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Boerhaave Syndrome, Emergency Medicine


Basics


Description


  • Spontaneous esophageal rupture from sudden combined increase in intra-abdominal pressure and negative intrathoracic pressure
    • Causes complete, full-thickness (transmural), longitudinal tear in esophagus
  • Esophagus has no serosal layer (which normally contains collagen and elastic fibers):
    • Results in weak structure vulnerable to perforation and mediastinal contamination
    • Esophageal wall is further weakened by conditions that damage mucosa (i.e., esophagitis is of various causes).
  • Majority of perforations occur at left posterolateral wall of the lower third esophagus.
  • Significant morbidity/mortality (most lethal GI tract perforation):
    • Owing to explosive nature of tear
    • Owing to almost immediate contamination of mediastinum with contents of esophagus
    • Overall mortality can approach 20%
    • Mortality can double if treatment is delayed >24 hr from rupture
    • Cervical rupture associated with the lowest mortality, followed by abdominal and thoracic rupture, respectively

Etiology


  • Associated with:
    • Forceful vomiting and retching (most common)
    • Heavy lifting
    • Seizures
    • Childbirth
    • Blunt trauma
    • Induced emesis
    • Caustic ingestions
    • Laughing
    • History of Barrett ulcer
    • History of HIV/AIDS
    • History of pill esophagitis
  • Common in middle-aged men
  • Medical procedures cause over 50% of all perforations.

  • Described in female neonates but rarely seen
  • Consider caustic ingestions

Diagnosis


Signs and Symptoms


History
  • Often no classic symptoms
  • Most common symptoms:
    • Chest or epigastric pain after vomiting/retching
  • Mackler triad:
    • Vomiting/retching
    • Chest pain
    • Subcutaneous emphysema
  • Retrosternal chest pain present in most patients:
    • Often pleuritic
    • Radiates to back or left shoulder
    • Worsens with swallowing
  • Odynophagia
  • Swallowing may precipitate coughing
  • Frequently, a history of alcoholism or heavy alcohol ingestion may be elicited

The vague nature of symptoms often lead to a delay in outcome and poorer prognosis  
Physical Exam
  • Dyspnea
  • Diaphoresis
  • Subcutaneous emphysema in neck and chest wall
  • Mediastinal crackling on auscultation (Hamman crunch)
  • Pleural effusions
  • Tachypnea
  • Fever
  • Shock, in more severe cases
  • If untreated, mediastinitis will develop and abscesses will form.
  • Not usually associated with bleeding

Essential Workup


  • Upright chest radiographs (preferably posteroanterior and lateral views if tolerated) evaluating for:
    • Pneumomediastinum
    • SC emphysema
    • Pleural effusion (left side)
    • Pneumothorax
    • Widened mediastinum
    • Hydropneumothorax
    • Empyema
    • Free peritoneal air
    • Naclerio "V" sign:
      • V-shaped radiolucency seen through the heart (air in left lower mediastinum)
  • Contrast esophagram identifies leak in esophagus:
    • Aids in decision of which type of surgical approach
    • Controversy exists regarding contrast use, water-soluble vs. barium
    • Water-soluble contrast material was thought to be less toxic if extravasated into the mediastinum; however, if aspirated may cause necrotizing pneumonitis and has a higher rate of false negatives
    • Barium, more sensitive for diagnosing perforation, but more irritating to the mediastinum
    • If esophagus is intact, use barium contrast for better detail

Diagnosis Tests & Interpretation


Lab
  • CBC
  • PT/PTT/INR
  • Blood cultures
  • Pleural effusion:
    • Amylase content
    • pH (<6)
    • Undigested food particles
  • ECG

Imaging
  • CXR
  • Endoscopy:
    • Controversial because this may extend perforation and/or introduce air into mediastinum
  • CT chest:
    • Sensitive for identifying free air, periesophageal fluid, mediastinal widening, air or fluid in pleural spaces; however, does not isolate lesion
    • Indicated if esophagram cannot be obtained
    • Evaluates other intrathoracic structures

Differential Diagnosis


  • Cholecystitis
  • Dissecting aortic aneurysm
  • Intestinal obstruction
  • Lung abscess
  • Mesenteric thrombosis
  • Myocardial infarction
  • Pneumothorax
  • Pericarditis
  • Pneumonia
  • Pancreatitis
  • Pulmonary thromboembolism
  • Ruptured abdominal viscus
  • Spontaneous pneumomediastinum (clinically benign)

Treatment


Pre-Hospital


  • Airway control must be established if patient unresponsive or airway patency in jeopardy.
  • Establish 2 large-bore intravenous catheters and treat hypotension with 0.9% NS.
  • Avoid opiates until patient is in ED to avoid complication of hypotension.

