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What is the frequency of atypical presentations of Boerhaave's syndrome?
J. Henderson (Ottawa)
This classic presentation and catastrophic course of spontaneous esophageal perforation is that described by Hermann Boerhaave in 1724 [1, 2], and the characteristic symptoms of vomiting, chest pain, and surgical emphysema were emphasized by Mackler [3] in 1950 (Table I).
When such a clinical picture consistent with spontaneous esophageal perforation is present, it may be sufficiently informative for the diagnosis to be made over the telephone by an astute consultant [4]. However, in a collective review Loop and Groves [5] remarked that although Mackler's triad is pathognomonic of spontaneous esophageal perforation, in their experience at least one-third of spontaneous perforations were atypical. Further to this point, Walker et al. [6] found that less than half of their patients presented in typical fashion. Similarly, Pate et al. [7] in their review of a 30-year experience, concluded that classic features were so frequently absent, that reliance on this clinical picture could result in substantial error or delay in diagnosis (Table II).
Complete disruption of the esophagus with gross pleural contamination such as was found in Boerhaave's original case is very rare [8], and represents one extreme of spontaneous esophageal perforation, with very small perforations confined to the mediastinum at the other extreme. In the exceptional circumstances of a small and confined leak, symptoms may be absent or very mild [9, 10].
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