Biliary Atresia

GENERAL INFORMATION:

What is biliary atresia?

• Biliary (bil-e-AR-e) atresia (ah-TRE-zhah) is a serious liver and bile duct disease that is seen in infants. This occurs when the bile ducts inside and outside the liver are blocked or missing. The liver makes liquid, called bile, which helps in the digestion (break down) of food and fats. With biliary atresia, bile flowing from the liver and gallbladder, which goes to the intestines (bowel), is blocked. The bile gets trapped and accumulates (stores up) in the liver. This causes inflammation (swelling), damage, and cirrhosis (scarring) of liver cells. Over time, this scarring may prevent blood flow through the liver and make it stop working permanently.
  
  
• The liver makes bile, which is carried by the biliary tract to the intestines. The biliary tract is a network of channels and ducts. The gallbladder stores the bile. The liver helps the body function properly, including making enzymes and removing harmful materials from the blood. The liver is in the upper right side of the abdomen (belly).

What causes biliary atresia? The exact cause of biliary atresia is not known. Caregivers are certain that this disease is not inherited. The bile ducts are believed to be damaged before or shortly after your baby's birth. The following are possible causes of biliary atresia:

• Autoimmune diseases: The immune system is the part of your baby's body that fights infection. His body might have attacked the bile duct cells before or after birth.
  
  
• Developmental defects: Bile ducts may not have formed properly during pregnancy. Other organs are sometimes involved, such as the heart, spleen, intestines, or blood vessels. Sometimes, defects occur with your baby's genes causing a problem in his liver or biliary tract.
  
  
• Infections: Germs, such as viruses, may have damaged the liver cells. These viruses include those that cause diarrhea (frequent watery stools), mumps, or hepatitis (swelling of the liver).
  
  
• Toxins: Toxins (harmful substances), such as alcohol or bile acids, may cause biliary atresia in your baby.

What are the signs and symptoms of biliary atresia? Jaundice (yellowing of the skin or the whites of the eyes) is the most important sign of biliary atresia. This may appear shortly after birth or from 2 to 8 weeks after birth. Your baby may also have one or more of the following:

• Abdominal (belly) pain or enlargement.
  
  
• Dark or cola-colored urine.
  
  
• Discomfort or irritability.
  
  
• Itchiness.
  
  
• Weight loss or poor growth.
  
  
• White, gray, or clay-colored stools.

How is biliary atresia diagnosed? Your baby may have one or more of the following tests:

• Blood and urine tests: Different tests, such as a liver profile and blood counts may be done to your baby. Tests to see how well your baby's blood clots may be done. A sample of his urine may also be collected and sent to the lab to help diagnose your baby's disease.
  
  
• Imaging tests:
  
  
  • Abdominal ultrasound: An abdominal ultrasound is a test that looks inside of your baby's abdomen. Sound waves are used to show pictures of his liver, gallbladder, and bile ducts on a TV-like screen.
    
    
  • Abdominal x-rays: Abdominal x-rays are pictures of the organs inside your baby's abdomen. Caregivers use these pictures to look for problems, such as an enlarged liver or spleen or blocked intestines.
    
    
  • Cholangiogram: This test checks to see if the bile ducts are open or blocked. A dye is injected into your baby's gallbladder and goes through the bile ducts. X-rays are taken to learn if the dye flows normally into the small intestines and the liver.
    
    
  • Liver and gallbladder scan: This test may also be called a HIDA scan. This is a test to look at the liver and gallbladder. Your baby will be given a small amount of radioactive dye in his IV. Pictures are then taken by a special scanner that can see the dye in his body. Caregivers look at the pictures to see if his liver and gallbladder are working normally.
    
    
  • Scintigraphy: This is a test to look at the flow or movement of blood through the liver and bile ducts. Your baby is given a small, safe amount of dye in an IV. Pictures are then taken of his abdominal organs and blood vessels.
  
  
• Liver biopsy: This checks your baby's liver by taking a sample from his liver. With an ultrasound as a guide, caregivers put a needle through your baby's skin and into his liver. Tissue samples may be taken and sent to a lab for tests.

How is biliary atresia treated? At this time, there is no cure for biliary atresia. Caregivers may do a surgery to bypass the damaged ducts and connect the liver to your baby's small intestine. This new duct allows bile to pass from the liver into the intestine. Success is more likely if this surgery is done before the baby is two months old. If this surgery fails, a liver transplant may be needed. This is done by removing your baby's whole liver, or a part of your baby's liver, and replacing it with a healthy, donated liver. Medicines may also be given to help ease your baby's signs and symptoms. With treatment, such as medicine and surgery, your baby's symptoms may be relieved and his quality of life may be improved.

Where can I get more information? Accepting that your baby has biliary atresia may be hard. You, your family, and those close to you may feel scared, sad, or angry. These are normal feelings. Talk to your baby's caregivers, your family, or friends about your feelings. You may also want to join a support group. This is a group of people who have babies with biliary atresia. Contact the following for more information:

• American Liver Foundation
  75 Maiden Lane Suite 603
  New York, New York 10038
  Phone: 1-212-668-1000
  Phone: 1-800-465-4837
  Web Address: http://www.liverfoundation.org
  

• National Digestive Diseases Information Clearinghouse (NDDIC)
  2 Information Way
  Bethesda, MD 20892-3570
  Phone: 1-800-8915389
  Web Address: www.digestive.niddk.nih.gov
  

CARE AGREEMENT:

You have the right to help plan your baby's care. To help with this plan, you must learn about your baby's health condition and how it may be treated. You can then discuss treatment options with your baby's caregivers. Work with them to decide what care may be used to treat your baby.

Copyright © 2008 Thomson Healthcare Inc. All rights reserved. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.

The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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