Mallory-Weiss Syndrome

In 1929, Kenneth Mallory and Soma Weiss first described a syndrome characterized by esophageal bleeding caused by a mucosal tear in the esophagus as a result of forceful vomiting or retching. The initial description was associated with alcoholic binging; however, with the advent of endoscopy, Mallory-Weiss tears have been diagnosed in many patients with no antecedent history of alcohol intake. The tear typically occurs after repeated episodes of vomiting or retching, but it may occur after a single incident.[1] Although most written reports of these tears relate to adults, Mallory-Weiss tears also occur in children.

In pediatrics, Mallory-Weiss tears are recognized in children with predisposing medical conditions, including portal hypertension, liver cirrhosis, and severe gastroesophageal reflux disease. The clinical implications in these pediatric patients are noteworthy, especially because these children are predisposed to recurrent upper GI bleeding.[2] The associated risk of morbidity and mortality must also be underscored.

Examples of Mallory-Weiss tears are shown in the images below.

Mallory-Weiss Syndrome. Typical longitudinal mucosal tear with overlying fibrinous exudate extending from the distal esophagus to the gastric cardia. Courtesy of C.J. Gostout, MD.

Mallory-Weiss Syndrome. Retroflexed view of the cardia showing the typical location of the tear with a clean base.

Any disorder that initiates vomiting may result in the development of a Mallory-Weiss tear, which develops as a linear laceration at the gastroesophageal junction because the esophagus and stomach are cylindrical. The cylindrical shape allows longitudinal tears to occur more easily than circumferential tears. These tears have been postulated to occur either by a rapid increase in intragastric pressure and distention, which increases the forceful fluid ejection through the esophagus, or secondary to a significant change in transgastric pressure (ie, difference in pressure across the gastric wall) because negative intrathoracic pressure and positive intragastric pressure leads to distortion of the gastric cardia, resulting in a gastric or esophageal tear. Aside from those patients who present with upper GI bleeding secondary to an alcohol binge, Mallory-Weiss tears occur more commonly in people with hiatal hernias.

Although most cases of Mallory-Weiss tears are self-limiting, patients with severe or recurrent episodes of bleeding that require intensive care therapy and interventional endoscopy have been reported. Typically, these patients have underlying conditions, including portal hypertension and hepatic insufficiency. Although upper GI bleeding is generally assumed to be secondary to varices in these patients, the physician must also be aware of the potential for Mallory-Weiss tears.

Many underlying disorders that cause vomiting and retching result in a Mallory-Weiss tear. Gastrointestinal (GI) diseases associated with Mallory-Weiss tears include the following:

• Bacterial gastroenteritis

• Gastric outlet obstruction

• Ulcers

• Hiatal hernias

• Malrotation

• Volvulus

• Inflammatory conditions of the stomach and intestine

• Hepatitis: Acute inflammation of the liver causes vomiting in 10-20% of patients.

• Cirrhosis

• Biliary tract disease: Although rare in children, these conditions can cause vomiting typically associated with meals and include gallstones, cholecystitis, and biliary cirrhosis.

• Cyclic vomiting syndrome

• Hyperemesis gravidarum: Some women develop hyperemesis gravidarum, a syndrome characterized by persistent severe vomiting and retching, in the first trimester of pregnancy. Gastric dysrhythmias and prolonged small-bowel motility cause the development of hyperemesis gravidarum. Some women lose as much as 10% of their body weight during this period.

Vomiting is often associated with diseases affecting the kidneys, including the following:

• Urinary tract infections

• Kidney stones

• Ureteropelvic junction (UPJ) obstruction

• Renal failure

Intracranial lesions that cause hydrocephalus or increased intracranial pressure may lead to vomiting in children. Most common causes of hydrocephalus include tumors, cysts, and congenital abnormalities. Other causes of increased intracranial pressure consist of trauma, infections (eg, meningitis), medications, and pseudotumor cerebri.

Iatrogenic causes of Mallory-Weiss tears include complications of endoscopy resulting in esophageal tears (< 0.01% in children) and are almost always associated with a patient who is retching or struggling during the procedure. The use of polyethylene glycol lavage, when used for ingestions, severe constipation, or preparation for colonoscopy, may cause severe vomiting.

Other causes of vomiting and retching that may result in a Mallory-Weiss tear include the following:

• Severe diabetic ketoacidosis

• Toxins

• Drugs (eg, chemotherapeutic agents)

United States data

Mallory-Weiss tears cause approximately 3-15% of all episodes of hematemesis in adults; however, these tears are less common in children (< 5% of all upper GI bleeding episodes).

Race-, sex-, and age-related demographics

In a retrospective study of Black and Hispanic patients presenting with upper GI bleeding over a 10-year period, Mallory-Weiss Tear was diagnosed in 84 of 698 of patients. Among these patients, more than half did not have a history of retching or vomiting, and 216 patients presented with hemodynamic instability. Overall, 10% of patients died from a failure to achieve hemostasis and secondary to associated comorbidities.[3]

Mallory-Weiss tears are equally common among male and female children. These tears also occur with equal frequency in both sexes in adults, although they have different causes. In women of childbearing age, the most common cause of these tears is hyperemesis gravidarum, which usually occurs in the first trimester, causing severe persistent nausea and vomiting. Any adolescent female presenting with a Mallory-Weiss tear should be evaluated for pregnancy, as well as bulimia and anorexia nervosa. Furthermore, the potential for drug and alcohol ingestion must also be underscored in the adolescent patient.

