Boerhaave syndrome: spontaneous oesophageal rupture

Boerhaave's Syndrome: Spontaneous Oesophageal Rupture

Boerhaave's syndrome is a rare but life-threatening condition where the oesophagus suddenly ruptures across its full thickness. This most often occurs after violent vomiting and is one of the most frequently missed diagnoses in emergency departments. Half of patients die if the condition is not detected within 24 hours. However, if it is diagnosed quickly, the vast majority recover.

The disease is named after the Dutch physician Herman Boerhaave, who described it in 1724 in a Dutch admiral who died after a large meal and severe vomiting. Unfortunately, we haven't made much progress with early diagnosis since then, and it remains a medical challenge.

What happens?

The oesophagus is a muscular tube that runs from the throat down to the stomach, behind the heart. When you vomit, the brain sends signals that coordinate the closure of the epiglottis, a contraction of the diaphragm, and a relaxation of the sphincter muscle at the top of the stomach. If this coordination fails, or if pressure builds up without the muscle at the top of the stomach relaxing, the pressure inside the oesophagus rises dramatically within seconds. The result is a vertical tear, most often on the left side of the lower part of the oesophagus. The tear is typically two to eight centimetres long.

It's not only vomiting that can trigger this. Rarer causes include severe coughing, heavy lifting, childbirth, and epileptic seizures. Often, a large meal or alcohol is involved.

What are the symptoms?

The classic symptom triad (Mackler's triad) is:

1. Violent vomiting, often immediately preceding the pain.
2. Sudden, severe pain behind the breastbone or in the upper abdomen. The pain can radiate to the back or left shoulder.
3. Subcutaneous emphysema, meaning air under the skin, which can be felt as a crackling sensation in the neck.

Only a third of patients experience all three symptoms simultaneously, which is part of the reason the diagnosis is often missed. Many believe they are having a heart attack, a perforated stomach ulcer, or a pulmonary embolism.

Later symptoms include shortness of breath, bluish lips, rapid pulse, and a drop in blood pressure. These are signs that digestive contents have leaked into the chest cavity and that sepsis is developing.

How is the diagnosis made?

Time is critical. Every minute matters. A CT scan with oral contrast is the fastest way to diagnose. It will show air outside the oesophagus, fluid in the chest cavity, and the tear in the wall itself.

A plain chest X-ray may show air in the mediastinum or a collection of fluid near the left lung, but it can also appear completely normal in the first few hours. Gastroscopy is rarely used as a first investigation due to the risk of worsening the injury, but it may play a role later.

Treatment

Treatment depends on how much time has passed, the size of the tear, and the patient's condition:

• Within 24 hours: Surgery is the first choice. The operation closes the tear with sutures, removes any necrotic tissue, and drains the chest cavity. The patient is given antibiotics and receives nutrition through a feeding tube in the intestine.
• Later than 24 hours, or if the tear is small and the patient is stable: In some cases, conservative treatment with antibiotics, drainage, and fasting may be used, possibly with a metal stent in the oesophagus to close the tear from within.
• When the patient is very ill: A stent can be the quickest solution to stop the leak and buy time.

Even with modern treatment, 10-25 percent of patients die. This underscores the importance of considering the diagnosis early.

When should you seek medical attention?

You should go to the emergency department or call 112 if you experience:

• Severe pain behind the breastbone or in the upper abdomen immediately after forceful vomiting.
• A crackling sensation in the neck after vomiting.
• Increasing shortness of breath after forceful vomiting.

These symptoms can resemble a heart attack, and it's not something you should ignore.

Frequently Asked Questions

Can I get Boerhaave's syndrome from ordinary vomiting due to stomach flu? It is extremely rare. The condition is most often seen in individuals with a combination of violent vomiting and an already weakened oesophageal wall, for example, after large meals, alcohol, or in patients with eosinophilic oesophagitis.

Can the tear heal on its own? Small perforations can, in rare cases, heal without surgery if antibiotics are administered quickly and the patient fasts. This is never something to rely on, but it explains why some patients survive without intervention.

How long is the hospital stay? Two to four weeks in typical cases, and longer with complications. Many patients are on tube feeding for the first few weeks.

Will there be problems with the oesophagus afterwards? Most people can eat normally again, but some develop scar tissue that narrows the oesophagus. This can be treated with balloon dilation during a gastroscopy.

References

1. Brinster CJ, Singhal S, Lee L, Marshall MB, Kaiser LR, Kucharczuk JC. Evolving options in the management of esophageal perforation. Ann Thorac Surg. 2004;77(4):1475-83. PubMed
2. de Schipper JP, Pull ter Gunne AF, Oostvogel HJ, van Laarhoven CJ. Spontaneous rupture of the oesophagus: Boerhaave's syndrome in 2008. Literature review and treatment algorithm. Dig Surg. 2009;26(1):1-6. PubMed
3. Sundhed.dk. Oesophageal perforation (doctor's handbook).
4. Chirica M, Champault A, Dray X, et al. Esophageal perforations. J Visc Surg. 2010;147(3):e117-28. PubMed

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This article provides general information and does not replace medical consultation. Contact your doctor if you experience symptoms.