Boerhaave’s syndrome is a spontaneous rupture of the esophagus that occurs during intense straining. It most typically occurs during an episode of forceful or repeated vomiting. When the esophagus tears, toxic contents can leak out and cause infection. This is an emergency. Without treatment, it can be fatal within days.
Boerhaave’s syndrome is a rupture of your esophagus caused by tremendous stress. It happens when forceful vomiting or excessive straining cause your esophagus wall to split open. Healthcare professionals call this an "effort rupture" or “spontaneous rupture" to distinguish it from ruptures caused by direct injury to your esophagus. Boerhaave's syndrome is an uncommon cause of esophageal rupture, affecting an estimated 0.0003% of the population. It represents 15% of all traumatic ruptures of the esophagus.
A Mallory Weiss tear is a tear in the inner lining of your esophagus. Like Boerhaave’s syndrome, it also occurs after forceful vomiting or straining, and both are associated with drinking too much alcohol. A Mallory Weiss tear will cause you to vomit blood, but it doesn't tear all the way through the esophagus. Boerhaave’s syndrome, on the other hand, ruptures the full thickness of the esophagus wall. This is also called a transmural tear. A transmural tear will likely need emergency repair, and repairing it can be a matter of life or death.
A split in the esophagus is an emergency and can be fatal. That’s because your esophagus, as part of your gastrointestinal tract, is designed to carry substances that are toxic to the rest of your body. If food particles, bacteria and digestive chemicals leak from your esophagus into your chest or abdomen, serious infection can result. Infection that enters your systemic bloodstream can lead to septicemia and sepsis, a life-threatening reaction that can include shock, multiple organ failure and death.
It can affect anyone but is more frequent in people assigned male at birth and in people older than 50. Up to 80% of those affected are middle-aged males. It's more common in people who drink heavily.
The pain is severe — some say excruciating — and vaguely localized around the site of the rupture. This is most often in the lower third of your esophagus, which passes through the bottom of your chest cavity and the top of your abdominal cavity. But it could be higher. It may also radiate to your back or shoulder.
Two factors seem to be involved. One is a build-up of pressure inside your esophagus from extraordinary effort or straining. The other is a build-up of negative pressure outside your esophagus when your upper esophageal sphincter (cricopharyngeus muscle) fails to relax in response to pressure.
The most common cause of strain in Boerhaave’s syndrome is forceful or repeated vomiting. Other possible causes include childbirth, seizures, weightlifting and ingestion of something caustic or corrosive.
Normally, in these circumstances, nerve signals should have triggered the cricopharyngeus muscle to relax to release the internal pressure. But in Boerhaave’s syndrome, this neuromuscular coordination fails for some reason.
Excessive alcohol use is certainly a risk factor. The classic presentation of Boerhaave’s syndrome follows repeated episodes of vomiting after excessive food and/or alcohol consumption. Alcohol use disorder sets the stage, as do binge eating and bulimia.
Other possible risk factors include underlying esophageal disorders such as esophagitis and eosinophilic esophagitis, Barrett’s esophagus and peptic ulcer disease. However, most people affected by Boerhaave’s syndrome have a normal esophagus.
Boerhaave’s syndrome can be difficult to diagnose, and timely diagnosis is important. It’s easier when it presents with the classic triad of symptoms known as the Mackler triad. These include severe or repeated vomiting, sudden onset chest pain and subcutaneous emphysema — a condition in which air is trapped in the tissues under your skin. This last symptom is especially telling of an esophageal rupture.
But not all cases present with the classic symptoms. The rupture may occur in an uncommon location, leading to pain in an uncommon area like your neck or clavicles. It may occur from less common causes than vomiting, or it may come with uncommon side effects. A standard chest X-ray might give some hints, but it will take a more sensitive imaging test to diagnose the condition.
A fluoroscopic X-ray with contrast dye (esophagram) is usually the first choice when your provider suspects Boerhaave’s syndrome, because it's quick and noninvasive with a high level of accuracy. For this test, you’ll swallow a solution with a water-soluble contrast dye that will highlight the inside of your esophagus in the X-ray. If you have a tear, they’ll be able to see where the contrast dye leaks.
A CT scan is an alternative choice if the esophagram won’t work for you, or if your healthcare provider needs to see the surrounding organs in more detail. It won’t show the exact location of the tear, but it can find small amounts of leaked contrast dye and air from your esophagus in the surrounding tissues. It’s also used to locate pockets of leaked fluids in your chest and abdominal cavities that will need to be drained.
Treatment follows these steps:
Early diagnosis and treatment is key to avoid the complications of infection, including death. Those who are treated within 24 hours have a good prognosis and a survival rate of 75%. After 24 hours, the chance of death is more than 50%, and after 48 hours it’s 90%. The overall mortality rate is around 35%. Those who are successfully treated in time can heal completely, but the recovery may take several months.
A note from Cleveland Clinic
Boerhaave’s syndrome is a rare but sobering anomaly. It can strike suddenly and without precedence — although chronic binge drinking is one clear precedent that we can avoid. We don’t understand everything about Boerhaave’s syndrome. We don’t know why the esophagus fails to preserve itself from harm in these cases, but we do know that it can be fatal without quick recognition and treatment.
Last reviewed by a Cleveland Clinic medical professional on 04/27/2022.
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