II. Pathophysiology

  1. Rupture results in linear tear of the lower esophagus
  2. Following rupture, only mediastinal pleura contains esophageal contents
    1. Lower esophagus lacks serosal covering
  3. Esophageal contents leaks into mediastinum, resulting in cardiopulmonary and systemic findings
    1. Pressure gradient changes with respirations
  4. Severe inflammation and infection follows
    1. Mediastinitis
    2. Empyema (following spread to pleural space)

III. Epidemiology

  1. Rare (but lethal if unrecognized)

IV. Causes

  1. Esophageal Cancer
  2. Esophageal Foreign Body
  3. Sudden increase in esophageal pressure
    1. Severe Retching or Vomiting (Mallory-Weis Tear)
    2. Valsalva Maneuver
  4. Traumatic Injury
    1. Upper endosccopy
    2. Penetrating Chest Trauma
    3. Blunt force to left chest, Sternum or epigastrium
      1. Forces gastric contents into the esophagus

V. Precautions

  1. Consider in the injured patient with a left Pneumothorax or Hemothorax without a Rib Fracture

VI. Findings: Presentations

  1. Mackler's Triad (occurs in <50% of patients)
    1. Severe Vomiting
    2. Chest Pain
    3. Subcutaneous Emphysema

VII. Symptoms

  1. Melena
  2. Vomiting
    1. Retching
    2. Hematemesis
  3. Chest Pain (70% of cases, most common presenting symptom)
    1. Follows Retching, Valsalva Maneuver or other sudden increase in esophageal pressure
    2. Pain distribution depends on perforation location
      1. Cervical Esophageal Perforation
        1. Dysphagia
        2. Pain increases with Swallowing or neck flexion
      2. Thoracic Esophageal Perforation
        1. Retrosternal pain radiates to back or epigastrium
      3. Distal Esophageal Perforation
        1. Pain radiation into Shoulders from diaphragmatic irritation
        2. Peritonitis if esophageal contents leak into Abdomen

VIII. Signs

  1. Sudden collapse and patient appears acutely ill
    1. Fever (delayed onset in up to 50% of cases)
    2. Shock
    3. Tachycardia
  2. Signs develop only gradually
    1. Subcutaneous Emphysema
    2. Mediastinal air
    3. Hamman's Crunch
    4. Pleural Effusion
    5. Pneumothorax

IX. Labs

  1. Broad based labs are typically performed as for any critically ill patient
    1. However diagnosis is based on imaging
  2. Complete Blood Count (CBC)
    1. Leukocytosis

X. Diagnostics

  1. Nasogastric Tube
    1. Aspirate with bloody fluid
  2. Chest XRay (Test Sensitivity: 90%)
    1. Anterior displacement of trachea
    2. Subcutaneous Emphysema
    3. Pneumomediastinum (Mediastinal air, Mediastinal Emphysema)
      1. Pathognomonic of Esophageal Rupture
    4. V-Sign
      1. Air outlines the medial left hemidiaphragm and lower mediastinal margin
    5. Pleural Effusion (esp. left sided)
    6. Wide Mediastinum
    7. Pneumothorax
      1. When Trauma-related it is often left sided and without Rib Fracture
      2. Chest Tube will show continued air leak and particulate matter
    8. Other findings
      1. Mediastinal air fluid level
      2. Free air under diaphragm
  3. Chest CT with IV Contrast (or CT Esophagography)
    1. Test Sensitivity: 92 to 100%
    2. Preferred study in patients stable enough to undergo CT imaging
    3. Guides surgical management, evaluates for associated injury, and excludes other causes
  4. Other studies (if CT imaging non-diagnostic)
    1. Esophagram (Gastrografin or dilute barium)
    2. Gastroscopy (False Negatives not uncommon)

