Boerhaave Syndrome - Causes, Symptoms, Diagnosis, and Management

What Is Boerhaave Syndrome?

Boerhaave syndrome is a serious condition of the gastrointestinal tract which is characterized by an increase in the pressure within the esophagus (the tube that connects the mouth to the stomach) due to strain by cough or forceful vomiting resulting in sudden bursting or rupturing of the esophagus. The rupturing of the esophagus leads to leakage of the toxic contents causing the infection. It is an emergency condition and needs urgent treatment as it can become fatal in a few days.

What Is the Prevalence of Boerhaave Syndrome?

Boerhaave syndrome accounts for almost 15 percent of all cases of esophageal rupture.

It is estimated to affect 0.0003 percent of the total population.

What Are the Causes of Boerhaave Syndrome?

Risk factors involved in Boerhaave syndrome are:

• The build-up of pressure within the esophagus due to excessive straining.

• Failure of cricopharyngeal muscle (muscles present in the upper portion of the esophagus or food pipe which opens and closes while swallowing) to relax resulting in the build-up of negative pressure outside the esophagus.

• Forceful or continuous vomiting.

• Straining while childbirth.

• Straining by weightlifting.

• Ingestion of any corrosive substance.

• Seizures (disorder in which nerve cell activity of the brain is hampered).

• Abdominal trauma.

• Excessive intake of alcohol.

What Are the Signs and Symptoms of Boerhaave Syndrome?

• Severe chest pain.

• Nausea and vomiting.

• Pain on swallowing.

• Shortness of breath.

• Fever.

• Excessive sweating.

• Tightness of the abdomen (belly).

• Swelling of the tissue of the chest cavity.

What Is the Classic Triad of Boerhaave Syndrome?

The Boerhaave syndrome typically manifests as Mackler's triad, which includes vomiting, chest pain, and subcutaneous emphysema (entrapment of air under the skin's tissues) as a result of an oesophageal rupture.

What Happens After the Rupture of the Esophagus?

The esophagus or food pipe is a part of the GIT (gastrointestinal tract) that carries chemicals and bacterial toxins that are not required by the body. Sudden rupture or bursting of the food pipe can lead to leakage of the food contents, chemicals, and toxins from the esophagus into the chest and abdomen, resulting in severe infections. This infection can further spread in the bloodstream. It can lead to septicemia (release of chemicals in the bloodstream) or septic shock, a life-threatening condition that can result in multiorgan failure and death.

How to Diagnose Boerhaave Syndrome?

Different diagnostic modalities of Boerhaave syndrome are:

• History and Clinical Examination: The patient's history of the frequency of vomiting, chest pain, intake of any corrosive material, and breathing difficulties is recorded. A clinical examination is done to rule out a tightening of the abdomen.

• Esophagogram: A radiograph using water-soluble contrast agents like gastrografin is performed to rule out any breach in the esophagus or food pipe. It is done by asking the patient to swallow the fluid containing the water-soluble contrast agent. The contrast dye highlights the complete esophagus from within and appears opaque on the radiograph. Any breach or tear in the esophagus will show leakage of the dye from the esophagus in the surrounding space, which can be easily detected in the radiograph. This test gives significant accuracy in the detection of Boerhaave syndrome.

• Computed Tomographic (CT) Scan: It is done to assess the other involved organs due to Boerhaave syndrome. It also detects esophageal wall thickening and the presence of fluid leaks in the chest and abdomen cavities so that treatment for it can be planned accordingly.

• Chest X-Ray: Chest X-ray helps detect signs of subcutaneous emphysema (entrapment of air under the skin's tissues) and pleural effusion (fluid build-up in the tissue lining the lungs and the chest).

• Endoscopic Examination: It is also one of the techniques used to examine the esophagus or food pipe for the presence of any rupture with the help of an endoscope (a thin, flexible tube-like apparatus with an attached camera used to see the internal body structures). The endoscopic examination should be done cautiously to prevent any further perforation in the esophagus. Examination with the help of a chest tube can also be done with the help of a methylene blue dye test. The patient is asked to swallow the sweetened methylene blue dye, and it gives bluish discoloration to the chest tube drainage within 12 to 24 hours when observed under X-Ray or computed tomography scans.

What Is the Treatment of Boerhaave Syndrome?

Treatment of the ruptured esophagus depends on the type, severity, and extent of the perforation. Different treatment methods are:

• Antibiotic Therapy: The patient is kept on antibiotics to control the infection.

• Rehydration Therapy: Intravenous fluids are started for the patient to recover the fluid volume loss from the body.

• Surgical Treatment: Surgical repair of the perforated esophagus should be done within 24 hours under the guidance of VATS (Video-Assisted Thoracoscopic Surgery).

• Endoscopic Endoluminal Vacuum Therapy (EVT): The leakage of the fluid content in the body cavities is drained, and any dead or necrotic tissue present is removed surgically. EVT (endoscopic endoluminal vacuum therapy) helps to clean the perforated cavity and drain the contents effectively. It also minimizes bacterial contamination and promotes esophageal healing.

• Esophagectomy: In cases of any delay in diagnosis when surgery cannot be performed within 24 hours, the affected part of the esophagus is removed surgically, followed by esophageal replacement after six weeks.

• Esophageal Stenting: Esophageal stenting by an endoscopic approach (under the guidance of an endoscope) is done to seal the perforated esophagus and prevent fistula (infected tract between two organs) formation in patients with delayed diagnosis.

Complications of Boerhaave Syndrome

• Swelling of the chest cavity.

• Formation of pus within the chest cavity (empyema).

• Pneumothorax (trapping of air between the chest wall and lungs).

• ARDS (acute respiratory distress syndrome).

• Entrapment of air under the tissues of the skin.

• Septicemia and septic shock syndrome (a life-threatening condition resulting in multiorgan failure and death).

Conclusion:

Boerhaave syndrome is a rare but life-threatening condition. Early diagnosis of the esophageal rupture is crucial for emergency management of the condition within 12 to 24 hours. Any delay in the diagnosis and management can lead to further complications and death due to septic shock.