Boerhaave's Syndrome

What is Boerhaave’s syndrome?

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.

What is the difference between Boerhaave’s syndrome and Mallory Weiss syndrome?

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.

What happens when the esophagus splits open?

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.

Who does Boerhaave’s syndrome affect?

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.

What are the symptoms of Boerhaave’s syndrome?

• Sudden, severe chest pain.
• Nausea and vomiting.
• Pain or coughing when swallowing.
• Short, rapid breathing.
• Swelling in the tissues of the chest cavity with air or fluid.
• Hard, rigid abdomen.
• Signs of infection like fever and sweating.

How painful is Boerhaave’s syndrome?

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.

What are the possible complications of Boerhaave’s syndrome?

• Inflammation and swelling in your chest cavity.
• A build-up of pus in your chest cavity (empyema).
• Trapped air in your chest cavity or in the tissues under your skin.
• A build-up of fluid in the lining of your chest cavity (pleural effusion).
• Acute respiratory distress syndrome (ARDS).
• Septicemia, sepsis and shock.

What causes Boerhaave’s syndrome?

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.

Can alcohol use cause a ruptured esophagus?

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.

How is Boerhaave's syndrome diagnosed?

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.

What type of radiology is used to diagnose Boerhaave’s syndrome?

Esophagram

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.

CT scan

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.

How is Boerhaave’s syndrome treated?

Treatment follows these steps:

• IV fluids. Most people need immediate IV fluid resuscitation due to overall volume loss.
• Antibiotics. A broad spectrum of antibiotics will be given intravenously to control infection.
• Surgical consult. Surgery is the standard of care for most people. Some small, contained ruptures may qualify for observation and IV antibiotics, or treatment by endoscopy instead. But in these cases, healthcare providers will still want to have a surgical backup plan.
• Surgical repair. Ideally, the rupture should be repaired directly within 24 hours. Depending on your condition, your surgeon may be able to use a minimally invasive surgery technique like video-assisted thoracoscopic surgery (VATS). Some emergency cases may need to be managed by open thoracotomy to allow quick and thorough access to your full thoracic cavity.
• Drainage/debridement. In addition to the direct repair of the tear, any infected fluid collections in the cavity will need to be drained and sterilized, and any infected or necrotic (dead) tissue will need to be removed. In some severe cases, this may mean removing part of your esophagus.
• Advanced management. When surgery isn’t possible within 24 hours of rupture, direct repair may not work. The edges of the wound may have begun to stiffen or fray. In this case, your surgeon may need to remove part or all of your esophagus (esophagectomy). If you need an esophagus replacement, you can have one after six weeks.
• Alternative nutrition. While your esophagus is healing, you won’t be able to use it to swallow food, so you’ll need an alternative way of feeding. This might be by tube or by vein.

What is the prognosis for someone with Boerhaave’s syndrome?

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.