Boerhaave Syndrome
Uploaded by
Francois Donatien De SadeBoerhaave Syndrome
Uploaded by
Francois Donatien De SadeAnswer
Boerhaave syndrome: Boerhaave syndrome is a spontaneous transmural
perforation of the esophagus resulting from a sudden rise in intraluminal
pressure caused by an uncoordinated act of forceful vomiting against a closed
cricopharyngeal sphincter. More than 90% of these perforations occur in the left
posterolateral wall of the lower third of the esophagus. The syndrome can also
occur after other spontaneous Valsalva-like maneuvers, such as childbirth,
coughing, straining during a bowel movement, or heavy lifting. Nonspontaneous
causes include iatrogenic perforation associated with endoscopy, ingestion of a
caustic substance, and blunt trauma to the neck and chest.
The classic presentation is that of a middle-aged man with recent dietary and
alcohol overindulgence who, after repeated episodes of retching and vomiting,
feels a sudden onset of severe chest pain in the lower thorax and upper
abdomen. The pain typically radiates to the back or left shoulder as a result of
the intense inflammatory response to the saliva and gastric contents entering
the mediastinum. Other symptoms are neck pain, dysphagia, odynophagia,
respiratory distress, and fever.
On physical examination, nonspecific findings, such as tachycardia,
diaphoresis, fever, hypotension, and generalized abdominal tenderness with
guarding and rebound, are typically noted. If present, subcutaneous mediastinal
and cervical emphysema are particularly helpful in confirming the diagnosis, but
these findings may take time to develop. Pneumomediastinum may manifest as
a crunching sound on chest auscultation during systole; this is known as the
Hamman sign. Septic shock and cardiovascular collapse develop as the
infectious and inflammation progress and are the major causes of mortality.
The differential diagnosis is broad and includes pulmonary embolism, acute
myocardial infarction, aortic dissection, perforated ulcer, acute pancreatitis or
cholecystitis, and pneumothorax. Upright chest radiography is the simplest and
most useful diagnostic test, as it shows abnormalities in 90% of patients. These
abnormalities include pneumomediastinum, left-sided pleural effusion, pleural
infiltrate, pneumothorax, hydropneumothorax, subcutaneous emphysema,
mediastinal widening, and subdiaphragmatic air. Image 1 shows air present in
the mediastinum and outlines of the aortic arch and larynx, as well as
subcutaneous air over the right clavicle with a left-sided effusion.
The diagnosis is established by means of water-soluble contrast esophagraphy
to reveal the location and extent of extravasation. In Image 2, contrast material
is leaking into the distal mediastinum, and a large collection of extraluminal
contrast agent is apparent, consistent with an esophageal tear. However, if
clinical suspicion is high and if imaging with a water-soluble contrast agent fails
to demonstrate a tear, a barium esophagraphy or chest CT may be useful.
Although barium sulfate is superior for identifying small tears, it is not initially
used because it causes inflammation in the mediastinum and pleural cavity.
Endoscopy has a limited role, as small tears are difficult to visualize on this
study. In addition, the insufflation of air required for the procedure can result in
the extension of the perforation and introduce additional air into the
mediastinum.
Prompt diagnosis and early surgical intervention is crucial because mortality
rates rise from 25% at 12 hours to 75% at 24 hours, and nearly 100% at 48
hours. Management includes strict adherence to giving the patient nothing by
mouth, administration of broad-spectrum antibiotics, fluid resuscitation,
nasogastric decompression, and early consultation with a surgeon. Depending
on the location of the tear, a chest or abdominal approach to repair the
perforation is performed, and parenteral nutrition is required. This patient's
esophageal tear was repaired by means of thoracotomy with the placement of 2
chest tubes for further drainage (Image 3), exploratory laparotomy, placement of
a G-tube and a J-tube, and broad-spectrum antibiotic therapy. Eight days later,
a repeat esophagram (Image 4) showed no evidence of an esophageal leak.
For more information on Boerhaave syndrome, see the eMedicine articles
Boerhaave Syndrome and Esophageal Rupture (within the Internal Medicine
specialty) and Esophageal Perforation, Rupture and Tears (within the
Emergency Medicine specialty).
References
Henderson JA, Peloquin AJ: Spontaneous esophageal perforation as a
diagnostic masquerader. Am J Med 1989;86(5):559-67.
Lemke T, Jagminas L: Spontaneous esophageal rupture: a frequently
missed diagnosis. Am Surg 1999;65(5):449-452.
Levy F, Mysko WK, Kelen GD: Spontaneous esophageal perforation
presenting with right-sided pleural effusion. J Emerg Med
1995;13(3):321-5.
Marx JA, ed: Rosen's Emergency Medicine: Concepts and Clinical
Practice. 5th ed. St Louis: Mosby-Year Book; 2002:1236-7.
BACKGROUND
An 88-year-old man who had a myocardial infarction 4 years ago went to bed
with indigestion. Several hours later, he awakened, vomited once, and then felt
the sudden onset of excruciating chest pain radiating to his back and shoulders.
He called for emergency assistance.
On his arrival in the emergency department, the patient's vital signs were an
oral temperature of 97.4°F, a blood pressure of 112/63 mm Hg, a heart rate of
99 beats per minute, a respiratory rate of 24 breaths per minute, and an oxygen
saturation of 95% while he was receiving oxygen 4 L/min by nasal cannula. The
patient, writhing on the gurney and clutching his chest, was yelling, "The pain is
going to my back and shoulders!"
On examination, clear lung sounds were heard on both sides. Cardiac
examination revealed tachycardia without murmurs or rubs. Abdominal
examination revealed mild epigastric tenderness and distension without
guarding or rebound. The patient's bowel sounds were depressed. ECG did not
demonstrate ischemic changes.
The patient was given morphine sulfate, which provided little relief. Before he
was transported for CT to rule out an aortic dissection, portable chest
radiography was performed (see Image).
What are the diagnosis and treatment?
Hint
On further examination, crepitus was palpable along the right side of the
patient's neck and sternum.
Authors: Patricia Rivera, MD, MPH,
Department of Emergency
Medicine, NYU/Bellevue
Emergency Medicine Residency
Program, New York City, NY
Robert Rothberg, MD, Attending
Physician, NYU/Bellevue
Emergency Medicine Residency
Program, New York City, NY
eMedicine Editor: Rick Kulkarni, MD, Attending
Physician, Department of
Emergency Medicine, Olive View
- UCLA Medical Center, Assistant
Professor of Medicine, David
Geffen School of Medicine at
UCLA
