Boerhaave's Syndrome: Treatment & Management, Diagnosis, Pathophysiology, Occurrence Rate
What is Boerhaave's Syndrome?
Boerhaave's syndrome is spontaneous esophageal perforation following forceful vomiting. In simple language Boerhaave syndrome's is an esophageal pathology. This syndrome is described as esophageal rupture too.
Boerhaave's syndrome can be morbid if not treated on time and appropriately, which means, if it occurs and appropriate treatment is not given on time, it is fraught with early complications, leading to a very high mortality rate.
Boerhaave syndrome's is a transmural perforation of the esophagus, which distinguishes it from Mallory-Weiss syndrome, which is a nontransmural esophageal tear with similar symptoms. Boerhaave's is uncommon in occurrence
Diagnosis of Boerhaave's Syndrome
Boerhaave's syndrome can be difficult to diagnose for the fact that most of the times no classic symptoms are present and delays in presentation for medical care are common. Roughly, one third of all cases of Boerhaave's syndrome are clinically atypical. Prompt recognition of this potentially lethal condition is vital to ensure appropriate treatment. Symptoms like Mediastinitis, sepsis, and shock frequently are seen late in the course of illness, which further confuses the diagnostic picture. Other symptoms that masquerade Boerhaave's syndrome are acute myocardial infarction, acute aortic dissection, tension pneumothorax or pneumoperitoneum, and this exactly accounts for the high mortality rate of 20–75% if appropriate treatment is not started on time. And if Boerhaave's syndrome is left untreated, the mortality reaches 100%.
X-ray is the most valuable diagnostic modality for this syndrome and the simple erect film of the chest which is rapidly obtainable yields the most information. The most common error in the diagnostic work-up is the failure to obtain a chest x-ray when a Boerhaave's syndrome presents as an "acute abdomen."
An urgent water-soluble contrast swallow and/or CT scan with oral contrast is another diagnostic tool in diagnosis of Boerhaave's syndrome.
CT scan is useful in patients too ill for contrast studies. It can localize fluid collections and perioesophageal air tracks suggestive of perforation but it cannot precisely localize the site of the perforation.
Occurrence Rate of Boerhaave's Syndrome
Esophageal perforations are rare, with an incidence of 3.1 per 1,000,000 per year. However, the most consistent feature in most cases is over indulgence in food and/or alcohol, followed by vomiting, then sudden onset of severe left sided chest pain. The pain occasionally radiates to the left shoulder giving the false impression of acute myocardial infarction.
In fact, Boerhaave's syndrome and acute myocardial infarction have a lot in common regarding the clinical presentation. In most cases, acute myocardial infarction is actually the first diagnosis considered. The common features are the age range (about 50 –70 years), the gender (male), the sudden nature of the pain (left sided chest pain), and the radiation to the left shoulder.
Pathophysiology of Boerhaave's Syndrome
Pathophysiologically, Boerhaave's syndrome occurs due to a sudden rise in intraluminal oesophageal pressure during vomiting, in the presence of a closed glottis. However there are several other reasons which can cause rupture of esophageal wall. Vomiting is the most frequent cause of Boerhaave's syndrome, but it is certainly not the only cause and this has led to some debate as to the name of the syndrome.
It has been reported with such diverse etiologies as straining, weight lifting, severe coughing, childbirth, blunt trauma, seizures, Cushing's ulcers due to central nervous system disease, seasickness, postoperative vomiting, esophagitis with perforated ulcer, asthma, smooth muscle hypertrophy of the esophagus, and the drug Antabuse (drug given to alcoholics attempting to reform, causing violent vomiting if alcohol is ingested while on this drug).
Age is an important prerequisite factor for this neuromuscular incoordination occurring in Boerhaave's syndrome. Anatomically, the tear is located at the left posterolateral wall in the lower third of the oesophagus (in the chest). The second commonest site of rupture is subdiaphragmatic, leading to abdominal symptoms.
Treatment & Management of Boerhaave Syndrome
A combination of both conservative and surgical interventions are considered ideal for treating Boerhaave's syndrome.
Foundation of therapy for Boerhaave's syndrome includes the following:
- Intravenous volume resuscitation
- Administration of broad-spectrum antibiotics
- Prompt surgical intervention
The decision to use a conservative (medical intervention only) or an aggressive (medical plus surgical intervention) approach for treating
Boerhaave's syndrome depends on the following factors:
- Time delay in presentation and diagnosis
- Extent of perforation
- Overall medical condition of the patient
Surgical intervention for Boerhaave's syndrome is the standard of care in most cases. The criteria in which conservative (nonsurgical) management might be appropriate include the following:
- The esophageal disruption should be well contained in the mediastinum.
- The cavity should be well drained back into the esophagus.
- Minimal symptoms should be present.
- Evidence of clinical sepsis should be minimal.
Conservative management of Boerhaave's syndrome consists of the following:
- Instituting intravenous fluids.
- Antibiotics with good broad-spectrum coverage like Imipenem/cilastatin (Primaxin).
- Application of nasogastric suction.
- Keeping the patient NPO.
- Adequate drainage with tube thoracostomy or formal thoracotomy is vital.
Early use of nutritional supplementation: Evidence suggests that for hastening recovery, a jejunostomy tube feeding may be favored over hyperalimentation.
The best outcomes in Boerhaave's syndrome are associated with early referral and surgical management in a specialist centre. Surgery appears to be superior to conservative treatment for patients referred late.
Nevertheless, a lack of consciousness of the disease, a strong tendency to diagnose peptic ulcer, pancreatitis or myocardial infarction, and a failure to recognize the importance of a left pleural effusion in a patient with a history of recent vomiting, too often leads to a delay that exceeds the critical first 12 hours of the disease.
A constant awareness of the possibility of esophageal rupture plus the knowledge that surgery is necessary and must be prompt should improve upon the formidable mortality and morbidity of Boerhaave's syndrome.