XI. Management

  1. Emergent surgical intervention
    1. Survival rates are best with the earliest interventions (within hours of onset)
    2. Wide mediastinal drainage and esophageal wall repair is often required
      1. However, minimally invasive procedures may be indicated in some cases
  2. ABC Management
    1. Aggressive fluid Resuscitation (and Vasopressors as needed) for shock
    2. Perform Endotracheal Intubation early in unstable patients (esp. with subcutaneous Emphysema)
    3. Avoid Noninvasive Ventilation (BiPAP, NIPPV)
      1. May worsen Esophageal Perforation and subcutaneous Emphysema
  3. Broad spectrum antibiotics
    1. Drug 1: Vancomycin
    2. Drug 2 (Choose 1)
      1. Piperacillin/Tazobactam (Zosyn) OR
      2. Meropenem OR
      3. Cefepime and Metronidazole
    3. Antifungal indications (consult infectious disease)
      1. Immunocompromised patient
      2. Prior esophageal infection
      3. Chronic Proton Pump Inhibitor (PPI)
  4. Prevent increases in esophageal pressure via symptom management
    1. Antiemetics (e.g. IV Ondansetron)
    2. Opioid Analgesics
  5. Other measures
    1. Keep patient NPO
    2. Proton Pump Inhibitor (e.g. IV Pantoprazole)
    3. Chest Tube for contaminated (esophageal contents) Pleural Effusion drainage
    4. Avoid Nasogastric Tube placement if possible
      1. Perform only under direction of managing surgical team
      2. Risk of increasing esophageal pressures on placement (Gag Reflex)
      3. Difficult placement and possible misplacement via perforation

XII. Resources

  1. Kassem (2021) Esophageal Perforation And Tears, StatPearls, Treasure Island
    1. https://www.ncbi.nlm.nih.gov/books/NBK532298/

XIII. References

  1. (2012) ATLS, 9th Ed, American College of Surgeons, Committee on Trauma, p. 108
  2. Long and Swaminathan in Swadron (2022) EM:Rap 22(7): 13-5

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Related Studies

Ontology: Esophageal Perforation (C0014860)

Definition (NCI_CTCAE) A disorder characterized by a rupture in the wall of the esophagus.
Definition (NCI) The presence of a hole or other type of opening in the esophageal wall through which the contents of the esophagus can pass into the mediastinum. The most common cause of esophageal perforation is injury during a medical procedure such as esophagoscopy or placement of a naso-gastric tube; and pathologic process such as neoplasm or gastric reflux with ulceration. Less common causes include injuries from penetrating or blunt trauma or injury to the esophagus during an operation on another organ, mechanical problem such as violent retching or vomiting; ingestion of a foreign body or caustic agents. The condition often results in infection of the mediastinum and mediastinitis.
Definition (MSH) An opening or hole in the ESOPHAGUS that is caused by TRAUMA, injury, or pathological process.
Concepts Disease or Syndrome (T047)
MSH D004939
ICD9 530.4
ICD10 K22.3
SnomedCT 155676000, 23387001
English Esophageal Perforations, Perforation, Esophageal, Perforations, Esophageal, ESOPHAGEAL PERFORATION, OESOPHAGEAL PERFORATION, esophageal perforation, esophageal perforation (diagnosis), Esophageal perforation, Perforation esophagus, Oesophageal perforation, Perforation Of Esophagus, Esophageal Perforation [Disease/Finding], esophagus perforation, perforation esophagus, oesophageal perforation, perforation of esophagus, Perforation of oesophagus (disorder), Perforation oesophagus, PERFORATION OF ESOPHAGUS, Perforation of Esophagus, ESOPHAGUS, PERFORATION OF, Perforation of esophagus, Perforation of oesophagus, Perforation of esophagus (disorder), esophagus; perforation, perforation; esophagus, Esophageal Perforation
Portuguese PERFURACAO ESOFAGICA, Perfuração do esófago, Perfuração esofágica, Perfuração Esofágica
German OESOPHAGUSPERFORATION, Perforation des Oesophagus, Oesophagusperforation, Ösophagusperforation
Italian Perforazione esofagea, Perforazione dell'esofago
Dutch perforatie oesofagus, slokdarmperforatie, perforatie van slokdarm, oesofagus; perforatie, perforatie; oesofagus, Perforatie van slokdarm, oesofagusperforatie, Oesofagusperforatie, Perforatie, oesofagus-, Perforatie, slokdarm-, Slokdarmperforatie
Spanish Perforacion de esófago, ESOFAGO, PERFORACION, perforación del esófago (trastorno), perforación del esófago, Perforación de esófago, Perforación del Esófago
Japanese 食道穿孔, ショクドウセンコウ
Swedish Esofagusperforation
Czech ezofágus - perforace, Perforace jícnu, Jícnová perforace
Finnish Ruokatorven perforaatio
Russian PISHCHEVODA PERFORATSIIA, ПИЩЕВОДА ПЕРФОРАЦИЯ
French PERFORATION DE L'OESOPHAGE, Perforation de l'oesophage, Perforation oesophagienne, Rupture de l'oesophage
Korean 식도의 천공
Polish Perforacja przełyku, Przedziurawienie przełyku
Hungarian Nyelőcső perforatio, Oesophagus perforatio, Nyelőcső perforatiója, Oesophagealis perforatio
Norwegian Oesophagusperforasjon, Øsofagusperforasjon, Spiserørsperforasjon, Perforasjon av spiserøret, Perforasjon av øsofagus

