emDOCs Revamp: Esophageal Perforation

Authors: Katey DG Osborne, MD (EM Attending Physician; Tacoma, WA), Rachel Bridwell, MD (EM Attending Physician; Tacoma, WA) // Reviewed by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital) and Brit Long, MD (@long_brit, EM Attending Physician, San Antonio, TX)

Welcome to emDOCs revamp! This series provides evidence-based updates to previous posts so you can stay current with what you need to know.

A 39-year-old male with history of achalasia with recent endoscopic dilation 24 hours prior presents to the ED for progressively worsening chest pain with radiation to his left shoulder. The pain is severe (10/10), worsened with swallowing and he has associated dyspnea.

Triage VS: BP 101/71 mm Hg, HR 124 BPM, T 102.4 F oral, RR 24 breaths per minute, SpO2 97% on room air.

Pertinent physical examination findings:
CV: Tachycardic, regular rhythm.
Lungs: Tachypneic, decreased breath sounds left lung base.
Abdomen: Soft, non-tender, without rebound or guarding.

What is the likely diagnosis, and what are your initial steps in evaluation and management?

Answer: Esophageal Perforation^1-6

Epidemiology:

• Most frequent etiology is iatrogenic injury from instrumentation accounting for 60% of cases (e.g. upper endoscopy, transesophageal echo, etc.).^1,2
• The most common non-iatrogenic cause is spontaneously due to increased intraesophageal pressure, Boerhaave syndrome, from forceful retching, coughing, straining, seizures, or even childbirth (15% of cases).²
• Additional etiologies include caustic ingestion, trauma, foreign body, or malignancy.²
• Despite advances in imaging and surgical methods, overall mortality approaches 20%, which doubles to 40% with delays to definitive management exceeding than 24 hours.³

Risk Factors: 

• Conditions pre-disposing a requirement for instrumentation, such as reflux esophagitis, hiatal hernia, strictures, achalasia
• Foreign body or caustic chemical ingestion
• HIV or immunosuppression with severe esophageal infection from HSV, CMV, candida species, or tuberculosis¹
• Injury during other thoracic or neck operative procedures¹

Clinical Presentation: 

• Depending on location of the perforation, presentation can vary to include pain (neck, chest, or epigastrium), radiation to back or shoulders, odynophagia, dyspnea, subcutaneous emphysema, emesis, fever, and/or hypotension.²
• Mackler’s triad: the classic presentation of Boerhaave syndrome includes severe vomiting, chest pain, and subcutaneous emphysema; however, all three findings are seen in < 50% of patients.^1,4
• If presenting later in course or with an intra-abdominal perforation, patients are more likely to show signs of sepsis and hemodynamic instability.⁵

Evaluation:

• Assess the ABCs and obtain vital signs
  • Fever, hypotension, tachypnea, and tachycardia may be present.
• Perform a thorough H&P:
  • Specifically ask about recent GI/ENT instrumentation
  • Physical Examination:
    • Neck / Chest Wall: Localized tenderness and/or evidence of crepitus.
    • Cardiac: Hamman’s crunch may be present if pneumomediastinum is present
    • Lungs: Rales or decreased lung sounds from pleural effusion.
    • Abdomen: Evidence of peritonitis if intra-abdominal rupture.
• ECG – May show tachycardia or rate related ST depressions
• Laboratory evaluation:
  • CBC w/ differential – may reveal leukocytosis with left shift
  • CMP, Lipase – can reveal alternative intra-abdominal diagnoses as well as show findings of end-organ hypoperfusion (elevated serum creatinine, transaminitis, etc.)
  • VBG with lactate⁴ – may show low pH with elevated lactate
  • Cardiac biomarkers – to evaluate for cardiac etiology in those presenting with chest pain or type II NSTEMI in those with sepsis.
• Imaging:
  • Chest radiograph (CXR): About 90% of radiographs demonstrate pneumomediastinum, pneumothorax, subcutaneous emphysema, pleural effusion, or pneumoperitoneum.^1,4
  • Computed tomography (CT) w/ IV contrast of neck and chest: 90% sensitive for rupture and more feasible in patients unable to tolerate swallowing contrast media or those intubated.⁵
  • Computed tomography (CT) esophagography: Addition of an oral contrast agent improves the positive predictive value of the CT chest for diagnosis of esophageal perforation in addition to evaluating for alternative extraesophageal diagnoses.
    • Performed in intubated patients via placement of nasogastric or orogastric tube proximal to area of suspected injury.⁶
  • Fluoroscopic esophagography: Reveals presence of as well as localizes perforation with 60% sensitivity for cervical perforations and 90% for intrathoracic perforations.^2,4,5
    • Recommended by the American College of Radiology (ACR) Appropriateness Criteria but otherwise limited for cervical perforations or alternative etiologies of chest pain.⁶

