals when treating a pneumothorax.  The first is the immediate removal of air from the space between the lung and the chest wall (the pleural space) so that the lung can re-expand.  The second, and equally as important, objective is the prevention of recurrent pneumothoraces. There is a procedure called pleurodesis that adheres the outside of the lung to the inside of the chest cavity to prevent the lung from collapsing again.  If you have a persistent leak and/or have had a pneumothorax previously, your doctor may ask you to consider pleurodesis as a preventative measure.  Although pleurodesis isn't a foolproof remedy against future pneumothoraces, it generally diminishes their likelihood.  Also, if you do have a pneumothorax after you’ve had pleurodesis, the collapse is more likely to be partial, and intervention may not be required.