Biliary Atresia: Causes, Symptoms, Diagnosis, and Treatment

A rare disease that affects only babies is called Biliary Atresia. This disease originates in the bile ducts, and the ratio of infants affected by it is 1:18,000. This disease is generally seen in premature babies, females, and those of African, American, or Asian ethnicity.

Here, the bile ducts are inflamed and blocked at birth. This causes bile to get trapped inside the liver, damaging liver cells over time and leading to scarring or cirrhosis. This is the No. 1 cause for children to have liver transplants.

Causes

The reason why biliary atresia develops is still unclear. However, in some babies, it is congenital. About one out of every 10 babies suffering from Biliary Atresia also suffers from other congenital problems. In such cases, viral infection could be the cause of Biliary Atresia.

According to scientists, this disease is not hereditary, contagious, or preventable. It is also not due to anything a pregnant woman did or didn’t do.

Symptoms

Between the first two and six months after birth, babies show symptoms of Biliary Atresia. These are a yellowing of the skin and corneas, hardening of the liver, and a swollen abdomen. The baby’s stools are light grey, his urine is unusually dark, and he may have a body itch.

Diagnosis

Doctors diagnose biliary atresia through a physical exam and several tests. If a baby has pale stools or jaundice lasting more than two weeks, they should be checked right away.

The doctor may:

• Examine the baby for jaundice and a swollen belly.
• Do blood and urine tests to check liver function and find the cause of jaundice.
• Use imaging tests like an ultrasound or HIDA scan to see how bile flows.
• Perform a liver biopsy to look for signs of biliary atresia.
• Use a blood test (MMP-7) to help confirm the diagnosis.

In some cases, a special X-ray called an intraoperative cholangiogram is done during surgery. If biliary atresia is confirmed, doctors can immediately perform the Kasai procedure to create a new path for bile flow.

Timely detection and proper treatment are essential to safeguard a baby’s liver health.

Treatment

So far, there is no cure for this disease, but a surgical procedure called the Kasai Procedure can help. Here, the doctor reconstructs the infant’s bile duct and restores bile flow by joining the baby’s liver with a part of his intestine.

If unsuccessful, the liver damage continues and eventually, cirrhosis develops. The doctor will then resort to a liver transplant, a treatment method that has proved to be very successful for children with this disease. Children who undergo this procedure survive it and grow into healthy adults.

Usually, even if the surgery is successful, infants develop progressively damaged livers. They will need focused medical care for their entire lives, leading to a liver transplant.