Treatment:

• Airway
  • Definitive airway management should be performed in those with respiratory distress with attention to appropriate resuscitation started beforehand in the setting of potential distributive shock from mediastinitis.
  • Caution with use of NIPPV – positive pressure can worsen injury as well as increase subcutaneous emphysema.⁴
• Fluid resuscitation and vasopressor use as appropriate.
• Initiate broad-spectrum antibiotics with both aerobic and anaerobic coverage: ⁴
  • Ampicillin/Sulbactam (3g IV), Piperacillin/tazobactam (3.375 g IV), or a carbapenem.
  • Beta lactam allergy: Clindamycin (900 mg IV) plus Ciprofloxacin (400 mg IV).
• Consider antifungal therapy with fluconazole 400 mg in select patients: ⁴
  • Recent hospitalization or broad-spectrum antibiotics prior to perforation
  • Long term proton pump inhibitor (PPI) therapy
  • Immunosuppression (i.e., HIV) or recent steroid use
  • Known esophageal candidiasis
  • Known esophageal dysmotility disorder
• Make patient NPO.
• PPI administration to decrease gastric acid secretion.
• Caution with nasogastric tube placement as there is theoretical risk of worsening of the tear. Discuss with GI/surgeons prior to placement. ⁴
• Prompt consultation is imperative with thoracic surgery, interventional radiology (IR), gastroenterology (GI), and/or critical care.
• Definitive management options include operative, non-operative, and a hybrid approaches: ^4,5
  • Operative: in the setting of large perforations, overwhelming fluid collections, and infection for mediastinal washout and direct esophageal repair
    • Traditional primary repair substantially reduces mortality when compared to conservative therapy
  • Non-operative: considered if found early in patients without systemic signs or symptoms and with evidence of contained leak in the cervical esophagus and/or mediastinum sparing the abdominal esophagus
  • Hybrid: minimally invasive approach which seals the perforation with esophageal stent placement by GI and drainage of fluid collections by IR
    • This is done in coordination with surgical and critical care specialists.
    • May not be an option.
  • If there will be a delay in definitive management and there is evidence of intra-thoracic gastric contents, consider chest tube placement.

Disposition:

• Admission to ICU with potential GI, IR, and/or surgical management.

Pearls:

• Increase index of suspicion in someone with recent EGD or ENT/GI instrumentation as iatrogenic injury is the most common cause.
• A chest radiograph is a good screening exam in patients for whom esophageal perforation is considered, but it is less sensitive early after injury.⁵
• Consider antifungal administration.
• In appropriate patients, there is the option for management with the minimally invasive hybrid approach with esophageal stenting and IR guided drainage.
• Prompt management is key as mortality is 20% and increases to 40% if definite management is delayed greater than 24 hours.

Further Reading: 

• emDOCs Esophageal perforation pearls and pitfalls
• emDOCs Podcast Esophageal Perforation

References:

1. Kemp CD, Yang SC. Esophageal Perforation. In: Yuh DD, Vricella LA, Yang SC, Doty JR, eds. Johns Hopkins Textbook of Cardiothoracic Surgery. McGraw-Hill Education; 2014. Accessed September 30, 2023. accesssurgery.mhmedical.com/content.aspx?aid=1104586430
2. Bono MJ. Esophageal Emergencies. In: Tintinalli JE, Ma OJ, Yealy DM, et al., eds. Tintinalli’s Emergency Medicine: A Comprehensive Study Guide. 9th ed. McGraw-Hill Education; 2020. Accessed September 30, 2023. accessemergencymedicine.mhmedical.com/content.aspx?aid=1166533659
3. Brinster CJ, Singhal S, Lee L, Marshall MB, Kaiser LR, Kucharczuk JC. Evolving options in the management of esophageal perforation. Ann Thorac Surg. 2004;77(4):1475-1483. doi:10.1016/j.athoracsur.2003.08.037
4. DeVivo A, Sheng AY, Koyfman A, Long B. High risk and low prevalence diseases: Esophageal perforation. Am J Emerg Med. 2022;53:29-36. doi:10.1016/j.ajem.2021.12.017
5. Muniappan A. Esophageal Perforation. In: Butler KL, Harisinghani M, eds. Acute Care Surgery: Imaging Essentials for Rapid Diagnosis. McGraw-Hill Education; 2015. Accessed October 1, 2023. accesssurgery.mhmedical.com/content.aspx?aid=1108260593
6. Norton-Gregory AA, Kulkarni NM, O’Connor SD, Budovec JJ, Zorn AP, Desouches SL. CT Esophagography for Evaluation of Esophageal Perforation. RadioGraphics. 2021;41(2):447-461. doi:10.1148/rg.2021200132