Biliary atresia is a rare congenital disease of the bile ducts. It occurs in approximately 1 in 10,000 to 15,000 births. It usually appears within the first few weeks after birth. The disease arises due to congenital abnormalities of the bile ducts in infants. It is observed about twice as often in girls compared to boys.

The underlying cause involves damage to the liver bile ducts that begins while the baby is still in the womb and continues in the weeks following birth. Symptoms typically appear within the first 2 months.

Symptoms

• Yellow discoloration of the skin and the whites of the eyes (jaundice)
• Darkening of the urine color, and lightening or whitening of the stool color
• Abdominal swelling due to fluid accumulation
• Feeding difficulties and weakness

Early Diagnosis

Early diagnosis is critical for children with biliary atresia. Babies diagnosed and operated on within the first 4 weeks after birth have a higher chance of living a healthy life. As the diagnosis and surgery are delayed, especially beyond 2-3 months, liver structure deteriorates, reducing the chance of restoring bile flow after surgery. Therefore, babies showing jaundice or white-colored stools should be urgently referred to a center experienced in liver surgeries.

Once diagnosed, the Kasai procedure (corrective surgery for biliary atresia) should be performed, and follow-up care should continue at the same center. Babies diagnosed late or not treated appropriately experience worsening liver function, which poses a life-threatening risk. In such cases, the only solution is liver transplantation. However, the younger and smaller the baby, the lower the chances of successful transplantation. Moreover, in Turkey, liver transplantation for very small infants is available only in a few centers.

Treatment

The treatment of biliary atresia is surgical. Babies diagnosed early undergo the Kasai procedure (hepatoportoenterostomy). This surgery aims to connect the liver’s bile ducts to the small intestine. It is generally performed within the first 2-3 months after birth. If the baby is diagnosed late or liver damage progresses after the Kasai surgery, liver transplantation becomes the alternative treatment.

Complications such as bleeding, infection, bile leakage, and liver damage can occur during or after surgery. Following liver transplantation, rare complications include organ rejection, organ dysfunction, and vascular problems.

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