Articles / Journals

Ramachandran, Priya, Mohamed Safwan, Mettu Srinivas Reddy, and Mohamed Rela. “Recent trends in the diagnosis and management of biliary atresia in developing countries.” Indian pediatrics 52, no. 10 (2015): 871-879.


What is biliary atresia?

Biliary Atresia – long term outcomes

What is Biliary Atresia?

Biliary atresia is a rare disease affecting the liver and bile ducts. The condition is seen immediately after birth. In an infant or baby with biliary atresia, bile flow from the liver to the gallbladder is blocked. The bile is trapped within the liver and causes scarring and damage leading to cirrhosis and ultimately liver failure. Biliary atresia is fatal if it is left untreated.

The incidence of biliary atresia is reported as 1 in every 9000 children.

Early surgical intervention is the best course of action for biliary atresia. Liver transplant is an option when the child is diagnosed late (beyond the age of 12 months).

Biliary atresia is not an inherited disorder. Hepatologists are still unsure of the origin of this condition. In some children, biliary atresia occurs because the liver or bile ducts were not properly formed during pregnancy. Other causes of biliary atresia include:
  • acquired viral or bacterial infection soon after birth
  • an issue in the immune system due to which the liver and bile ducts are attacked
  • an abnormal bile
  • component
  • certain spontaneous genetic mutations which predispose the baby to biliary atresia
Biliary atresia has two major types:
  • fetal form which develops during the development of fetus in the womb and presents itself at birth. Children with this form of biliary atresia may also present with other abnormalities like defects in the spleen, heart and intestines
  • perinatal form which presents itself in the second to fourth week of the child’s birth

Biliary atresia usually presents itself as jaundice with yellowing of the eyes and skin. Jaundice is caused by the liver’s inability to get rid of the excess bilirubin (yellowish pigment) from the blood. Hence it enters the bloodstream and leads to yellow eyes and skin. The excess bilirubin pile up in the liver is caused by the blocked bile ducts.

The problem in detecting biliary atresia is jaundice is usually normal even in healthy newborns during the first 2 weeks. This is due to an immature liver. The jaundice clears up after 2 weeks on its own.

If the cause of jaundice is biliary atresia, it will be a persistent sickness which does not clear up. In the case, the infant must be immediately checked for liver dysfunction. The infant may also present with dark color urine and pale stools.

Progressive jaundice in infants with no sign of abatement is usually an alarm bell to check for biliary atresia. Biliary atresia is usually diagnosed using a combination of lab tests and imaging studies.
  • Lab tests – blood tests like liver function indicate elevated levels of bilirubin and other components
  • Abdominal ultrasound – gives the image of the liver and associated structures like gallbladder and bile ducts
  • Hepatobiliary Iminodiacetic (HIDA) scan – gives an image of bile flow from the liver through the gallbladder and extrahepatic biliary ducts into the intestine
  • Liver biopsy – where a tissue sample is taken with a needle and examined under a microscope. This may indicate the need for surgery
  • Intraoperative cholangiogram – is the final confirmatory test for biliary atresia. A contrast dye is injected into the gallbladder and the bile flow is constantly monitored. If there is no flow into the extrahepatic biliary ducts, a diagnosis of biliary atresia is confirmed

Surgical interventions are the only available treatment option for biliary atresia. If the diagnosis is confirmed in the early stages, a Kasai surgery procedure ((hepato-portoenterostomy) is possible to reconstruct the bile ducts and get the flow channeled properly. In this procedure, the blocked extrahepatic bile ducts and the gallbladder are replaced by a portion of the child’s intestine, which is sewn directly to the liver. This creates a new extrahepatic bile duct system.

Despite a Kasai procedure, many babies will still require a liver transplant before the age of 5. Usually babies with the fetal form of biliary atresia require a transplant immediately whereas those with the perinatal form can wait till about 3-4 years of age. Liver transplant is usually successful in and survival post-surgery has increased in the last decade with newer immunosuppressants and transplantation medicine.

Parents or other relatives are most eligible to donate a portion of their liver for the baby/child. Healthy liver tissue regenerates very fast so the donor and child can grow back their livers.

Individuals with biliary atresia who have undergone Kasai surgery or liver transplant must be encouraged to participate in all life activities. Children with liver transplants need to be careful about catching infections and must consult a doctor whenever he/she catches an infection. Normal diet and exercise is encouraged for post-transplant children.