Esophageal perforation

Perforation of the esophagus is most commonly caused by upper endoscopy (iatrogenic), foreign body ingestion, or trauma. It can be located at any point along the esophagus, in the cervical, thoracic, or abdominal region. Boerhaave syndrome is a spontaneous subtype of esophageal perforation characterized by transmural rupture of the esophagus following an episode of forceful vomiting/retching or increased intrathoracic pressure. The condition is associated with recent consumption of large amounts of alcohol or food, repeated episodes of vomiting, and other causes of elevated intrathoracic pressure (e.g., childbirth, seizure, prolonged coughing). The classic symptom is severe retrosternal pain, which is due to the development of mediastinal emphysema after massive emesis. Diagnosis of esophageal perforation is confirmed via neck or chest x-ray, esophagram, and/or CT of the neck and chest. Surgical repair of the esophageal rupture is often necessary, although conservative treatment alone may be considered in select cases (e.g., if the perforation is very small and the patient is stable).

• Boerhaave syndrome: ♂ > ♀ (3:1)

Epidemiological data refers to the US, unless otherwise specified.

Esophageal perforation (general) ^[1][2]

• Iatrogenic esophageal perforation
  • Most common cause of esophageal perforation
  • Most often injury during upper endoscopy
  • Injury related to surgery
• Ingestion of a foreign body or caustic material
  • Bone, dentures
  • Alkali or acidic agents (e.g., batteries)
• Trauma (blunt or penetrating)
• Malignancy
• Infection
  • Candida spp.
  • Herpes simplex
  • Tuberculosis
  • Syphilis
• Spontaneous rupture

Boerhaave syndrome

• Risk factors
  • Intake of large amounts of alcohol or food in the recent past
  • Repeated episodes of vomiting
  • Prolonged coughing
  • Childbirth
  • Seizures
  • Weightlifting
• Pathophysiology
  • Severe vomiting/increased intrathoracic pressure → rupture of all layers of the esophageal wall (transmural perforation)
  • In > 90% of cases, the rupture occurs in the distal third of the esophagus on the left dorsolateral wall surface.

• Mackler triad (esp. in Boerhaave syndrome)
  • Vomiting and/or retching
  • Severe retrosternal pain that often radiates to the back
  • Subcutaneous or mediastinal emphysema: crepitus in the suprasternal notch and neck region or crunching/crackling sound on chest auscultation (Hamman sign)
• Dyspnea, tachypnea, tachycardia
• Dysphagia
• Signs of sepsis
• History of recent endoscopy: Symptoms usually occur within 24 hours of endoscopy.
• Delayed presentations: critically ill with sepsis and multiorgan dysfunction

If esophageal perforation or Boerhaave syndrome is suspected, a neck or a chest x-ray is first conducted, followed by contrast esophagography. If inconclusive, or the patient is unstable/uncooperative, a CT scan is conducted to confirm the diagnosis. ^[1][2]

Imaging

• Initial diagnostic study
  • Neck x-ray lateral : subcutaneous emphysema
  • Chest x-ray posteroanterior and lateral, upright AXR
    • Widened mediastinum
    • Pneumomediastinum, pneumothorax, pneumoperitoneum, subcutaneous emphysema
    • Pleural effusion
    • Mediastinal air-fluid levels
• Confirmatory test: : Contrast leak on contrast esophagography (gold standard) reveals the location and size of the rupture. ^[3]
• CT scan (with oral contrast)
  • Indications
    • The patient is unstable/uncooperative.
    • Pneumoperitoneum is detected on x-ray.
    • X-rays and contrast esophagography are inconclusive.
  • Findings
    • Widened mediastinum
    • Esophageal wall thickening, hematoma
    • Extraluminal air: pneumomediastinum, pneumoperitoneum, pneumothorax, subcutaneous emphysema
    • Pleural effusion
• Flexible endoscopy
  • Consider if the CT scan is inconclusive.
  • Should be reserved for patients with a poorly localized esophageal perforation and those with a therapeutic indication for endoscopy

• Mallory-Weiss syndrome
• See “Differential diagnosis of chest pain”.

The differential diagnoses listed here are not exhaustive.

Initial approach ^[1][2][4]

• ABCDE survey
• Establish airway and/or provide supplemental oxygen as needed.
• IV fluid resuscitation
• Nothing by mouth (NPO) and supply nutritional support
• Broad-spectrum IV antibiotics (see empiric antibiotic treatment for intra-abdominal infection)
• IV proton pump inhibitor (e.g., pantoprazole )
• Parenteral analgesics (see acute pain management)

Nonsurgical treatment ^[1][2][4]

• Indications
  • Small, contained perforation, demonstrated by:
    • Either a contained leak with the neck, within the mediastinum, or between the mediastinum and visceral lung pleura
    • Contrast can flow back into the esophagus from the cavity surrounding the perforation.
  • The perforation site is benign, outside of the abdomen, and distal to an obstruction.
  • The patient is stable with no evidence of sepsis.
  • Contrast studies are available at any time for follow-up evaluation.
  • A skilled thoracic surgeon is continuously available.
• Consider endoscopic intervention
  • Esophageal stent placement
  • Endoclip
  • Fibrin glue application

Surgical treatment ^[1][2][4]

• Indications
  • Hemodynamic instability
  • Patients who do not fulfill the criteria for conservative management
  • Clinical deterioration during conservative management
• Surgical repair
  • Closure of the ruptured esophageal segment
  • Last resort: esophagectomy

• Supplemental oxygen as needed
• IV fluid resuscitation
• NPO
• Empiric broad-spectrum intravenous antibiotics
• Immediate thoracic surgery and GI consults for consideration of endoscopic vs. surgical management
• Consider nasogastric tube insertion after discussion with surgical consult.
• Parenteral analgesics: See acute pain management.
• Intravenous proton pump inhibitor
• Chest tube placement if pneumothorax or pleural effusion is present
• ICU transfer and close monitoring

Mediastinitis

• Definition: inflammation of the tissues in the mediastinum
• Classification ^[5][6]
  • Acute mediastinitis: acute infection of the mediastinum
  • Chronic mediastinitis (fibrosing mediastinitis): proliferation of fibrous and collagenous tissue in the mediastinum
• Etiology ^[5][6]
  • Acute mediastinitis
    • Cardiothoracic surgical procedures (most common cause): Mediastinitis typically occurs within 14 days of the procedure. ^[7]
    • Perforation of mediastinal structures (e.g., esophagus, trachea)
    • Descending spread of infection from oropharyngeal foci
  • Chronic mediastinitis
    • Etiology remains unclear.
    • Several studies report that Histoplasma capsulatum is causative.
• Clinical features
  • Retrosternal and/or back pain
  • Subcutaneous emphysema in the neck and face
  • Fever, tachycardia, tachypnea
  • Sternal wound drainage
  • Superior vena cava syndrome
  • Obstruction of the upper airways
  • Pleuritis and pericarditis
  • Bacteremia leading to sepsis and signs of shock
• Diagnostics
  • Chest x-ray (posteroanterior and lateral views) shows a widened mediastinum and mediastinal emphysema.
  • Chest CT (confirmatory test) shows attenuation of mediastinal fat, as well as mediastinal fluid collections and gas. ^[8][9]
  • CBC may show leukocytosis.
• Management: resuscitation, IV antibiotic therapy, and surgical debridement

Others

• Peritonitis in intraabdominal perforations
• Empyema
• Severe sepsis or shock
• Multiorgan dysfunction

We list the most important complications. The selection is not exhaustive.

• Mortality: 10–50% ^[2]