Boerhaave Syndrome

Background: Herman Boerhaave first described spontaneous rupture of the esophagus in 1724. It typically occurs after forceful emesis. Boerhaave syndrome is a transmural perforation of the esophagus to be distinguished from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting. Because it usually is associated with emesis, Boerhaave syndrome usually is not truly spontaneous. However, the term is useful for distinguishing it from iatrogenic perforation, which accounts for 85-90% of cases of esophageal rupture.

Diagnosis can be difficult because often no classic symptoms are present and delays in presentation for medical care are common. Approximately one third of all cases are clinically atypical. Prompt recognition of this potentially lethal condition is vital to ensure appropriate treatment. Mediastinitis, sepsis, and shock frequently are seen late in the course of illness, which further confuses the diagnostic picture.

A reported mortality estimate is approximately 35%, making it the most lethal perforation of the GI tract. The best outcomes are associated with early diagnosis and definitive surgical management within 12 hours of rupture. If intervention is delayed longer than 24 hours, the mortality rate (even with surgical intervention) rises to higher than 50% and to nearly 90% after 48 hours. Left untreated, the mortality rate is close to 100%.

Pathophysiology: Esophageal rupture in Boerhaave syndrome is postulated to be the result of a sudden rise in intraluminal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle to relax. The syndrome commonly is associated with overindulgence in food and/or alcohol. The most common anatomical location of the tear in Boerhaave syndrome is at the left posterolateral wall of the lower third of the esophagus, 2-3 cm proximal to the gastroesophageal junction, along the longitudinal wall of the esophagus. The second most common site of rupture is in the subdiaphragmatic or upper thoracic area.

Frequency:

• Internationally: Although likely underreported, incidence is relatively rare. A 1980 review by Kish cited 300 cases in the literature worldwide. A 1986 summary by Bladergroen and Postlethwait described 127 cases. Of these, 114 were diagnosed antemortem; the others were diagnosed at autopsy. Overall, Boerhaave syndrome accounts for 15% of all traumatic rupture or perforation of the esophagus.

Mortality/Morbidity: The mortality rate is high. It is the most lethal perforation of the GI tract. Survival is contingent largely upon early recognition and appropriate surgical intervention.

• Overall mortality rate is approximately 30%. Mortality is usually due to subsequent infection including mediastinitis, pneumonitis, pericarditis, or empyema

• Patients who undergo surgical repair within 24 hours of injury have a 70-75% chance of survival. This falls to 35-50% if surgery is delayed longer than 24 hours and to approximately 10% if delayed longer than 48 hours.

• Cases of patients surviving without surgery exist but are rare enough to warrant case reports in the medical literature.

Race:

• Cases have been reported in all races and on virtually every continent.

strong>Sex:

• The syndrome is described more commonly in males than in females, with ratios ranging from 2:1 to 5:1.

Age:

• It is seen most frequently among patients aged 50-70 years.

• Reports suggest that 80% of all patients are middle-aged men.

• Cases have been described in neonates and in persons older than 90 years. Although no clear explanation exists for this, the least susceptible age group appears to be children aged 1-17 years.

Treatment
Medical Care:

• Ideal management involves a combination of both conservative and surgical interventions.
• Mainstays of therapy include the following:

• Intravenous volume resuscitation
• Administration of broad-spectrum antibiotics
• Prompt surgical intervention

• The decision to use a conservative (medical intervention only) or an aggressive (medical plus surgical intervention) approach depends on the following factors:
  • Time delay in presentation and diagnosis
  • Extent of perforation
  • Overall medical condition of the patient

• Surgical intervention is the standard of care in most cases, but Cameron et al established a set of criteria in which conservative (nonsurgical) management might be appropriate. These include the following:

• The esophageal disruption should be well contained in the mediastinum.
• The cavity should be well drained back into the esophagus.
• Few symptoms should be present.
• Evidence of clinical sepsis should be minimal.

