What is Boerhaave Syndrome?
Boerhaave syndrome generally happens after intense vomiting. It’s a complete tear in the esophagus – the tube that connects your mouth to your stomach. This condition is different from Mallory-Weiss syndrome, in which the tear in the esophagus is not complete. Also, while Boerhaave syndrome is commonly caused by vomiting, it can also occur from any activity that increases pressure inside the esophagus. Boerhaave syndrome can happen to anyone, even if their esophagus is normal, but there is a specific group with certain abnormalities of the esophagus who are more likely to get this condition. About 10% to 15% of all esophagus tears are due to Boerhaave syndrome.
Diagnosing this condition could be complex because the symptoms can differ widely among patients. Traditionally, Boerhaave syndrome has been linked to a trio of symptoms: vomiting, chest pain, and air trapped under the skin. However, patients don’t always show all these symptoms and sometimes may have vague complaints that aren’t very specific. This can cause delays in diagnosing the syndrome and lead to worse health outcomes. Boerhaave syndrome is one of the most dangerous diseases of the gastrointestinal tract – the system that processes food. The mortality rate, or the risk of death, is as high as 60% even with treatment, and without treatment, it nearly reaches 100%.
The type of treatment used depends on when the syndrome is diagnosed and the patient’s health condition at the time they first had symptoms. The treatment approach can range from simply giving the body a chance to heal itself to performing major surgery to remove the affected part of the esophagus.
What Causes Boerhaave Syndrome?
Boerhaave syndrome is a type of injury that is caused by a sudden increase in pressure within the esophagus, the tube that connects your throat to your stomach. This pressure increase typically happens when a muscle near the top of the throat, called the cricopharyngeus, doesn’t relax properly. This builds up intense pressure, and eventually, it causes the wall of the esophagus to tear apart at its weakest point.
In adults, this tear usually happens in the lower part of the esophagus on the left side, just beneath the diaphragm, which is a muscle that helps us breathe. In very young patients, the tear generally occurs on the right side and can reach into the body cavity near the lung.
Certain things increase the risk of Boerhaave syndrome, like alcohol misuse and eating too much. Both can potentially lead to violent vomiting, which is usually the most common cause. Other activities that increase pressure in the esophagus can also cause this condition, including weightlifting, straining while going to the bathroom, having a seizure, experiencing stomach trauma, suffering injuries from pressurized air, or giving birth. Although it’s most common in people with a healthy esophagus, this condition can also occur in individuals with inflammation or ulcers in the esophagus. Currently, there’s no evidence that indicates a genetic risk for developing Boerhaave syndrome.
Risk Factors and Frequency for Boerhaave Syndrome
Boerhaave syndrome is a type of traumatic esophageal rupture, making up roughly 15% of these kinds of cases. It’s estimated that this condition happens to about 3.1 out of every 1,000,000 people each year globally, but this number may be underestimated due to not all cases being reported. Boerhaave syndrome can happen to anyone, regardless of race, but it mostly affects males. The ratio of males to females with this condition ranges from 2:1 to 5:1.
- Boerhaave syndrome is particularly common in men in their sixties and seventies.
- However, it has been observed in everyone from newborn babies to individuals over 90 years old.
- Children aged 1 to 17 years seem to be the least affected by this condition.
Signs and Symptoms of Boerhaave Syndrome
Boerhaave syndrome is a condition that can affect people of all ages, ethnicities, and genders, not just middle-aged men after binge eating or drinking, as traditionally believed. Its symptoms vary widely due to the esophagus’s location in different bodily spaces like the neck, chest, and abdomen. It’s also important to remember that up to 45% of patients might not have a history of vomiting when they come in for a checkup.
Timing plays a big part in how this condition presents itself. Depending on how long it’s been since the initial injury and how widespread the leakage is, patients can experience a wide range of symptoms. Both how sudden the affliction strikes and what particular ailments they suffer from will be dictated by these factors.
