Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence. Biliary atresia is a serious condition that affects infants. It is characterized by hepatic bile ducts, the bile ducts in the liver that do not. Liver and intrahepatic bile ducts – nontumor – Extrahepatic biliary atresia.

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Early occurrence of hepatocellular carcinoma in biliary atresia treated by liver transplantation. The intussusception antireflux valve is ineffective for preventing cholangitis in biliary atresia: Small veins from the portal vein to the portal remnant should also be divided to expose the caudate lobe posteriorly. Bileir Source of Support: Most common cause of pathologic infant jaundice; common reason for pediatric liver transplantation Stenotic or atretic portions nilier extrahepatic biliary tree cause chronic extrahepatic large duct obstruction Histologically resembles choledochal cyst or other causes of large duct obstruction Note: This page was last edited on 30 Atrewiaat A multicenter study of the outcome of biliary atresia in the United States, to The bilirubin is then filtered by the kidney and removed in the urine.

In some infants, there is evidence to suggest that the process begins atrewia in gestation. Moreover, the baby feeds aflatoxin M1 from its mom, as aflatoxin M1 is the detoxification product of aflatoxin B1. A possible link between cytomegalovirus infection and extrahepatic biliary atresia.


National Center for Biotechnology InformationU. See other articles in PMC that cite the published article.

Increased expression of intercellular adhesion molecules in biliary atresia. A number of factors contribute to good outcome and may be listed as:.

Biliary atresia in two sets of twins.

Biliary atresia | Radiology Reference Article |

Prolonged neonatal jaundice beyond 2 weeks of age and identification of primarily conjugated hyperbilirubinemia frequently raise the index of suspicion for the condition. For these infants the aetiology lies within the first trimester of gestation. There are also some cases wherein the Kasai procedure was deemed successful but the patient still developed cirrhosis of the liver. Key investigations include ultrasonography, biochemical liver function tests, viral serology, and a percutaneous liver biopsy.

However, if a Kasai procedure is not performed in a child with biliary atresia, the patient will not be expected to live beyond the age of 2. Hepato-pulmonary HP syndrome is characterized by hypoxia, cyanosis, dyspnoea and clubbing and is due to development atfesia pulmonary arterio-venous shunts.

Am J Dis Child. Neonatal hepatitis and extrahepatic biliary atresia associated with cytomegalovirus infection in twins.

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Biliary Atresia

Please review our privacy policy. An attempt to preserve the infant’s own liver. Bacterial cholangitis after surgery for biliary atresia. If successful, any patent intrahepatic bile ducts will drain into the roux limb allowing relief of the biliary obstruction.


The outcome following the Kasai operation can be assessed in two ways: Although the procedure is not thought of as curative, it may relieve jaundice and atrexia liver fibrosis, allowing normal growth and development. It also carries waste products from the liver to the intestines for excretion. Some may be due to a defect in early bile duct development particularly those with other abnormalities and some may arise in the perinatal period due to an external cause such as an hepatotropic virus reovirus 3 infection, [9] congenital cytomegalovirus infection, [10] and autoimmunity.

Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and in our centre a percutaneous liver biopsy.

Biliary atresia

Genetics may play a role in the pathogenesis of BA, perhaps in predisposing to the detrimental effects of hepatotropic viral infection. D ICD – These changes may produce problems such as easy bruising of the skin, nosebleeds, retention of body fluid and enlarged veins varices in the stomach and esophagus.

The Kasai procedure is biler operation to re-establish bile flow from the liver into the intestine.

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