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Boerhaave's Syndrome: Still a Diagnostic and Therapeutic Challenge in the 21st Century

DOI: 10.1155/2013/161286

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Abstract:

Boerhaave's syndrome is a rare but potentially fatal condition characterised by a transmural tear of the distal oesophagus induced by a sudden increase in pressure. Diagnosis is challenging as the classic triad of vomiting, abdominal or chest pain, and subcutaneous emphysema is absent in many patients. Management is multidisciplinary and relies on rapid, distinct, and repeated imaging. Treatment has not been standardised and may be conservative, endoscopic, or surgical. We present a typical case which illustrates possible diagnostic pitfalls and the therapeutic conundrum surrounding management of the syndrome. Based on time of presentation and eventual presence of sepsis, a therapeutic algorithm is proposed. 1. Introduction Boerhaave’s syndrome, first described in the 18th century by the Dutch physician Herman Boerhaave, refers to an oesophageal tear caused by an abrupt rise of intraluminal pressure [1]. It is a rare but life-threatening condition that requires urgent diagnosis and treatment. If treatment is delayed, severe and potentially lethal complications such as mediastinitis, pleural empyema, septic shock, and multiple organ failure may develop. A high index of suspicion is imperative for timely diagnosis and to assure well-selected radiological and endoscopic investigations. Prompt and adequate therapy reduces mortality. Some centers advocate early and extensive surgery as the cornerstone of treatment, yet others prefer a more conservative, endoscopic, or less invasive surgical approach. Such experience-based difference in attitude likely explains the lack of well-established treatment guidelines. 2. Case Report A 41-year-old man with an extensive medical history, including Child-Pugh B liver cirrhosis and chronic pancreatitis, consulted his family doctor with progressive dyspnea, retrosternal pain radiating to the back, repeated vomiting, and fever for 3 days. On admission at the emergency ward, the patient appeared confused and agitated. Core temperature was 38.5°C, heart rate was regular at 150?beats/min, and respiratory rate was 40?breaths/min. Physical examination revealed a supple but tender abdomen, normal peristalsis, and muffled breath sounds over the right lung. Blood analysis showed macrocytic anemia, 18500?leukocytes/mm3, normal enzymes, a C-reactive protein (CRP) of 303?mg/L, and a lactate level of 7.4?mmol/L. Chest X-ray showed a silhouette sign over the right heart border and small mediastinal radiolucent streaks of air (Figure 1). A contrast-enhanced computed tomography (CT) scan of the thorax confirmed the pneumomediastinum

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