Biliary Atresia

Biliary atresia is a rare but serious liver disease that affects infants, causing bile ducts to become blocked or absent. This condition prevents bile from flowing from the liver to the intestine, leading to liver damage and, if left untreated, liver failure. Early diagnosis and intervention are crucial for improving outcomes

The exact cause of biliary atresia is not well understood, but it is believed to involve genetic and environmental factors, an abnormal inflammatory response, and congenital factors (birth defects).

Symptoms of biliary atresia typically appear within the first few weeks of life and may include:

Jaundice (yellowing of the skin and eyes) that persists beyond two weeks after birth.
Dark urine and pale, clay-colored stools.
Enlarged liver and spleen.
Poor weight gain and slow growth.

Timely diagnosis is critical for effective treatment. Diagnostic procedures include:

Blood tests to assess liver function and detect abnormal bilirubin levels.
Ultrasound to examine the liver and bile ducts.
Hepatobiliary scintigraphy – a nuclear medicine test to track bile flow.
Liver biopsy to confirm liver damage and inflammation.

The primary treatment for biliary atresia is surgery, followed by long-term management:

Kasai procedure (Hepatoportoenterostomy) – a surgical procedure that creates a new pathway for bile drainage. It is most effective when performed before the infant is 2-3 months old.
Liver transplantation. In cases where the Kasai procedure is unsuccessful or liver failure progresses, a liver transplant may be necessary.
Nutritional support. Infants with biliary atresia may require a specialized diet to support growth and development.
Medical management. Monitoring and treatment of complications such as infections, liver fibrosis, and vitamin deficiencies.

With timely intervention, many infants with biliary atresia can live healthy lives. However, long-term monitoring and potential liver transplantation are often required to manage liver disease progression.

At Karolinska University Hospital, our experienced team can provide comprehensive diagnosis, treatment, and rehabilitation.

Pediatric Surgery

Our aim is to provide high-quality management, in collaboration with multiple specialties, of the most complex cases from the prenatal period to the transition to adult care. Pediatric Surgery

Liver Transplant

Karolinska University Hospital runs one of the more extensive liver surgery units in the Nordics, and has a special assignment for liver transplants on behalf of the Swedish National Board of Health and Welfare. Both children and adults undergo transplants with excellent results at Karolinska. Liver Transplant

Center

Transplantation Center

We aim to significantly improve the quality of life for a vast number of patients with various diseases, as well as give new life to those for whom there is no other treatment.