What is Boerhaave syndrome?
Boerhaave syndrome is a transmural (full-thickness) perforation of the oesophagus that typically follows forceful vomiting or retching. It is called 'spontaneous' rupture to distinguish it from iatrogenic perforation (after endoscopy or surgery), even though a trigger — usually emesis — is almost always present. It accounts for about 10–15% of all oesophageal perforations.
The condition is named after the Dutch physician Hermann Boerhaave (1668–1738), whose account of the fatal case of Admiral Baron Jan von Wassenaer gave the syndrome its name.
Why does the oesophagus rupture, and where?
Boerhaave syndrome is a barogenic injury. During vomiting the cricopharyngeus fails to relax, so the gastric contents are forced up against a closed upper sphincter. The pressure surge overwhelms the oesophageal wall at its weakest point.
- Site: the left posterolateral wall of the distal (lower third) oesophagus, just above the gastro-oesophageal junction. StatPearls places it about 2–3 cm proximal to the GOJ; Radiopaedia says about 90% occur on the left posterolateral wall, 3–6 cm above the oesophageal hiatus.
- Why there: that segment is weaker because of entering neurovascular structures and a relative lack of longitudinal muscle fibres.
- Shape: usually a longitudinal tear, averaging 3–8 cm.
- Side of effusion: the lower oesophagus abuts the left pleura, so leakage usually enters the left pleural cavity. Mid-oesophageal tears tend to give a right-sided effusion or hydropneumothorax.
- Cervical ruptures are usually localised and benign, because spread to the mediastinum through the retro-oesophageal space is slow.
Triggers and risk factors: vomiting after alcohol excess or overeating is the classic story, but any sudden rise in intra-oesophageal pressure can do it — weightlifting, straining at stool, epileptic seizures, blunt abdominal trauma, compressed-air injury and childbirth. Most patients have a normal oesophagus, though oesophagitis or ulcers are found in a subset.
Who gets Boerhaave syndrome?
- Incidence: about 3.1 per million people per year (probably under-reported).
- Sex: male predominance, with male-to-female ratios of 2:1 to 5:1.
- Age: highest risk in men in their sixth and seventh decades, though cases are reported from neonates to people over 90; children aged 1–17 are least affected.
- Classic patient: a middle-aged man who vomits forcefully after a heavy meal or a bout of drinking.
What are the Mackler triad and Hamman sign?
The Mackler triad is vomiting, lower thoracic (chest) pain and subcutaneous emphysema. It is the textbook picture, but StatPearls stresses that it is rarely seen in full — and 25–45% of patients give no history of vomiting at all. That is why the diagnosis is so often delayed.
| Symptoms | Signs |
|---|---|
| Sudden severe chest, epigastric or neck pain after vomiting | Subcutaneous emphysema (crepitus in the neck or chest wall) |
| Odynophagia, dysphagia, hoarseness | Hamman sign — a mediastinal crunch synchronous with the heartbeat, heard best in the left lateral decubitus position |
| Haematemesis (usually small) | Fever, tachycardia, tachypnoea, hypotension, cyanosis |
| Breathlessness | Decreased breath sounds (pleural effusion), abdominal rigidity, shock |
How is Boerhaave syndrome different from Mallory-Weiss syndrome?
| Feature | Boerhaave syndrome | Mallory-Weiss syndrome |
|---|---|---|
| Depth | Transmural (full thickness) | Mucosal ± submucosal longitudinal laceration |
| Site | Left posterolateral distal oesophagus | Gastro-oesophageal junction |
| Main problem | Leak → mediastinitis, empyema, sepsis | Bleeding → haematemesis |
| Key signs | Chest pain, subcutaneous emphysema, Hamman sign | Haematemesis after retching; often stable |
| Diagnosis | Water-soluble contrast swallow or CT | Upper GI endoscopy |
| Natural course | Fatal without treatment | Up to 90% stop bleeding spontaneously |
| Treatment | Nil by mouth, antibiotics, drainage, early repair or stent | Supportive; endoscopic haemostasis if bleeding persists |
Mallory-Weiss tears account for 3–10% of non-variceal upper GI bleeding, and heavy alcohol use is the commonest risk factor (50–70% of cases). Endoscopy, which is the test of choice for Mallory-Weiss, is controversial in suspected Boerhaave because it can extend the perforation.
Which investigations confirm oesophageal rupture?
