What is Boerhaave syndrome?

What is Boerhaave Syndrome?

Boerhaave syndrome is a spontaneous, full-thickness rupture of the esophagus caused by forceful vomiting against a closed glottis, typically occurring in the distal esophagus on the left posterolateral wall, and represents a life-threatening emergency with 10-20% mortality that increases exponentially if treatment is delayed beyond 24 hours. 1, 2

Mechanism and Pathophysiology

• The rupture results from an abrupt increase in esophageal pressure during forceful vomiting when the upper esophageal sphincter fails to relax 2
• The tear characteristically occurs on the left border of the lower third of the thoracic esophagus where the wall is anatomically weaker on the posterolateral aspect 2
• The wall defect is typically large (3-8 cm) and causes contamination of the left thorax with air and digestive contents 2
• This accounts for approximately 15% of all esophageal perforations 1

Clinical Presentation

The classic presentation includes chest pain (70% of cases), fever (50%), dyspnea (25%), and subcutaneous emphysema (19%), though the complete Mackler's triad (chest pain, vomiting, subcutaneous emphysema) is rarely present. 1, 3

Key warning signs requiring immediate attention:

• Tachycardia is the most critical vital sign abnormality that should raise immediate suspicion 4
• Development of shock (pulse >100 beats/min and systolic BP <100 mmHg) 4
• Persistent rather than transient pain 4
• Rapid clinical deterioration suggesting mediastinal contamination and developing sepsis 4

Common Diagnostic Pitfalls

• Symptoms are frequently misdiagnosed as cardiogenic in origin 3
• Over 50% of cases have delayed diagnosis due to atypical presentation 4
• Can present as tension hydropneumothorax, further obscuring the diagnosis 3
• Must be distinguished from Mallory-Weiss syndrome, which shares similar history and symptoms but involves only mucosal tears without perforation 5

Diagnostic Approach

Contrast-enhanced CT is the imaging examination of choice with 92-100% sensitivity and should be performed immediately in suspected cases. 4

Imaging findings include:

• Pneumomediastinum 4
• Pneumothorax 4
• Air under the diaphragm 4
• Pleural effusion (typically left-sided) 4
• Subcutaneous emphysema 4

CT esophagography can confirm the diagnosis and show the location and extent of the perforation 6

Critical Distinction from EoE-Related Perforations

Boerhaave syndrome differs fundamentally from eosinophilic esophagitis (EoE) perforations in both presentation and prognosis. 6

Key differences:

• Boerhaave syndrome: Large full-thickness tear (3-8 cm) in the lower third of the distal esophagus with massive contamination of the right thorax, often requires surgical intervention, and has mortality >50% 6
• EoE perforations: Usually multiple small partial tears or tissue plane dissections with limited extravasation (mostly air and liquid, not large amounts of food), surgery required in only 30% of cases, and no reported mortality 6

Treatment Algorithm Based on Timing

Time to diagnosis is the most important survival predictor—treatment within 24 hours significantly improves outcomes. 1, 2

Early Diagnosis (<24 hours) WITHOUT Sepsis:

• Endoscopic treatment with stent placement is the preferred approach 7
• Survival rate with endoscopic approach: 100% 7

Early Diagnosis (<24 hours) WITH Sepsis:

• Thoracotomy with hemifundoplication and pleural/mediastinal drainage 7
• For intra-abdominal leakage: laparotomy for local repair 7
• Surgical approach survival rate: 81% 7

Late Diagnosis (>24 hours):

• Conservative treatment should be initiated (bowel rest, IV antibiotics, drainage) 7
• Surgical treatment only indicated if patient develops septic profile 7
• Late diagnosis carries significantly higher mortality (38% vs 0% in early diagnosis) 8

Multidisciplinary Management Required:

• All cases require management by a team including gastrointestinal surgeon, gastroenterologist, and radiologist 6
• This is now standardized practice given the complexity of decision-making regarding drainage, nutrition, stenting, and surgical intervention 6

Prognosis

• Overall 90-day mortality: 14% with appropriate treatment 8
• Mortality ranges 10-20% overall but increases exponentially with delayed diagnosis 1, 2
• Late-diagnosed patients (>24 hours) have 38% mortality compared to 0% in early-diagnosed patients 8
• Stent complications occur in 37% of cases, including leakage (16%), migration (16%), and bleeding (5%) 8
• Additional interventions required in 67% of patients 8
• Median hospital stay: 32 days 8