Boerhaave Syndrome Definition

Boerhaave syndrome (BS) is a spectrum of medical conditions where there is spontaneous rupture of the esophagus. This means that tears form through the wall of the esophagus, which is known as an esophageal perforation. Boerhaave syndrome is named after its discoverer, Dr. Hermann Boerhaave, a physician. Often the esophageal perforations occur as a complication associated with medical interventions. However, it is forceful vomiting which increases pressure within the esophagus that leads to the actual tear.

Boerhaave Syndrome Incidence

Boerhaave syndrome does not present with any defined symptoms and is considered a rare clinical condition, yet it accounts for 15% of esophageal ruptures. It is associated with high mortality rates and delays in diagnosis reduces the chances of survival in Boerhaave syndrome. If left unattended for more than 12 hours, a rupture may become life-threatening. Middle-aged adults are at a greater risk, especially where there is excessive alcohol consumption and food intake.

Boerhaave Syndrome Pathophysiology

The esophagus is a muscular tube that connects mouth to abdomen. The wall of esophagus is made up of different esophageal muscles. The constriction and relaxation of these muscles aid in proper movement of food across the tract and also support breathing. It occurs in a coordinated manner known as peristalsis. The movement down the esophagus is an involuntary phase of the swallowing process that starts at the back of the mouth.

There are two sphincters that regulate the passage of food and fluids through the esophagus. The upper esophageal sphincter at the top relaxes to allow food from the throat to enter the esophagus during swallowing. The lower esphageal sphincter (LES) opens as food reaches the bottom of the esophagus to allow it to pass into the stomach. These sphincters ensure that food only moves in one direction – from the throat to the stomach. It prevents the backward movement of food, fluid or partially digested contents by contracting forcefully.

Increased pressure in the esophagus

When there is an excess of food or alcohol consumption, the brain initiates antiperistaltic waves. This pushes contents forcefully backwards – from the stomach into the esophagus. It is simply known as vomiting. However, sometimes the esophageal sphincters, especially the upper esophageal sphincter, fails to relax. The contents being forced into the esophagus are therefore restricted by the sphincter and the pressure within the esophagus builds up.

If the walls of the esophagus are not able to withstand the high pressure, it can eventually rupture or tear. The contents within the esophagus can therefore spill into neighboring structures like the chest cavity or lungs. Most tears arise a few centimeters away from the abdomen on the left posterolateral side which is the back and left side of the esophageal wall.

Boerhaave Syndrome Causes

Most esophageal perforations are caused due to medical interventions (iatrogenic). The instruments entering the esophagus can damage the esophageal wall and subsequent violent vomiting eventually leads to a tear. However, overall the cause of Boerhaave syndrome is any excessive build up of pressure within the esophagus (intraluminal pressure) beyond the capacity of the wall to withstand it. It is therefore a pressure-dependent event.

Other less likely events that may cause an increase in intraluminal pressure include :

• Child birth
• Weight lifting
• Unstoppable fits of coughing or laughing
• Hiccups
• Seizures

However, even in this instance there has to be some major defect of the esophageal wall for a rupture to occur.

Risk factors

• Violent vomiting or retching induced by heavy food or alcohol intake.
• Swallowed pills or coins damage the esophagus walls could increase intra-esophageal pressure when it becomes impacted in a narrow zone.
• Intake of caustic toxins erodes esophagus walls and cause inflammation.
• Penetrating wounds from knife stabbing or gunshot.
• Intraoperative perforations occur during surgeries.
• Forceful swallowing or force feeding.
• Iatrogenic procedures rarely cause the actual perforation but weaken the wall and may even trigger vomiting which then causes the tear.

A person who has a history of esophageal disease, such as GERD, a hiatal hernia or Barett esophagus, is more likely to experience a perforation. Similarly alcoholism is another major risk factor.

Boerhaave Syndrome Symptoms

Early Symptoms

Boerhaave syndrome usually presents itself as a consequence of forceful vomiting or retching followed by severe chest pain. Therefore Boerhaave syndrome should be suspected when these events occur :

• Single forceful vomit or repeated episodes of vomiting or retching
• Excruciating chest pain that may spread from upper abdomen to shoulders
• Coughing upon swallowing due to non-coordinated activities of food and wind pipes
• Uncomfortable breathing episodes

Later Symptoms

Esophageal rupture releases its acidic content into chest that gets inflamed and infected (chest mediastinitis). Owing to this inflammation, fluid enters the pleural cavity, covering the lungs and contributes to appearance of further secondary symptoms including:

• Lower back pain
• Acute shoulder pain
• Discomfort while lying flat
• Acute abdominal distress

Boerhaave Syndrome Complications

In acute cases, Boerhaave syndrome may become morbid due to:

• Pneumonia
• Deposition of pus in the pleural covering of lungs (empyema)
• Ulcers
• Respiratory distress
• Polymicrobial infections (sepsis)
• Multi-organ failure

Since iatrogenic causes of Boerhaave syndrome are more common, appearance of these symptoms immediately after medical interventions may indicate esophageal perforations:

• Fever
• Pain in neck, abdomen or chest
• Difficulty swallowing (dysphagia)
• Pain when swallowing (odynophagia)
• Difficulty breathing (dyspnea)

Boerhaave Syndrome Diagnosis

Mackler triad

Although Boerhaave syndrome does not present with symptoms specific to the condition, the Mackler triad comprising of these signs is often useful in diagnosing the condition:

• Vomiting
• Chest pain
• Subcutaneous emphysema – air enters into the tissue under the skin, usually develops in chest and neck area, almost an hour after injury.

Physical examination

Initial physical examination may also reveal :

• Fast or irregular heart rate (tachycardia)
• Rapid breathing (tachypnea)
• Fever

Signs of complications

Owing to rupture of the esophagus and contamination of food and air pipes, air fills in most of the cavity surrounding the lungs and heart causing:

• Crackling sound with the heartbeat due to entry of air in between lungs (mediastinal emphysema)
• Reduced breathings sounds due to filling of pleural space

Tests

Detection of food particles or increased amylase with pH<6.0 (acidic) in the pleural fluids, is suggestive of an esophageal perforation.  Imaging studies like chest or neck x-rays or high contrast CT scan of chest help in diagnosing Boerhaave syndrome. Absence of esophageal bleeding helps in differentiating Boerhaave syndrome from similar esophageal tears like Mallory-Weiss Syndrome.

Boerhaave Syndrome Differential Diagnosis

Confirmation of any other disease showing symptoms common to Boerhaave syndrome, also excludes the possibility of esophagus tear:

• Myocardial infarction
• Pericarditis
• Pancreatitis
• Lung abscess
• Pneumothorax

Boerhaave Syndrome Death

Even with prompt diagnosis, Boerhaave syndrome remains the most fatal of all esophageal ruptures. There about a 72% mortality rate. Location of perforations also determines the risk of complications and death. Cervical perforations (closer to the neck) are less fatal when compared to thoracic (chest) or abdominal perforations.

Boerhaave Syndrome Treatment

Emergency medical attention is required if Boerhaave syndrome is suspected. Initial measures include intravenous (IV) fluid administration and oxygen therapy, along with pain medication if necessary. The spillage of esophageal content affects neighboring organs leading to a host of complications. Therefore, broad-spectrum antibiotics and fluids are given to overcome these secondary effects.

Surgically repairing the torn esophagus and draining the spilled contents from the surrounding areas are the main approaches. These procedures also include installing stents or drains to facilitate proper drainage of esophageal contents over time. Despite these measures, Boerhaave syndrome may still be fatal.