What is Esophageal Varices?
Esophageal varices are enlarged veins in the esophagus, the tube connecting your mouth and stomach. This can occur when there is increased pressure in the portal vein, the main vein supplying your liver, generally due to liver scarring called cirrhosis. The increased pressure and resistance to blood flow causes more blood than usual to enter these veins. The most deadly complication of cirrhosis is a rupture, or bursting, of these varices, and the risk of bleeding goes up with how severe the liver disease is.
The portal vein typically handles over 1500 ml/min of blood. When this flow is blocked, pressure builds up. To relieve that pressure, your body might form new pathways for the blood, called “collaterals”. These collaterals can divert blood away from the portal vein and towards other large veins in the body. One such pathway drains into the azygos vein and can lead to the development of esophageal varices. When these veins get too enlarged, they can burst, causing severe bleeding. This bleeding is the third most common cause of upper digestive tract bleeding, after ulcers in the small intestine and stomach.
What Causes Esophageal Varices?
Portal hypertension is a condition where there’s high blood pressure in the veins that lead to your liver. This can be caused by various factors:
Pre-liver issues: This can happen when either the vein leading to your liver (Portal vein) gets blocked (a condition known as EHPVO) or when your spleen is abnormally large, causing too much blood flow in the spleen’s vein.
Post-liver issues: Specific heart problems can cause this, like serious issues with the right side of your heart or a condition where your heart’s outer layer becomes rigid (constrictive pericarditis). It can also happen if there’s an obstruction in your hepatic veins (veins in your liver), a condition called Budd-Chiari syndrome.
Inside the liver issues: Cirrhosis, a condition where your liver is damaged and scar tissue replaces healthy liver tissues, is the most common cause of portal hypertension.
There are less common causes like a parasitic disease called schistosomiasis, massive fatty changes in your liver, nodular regenerative hyperplasia (when tiny bumps form in your liver disrupting blood flow), and sarcoidosis (a disease causing inflammation in your organs).
There are also rare situations where certain diseases could lead to portal hypertension, including Wilson disease (a genetic condition causing copper buildup in your organs), alpha-1 antitrypsin deficiency (a genetic condition affecting your lungs and liver), primary biliary cirrhosis (a slow damage to your bile ducts in the liver), tuberculosis (an infectious disease affecting the lungs), and constrictive pericarditis.
Risk Factors and Frequency for Esophageal Varices
Cirrhosis, a chronic liver disease, is quite common, especially in children. With this condition, about 30% of patients will have varices (or swollen blood vessels) at the time of diagnosis. However, this can increase to 90% after 10 years. The chances of these varices bleeding within a year are 5% if they’re small, and 15% if they’re large.
Interestingly, chronic liver disease seems to affect males more than females. In fact, half of the patients with a particular type of swollen blood vessels, called esophageal varices, will experience bleeding at some point. Sadly, this kind of bleeding has a 10% to 20% mortality rate in the 6 weeks following the episode.
What causes this condition? In the West, the two common causes of portal hypertension (a type of high blood pressure) are alcohol and viral hepatitis. However, in Asia and Africa, the most common causes are a parasitic disease called schistosomiasis and hepatitis B and C.
- 30% of cirrhotic patients have varices at diagnosis, rising to 90% after 10 years.
- The 1-year rate of first-time variceal bleeding is 5% for small varices and 15% for large ones.
- Chronic liver disease is more common in males. Half of patients with esophageal varices will bleed at some point.
- Variceal bleeding has a 10% to 20% mortality rate within 6 weeks of the episode.
- In the West, alcohol and viral hepatitis are the leading causes of portal hypertension while in Asia and Africa, schistosomiasis and hepatitis B/C are most common.
Signs and Symptoms of Esophageal Varices
Varices, which are enlarged and twisted veins, often show up first when a person experiences some type of bleeding in the gastrointestinal system, such as vomiting blood, finding blood in the stool, or having black, tarry stools. Anemia due to hidden bleeding is not common.
