What is Primary Spontaneous Pneumothorax?

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Primary Spontaneous Pneumothorax is a condition that predominantly affects young, otherwise healthy, people with no underlying lung disease. It is much more common in men than women and seems to be associated with tall, slim body types. Factors that can increase risk are smoking, smoking cannabis, scuba diving, flying (especially in an unpressurised air craft) and a family history of pneumothorax.

The only one of these risk factors that applies to me, aside from being slim (though not tall!) is a family history. My father had a pneumothorax in 1964 when he was eighteen. Although his was not as severe as mine and resolved itself with bed rest. He did not have a repeat episode.

Once a person suffers one pneumothorax their chances of experiencing another dramatically increase. Infact the reoccurrence rate after a first episode is as high as 50% in the same lung and 10% in the other lung. After a second episode reoccurrence rates reach an incredible 80%.

Primary Spontaneous Pneumothorax is poorly understood but it is believed to be caused by the formation of tiny blebs and bullae (little air blisters – a ‘bleb’ under 1cm in size, a ‘bulla’ over 1cm) at the apex of the lung which subsequently rupture leaking air into the plural space and cause the lung to collapse.

Why these blebs and bullae form is not certain and neither is what causes them to suddenly rupture although it is believed to be related to pressure in the chest space relative to external air pressure hence its association with scuba diving and flying.

It’s also possible to live your whole life with multiple blebs and bullae on your lungs and never have a pneumothorax. The presence of these little air pockets alone does not necessarily indicate a future collapsed lung although it does certainly increase your risk.

Once a bleb or bulla has ruptured the pneumothorax can be partial, meaning only some of the lung has collapsed or complete, meaning the whole lung is down.

Small pneumothoraces can be treated conservatively with observation and bed rest but larger and complete ones usually require some kind of medical intervention. This can be in the form of aspiration, using a large syringe to puncture the chest and suck the air out, or with the insertion of an Intercostal Chest Drain.

In most cases the lung will repair the tiny hole and reinflate without surgical intervention, even in cases of large pneumothorax.

In cases where the lung fails to reinflate (as was the case for me) or when a person has a repeat pneumothorax, surgery becomes the best option.

Nowadays this is usually done using the minimally invasive VATS technique (Video Assisted Thorasic Surgery) where two or three small incisions are made in the side and back of the patient. The blebs and bullae are then stapled to prevent further leakage and some form of ‘pleurodesis’ is performed on the lung to artificially stick it to the chest wall in the hope of preventing it from collapsing again in the future.

In my case my pneumothorax was complete so I required an Intercostal Chest Drain which was in place for fourteen days. During that time unfortunately my lung failed to reinflate so I had the VATS procedure performed at St Barts in London, through two incisions on my left side (my surgeon very cleverly used my existing armpit wound from the chest drain as one of the surgical incisions). They found only one bulla – this was what was causing the leak so it was stapled and pleurodesis was performed. I am still recovering from this painful procedure, but as far as external wounds go they are very tiny (one in my armpit just less than an inch long and one slightly further down measuring around a centimetre).

It really is incredible what modern surgery can do. If I had this same surgery performed in the 1970s I would now have a wound about six to nine inches long…!

Although rarely life threatening, except in cases of Tension Pneumothorax (where the pressure of the air builds up in the chest cavity so much it causes cardiac arrest) or in Bilateral Pneumothorax (where both lungs go down at the same time), Primary Spontaneous Pneumothorax is a debilitating and painful condition which can require a lengthy hospital stay (as was the case for me) and a lot of psychological trauma.

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