Fibrothorax

Fibrothorax is fibrosis of the pleural space, and inflammatory response to a precipitating event.

Relevant physical signs

• Hands and wrists
  • Clubbing – may be related to initiating disease process
  • Typically not cyanosed
  • Pulse and respiratory rate usually normal
• Head and neck
  • No central cyanosis
  • Trachea deviated towards affected side
  • Jugular venous pressure usually not elevated
  • Cervical lymphadenopathy for underlying malignancy
• Chest
  • Scars
    • Lateral thoracotomy scar – previous pneumonectomy / lobectomy, or previous decortication
    • Smaller, anterior axillary line scars suggesting previous chest drain
    • Multiple small scars suggesting previous VATS
    • If no scar, excludes pneumonectomy / lobectomy as a differential diagnosis
  • Chest expansion reduced on the affected side
  • Percussion note dull, but not stony dull
  • Breath sounds reduced on the affected side
  • May have evidence of underlying aetiology
    • Apical fine end-inspiratory crepitations representing post-TB fibrosis
    • Coarse crepitations of bronchiectasis complicated by pleural infection

Differential diagnosis

• Lung collapse
• Solid tumour
• Pneumonectomy / lobectomy (would expect lateral thoracotomy scar)

Causes of fibrothorax

• Pleural infections and injury
  • Pleural tuberculosis
  • Pleural empyema
  • Haemothorax, especially if incompletely drained
  • Lung abscess
  • Bronchiectasis
• Occupational lung disease
  • Asbestos-related pleural disease
• Inflammatory connective tissue disease
  • Systemic lupus erythematosus
  • Rheumatoid arthritis

Investigations

• Chest radiograph
  • Smooth thickening of pleural spaces, usually with sparing of mediastinal pleura
  • Marked volume loss of affected hemithorax
• Spirometry showing restrictive defect: decreased lung volume and diffusion capacity
• Computed tomography to rule out collapse and underlying lung malignancy
• Consider pleural biopsy to rule out pleural malignancy

Management

• Multidisciplinary team approach
• Patient education
• Smoking cessation, if applicable
• Yearly influenza vaccine, five-yearly pneumococcal vaccine
• Chest drainage of pleural effusion, if any
• Consider surgical decortication if respiratory symptoms are disabling

Summary

Sir, this patient has a reduced lung volume on the left side in the absence of a lateral thoracotomy scar and palpable lymphadenopathy. The most likely diagnosis is a fibrothorax. On examination of the peripheries, there is no clubbing or cyanosis, and the patient is comfortable at rest. The trachea is deviated to the left side. Chest expansion is reduced over the whole of the left hemithorax, as are the breath sounds. There are no other added sounds. There are no scars to suggest previous pneumonectomy or lobectomy, although I note the presence of a small scar just lateral to the anterior axillary line, which may indicate previous chest tube placement. Possibilities for such findings include a fibrothorax, lung collapse secondary to an endo-bronchial mitotic process or a primary pleural mitotic lesion. Causes of fibrothorax include infections such as tuberculousis, pleural empyemas, traumatic injury such as a haemothorax, occupational-related asbestos exposure and inflammatory conditions such as systemic lupus erythematosus and rheumatoid arthritis.

In summary, this patient has a restrictive defect on the left side associated with tracheal deviation. The most likely aetiology is a fibrothorax.