Mallory-Weiss tears usually occur in the fifth and sixth decades of life. In children, tears are more commonly observed in older children and adolescents secondary to increased intragastric and transgastric pressures that develop at an older age. However, children with underlying medical conditions, including gastroesophageal reflux and liver disease, can present at any age.

Prognosis is extremely good in children, with a less than 0.01% mortality rate. These tears almost always respond to conservative therapy and supportive care.[4]

Morbidity/mortality

Although children rarely have hemodynamic instability secondary to upper GI bleeding associated with Mallory-Weiss tears, the risk for potential complication, including shock, must be underscored in patients with portal hypertension and hepatic insufficiency.

The major factor in a poor outcome of a Mallory-Weiss tear is bleeding.[5, 6] Patients present with variable bleeding, which can range from a few specks or streaks of blood mixed with mucus to copious amounts of fresh red blood. In adults, shock occurs in as many as 20% of patients bleeding from Mallory-Weiss tears who present to emergency departments; as many as 45% of patients develop postural hypotension. Indeed, studies have also shown that those patients who present clinically in shock are more likely to require intensive care management and interventional endoscopic procedures. Moreover, these patients are more likely to experience recurrent episodes of bleeding secondary to Mallory-Weiss tears.[7]

Predictive factors for recurrent bleeding include the following[8] :

• Initial presentation of shock: In a study of 159 patients treated for Mallory-Weiss tears, 17 patients (10.7%) experienced recurrent bleeding. Those patients were in shock at initial presentation and had active bleeding on endoscopic evaluation.[9]

• Liver cirrhosis

• Decreased hemoglobin and platelet count: In a study of 93 patients who were managed for Mallory-Weiss tears, 9 had a fatal outcome. In that study, multivariant analysis showed that advanced age, a low hemoglobin level at clinical presentation, and the presence of tarry stool were associated with an increased risk of dying.[10]

• Need for blood transfusion

• Intensive care management

• Active bleeding noted at the time of endoscopy

Future studies are necessary to validate these clinical observations, and they may perhaps lead to the development of a measure tool for clinical risk among patients presenting with Mallory-Weiss tears.

Complications

Complications include the following:

• Anemia

• Dehydration

History

Presenting symptoms of Mallory-Weiss syndrome include hematemesis, melena, light-headedness, dizziness, syncope, and abdominal pain.

• Hematemesis: Hematemesis is the presenting symptom in all patients diagnosed with a Mallory-Weiss tear.[7] The diagnosis does not depend on the amount of hematemesis because it can vary from blood flecks or streaks of blood mixed with gastric contents or mucus to several ounces of bright red bloody emesis. In most children, hematemesis is preceded by one or more episodes of nonbloody emesis; however, hematemesis secondary to a Mallory-Weiss tear has been reported to occur during the first bout of vomiting.

• Melena: Melena has been reported to occur in as many as 10% of patients who have been diagnosed with a Mallory-Weiss tear.

• Light-headedness, dizziness, or syncope: Patients with severe vomiting can develop light-headedness or dizziness. These symptoms usually occur secondary to dehydration from the underlying cause of vomiting and are not secondary to blood loss from the Mallory-Weiss tear. Only in rare cases does a Mallory-Weiss tear lead to anemia requiring transfusions.

• Abdominal pain, dyspepsia: As many as 40% of patients may experience epigastric pain or symptoms of heartburn. These symptoms are often related to the underlying cause of vomiting and not specifically to the Mallory-Weiss tear.

Physical examination

No specific physical findings can be linked to the diagnosis of a Mallory-Weiss tear. Physical findings are linked to the underlying disorder causing the vomiting and retching.

No specific laboratory tests are indicated for determining the etiology of a Mallory-Weiss tear. Coagulation parameters should be assessed. Obtain a CBC count to determine the severity of bleeding.

No specific imaging studies exist that can positively identify an esophageal tear. Several retrospective studies have demonstrated that barium esophagography may reveal small esophageal hematomas or thick streaks where the barium becomes trapped in the tear. However, only 20% of patients with a Mallory-Weiss tear had positive radiologic findings, and, in all cases, an upper endoscopy was performed for definitive diagnosis.

The hallmark of a Mallory-Weiss tear is the visual appearance of one or more linear bleeding lesions at or just proximal to the esophagogastric junction. Visual inspection of the esophagus, stomach, and duodenum is essential in the evaluation of a child presenting with hematemesis.

Upper endoscopy is the diagnostic tool of choice for esophageal tears.[11] Perform endoscopy within 24 hours of the bleeding episode. In cases of severe bleeding with hemodynamic instability, the patient should be stabilized prior to performing endoscopy. Note the following:

• Tears visualized within 24 hours usually have a soft, fresh, mounded, brownish-red appearance on the surface of the mucosa.

