Portal Hypertension Liver Disease

  • While liver disease is rare in children, portal hypertension is even less common. For this reason, few centers are able to adequately manage the care of children with this serious condition. Over the next few pages we will introduce you to the basics of portal hypertension, its causes, signs and symptoms, and how the physicians in the Center for Childhood Liver Disease at Boston Children's Hospital care for children with portal hypertension.

    The liver is a large organ (the second largest in the body) responsible for processing nutrients from our food and medications in ways that allow the rest of the body to make use of them. It also acts as a big filter for the bloodstream, removing poisons and toxins as well as byproducts made by our cells and tissues. The liver helps to control our blood sugar and cholesterol, and it produces chemicals that help our blood to clot.

    The liver is truly unique in how it receives its blood supply. Nearly every tissue and organ in the body receives nourishment and oxygen from blood delivered through an artery, which carry blood under high pressure due to the heart’s pumping action. The liver, however, is the only organ to receive the majority of its blood supply through a large vein. Called the portal vein, this vein carries blood to the liver from the intestines and the spleen.

    Why would the liver work this way? The pressure in veins, including the portal vein, is much lower than in arteries. The reduced pressure allows blood to percolate through the liver and gives the liver’s cells the time they need to do their work.

    Obstruction of the portal vein – caused primarily by clots in or narrowing of the vein before it reaches the liver, cirrhosis, or high pressure in the veins that drain the liver into the heart – can cause the pressure in the vein to build up, much as blocking the end of a hose causes the pressure in the hose to climb. Increased portal vein pressure – known medically as portal hypertension – causes blood to back up in the organs that send blood to the liver. The body tries to relieve the pressure by generating new blood vessels that bypass the blockage, but such vessels are often weak and twisted, and tend to bleed easily. These vessels, called varices, may also bypass the liver itself, allowing toxins and nutrients to travel through the bloodstream unprocessed.
     

    How Boston Children's Hospital approaches portal hypertension

    The weak blood vessels produced by the body in response to portal hypertension put a child at risk for gastrointestinal bleeding (bleeding into the intestine that causes vomiting of blood and/or passage of blood from the rectum). The major goals of the physicians, nurses, and staff in the Center for Childhood Liver Disease in treating children with portal hypertension are to reduce that risk and, ideally, to reduce the increased blood pressure in the portal vein by identifying its root cause. At every step, our specialists endeavor to provide compassionate care that respects the values of each family and addresses their hopes and concerns for their child’s present and future health.

    Center for Childhood Liver Disease 

     

    Boston Children's Hospital
    300 Longwood Avenue
    Fegan 5
    Boston MA 02115

     617-355-5837 
     fax: 617-730-0716

  • What is the liver, and what does it do?

    The liver is the body’s second largest organ, located in the right side of the abdominal cavity below the diaphragm and above the right kidney and intestines. The liver helps the body in hundreds of ways.

    • All of the blood coming from the stomach and intestines passes through the liver through a large vein called the portal vein. The liver turns nutrients from the food we eat and chemicals from the medicines we take into forms that the rest of our bodies can use.

    • The liver helps clean the bloodstream of harmful substances and poisons.

    • The liver makes bile, which contains chemicals to help us digest the food we eat.

    • The liver helps control blood sugar and cholesterol levels

    • The liver makes the proteins that allow blood to clot normally.

    What is portal hypertension?

    Portal hypertension describes an increase in pressure within the portal vein, the vessel that carries blood from the organs of the digestive system (the intestines and the spleen) to the liver.

    What happens to the liver and other organs in portal hypertension?

    The increased blood pressure in the portal vein causes pressure in other blood vessels to increase as well. For instance, rising blood pressure can force blood to back up into the splenic vein, causing the spleen to swell. Pressure back into the veins of the intestines can cause diarrhea.

    As the body senses the increased portal vein pressure, it tries to compensate by growing new veins that bypass the liver. These new veins, called varices, tend to be full of twists and turns, are much weaker than normal veins, and can bleed very easily. The varices tend to grow around the esophagus, spleen, stomach, and colon.

    Because varices bypass the liver, toxins and nutrients that the liver would normally process can travel into the rest of the bloodstream.

    Causes

    What causes portal hypertension?

    Portal hypertension results from a blockage in the portal vein before (“prehepatic”), within (“hepatic”), or past (“posthepatic”) the liver.

    Prehepatic blockages, the most common cause of portal hypertension in children, stem from blood clots or narrowing of the portal vein before it reaches the liver. In response, the body grows varices that bypass the blockage but which, instead of relieving the pressure, lead to what is called “cavernous transformation of the portal vein.”

    Cirrhosis is the most common cause of portal hypertension in adults, and the second most common in children. This progressive scarring of the liver is a result of long-term illness or damage to the liver caused by a wide range of conditions. In a child with cirrhosis, the liver’s soft, healthy tissue is gradually replaced with hard, nodular tissue that blocks the flow of blood through the portal vein.
     

    Signs and symptoms

    What are the symptoms of portal hypertension?

