What are the typical characteristics and presentation of spontaneous perforation of the esophagus?

Spontaneous Perforation of the Esophagus: Characteristics and Presentation

Spontaneous perforation of the esophagus (Boerhaave syndrome) typically presents with sudden onset of severe chest or abdominal pain following forceful vomiting, most commonly affects the lower third of the esophagus, and is not the most common cause of esophageal perforation. 1, 2

Etiology and Epidemiology

• Iatrogenic injury is the most common cause of esophageal perforation (approximately 60% of cases), not spontaneous perforation 2, 3
• Spontaneous perforation (Boerhaave syndrome) accounts for only about 15% of all esophageal perforations 2
• Eosinophilic esophagitis (EoE) has emerged as an important cause of spontaneous esophageal perforation in both children and adults 1

Anatomical Location

• Spontaneous perforation typically occurs in the lower third of the thoracic esophagus, usually on the left posterolateral wall 2, 4
• This is in contrast to EoE-related perforations which can occur at any level of the esophagus 1
• The perforation is usually a large wall defect (3-8 cm) in Boerhaave syndrome 2

Clinical Presentation

• Sudden onset of severe chest or abdominal pain is the hallmark presentation 1, 2, 4
• The classic triad includes:
  • Vomiting (preceding the perforation)
  • Chest pain
  • Subcutaneous emphysema
• However, this classic triad is present in only a minority of cases 4
• Additional symptoms may include:
  • Dyspnea
  • Signs of sepsis in later stages
  • Pleural effusion (can be right-sided in some cases) 5

Risk Factors and Demographics

• More common in middle-aged adults
• Often associated with:
  • Forceful vomiting (often after heavy meals or alcohol consumption)
  • Increased abdominal pressure
• Not typically associated with elderly females 5
• Not usually preceded by a history of dysphagia, unless the perforation is related to underlying EoE 1

Diagnostic Considerations

• Misdiagnosis is common and contributes to high morbidity and mortality 5
• Contrast-enhanced CT and CT esophagography have high sensitivity (92-100%) 1
• Diagnostic delay beyond 24 hours significantly increases mortality 2
• Radiographic findings may include:
  • Pneumomediastinum
  • Pleural effusion
  • Subcutaneous emphysema
  • Pneumothorax

Important Distinctions

• Boerhaave syndrome differs from EoE-related perforations:
  • Boerhaave: large full-thickness tear in lower esophagus with massive contamination, high mortality (>50%) 1
  • EoE perforation: usually multiple small tears or tissue plane dissection, limited extravasation, lower mortality 1

Treatment Approach

• Early diagnosis and prompt treatment are critical
• Management options include:
  • Surgical repair for large perforations
  • Conservative management for contained perforations
  • Endoscopic interventions (clips, stents) for selected cases 1

In summary, spontaneous esophageal perforation is characterized by sudden onset of pain, typically affects the lower esophagus, is not the most common cause of esophageal perforation, is not typically preceded by dysphagia, and does not predominantly affect elderly females.