Improving treatment outcomes in patients with biliary atresia

Abstract

Introduction: Biliary atresia (BA) remains one of the most challenging conditions in pediatric hepatology and surgery. The main therapeutic approach is entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai portoenterostomy (KPE). Liver transplantation is usually offered if this fails and it remains the biggest single indication during childhood.

Areas covered: The role of adjuvant medical therapy is still unclear and conclusive evidence of benefit is lacking. The review covers the current evidential basis for corticosteroids, prophylactic antibiotics, and choleretic agents such as ursodeoxycholic acid.

Expert opinion: There are obvious areas for improving outcome in BA such as diminishing the time to KPE and concentration of resources to achieve a throughput of > 5 cases/year. High-dose steroids can improve the proportion of infants who clear their jaundice to normal levels by about 10 – 15%. It is not clear whether such improvements can be translated into a reduction in the number of transplants required however.

Keywords::

• biliary atresia
• cholangitis
• kasai
• liver transplant
• portoenterostomy
• steroids
• ursodeoxycholic acid

Notes

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