What are esophageal varices?

What Are Esophageal Varices

Esophageal varices are abnormally dilated submucosal veins in the lower esophagus that develop as porto-systemic collaterals in response to portal hypertension, representing the most clinically significant complication because their rupture causes life-threatening hemorrhage with mortality of at least 20% at 6 weeks. 1

Pathophysiology and Formation

Esophageal varices form through a specific hemodynamic cascade:

• Portal hypertension develops when hepatic venous pressure gradient (HVPG) reaches at least 10-12 mm Hg, driven by increased intrahepatic resistance (70-80% structural from fibrosis and regenerative nodules, 20-30% from active vasoconstriction due to decreased nitric oxide production) 1

• Collateral vessel formation occurs as blood seeks alternative pathways around the obstructed liver, with normally thin parallel veins in the lamina propria mucosae of the palisade zone becoming enlarged and joining larger submucosal veins 2

• The critical anatomic vulnerability exists where enlarged parallel veins pile up and join submucosal veins at acute angles in the distal esophagus—this is where most ruptures occur 2

Epidemiology and Natural History

The progression of varices follows a predictable pattern with significant mortality implications:

• Approximately 50% of cirrhotic patients have gastroesophageal varices at diagnosis, with prevalence correlating directly with liver disease severity: 40% in Child A patients versus 85% in Child C patients 1

• Development rate is 8% per year in patients without varices, with HVPG ≥10 mmHg being the strongest predictor 1

• Progression from small to large varices occurs at 8% per year, accelerated by decompensated cirrhosis (Child B/C), alcoholic etiology, and presence of red wale marks (longitudinal dilated venules on variceal surface) 1

Bleeding Risk and Mortality

The hemorrhagic complications define the clinical significance:

• Variceal hemorrhage occurs at a yearly rate of 5-15%, with the highest risk (15% per year) in patients with large varices 1

• Key predictors of first hemorrhage include varix size (most important), decompensated cirrhosis (Child B/C), and endoscopic red wale marks 1

• Mortality remains substantial at 20% at 6 weeks despite therapeutic advances, with patients having HVPG ≥20 mmHg within 24 hours of hemorrhage facing 64% one-year mortality versus 20% in those with lower pressures 1

• Rebleeding risk is 60% in untreated patients, predominantly within 1-2 years of the index hemorrhage 1

Anatomic Classification

Varices are classified by size and location for treatment planning:

• Size classification uses either two grades (small vs. large with 5 mm cutoff) or three grades: small (minimally elevated above mucosa), medium (tortuous, <1/3 lumen), and large (>1/3 lumen), with medium and large grouped together for treatment decisions 1

• Gastric varices are less common (5-33% of portal hypertension patients) but important: Type 1 gastroesophageal varices (GOV1) extend along lesser curvature; Type 2 (GOV2) extend to fundus; isolated gastric varices occur without esophageal varices 1

Diagnostic Approach

Esophagogastroduodenoscopy (EGD) is the gold standard for diagnosis, with specific screening recommendations 1:

• All cirrhotic patients should undergo endoscopic screening at diagnosis 1
• Decompensated patients (those with ascites, variceal hemorrhage, or hepatic encephalopathy) require EGD to screen for varices unless previously diagnosed and treated 1
• Annual repeat screening is indicated for patients without varices if the etiological factor persists or decompensation continues 1

Clinical Context and Special Populations

Important clinical variations exist:

• Primary biliary cirrhosis patients may develop varices and hemorrhage early, even without established cirrhosis 1
• Hepatitis C with bridging fibrosis shows 16% prevalence of esophageal varices even before cirrhosis develops 1
• Downhill varices (rare variant) occur from superior vena cava obstruction rather than portal hypertension, appearing in upper/middle esophagus and requiring different management 3