Biliary Atresia

Biliary atresia is a rare and serious liver disease in infants where the bile ducts inside or outside the liver become blocked or absent. This prevents bile from flowing out of the liver, leading to bile buildup, liver inflammation, and ultimately liver damage and cirrhosis. Without timely surgical treatment, the condition is fatal within the first few years of life.

Biliary atresia is typically classified into the following types:

Isolated (perinatal) biliary atresia

• The most common form, occurring in infants who are otherwise healthy. This form accounts for 70 to 85 percent of infants with biliary atresia.

Syndromic (fetal/embryonic) biliary atresia

• Occurs with other congenital abnormalities such as polysplenia, heart defects, or situs inversus. This form accounts for 10 to 15 percent of infants with biliary atresia.

Symptoms usually appear within the first 2 months of life:

• Prolonged jaundice (lasting >2 weeks after birth)
• Pale or clay-colored stools
• Dark urine
• Enlarged liver or spleen (hepatosplenomegaly)
• Poor weight gain or growth (failure to thrive)

These signs should prompt immediate medical evaluation.

The exact cause is unknown, but several theories exist:

• Abnormal bile duct development in the womb
• Autoimmune or inflammatory response after birth
• Viral infections (e.g., CMV, rotavirus)

• Slightly more common in females
• Higher incidence in certain geographic regions (e.g., East Asia)

Currently, there is no known way to prevent biliary atresia, as its cause remains unclear. However, Early detection is crucial to improve outcomes. Newborn screening programs (e.g., stool color cards) in some countries help identify the disease early. Prompt surgical intervention offers the best chance of survival and improved liver function.