Long-term results of biliary atresia in the era of liver transplantation.
OBJECTIVE
The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival.


METHODS
Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed.


RESULTS
Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002).


CONCLUSIONS
Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.
Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, 135-710, Korea.
Sanghoon
S
Lee
Hyojun
H
Park
Suk-Bae
SB
Moon
Soo-Min
SM
Jung
Jong Man
JM
Kim
Choon Hyuck David
CH
Kwon
Sung Joo
SJ
Kim
Jae-Won
JW
Joh
Jeong-Meen
JM
Seo
Suk-Koo
SK
Lee
D001656:Biliary Atresia / Q000150:complications / Q000401:mortality* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D007223:Infant; D008105:Liver Cirrhosis, Biliary / Q000150:complications; D016031:Liver Transplantation / Q000379:methods* / Q000706:statistics & numerical data; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000379:methods* / Q000401:mortality* / Q000706:statistics & numerical data; D011183:Postoperative Complications; D056910:Republic of Korea; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome