Boerhaave syndrome is a rupture (tear) in the wall of the esophagus. It usually results from an increase in pressure caused by severe vomiting. Mortality is more than 90 percent if the perforation is not treated promptly.
What is Boerhaave syndrome?
Boerhaave syndrome should be distinguished from Mallory-Weiss syndrome. In the latter, perforation of the mucosa between the esophagus and stomach occurs, as well as traumatically triggered esophageal perforations (esophageal ruptures). Boerhaave’s disease, on the other hand, is confined to the esophagus itself and penetrates all wall layers of the esophagus. Rupture occurs spontaneously and requires immediate surgical treatment. The condition is very rare and there is little epidemiologic data. Approximately 10 to 15 percent of all esophageal perforations are due to the syndrome, although only about 900 cases had been reported in the literature worldwide by 1990. Men are more commonly affected than women, which has been attributed to a higher proportion of alcohol-dependent men. Boerhaave syndrome affected men in more than 80 percent of known cases. Rarely, the disorder also occurs in children, and on average, it primarily affects individuals between the ages of 20 and 40.
Causes
The cause of Boerhaave syndrome is a sudden, sharp increase in pressure in the trachea. At the same time, a negative pressure develops in the chest, called intrathoracic pressure. Conjecture suggests that the magnitude of the pressure increase is less important than the rate at which the pressure increases. This is due to studies on cadavers. These revealed perforation of the lower esophagus in the distal third with a sudden increase in pressure between 150 and 200 mmHG. Over 90 percent of ruptures are in the lower third left posteriorly. The reason for this is the anatomically determined low muscular resistance. In most cases, the perforation is triggered by massive vomiting, which earned it the name emetogenic esophageal perforation. In most cases, this vomiting is triggered by excessive alcohol intake that the body, and especially the stomach, can no longer handle. Other causes can be strong physical tension or excessive pressing, as shown in the literature. However, a large number of diseases can also trigger spontaneous rupture of the esophagus. These include gastroesophageal reflux disease or esophagitis. The former is a condition in which gastric secretions flow back into the esophagus.
Symptoms, complaints, and signs
Boerhaave syndrome has a very typical set of symptoms (Mackler triad). Excessive vomiting is followed by severe pain. These are referred to as annihilation pain. In addition, either skin emphysema or mediastinal emphysema may occur. This is a rising or spreading accumulation of gas under the subcutaneous tissue or in the mediastinal pleura. In addition, other signs may indicate Boerhaave’s syndrome. On the one hand, there may be signs of shock, such as a drop in blood pressure and cold sweat. On the other hand, many affected individuals suffer from shortness of breath (dyspnea) and lack of oxygen (cyanosis). Vomiting of blood, which is called hematemesis, is also possible.
Diagnosis and course
If a rupture of the esophagus is suspected, an ambulance should be called immediately. Diagnosis is made after first-aid measures via radiographs. Images show crescents of air under the domes of the diaphragm. In addition, air leakage into the mediastinum may be visible. Other tests that a medical professional may initiate include esophagography and esophagoscopy. The former involves a contrast agent examination. In this procedure, the contrast material enters the mediastinum if there is a perforation. This examination is considered to be lower risk. An esophagoscopy, on the other hand, is an endoscopic examination of the esophagus. Depending on the case, the tear can also be sutured. However, this method can lead to complications and further tearing of the rupture. Boerhaave’s syndrome must be differentiated from acute pancreatitis, ulcer perforation, and myocardial infarction, among others. In addition, the symptoms resemble pneumothorax, in which air in the pleural space obstructs expansion of the lungs.In addition, a differential diagnosis should be made to rule out aortic dissection. In this case, internal vessel walls tear, which in turn leads to bleeding and splitting of the wall layers of the aorta. To rule out a heart attack, it is also useful to initiate an ECG. In addition, a computer tomography is performed. It is not possible to make a diagnosis by pure, external observation of the patient.
Complications
In Boerhaave syndrome, death occurs in a very large number of cases if treatment is not given promptly. Usually, Boerhaave syndrome occurs after or during vomiting, after which extremely severe pain is experienced by the patient. In many cases, this pain leads to unconsciousness. The patient suffers from a sharp drop in blood pressure, with damage to the heart and other organs. Panic attacks and the development of the so-called cold sweat also occur. Most patients show gasping for breath. If vomiting continues, blood may also be vomited in the process. If the emergency physician cannot treat the patient immediately, death will result. The treatment of Boerhaave’s syndrome is surgical. It leads to success if it is initiated immediately after onset and without delays. In most cases, the affected person still has to take antibiotics after treatment to ward off inflammation and infection. This is where complications can arise if hygiene is poor or if the medication is not taken. People who suffer from alcohol dependence are more affected by Boerhaave syndrome.