Initial Stabilization/Therapy


  • ABCs
  • Airway control: 100% oxygen or intubate patient if unresponsive or airway patency is in jeopardy.
  • Establish intravenous access and treat hypotension:
    • Administer 1 L (20 mL/kg) bolus with 0.9% NS (or lactated Ringer solution).
    • Initiate dopamine if blood pressure does not respond to fluids.
    • Central catheter placement if condition of patient remains unstable for more efficient delivery of fluids and monitoring of central venous pressure

Ed Treatment/Procedures


  • NPO
  • Careful placement of a nasogastric tube to decompress the stomach
  • Bladder catheter to monitor urine output
  • Expedient diagnosis to decrease incidence of morbidity/mortality
  • Prompt surgical consultation
  • Definitive treatment:
    • Surgical repair
    • Endoscopic stent placement, considered in appropriate patients
    • Conservative management, may be considered in patients with a contained perforation
  • Initiate broad-spectrum antibiotics directed against oral microflora and gastrointestinal pathogens:
    • Ampicillin/sulbactam + gentamicin
    • Imipenem/Cilastatin

Medication


  • Ampicillin/sulbactam: 3 g IV q6h
  • Dopamine: 2-20 μg/kg/min IV per bolus
  • Gentamicin: 2 mg/kg load, then 1.7 mg/kg IV q8h or 5-7 mg/kg IV QD (assuming normal renal function)
  • Imipenem/cilastatin: 250-500 IV q6h

Follow-Up


Disposition


Admission Criteria
All cases of Boerhaave syndrome must be admitted to surgical ICU:  
  • Cervical esophageal perforations may be treated by drainage alone.
  • All thoracic and abdominal perforations require surgical intervention.

Discharge Criteria
None  
Issues for Referral
Thoracic or general surgeon must be consulted for admission and possible operative intervention.  

Followup Recommendations


As per surgeon recommendations  

Pearls and Pitfalls


  • Chest radiographs done immediately after injury may be normal.
  • Left pleural space involvement is usually associated with a distal esophageal perforation.
  • Right pleural space involvement is usually associated with proximal esophageal perforations.
  • If esophagram is negative and there is high suspicion, repeat with patient in left and right decubitus positions.
  • Immediate surgical consultation is the keystone of management.
  • Significant increases in mortality are seen with delay in diagnosis and management.

Additional Reading


  • Brinster  CJ, Singhal  S, Lee  L, et al. Evolving options in the management of esophageal perforation. Ann Thoracic Surg.  2004;77:1475-1483.
  • Katabathina  VS, Restrepo  CS, Martinez-Jimenez  S, et al. Nonvascular, nontraumatic mediastinal emergencies in adults: A comprehensive review of imaging findings. Radiographics.  2011;31:1150-1153.
  • Onat  S, Ulku  R, Cigdem  KM, et al. Factors affecting the outcome of surgically treated non-iatrogenic traumatic cervical esophageal perforation: 28 years experience at a single center. J Cardiothorac Surg.  2010;5:46.
  • Vogel  SB, Rout  WR, Martin  TD, et al. Esophageal peforation in adults: Aggressive, conservative treatment lowers morbidity and mortality. Ann Surg.  2005;241:1016-1023.
  • Wu  JT, Mattox  KL, Wall  MJ Jr. Esophageal perforations: New perspectives and treatment paradigms. J Trauma.  2007;63:1173-1184.

Codes


ICD9


530.4 Perforation of esophagus  

ICD10


K22.3 Perforation of esophagus  

SNOMED


  • 19995004 Spontaneous rupture of esophagus (disorder)
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