A 27-Year-Old Man With Hyperemesis and Hematemesis

Gerard J. Fitzmaurice, BSc, MBBCh BAO; Robin Brown, MD, FRCS; Mark E. O'Donnell, DSEM, MFSEM, MRCS; Fionnuala Mone, MBBCh BAO; Angela McGreevy, MBBCh BAO

Disclosures

June 27, 2017

The clinical presentation of Boerhaave syndrome typically includes a history of nausea and vomiting, followed by severe lower thoracic and epigastric pain. The pain may radiate to the back or to the left shoulder, and it may be worsened by swallowing. Hematemesis is typically not a symptom with esophageal rupture, and this can help to differentiate it from a Mallory-Weiss tear. Shortness of breath can also be a common symptom, resulting from pleuritic chest pain or a pleural effusion.[3]

The Mackler triad is the classic presentation of vomiting, lower chest pain, and subcutaneous emphysema that is seen in Boerhaave syndrome[2,3]; however, this sign is seen only in approximately one half of patients at the initial presentation (typically in those who present later).[3] Other classic findings include tachypnea and abdominal rigidity.[3] Atypical findings include peripheral cyanosis, hoarseness caused by involvement of the recurrent laryngeal nerve, tracheal shift, cervical vein distention, and proptosis.[3] Nonspecific findings include tachycardia, diaphoresis, fever, and hypotension, especially as the condition evolves.[3]

The Hamman crunch, a crackling sound heard on auscultation of the chest, demonstrates pneumomediastinum and can be present in 20% of patients.[3] More advanced stages of rupture typically present as sepsis with progression toward multiorgan failure.[3] Despite these symptoms and signs, the clinical presentation can vary, and often the classic triad of symptoms and signs (ie, the Mackler triad) can be absent, which can make establishing the diagnosis difficult.[5,6,7] This multitude of presenting symptoms and clinical signs leads to a wide differential diagnosis, including acute coronary syndrome, aortic dissection, Mallory-Weiss tear, pericarditis, acute pancreatitis, and spontaneous pneumothorax.[3]

The initial workup should involve basic laboratory investigations; however, these tests are often nonspecific and may simply show elevated inflammatory markers and, perhaps, deranged liver function (in keeping with significant alcohol intake). An early ECG is mandatory to exclude myocardial infarction. Erect chest radiography is the initial radiographic investigation; as many as 90% of patients have an abnormal finding after esophageal perforation.[2,3]

The most common abnormality is a unilateral pleural effusion (usually on the left side), which reflects the fact that most ruptures affect the left lower esophagus.[3] Other findings can include pneumothorax, hydropneumothorax, pneumomediastinum, subcutaneous emphysema, or mediastinal widening.[3]

CT allows visualization of the surrounding structures to assist with the elimination of other diagnoses, and it can demonstrate periesophageal air, which can be suggestive of a perforation[2,3]; however, the mainstay of investigation used to determine the exact location and size of an esophageal perforation is an esophageal swallow or esophagraphy with, preferably, water-soluble contrast.[8,9] Esophagraphy has a sensitivity of 90%, but it has a false-negative result in 20% of cases.[3]

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