Mallory-Weiss syndrome (MWS) is characterised by upper gastrointestinal bleeding (UGIB) from mucosal lacerations in the upper gastrointestinal tract (GIT) (usually at the gastrooesophageal junction or gastric cardia). Mallory and Weiss described the syndrome in 1929 in patients retching and vomiting after an alcoholic binge.

MWS may also occur with other events causing a sudden rise in intragastric pressure or gastric prolapse into the oesophagus. Acute distension of the nondistensible lower oesophagus causes tearing. It is a feature of about 10% (ranging from 1% to 15%) of upper gastrointestinal bleeds and causes significant hypovolaemia in about 10% of these. There appears to be a trend towards less associated blood loss and lower mortality. It is often associated with hiatus hernia and is also associated with alcoholism and dialysis.

Epidemiology

In recent years MWS may have become more frequent.¹

• The incidence of UGIB is between 47 and 116 per 100,000 population (most from ulcers)²
• MWS occurs in about 10% of upper gastrointestinal bleeds (between about 5 and 12 per 100,000 population)
• It occurs more often in men (between 2 and 4 times more common)
• There is a wide age range
• It is most common between age 40 and 50 years

Presentation

History

• The classic presentation is of haematemesis following a bout of retching or vomiting.
• However in 50% of cases haematemesis occurs with the first vomit.
• Only about 1/3 of cases have the classic presentation above. Other symptoms include melaena, haematochezia, syncope, and abdominal pain.
• Excessive alcohol ingestion is reported in between 40% and 75% of cases.³
• Aspirin ingestion is associated with 30% of cases.
• There may be clues as to the cause or precipitating factors.
  • Hiatus hernia is a predisposing factor (35-100% of patients with MWS). During retching or vomiting, the transmural pressure gradient is greater within the hiatus hernia than the rest of the stomach.
  • Other precipitating factors include retching, vomiting, straining, hiccuping, coughing, blunt abdominal trauma, cardiopulmonary resuscitation and even primal scream therapy.
  • Other gastrointestinal diseases (gastroenteritis, gastric outlet obstruction, malrotation, volvulus).
  • Hyperemesis gravidarum.⁴
  • Hepatitis (causes vomiting in 10-20% of patients).
  • Biliary disease (gallstones and cholecystitis).
  • Renal disease - vomiting is often associated with diseases affecting the kidneys (from urinary tract infections to renal failure).
  • Raised intracranial pressure may lead to vomiting (particularly in children).
  • Cyclical vomiting syndrome.
  • Other causes (drugs, severe diabetic ketoacidosis).
  • Iatrogenic tears are uncommon even with a high incidence of retching during endoscopy. The reported prevalence is 0.07-0.49%. It has also been reported in transoesophageal echocardiography.^5,6
  • No apparent precipitating factor can be identified in about 25% of patients.

Examination

• There are no specific physical signs.
• An assessment of the degree of blood loss should be made. The Rockall scoring system can be used to assess UGIB.⁷ A score of less than 3 is associated with an excellent prognosis and 8 or above an extremely poor prognosis. MWS is usually associated with a score of 3 or less. Predicting which patients with MWS are at greatest risk is largely on empirical grounds.³

Differential diagnosis

Haematemesis as a symptom has quite a long differential diagnosis. The following are important to consider (particularly with the retching and sudden bright bleeding associated with MWS):

• Boerhaave's syndrome (oesophageal rupture)⁸
• Oesophagitis
• Peptic ulcers (particularly gastric ulcers)

Investigations

Endoscopy is the primary diagnostic investigation. Other relevant investigations include:

• Full blood count, including haematocrit to assess the severity of the initial bleeding episode and to monitor patients.
• Coagulation studies and platelet counts to detect coagulopathies and thrombocytopenias (routine platelet count, prothrombin time, and activated partial thromboplastin time).
• Urea, creatinine, and electrolyte levels (to guide intravenous fluid therapy).
• Cross matching/ blood grouping and antibody screen (potential blood transfusion).
• Electrocardiogram and cardiac enzymes (may be indicated if myocardial ischaemia is suspected).

