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Boerhaave syndrome

Updated : July 22, 2023





Background

Boerhaave syndrome, also known as spontaneous esophageal rupture, is a rare and life-threatening condition which is characterized by a full-thickness tear or rupture of the esophageal wall. It was first described by the Dutch physician Herman Boerhaave in the 18th century.

Boerhaave syndrome typically occurs due to a sudden increase in intraluminal esophageal pressure combined with poor esophageal wall integrity. The most common cause of Boerhaave syndrome is forceful vomiting, which leads to a sudden rise in intraesophageal pressure.

Other factors that can contribute to Boerhaave syndrome include vigorous coughing, childbirth, epileptic seizures, and procedures involving esophageal instrumentation. Boerhaave syndrome requires urgent medical attention and typically requires surgical repair. The prognosis is better if the diagnosis is made early and appropriate treatment is initiated promptly.

Epidemiology

  • Incidence: Boerhaave syndrome is considered a rare condition, accounting for 10-15% of all cases of esophageal perforation.
  • Age and gender: Boerhaave syndrome can occur at any age, but it is most observed in middle-aged adults, with a peak in the fifth and sixth decades of life.
  • Predisposing factors: The majority of cases of Boerhaave syndrome occur in individuals who have engaged in forceful vomiting, often related to alcohol intoxication, or eating disorders. Other predisposing factors include vigorous coughing, childbirth, epileptic seizures, and esophageal instrumentation.
  • Underlying conditions: Boerhaave syndrome may be associated with underlying conditions that weaken the esophageal wall, such as hiatal hernia, esophageal motility disorders, connective tissue disorders, and previous esophageal surgeries.

Anatomy

Pathophysiology

Increased intraluminal pressure: Boerhaave syndrome typically occurs due to a sudden increase in intraesophageal pressure. This can happen during episodes of forceful vomiting, retching, or coughing. The increased pressure causes a rupture or tear in the wall of the esophagus.

  • Full-thickness esophageal wall tear: The rupture in Boerhaave syndrome usually occurs in the distal part of the esophagus, specifically the left posterolateral aspect. It starts as a mucosal tear and progresses to involve all layers of the esophageal wall, resulting in a full-thickness defect.
  • Leakage of gastric contents: Once the esophageal wall is ruptured, gastric contents, including gastric acid, food particles, and bacteria, can leak into the mediastinum. This leads to the development of mediastinitis, which is a serious and potentially life-threatening condition.
  • Local and systemic inflammation: The leakage of gastric contents triggers an inflammatory response in the mediastinum, leading to recruitment of immune cells and release of pro-inflammatory mediators. This results in local tissue damage, abscess formation, and systemic manifestations such as fever, leukocytosis, and sepsis.

Etiology

Increased intraesophageal pressure: Boerhaave syndrome is commonly caused by a sudden increase in intraesophageal pressure. This can occur during episodes of forceful vomiting, retching, or coughing. The excessive pressure can lead to a rupture or tear in the esophageal wall.

  • Esophageal structural weakness: The esophagus can be predisposed to rupture if there is a structural weakness in its wall. Chronic conditions that weaken the esophageal wall, such as long-standing gastroesophageal reflux disease (GERD), esophageal strictures, or esophageal cancer, may increase the risk of developing Boerhaave syndrome.
  • Trauma or iatrogenic causes: Boerhaave syndrome can also occur as a result of trauma or iatrogenic injury to the esophagus. This can happen during medical procedures such as endoscopy, esophageal dilation, or esophageal surgery.
  • Alcohol or substance abuse: Excessive alcohol consumption or illicit drug use that leads to repeated episodes of forceful vomiting can increase the risk of developing Boerhaave syndrome.

Genetics

Prognostic Factors

Delay in Diagnosis and Treatment:

  • The time between onset of symptoms and initiation of treatment is a critical prognostic factor.
  • Early diagnosis and prompt surgical intervention are associated with better outcomes.

Degree of Esophageal Perforation:

  • The size and location of the esophageal perforation can impact the prognosis.
  • Larger or more extensive perforations may be associated with higher morbidity and mortality rates.

Presence of Complications:

  • Complications such as mediastinitis, sepsis, or pleural effusion can influence the prognosis.
  • The severity and management of these complications can affect patient outcomes.

Patient Factors:

  • The overall health status and comorbidities of the patient can influence prognosis.
  • Patients with pre-existing medical conditions may have higher surgical risks and poorer outcomes.

