Cirrhosis is a condition in which the cells of the liver are damaged and can't repair themselves. As liver cells die, scar tissue forms. Cirrhosis occurs with many liver diseases.

Blood is carried to the liver by a major blood vessel called the portal vein. If blood can't flow easily through the liver because of cirrhosis, the blood gets slowed down in this vein and the pressure inside the vein increases. This higher blood pressure in the portal vein is called portal hypertension.

When blood doesn't flow normally through the portal vein, it must return to the heart using other blood vessels. These vessels become swollen because of the increased amount of blood flowing through them. They are called varices. Varices have thin walls and can easily break open because they aren't meant to handle such high-pressure blood flow. Bleeding from a broken blood vessel (variceal bleeding) is serious and can be life-threatening. Also, because the portal vein is blocked, toxins in the blood are not cleaned by the liver and therefore remain in the body.

Symptoms

• Loss of appetite
• Weakness and fatigue
• Nausea
• Jaundice (yellowing of the skin and eyes)
• Dark brown urine
• Red palms
• Vomiting blood
• Itchiness of the skin
• Abdominal swelling

Exams and Tests

During a physical examination your child's pediatric GI specialist may find:

• An enlarged liver or spleen
• Excess fluid in the tissues lining the abdomen (ascites)
• Swollen abdomen
• Reddened palms
• Red spider-like blood vessels on the skin
• Smaller (contracted) fingers
• Widened (dilated) veins in the abdomen wall
• Yellow eyes or skin (jaundice)

Liver Biopsy

A liver biopsy may be done to confirm cirrhosis. Your child will receive sedation medications or a general anesthetic before the biopsy. These medications will prevent your child from feeling pain or anxiety or remembering the procedure. An area on the right side of your child's abdomen or mid-upper abdomen will be cleansed with an antibacterial soap. The biopsy site will then be numbed with an injection of lidocaine (numbing medicine). The pediatric gastroenterologist will insert a needle through the skin and into the liver to obtain a very small piece of liver tissue. The sample will be sent to a lab and analyzed. The doctor will apply a small pressure dressing (bandage) to the site.

After the biopsy your child will stay in or return to his/her room for four hours, when a repeat blood count is checked. (Children less than one year are observed overnight.) He or she should lie on the right side or back, depending on the biopsy site, for two hours. Being in bed will reduce the chance of bleeding after the biopsy. Once your child is awake and alert, he/she will be allowed to drink fluids. If fluids are tolerated, a regular diet can be restarted. For the remainder of the day, your child will need to participate in quiet activity. The results of the liver biopsy are usually available two weeks after the procedure.

Treatment

Once liver cells have been damaged, nothing can be done to repair the liver or cure cirrhosis. Treatment is aimed at avoiding further damage to the liver and preventing and treating complications (such as variceal bleeding).

Medications

Your child's pediatric GI specialist may prescribe a medicine to help prevent your child's blood vessels from breaking open. Drugs that prevent broken blood vessels have some side effects. Not everyone can take them.

Endoscopy

If medicine isn't enough, a scope procedure may help stop the bleeding from broken blood vessels. One option is to interrupt the flow of blood to swollen varices in the area where the esophagus (the tube leading to the stomach) attaches to the stomach. With the child asleep under anesthetic, a long lighted tube is passed through the mouth to the stomach. Then, rubber bands or hardening chemicals are placed on the swollen blood vessels to block them off.

Surgery

A person with portal hypertension may need to have a shunt surgery to drain the portal vein blood into another vein so it won't back up into the varices. Another kind of procedure, called TIPS (transjugular intrahepatic portosystemic shunt), may be done in some cases.

For More Information

American Liver Foundation - liverfoundation.org

National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health - digestive.niddk.nih.gov/ddiseases/pubs/cirrhosis/index.htm