    Portal hypertension itself usually causes no symptoms, though its complications can. For instance, varices can rupture and bleed easily, causing gastrointestinal bleeding evidenced by black, tarry stools or vomiting of blood. Fluid buildup, called ascites, can cause the abdomen to swell and enlarge. Some children feel vague discomfort in the upper left part of the abdomen, caused by enlargement of the spleen.

    Because portal hypertension is itself often a complication of advanced liver disease, children with it may also experience symptoms of poor liver function. They may experience poor weight gain or weight loss, jaundice and confusion or forgetfulness due to the presence in the bloodstream of substances, such as toxins, that are normally filtered by the liver.
     

    How is portal hypertension diagnosed?

    Portal hypertension is often diagnosed through a combination of imaging and endoscopic studies. These techniques give physicians a comprehensive picture of the state of the portal vein and the organs and vessels connected to it.

    How do you treat portal hypertension?

    With portal hypertension, the main goal of therapy is to prevent further complications and reduce the risk of variceal bleeding while, ideally, reducing the pressure in the portal vein. This is accomplished through a combination of medication, endoscopic treatment (“banding” of varices), and surgical therapies (shunting of the blood flow in the portal venous system into the systemic circulation).

    Because portal hypertension can be a late complication of liver disease, such as cirrhosis, it is important to try to manage the conditions that caused damage to the organ in the first place. Should the liver begin to fail, a liver transplant may become necessary.

    How can portal hypertension affect my child in the long term?

    Because portal hypertension is typically a chronic condition, your child may have to seek care for it for the rest of his or her life. The Center for Childhood Liver Disease can help you and your child plan for the eventual transition from pediatric to adult care.

  • The first step in treating your child is forming an accurate and complete diagnosis. Because portal hypertension can cause a variety of complications, your child’s doctor will likely already be on the lookout for signs such as gastrointestinal bleeding, enlargement of the spleen, the development of varices, and the presence of abdominal swelling (ascites).

    Imaging technologies can provide a great deal of information on the anatomy and function of the portal vein and surrounding organs and vessels. Using ultrasound, a painless and non-invasive imaging technology, physicians can see the direction and speed of the blood flow through the portal vein. In addition, the technology lets them assess the state of the liver, spleen, and gallbladder, and also see whether varices have developed. Often, ultrasound is the first way in which “cavernous transformation of the portal vein” is diagnosed. The portal vein and related blood vessels can also be seen using other techniques, such as a special CT scan (called a “CTA” or “CT angiogram”) or magnetic resonance imaging (MRI).

    If your child has portal hypertension, even if he or she has not yet had gastrointestinal bleeding, physicians may use an endoscope – a thin, flexible, lighted tube – to look for varices in the esophagus. If your child is old enough and can swallow a capsule, a wireless capsule endoscopy may be done instead. In this case, a tiny camera in a capsule sends digital pictures to a computer as the capsule itself goes down the esophagus.

    If varices are bleeding, our physicians can also use an endoscope to deliver some forms of treatment aimed at controlling this complication.

  • As there is no simple cure for portal hypertension, our Center for Childhood Liver Disease takes a multidisciplinary approach to preventing the condition from becoming worse while addressing the risk of gastrointestinal bleeding.

    Physicians often prescribe treatment with a medication called a nonselective beta blocker, such as propranolol or nadolol, which can help lower the blood pressure within the portal vein.

    Control or prevention of bleeding from varices is a high priority with portal hypertension. To do this, physicians often use an endoscope to tie off varices using a rubber band (a procedure known as “banding”) or to deliver sclerosing therapy. In this kind of therapy, a physician injects a chemical into the varices directly, causing them to clot.

    If your child develops significant ascites, our physicians may try to relieve the fluid load with diuretic medications or, if necessary, by draining the fluid from the abdomen with a needle (a non-surgical procedure called abdominal paracentesis).

    Should your child experience continued internal bleeding, doctors may create a bypass or shunt between the portal vein and the rest of the bloodstream. Our physicians often use one of two types of shunting procedures, transjugular intrahepatic portal-systemic shunting (TIPSS, a non-surgical procedure involving use of a catheter) or surgical shunting. Both procedures relieve the pressure on the portal vein and redistribute it to the rest of the bloodstream.

    Because portal hypertension is an advanced complication of other forms liver disease, such as cirrhosis, it is important to try to manage the conditions that caused damage to the organ in the first place. Should liver function begin to fail, a liver transplant may become necessary.

  • Many of the drugs and procedures used to treat portal hypertension originated in the realm of adult care. The Center for Childhood Liver Disease has been at the forefront of adapting these adult procedures for children, including development of techniques and tools appropriate for a child’s smaller body.

    Our physicians are also early adopters of a new wireless endoscopy technology for liver disease surveillance and treatment. Wireless esophageal endoscopy centers on the use of a capsule containing two cameras. The child swallows the battery-powered capsule; the cameras take many photographs per second as the capsule travels through the esophagus. This gives physicians a very clear view of any varices in the esophagus or gastrointestinal tract, does not require any sedation or anesthesia, and is much more comfortable than standard endoscopy.

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The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
- Sandra L. Fenwick, President and CEO
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