Biliary atresia is a rare condition present in newborn infants in which the common bile duct between the liver and the small intestine is blocked or absent. The obliteration or discontinuity of the extrahepatic biliary system results in obstruction to bile flow. If the condition is undetected, it leads to liver failure. The cause of the condition is unknown.

Symptoms

The symptoms are indistinguishable from neonatal jaundice amd are usually evident between one and six weeks after birth. Other symptoms include:

• clay colored stools,
• dark urine,
• swollen abdomen,
• large hardened liver.

Diagnosis

Prolonged jaundice that is resistant to phototherapy should prompt a search for secondary causes.Doctors may perform ultrasound investigation or other forms of imaging. Further testing include radioactive scans of the liver and a liver biopsy.

Treatment

Possible surgical reconstruction of the extrahepatic biliary tract (called a Kasai procedure) or hepatoportoenterostomy. In case of the complete atresia, liver transplantation is the only option. A considerable number of the patients who have undergone a successful Kasai procedure eventually undergo liver transplantation within a couple of years following the procedure.

Corticosteroid treatment and antibiotics have a beneficial effect on the postoperative bile flow and, thereby clear the jaundice of the patients, but the dose and duration of the ideal steroid protocol has been controversial.

Prevalence

Biliary atresia is a very rare disorder. About one in 10,000 to 20,000 babies in the U.S are affected every year. Biliary atresia seems to affect girls slightly more often than boys. Asians and African-Americans are affected more frequently than Caucasians.