Esophageal Perforation

Overview

Definition

Esophageal perforation, also called esophageal rupture, is a transmural defect of the esophageal wall that exposes the mediastinum to GI content.

Epidemiology

• Incidence in relation to causes:
  • Most cases are associated with invasive procedures (e.g., endoscopy or surgery).
  • Penetrating injuries > blunt injuries
• Age > 65 years: ↑ risk of perforation via instrumentation
• Predilection vary by etiology:
  • Spontaneous esophageal rupture or Boerhaave syndrome: more common in men (3:1)
  • No difference in sex predilection in terms of iatrogenic perforation
• Mortality rate:
  • Ranges from 10%–50%
  • Delay in diagnosis and treatment contributes to higher mortality.

Etiology

• Iatrogenic esophageal perforation 
• Noniatrogenic esophageal perforation:
  • Caustic ingestion
  • Spontaneous rupture (Boerhaave syndrome)
  • Foreign body ingestion
  • Penetrating or blunt trauma
  • Esophageal or mediastinal malignancy
  • Intrinsic diseases of the esophagus:
    • Crohn’s disease
    • Pill esophagitis
    • Infectious esophagitis
    • Eosinophilic esophagitis

Pathophysiology

The pathogenesis of esophageal perforation depends on the cause.

• Iatrogenic:
  • Most common cause of esophageal rupture
  • Upper endoscopy and/or dilatation:
    • Piercing or shearing can accidentally damage the esophageal wall.
    • Most common in the pharyngoesophageal junction
  • Esophageal surgery: perforation more common in the abdominal or lower esophagus 
• Foreign body or caustic material ingestion:
  • Acidic or basic solutions can cause thermal reactions that damage the esophageal mucosa.
  • Basic solutions: more deleterious for the esophageal mucosa than acidic solutions
  • Sharp foreign bodies (e.g., impacted bones) can mechanically damage the esophagus.
• Trauma:
  • Penetrating trauma (gunshot wound: most common in the United States)
  • Blunt trauma (e.g., falling from a great height or a vehicle accident)
• Malignancy:
  • Endophytic esophageal carcinoma
  • Mediastinal lymphadenopathy, in cases of metastatic malignancy, can also erode into the esophagus.
• Infection:
  • Infectious etiology can result in esophageal ulcers that lead to perforation.
  • Examples:
    • Viral esophagitis (e.g., herpes simplex virus, cytomegalovirus)
    • Fungal esophagitis (e.g., candidiasis)
    • Tuberculosis
• Boerhaave syndrome:
  • A sudden increase in intraluminal pressure in the esophagus, coupled with negative intrathoracic pressure, can lead to rupture.
  • Intrathoracic pressure can be increased by:
    • Forceful retching and vomiting (e.g., after excessive alcohol consumption)
    • Self-induced vomiting in bulimia nervosa or anorexia nervosa
    • Childbirth
    • Weightlifting
    • Persistent cough
  • Most common in the posterolateral side of the lower/distal esophagus

Clinical Presentation

Clinical features

History:

• Endoscopy or surgery near the esophagus within the last 24 hours
• Associated conditions: malignancy, radiation therapy, strictures
• Severe retching, vomiting
• Caustic or foreign body ingestion
• Traumatic injury
• Risk factors for infectious esophagitis

Manifestations:

• Acute in onset
• Symptoms vary by location of injury, which can include:
  • Pharyngeal or neck pain
  • Chest pain
  • Shortness of breath
  • Dysphagia and/or odynophagia
  • Abdominal pain
• Mackler triad (associated with Boerhaave syndrome): 
  • Retrosternal chest pain radiating to the back
  • Subcutaneous emphysema
  • Vomiting

Exam findings:

• Tachypnea
• Tachycardia
• Crepitus on the chest wall (from subcutaneous emphysema)
• Hamman’s sign: 
  • Mediastinal crunching sound over the precordium synchronous with the heartbeat 
  • From mediastinal emphysema
• Reduced breath sounds on the side of perforation
• Abdominal tenderness (in lower esophageal perforation)
• In severe and/or delayed presentation: fever, hypotension

