Boerhaave syndrome

Description

Boerhaave syndrome: spontaneous oesophageal rupture resulting from sudden increased intra-oesophageal pressure. Most commonly associated with emesis with incomplete cricopharyngeal relaxation.

The classic ‘‘Mackler’s triad’’ of esophageal rupture—chest pain, vomiting, and subcutaneous emphysema—has been estimated to occur in only 14-25% of cases. Up to two-thirds of patients may demonstrate subcutaneous emphysema on physical examination, whereas Hamman’s sign (a crunching sound synchronous with each heart beat) is rare. Other nonspecific signs include tachypnea and tachycardia, with 82% of patients meeting systemic inflammatory response syndrome criteria

Oesophageal contents may leak into the mediastinum and pleural space resulting in mediastinitis and a mortality of 40–90% if not recognised and treated promptly

Left posterolateral distal oesophagus is most commonly the site of rupture. Radiographic imaging (CXR, CT scan) commonly reveal pneumomediastinum, mediastinal fluid, and pleural effusion

History

1724 – Baron Jan von Wassenaer, the Grand Admiral of the Dutch Fleet and Prefect of Rhineland, ingested an emetic after eating a large meal. The forceful vomiting was associated with a tearing pain in the chest and the Baron died 24 hours later.

The Baron’s demise was described in minute detail by Dutch physician Herman Boerhaave as a rupture of the esophagus with the exit of the gastric content into the mediastinum [PMC199852]

Boerhaave: Advice for future cases

…when the disease I described occurred we could not diagnose it by its symptoms, but if it had been recognized it would have ignored every remedy. When it recurs again it can be recognized with the help of this description, but cannot be remedied by any assistance of the medical profession.

Boerhaave, 1724

Associated Persons

• Herman Boerhaave (1668–1738)
• Saul Allen Mackler (1913 – 2007)

References

• Boerhaave H. Atrocis, nec descripti prius, morbi historia. 1724 [English translation: History of a grievious disease not previously described. Derbes VJ, Mitchell RE Jr 1955]
• Mackler SA. Spontaneous rupture of the esophagus. An experimental and clinical study. Surgery, Gynecology & Obstetrics 1952; 95: 345-356.
• Kanne JP, Rohrmann CA Jr, Lichtenstein JE. Eponyms in radiology of the digestive tract: historical perspectives and imaging appearances. Part I. Pharynx, esophagus, stomach, and intestine. Radiographics. 2006 Jan-Feb;26(1):129-42. [PMID 16418248]
• Young CA, Menias CO, Bhalla S, Prasad SR. CT features of esophageal emergencies. Radiographics. 2008 Oct;28(6):1541-53. [PMID 18936020]
• Neff C, Lawson DW. Boerhaave syndrome: interventional radiologic management. AJR Am J Roentgenol. 1985 Oct;145(4):819-20. [PMID 3876005]
• Katabathina VS, Restrepo CS, Martinez-Jimenez S, Riascos RF. Nonvascular, nontraumatic mediastinal emergencies in adults: a comprehensive review of imaging findings. Radiographics. 2011 Jul-Aug;31(4):1141-60. [PMID 21768244]
• Ng P. An unusual case of chest pain. BMJ Case Rep. 2013; 2013: bcr2013008849. [PMC3604495]
• Young P. Roast duck and juniper beer