- Sudden chest, neck, and abdominal pain
- Difficulty or pain during swallowing (odynophagia and dysphagia)
- Changes in voice such as hoarseness and dysphonia
- Vomiting
- Vomiting blood (hematemesis)
- Breathing problems
- Physical signs like a crackling sensation under the skin (subcutaneous crepitation), a crunching sound in sync with the heartbeat when lying on the left side (Hamman sign), fever, low blood pressure, rapid heart rate, rapid breathing, bluish skin or lips (cyanosis), reduced breath sounds, stomach pain, stiff abdomen, neck pain, shift in wind pipe location, bulging eyes (proptosis), and mental confusion.
Testing for Boerhaave Syndrome
The main steps in evaluating a person’s condition include a detailed physical examination and imaging tests. In a physical examination, the doctor assesses your physical wellbeing to determine how severe the condition is.
Imaging tests are very important when diagnosing Boerhaave syndrome, a potentially life-threatening condition that involves a rupture in your esophagus. Simple x-rays of your chest and abdomen can show signs of this syndrome, such as the presence of air under the skin or within the mediastinum (the area between the lungs), widening of the mediastinum, and fluid around the lungs. In some cases, these X-rays might also show a specific sign of an esophageal tear known as the Nacleario V-sign. However, this isn’t always present.
Lab tests, while often not specific enough to confirm the diagnosis, can show increased white blood cells pointing to infection and dehydration. Other significant findings may include the presence of undigested food particles from the digestive tract in potentially leaked fluid in the chest cavity, a low pH level indicating the presence of stomach acid, and an increased level of a digestive enzyme called salivary amylase.
To get a clearer view of the esophagus, a contrast esophagogram may be performed. This is a special X-ray test where you first swallow a special dye that makes the esophagus visible on the X-ray scan. It’s crucial to use a safe dye for this test since leakage of some agents can cause inflammation and scarring in the tissue surrounding the esophagus. However, sometimes this test might not detect the esophageal rupture, especially if the tear is small or in a tricky location. If the initial test doesn’t show anything, a repeat test might be done with a different contrast agent.
A CT scan can provide a more precise diagnosis, especially in very sick patients, as it offers more detailed images and can highlight the exact location of the rupture and any related complications. When used along with a contrast medium, the CT scan can outline the extent of the injury and help speed up the diagnosis. Common findings on a CT scan for Boerhaave syndrome include air or fluid collections around the esophagus, thickening of the esophageal wall, and fluid or air in the chest cavity.
Endoscopy, which involves inserting a small camera through your mouth to see the inside of your esophagus, might be considered useful but is risky as it could further tear the esophagus. So, it is typically used only when the location of the perforation is hard to determine, and the patient is a good candidate for endoscopic treatment.
Treatment Options for Boerhaave Syndrome
Boerhaave syndrome, a medical condition where the esophagus bursts or tears, can be managed in three ways – nonsurgical, endoscopic (using a special instrument to view and work inside the body), and surgical (either a major open surgery or a less invasive surgery). It is crucial to have a team of health care professionals decide on the best treatment option, as often a combination of medical and surgical interventions will be used depending on the unique circumstances of the patient.
Key parts of the treatment include not eating or drinking by mouth, ensuring adequate fluids, giving a wide range of antibiotics, providing nutritional support (often directly into the vein), controlling any leaks (via IR or VATS – medical procedures used to diagnose and treat conditions in your chest), and performing surgery or endoscopy when necessary. The treatment plan is usually tailored based on where and how big the tear in the esophagus is, how long ago the injury happened, and the overall health of the patient. The earlier the condition is diagnosed (within 12 to 24 hours), the better the outcome is likely to be.
Certain patients might be considered suitable for nonsurgical treatment. This includes those whose leak is restricted to certain areas, who can have contrast (a type of dye used in medical imaging) flow back into the esophagus from the surrounding area, whose injury is not in cancerous tissue or certain other areas, and who have minimal symptoms without signs of a severe infection called sepsis. These patients must also be able to get special imaging tests at any time and have surgical specialists available without delay if their condition worsens.
Nonsurgical treatment involves not eating or drinking by mouth for at least seven days, getting nutrition through the veins, getting antibiotics directly into the vein for 7 to 14 days, and draining any build-up of fluid. If patients start to worsen during nonsurgical treatment, surgery is required. Specific signs indicating the need for surgery include having the tear get larger or lead to a severe type of rupture, ongoing fevers, worsening condition including the development of sepsis, increased air in the chest area outside the lungs, or the development of an infected collection of pus.
