What is Biliary Atresia?
Cells within the liver secrete a liquid called bile, which is made up of cholesterol, bile salts and waste products, including bilirubin. A network of tubular structures and tiny ducts form the biliary system to drain bile from the liver to the small intestine where it aids in the digestive process. Biliary atresia is the closure or disappearance of the biliary system.
Biliary atresia is a progressive inflammatory process that begins very soon after birth. In the most common form, called extrahepatic biliary atresia, the delicate ducts outside the liver are affected first. White blood cells invade the ducts, which become damaged and may whither or completely disappear. Bile is trapped inside the liver and rapidly causes damage and scarring to the liver cells. Further scarring of the liver tissue may result in cirrhosis.
On average, there is one case of biliary atresia out of every 15,000 live births. Females are affected slightly more often than males. In the United States, approximately 300 new cases are diagnosed each year.
What Causes Biliary Atresia?
The cause of biliary atresia is unknown. Auto-immune mechanisms may be partly responsible for the progressive process that takes place. Recent research suggests that a viral infection in susceptible infants could result in biliary atresia. About 10% of cases have other associated congenital defects in the heart, blood vessels, intestine, or spleen.
Although the cause is uncertain, it is known that biliary atresia affects only newborns; it is not hereditary; it is not contagious; and it is not preventable. Parents should be assured that biliary atresia is not caused by anything the mother did during pregnancy.
What Are The Symptoms of Biliary Atresia?
Symptoms
Newborns with this condition may appear normal at birth. However, jaundice (a yellow color to the skin and mucous membranes) develops by the second or third week of life. The infant may gain weight normally for the first month, but then will lose weight and become irritable, and have worsening jaundice.
Other symptoms may include:
Dark urine
Enlarged spleen
Floating stools
Foul-smelling stools
Pale or clay-colored stools
Slow growth
Slow or no weight gain
What Test Are Done To Diagnose Biliary Atresia?
The health care provider will perform a physical exam, which includes feeling the patient's belly area. The doctor may feel an enlarged liver.
Tests to diagnose biliary atresia include:
Abdominal x-ray*
Abdominal ultrasound*
Blood tests to check total and direct bilirubin levels*
Hepatobiliary iminodiacetic acid (HIDA) scan, also called cholescintigraphy, to help determine whether the bile ducts and gallbladder are working properly*
Liver biopsy to determine the severity of cirrhosis or to rule out other causes of jaundice*
X-ray of the bile ducts (cholangiogram)****In Nates case, none of these showed for sure biliary atresia,but all other causes had been ruled out. So he had to have an exploratory surgery to confirm diagnosis. Once visual diagnosis was made, they did the Kasai(pronounced KA-Sigh)
Whats the Treatment for Biliary Atresia?
Once the diagnosis is confirmed, the preferred treatment is to remove the atretic biliary ducts outside the liver and attach the small intestine directly to the liver at the spot where bile is found or expected to drain. The segment of intestine that connects to the liver also connects to the rest of the intestine and forms a Y connection called a "Roux-en-Y hepatoportojejunostomy," or Kasai procedure.
Bile flow is re-established in approximately 80% of infants who are operated on when younger than 3 months of age. Of these, about 50% will have some bile drainage and as many as 30% will have complete bile drainage with a return to normal bilirubin. About 20% of infants will not be helped by the Kasai procedure. In these cases, the only other treatment option is a liver transplant.
Early diagnosis of this disease is very important. If surgery is performed before the baby is 2 months old, success is much more likely. After 3 months, success of the operation is poor. For this reason, all infants who are jaundiced after the age of 4 weeks should be evaluated for biliary atresia
Whats the outcome of Biliary Atresia?
If left untreated, the result of blocked bile flow is damage to the liver such that few children survive beyond the age of two. When bile flow is only partly restored by surgery, the complications of cirrhosis will gradually develop. If the Kasai procedure is successful in draining the liver and returning the bilirubin back to normal, children may live many years with normal growth and activities. Some will grow into adulthood and some may never need a transplant.
More often, despite successful surgery, slow, progressive damage to the liver continues. When progressive cirrhosis occurs, it will eventually require liver transplantation.
Liver transplantation plays an important role in the long-term treatment of biliary atresia. Pediatric liver transplantation has evolved into a highly successful therapy and now offers significant hope for all children born with biliary atresia