• Conservative management consists of the following:
  • Intravenous fluids should be instituted.
  • Antibiotics: Imipenem/cilastatin (Primaxin) offers good broad-spectrum coverage.
  • Nasogastric suction should be applied.
  • Keep the patient NPO.
  • Adequate drainage with tube thoracostomy or formal thoracotomy is vital.
  • Early use of nutritional supplementation: Recent evidence suggests that, for hastening recovery, a jejunostomy tube feeding may be favored over hyperalimentation.

Surgical Care:

• Barrett described the first successful surgical repair of the esophagus in 1947. Prior to this, Boerhaave syndrome had virtually 100% mortality.
• Most physicians advocate surgical intervention if the diagnosis is made within the first 24 hours after perforation.
• Direct repair of the rupture and adequate drainage of the mediastinum and pleural cavity provide the best survival rates.

• A left thoracotomy is the preferred approach, although laparotomy may be necessary if the tear extends into the distal esophagus.

• Various techniques, such as the use of an omental flap, may be used to support the primary closure.
• Gastrostomy and jejunostomy tubes often are placed to aid in drainage and nutrition, respectively.

• The vitality of the surrounding tissue is an important factor in selecting the surgical procedure.

• For patients in whom a delay in diagnosis (>24 h) occurred, primary repair may not be possible.
• After 24 hours, the wound edges frequently are edematous, stiff, and friable.

• Various alternatives to primary repair are available.

• The most common includes the creation of an esophageal diversion through the use of a loop or end-cervical esophagostomy. This allows the wound to heal by secondary intention.
• The use of T-tubes also has been described. T-tubes result in the formation of a controlled fistula and a route for drainage of esophageal secretions and refluxed gastric materials.
• One recent study noted that the option of primary repair may be considered for perforations as old as 72 hours.

• Newer techniques involve the use of plastic-covered self-expanding metallic stents.

• They are considered acceptable alternatives only when all other interventional options have been exhausted. Their use in nonmalignant disease is highly controversial because they cannot be removed without considerable risks or not at all.
• Currently, the use of stents in Boerhaave syndrome is recommended for cases that involve extreme delays in diagnosis or a failure of conservative management.
• Expandable metal stents are most commonly used as palliative interventions for unresectable malignant esophageal obstruction.
• These devices bridge the esophageal tear.
• Several types of stents are available, and they vary in flexibility. Recent research holds the promise of biodegradable stents, obviating the necessity for removal.
• Esophageal stents have been associated with a risk of delayed massive hemorrhage in patients with esophageal malignancy.
• The long-term effects of stent placement in Boerhaave syndrome have not been adequately evaluated.

• Late complications of surgical intervention may include the following:
  • Empyema that often requires tube drainage or decortication
  • Esophagotracheal or esophagobronchial fistulas

Consultations:

• Consultation with a thoracic or general surgeon is indicated as soon as the diagnosis is suspected.

• Infectious disease specialist should be consulted for assistance with antimicrobial therapy.

Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Antibiotics -- Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting. A broad-spectrum antibiotic, such as imipenem/cilastatin (Primaxin), is recommended.

Drug Name	Imipenem/cilastatin (Primaxin) -- Offers good broad-spectrum coverage. For treatment of multiple-organism infections in which other agents do not have wide-spectrum coverage or are contraindicated due to potential for toxicity.
Adult Dose	Base initial dose on severity of infection, and administer in equally divided doses 250-500 mg IV q6h, not to exceed 3-4 g/d Alternatively, 500-750 mg IM q12h or intra-abdominally
Pediatric Dose	<12>3 months Fully susceptible organisms: not to exceed 2 g/d Infections with moderately susceptible organisms: not to exceed 4 g/d
Contraindications	Documented hypersensitivity
Interactions	Coadministration with cyclosporine may increase adverse CNS effects of both agents; coadministration with ganciclovir may result in generalized seizures
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Adjust dose in renal insufficiency; avoid use in children <12>