When should you see a doctor?
Immediate treatment is needed for Boerhaave syndrome. If this complaint is not treated immediately, the patient usually dies. In the case of Boerhaave syndrome, call an emergency physician or go to the hospital immediately. Options for self-help are unfortunately not available to the affected person. The emergency physician must be consulted if there is extremely severe pain after vomiting. This pain is described by patients as excruciating pain. Gas may also accumulate under the skin, which is also indicative of Boerhaave’s syndrome. An emergency physician must also be called if the patient suffers from difficulty breathing or cold sweats. Blue discoloration of the skin and lips may also indicate the syndrome and must be treated immediately. In many cases, however, patients also lose consciousness. As a rule, the emergency physician must be called in the event of Boerhaave syndrome. The sooner this arrives, the higher the patient’s likelihood of survival.
Treatment and therapy
Treatment is via thoracotomy or laparoscopy, in which the rupture is sutured. Thoracotomy involves surgically opening the chest through an incision in the intercostal space. This should be done within 24 hours of the rupture. Laparoscopy (abdominal endoscopy) involves procedures within the abdominal cavity. In some circumstances, Boerhaave’s syndrome is covered plastically with surrounding tissue. Thus, the suture is appropriately stabilized with the body’s own tissue. After the operation, an accompanying therapy with antibiotics is necessary, as there is a risk of infection. In addition, the patient must also remain under intensive medical observation for some time. The mortality rate (lethality) for the syndrome is between 20 and 40 percent.
Outlook and prognosis
Boerhaave syndrome is an extremely severe disease that is always fatal if left untreated. If treatment is started immediately, the lethality decreases. It is then still 20 to 40 percent. The healing process is also influenced by the possible complications. Even the usual symptoms of the disease, such as shortness of breath, circulatory shock or vomiting blood, can quickly lead to death. Death can occur by bleeding to death, by cardiac arrest or by the undersupply of oxygen to the organism and especially to the brain. The danger is even greater if, in addition, such complications as mediastinitis or sepsis occur. The fastest possible surgery helps to stop bleeding and stabilize the circulation. The patient’s further recovery now depends on how severely he is already impaired by the undersupply of oxygen to the body.Furthermore, immediate treatment with antibiotics is necessary to avert a bacterial infection with all its complications (sepsis, mediastinitis). Both sepsis and mediastinitis may result in death due to multiple organ failure. The only way to avoid a fatal course of the disease is to surgically open the thorax (thoracotomy) or open the abdominal cavity (laparotomy) to close the tear in the esophagus, with concomitant antibiotic treatment with broad-spectrum antibiotics.
Prevention
Boerhaave syndrome is extremely rare, as mentioned earlier. However, preventive measures are not known. However, there are risk groups in which the syndrome occurs more frequently. These include, in particular, individuals who suffer from severe alcohol problems.
Follow-up
Follow-up care for Boerhaave syndrome depends on the course of the syndrome and any complications during treatment. If the tear in the esophagus is detected and treated early, the prognosis is generally good. Follow-up care focuses on regular check-ups by the physician. Among other things, the physician will perform ultrasound examinations and check the wound healing in the esophagus. During a patient interview, any accompanying symptoms will be discussed and the physician may prescribe appropriate medications. In addition, the rare syndrome requires that the esophagus be spared. This is achieved by an adapted diet, which, however, must be regularly adjusted to the progressively improving state of health. If the injury has been treated surgically, the patient must initially stay in the hospital for a few days. Provided that no further complications arise, the patient can leave the hospital after a week. Before that, the doctor will perform a final examination and give the patient general tips regarding hygiene, diet and stress on the esophagus. One week after the patient has left the hospital, the otolaryngologist should perform another follow-up examination, primarily checking for wound healing and adjusting medication if necessary.
What you can do yourself
Boerhaave syndrome is treated with medication and surgery. Supportive dietary measures and sparing are recommended as means of self-help. The patient should avoid physical exertion, especially in the days after surgery. Any surgical wounds must be cared for according to the physician’s instructions. If a wound opens or becomes infected, a visit to the doctor is recommended. If pancreatitis is part of the condition, regular hydration must be maintained in addition to a healthy and balanced diet. The painkillers prescribed by the doctor can be supplemented with remedies from natural medicine in consultation with the doctor. For acute symptoms such as nausea and vomiting, warm compresses or compresses with ointments are recommended. Pancreatitis should heal within one to two weeks if the patient takes it easy and supports recovery with the above-mentioned measures. If signs of a heart attack appear, the ambulance service must be called. The ill person should be placed in a quiet position until medical help arrives. Any first responders must reassure the sufferer and, in the event of cardiac arrest, initiate resuscitative measures such as chest compressions. Ulcer perforation or aortic dissection are also medical emergencies for which emergency medical services must be called.
