Definition And Epidemiology

  • Accumulation extrapulmonary air within chest cavity between parietal, visceral pleura.
  • Incidence: 4/100,000 boys, 1/100,000 girls.

Etiology And Classification

Spontaneous Pneumothorax

ClassificationCharacteristics And Associations
Primary Spontaneous (PSP)Lacks underlying lung disease. Typically tall, thin adolescent boys. Associated smoking (tobacco, marijuana, crack cocaine, e-cigarettes), MDMA, Valsalva maneuver, apical blebs (controversial).
Secondary Spontaneous (SSP)Underlying lung disease present. Congenital: Congenital Pulmonary Airway Malformation (CPAM), Congenital Lobar Emphysema (CLE), bronchogenic cysts, Birt-Hogg-Dube syndrome. Increased Intrathoracic Pressure: Asthma, bronchiolitis, Cystic Fibrosis (CF), foreign body. Infections: Tuberculosis, Pneumocystis jirovecii, pneumatocele, lung abscess, COVID-19. Lung Diseases: Langerhans cell histiocytosis, Marfan syndrome, Ehlers-Danlos syndrome, pulmonary fibrosis, metastatic osteosarcoma.

Traumatic And Neonatal Pneumothorax

CategoryCauses
TraumaticNoniatrogenic: Blunt, penetrating trauma. Iatrogenic: Thoracotomy, thoracentesis, tracheostomy, mechanical ventilation, high-flow therapy, tube/needle puncture.
NeonatalRespiratory Distress Syndrome (RDS), meconium aspiration, mechanical ventilation. Oligohydramnios (renal agenesis, obstructive uropathy) leading pulmonary hypoplasia.

Contributory Factors

  • Apical blebs/bullae.
  • Pleural porosity (disturbed mesothelial cells).
  • Folliculin gene mutations.

Pathophysiology

Mechanism Of Air Leak

  • Normal resting state balances outward chest wall expansion against inward lung elastic recoil, generating negative intrapleural pressure.
  • Air entering pleural space collapses lung.
  • Alveolar hypoventilation, ventilation-perfusion mismatch, intrapulmonary shunt induce hypoxemia.
  • Simple pneumothorax: Atmospheric intrapleural pressure, lung collapses up 30%.

Tension Pneumothorax

  • Continuous air leak creates increasing positive intrapleural pressure.
  • Compresses lung, shifts mediastinal structures contralaterally.
  • Decreases venous return, cardiac output, precipitating hemodynamic instability, shock.
  • Frequent Secondary Spontaneous Pneumothorax, Traumatic Pneumothorax; uncommon Primary Spontaneous Pneumothorax.

Clinical Manifestations

Symptoms

  • Abrupt onset.
  • Sharp chest pain, pleuritic nature, worsening during inspiration, cough.
  • Dyspnea, cyanosis, breathlessness.
  • Potentially minimal/absent depending collapse extent.

Physical Signs

  • Tachypnea.
  • Affected hemithorax: Decreased chest expansion, hyper-resonance percussion, diminished/absent breath sounds.
  • Larynx, trachea, heart shifted toward unaffected side.
  • Fluid presence: Gurgling sounds synchronous respirations.
  • Tension Pneumothorax specific: Cyanosis, sweating, severe tachypnea, tachycardia, shock.

Evaluation And Diagnosis

Imaging Modalities

ModalityDiagnostic Findings
Chest Radiograph (Upright)First-line investigation. Black radiolucent rim between visceral pleura, chest wall. Absent bronchovascular markings. Expiratory views accentuate contrast.
Chest UltrasoundReplacing Chest X-Ray Pediatric Intensive Care Unit (PICU) point-of-care tool. Excludes Pneumothorax: Lung sliding, lung pulse, B lines. Confirms Pneumothorax: Absent lung slide, presence lung point, barcode/stratosphere sign.
Computed Tomography (CT)Not routinely recommended initial diagnosis. Identifies underlying disease (apical blebs), evaluates contralateral normal lung recurrences.
TransilluminationUseful infants <6 months using fiberoptic light probe. Unreliable older patients, subcutaneous emphysema, atelectasis.

Sizing Criteria

  • Large Pneumothorax (British Thoracic Society): >=2 cm lung margin chest wall hilum level.
  • Large Pneumothorax (American College Chest Physicians): >=3 cm apex-to-cupola distance.

Differential Diagnosis

  • Localized/generalized emphysema, large emphysematous blebs.
  • Large pulmonary cavities, cystic formations.
  • Diaphragmatic hernia (evaluate via Barium swallow).
  • Compensatory overexpansion concurrent contralateral atelectasis.
  • Gaseous gastric distention.

Management

Stepwise Interventions

  • Goal encompasses air removal, recurrence prevention.

Observation And Oxygenation

  • Indicated small Pneumothorax (<2 cm), lacking respiratory distress.
  • High-flow oxygen (10-100%) decreases nitrogen partial pressure, hastens lung reexpansion.
  • Avoid observation very young children, traumatic Pneumothorax.

Needle Aspiration

  • Indicated large Pneumothorax (>2 cm), respiratory distress, Tension Pneumothorax.
  • Execute utilizing large bore intravenous catheter, 3-way stopcock.
  • Tension Pneumothorax requires emergent needle decompression prior radiographic confirmation.

Tube Thoracostomy

  • Indicated failed simple aspiration, recurrence <7 days, underlying lung disease.
  • Site: Midclavicular line, second intercostal space.
  • Size: 10 F pigtail catheter minimizes pain, scarring.
  • Suction: -20 cmH2O required solely failure inflate >24 hours.
  • Removal criteria: Complete reinflation 24 hours, absent bubbling.

Pleurodesis

  • Obliterates pleural space adhering parietal, visceral pleura.
  • Indications: First occurrence Secondary Spontaneous Pneumothorax, second recurrence Primary Spontaneous Pneumothorax, bilateral spontaneous Pneumothorax, persistent air leak >5-7 days.
  • Surgical: Video-Assisted Thoracoscopic Surgery (VATS) or open thoracotomy utilizing mechanical abrasion.
  • Chemical: Talc, doxycycline, povidone-iodine, autologous blood patch. Painful, 25% recurrence rate.

Complications And Follow-Up

Complications

  • Reexpansion Pulmonary Edema: Suspect developing new-onset cough, breathlessness, chest tightness post-drain insertion. Treat utilizing Positive End-Expiratory Pressure (PEEP).

Follow-Up Guidelines

  • Air Travel: Delay flying 7 days to 6 weeks post-resolution. Risk ipsilateral, contralateral recurrence.
  • Scuba Diving: Absolutely contraindicated lifelong post-Pneumothorax children.