The remainder of the patient's medical course was complicated by persistent right-sided empyema, ultimately requiring video-assisted thoracoscopic surgery with decortication. Upper endoscopy 2 months after the stent placement revealed a healed perforation, at which point the stent was removed. Currently, the patient is being slowly reintroduced to a mechanical soft diet with a tube-fed nutritional supplementation.
Boerhaave syndrome's classic presentation, Meckler's triad (retching, chest pain, and subcutaneous emphysema), is present in less than 10% of cases, which frequently leads to a delayed or missed diagnosis.1 Clinical presentation varies by severity, although, if left untreated, it carries a high mortality rate. Therefore, it should be considered when any 2 of Meckler's triad are present in a patient's history.1,2
A Boerhaave perforation is classically localized to the left distal third of the esophagus, likely because of a weak zone in the smooth muscle fibers at this level.3 Owing to the usual location of perforation, 75%–90% of cases result in a unilateral left-sided pleural effusion that can progress to empyema.4 Although unilateral effusion is somewhat common with this pathology, only 1 case report has demonstrated bilateral empyemas as a consequence of Boerhaave syndrome.5,6 This patient's fluoroscopy only demonstrated a leak into the mediastinum and right pleural space, which is likely because of delayed imaging, allowing a left-sided tear to heal.
Our patient was found to have a hiatal hernia, which may lead one to question whether it predisposed to bilateral esophageal perforations. This association is not well studied; however, a review of 34 patients with Boerhaave syndrome found 8 with a hiatal hernia, indicating this pathology is of relatively increased frequency in patients with esophageal rupture.7 This idea is supported by the proposed theory that the presence or absence of a hiatal hernia can influence the location of a mucosal tear.8 Further study is warranted to better assess this relationship because the presence of a hiatal hernia could heighten suspicion for Boerhaave syndrome in a patient with an otherwise equivocal presentation.
Mortality rates from Boerhaave syndrome have improved compared with previous decades, given the advances in treatment and imaging.9 Fluoroscopic esophagography was standard for years, which has now changed to CT.2,8,10 The literature comparing these 2 modalities in diagnosing Boerhaave syndrome suggest a higher sensitivity and negative predictive value of CT in comparison with fluoroscopic esophagography.11 Studies have documented 100% sensitivity and 100% negative predictive value with CT compared with 73.3% and 95.7%, respectively, for fluoroscopic esophagography, for identifying esophageal perforation.12,13 Importantly, on multiple CTs, our patient did not have convincing evidence of pneumomediastinum but did have a definitive diagnosis made by a swallow study.4,14
Current treatment approaches for Boerhaave syndrome include conservative, endoscopic, or surgical management, with mortality rates documented as 75%, 100%, and 81%, respectively.11 Ongoing debates exist regarding best practices based on a patient's presenting clinical scenario, with the current literature suggesting that endoscopic therapy be reserved for early presenters, those with a defect less than 1 cm and without evidence of sepsis, thus indicating a promising role for endoscopy in a carefully selected population with Boerhaave syndrome.15,16
Boerhaave syndrome is a life-threatening condition with several modalities for diagnosis and treatment. Its presentation can be variable, but even in those with atypical preceding symptoms, it is important to keep in the differential because of varying sensitivities and predictive values of imaging tests. Multiple treatment options exist, but a minimally invasive endoscopic approach, perhaps with endoluminal stent placement, clip closure, or suturing, should be considered in the right patient population.
Author contributions: D. Chalikonda, J. Yoo, and D. Johnson wrote the manuscript. C. Tofani edited the manuscript. D. Chalikonda is the article guarantor.
Financial disclosure: None to report.
Informed consent was obtained for this case report.
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© 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.
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