Biliary atresia

Biliary Atresia

Overview

Biliary atresia is a congenital condition in which the bile ducts do not develop properly. Babies develop biliary atresia in utero, but most infants show symptoms 2-6 weeks after birth. Prolonged obstruction leads to accumulation of the bile secretions within the liver cells, damaging them and leads to complications.

Signs and symptoms

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.

Causes and risk factors

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.

Complications

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.

Diagnosis

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.

Treatment and Surgical Interventions

Biliary atresia can sometimes be corrected by performing a surgery called Kasai procedure early after birth. The procedure connects the liver to the small intestine, bypassing the abnormal bile ducts. The liver can recover in about half the children with reduction in jaundice depending the severity of the biliary atresia and the age at surgery (ideally within 3 months). Non-treatment or an unsuccessful result can lead to continued jaundice, portal hypertension, repeated infections and failure to grow Liver transplantation is the only curative option in this stage and gives very successful long-term results.

Prevention

Biliary atresia is a congenital condition and cannot be prevented.

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