Clinical History

CLINICAL HISTORY

Age group: Boerhaave syndrome can occur in individuals of any age, but it is mostly seen in adults between 30 and 70 years.

 

Physical Examination

PHYSICAL EXAMINATION

  • General Appearance: The patient may appear in distress, with signs of respiratory distress such as rapid breathing, increased heart rate, and sweating.
  • Vital Signs: The vital signs may reveal signs of shock, including low blood pressure (hypotension) and a rapid heart rate (tachycardia).

Chest Examination:

  • Inspection: There may be signs of respiratory distress, such as increased respiratory effort, use of accessory muscles, and cyanosis (bluish discoloration of the skin).
  • Palpation: Palpation of the chest may reveal tenderness or crepitus (a crackling sensation) over the affected area.
  • Auscultation: Auscultation of the chest may reveal decreased breath sounds or abnormal breath sounds, such as crackles or wheezes.

Abdominal Examination:

  • Palpation: Palpation of the abdomen may reveal tenderness or guarding, especially in the upper abdomen.
  • Percussion: Percussion of the abdomen may reveal dullness or tympany over the affected area.
  • Neck Examination: Examination of the neck may reveal subcutaneous emphysema (air under the skin) or swelling in the neck, indicating a possible esophageal perforation.

Age group

Associated comorbidity

Associated comorbidity or activity:

Boerhaave syndrome is often associated with history of excessive alcohol consumption or substance abuse. It may also be seen in individuals with underlying esophageal disorders, such as gastroesophageal reflux disease (GERD), esophageal strictures, or esophageal cancer. Activities that involve forceful vomiting or retching, such as bulimia or weightlifting, can also increase the risk of Boerhaave syndrome.

Associated activity

Acuity of presentation

Acuity of presentation: Boerhaave syndrome typically presents acutely and is considered a medical emergency. The classic presentation includes a sudden onset of severe chest pain, often described as a tearing or ripping sensation, following a bout of forceful vomiting, or retching.

Differential Diagnoses

DIFFERENTIAL DIAGNOSIS

Gastroesophageal Reflux Disease (GERD):

  • History of chronic acid reflux or heartburn.
  • Absence of severe chest pain or signs of respiratory distress.
  • Presence of typical GERD symptoms such as regurgitation and dysphagia.

Acute Coronary Syndrome (ACS):

  • Severe chest pain radiating to the arm, jaw, or back.
  • ECG changes indicative of myocardial ischemia or infarction.

Esophageal Perforation due to Other Causes:

  • Traumatic esophageal injury, such as from instrumentation or foreign body ingestion.
  • Esophageal malignancy with perforation.
  • Infectious esophagitis with severe ulceration or abscess formation.

Pulmonary Embolism:

  • Acute onset of pleuritic chest pain and shortness of breath.
  • Presence of risk factors for venous thromboembolism, such as recent surgery or immobility.
  • Positive findings on imaging studies, such as CT pulmonary angiography.

Pneumothorax:

  • Sudden onset of pleuritic chest pain and shortness of breath.
  • Presence of risk factors for spontaneous pneumothorax, such as a history of lung disease or smoking.
  • Physical examination findings of decreased breath sounds and hyperresonance on percussion.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

TREATMENT PARADIGM

Modification of Environment:

  • NPO (nothing by mouth): Oral intake should be stopped to prevent further esophageal injury and minimize the risk of contamination.
  • Intravenous fluid administration: Adequate hydration and electrolyte balance should be maintained.

Administration of Pharmaceutical Agents with Drugs:

  • Broad-spectrum antibiotics: Given empirically to cover potential bacterial infections associated with esophageal perforation.
  • Proton pump inhibitors (PPIs): Used to reduce gastric acid secretion and minimize further damage to the esophageal tissues.

Intervention with a Procedure:

  • Surgical repair: The mainstay of treatment for Boerhaave syndrome is early surgical intervention, which involves primary closure of the esophageal perforation and reinforcement with tissue flaps or muscle patches. Minimally invasive approaches, such as thoracoscopic or laparoscopic surgery, may be considered in select cases.

