Esophageal varices are present in ~50% of patients with portal hypertension . They occur in greater frequency in patients with more severe cirrhosis, for example esophageal varices may be present in ~40% of patients with Child-Pugh A, but in ~85% of patients with Child-Pugh C cirrhosis .
Patients will generally be asymptomatic until a variceal hemorrhage, which occur at a yearly rate of 5-15% . In the case of a variceal bleed, patients will usually present with stigmata of upper gastrointestinal bleeding, such as hematemesis, melena, presyncope and syncope, and progression to hypovolemic shock . The primary predictor of variceal bleed is variceal wall tension, which is a metric that encompasses vessel diameter (larger is worse) and pressure (higher is worse) .
Additionally, patients will often have stigmata of portal hypertension and cirrhosis – the most common etiological basis for esophageal varices.
Two types of esophageal varices have been described.
- uphill esophageal varices
- most common form
- typically caused by portal hypertension, as a collateral pathway between the portal vein and the superior vena cava (via the azygos vein)
- typically present in the lower third of the esophagus
- commonly co-occur with gastric varices, which have an identical pathophysiology, but are notably less common
- when co-occurring, most commonly there is extension of the esophageal varices along the lesser curvature of the stomach (Type GOV1 in the Sarin classification)
- may be associated with paraesophageal varices, which are varices of the adventitial esophageal veins, and confer a low bleeding risk
- downhill esophageal varices
- relatively rare
- typically caused by superior vena cava obstruction, as part of superior vena cava syndrome, as a collateral pathway between the superior vena cava into the portal circulation and/or the inferior vena cava
- typically present in the upper third of the esophagus, although can span the entire esophagus if the superior vena cava obstruction is proximal to the inflow of the azygos vein
- do not co-occur with gastric varices due to a different pathophysiological basis
- have a relatively lower bleeding risk
The gold-standard investigation for the evaluation of esophageal varices is esophagogastroduodenoscopy, however radiographic investigations may serve as a useful non-invasive adjunct.
Esophageal varices may be visible on plain radiograph if they are large. In such instances, they will have a non-specific appearance of a mass in the posterior mediastinum .
Barium swallow may reveal longitudinal esophageal luminal filling defects, representing esophageal varices .
Esophageal and paraesophageal varices are readily visible on contrast-enhanced cross-sectional imaging, as torturous, enlarged, smooth enhancing tubular structures . They, depending on size and pressure, may protrude into the esophageal lumen . In association, the esophageal wall is also often thickened .
Digital subtraction angiography can also be a useful imaging modality in the assessment of esophageal and paraesophageal varices, providing direct visualization through catheterization and contrast injection of the left gastric vein . However, smaller varices are often not appreciated .
Treatment and prognosis
The mainstay of management for uphill esophageal varices is primary prevention, which can be pharmacological (typically with non-selective beta-blockers), or with invasive endoscopic variceal ligation . The decision between pharmacological or endoscopic prevention is rather complex, and depends on factors such as variceal size and severity of cirrhosis . In patients with a prior variceal hemorrhage, the focus of management is secondary prevention, which can again be pharmacological (typically with non-selective beta-blockers), with invasive endoscopic variceal ligation, or through surgical creation of a portosystemic shunt (e.g. transjugular intrahepatic portosystemic shunt) .
In the event of acute variceal hemorrhage, management includes resuscitation with intravenous fluids and blood products, administration of vasoactive drugs (such as terlipressin or octreotide), intravenous antibiotics, and endoscopic ligation . In severe cases refractory to standard management, salvage therapy with a Sengstaken-Blakemore tube or a Minnesota tube can be attempted .
The management of downhill esophageal varices is quite different, and the focus is on treating the cause of the superior vena cava obstruction . Unlike uphill esophageal varices, beta-blockade may not be useful, however endoscopic variceal ligation or banding may be appropriate .