Boerhaave syndrome

Overview

Boerhaave syndrome is a rare but life-threatening condition characterized by spontaneous rupture of the esophagus, typically following forceful vomiting. Unlike other esophageal injuries, Boerhaave syndrome involves a full-thickness tear of the esophageal wall, allowing gastric contents to leak into the mediastinum and surrounding structures. This can lead to severe infection, inflammation, and shock if not treated promptly. The syndrome was first described by Dutch physician Hermann Boerhaave in the 18th century and remains a surgical emergency today.

Causes

The most common cause of Boerhaave syndrome is a sudden increase in intraesophageal pressure, usually due to intense vomiting or retching. Contributing factors include:

• Excessive alcohol intake – which often precedes episodes of forceful vomiting

• Overeating or consuming a large meal followed by vomiting

• Esophageal instrumentation – such as during endoscopy or nasogastric tube insertion (rarely)

• Severe coughing or straining – causing abrupt pressure changes

• Trauma or barotrauma – due to rapid pressure changes, e.g., during seizures or childbirth

Symptoms

Boerhaave syndrome typically presents suddenly and dramatically. Classic symptoms include:

• Severe chest pain – often described as tearing or ripping, radiating to the back or left shoulder

• Pain after vomiting – the most characteristic feature

• Difficulty swallowing (dysphagia)

• Shortness of breath

• Subcutaneous emphysema – crackling sensation under the skin due to air escaping into soft tissues

• Fever and signs of sepsis – due to mediastinal contamination and infection

• Shock and rapid deterioration – in advanced or untreated cases

The classic triad of vomiting, chest pain, and subcutaneous emphysema is known as Mackler’s triad, but it is present in only a minority of cases.

Diagnosis

Prompt diagnosis of Boerhaave syndrome is critical to improving survival. Diagnostic methods include:

• Chest X-ray – may show mediastinal air, pleural effusion, or pneumothorax

• CT scan of the chest – highly sensitive for detecting esophageal rupture and associated complications

• Contrast esophagography – using water-soluble contrast (e.g., Gastrografin) to visualize leakage from the esophagus

• Endoscopy – may be used cautiously to confirm diagnosis if imaging is inconclusive

• Laboratory tests – showing elevated white blood cell count, signs of infection, or metabolic acidosis

Treatment

Treatment for Boerhaave syndrome depends on the timing of diagnosis and the extent of the rupture. It often requires emergency intervention and includes:

• Emergency surgery – the mainstay treatment for full-thickness esophageal tears, especially if diagnosed within 24 hours

• Endoscopic stent placement – may be considered in select stable cases as a non-surgical alternative

• Broad-spectrum intravenous antibiotics – to prevent or treat mediastinitis and sepsis

• Nil per os (NPO) – withholding oral intake to allow healing

• Parenteral nutrition and fluid resuscitation

• Drainage of pleural effusions or abscesses – through chest tubes or surgical procedures

Prognosis

Boerhaave syndrome carries a high mortality rate if not diagnosed and treated promptly. Early surgical intervention within the first 24 hours significantly improves survival rates, which can exceed 80% with timely care. Delays beyond 24–48 hours are associated with increased risk of sepsis, multi-organ failure, and death. Long-term outcomes depend on the severity of the initial rupture, speed of treatment, and presence of complications such as infection or esophageal strictures. With proper and timely management, many patients recover fully.