Surgical repair of the esophagus, particularly within the first four hours of the tear, is the most successful method of treatment. This can be done either through a large incision (open thoracotomy) or a less invasive method (VATS) with the support of the upper portion of the stomach, which is considered the gold standard of treatment.
In cases where a section of the esophagus is too damaged, it may need to be removed. If the tear is diagnosed after 24 hours, the tissue around the wound is often swollen, stiff, or easily breakable, making a primary repair risky. In these cases, many manage late tears with cleaning out the pleural cavity (the space between the two layers of tissue that line the lungs) and mediastinum (the area between the lungs), making an opening into the esophagus, and making a feeding opening into the stomach. The definitive rebuilding is often done after about six weeks.
While surgery is the most common treatment of Boerhaave syndrome, in some cases, using a stent (a small tube) to bridge the tear has shown promising results. This could be an option for patients with serious other health conditions. Advanced endoscopic techniques can offer patients a less invasive therapeutic option. These endoscopic approaches to manage an esophageal tear include placing fully covered esophageal stents, using a variety of clips, and doing endoscopic suturing, esophageal resection (removal), and diversion (altered routing of body fluids).
What else can Boerhaave Syndrome be?
Boerhaave syndrome has symptoms that can be mistaken for a range of other conditions. These include:
- Aortic dissection
- Pancreatitis
- Heart attack
- Pulmonary embolus (or blood clot in the lung)
- Perforated peptic ulcer
- Spontaneous pneumothorax (collapsed lung)
- Pneumonia
- Pericarditis (inflammation of the lining around the heart)
- Mallory-Weiss tear (a tear in the esophagus)
Physicians can tell Boerhaave syndrome apart from these conditions by taking a patient’s history, doing physical exams, conducting lab tests, evaluating EKG results, and looking at images taken of the body’s interior.
What to expect with Boerhaave Syndrome
The chances of recovery often hinge on how quickly an injury is diagnosed and treated. The longer the delay in diagnosis and treatment, the worse the outcome can be, and it could even increase the risk of death. If an injury is diagnosed and treated properly within 12 to 24 hours, patients generally have a good chance of recovery, with survival rates up to 75%. However, if a condition called Boerhaave syndrome is not treated, the risk of death rises to over 90%.
Possible Complications When Diagnosed with Boerhaave Syndrome
Boerhaave syndrome is a rare condition that can often be overlooked or diagnosed late, which can lead to serious complications. These can include severe dehydration, infection in the chest, blood poisoning, a type of severe lung injury called acute respiratory distress syndrome (ARDS), an abnormal buildup of fluid in the lungs, an abnormal connection in the esophagus, pus-filled infection in the cavity between the lungs and chest wall, shock, and even death.
Potential Complications:
- Severe dehydration
- Infection in the chest (mediastinitis)
- Blood poisoning (sepsis)
- Severe lung injury (ARDS)
- Abnormal buildup of fluid in the lungs
- An abnormal connection in the esophagus
- Pus-filled infection in the cavity (empyema)
- Shock
- Potential death
Recovery from Boerhaave Syndrome
The healing process after an esophageal rupture, a tear in the esophagus, largely depends on how severe the tear is, the treatment given, and how much it has affected the body’s system. The path to recovery may include gradually starting to eat again, bringing body systems back to normal, reducing reliance on intense treatments like mechanical breathing assistance and continuous health monitoring, and working towards rebuilding strength and stamina. This is all done with the goal of enabling patients, when possible, to return to their normal everyday activities independently.
Preventing Boerhaave Syndrome
Boerhaave syndrome is not very common and there are no specific criteria to predict who might get it. Individuals who frequently consume too much alcohol or eat excessively should be made aware of this condition. They should know that any sudden chest, neck, or stomach pain accompanied by violent vomiting when indulging in these behaviors, is a serious matter and they should seek immediate medical attention. For those diagnosed with Boerhaave syndrome, regular follow-ups with their stomach specialists (gastroenterologists) and their primary healthcare provider would be necessary.
