Biliary Atresia

The most obvious symptom of biliary atresia is jaundice in the infant. While many newborns have jaundice, it usually improves over the first few weeks. Persistent jaundice beyond the first month, especially in association with pale clay-coloured stools, is strongly suspicious of biliary atresia and needs urgent medical attention.

The exact cause of the disease is unknown but it appears to be triggered by viral infections, auto-immune conditions, exposure to harmful chemicals or a gene mutation. Premature babies seem to be most at risk.

Biliary atresia causes early permanent liver scarring, loss of liver function and cirrhosis. When untreated children develop serious complications within the first few years of age.

Jaundice beyond the first month after birth, along with pale clay coloured stools, is an important finding and can be easily detected by the parents. Diagnosis is based on a physical exam, an abdominal ultrasound, a panel of blood tests, a cholangiogram and possibly a liver biopsy.

Biliary atresia can be corrected by performing a Kasai procedure. The procedure connects the liver to the small intestine, bypassing the abnormal bile ducts. The procedure is most successful when performed before three months of age. When performed in a timely fashion, the liver can recover in about half the children. Failure of the procedure leads to continued liver failure and episodes of infection. Liver transplantation is the only curative option in this stage and gives very successful long-term results.

Biliary atresia is a congenital condition and cannot be prevented.