Mallory-Weiss Syndrome (Mallory-Weiss Tear)

Mallory-Weiss syndrome (MWS) is defined by the presence of longitudinal mucosal lacerations in the distal esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus and proximal stomach Stomach The stomach is a muscular sac in the upper left portion of the abdomen that plays a critical role in digestion. The stomach develops from the foregut and connects the esophagus with the duodenum. Structurally, the stomach is C-shaped and forms a greater and lesser curvature and is divided grossly into regions: the cardia, fundus, body, and pylorus. Stomach, which are usually associated with any action that provokes a sudden rise in intraluminal esophageal pressure, such as forceful or recurrent retching, vomiting, coughing, or straining. Hematemesis results from bleeding from submucosal blood vessels and is self-limited in 80%–90% of patients. Treatment includes gastric acid suppression, endoscopic intervention, and angiotherapy if there is active bleeding. Blood transfusions and surgery are not usually required.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Epidemiology and Pathogenesis

Epidemiology

  • 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 Gastrointestinal bleeding Gastrointestinal bleeding (GIB) is a symptom of multiple diseases within the gastrointestinal (GI) tract. Gastrointestinal bleeding is designated as upper or lower based on the etiology's location to the ligament of Treitz. Depending on the location of the bleeding, the patient may present with hematemesis (vomiting blood), melena (black, tarry stool), or hematochezia (fresh blood in stools). Gastrointestinal Bleeding

Risk factors

  • Alcohol use disorder Alcohol use disorder Alcohol is one of the most commonly used addictive substances in the world. Alcohol use disorder (AUD) is defined as pathologic consumption of alcohol leading to impaired daily functioning. Acute alcohol intoxication presents with impairment in speech and motor functions and can be managed in most cases with supportive care. 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 Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. 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 Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus. Increased thoracic (and therefore increased esophageal intraluminal) pressure transmitted from the abdomen, or prolapse of the stomach Stomach The stomach is a muscular sac in the upper left portion of the abdomen that plays a critical role in digestion. The stomach develops from the foregut and connects the esophagus with the duodenum. Structurally, the stomach is C-shaped and forms a greater and lesser curvature and is divided grossly into regions: the cardia, fundus, body, and pylorus. Stomach into the esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. 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 Back pain Back pain is a common complaint among the general population and is mostly self-limiting. Back pain can be classified as acute, subacute, or chronic depending on the duration of symptoms. The wide variety of potential etiologies include degenerative, mechanical, malignant, infectious, rheumatologic, and extraspinal causes. 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
    • 2nd 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 Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus resulting from a full-thickness tear, which requires immediate surgery to lessen the risk of mediastinitis Mediastinitis Mediastinitis refers to an infection or inflammation involving the mediastinum (a region in the thoracic cavity containing the heart, thymus gland, portions of the esophagus, and trachea). Acute mediastinitis can be caused by bacterial infection due to direct contamination, hematogenous or lymphatic spread, or extension of infection from nearby structures. Mediastinitis and sepsis Sepsis Organ dysfunction resulting from a dysregulated systemic host response to infection separates sepsis from uncomplicated infection. The etiology is mainly bacterial and pneumonia is the most common known source. Patients commonly present with fever, tachycardia, tachypnea, hypotension, and/or altered mentation. Sepsis and Septic Shock. 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 Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea.
  • Esophageal varices: may present with a life-threatening GI bleed. Occurs secondary to portal hypertension Portal hypertension Portal hypertension is increased pressure in the portal venous system. This increased pressure can lead to splanchnic vasodilation, collateral blood flow through portosystemic anastomoses, and increased hydrostatic pressure. There are a number of etiologies, including cirrhosis, right-sided congestive heart failure, schistosomiasis, portal vein thrombosis, hepatitis, and Budd-Chiari syndrome. Portal Hypertension, usually caused by cirrhosis Cirrhosis Cirrhosis is a late stage of hepatic parenchymal necrosis and scarring (fibrosis) most commonly due to hepatitis C infection and alcoholic liver disease. Patients may present with jaundice, ascites, and hepatosplenomegaly. Cirrhosis can also cause complications such as hepatic encephalopathy, portal hypertension, portal vein thrombosis, and hepatorenal syndrome. Cirrhosis. May coexist with MWS.

References

  1. Guelrud M. Mallory Weiss Syndrome. UpToDate. Retrieved on July 24, 2020 from: https://www.uptodate.com/contents/mallory-weiss-syndrome#H2568211429
  2. Adler DG. Mallory Weiss Tear. BMJ Best Practice. Last updated: March 19, 2018, Retrieved on July 24, 2020 from: https://bestpractice.bmj.com/topics/en-gb/1145/pdf/1145/Mallory-Weiss%20tear.pdf
  3. Corral JE, Keihanian T, Kröner PT, et al. Mallory Weiss syndrome is not associated with hiatal hernia: A matched case-control study. Scand. J. Gastroenterol. 2017 Apr; 52(4):462-464.
  4. Kortas DY, Haas LS, Simpson WG, Nickl NJ 3rd, Gates LK Jr. Mallory-Weiss tear: Predisposing factors and predictors of a complicated course. Am J Gastroenterol. 2001;96(10):2863-2865. doi:10.1111/j.1572-0241.2001.04239.x

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