- Chest X-ray: pneumomediastinum, mediastinal widening, left pleural effusion, left pneumothorax, subcutaneous emphysema.
- Naclerio V sign: a V-shaped lucency of air dissecting along the left paraspinal region just above the diaphragm — seen in up to 20%; specific but insensitive.
- Contrast oesophagogram with a water-soluble agent (Gastrografin) shows the leak, usually just above the diaphragm. Barium is avoided first because extravasated barium causes mediastinitis and fibrosis; if the water-soluble study is negative, it can be repeated with barium. False negatives occur in 10–38%.
- CT chest (often with oral contrast): periesophageal and mediastinal gas, fluid collections, oesophageal wall thickening, effusion or pneumothorax. It is better tolerated by sick patients and maps collections for drainage.
- Pleural fluid: undigested food particles, pH below 6 and a raised salivary amylase strongly suggest oesophageal rupture.
- Bloods: leukocytosis with left shift; haemoconcentration (haematocrit around 50%).
How is Boerhaave syndrome managed?
Management depends on time since rupture, the size and containment of the leak, and how sick the patient is. Every patient needs the same foundation: nil by mouth, aggressive fluid resuscitation, broad-spectrum IV antibiotics, parenteral nutrition and drainage of pleural or mediastinal collections, with early thoracic surgical input.
| Situation | Usual approach |
|---|---|
| Early (within about 24 hours), free leak, fit patient | Primary surgical repair — open thoracotomy or VATS, with fundic reinforcement (described as the gold standard) |
| Small, contained leak, contrast drains back into the oesophagus, no sepsis | Conservative: nil by mouth for at least 7 days, IV antibiotics for 7–14 days, parenteral nutrition, drainage — with surgeons ready if the patient deteriorates |
| Late (after 24 hours) with friable, oedematous edges | Debridement and drainage of pleura and mediastinum, oesophagostomy and feeding gastrostomy; reconstruction after about 6 weeks |
| Diseased, non-viable oesophagus | Resection |
| High surgical risk | Endoscopic therapy — fully covered stent, through-the-scope or over-the-scope clips, endoscopic suturing |
Indications to abandon conservative treatment include a contained leak becoming free, extension of the injury, persistent fever, sepsis, progression of pneumothorax or pneumomediastinum, and empyema.
Which Boerhaave facts do examiners test most?
- Pathology: transmural (full-thickness) tear — not a mucosal tear. A mucosal tear with haematemesis is Mallory-Weiss.
- Mechanism: sudden rise in intraluminal pressure against a non-relaxing cricopharyngeus.
- Commonest site: left posterolateral wall of the distal oesophagus, a few centimetres above the GOJ.
- Commonest pleural side: left. A right-sided effusion suggests a higher (mid-oesophageal) tear.
- Triad: Mackler — vomiting, lower chest pain, subcutaneous emphysema. Do not confuse it with Beck's triad (tamponade) or Virchow's triad (thrombosis).
- Auscultation: Hamman sign — precordial crunch with each heartbeat.
- X-ray sign: Naclerio V sign — specific but seen in only about one in five.
- First contrast study: water-soluble (Gastrografin), not barium.
- Avoid routine diagnostic endoscopy when rupture is suspected — it can enlarge the tear.
- Prognosis hinges on time: best within 12–24 hours; primary repair is risky after 24 hours because the edges become oedematous and friable.
- Gold-standard repair: primary repair by thoracotomy or VATS with fundic reinforcement.
Compare the triad with Virchow's triad, and see anti-emetic drugs for the drugs used to control the vomiting that sets the stage.
What complications and outcomes should you know?
- Complications: mediastinitis, empyema, massive pleural effusion, oesophageal fistula, sepsis, ARDS, shock and death.
- Mortality figures vary widely by series and delay. StatPearls quotes up to 60% even with intervention; a 2024 single-centre series of 33 surgically treated patients (2012–2022) reported 24% in-hospital mortality, rising to 31% with delayed presentation.
- Morbidity remains high even with modern care — 57% had postoperative complications in that series.
- Prevention and counselling: people who binge-drink or binge-eat should seek urgent care for sudden chest, neck or abdominal pain after forceful vomiting.
Related reading: airway and ventilation choices for a septic thoracic patient in mechanical ventilation modes, and the acid-base work-up of a septic, shocked patient via the anion gap. More surgical one-liners are on most repeated topics.