Esophageal varices

Esophageal varices are dilated collateral veins resulting from increased blood flow due to portal hypertension, often caused by cirrhosis. Nonbleeding varices are typically asymptomatic. Screening for varices with esophagogastroduodenoscopy (EGD) is recommended at the time of cirrhosis diagnosis. Management of nonbleeding esophageal varices focuses on the prevention of bleeding and involves regular surveillance and, in some cases, primary prophylaxis of bleeding using nonselective beta blockers or eradication of varices using endoscopic variceal ligation (EVL).

Acute variceal hemorrhage is a potentially life-threatening condition. Patients present with clinical features of gastrointestinal bleeding, e.g., sudden hematemesis and melena, and, in some cases, hypovolemic shock. In addition to stabilizing the patient, management involves administration of vasoactive medication and antibiotic prophylaxis in combination with endoscopic treatment. If the hemorrhage persists, balloon tamponade of the bleeding and/or an emergent transjugular intrahepatic portosystemic shunt (TIPS) may be necessary. Secondary prophylaxis of variceal bleeding involves nonselective beta blockers, EVL, and/or TIPS placement.

• Affects ∼ 50% of patients with cirrhosis ^[1][2]
• Variceal hemorrhage is the most common lethal complication in patients with cirrhosis. ^[3]

Epidemiological data refers to the US, unless otherwise specified.

• Cirrhosis → portal hypertension → dilated sub-mucosal veins (varices) of the distal esophagus
• See “Etiology” in “Portal hypertension.”

Several classification methods exist for esophageal varices.

• Bleeding (i.e., esophageal variceal hemorrhage) vs. non-bleeding
• Degree of extension into the stomach
• Size on endoscopy: ^[4]
  • Small esophageal varices: < 5 mm
  • Medium/large esophageal varices: ≥ 5 mm ^[4][5]

• Nonbleeding varices: typically asymptomatic
  • Patients may have other clinical features of portal hypertension.
• Bleeding varices: sudden onset of severe symptoms of gastrointestinal bleeding
  • Signs of hemorrhagic shock
  • Hematochezia
  • Melena
  • Hematemesis

Consider also a diagnosis of Mallory-Weiss syndrome if bleeding occurs following retching or vomiting.

Esophagogastroduodenoscopy (EGD)

Diagnosis and surveillance of esophageal varices requires esophagogastroduodenoscopy (EGD), with the goal of establishing: ^[1]

• Presence of varices
• Size of varices
• Stigmata of recent or impending bleeding (i.e., high-risk endoscopic findings): ^[3][6]
  • Red wale marks: longitudinal red streaks on the surface of a varix
  • Cherry-red spots
  • Hematocystic spots: raised spots that appear as blisters

Additional studies

• Imaging is not routinely indicated but large esophageal varices may be incidentally identified.
• Transient elastography and CBC may be used to rule out high-risk esophageal varices but are not routinely used for confirming the diagnosis.

Approach ^[1][2][5]

• Obtain EGD to screen for esophageal varices at the time of cirrhosis or portal hypertension diagnosis. ^[1]
• Assess for high-risk features for esophageal variceal hemorrhage (see “Risk stratification”).
  • Low-risk varices: Monitor for development to high-risk varices.
  • High-risk varices: Start pharmacologic prophylaxis.
• Identify and treat the underlying cause of portal hypertension.

Risk stratification ^[4]

• High-risk features for esophageal variceal hemorrhage
  • Large esophageal varices
  • Small esophageal varices with:
    • High-risk endoscopic findings , e.g., red wale marks, cherry-red spots, hematocystic spots
    • And/or presence of decompensated cirrhosis
• Low-risk features for esophageal variceal bleeding: small esophageal varices without high-risk endoscopic findings

Monitoring of low-risk varices ^[1][5]

EGD surveillance is indicated every 1–3 years for patients with low-risk features for esophageal variceal bleeding to screen for the development of high-risk varices.

Patients with esophageal varices have a 10–15% annual risk of variceal hemorrhage; the risk increases with the severity of liver disease, size of varices, and presence of variceal wall thinning. ^[1][5]

Prevention of first episode of variceal bleeding ^[1][4][5]

• Medium or large esophageal varices: Provide either pharmacological prophylaxis or EVL. ^[1]
• Small esophageal varices with high-risk features for esophageal variceal hemorrhage: Provide pharmacological prophylaxis as indicated.

Pharmacological prophylaxis (off-label) ^[2]

• Nonselective beta blockers (recommended) ; ^[1]
  • Propranolol; OR nadolol ^[1]
  • Carvedilol (alternative) ^[1][7]
• Can be continued indefinitely if tolerated
• No EGD surveillance is necessary

Reduce the dose or discontinue beta blockers if ascites or hepatorenal syndrome develop or systolic blood pressure is < 90 mm Hg. ^[4]

In patients without varices, there is no evidence to support the use of beta blockers to prevent the development of gastroesophageal varices; however, beta blockers may be used for other indications in patients with clinically significant portal hypertension. ^[1]

Endoscopic variceal ligation (EVL) ^[1][5]

• Repeat every 1–8 weeks until varices are eradicated.
• Obtain surveillance EGD within 1–6 months of eradication and every 6–12 months thereafter. ^[1][5]

Combination therapy with EVL and pharmacotherapy is not recommended for primary prophylaxis of esophageal variceal hemorrhage.

