What is the recommended treatment for fibrothorax in an otherwise healthy adult presenting with dyspnea, reduced chest expansion, and a restrictive pattern on pulmonary function testing?

Fibrothorax Treatment

For an otherwise healthy adult with fibrothorax causing dyspnea and restrictive lung disease, surgical decortication is the definitive treatment to restore lung function and improve quality of life. 1

Understanding Fibrothorax

Fibrothorax represents dense pleural fibrosis that entraps the lung, preventing normal expansion and causing restrictive physiology. The condition can result from:

• Chronic pleural inflammation (empyema, hemothorax, complicated parapneumonic effusions) 2
• Asbestos exposure with diffuse pleural thickening 3
• Autoimmune pleuritis (rheumatoid arthritis, systemic lupus erythematosus) 1, 4
• Post-traumatic complications when inadequately drained 5

The clinical presentation typically includes progressive dyspnea on exertion, reduced chest expansion, and restrictive defects on pulmonary function testing with reduced FVC and total lung capacity 3, 1.

Treatment Algorithm

Initial Assessment and Medical Management

• Identify and treat underlying cause: Address any active infection, autoimmune disease, or ongoing inflammatory process before considering surgery 4
• Optimize medical therapy first: For autoimmune causes (SLE, rheumatoid arthritis), maximize immunosuppressive therapy with corticosteroids and agents like cyclophosphamide 1, 4
• Trial of conservative management: In early or mild cases, medical therapy alone may suffice, particularly for autoimmune etiologies 4

However, medical therapy alone is insufficient when fibrothorax causes significant functional impairment. 1

Surgical Intervention: The Definitive Treatment

Surgical decortication (pleurectomy) should be performed when:

• Severe restrictive defect persists despite maximal medical therapy (FVC <50% predicted, TLC <50% predicted) 1
• Disabling dyspnea significantly impairs quality of life 1
• Dense visceral pleural thickening entraps the lung on CT imaging 1

Surgical Approach

Video-assisted thoracoscopic surgery (VATS) or open thoracotomy with decortication removes the thickened visceral pleura, allowing lung re-expansion 1, 5.

The procedure involves:

• Complete removal of the fibrous peel from the visceral pleura 1
• Release of entrapped lung parenchyma 1
• Restoration of chest wall mechanics 1

Expected Outcomes

Surgical decortication in appropriate candidates yields:

• Significant improvement in dyspnea: Borg dyspnea scale improvements from 4 to 2 1
• Substantial gains in lung function: FVC improvements from 24% to 47% predicted, TLC from 35% to 54% predicted 1
• Enhanced functional capacity: 6-minute walk distance increases (220m to 384m) 1
• Prevention of permanent disability: Early intervention prevents progression to irreversible fibrothorax 5

Special Considerations and Pitfalls

Timing of Surgery

Early surgical intervention (within 48 hours to weeks) prevents chronic fibrothorax formation in post-traumatic or post-infectious cases 5. Delayed treatment allows dense fibrosis to develop, making decortication more difficult and outcomes less favorable 5.

Patient Selection

Surgery is most beneficial for:

• Otherwise healthy patients with isolated fibrothorax 1
• Patients with preserved underlying lung parenchyma 1
• Those who have failed maximal medical therapy for at least 3-6 months in autoimmune cases 1, 4

Avoid surgery in:

• Active empyema or infection (drain and treat infection first) 4
• Extensive underlying lung parenchymal disease (asbestosis, COPD) where decortication won't improve function 3
• Patients too frail for thoracic surgery 3

Asbestos-Related Fibrothorax

For asbestos-related diffuse pleural thickening causing restrictive defects, the evidence is more limited 3. While decortication has been described in early literature for severe cases, the functional benefit is less predictable when underlying parenchymal asbestosis coexists 3, 6. The pleural fibrosis contributes independently to FVC reduction, but surgery should be considered only when pleural disease is the dominant pathology 6.

Post-Operative Management

• Aggressive pulmonary rehabilitation maximizes functional gains 1
• Continue immunosuppression in autoimmune cases to prevent recurrence 1, 4
• Monitor for complications: Prolonged air leak, infection, incomplete lung expansion 1

Conservative Management Alternatives

When surgery is contraindicated or refused:

• Supportive care: Oxygen therapy, pulmonary rehabilitation, treatment of underlying disease 3
• Pleural drainage procedures: Limited role once dense fibrosis established 3
• Intrapleural corticosteroids: Anecdotal use in rheumatoid cases, but evidence is weak 4

These alternatives provide only symptomatic relief and do not restore lung function. 1