• Dilated collateral veins in the lamina propria of the distal esophagus connecting the portal and systemic circulations
• Results from chronic hypertension in the portal circulation due to increased resistance to blood flow
• Increased pressure and turbulent flow within these vessels as well as their superficial location in the distal esophagus make them prone to rupture with significant morbidity and mortality

• Esophageal varices occur in ~50% of patients with cirrhosis.
• 50% of patients with esophageal varices bleed during their lifetime.
• Bleeding from esophageal varices is associated with 15–20% mortality.
• Predominant sex: Male > Female.

Cirrhosis of the liver

Genetics
No known pattern

• Endoscope esophagus annually in patients with cirrhosis
• Consider use of non-selective beta-blockers or obliteration of varices with esophageal banding for those intolerant of medication to prevent bleeding

Portal Hypertension is caused by elevated portal pressure due to splanchnic arteriolar vasodilatation and increased resistance through dilated hepatic sinusoids.

• Portal Hypertension is defined as a pressure gradient >10 mmHg.
• Cirrhosis accounts for >90% of cases. Alcohol and hepatitis C are the most common etiologies.
• Hemochromatosis, hepatitis B, nonalcoholic fatty liver disease, biliary cirrhosis, and autoimmune cirrhosis account for remainder. Extrahepatic portal vein thrombosis from umbilical vein infection, trauma, chronic pancreatitis, thrombotic conditions, and polycythemia.
• Malignant invasion of liver sinusoids or portal vein; seen in lymphoma, leukemia, hepatocellular carcinoma, and pancreatic carcinoma.
• Metabolic diseases altering liver sinusoids—amyloid, Gaucher disease, fatty liver
• Budd-Chiari syndrome
• Veno-occlusive disease

• Portal hypertensive gastropathy
• Hemorrhoids

• GI bleeding:
  • 75% of time, painless hematemesis and/or melena
  • Occult bleeding with anemia 25%
• Signs of cirrhosis

History

• Generally a history of cirrhosis or liver disease
• Painless hematemesis or melena

Physical Exam

• Possible hypotension/tachycardia
• Small, hard liver
• Splenomegaly
• Ascites
• Visible abdominal periumbilical collateral circulation (Caput medusae)
• Spider angiomata on upper chest/back
• Palmar erythema

Lab
Initial Labs

• Anemia related to blood loss
• Possibly abnormal liver function tests, thrombocytopenia, prolonged prothrombin time or low albumin-reflecting cirrhosis

Imaging
Initial Imaging Approach

• Barium swallow
  • Adequate for advanced varices, but is insensitive to small ones
  • Precludes possible urgent endoscopy
• Doppler sonography: Demonstrates patency, diameter, and flow in portal vein, and splenic vein, and large collaterals intra-abdominally
• MRI:
  • Demonstrates large vascular channels intra-abdominally, and in the mediastinum
  • Can demonstrate patency of the intrahepatic portal vein and splenic vein
• Venous phase celiac arteriography: Demonstrates portal vein and its collaterals, also can diagnose hepatic vein occlusion

Diagnostic Procedures/Other

• Esophagoscopy as part of esophagogastroduodenoscopy:
  • Can identify and treat varices that appear as protruding submucosal veins in the distal 3rd of the esophagus
  • Can identify actively bleeding varices as well as those with stigmata of recent hemorrhage
  • Can treat actively bleeding vessels with sclerotherapy or esophageal band ligation or can obliterate vessels to prevent rebleeding. Can also identify associated conditions, including gastric varices and portal hypertensive gastropathy.
• Endoscopic ultrasound is particularly sensitive to gastric varices.
• Portal pressure measurement
  • Radiologist introduces a catheter retrograde into the hepatic vein in a wedged position to occlude flow
  • The catheter is withdrawn to a free position and pressure again measured. The difference between wedged and free is the portal pressure. If <12 mm Hg, bleeding is less likely. Progressive increases above 12 correlate with the likelihood of hemorrhage.
  • This is sometimes used to monitor successful treatment with beta-adrenergic blocking agent though it is not widely available.

Pathological Findings

• Extensive collateral circulation in the mediastinum and in the abdomen in addition to large vessels in the submucosa of the esophagus
• When bleeding occurs, these large veins explode into the submucosa of esophagus and rupture into the lumen.

• Upper GI bleeding:
  • Pulmonary bleeding; hemoptysis
  • Peptic ulcer disease
  • Gastric or esophageal malignancy
  • Arteriovenous malformation (AVM)
  • Nosebleed
• Lower GI bleeding:
  • Hemorrhoids
  • Colonic neoplasia
  • Diverticulosis
  • AVMs

• For varices:
  • B-Blockers: Decrease risk of first bleed by 45–50%, in primary prophylaxis of variceal hemorrhage (1)[A].
  • Propranolol: 40 mg b.i.d. increase until heart rate decreased by 25% from baseline
  • Nadolol 80 mg daily, increase as above
  • Isosorbide mononitrate further reduces portal pressure. Begin at 20 mg b.i.d. No significant benefit in preventing first bleeds when given in combination with beta-blockers. Should not be given as monotherapy (2)[B].
  • During banding or sclerotherapy: Proton pump inhibitor, such as lansoprazole 30 mg/d until varices obliterated
• During bleeding, consider antibiotic prophylaxis for spontaneous peritonitis and other infections with ciprofloxacin for 7–10 days.
• Contraindications: Severe asthma with beta-blockers
• Precautions: Symptomatic hypotension