Ontology: Boerhaave syndrome (C0238115)

Concepts Disease or Syndrome (T047)
MSH D004939 , C536571
SnomedCT 19995004, 307217000
English Spontaneous rupture esophagus, Spontaneous rupture oesophagus, Spontan perforation esophagus, Spontan perforation oesophagus, Boerhaave's syndrome (diagnosis), Boerhaave syndrome, Boerhave syndrome, boerhaave's syndrome, boerhaave syndrome, boerhaaves syndrome, Spontaneous esophageal perforation, Spontaneous oesophageal perforation, Spontaneous perforation of oesophagus (disorder), Boerhaave's syndrome, Spontaneous rupture of esophagus, Spontaneous rupture of oesophagus, Spontaneous perforation of esophagus, Spontaneous perforation of oesophagus, Spontaneous rupture of esophagus (disorder)
Dutch Boerhaave-syndroom
German Boerhaave-Syndrom
Italian Sindrome di Boerhaave
Portuguese Síndrome de Boerhaave
Spanish Síndrome de Boerhaave, perforación esofágica espontánea, ruptura esofágica espontánea, ruptura espontánea del esófago (trastorno), ruptura espontánea del esófago, síndrome de Boerhaave
Japanese ブールハーフェ症候群, ブールハーフェショウコウグン
French Syndrome de Boerhaave
Czech Boerhaaveův syndrom
Hungarian Boerhaave syndroma

Ontology: Rupture of esophagus (C0281839)

Concepts Injury or Poisoning (T037)
ICD10 K22.3
SnomedCT 235626005
Italian Rottura esofagea, Rottura dell'esofago
French Rupture oesophagienne, Rupture de l'oesophage
Portuguese Rutura do esófago, Ruptura esofágica
Spanish Rotura esofágica, disrrupción esofágica, Rotura de esófago, ruptura esofágica (trastorno), ruptura esofágica
Japanese 食道破裂, ショクドウハレツ
Czech Ruptura jícnu
English esophagus rupture, oesophageal rupture, rupture esophagus, esophageal rupture, rupture of esophagus, Oesophageal rupture, Esophageal rupture, Bursting injury of esophagus, Bursting injury of oesophagus, Disruption of esophagus, Disruption of oesophagus, Rupture of oesophagus, Rupture of esophagus (disorder), esophagus; rupture, rupture; esophagus, Rupture of esophagus
Hungarian Oesophagealis szakadás, Oesophagus ruptura
Dutch oesofagus; ruptuur, ruptuur; oesofagus, slokdarmruptuur
German Speiseroehrenriss