Friday Factoid: Pneumothorax
Sometimes it's the first clue ...
Fuelling our body with life-sustaining oxygen, our lungs are our lifeline but can be compromised in Marfan syndrome by a pneumothorax.
Put simply, pneumothorax means air in the cavity between the chest wall and the lungs, sometimes referred to as a ‘collapsed lung’. This is sometimes the first clue that prompts doctors to consider a diagnosis of Marfan syndrome.
Pneumothorax is part of the Systemic Scoring system of the Ghent Nosology (2010).
- Approximately 15% of people with spontaneous pneumothorax have a genetic cause and this could be a connective tissue disorder like Marfan syndrome (MFS) or Loeys Dietz
- 4-14% of people with MFS will suffer from spontaneous pneumothorax and it’s more common in young men
Symptoms
- Chest pain
- Pain that is worse when breathing in
- Cough
- Increased shortness of breath
Diagnosis is made with a Chest X-ray and treatment will be planned depending on the severity of the condition but could include oxygen, pain relief, chest drain.
CXR with blue arrows showing the area of collapsed lung.
