Boerhaave’s Syndrome – Know About This Rare Condition

Overview

The Boerhaave’s syndrome is a spontaneous perforation of the esophagus that usually affects the lower one-third of the food pipe. The condition was first described by a Dutch physician, Hermann Boerhaave in 1724. It is also known as ‘spontaneous esophageal rupture’. However, it is not truly spontaneous as it typically results from violent vomiting or retching. The word ‘spontaneous’ here denotes that the perforation is not caused by any foreign body or direct trauma.

It is a relatively rare condition. It is also likely to be under-reported as most of the cases are misdiagnosed or diagnosed only post-mortem. This is because the condition rarely presents with the classical picture. Most of the time, the patient seems like suffering from an abdominal or cardiac pathology while the real problem is in his food pipe.

Causes

The Boerhaave’s syndrome is often reported to be associated with overindulgence in food and alcohol. Such an activity is followed by violent vomiting against a closed glottis. This causes a sudden increase in the esophageal pressure and results in rupture.

Symptoms and presentations

The classical symptoms of the Boerhaave’s syndrome are:

  1. Vomiting
  2. Lower chest pain
  3. Subcutaneous emphysema – collection of air under the skin, especially the skin that covers the chest and neck.

This is called the ‘Mackler’s triad‘. But in most of the cases, the presentation is not classical.

A patient who presents with a history of overindulgence in food or alcohol followed by vomiting, severe chest pain, breathlessness and shock is likely to have a spontaneous esophageal rupture.

Other reported unusual presentations are changes in voice, extreme facial and neck swelling and thirst.

Complications

  1. During the later course of the disease, the patient can also present with infection of the mediastinum. This is the middle part of the chest cavity and it is prone to infection when any organ in this part of the chest cavity gets injured. The infection can also become full-fledged and spread to the blood causing sepsis.
  2. Pericarditis can occur. This is nothing but an infection of the pericardium which is the outermost covering of the heart.
  3. Air can get collected under the pericardium leading to a condition called pneumopericardium.
  4. Another possible complication is pneumoperitoneum, where air gets collected in the abdominal cavity.

These are rare but possible complications of this condition. Such unspecific presentations and complications are the main causes of delay in delivering relevant medical care to the patients. Hence the mortality rate is very high ranging from 20-40%. Spontaneous esophageal rupture is the most lethal gastrointestinal perforation.

Other conditions that mimic the Boerhaave’s syndrome

  1. The Boerhaave’s syndrome is commonly confused with peptic ulcer. But in this syndrome, a history of peptic ulcer and the usual symptoms of ulcer like heart burns and indigestion are absent.
  2. It is also common to mistake this condition for a heart attack. The crucial point is to differentiate the chest pain due to this syndrome from that of a heart attack because both the conditions need immediate medical intervention, but in entirely different ways.
  3. The third most common condition mimicking the Boerhaave’s syndrome is acute pancreatitis. This pancreatic disorder is also associated with alcohol consumption, vomiting and abdominal pain. Serum amylase is an enzyme secreted by the liver and the level of this enzyme is elevated in case of acute pancreatitis. This laboratory test differentiates acute pancreatitis from the Boerhaave’s syndrome.

Diagnosis

When a doctor suspects a spontaneous esophageal rupture in a patient, he demands for an erect chest X-ray film. A barium contrast study is also usually advised regardless of the inferences from an X-ray film. This radiological test can show where the perforation is located. If a perforation is present the radio-opaque barium ingested by the patient leaks out of the esophagus into the chest cavity. It can then be captured by X-ray films. If contrast studies are negative despite strong suspicion, the doctor then advises to perform a chest CT.

Management

The preferred treatment for spontaneous esophageal rupture is a surgical repair of the rupture. But the choice of the treatment and method of operative repair depends on various factors like:

  1. The size of the
  2. The site of the rupture
  3. Age of the patient
  4. General health condition of the patient
  5. The time lapsed from perforation to diagnosis.

Prolonged gastric (stomach) drainage and chest drainage with tubes may also be necessary. Survival rate and response to treatment depend mainly on early diagnosis and the appropriateness of the intervention.