Associated diseases

• Alcohol intoxication
• Aspirin ingestion
• Biliary disease
• Cyclical vomiting syndrome
• Diabetic ketoacidosis
• Gastroenteritis
• Hiatus hernia
• Hyperemesis gravidarum
• Hepatitis (cirrhosis carries poor prognosis)⁹
• Raised intracranial pressure
• Renal disease

Management

Initial management is described in the article on Upper Gastrointestinal Bleeding.

Initial assessment and management

• Resuscitation is a priority - maintain airway, provide high flow oxygen, correct fluid losses (place 2 wide bore cannulae and also send bloods at the same time). Initial fluid resuscitation may be with crystalloids or colloids; give intravenous blood when 30% of circulating volume is lost.¹⁰ Major haemorrhage protocols should be in place.¹⁰
• Once patient is more stable - take a history and perform an examination as above, identify severity of blood loss and treat any co-morbid conditions.
• MWS usually follows a benign course but occasionally endoscopic treatment is required to stop bleeding. Sclerotherapy, electrocoagulation and YAG laser treatment can all be used to arrest bleeding. Banding and clipping techniques have also been used.^11,12

Endoscopy¹⁰

• Ideally endoscopy should be performed within 24 hours, as tears heal rapidly and may not be readily apparent at endoscopy after 2-3 days. PPI use is not recommended prior to diagnosis by endoscopy.
• 5 to 35% of patients require some form of intervention, usually endoscopic.
• Most patients (>80%) present with a single tear. The tear is usually just below the gastrooesophageal junction on the lesser curvature of the stomach.
• Tears are usually associated with other mucosal lesions (83% of patients). These may contribute to bleeding and/or cause the retching and vomiting. Endoscopic examination should be thorough because such co-existing lesions are common.
• Several endoscopic modalities are effective for treating a bleeding Mallory-Weiss tear. The choice usually depends on the endoscopist's experience and training.
• Fasting is restricted to haemodynamically unstable patients and to those who require repeat endoscopy.
• Patients can resume oral intake following endoscopy (starting with a liquid diet and advancing as tolerated to a normal diet) within 48 hours (unless nausea or vomiting is a problem).

Post-initial endoscopy¹⁰

Calculate the full (post-endoscopic) Rockall score as described in upper gastrointestinal bleeding article - score <3 is associated with low risk of re-bleeding or death and can be considered for early discharge, whereas a score >3 indicates patients need further close observation as an inpatient.

• Careful monitoring is needed after endoscopy for UGIB (pulse, blood pressure, urine output). It is imperative to identify re-bleeding or continuing bleeding.
• Patients with clinical risk factors for re-bleeding (for example portal hypertension, coagulopathy) comprise about 10% of cases. These and those with certain endoscopic findings (non-bleeding visible vessel, pigmented protuberance, or adherent clot) should be observed for 48 hours.
• If patients are stable 4-6 hours after endoscopy they should be put on a light diet as there is no benefit in continued fasting.
• If re-bleeding occurs, it usually takes place within 48 hours. Shock at initial manifestation and active bleeding at endoscopy are independent risk factors predicting recurrent bleeding in patients with MWS.¹³

Complications

These relate to:

• Symptoms:
  • Vomiting (hypokalaemia and other metabolic disturbance, aspiration pneumonia, perforation and mediastinitis)
• Severity of bleeding:
  • Hypovolaemic shock, and death (very rare with good care)
  • Myocardial ischaemia or infarction
• Co-morbidities:
  • Myocardial ischaemia (precipitating, for example, myocardial infarction)
  • Hepatitis (precipitating, for example, liver failure)
  • Renal disease (precipitating, for example, renal failure)
  • Diabetes (worsening control and diabetic coma)
• Treatment or investigation:
  • Endoscopy (mediastinitis, aspiration pneumonia, perforation or aggravation of bleeding)
  • Angiotherapy (organ ischaemia and infarction, aggravation of bleeding)

Prognosis

The prognosis is generally excellent. Most patients usually stop bleeding spontaneously and the tears heal rapidly, usually within 48-72 hours. Associated diseases may have a significant effect on prognosis; for example, cirrhosis carries a very poor prognosis.⁹

Prevention

Recurrence is rare but it makes sense to counsel patients about precipitating factors (for example binge drinking, alcohol consumption, excessive straining and lifting, violent coughing) that may lead to a recurrence and are generally hazardous to health.