Approach ^[1][4][5]

• Stabilize the patient: See “Initial management of overt gastrointestinal bleeding.”
  • Initiate IV fluid resuscitation.
  • Transfuse packed red blood cells to maintain hemoglobin > 7 g/dL.
  • Consider initiating massive transfusion protocol.
  • Intubate patients who have altered mental status and/or severe, ongoing hematemesis.
• Consult gastroenterology for EGD and further management immediately.
• Start vasoactive medication: octreotide OR vasopressin infusion
• Administer antibiotic prophylaxis.
• Begin prophylaxis to prevent recurrence.

Esophageal variceal hemorrhage is a medical emergency.

Airway management ^[8][9]

• Indications for tracheal intubation ^[4][10]
  • Altered mental status or active hematemesis
  • Emergent balloon tamponade procedure
• Interventions to reduce the risk of pulmonary aspiration
  • Consider rapid sequence intubation.
  • Have adequate suction immediately available.
  • Consider gastric decompression prior to induction.
• Interventions to mitigate hemodynamic instability
  • Administer IV fluid bolus or blood before induction.
  • Consider etomidate or ketamine as the intubation induction agent.
  • For details, see “Intubation of hemodynamically unstable patients.”

Anticipate difficult airway management in patients with ongoing bleeding and consider using a video laryngoscope for the first attempt.

Pharmacological treatment

Vasoactive medication and antibiotic prophylaxis are indicated for all patients. ^[1][4]

• Vasoactive medication
  • Reduces mortality and the need for blood transfusions by reducing splanchnic blood flow
  • Preferred agents
    • Octreotide for 2–5 days
    • OR vasopressin for 24 hours ^[1][4]
• Antibiotic prophylaxis ; ^[1]
  • Associated with decreased rates of infection, recurrent hemorrhage, and death
  • Preferred agent: ceftriaxone for a maximum of 7 days
• Hepatic encephalopathy prevention: Consider lactulose (PO or PR). ^[4]

Esophageal variceal bleeding is a consequence of portal hypertension, and therefore treatment focuses on reducing portal hypertension rather than the correction of coagulation abnormalities. ^[4]

Endoscopic treatment ^[1][5]

EGD should be performed as soon as possible in unstable patients and within 12 hours in all other patients.

• Variceal ligation
  • Preferred intervention
  • Consider prokinetic treatment with erythromycin (off-label) prior to EGD. ^[11]
• Variceal sclerotherapy
  • Injection of a sclerosant into, or adjacent to, the varix
  • Used when variceal ligation is technically difficult
• Self-expanding metal stents: bridge therapy to TIPS in refractory bleeding ^[4]

Other interventional treatments

Balloon tamponade ^[1][10]

• Definition: orogastric tubes with esophageal and gastric balloons that tamponade bleeding when inflated
  • Sengstaken-Blakemore tube: orogastric tube with an esophageal balloon, gastric balloon, and gastric aspiration port
  • Minnesota tube: modification of Sengstaken-Blakemore tube with an additional aspiration port above the esophageal balloon and larger gastric balloon
• Indication: bridge to definitive treatment if ^[10]
  • Endoscopy is unavailable and vasoactive medications are ineffective
  • Endoscopic treatment is unsuccessful
• Complications
  • Airway obstruction
  • Pressure-induced tissue necrosis
  • Esophageal rupture
  • Aspiration pneumonitis

TIPS

• Consider if pharmacological and endoscopic treatment are unsuccessful. ^[5]
• Consider early TIPS (within 72 hours of EVL) in patients at high risk of rebleeding.

Prevention of recurrent variceal bleeding ^[1][5]

• Patients without TIPS: combination therapy with nonselective beta blockers and EVL
  • See “Prevention of first variceal bleed” for information on dosing and frequency.
  • Refer for TIPS if combination therapy is unsuccessful or not tolerated.
• Patients with TIPS: No additional treatment is indicated.
  • Evaluate TIPS patency using Doppler ultrasound every 6 months.
  • Consider referral for liver transplantation evaluation.

The combination of EVL and nonselective beta blockers for the prevention of recurrent esophageal variceal hemorrhage is more effective than either therapy alone.

• Keep NPO.
• Follow ABCDE approach and stabilize patient in an ICU setting.
• Consider intubation for airway protection.
• Initiate IV fluid resuscitation.
• Consult gastroenterology for endoscopic hemostasis.
• Hold anticoagulants if needed.
• Transfuse packed red blood cells if Hb is ≤ 7 g/dL.
• Consider initiating massive transfusion protocol.
• Start octreotide or vasopressin infusion.
• Start antibiotic prophylaxis (e.g., ceftriaxone).

• Esophageal perforation
• Infection: e.g., aspiration pneumonia, spontaneous bacterial peritonitis, bacteremia
• Acute kidney injury: e.g., hepatorenal syndrome, acute tubular necrosis
• Hepatic encephalopathy

We list the most important complications. The selection is not exhaustive.

• Six-week mortality rate after a variceal bleeding event is ∼ 20%. ^[2][5]
• Risk of rebleeding within 1 year if left untreated is ∼ 60%. ^[2][5]