Mallory-Weiss Syndrome (Mallory-Weiss Tear)

• 3 times more common in men than women, rare in children
• History of heavy alcohol use in 40%–80% of patients
• In women of childbearing age, the most common cause is hyperemesis gravidarum.
• Accounts for approximately 8%–15% of upper gastrointestinal bleeding

Risk factors

• Alcohol use disorder: seen in 40%–80% of patients. Mallory-Weiss syndrome (MWS) may coexist with esophageal varices.
• Any event that provokes a sudden rise in the pressure gradient across the gastroesophageal junction (e.g., forceful or recurrent retching, vomiting, hiccupping [singultus], violent coughing spasms, blunt abdominal trauma)
• Many patients have no risk factors (23% in a study by Kortas DY, 2001).
• Conflicting studies exist regarding hiatal hernia as a risk factor: A large study in 2017 showed no correlation (Corral, 2017).

Pathogenesis

• The pathogenesis has not been entirely elucidated and several mechanisms are possible.
• Retching and vomiting normally cause a rapid increase in intraabdominal pressure, which causes a rise in intragastric pressure; this pressure overcomes the normally high lower esophageal sphincter pressure so the gastric contents are released into the esophagus. Normal autonomic reflexes cause the upper esophageal sphincter (UES) to relax at this point and the gastric contents to be expelled as part of the normal vomiting process.
• It is postulated that longitudinal esophageal tears may result from very high intra-abdominal pressures alone, possibly combined with the failure of synchronous relaxation of the UES at the time of expulsion of the gastric contents into the esophagus. Increased thoracic (and therefore increased esophageal intraluminal) pressure transmitted from the abdomen, or prolapse of the stomach into the esophagus at the time of gastric expulsion, may also be the principal pathogenetic mechanisms in some cases.
• Bleeding occurs when the tears involve the submucosal blood vessels. If a tear becomes full thickness and perforates, it is transformed into a much rarer condition, Boerhaave’s syndrome, which is a surgical emergency.

Clinical Presentation and Diagnosis

Clinical presentation

• Typically presents with acute onset hematemesis with a history of non-bloody emesis, retching, or coughing
• Epigastric or back pain
• May be asymptomatic

Diagnosis 

Diagnosis is established by endoscopy, which shows a longitudinal tear (usually single) limited to the mucosa and submucosa at the gastroesophageal junction.

Management

• Acute management of an upper gastrointestinal (GI) bleed:
  • Assess hemodynamic stability: Administer fluids and transfuse packed red blood cells (PRBCs) if needed.
  • IV proton pump inhibitors
  • Upper endoscopy
• In 80% of cases, bleeding stops spontaneously. However, for actively bleeding lesions, treatment options include:
  • 1st line: endoscopic interventions, including injection of epinephrine, electrocoagulation, or band ligation
  • 2ns line: arteriography with embolization if endoscopic interventions fail; vasopressin infusion used if embolization not possible
  • 3rd line: surgery if angiography fails

Differential Diagnosis

• Boerhaave’s syndrome: perforated esophagus resulting from a full-thickness tear, which requires immediate surgery to lessen the risk of mediastinitis and sepsis. The pathogenic mechanism is identical to that of MWS (sudden increased intraluminal esophageal pressure due to forceful vomiting or retching). Symptoms may include subcutaneous emphysema with crepitus on examination, pneumomediastinum, odynophagia, and dyspnea.
• Esophageal varices: may present with a life-threatening GI bleed. Occurs secondary to portal hypertension, usually caused by cirrhosis. May coexist with MWS.