Complications

• Acute mediastinitis:
  • Acute inflammation of the mediastinal tissues due to mediastinal spread of the esophageal and oropharyngeal flora
  • Presents with severe retrosternal chest pain, fever, tachypnea, tachycardia, or septic shock
• Sepsis can develop in cases of delayed presentation.
• Pleuritis
• Pericarditis
• Empyema: a collection of pus in the pleural cavity

Diagnosis

• Cervical X-ray:
  • Performed when cervical esophageal perforation is suspected
  • Finding(s): subcutaneous emphysema
• Chest X-ray:
  • Performed when thoracic or intraabdominal esophageal perforation is suspected
  • Finding(s): 
    • Pneumomediastinum
    • Pneumopericardium
    • Hydropneumothorax
    • Widened mediastinum
    • Subdiaphragmatic air
• Contrast esophagography:
  • Diagnostic
  • Use water-soluble contrast (Gastrografin):
    • For initial study
    • Finding: contrast leakage from the esophagus to the mediastinum
  • Barium:
    • Not used initially due to the risk of developing acute mediastinitis
    • Utilized if water-soluble study is negative (as barium demonstrates small perforation(s) well)
• CT scan of the chest:
  • Performed when:
    • Chest X-ray or esophagography is inconclusive
    • Patient is unstable
  • Finding(s): 
    • Esophageal wall thickening
    • Pneumomediastinum
    • Pneumopericardium
    • Pneumothorax
    • Widened mediastinum
• Upper endoscopy:
  • Performed when:
    • Location of the perforation is not clear
    • CT scan is inconclusive
  • Caution must be taken as insufflation of air can lead to extension of the perforation.

Management

Stabilization

Initial approach:

• ABCDE survey:
  • Airway: Ensure the patency of the airway.
  • Breathing: Ensure proper ventilation is occurring.
  • Circulation: Measure blood pressure and pulse, and administer IV fluids. 
  • Disability: Perform basic neurologic examination.
  • Exposure: Search for injuries, and perform environmental control. 
• Nothing by mouth
• Broad-spectrum IV antibiotics
• IV analgesic
• IV proton pump inhibitor
• Parenteral nutrition

Obtain surgical (including cardiothoracic) consult, as even stable patients can deteriorate and require surgery.

Further intervention determined by:

• Size and location of perforation
• Comorbidities

Nonsurgical management

• Indications:
  • Patient is stable with no signs of sepsis.
  • Perforation is contained:
    • Within the neck OR
    • Between visceral lung pleura and mediastinum
  • Perforation site is located outside of the abdomen.
  • Does not involve a neoplasm or obstruction
  • Contrast drains back to the esophagus. 
  • Contrast esophagography is available for follow-up assessment.
  • A skilled thoracic surgeon is readily available.
• Critical care monitoring
• Any sign of sepsis or deterioration → immediate surgery

Surgical management

Indications:

• Patient is hemodynamically unstable.
• Patient with intraabdominal esophageal perforation
• Patient with esophageal malignancy
• Respiratory complications

Procedure:

• Surgical repair of perforation (standard of care)
• Esophageal stent: may be used in select patients (with severe comorbidities and/or cannot undergo surgery)
• Endoscopic clipping: an option in small perforations that can be corrected with minimal tension
• Esophagectomy is used as a last resort.

Differential Diagnosis

• Mallory-Weiss syndrome: a superficial longitudinal laceration of the esophageal mucosa and/or submucosa at the gastroesophageal junction. As with esophageal perforation, the condition may be caused by increased intrathoracic pressure (e.g., by vomiting). The condition presents with hematemesis. Diagnosis is mostly clinical and treatment is supportive, with IV fluids and analgesics. 
• Esophageal spasm: also presents with dysphagia to solids and liquids but is associated with sudden onset of chest pain that is not exertion related. Two types of this condition are distal esophageal spasm and hypercontractile (jackhammer) esophagus. Manometry shows characteristic esophageal contractions with normal relaxation of the esophagogastric junction. Management involves treating reflux disease (if present) and a trial of calcium channel blocker.
• Gastroesophageal reflux disease (GERD): symptoms of heartburn and regurgitation caused by the reflux of stomach contents. The patient usually complains of a burning epigastric pain radiating up the chest with a sour or metallic taste in the mouth. The disease is due to inappropriate relaxation of the lower esophageal sphincter. Proton pump inhibitors are used to control symptoms of GERD.