Boerhaave Syndrome

What is Boerhaave Syndrome?

Boerhaave syndrome is a full- thickness perforation of the esophagus. It usually occurs spontaneously. Boerhaave syndrome can affect otherwise healthy individuals. Because of the sudden onset and difficulties of the diagnostic process, it is potentially lethal ^[1].

Causes

Boerhaave syndrome usually occurs when there is a sudden increase in the intra-esophageal pressure in combination with negative intrathoracic pressure. The pressure usually rises while vomiting, because of neuromuscular failure- the cryptopharyngeal muscle is not able to relax. The intense vomiting usually occurs after over-eating or using too much alcohol, or in other cases (see cyclic vomiting syndrome). Rupture of the esophagus is very dangerous because of its anatomical location ^[1,2].

Esophagus Anatomy and rupture location

Esophagus is a tube shaped, muscular organ that connects the throat to the stomach. Anatomically it is located behind the trachea and the heart. It has upper and lower sphincter. The upper sphincter (cryptopharyngeal muscle) is located just below the place where throat connects with esophagus. The lower sphincter is located where esophagus connects with the stomach.

Esophagus is made from muscle tissue. From inside it is covered with connective tissue- mucosa and submucosa, and from outside with a layer of connective tissue. The upper part of esophagus has striated and smooth muscle, but the lower two thirds consist only from smooth muscle. When food is swallowed, it is moved down to the stomach by peristaltic movements of the esophageal muscle.

The rupture usually occurs on the lower part of esophagus, around 2-3 cm proximally to the esophagus junction with stomach. The next most common place is just beneath the diaphragm ^[4].

Symptoms

Usually the onset of symptoms is after vomiting episodes. The classic patient is a middle aged man, who has started vomiting after consuming a lot of food and/or alcohol. When the rupture occurs, the patient feels severe pain in the chest that can irradiate to the left arm or back, similar to symptoms of heart attack or aortic dissection which is common in patients with Marfan syndrome. The pain usually gets worse when swallowing. Some patients might present with cough, due to connection with the pleural cavity. Patients might also complain about shortness of breath, which is usually caused by pleural effusion or pleuritic pain. Patients try to take shorter breaths with more frequency ^[1,3].

Boerhaave syndrome Vs Mallory-Weiss syndrome

In case of Boerhaave syndrome, the tear in the esophagus affects all tissue layers. The perforation causes the content of esophagus and the stomach to freely low in the chest cavity. Mallory-Weiss syndrome is longitudinal tears in the mucosa of esophagus. It is characterized by bleeding and vomiting of the blood. Mallory-Weiss syndrome also can occur after vomiting, or some medical procedures like trans-esophageal ehocardiography. Although Mallory-Weiss syndrome can cause many complications, it is not as life threatening as Boerhaave syndrome ^[5].

Diagnosis

Boerhaave syndrome can be diagnosed by physical examination of the patient and obtaining series of laboratory and imaging studies.

Physical examination

• The onset of symptoms is usually after vomiting
• Patient complains about serious pain in lower thoracic/upper abdominal region
• When palpating abdomen, pain increases.
• Subcutaneous emphysema (air under the skin) can be observed
• Rapid heartbeat and breathing
• In some cases, the abdomen is rigid
• Very low blood pressure and fever is common as the condition progresses.
• On auscultation the doctor might hear crackling noise in the upper part of the lungs, because there is air in the mediastinum.

In cases of atypical location, for example in case of cervical rupture, patient might feel pain in the neck or upper chest area, or in case the perforation is located in the middle part of esophagus, the pain might irradiate to the shoulder blade.

Laboratory studies

• Blood analysis usually are not specific to the condition
• Leukocytosis might be present
• Hematocrit level close to 50%
• Serum albumin is normal or slightly lowered, but globulin can be slightly increased

If thoracocenthesis is performed, particles of food, gastric juices and air can be found. If the food particles are not seen, they can be confirmed by microscopic examination of the fluid. Also saliva and epithelial cells can be found ^{[1, 3]}.

Imaging

Imaging studies can confirm the perforation of esophagus.

Chest X-ray typically reveals effusion on one side, usually on the left. Other visual findings are pneumothorax, hydropneumothorax, pneumomediastinum or mediastinal widening. In X-ray subcutaneous emphysema can also be seen. In around 10% of the cases chest X-ray is normal. That is because it might take time for the previously mentioned symptoms to appear.

Esophagography is a procedure in which a contrast fluid is given to the patient and following X-ray imaging is done. In this case the contrast fluid goes in the pleural cavity, because the esophagus is perforated. This procedure is helpful in order to determine the precise location of the tear, which can aid to choose the surgical approach. Barium contrast is usually used to diagnose other conditions, for example Precordial catch syndrome, but in Boerhaave syndrome it is associated with severe mediastinitis, therefore Gastrografin (water-soluble iodinated contrast) indicated for use ^[6].

CT scanning is beneficial for patients who are not able to tolerate esophagogram. It gives a better image of localization of the fluid in the thoracic cavity, but it doesn´t always provide precise localization of the perforation. Also, other anatomical structures can be seen, and it helps to exclude other possible conditions, like afferent loop syndrome ^[1].

Treatment

Surgical repair of esophageal tear

The treatment of Boerhaave syndrome is usually surgical reparation of the esophageal tear. The patient is usually given broad spectrum antibiotics and intravenous fluids.

Esophagus can be approached either from thorax or abdomen. The standard treatment involves direct repair of the perforation and drainage of the thoracic cavity.

Non-surgical treatment

There are some criteria for when non-surgical treatment is applicable:

• The rupture is well contained in the mediastinum
• The cavity drains back to esophagus
• Symptoms are minor
• No or minimal symptoms of sepsis

Non surgical treatment includes the use of antibiotics, intra-venous fluids and nutritional supplementation (treatment also used in Blind loop syndrome), suction through a naso-gastric tube, drainage of thoracic cavity ^[1].

Prognosis

Boerhaave syndrome is very rare- the incidence is 3 cases per 1 million people per year. It is more common in males than in females.

If the diagnosis is made in the first 24 hours after the rupture, survival rate is 75%. Mortality rate increases the longer diagnosis has not been made, reaching only 10% survival after 48 hours ^[3].

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References

1. Information about causes, diagnostics and treatment: http://emedicine.medscape.com/article/171683-overview
2. Information about Boerhaave syndrome and diagnostics: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3589863/
3. Information about causes, diagnostics and treatment: http://www.uptodate.com/contents/boerhaave-syndrome-effort-rupture-of-the-esophagus
4. Anatomy of the esophagus: https://www.merckmanuals.com/home/digestive-disorders/esophageal-and-swallowing-disorders/overview-of-the-esophagus
5. Information about Mallory-Weiss syndrome: http://emedicine.medscape.com/article/187134-overview
6. https://www.drugs.com/pro/gastrografin.html