VIDEO: What’s in a name? How tears of the esophagus became known as Boerhaave syndrome
In this Endo-Sketch, a Healio video series on clinical conditions named after famous colleagues, Klaus Mergener, MD, of the University of Washington School of Medicine, discusses the origin of Boerhaave syndrome.
These full-thickness tears of the esophagus typically occur after violent vomiting and retching, Mergener said. Patients quickly become sick and may develop retrosternal pain, fever, tachycardia and eventually sepsis.
Before the availability of surgical treatment, these tears were largely considered fatal. Now, if the defect occurs in the typical location, the treatment is left-sided thoracotomy with repair of the tear and flap closure. More recently, endoscopists have assisted in the identification and treatment of certain tears.
According to Mergener, these esophageal tears were named after Herman Boerhaave, who was born in 1668 in the Netherlands. Although he initially studied to become a preacher like his father, Boerhaave later added philosophy, mathematics, basic sciences, botany and medicine to his studies and became a physician, serving as professor of medicine, botany and chemistry at the University of Leiden. He eventually became president of the university.
The syndrome was named after Boerhaave, because he was the first to describe it in detail. Although Boerhaave’s patient died as a result of the esophageal rupture, he described his symptoms to Boerhaave, who later performed the patient’s autopsy.
Mergener noted that Boerhaave, in addition to being an outstanding educator, made many other contributions in medicine, as well as in his other fields of interests, including serving as director of the Leiden botanical garden.
“Dr. Boerhaave was the classical renaissance man in the sense that we use the term nowadays,” Mergener said. “He had extremely broad knowledge and interests.”
Perspective
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Boerhaave syndrome, also called effort rupture of the esophagus, is defined by spontaneous perforation of the esophagus caused by forceful vomiting or excessive straining. The classic history of Boerhaave syndrome is characterized by severe retching and vomiting followed by excruciating retrosternal chest pain due to an intrathoracic esophageal perforation. Physical examination may show subcutaneous emphysema (crepitus), pleural effusion or audible mediastinal crackling with each heartbeat, known as the Hamman’s sign. If untreated, sepsis, multiorgan failure and death ensues.
It is important to recognize that although well-characterized, the classic signs of Boerhaave syndrome require time to develop and even then are present in only a small number of patients. In addition, up to 45% of patients have no history of vomiting. The presentation also differs depending on location of perforation. Typically, Boerhaave syndrome involves linear perforation along the left posterolateral aspect of the distal intrathoracic esophagus. However, perforation can occur in the intraabdominal esophagus or cervical esophagus, leading to epigastric pain or neck pain instead of retrosternal chest pain, respectively.
The mainstay of treatment for esophageal perforation is by avoidance of oral intake, antibiotics, drainage of fluid collections and, when indicated, surgery. The role of endoscopy in management of esophageal perforation is controversial, as insufflation may extend the line of perforation. However, advances in endoscopy have led to the development of stents, clips, suturing, drains and EUS-guided drainage of paraesophageal abscesses. The advent of this new endoscopic armamentarium, in combination with highly experienced advanced endoscopists, has led to an emerging role of endoscopic therapy as primary repair for patients with esophageal perforation. However, this needs to be evaluated based on available endoscopic expertise and only applied to patients who are not surgical candidates.
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