Phase of Management:

  • Postoperative care: After surgical repair, patients require intensive care monitoring and management, including respiratory support, pain control, and nutritional support.
  • Long-term follow-up: Regular evaluation and surveillance to monitor for potential complications, such as anastomotic leaks, strictures, or recurrent fistulas.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

Boerhaave Syndrome.nlm.nih.gov.in

Boerhaave’s Syndrome: Still a Diagnostic and Therapeutic Challenge.ncbi.nlm.nih.gov

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Boerhaave syndrome

Updated : July 22, 2023




Boerhaave syndrome, also known as spontaneous esophageal rupture, is a rare and life-threatening condition which is characterized by a full-thickness tear or rupture of the esophageal wall. It was first described by the Dutch physician Herman Boerhaave in the 18th century.

Boerhaave syndrome typically occurs due to a sudden increase in intraluminal esophageal pressure combined with poor esophageal wall integrity. The most common cause of Boerhaave syndrome is forceful vomiting, which leads to a sudden rise in intraesophageal pressure.

Other factors that can contribute to Boerhaave syndrome include vigorous coughing, childbirth, epileptic seizures, and procedures involving esophageal instrumentation. Boerhaave syndrome requires urgent medical attention and typically requires surgical repair. The prognosis is better if the diagnosis is made early and appropriate treatment is initiated promptly.

  • Incidence: Boerhaave syndrome is considered a rare condition, accounting for 10-15% of all cases of esophageal perforation.
  • Age and gender: Boerhaave syndrome can occur at any age, but it is most observed in middle-aged adults, with a peak in the fifth and sixth decades of life.
  • Predisposing factors: The majority of cases of Boerhaave syndrome occur in individuals who have engaged in forceful vomiting, often related to alcohol intoxication, or eating disorders. Other predisposing factors include vigorous coughing, childbirth, epileptic seizures, and esophageal instrumentation.
  • Underlying conditions: Boerhaave syndrome may be associated with underlying conditions that weaken the esophageal wall, such as hiatal hernia, esophageal motility disorders, connective tissue disorders, and previous esophageal surgeries.

Increased intraluminal pressure: Boerhaave syndrome typically occurs due to a sudden increase in intraesophageal pressure. This can happen during episodes of forceful vomiting, retching, or coughing. The increased pressure causes a rupture or tear in the wall of the esophagus.

  • Full-thickness esophageal wall tear: The rupture in Boerhaave syndrome usually occurs in the distal part of the esophagus, specifically the left posterolateral aspect. It starts as a mucosal tear and progresses to involve all layers of the esophageal wall, resulting in a full-thickness defect.
  • Leakage of gastric contents: Once the esophageal wall is ruptured, gastric contents, including gastric acid, food particles, and bacteria, can leak into the mediastinum. This leads to the development of mediastinitis, which is a serious and potentially life-threatening condition.
  • Local and systemic inflammation: The leakage of gastric contents triggers an inflammatory response in the mediastinum, leading to recruitment of immune cells and release of pro-inflammatory mediators. This results in local tissue damage, abscess formation, and systemic manifestations such as fever, leukocytosis, and sepsis.

Increased intraesophageal pressure: Boerhaave syndrome is commonly caused by a sudden increase in intraesophageal pressure. This can occur during episodes of forceful vomiting, retching, or coughing. The excessive pressure can lead to a rupture or tear in the esophageal wall.

  • Esophageal structural weakness: The esophagus can be predisposed to rupture if there is a structural weakness in its wall. Chronic conditions that weaken the esophageal wall, such as long-standing gastroesophageal reflux disease (GERD), esophageal strictures, or esophageal cancer, may increase the risk of developing Boerhaave syndrome.
  • Trauma or iatrogenic causes: Boerhaave syndrome can also occur as a result of trauma or iatrogenic injury to the esophagus. This can happen during medical procedures such as endoscopy, esophageal dilation, or esophageal surgery.
  • Alcohol or substance abuse: Excessive alcohol consumption or illicit drug use that leads to repeated episodes of forceful vomiting can increase the risk of developing Boerhaave syndrome.

Delay in Diagnosis and Treatment:

  • The time between onset of symptoms and initiation of treatment is a critical prognostic factor.
  • Early diagnosis and prompt surgical intervention are associated with better outcomes.

Degree of Esophageal Perforation:

  • The size and location of the esophageal perforation can impact the prognosis.
  • Larger or more extensive perforations may be associated with higher morbidity and mortality rates.

Presence of Complications:

  • Complications such as mediastinitis, sepsis, or pleural effusion can influence the prognosis.
  • The severity and management of these complications can affect patient outcomes.

Patient Factors:

  • The overall health status and comorbidities of the patient can influence prognosis.
  • Patients with pre-existing medical conditions may have higher surgical risks and poorer outcomes.

