emDOCs Podcast – Episode 62: Esophageal Perforation/Rupture

Today on the emDOCs cast with Brit Long, MD (@long_brit), we cover esophageal rupture and perforation.

Episode 62: Esophageal Perforation/Rupture

Background:

• Full-thickness tear of the mucosal/muscular layers of the esophagus from sudden increase in esophageal pressure
• Esophagus lacks a serosal layer (more susceptible to perforation)
• With perforation, gastric contents and bacteria extravasate into the mediastinum => mediastinitis, systemic infection, empyema
• Mortality rates reach 90% if not diagnosed and treated

Causes:

• Boerhaave Syndrome: spontaneous rupture, defined as “rupture of the esophagus due to forceful emesis”
  • Rapid increase in intraesophageal pressures and opposing negative intrathoracic pressure
  • Accounts for 15% of cases
• Most common etiology of esophageal rupture is post endoscopy
  • Perforation associated with endoscopy is still very rare
• Other causes: foreign body or caustic ingestion, trauma, tumor, aortic pathology, infection

History and Exam:

• Must consider in patients with chest/abdominal pain
• Evaluate for risk factors (prior esophageal pathology and endoscopic procedures)
• Vomiting classically associated with Boerhaave, but this is just one cause of sudden increase in intraesophageal pressure
  • Others: childbirth, seizure, prolonged coughing/laughing, extreme exertion/weightlifting
• Mackler’s triad of Boerhaave syndrome: 1) severe vomiting followed by 2) chest pain and 3) subcutaneous emphysema. Present in less than 50%
• Nausea and vomiting present in less than 20%
• Pain is most common presenting symptom (70%), but depends on perforation location
• Other symptoms also depend on location
  • Cervical esophageal perforation – dysphagia and pain with neck flexion; dysphagia is present in 10%
  • Thoracic esophageal perforation – chest, back, and/or epigastric pain; dyspnea in 26%
  • Distal esophageal perforation with leakage into abdominal cavity – peritonitis and radiation of pain to shoulders as a result of diaphragmatic irritation
• Systemic symptoms occur with severe pain and mediastinal perforation; fever may be delayed (40-50%)
• Subcutaneous emphysema in the neck and/or chest suggestive, but occurs in less than 60% of patients
  • Hamman’s crunch: crunching sound over the precordium that occurs with each heartbeat with auscultation of the chest
  • Abnormal breath sounds due to pleural effusion or pneumothorax

ED Evaluation

• Labs: Not specific, cannot exclude diagnosis, need imaging
  • World Society for Emergency Surgery (WSES) recommends CBC, electrolytes, creatinine, liver function tests, pH, and lactate in the initial evaluation (Grade 1C recommendation).
  • Inflammatory markers may be elevated
• Imaging:
  • Chest X-ray abnormal in 90%
    • May see pneumomediastinum and subcutaneous emphysema
    • V sign – air outlining the medial left hemidiaphragm and left lower mediastinal border
    • Later findings include pleural effusion (usually left sided), mediastinal air-fluid levels, free air under the diaphragm, hydrothorax, pneumothorax
  • Definitive imaging: CT chest with IV contrast or CT esophagography
    • CT chest with IV contrast: 92-100% sensitivity; can rule out alternate diagnoses, evaluate for involvement of surrounding structures, guide management
    • If suspicious but CT negative, other tests include diagnostic endoscopy or fluoroscopy using water-based contrast

Management:

• Broad-spectrum intravenous antibiotics, manage symptoms with antiemetics and analgesics, PPI administration, make patient NPO, consult specialists for definitive therapy
• Specialists include thoracic surgery, interventional radiology, gastroenterology, and critical care
• Several options available for definitive therapy: direct repair and washout, hybrid approach, non-operative
  • Primary repair with washout: large perforations, overwhelming mediastinal infection, or large infectious collection formation
  • If surgical intervention is necessary but will be delayed and the patient has significant GI material in the pleural cavity with hemodynamic compromise, consider chest tube
  • Hybrid approach: minimally invasive, depends on location of the perforation, underlying pathologies, and clinical status of the patient
    • Example: GI stent placement, IR fluid drainage, admission to ICU
  • Non-operative: patients with early diagnosis and contained leak within the cervical esophagus and/or mediastinum; no abdominal involvement; no signs or symptoms of systemic infection
• Airway: May need airway management if hemodynamically unstable/respiratory distress or with accumulation of profound amounts of subcutaneous air
  • Caution with NIPPV: the potential increase in transluminal pressure within the esophagus could potentially worsen the tear, as well as increase subcutaneous air, leading to more difficulty in establishing a definitive airway
  • If toxic, respiratory distress, intubation is a good idea
• NG tube placement:
  • Place with care and speak with GI/surgeons first; increases in intraluminal pressure from eliciting a gag reflex could theoretically worsen the tear
• Antifungals:
  • Some patients may benefit: history of immunocompromise, esophageal lesions or infections, prolonged proton pump inhibitor use, or other risk factors for fungal infection should receive intravenous antifungal coverage such as fluconazole or caspofungin
  • Discuss with ID and surgical specialists

Reference:

• DeVivo A, Sheng AY, Koyfman A, Long B. High risk and low prevalence diseases: Esophageal perforation. Am J Emerg Med. 2022 Mar;53:29-36.