Your body may show signs of varices even if you didn’t know you had a liver disease like cirrhosis. Signs could include:
- Liver disease diagnosed due to bleeding from varices
- Alcohol abuse or exposure to viruses floating in the blood
- Vomiting blood, black and tarry stools, or bloody stools
- Rapid bleeding from the upper part of your gastrointestinal system that shows up as bleeding from the rectum
- Weight loss in people with chronic liver disease
- Loss of appetite
- Abdominal discomfort
- Yellowing of the skin and eyes (jaundice)
- Itchy skin
- Confusion or altered mental status
- Muscle cramps
During a physical exam, a healthcare provider may look for specific signs linked to varices, such as:
- Indications of active bleeding, like low blood pressure or a fast heartbeat
- Signs of liver damage upon abdominal examination (the liver may feel small and firm if cirrhosis is present)
- An enlarged spleen, excess fluid in the abdomen
- Visible twisted blood vessels around your belly button
- Signs often found in people who consume a lot of alcohol such as red palms, testicular atrophy, breast development in men, and red spider-like blood vessels on the skin
- Hearing a buzzing sound in the veins (venous hum)
- Enlarged veins or blood present on a rectal examination
- Trembling hands or confusion, both signs of a condition called hepatic encephalopathy which is caused by liver disease
Testing for Esophageal Varices
If your doctor suspects that you’re dealing with issues related to your liver or digestive tract, they may suggest a few different tests to help reach a conclusion. Firstly, they might recommend a blood test. A low number of red blood cells, or anemia, is one possibility they might find – however, your hemoglobin may be normal if you’re currently bleeding, and it can take between six to 24 hours for it to change. A low platelet count is another common sign of issues such as portal hypertension and large esophageal varices, conditions associated with liver disease. Elevated levels of liver enzymes such as aspartate aminotransferase (AST) and alanine aminotransferase (ALT), an increased alkaline phosphatase, and a high amount of bilirubin in the blood might suggest liver cirrhosis. Furthermore, your doctor may look at your BUN (a measure of kidney function), sodium level, blood clotting profile, kidney function, and arterial blood gas.
Additionally, the doctor might do a specific type of endoscopy called an esophagogastroduodenoscopy. This procedure allows the doctor to look at your esophagus, stomach, and the upper part of your small intestine. By doing this, they can identify any varices (swollen veins) that are actively bleeding along with large varices and signs of recent bleeding. This procedure can also be used to treat bleeding by putting a band around the varices, known as esophageal band ligation. The doctor might also discover other conditions such as gastric varices, portal hypertensive gastropathy, or alternative bleeding sites.
There are a few other procedures that your doctor might suggest such as Transient Elastography (TE), which measures the stiffness of your liver to help identify patients with a type of liver disease known as Chronic Liver Disease (CLD) who are at risk of developing Clinically Significant Portal Hypertension (CSPH). Another method includes the Hepatic Vein Pressure Gradient (HVPG), which measures the blood pressure in the hepatic vein – the main vessel that carries blood away from the liver. If this pressure is above 10 mmHg, it’s an indication of CSPH. There’s also video capsule endoscopy which is an alternative to traditional endoscopy and checks your digestive tract for any issues. A Doppler sonogram, which is an ultrasound that can show how blood moves through your vessels, is another test they may use to check the veins in your liver and spleen.
Lastly, your doctor may recommend a CT or MRI-angiography. This type of imaging uses a special dye to make the blood vessels more visible, which can help to identify larger vascular passages throughout your body, as well as highlighting any issues with your veins.
Treatment Options for Esophageal Varices
In certain cases, treating cirrhosis can also help with managing variceal bleeding. Variceal bleeding, or bleeding from large veins, can often be complicated by hepatic encephalopathy, which is a decline in brain function due to severe liver disease, and by infection.
During moments of active bleeding from varices, the first steps of treatment include establishing an intravenous, or IV, line and stabilizing the patient’s vital signs. It’s important to avoid over-transfusion of fluids, as this can increase the pressure in the portal vein and potentially trigger more bleeding.