First Line
Beta-blockers, proton pump inhibitors, antibiotics

Second Line
Isosorbide mononitrate

General Measures

• Treat comorbidities, generally related to cirrhosis
• Hospital management of bleeding varices:
  • Appropriate resuscitation and maintenance of blood volume
  • Treat coagulopathy, if necessary.
  • IV somatostatin to lower portal venous pressure usually used as adjuvant to endoscopic management. Begin with IV bolus of 50 mg followed by drip of 50 mg/h (3)[A].
  • Urgent upper endoscopy for diagnosis and treatment. Variceal band ligation or sclerotherapy for bleeding varices or those not bleeding, which are medium to large in size to decrease risk of bleeding. Variceal band ligation is preferred due to better bleeding cessation with fewer complications (4)[A].
  • Repeat ligation or sclerosant injection if bleeding recurs.
  • If endoscopic treatment fails to stop bleeding or cannot be accomplished, may need to use Sengstaken Blakemore or Minnesota tube to stabilize patient for a transjugular intrahepatic portosystemic shunt
• Management of nonbleeding varices:
  • If ligation started, usually in medium to large varices (grade 2–4), repeat banding at 1–3 week intervals. 4–6 treatments are usually required to obliterate varices.
  • For those not treated endoscopically, begin non-selective beta-blockers such as propranolol or nadolol. Increase dose for goal of heart rate reduction of 25% of baseline (SBP >90, HR >50). For those who do not tolerate the side effects of this regimen, proceed with endoscopic variceal band ligation as primary prophylaxis (5)[A].
  • If bleeding recurs, or portal pressure measurement shows portal pressure still >12 mm Hg, isosorbide mononitrate may be added, though endoscopic band ligation preferred if possible (6)[B].
  • Refractory bleeding may require use of TIPS, or portocaval shunt (7)[B]
  • Refer for liver transplantation where appropriate.

Issue for Referral
Primarily those associated with liver transplantation

• Endoscopic variceal ligation- preferred approach to those who cannot tolerate beta-blockers.
• Endoscopic sclerotherapy
• Transjugular intrahepatic portasystemic shunt (TIPS)
• Portacaval shunt
• Esophageal transection
• Liver transplantation
• In patients with current or prior bleeding from esophageal varices, endoscopic variceal ligation is superior to endoscopic sclerotherapy (8)[A].

Admission Criteria
Inpatient for acute bleeding

Discharge Criteria
Cessation of bleeding, stability of other comorbidities

Patient Monitoring

• Close monitoring of vital signs if actively bleeding
• Endoscopic variceal ligation, repeated every 1–4 weeks until varices eradicated
• All patients with cirrhosis should undergo endoscopy to document the presence of varices and risk of hemorrhage (1)[B].
• If transjugular intrahepatic portasystemic shunt or other portacaval shunt, repeat endoscopy only if clinically bleeding
• If transjugular intrahepatic portasystemic shunt present, follow-up as recommended by radiologist; usually Doppler sonogram each 6 months

• Appropriate to cirrhosis
• National Digestive Information Clearinghouse, 2 Information Way, Bethesda, MD 20892 or American Liver Foundation, 1425 Pompton Way, Cedar Grove, NJ 07009

• Depends heavily on ability to treat or reverse underlying condition
• In those with cirrhosis, 1 year survival for those who are alive 2 weeks after variceal bleed is ~50%.

• Bleeding
• Gastric or other uncommon varices may occur following successful eradication of esophageal varices.
• Esophageal varices can recur after obliteration.

See Also (Topic, Algorithm, Electronic Media Element)
Cirrhosis of the Liver; Hemorrhoids; Portal Hypertension

• 456.0 Esophageal varices with bleeding
• 456.1 Esophageal varices without mention of bleeding
• 456.20 Esophageal varices in diseases classified elsewhere, with bleeding
• 456.21 Esophageal varices in diseases classified elsewhere, without mention of bleeding

• 28670008 esophageal varices (disorder)
• 17709002 bleeding esophageal varices (disorder)
• 14223005 esophageal varices without bleeding (disorder)

• Esophageal varices occur in ~50% of patients with cirrhosis.
• 50% of patients with esophageal varices bleed during their lifetime.
• Bleeding from esophageal varices is associated with 15–20% mortality
• Endoscopy can identify and treat actively bleeding varices

James G. Nee, MD

1. Groszmann, et al. Beta-Blockers to prevent gastroesophageal varices in patients with cirrhosis. N Engl J Med 2005;353:2254.  [PMID:16306522]
2. D'Amico, et al. Pharmacological treatment of portal hypertension: An evidence based approach. Semin Lever Dis 1999;19:475.
3. Zhou Y, et al. Comparison of the efficacy of octreotide, vasopressin, and omeprazole in the control of acute bleeding in patients with portal hypertensive gastropathy: A controlled study. J Gastroenterol Hepatol 2002;17:973–979.  [PMID:12167118]
4. Laine L, et al. Endoscopic ligation compared with sclerotherapy for the treatment of bleeding esophageal varices. Ann Intern Med 1993;119:1–7.  [PMID:8498757]
5. Boyer TD. Primary prophylaxis for variceal bleeding: Are we there yet? Gastroenterology 2005;128:1120–112.  [PMID:15825093]
6. Merkel C, et al. Randomised trial of nadolol alone or with isosorbide mononitrate for primary prophylaxis of variceal bleeding in cirrhosis. Lanc 1996;348:1677.
7. Sanyal AJ, et al. Transjugular intrahepatic portosystemic shunts for patients with active variceal hemorrhage unresponsive to sclerotherapy. Gastroenterology 1996;111:138.  [PMID:8698192]
8. American Society for Gastrointestinal Endoscopy, The role of endoscopy in the management of variceal hemorrhage. Gastrointestinal Endoscopy 2005;62:651–655.  [PMID:16246673]