Esophageal rupture, or Boerhaave syndrome, is a rupture in the esophageal wall. Medically induced ruptures account for approximately 56% of esophageal perforations and are usually caused by endoscopy or esophageal surgery. Other causes include vomiting, which accounts for 10% of esophageal perforations, also known as Borhave syndrome. Spontaneous esophageal perforation usually results from a sudden increase in esophageal pressure combined with relative negative thoracic pressure, which can occur during vigorous vomiting.
"Spontaneous perforation may often be unrelated to preexisting disease of the esophagus, but it is associated with an increased risk of death."
The rupture in Borhave syndrome usually occurs in the left posterolateral part of the lower third of the esophagus and extends for several centimeters. This condition has very high morbidity and mortality rates and is almost always fatal if not treated promptly. Because symptoms are sometimes nonspecific, diagnosis may be delayed, leading to adverse outcomes.
Classic symptoms of esophageal rupture include severe vomiting followed by severe pain behind the sternum and in the upper abdomen. Symptoms that may quickly follow include odynophagia, shortness of breath, dyspnea, cyanosis, fever, and shock. Although the physical examination is usually not very helpful, subcutaneous emphysema is an important diagnostic finding. Additionally, in some cases, chest pain may radiate to the left shoulder, misleading doctors into thinking it is a myocardial infarction. It is worth noting that the Macckler Trinity refers to the combination of chest pain, vomiting, and subcutaneous emphysema, but in fact only 14% of people will show this typical pattern.
Pathophysiology"Upon examination, abnormalities consistent with Borjave syndrome are almost always found on X-rays."
In Borhave syndrome, the esophagus ruptures because of a sudden increase in internal pressure caused by vomiting, which is usually related to a neuromuscular disorder that prevents the circular muscle in the upper part of the esophagus, the epiglottis, from relaxing. As the pressure in the esophagus increases, food in the esophagus cannot move upward, eventually leading to a rupture. The syndrome is often associated with overeating, alcohol abuse, or eating disorders, especially anorexia nervosa and bulimia nervosa.
The diagnosis of Borhave syndrome is usually based on abnormalities seen on a plain chest X-ray and is confirmed with a chest CT scan. Simple radiographs will almost always reveal abnormalities, commonly the presence of mediastinal or free peritoneal gas. Compared with endoscopy, water-soluble contrast agent esophagecography is more valuable in confirming the location and extent of esophageal perforation.
TreatmentBorhaven syndrome has an almost 100% mortality rate without surgical treatment. After surgical intervention, the mortality rate dropped to about 30%. Treatment usually includes immediate antibiotic therapy to prevent adenitis and sepsis, surgical repair of the perforation, and intravenous fluid replacement if fluid loss is severe. In some cases, even with early surgical intervention (completed within 24 hours), the risk of death is still as high as 25%.
The symptoms of esophageal rupture should not be underestimated. Timely identification and early treatment are the keys to survival. Are you able to quickly recognize these potential warning signs?