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Biliary atresia remains one of the most challenging conditions in pediatric surgery. The incidence ranges from 1 in 5,000 in Asia to about 1 in 16,000 live births in Europe with a slight female preponderance. The cause is still unknown with a number of possible causes giving rise to a range of different presentations. It is usually an isolated condition but can be associated with other anomalies.
Currently the management is almost entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai portoenterostomy. Liver transplantation is an option if this fails or for those infants who present late with obvious end-stage cirrhosis. Beyond effective surgical technique, the role of adjuvant medical therapy is unclear and evidence of benefit is lacking. Despite this the use of postoperative steroids, prophylactic antibiotics, and choleretic agents such as ursodeoxycholic acid is common.
Experience from high-volume centers suggests clearance of jaundice can be achieved in 50–60% of infants with 10-year native liver and real survival rates of 40% and 90%, respectively.
KeywordsBiliary atresia Kasai portoenterostomy Surgical jaundice Cirrhosis BASM Cystic biliary atresia
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