Boerhaave syndrome

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Classification according to ICD-10
K22.3 Esophageal perforation. Esophageal
rupture
ICD-10 online (WHO version 2019)

As Boerhaave syndrome is called a rare condition in which a spontaneous esophageal perforation , that is a spontaneous tear ( rupture ) through all layers of the wall of the esophagus (gullet) is formed. It was first described by Hermann Boerhaave in 1724 .

Boerhaave's syndrome occurs as a result of a sudden and sharp increase in intra-oesophageal pressure, mainly as a result of massive vomiting . The occurrence of Boerhaave's syndrome is an emergency situation. Immediate surgical treatment of the perforation is by far the most important therapeutic option. Untreated spontaneous esophageal perforation has a mortality rate of over 90 percent.

Boerhaave's syndrome is differentiated from Mallory-Weiss syndrome , in which the mucous membrane at the junction of the esophagus and stomach is perforated, and from iatrogenic and traumatic esophageal perforations.

frequency

Boerhaave syndrome is a very rare disease for which there is little epidemiological data. By 1990 a total of about 900 cases had been recorded in the medical literature worldwide. About 10 to 15 percent of all esophageal perforations are due to this syndrome. There is a clear preference for the male sex. In about 80 percent of the cases, men develop Boerhaave syndrome. The maximum age is between 20 and 40 years and makes up about two thirds of the cases. Boerhaave syndrome rarely occurs in children, especially newborns.

Causes and Pathogenesis

Boerhaave's syndrome usually results from a sudden, strong and unexpected increase in pressure in the esophagus with simultaneous negative pressure within the chest (intrathoracic pressure). The speed of the pressure increase probably plays a more important role than the absolute amount of the pressure increase. In experimental studies on the esophagus of cadavers, it was found that sudden increases in pressure of 150 to 200 mmHg lead to perforation of the esophagus in the lower (distal third) of the esophagus. Most perforations - over 90 percent - are located in the lower (distal) third of the esophagus dorsolaterally (left rear), as anatomically the least muscular resistance is here.

In most cases, spontaneous esophageal perforation is preceded by massive vomiting. It is therefore also known as emetogenic esophageal perforation . There are different statements in the literature about other causes. In addition to vomiting, strong straining and vigorous physical exertion are also considered possible causes.

Spontaneous esophageal perforation can be promoted by a variety of diseases such as esophagitis and gastroesophageal reflux disease . Chronic alcohol abuse is the most important predisposing factor.

clinic

The classic symptom triad (so-called Mackler triad) consists of

  1. explosive vomiting
  2. severe retrosternal pain ( annihilation pain )
  3. Cutaneous emphysema or mediastinal emphysema .

Other symptoms are

diagnosis

In X-rays to air sickles show as a sign of the air outlet under both diaphragmatic domes or air leakage into the mediastinum .

The following follow-up examinations can be useful as part of the diagnosis:

  • Esophagography : X-ray contrast agent examination in different planes to detect a tear caused by contrast agent leakage into the mediastinum (lower risk)
  • Esophagoscopy : Esophagoscopy as an endoscopic , diagnostic and, under favorable circumstances, therapeutic method through direct suturing of the tear. As a complication of esophagoscopy, there is a risk of the tear expanding as a result of the procedure.

Differential diagnoses

therapy

The defect is sutured via a thoracotomy - or in the case of deeper lesions, a laparoscopy - and the suture is possibly covered and stabilized by plastic covering with tissue from nearby (e.g. greater omentum , pleura , diaphragm ).

As an accompanying therapy, broad-spectrum antibiosis should be initiated pre- and post-operatively . Intensive care monitoring is required.

forecast

The mortality rate in Boerhaave syndrome is around 20–40%.

Individual evidence

  1. ↑ A. Matsuda et al. a .: Boerhaave syndrome treated conservatively following early endoscopic diagnosis: a case report. In: J Nippon Med Sch. 2006 Dec; 73 (6), pp. 341-345. PMID 17220586
  2. ↑ a b H. J. Mota et al. a .: Postemetic rupture of the esophagus: Boerhaave's syndrome. In: J Bras Pneumol. 2007 Aug; 33 (4), pp. 480-483. PMID 17982542 .
  3. ↑ a b D. Decker, U. Herrmann: Rare cause of pleural empyema - the Boerhaave syndrome . ( Memento of the original from March 4, 2016 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. (PDF) In: Ärzteblatt Sachsen. 2/2001: pp. 62-64. @1@ 2Template: Webachiv / IABot / www.slaek.de
  4. ↑ a b Michael J. Lentze, Jürgen Schaub, Franz-Josef Schulte (ed.): Pediatrics. Basics and practice. 2nd Edition. Springer Verlag, 2002, ISBN 3-540-43628-6 , p. 860.
  5. ↑ FA Herbella et al. a .: Eponyms in esophageal surgery, part 2 . In: Dis Esophagus. 2005; 18 (1), pp. 4-16. PMID 15773835
  6. ↑ a b J. R. Siewert, Felix Harder, M. Rothmund (ed.): Practice of visceral surgery - gastroenterological surgery. 1st edition. Springer Verlag, 2002, ISBN 3-540-65950-1 , p. 330 ff.
  7. ^ A b Wolfgang Remmele: Pathology Volume 2: Digestive Tract . 2nd Edition. Springer-Verlag, 2001, ISBN 3-540-60119-8 , p. 105.
  8. ↑ a b c d A. M. Nia, J. Abel, N. Semmo, N. Gassanov, F. He: 86-year-old patient with vomiting and loss of consciousness: the Mackler triad . In: Dtsch. Med. Wochenschr. tape 136 , no. 36 , September 2011, p. 1779-1780 , doi : 10.1055 / s-0031-1286100 , PMID 21882132 .