Gastrointestinal · PANCE / PANRE

Boerhaave Syndrome (Esophageal Perforation)

Spontaneous full-thickness esophageal rupture from forceful vomiting — high mortality, time-critical surgical emergency.

Also known as: Boerhaave, esophageal rupture, spontaneous esophageal perforation

Overview

Spontaneous transmural rupture of the esophagus, typically in the left posterolateral distal third, classically caused by a sudden rise in intraesophageal pressure against a closed cricopharyngeus during forceful vomiting. Distinct from iatrogenic perforation (most commonly from endoscopy).

Epidemiology

Rare (~3.1 per 1,000,000 per year). Most cases occur in men 50-70. Mortality approaches 50% if diagnosis is delayed beyond 24 h, but falls below 20% with treatment within 12 h.

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Risk factors

  • Forceful or recurrent vomiting (alcohol binge, eating disorder, gastroenteritis)
  • Predisposing esophageal disease: eosinophilic esophagitis, peptic stricture, Barrett, malignancy
  • Iatrogenic: dilation, EGD with biopsy, TEE, NG/OG tube placement
  • Trauma, caustic ingestion, foreign body, pill esophagitis

Pathophysiology

A sudden rise in intraluminal pressure exceeds esophageal wall tensile strength, producing a longitudinal full-thickness tear, most often in the left posterolateral distal esophagus where the wall is thinnest. Gastric contents enter the mediastinum and pleural space, producing chemical and then bacterial mediastinitis, pleural effusion, empyema, and sepsis.

Clinical presentation

Symptoms

  • Sudden severe retrosternal, epigastric, or back pain immediately after forceful vomiting
  • Odynophagia, dysphagia
  • Dyspnea, tachypnea
  • Fever and signs of sepsis within hours if diagnosis delayed

Signs / physical exam

  • Mackler triad: vomiting, chest pain, subcutaneous emphysema (present in only ~14%)
  • Hamman sign: crunching sound on auscultation synchronous with heartbeat (mediastinal air)
  • Decreased breath sounds, dullness to percussion (pleural effusion, usually left)
  • Tachycardia, hypotension, fever, leukocytosis

Classic findings

Subcutaneous emphysema of the neck and chest in a patient with severe chest pain after vomiting is highly suggestive.

Differential diagnosis

  • Acute coronary syndrome — Substernal pressure with diaphoresis, ECG changes, troponin elevation; no vomiting trigger
  • Aortic dissection — Tearing chest/back pain, BP differential, widened mediastinum; CTA diagnostic
  • Pulmonary embolism — Pleuritic pain, hypoxia, tachycardia; CTPA diagnostic
  • Pneumothorax / tension pneumothorax — Unilateral absent breath sounds, hyperresonance, tracheal deviation
  • Mallory-Weiss tear — Mucosal only; hematemesis without perforation signs; no pneumomediastinum
  • Perforated peptic ulcer — Sudden epigastric pain with rigid abdomen, free air under diaphragm
  • Pancreatitis — Epigastric pain radiating to back, elevated lipase; may coexist with esophageal injury after vomiting

Diagnostic workup

Diagnostic criteria

Demonstration of extravasation of contrast from the esophagus, with extraluminal air or pleural communication on CT or esophagram.

Labs

  • CBC (leukocytosis), BMP, LFTs, lactate, coagulation studies, type and crossmatch
  • Blood cultures, pleural fluid analysis if effusion tapped (pH <6, elevated amylase from saliva)

Imaging

  • Upright CXR — pneumomediastinum, subcutaneous emphysema, left pleural effusion, hydropneumothorax
  • CT chest with water-soluble oral contrast — preferred initial study; shows extraluminal air, fluid collections, perforation site
  • Water-soluble contrast (Gastrografin) esophagram — confirms perforation; barium reserved if Gastrografin negative (more sensitive but more inflammatory if extravasated)
  • EGD — limited role; risk of extending perforation

Diagnostic algorithm

flowchart TD
  A[Severe chest pain<br/>after forceful vomiting] --> B[Upright CXR + IV access<br/>broad-spectrum antibiotics, PPI]
  B --> C{Pneumomediastinum,<br/>SQ emphysema, or<br/>L pleural effusion?}
  C -->|Yes| D[CT chest with water-soluble<br/>oral contrast]
  C -->|No but high suspicion| D
  D --> E{Perforation confirmed?}
  E -->|Yes| F[Surgical consult emergently]
  F --> G{<24 h, stable,<br/>contained?}
  G -->|Yes| H[Primary repair<br/>+ drainage]
  G -->|No / delayed / unstable| I[Stent, esophageal diversion,<br/>or esophagectomy]
  E -->|No / equivocal| J[Esophagram<br/>Gastrografin then barium]
Emergency evaluation and management algorithm for suspected Boerhaave syndrome.

Treatment

First-line

  • NPO, IV fluids, NG decompression (carefully, often under fluoroscopy)
  • Broad-spectrum IV antibiotics: piperacillin-tazobactam, or carbapenem (meropenem); add antifungal (fluconazole) if immunocompromised or high suspicion of Candida
  • IV PPI (pantoprazole) to reduce gastric acid contamination
  • Surgical consultation immediately — primary repair within 24 h is the goal for hemodynamically stable patients

Complications

  • Mediastinitis, empyema, pleural effusion
  • Septic shock and multiorgan failure
  • Esophageal stricture or fistula (esophagopleural, esophagobronchial)
  • ARDS
  • Death (mortality 20-50% depending on time to diagnosis)

PANCE pearls

  • Time is esophagus — mortality doubles after 24 h delay.
  • Mackler triad is classic but only present in a minority; do not exclude Boerhaave by its absence.
  • Always get an upright CXR in any patient with severe chest pain after vomiting — pneumomediastinum is the giveaway.
  • Use water-soluble (Gastrografin) contrast first; barium gives better detail but is more inflammatory if it extravasates.
  • Iatrogenic perforation (most commonly cervical from EGD) is the most common cause of esophageal perforation overall; Boerhaave is spontaneous and usually distal.

References

  • WSES 2019 — World Society of Emergency Surgery (WSES) guidelines for the management of esophageal perforations (Chirica et al., World J Emerg Surg 2019)
  • SAGES 2014 — SAGES guidelines for the diagnosis and treatment of esophageal perforation

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