Fibrothorax

Etiology

  • Empyema (common etiology) (see Pleural Effusion-Parapneumonic, [[Pleural Effusion-Parapneumonic]]): unilateral
  • Tuberculosis (see Tuberculosis, [[Tuberculosis]]): unilateral
  • Paragonimiasis (see Paragonimiasis, [[Paragonimiasis]]): unilateral
  • Hemothorax (common etiology) (see Pleural Effusion-Hemothorax, [[Pleural Effusion-Hemothorax]]): unilateral
  • Pancreatitis (see Chronic Pancreatitis, [[Chronic Pancreatitis]]): unilateral
  • Collagen Vascular Disease: unilateral
  • Uremia (see Uremic Pleurisy, [[Uremic Pleurisy]]): unilateral
  • Asbestos Exposure-Related Pleural Plaques (see Pleural Plaques, [[Pleural Plaques]]): bilateral
    • Deposition is mainly on the parietal pleural surface
    • Predisposed by occurrence of pleural effusion after asbestos exposure

Physiology

  • Hypoventilation with acidosis and hypoxia -> pulmonary vasoconstriction
    • Pulmonary hypertension due to chest wall disease (due to development of thick visceral pleural fibrous peel/parietal surface in asbestos exposure)
  • On the side of the pleural peel, blood flow is decreased out of proportion to ventilation
  • Progression of fibrothorax leads to restriction of chest wall movement, narrowing of intercostal spaces, decreased size of hemithorax, and displacement of medistinum to the affected side

Diagnosis

  • ABG: hypoxemia/hypercapnia may be seen in bilateral cases (associated with asbestos exposure)
  • PFT’s: mild-severe restrictive pattern
  • CXR/Chest CT: pleural plaques may be seen and are often calcified (on the inner aspect of the peel)

Clinical

  • Dyspnea: depends on extent of disease
  • Pulmonary Hypertension/Cor Pulmonale (see Pulmonary Hypertension, [[Pulmonary Hypertension]])
  • Acute/Chronic Hypoventilation (see Acute Hypoventilation, [[Acute Hypoventilation]] and Chronic Hypoventilation, [[Chronic Hypoventilation]])
    • Respiratory failure may be seen in bilateral cases associated with asbestos exposure

Treatment

  • Surgical Decortication: only available treatment
    • Degree of success depends on integrity of unerlying lung (bilateral fibrothoraces do not respond well to surgery, probably due to underlying parenchymal lung disease)
    • VC may improve >50% after surgery if underlying lung is normal (VC may decrease in some cases where underlying lung is abnormal)
    • Duration of fibrothorax: does not affect surgical success
    • Timing of surgery: improve-ment occurs in first 3-6 months after acute insult that produced fibrothorax (sur-gery should be delayed until later to observe for any spontaneous improvement)

References

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