Description

• Dilated submucosal esophageal veins connecting the portal and systemic circulations
• Most commonly results from portal hypertension (typically a result of cirrhosis)
• Variceal rupture: most common fatal complication of cirrhosis; severity of liver disease correlates with presence of varices and risk of bleeding.

Epidemiology

Incidence

• 30% of cirrhotic patients have varices at the time of diagnosis; 90% at 10 years
• 1-year rate of first variceal bleeding is 5% for small varices, 15% for large varices.

Prevalence

• 50% of patients with esophageal varices experience bleeding at some point.
• Variceal bleeding: 10–20% mortality in the 6 weeks following the episode
• Gender: male > female

Etiology and Pathophysiology

• Portal hypertension causes the formation of portacaval anastomoses to decompress the portal circulation. This leads to a congested submucosal venous plexus with tortuous dilated veins, particularly in the distal esophagus. Variceal rupture results in hemorrhage.
• Pathophysiology of portal hypertension:
  • Increased resistance to portal flow at the level of hepatic sinusoids caused by
    • Intrahepatic vasoconstriction due to decreased nitric oxide production and increased release of endothelin-1 (ET-1), angiotensinogen, and eicosanoids
    • Sinusoidal remodeling causes disruption of blood flow.
  • Increased portal flow caused by hyperdynamic circulation due to splanchnic arterial vasodilation through mediators such as nitric oxide, prostacyclin, and TNF
• Causes of portal hypertension:
  • Prehepatic:
    • Extrahepatic portal vein obstruction (EHPVO)
    • Massive splenomegaly with increased splenic vein blood flow
  • Posthepatic:
    • Severe right-sided heart failure, constrictive pericarditis, and hepatic vein obstruction (Budd-Chiari syndrome)
  • Intrahepatic:
    • Cirrhosis (accounts for most cases of portal hypertension)
  • Less frequent causes are schistosomiasis, massive fatty change, diseases affecting portal microcirculation as nodular regenerative hyperplasia, and diffuse fibrosing granulomatous disease as sarcoidosis.

Genetics
Cirrhosis is rarely hereditary.

Risk Factors

• Cirrhosis
• In cirrhotic patients, thrombocytopenia and splenomegaly are independent predictors of esophageal varices.
• Noncirrhotic portal hypertension
• Increased bleeding risk for known varices is associated with varix size, endoscopic signs (red wale marks, cherry-red spots), vessel wall thickness, and abrupt increase in variceal pressure (i.e., Valsalva maneuver).
• MELD/Child-Pugh score; presence of portal vein thrombosis; high hepatic venous pressure gradient (HVPG)

General Prevention

Prevent underlying causes: Prevent alcohol abuse, administer hepatitis B vaccine, needle hygiene, intravenous (IV) drug use (needle exchange programs reduce risk of hepatitis); specific screening and therapy for hepatitis B and C, hemochromatosis

Commonly Associated Conditions

• Portal hypertensive gastropathy; varices in stomach, duodenum, colon, rectum (causes massive bleeding, unlike hemorrhoids); rarely at umbilicus (caput medusae) or ostomy sites
• Isolated gastric varices can occur due to splenic vein thrombosis/stenosis from hypercoagulability/contiguous inflammation (most commonly, chronic pancreatitis).
• Other complications of cirrhosis: hepatic encephalopathy, ascites, hepatorenal syndrome, spontaneous bacterial peritonitis, hepatocellular carcinoma