Ensuring the patient’s mental state is alert is crucial. Strong medications that could potentially harm the kidneys, sleep-inducing medications, and certain heart medications should be avoided.
Medications such as octreotide, which is given through an IV, can help lower the pressure in the portal vein. An alternative medicine that has similar effects is terlipressin. Another medication, erythromycin, can also be given before an upper GI endoscopy, which is a procedure that allows doctors to look at the upper parts of the digestive system and provide treatment if needed.
If conditions permit, a certain type of heart medicine, such as beta-blockers, could be used. If varices are spotted during the endoscopy, a common approach is the use of variceal banding, a technique that uses bands to decrease bleeding risk. If banding does not stop the bleeding, treatments may include the placement of self-expanding esophageal metal stents or the use of a Sengstaken-Blakemore-type tube to stabilize the patient.
If the patient actively bleeds, it is best not to use beta-blockers, as these lower blood pressure and interfere with the heart’s normal responses to bleeding.
To prevent recurrent episodes of bleeding, doctors may use medicines that reduce pressure in the portal vein, perform repeated endoscopic banding procedures, or put in place a Transjugular Intrahepatic Portosystemic Shunt (TIPS)—a procedure where a shunt is used to create a pathway between the hepatic vein and the portal vein in the liver. This decreases the pressure by redirecting the blood flow.
For longer-term treatment, medications that reduce portal pressure are usually the first choice, reducing the risk of initial bleeding. Carvedilol, a type of beta-blocker, may also be prescribed for daily use, and can be even more effective in reducing pressure in the hepatic venous pressure gradient (HVPG), a measure of the blood pressure in the liver’s veins.
For patients who cannot tolerate medication, binding the varices with bands is an alternative. When using this alternative treatment, proton pump inhibitors like lansoprazole can be used until varices are obliterated.
Some patients may be referred for further assessment, such as endoscopy, liver transplant evaluation, and a potential consultation with interventional radiologists for TIPS procedure. Vaccines against pneumococcus and hepatitis both A and B should be considered.
In extreme cases of uncontrolled, life-threatening bleeding, esophageal transection or liver transplantation may be required. Patients may also need to be admitted to the intensive care unit to manage acute bleeding and stabilize vital signs.
Treatments like percutaneous transhepatic embolization have been used to stop variceal bleeding. However, its effectiveness remains questionable. It is generally reserved for patients who are not candidates for surgery. Similarly, TIPS is a procedure that can be used to stop variceal bleeding. It comes with risks, such as encephalopathy and blocking of the shunt, and is often seen as a bridge to a liver transplant.
What else can Esophageal Varices be?
- Acute stomach lining damage
- Sores in the small intestine (duodenal ulcers)
- Sores in the stomach (gastric ulcers)
- Stomach cancer
- Mallory-Weiss tear (a tear in the lining of the throat or stomach usually due to prolonged vomiting)
- Injuries caused by a nasogastric tube (a tube inserted through the nose into the stomach)
- Disease of the stomach lining due to high blood pressure in the liver (portal hypertensive gastropathy)
What to expect with Esophageal Varices
If a patient experiences variceal bleeding once, there’s a 70% likelihood that it could happen again. Out of the repeated occurrences, about 30% could be fatal. Most fatalities usually happen in the first few days after the bleeding episode. The chances of death are significantly higher if surgery is required or in cases of intense, sudden variceal bleeding.
Variceal bleeding involves enlarged veins usually found in the esophagus or stomach. This generally happens when live damage leads to high blood pressure in the veins that carry blood to the liver, causing the veins in the esophagus and stomach to swell.
Possible Complications When Diagnosed with Esophageal Varices
Possible Risks:
- Inhaling foreign material into the lungs (Aspiration)
- Failure of multiple bodily organs (Multiorgan failure)
- Brain condition that causes confusion, memory loss, and physical instability (Encephalopathy)
- Esophagus rupture (Perforation of the esophagus)
- Potential death