CLINICAL HISTORY

Age group: Boerhaave syndrome can occur in individuals of any age, but it is mostly seen in adults between 30 and 70 years.

 

PHYSICAL EXAMINATION

  • General Appearance: The patient may appear in distress, with signs of respiratory distress such as rapid breathing, increased heart rate, and sweating.
  • Vital Signs: The vital signs may reveal signs of shock, including low blood pressure (hypotension) and a rapid heart rate (tachycardia).

Chest Examination:

  • Inspection: There may be signs of respiratory distress, such as increased respiratory effort, use of accessory muscles, and cyanosis (bluish discoloration of the skin).
  • Palpation: Palpation of the chest may reveal tenderness or crepitus (a crackling sensation) over the affected area.
  • Auscultation: Auscultation of the chest may reveal decreased breath sounds or abnormal breath sounds, such as crackles or wheezes.

Abdominal Examination:

  • Palpation: Palpation of the abdomen may reveal tenderness or guarding, especially in the upper abdomen.
  • Percussion: Percussion of the abdomen may reveal dullness or tympany over the affected area.
  • Neck Examination: Examination of the neck may reveal subcutaneous emphysema (air under the skin) or swelling in the neck, indicating a possible esophageal perforation.

Associated comorbidity or activity:

Boerhaave syndrome is often associated with history of excessive alcohol consumption or substance abuse. It may also be seen in individuals with underlying esophageal disorders, such as gastroesophageal reflux disease (GERD), esophageal strictures, or esophageal cancer. Activities that involve forceful vomiting or retching, such as bulimia or weightlifting, can also increase the risk of Boerhaave syndrome.

Acuity of presentation: Boerhaave syndrome typically presents acutely and is considered a medical emergency. The classic presentation includes a sudden onset of severe chest pain, often described as a tearing or ripping sensation, following a bout of forceful vomiting, or retching.

DIFFERENTIAL DIAGNOSIS

Gastroesophageal Reflux Disease (GERD):

  • History of chronic acid reflux or heartburn.
  • Absence of severe chest pain or signs of respiratory distress.
  • Presence of typical GERD symptoms such as regurgitation and dysphagia.

Acute Coronary Syndrome (ACS):

  • Severe chest pain radiating to the arm, jaw, or back.
  • ECG changes indicative of myocardial ischemia or infarction.

Esophageal Perforation due to Other Causes:

  • Traumatic esophageal injury, such as from instrumentation or foreign body ingestion.
  • Esophageal malignancy with perforation.
  • Infectious esophagitis with severe ulceration or abscess formation.

Pulmonary Embolism:

  • Acute onset of pleuritic chest pain and shortness of breath.
  • Presence of risk factors for venous thromboembolism, such as recent surgery or immobility.
  • Positive findings on imaging studies, such as CT pulmonary angiography.

Pneumothorax:

  • Sudden onset of pleuritic chest pain and shortness of breath.
  • Presence of risk factors for spontaneous pneumothorax, such as a history of lung disease or smoking.
  • Physical examination findings of decreased breath sounds and hyperresonance on percussion.

TREATMENT PARADIGM

Modification of Environment:

  • NPO (nothing by mouth): Oral intake should be stopped to prevent further esophageal injury and minimize the risk of contamination.
  • Intravenous fluid administration: Adequate hydration and electrolyte balance should be maintained.

Administration of Pharmaceutical Agents with Drugs:

  • Broad-spectrum antibiotics: Given empirically to cover potential bacterial infections associated with esophageal perforation.
  • Proton pump inhibitors (PPIs): Used to reduce gastric acid secretion and minimize further damage to the esophageal tissues.

Intervention with a Procedure:

  • Surgical repair: The mainstay of treatment for Boerhaave syndrome is early surgical intervention, which involves primary closure of the esophageal perforation and reinforcement with tissue flaps or muscle patches. Minimally invasive approaches, such as thoracoscopic or laparoscopic surgery, may be considered in select cases.

Phase of Management:

  • Postoperative care: After surgical repair, patients require intensive care monitoring and management, including respiratory support, pain control, and nutritional support.
  • Long-term follow-up: Regular evaluation and surveillance to monitor for potential complications, such as anastomotic leaks, strictures, or recurrent fistulas.

Boerhaave Syndrome.nlm.nih.gov.in

Boerhaave’s Syndrome: Still a Diagnostic and Therapeutic Challenge.ncbi.nlm.nih.gov

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