J Korean Assoc Pediatr Surg.  2007 Jun;13(1):1-12.

Long-term Outcome after Surgery for Biliary Atresia (Study of 10 patients surviving more than 10 years)

• Rhim SY
• Jung PM

Abstract

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

Keyword

Biliary atresia; Kasai operation; Prognosis

MeSH Terms

Alanine
Aspartate Aminotransferases
Bile Ducts, Extrahepatic
Biliary Atresia*
Bilirubin
Cholangitis
Fibrosis
Follow-Up Studies
Hepatomegaly
Humans
Hypertension, Portal
Korea
Liver
Prognosis
Retrospective Studies
Splenomegaly
Surgical Procedures, Operative
Survival Rate
Survivors
Alanine
Aspartate Aminotransferases
Bilirubin