There are some diseases we will never see or see only once. Boerhaave Syndrome, or spontaneous perforation of the esophagus is probably one of those. It was first described in 1729 by Herman Boerhaave, happens due to increased esophageal pressure, usually after vomiting, and has a mortality rate of 30-50% (100% without adequate treatment). The lower third of the esophagus is the most common location of the perforation, 2-4 cm above the gastroesophageal junction (thinner wall) and on the left side. Patients are usually males, between 50 and 70 years old, with a previous history of alcohol abuse or psychiatric disorders. Although surgical treatment is generally necessary, some patients may be conservatively managed. Who are the best candidates for conservative treatment of Boerhaave Syndrome? A recent paper (2023) from Dr. Maria Tarazona et al. from Colombia may give us some answers.
The Mackler triad is the classical presentation of Boerhaave Syndrome: lower thoracic pain, vomiting, and subcutaneous emphysema. However, the Mackler triad is only present in 15% of patients, making the diagnosis a clinical challenge. When a spontaneous esophageal tear is suspected, contrast-enhanced CT and esophagogram are the best tools to confirm this life-threatening entity and evaluate the extension and involvement of adjacent areas and structures.
The authors diagnosed Boerhaave Syndrome in a 19yo female patient with a previous history of postprandial vomiting for three months. In the last 24h, she initiated high-intensity epigastric pain, edema of the anterior surface of the neck, and dysphagia. On physical examination, subcutaneous emphysema was noticed from the submandibular area to the second intercostal space. A neck and thoracic CT scan showed extensive pneumomediastinum and emphysema, but “without signs of mediastinitis”. Maria Tarazona et al. concluded the patient had a contained leak because the esophagogram showed no leak. She was hemodynamically normal. The authors decided on a conservative treatment based on a Pittsburgh score of 1 (leukocytosis > 10000). Ten days later, the patient had no subcutaneous emphysema, no signs of a leak, was tolerating oral intake, and was discharged home. At three months follow-up, she was free of symptoms with normal upper endoscopy. Impressive! In my opinion, this great outcome was possible due to the young age of the patient. Can we use the same approach in older patients?
Conservative, surgical, percutaneous, and endoscopic treatment are possibilities. Which one to choose can be difficult. The Pittsburgh Score (PS) can be of good help. This score is a group of clinical signs to which a specific value corresponds. The sum of all values gives the final score.
Treatment decision based on the Pittsburgh Score (PS) for Esophageal Perforation:
PS < 2 points with a contained leak – conservative treatment
PS < 2 points with an uncontained leak – endoscopic treatment
PS = 3-5 points with a contained leak – consider conservative treatment with close surveillance for early diagnosis of sepsis
PS = 3-5 points with an uncontained leak – surgical treatment (esophagectomy or primary repair)
PS > 5 – surgical treatment (evaluate functional status to define aggressive treatment)
Essentially, in cases of early presentation (<24h), without surrounding areas contamination and contained leak, conservative treatment can be offered if the patient is hemodynamically stable. If these features are not fully observed, an intervention (surgery or endoscopy) is mandatory.
Conservative treatment includes nil per os, board spectrum antibiotics, proton pump inhibitors, nutritional support (e.g., parenteral), and constant clinical evaluation. In the case of abscesses, percutaneous drainage is preferred over surgical management in hemodynamically stable patients.
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Costa Almeida CE, editor. Posterior Retroperitoneoscopic Adrenalectomy. Indications, Technical Steps and Outcomes. Switzerland: Springer; 2023.
As the authors state, “early recognition of the disease, and effective classification of the patient in low, medium, or high-risk groups according to Pittsburgh score leads to timely and targeted treatment”. However, surgeons must not be stuck with scores and guidelines, but follow clinical perception of the severity of the patient they are treating. Surgeons must never forget that Boerhaave Syndrome is a life-threatening condition with high mortality. Conservative treatment can be a valid option with few iatrogenic side effects. However, if we do not promote intensive clinical surveillance of this kind of patient, the correct timing for surgical intervention can be missed. The clinical evaluation of a Boerhaave Syndrome patient is always the most important factor throughout the decision-making process. In the end, is not the Pittsburgh score just the usual clinical evaluation presented in a systematized way?
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Dr. Carlos Eduardo Costa Almeida
General Surgeon