Fibrothorax is a medical condition characterised by severe scarring (fibrosis) and fusion of the layers of the pleural space surrounding the lungs resulting in decreased movement of the lung and ribcage. The main symptom of fibrothorax is shortness of breath. There also may be recurrent fluid collections surrounding the lungs. Fibrothorax may occur as a complication of many diseases, including infection of the pleural space known as an empyema or bleeding into the pleural space known as a haemothorax.Fibrosis in the pleura may be produced intentionally using a technique called pleurodesis to prevent recurrent punctured lung (pneumothorax), and the usually limited fibrosis that this produces can rarely be extensive enough to lead to fibrothorax. The condition is most often diagnosed using an X-ray or CT scan, the latter more readily detecting mild cases. Fibrothorax is often treated conservatively with watchful waiting but may require surgery. The outlook is usually good as long as there is no underlying pulmonary fibrosis or complications following surgery. The disease is highly uncommon. Signs and symptoms
Signs
Reduced movement of the ribcage during breathing, reduced breath sounds on the affected side(s), and a dull feeling when the chest is pressed are common signs observed during examination for fibrothorax. Sharp chest pain with deep breaths or coughing may be seen in some cases. Severe cases of fibrothorax can lead to respiratory failure due to inadequate ventilation and cause abnormally high levels of carbon dioxide in the bloodstream. Symptoms
The condition only causes symptoms if the visceral pleura is affected.
Tobacco smoking cessation is strongly recommended since tobacco smoke exposure can worsen fibrosis. Severe cases of fibrothorax may require supportive mechanical ventilation if the affected person is unable to breathe adequately on their own.In cases of fibrothorax caused by medication, it is recommended that the offending medications be stopped. Ergot alkaloid medications, which can worsen pleural fibrosis, are typically avoided. Cases of fibrothorax attributable to medication typically stop worsening if the provoking medication is stopped. In some situations, medication-induced fibrothorax improves after stopping the causative medication but fibrothorax usually does not completely resolve.Watchful waiting is appropriate for milder cases of fibrothorax in certain situations. Fibrothorax caused by tuberculosis, empyema, or haemothorax often improves spontaneously 3–6 months after the precipitating illness. Corticosteroids are commonly used to treat fibrothorax but are not well-supported by available evidence. Surgical
In severe cases of fibrothorax that are compromising a persons ability to breathe, the scar tissue (fibrous peel) causing fibrothorax can be surgically removed using a technique called decortication. However, surgical decortication is an invasive procedure which carries the risk of complications including a small risk of death, and is therefore generally only considered if severe symptoms are present and have been for many months. Surgical decortication is generally considered for people with fibrothoraces that are severe, causing significant shortness of breath, and have otherwise relatively healthy lungs since this enhances the likelihood of a better outcome. Surgical removal of the pleura (pleurectomy) may be performed in refractory cases, as often happens when asbestosis is the cause.