Cirrhosis in Children

Cirrhosis is a progressive liver disease in which scar tissue replaces soft, healthy liver tissue. As scar tissue builds up, the liver becomes hard and slowly cuts off blood circulation in the liver. This interferes with the liver's many important functions, including the ability to process nutrients and filter toxins. In extreme cases, the liver stops working and the child will need a liver transplant.

Although a healthy liver has a remarkable ability to repair itself, chronic liver disease can lead to cirrhosis. In very young children, cirrhosis is most often caused by a genetic (inherited) liver problem such as biliary atresia. In older children, conditions such as Wilson disease and autoimmune hepatitis can cause cirrhosis.

While cirrhosis in adults is often caused by heavy alcohol consumption, a mother or father's drinking cannot cause cirrhosis in a child.

Cirrhosis is the most severe stage of liver scarring. If left untreated, cirrhosis can lead to serious complications such as:

• Kidney failure
• Malnutrition
• Diabetes
• Liver cancer

Children with cirrhosis bruise and bleed easily and are more susceptible to infection than other children.

The liver is the second largest organ in the body, located in the abdominal cavity. The liver helps the body in many ways:

• Produces proteins that allow blood to clot normally, transport oxygen, and support the immune system
• Produces bile, a substance that helps digest food
• Stores extra nutrients
• Helps clean the bloodstream of harmful substances
• Helps control blood sugar and cholesterol levels

Cirrhosis is a chronic condition. Children with cirrhosis will need ongoing medical monitoring and treatment for the rest of their lives.

Cirrhosis often produces no symptoms early on, though a child with cirrhosis may have symptoms related to the underlying medical condition causing the liver damage. As cirrhosis progresses, a child may have the following symptoms:

• Loss of appetite
• Nausea or vomiting
• Weight loss or difficulty gaining weight
• Weakness
• Abdominal pain or swelling
• Spider-like blood vessels on the skin

With time, cirrhosis may also lead to additional serious problems, including:

• Yellowing of the skin or the whites of the eyes, called jaundice
• Bruising or bleeding easily or nosebleeds
• Swelling of the legs or abdomen from built-up fluid — in the legs this fluid buildup is called edema; in the abdomen it is called ascites
• Confusion or difficulty thinking (encephalopathy) caused by buildup of waste products from food in the bloodstream
• Kidney failure

Because blood cannot flow as easily through a liver with cirrhosis, the pressure in the vein that enters the liver, called the portal vein, may increase. This condition is called portal hypertension and it can cause its own symptoms and complications. The increased blood pressure in the portal vein can also affect how blood circulates in the lungs, causing:

• Hepatopulmonary syndrome, a rare condition that interferes with the lungs' ability to supply oxygen to the rest of the body
• Esophageal varices, enlarged or swollen veins on the lining of the tube that connects the throat to the stomach

The liver has a great capacity to heal itself, but long-term illness or injury can scar the liver.

As liver damage continues, hard scar tissue gradually replaces the liver's healthy tissue and partially blocks blood circulation through the liver. The organ may shrink and take on a shriveled appearance.

Eventually the soft, smooth surface of the liver becomes covered in scars.

The following conditions can lead to cirrhosis in children:

• Biliary atresia, a blockage of the ducts that allow bile to pass from the liver to the gallbladder and intestines
• Autoimmune hepatitis, a disorder of the immune system that causes immune cells attack the liver as if it were an infection
• Chronic viral hepatitis or hepatitis C, which cause the liver to swell and can encourage scar tissue to form
• Cystic fibrosis, an inherited chronic disease that clogs the liver and other organs with thick mucus and can lead to scarring
• Alpha-1 antitrypsin deficiency, a genetic disease that prevents the liver from building a protein it normally releases into the bloodstream
• Primary sclerosing cholangitis, an acquired condition that causes inflammation and narrowing of the bile ducts around the liver
• Wilson disease, an uncommon inherited disorder in which the body cannot rid itself of excess copper found in many foods and allows copper to build up in the liver
• Fatty liver disease, a buildup of fat in the liver that can lead to scarring
• Some congenital (inherited) heart defects

While overconsumption of alcohol is the most common cause of cirrhosis in adults, no amount of alcohol consumption by either parent, even during pregnancy, can cause a child to develop cirrhosis.

Doctors usually base a diagnosis of cirrhosis on a combination of symptoms, medical history, physical exam, and blood tests. In some cases, the doctor may order a liver biopsy to confirm the diagnosis and determine how badly the liver is damaged.

In some cases, doctors use a system called the CTP or Child-Turcotte-Pugh ("Child") score to measure the severity of a child's cirrhosis. This scoring system, named for the surgeons who developed it, can provide a helpful picture of the status of a child's liver and other changes in the body caused by cirrhosis.

In most cases, there is no way to cure cirrhosis. Children with cirrhosis typically need ongoing care for the rest of their lives. The main goal of treatment is to protect the liver from further scarring and address the underlying medical condition that damaged the liver.

While the specific treatment may vary, healthy eating and regular monitoring are important for any child with cirrhosis.

Healthy eating

Because of the liver's important function in helping the stomach absorb nutrients, healthy eating is doubly important for children with cirrhosis or any other liver disease. The child's doctor may also prescribe caloric supplements or special formulas to promote healthy weight gain and development.

Regular monitoring

Cirrhosis can lead to complications in many organs and systems in the body. Regular checkups and monitoring enable clinicians to keep a close eye on the child and treat complications early. This may include:

• Diuretics to reduce swelling in a child's legs or abdomen
• Regular screening for esophageal varices (enlarged veins on the lining of the esophagus)
• Regular screening for portal hypertension or hepatopulmonary syndrome

Cirrhosis and medication

It is important to talk to a child’s pediatrician before giving the child any new medications or supplements. This even includes vitamins.

A damaged liver cannot break down medicine as quickly as healthy liver, so prescription drugs, over-the-counter drugs, and vitamin or herbal supplements may be more potent for a child with cirrhosis. Other times, the damaged liver may not convert the medicine or supplement into an active form.

Liver transplant

Two of the most serious risks of cirrhosis are liver failure and liver cancer. If either of these conditions develops, the child may need a liver transplant.

The physicians, nurses, and staff in the Center for Childhood Liver Disease at Boston Children’s Hospital have a deep understanding of the complexities and complications of cirrhosis. We have one of the few dedicated teams of specialists who are board-certified in pediatric hepatology and transplant, and we offer comprehensive resources to help families manage cirrhosis and plan for their child’s eventual transition to adult care.

Doctors rely on biopsies to diagnose and monitor cirrhosis and other forms of liver disease. A group of physicians and researchers at Boston Children’s is working on an alternative, non-invasive method to determine the amount of scar tissue present in a child’s liver. Through a combination of blood tests and ultrasound technology, it may be possible to measure the stiffness of the liver and diagnose cirrhosis with a quick, painless exam. In the future, this research could reduce the number of biopsies needed to effectively monitor and manage liver disease.