Digestive System Disorders

Resources and information on ulcerative colitis, crohn's disease, IBS, diverticulitus, and other common digestive ailments

Home

Common Ailments:

Other Articles:

 

Biliary Atresia

What Is Biliary Atresia?

Biliary atresia is a rare condition that affects newborn infants and is considered a type of birth defect. It blocks the tubes that carry a digestive liquid, called bile, from the liver to the gallbladder. This blockage occurs when the bile ducts, located inside or outside the liver, do not develop normally. This lack of development is very serious due the nature of the bile ducts themselves. The function of the bile ducts is to help remove waste from the liver and carry salts that help the small intestine break down (digest) fat. When this bile flow is blocked, it can lead to potentially fatal conditions such as liver damage and cirrhosis of the liver if it is left untreated.

How Do I Know My Baby Has Biliary Atresia? What Are The Symptoms?

Babies that are born with biliary atresia appear to be normal at birth; however, symptoms start showing by the time they reach 2 to 3 weeks old. Yellowing of the skin and eyes, known as jaundice, might develop as the first symptom at around 2 to 3 weeks, while weight loss and irritability might appear after the first month. It is noted that some babies might experience jaundice at birth while some develop it over several months. It might either get worse or come and go. Other symptoms to look for would be:

  • Dark urine
  • Slow growth in a child 0 - 5 years old
  • Pale or clay-colored, foul smelling, floating stools
  • Enlarged spleen

    If you notice any of these symptoms with your infant, please contact your child's pediatrician immediately.

How Is Biliary Atresia Diagnosed?

If the diagnosis of biliary atresia is suspected, your child's pediatrician will refer your newborn to a specialist to treat the condition. Specialists who treat biliary atresia include a pediatric hepatologist, who is an expert in liver disease of children, a pediatric gastroenterologist, who is an expert in digestive diseases of children and a pediatric surgeon.

Initial tests will include a physical exam where the physician will press on the baby's abdomen to check for an enlarged liver. Other test will include blood, stool and urine samples to check for liver disease. In the blood test, the level of bilirubin will be measured as well as other tests for other problems will be done. These other tests would include abdominal x-ray and ultrasound, liver scans such as Hepatobiliary iminodiacetic acid (HIDA) scan, an x-ray of the bile ducts and a liver biopsy.

While abdominal x rays and ultrasounds cannot be used to make the diagnosis of Biliary atresia, they can help rule out the common causes of the jaundice and can show whether the liver or bile ducts are enlarged and whether tumors or cysts are blocking the flow of bile.

Liver scans such as the Hepatobiliary iminodiacetic acid (HIDA) scan, can detect a blockage of bile flow. These liver scans use substances that can be detected by the special cameras to create an image of the liver and bile ducts.

In a liver scan, the baby will be sedated while a needle is injected through the skin and then quickly in and out of the liver. A small piece of liver is extracted from the infant for examination under a microscope. This examination will not only rule out other liver conditions, such as hepatitis, but can also usually show whether biliary atresia is likely.

How Is Biliary Atresia Treated?

The only treatment for biliary atresia at this time is surgery. In the procedure, the baby will be under general anesthesia while the physician will make an incision to examine the bile ducts and liver. If the diagnosis of biliary atresia is confirmed, one of the surgical options, the Kasai procedure, is performed while the baby is still asleep. In this procedure, the bile ducts are removed and a loop of small intestine is brought up to replace the bile ducts and drain the liver resulting in bile flowing from the small bile ducts straight into the intestine. If the Kasai procedure is unsuccessful, the second option, a liver transplant, is necessary. A liver transplant, if done within the first 1 to 2 years of life, will improve the infant's chances of survival.

***DISCLAIMER***
This site is meant only to provide information and resources about digestive system disorders. It is not meant to diagnose any digestive problems. If you suspect you may have some digestive disorder, then please, see your doctor, and get his professional opinion and advice.