NEW YORK (Reuters Health) – The earlier the Kasai operation is performed to bypass extrahepatic bile ducts in cases of biliary atresia, the better is the long-term outcome, according to a report in the May issue of Pediatrics. The finding makes a case for routine neonatal screening for biliary atresia.

“The earlier biliary atresia is diagnosed, the earlier the Kasai operation can be performed, the better the chances for the child to live for a long time with its own liver, and thus the better the chances to prevent an early liver transplantation in childhood, or to prevent a liver transplantation for good,” Dr. Barbara E. Wildhaber told Reuters Health.

Dr. Wildhaber, from the University of Geneva Children’s Hospital, Switzerland and colleagues examined the impact of age at Kasai operation on survival with native liver in later childhood and adolescence.

The team examined data on all patients with biliary atresia in France who were born between 1986 and 2002. Of the 743 patients, 695 underwent a Kasai operation.

Survival with native liver decreased significantly when the age at Kasai operation increased, the authors report.

The best results were obtained when the operation was performed before the patient was 30 days old, yielding a 42.5% rate of survival with native liver at 10 years. Children operated after the age of 90 days still had a 13% chance of surviving with their native liver until adolescence, the report indicates.

If every child with biliary atresia underwent the Kasai operation before 46 days of life, 4.5 liver grafts per year would be saved, Dr. Wildhaber and colleagues estimate. This represents 5.7% of all pediatric liver transplants performed annually in France.

“In Switzerland a neonatal screening with a Stool Color Card will be launched this spring/summer,” Dr. Wildhaber said. “This will be a pilot study in order to know if with such screening the age at diagnosis and thus at Kasai operation will be lower.”

She added: “Physicians should always ask parents of a neonate about the color of the baby's stools and not just believe when they say ‘it is normal.' Look at it, and think of biliary atresia in the case of a well thriving yellow baby with pale stools.”

Reference:
Pediatrics 2009;123:1280-1286.