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. Evidence of esophageal perforation may be seen on neck, chest, and/or abdominal x-ray and the diagnosis is confirmed with esophagram and/or CT esophagography. 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

Symptoms are often nonspecific; maintain a high index of suspicion in patients with recent retching, vomiting, upper endoscopy, trauma, or known esophageal or mediastinal malignancy.

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

Imaging

Initial diagnostic studies

• Chest x-ray posteroanterior and lateral, upright AXR
  • Widened mediastinum
  • Pneumomediastinum, pneumothorax, pneumoperitoneum, subcutaneous emphysema
  • Pleural effusion
  • Mediastinal air-fluid levels
• Neck x-ray lateral : subcutaneous emphysema

As radiographic abnormalities may not be immediately apparent after injury, negative results on early plain x-rays do not rule out acute perforation. ^[3]

Confirmatory tests

• Contrast esophagography (gold standard): Contrast leak reveals the location and size of the rupture. ^[4][5]
• CT chest and CT esophagography (with oral contrast) ^[6][7]
  • 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, inflammation, and/or hematoma
    • Extraluminal air: pneumomediastinum, pneumoperitoneum, pneumothorax, and/or subcutaneous emphysema
    • Extraluminal contrast and/or periesophageal fluid collection(s)
    • Pleural effusion
• Flexible endoscopy: direct visualization of the perforation ^[1][3]
  • Typically reserved for patients with penetrating external esophageal injury
  • Avoided in nonpenetrating injury unless there is a specific therapeutic indication
• Caustic ingestion: urgent endoscopy recommended; oral contrast radiography typically not recommended

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

The differential diagnoses listed here are not exhaustive.

Initial management ^[1][3][8]

• ABCDE survey
  • Airway management for patients with signs of airway compromise
  • Supplemental oxygen as needed
  • IV fluid resuscitation
• Nothing by mouth (NPO) ^[2]
• IV proton pump inhibitor (e.g., pantoprazole )
• Broad-spectrum IV antibiotics: Initiate early in all patients.
  • Piperacillin/tazobactam OR meropenem ^[3][9]
  • PLUS vancomycin ^[9][10]
  • Consider adding fluconazole in patients at risk of fungal colonization. ^[3][9]
• Chest tube placement: Consider for pneumothorax or pleural effusions.
• Parenteral analgesia and antiemetics as needed
• Urgent thoracic surgery and GI consult
• Management of caustic ingestion, if present

Do not attempt blind nasogastric tube placement to avoid further damage to the esophagus. ^[9][11]

Patients with esophageal perforation can deteriorate rapidly and benefit from close monitoring in the ICU and early surgical consultation. ^[3]

Nonsurgical treatment ^[1][2][8]

• 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][8]

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

• Airway management and supplemental oxygen as needed
• IV fluid resuscitation
• NPO
• Empiric broad-spectrum IV antibiotics
• Immediate thoracic surgery and GI consults for consideration of endoscopic vs. surgical management
• Consider nasogastric tube insertion only after discussion with surgical consult.
• Parenteral analgesics and antiemetics
• 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 ^[12][13]
  • Acute mediastinitis: acute infection of the mediastinum
  • Chronic mediastinitis (fibrosing mediastinitis): proliferation of fibrous and collagenous tissue in the mediastinum
• Etiology ^[12][13]
  • Acute mediastinitis
    • Postoperative acute mediastinitis after cardiothoracic procedures (most common cause): Mediastinitis typically occurs within 14 days of the procedure. ^[14]
    • 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.
    • IgG4-related fibrosing mediastinitis
• 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 ^[15]
  • Chest x-ray (posteroanterior and lateral views) shows a widened mediastinum and mediastinal emphysema.
  • CT neck and chest (confirmatory test) shows attenuation of mediastinal fat, as well as mediastinal fluid collections and gas. ^[16]
  • CBC may show leukocytosis.
  • Blood, tissue, and/or fluid (mediastinal, pleural, or bronchoalveolar) cultures can help determine the causative organism.
• Management ^[15]
  • Resuscitation using the ABCDE approach
    • Difficult airway management for patients with soft tissue swelling or trismus
    • Immediate hemodynamic support for patients with septic shock
  • Broad spectrum IV antibiotics depending on the underlying etiology; see: ^[15]
    • Treatment of esophageal perforation
    • Empiric antibiotic therapy for deep neck infections
  • Urgent surgical consultation for debridement and drainage ^[17]
  • Disposition: Patients often require ICU admission.

Consider acute mediastinitis in any patient with recent cardiothoracic surgery, deep neck infection, or potential esophageal injury who presents with chest pain and/or sepsis. ^[13][18]

Early diagnosis and treatment are essential to prevent significant morbidity and mortality associated with acute mediastinitis. ^[15]

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]