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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: Anatomy 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: Anatomy, which are usually associated with any action that provokes a sudden rise in intraluminal esophageal pressure, such as forceful or recurrent retching, vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia, coughing, or straining. Hematemesis results from bleeding from submucosal blood vessels and is self-limited in 80%–90% of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship. Treatment includes gastric acid Gastric acid Hydrochloric acid present in gastric juice. Gastroesophageal Reflux Disease (GERD) suppression Suppression Defense Mechanisms, endoscopic intervention, and angiotherapy if there is active bleeding. Blood transfusions Blood transfusions The introduction of whole blood or blood component directly into the bloodstream. Transfusion Products and surgery are not usually required.

Last updated: Oct 21, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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 Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship
  • 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 Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship. Mallory-Weiss syndrome (MWS) may coexist with esophageal varices.
  • Any event that provokes a sudden rise in the pressure gradient Pressure gradient Vascular Resistance, Flow, and Mean Arterial Pressure across the gastroesophageal junction Gastroesophageal junction The area covering the terminal portion of esophagus and the beginning of stomach at the cardiac orifice. Esophagus: Anatomy (e.g., forceful or recurrent retching, vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia, hiccupping [singultus], violent coughing spasms Spasms An involuntary contraction of a muscle or group of muscles. Spasms may involve skeletal muscle or smooth muscle. Ion Channel Myopathy, blunt abdominal trauma)
  • Many patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship have no risk factors (23% in a study by Kortas DY, 2001).
  • Conflicting studies exist regarding hiatal hernia Hiatal hernia Stomach herniation located at or near the diaphragmatic opening for the esophagus, the esophageal hiatus. Congenital Diaphragmatic Hernias as a risk factor: A large study in 2017 showed no correlation Correlation Determination of whether or not two variables are correlated. This means to study whether an increase or decrease in one variable corresponds to an increase or decrease in the other variable. Causality, Validity, and Reliability (Corral, 2017).

Pathogenesis

  • The pathogenesis has not been entirely elucidated and several mechanisms are possible.
  • Retching and vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia normally cause a rapid increase in intraabdominal pressure, which causes a rise in intragastric pressure; this pressure overcomes the normally high lower esophageal sphincter Lower Esophageal Sphincter Esophagus: Anatomy 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: Anatomy. Normal autonomic reflexes cause the upper esophageal sphincter Upper esophageal sphincter The structure at the pharyngoesophageal junction consisting chiefly of the cricopharyngeus muscle. It normally occludes the lumen of the esophagus, except during swallowing. Esophagus: Anatomy (UES) to relax at this point and the gastric contents to be expelled as part of the normal vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia 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: Anatomy. 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: Anatomy 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: Anatomy 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 Surgical Emergency Acute Abdomen.

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 Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways
  • May be asymptomatic

Diagnosis 

Diagnosis is established by endoscopy Endoscopy Procedures of applying endoscopes for disease diagnosis and treatment. Endoscopy involves passing an optical instrument through a small incision in the skin i.e., percutaneous; or through a natural orifice and along natural body pathways such as the digestive tract; and/or through an incision in the wall of a tubular structure or organ, i.e. Transluminal, to examine or perform surgery on the interior parts of the body. Gastroesophageal Reflux Disease (GERD), which shows a longitudinal tear (usually single) limited to the mucosa and submucosa at the gastroesophageal junction Gastroesophageal junction The area covering the terminal portion of esophagus and the beginning of stomach at the cardiac orifice. Esophagus: Anatomy.

Management

  • Acute management of an upper gastrointestinal (GI) bleed:
    • Assess hemodynamic stability: Administer fluids and transfuse packed red blood cells Red blood cells Erythrocytes, or red blood cells (RBCs), are the most abundant cells in the blood. While erythrocytes in the fetus are initially produced in the yolk sac then the liver, the bone marrow eventually becomes the main site of production. Erythrocytes: Histology (PRBCs) if needed.
    • IV proton pump Pump ACES and RUSH: Resuscitation Ultrasound Protocols inhibitors
    • Upper endoscopy Endoscopy Procedures of applying endoscopes for disease diagnosis and treatment. Endoscopy involves passing an optical instrument through a small incision in the skin i.e., percutaneous; or through a natural orifice and along natural body pathways such as the digestive tract; and/or through an incision in the wall of a tubular structure or organ, i.e. Transluminal, to examine or perform surgery on the interior parts of the body. Gastroesophageal Reflux Disease (GERD)
  • In 80% of cases, bleeding stops spontaneously. However, for actively bleeding lesions, treatment options include:
    • 1st line: endoscopic interventions, including injection of epinephrine Epinephrine The active sympathomimetic hormone from the adrenal medulla. It stimulates both the alpha- and beta- adrenergic systems, causes systemic vasoconstriction and gastrointestinal relaxation, stimulates the heart, and dilates bronchi and cerebral vessels. Sympathomimetic Drugs, electrocoagulation, or band ligation Ligation Application of a ligature to tie a vessel or strangulate a part. Esophageal Atresia and Tracheoesophageal Fistula
    • 2nd line: arteriography with embolization Embolization A method of hemostasis utilizing various agents such as gelfoam, silastic, metal, glass, or plastic pellets, autologous clot, fat, and muscle as emboli. It has been used in the treatment of spinal cord and intracranial arteriovenous malformations, renal arteriovenous fistulas, gastrointestinal bleeding, epistaxis, hypersplenism, certain highly vascular tumors, traumatic rupture of blood vessels, and control of operative hemorrhage. Gastrointestinal Bleeding if endoscopic interventions fail; vasopressin infusion used if embolization Embolization A method of hemostasis utilizing various agents such as gelfoam, silastic, metal, glass, or plastic pellets, autologous clot, fat, and muscle as emboli. It has been used in the treatment of spinal cord and intracranial arteriovenous malformations, renal arteriovenous fistulas, gastrointestinal bleeding, epistaxis, hypersplenism, certain highly vascular tumors, traumatic rupture of blood vessels, and control of operative hemorrhage. Gastrointestinal Bleeding not possible
    • 3rd line: surgery if angiography Angiography Radiography of blood vessels after injection of a contrast medium. Cardiac Surgery 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: Anatomy 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 Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by hypotension despite adequate fluid infusion, it is called septic shock. Sepsis and Septic Shock. The pathogenic mechanism is identical to that of MWS (sudden increased intraluminal esophageal pressure due to forceful vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia or retching). Symptoms may include subcutaneous emphysema Emphysema Enlargement of air spaces distal to the terminal bronchioles where gas-exchange normally takes place. This is usually due to destruction of the alveolar wall. Pulmonary emphysema can be classified by the location and distribution of the lesions. Chronic Obstructive Pulmonary Disease (COPD) with crepitus Crepitus Osteoarthritis on examination, pneumomediastinum Pneumomediastinum Mediastinitis, odynophagia Odynophagia Epiglottitis, 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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