Biliary atresia in characterized by the obstruction of bile ducts within the first weeks and months of life. The etiology not yet fully understood.
Bile is produced by the liver and normally released into the intestine via the bile ducts, where it plays an important role in digestion and absorption of nutrition components. If the bile cannot exit the liver, this is known as cholestasis. Cholestasis over a longer period of time can damage the liver and can even lead to liver failure.
Without treatment, the liver of babies with Biliary atresia is so severely and irreversibly damaged within the first few months that the only cure is a liver transplant ¹.
In the early stages of the disease, the symptoms are often not obvious. Children usually grow and thrive normally during the first few weeks of life.
However, the pale stools mentioned above occur in the early stages of the disease and, if recognized, can lead to a timely diagnosis.
Children’s eyes and skin may also appear yellow, a symptom called jaundice. It can sometimes be difficult to distinguish from the often harmless ‘neonatal jaundice’. In any case, jaundice that persists beyond 14 days of life should be investigated by a paediatrician ².
In later stages, when the liver has already suffered significant damage, the babies are seriously ill and require extensive medical care.
Biliary atresia is a rare disease and affects around 1 in 15,000 newborns.
For Germany, this means a case count of around 60 per year.
The treatment of choice is the Kasai operation, which is currently the only way to preserve the baby’s native liver.
During the operation, the connection between the patient’s own liver and the intestine is restored so that the bile can flow out again. The earlier the Kasai operation is performed, the higher the chances of success. Ideally, it is performed before the 6th week of life.