• After 48-72 hours, the tear looks like a mucosal cleft that may be surrounded by erythematous mucosa.

• By 96 hours, most Mallory-Weiss lesions are well-healed and may be difficult to visualize.

• Mallory-Weiss tears can heal quickly after the cessation of vomiting and retching and may not be diagnosed if performance of the upper endoscopy is delayed.

An upper endoscopy (performed by a trained pediatric gastroenterologist) should be considered for all patients with persistent bleeding for whom medical therapy is unsuccessful.

Only in extraordinary cases should surgical intervention be required. A consultation with a surgeon should be considered only in patients with persistent bleeding requiring transfusions and in whom the bleeding cannot be controlled by medication or by therapeutic upper endoscopy.

Transfer children with severe uncontrolled bleeding to a tertiary care hospital with an in-house pediatric gastroenterologist.

Once the esophageal bleeding has stopped, no activity restrictions are required.

Mallory-Weiss tears almost never rebleed; thus, follow-up is not usually indicated.

Initial medical management is always supportive. Patients in whom conservative medical therapy is ineffective should have a consultation with a gastroenterologist for possible endoscopy. Note the following:

• Closely monitor vital signs, obtain a CBC count, and place a large-bore intravenous tube for fluid resuscitation.

• Less than 5% of children require a blood transfusion.

• Begin workup to determine the underlying cause of the retching and vomiting.

• In most cases, Mallory-Weiss tears resolve spontaneously; however, consider pharmaceutical therapy in cases of persistent bleeding or complications (see Medication).

• Esophageal balloon tamponade, although useful for patients with esophageal varices, should be considered only in extreme cases because the use of an esophageal balloon increases the risk of extending the esophageal tear.

• Esophageal clips should be applied at the site of active bleeding.

• Endoscopic band ligation has been used and was shown to be an effective and safe procedure for patients with severe bleeding.[3]

With the increased availability of certain endoscopic techniques, including mucosal resection, sclerotherapy, and endoscopic retrograde cholangiopancreatography (ERCP), Mallory-Weiss tear has now become a relatively common nosocomial complication of endoscopic procedures. In one study, metallic hemoclips were used to achieve hemostasis among patients presenting with Mallory-Weiss tears. In that study, patients with bleeding Mallory-Weiss tears secondary to endoscopy were compared with patients presenting with Mallory-Weiss tears from other etiologies. In both groups of patients, hemoclips were effective in achieving hemostasis.[10, 12]

In another study, endoscopic band ligation was compared with hemoclip plus epinephrine injection among patients presenting with upper gastrointestinal bleeding from Mallory-Weiss tears. In 218 consecutive patients with Mallory-Weiss tears, 56 patients required endoscopic hemostasis because of active bleeding. Band ligation was performed in 29 patients, and hemoclip application plus epinephrine injection was performed in 27 patients. Recurrent bleeding occurred in no patients in the banding group and in 18% of patients in the latter group. The investigators concluded that band ligation should be considered as the endoscopic treatment of choice.[13]

In a similar comparative study from Korea, no significant difference was noted between hemoclip and band ligation in patients treated with Mallory-Weiss tears, even among those patients treated with comorbid conditions and hemodynamic instability.[14] In comparison, a similar small comparative study noted no difference in the efficacy or the safety of band ligation versus epinephrine injection for the treatment of active bleeding in Mallory-Weiss tears.[15] Future comparative studies are needed to establish treatment guidelines in patients presenting with Mallory-Weiss tears.

Angiographic embolization of the vessels supplying blood flow to the esophageal tear has been reported in the adult literature but should be considered in children only under dire circumstances.

Two types of endoscopic therapy can be used to control severe bleeding in patients who are hemodynamically unstable because of bleeding from a Mallory-Weiss tear. Note the following:

• Injection therapy is favored as the first-line therapy by most endoscopists for control of bleeding esophageal lesions because of its ease of use, safety, and cost. Typically, the injections are made 3-5 mm apart circumferentially around the site of bleeding in four areas. The chemical agents used for injection therapy include dilute epinephrine, sodium morrhuate, ethyl alcohol, or sodium tetradecyl sulfate.

• Heater probe or bipolar coagulation therapies use electrical current supplied by catheters that can be inserted into an endoscope to control bleeding. Approximately 20 joules (10-15 Watts) of current are used per individual pulse, and treatment is complete when the bleeding has ceased. The current is usually delivered in repeated time-limited pulses.

Evaluate the underlying cause of vomiting; advise patients to avoid identified triggers.

During the acute problem, keep patients on nothing by mouth (NPO). Once resolved, provide the patient clear liquids and advance the diet as tolerated. After complete resolution, no special diet is required. However, foods or liquids that may have been identified as contributing to the cause of the underlying problem (eg, excessive alcohol intake, food allergies) should be avoided.

Vasopressin administered systemically may control cases of severe bleeding in patients with Mallory-Weiss syndrome.

Class Summary

These agents are used to decrease blood flow in the distal esophagus.

Vasopressin (Pitressin)

DOC when attempting to systemically control esophageal bleeding from a Mallory-Weiss tear. Titrate dosage to produce desired clinical effect.