Biliary Atresia—A Fifteen-Year Review of Clinical and Pathologic
Factors Associated With Liver Transplantation
By Joanne Baerg, Craig Zuppan, and Marquelle Klooster
Loma Linda, California
Purpose: The aim of this study was to identify clinical and
pathologic factors associated with liver transplantation in
infants with biliary atresia initially treated with Kasai hepatic
Methods: Institutional Review Board approval was obtained.
Records of patients with biliary atresia diagnosed between
January 1986 and December 2000 were reviewed. Patients
were divided into those who never required transplantation,
those who underwent transplant in the ﬁrst year after KHPE,
and those who required transplantation later in childhood.
Analysis of variance (ANOVA) compared multiple factors
among the 3 groups. Proportional analysis compared those
who required transplantation against those who did not.
Statistical signiﬁcance was considered achieved if P was less
Results: Forty-ﬁve patients were identiﬁed. Survival after
KHPE was 96% (43 of 45). Sixteen (37%) never required
transplantation, 13 (30%) underwent transplant within 1 year
after KHPE, and 14 (33%) underwent transplant more than 1
year after KHPE. ANOVA comparison showed that the dura-
tion of jaundice before KHPE as a predictor for liver trans-
plantation approached signiﬁcance (P ϭ .082). Proportional
analysis found that a longer initial duration of jaundice be-
fore KHPE (P ϭ .016) and failure to establish biliary ﬂow (P ϭ
.033) were also signiﬁcant predictive factors. An initial re-
quirement for phototherapy (P ϭ .057) and ductules less than
m in diameter (P ϭ .060) showed a trend toward
predictor of liver transplantation.
Conclusions: A longer duration of jaundice before KHPE,
failure to establish bile ﬂow, requirement for phototherapy in
the neonatal period, and ductules smaller than 200
associated with liver transplant after KHPE.
J Pediatr Surg 39:800-803. © 2004 Elsevier Inc. All rights
INDEX WORDS: Biliary atresia, phototherapy, Kasai opera-
ILIARY ATRESIA is a disorder of infants in which
perinatal obliteration of the extrahepatic biliary
system occurs. This idiopathic process is the most com-
mon indication for pediatric liver transplant.
the era of surgical management, most children died of
hepatic failure, variceal bleeding, and sepsis. Three-year
survival without treatment has been reported as less than
10%, with a median survival of 8 months.
In 1959, Kasai and Suzuki
reported that relief of
biliary obstruction in these infants could be achieved by
This procedure is now the
accepted initial treatment for infants with biliary atresia.
The response, however, is variable. The aim of this study
was to identify clinical and pathologic factors associated
with transplantation in patients who were treated initially
with a Kasai hepatic portoenterostomy.
MATERIALS AND METHODS
After Institutional Review Board approval was obtained (LLU IRB
# 51025), records of all patients with a pathologic diagnosis of biliary
atresia who underwent a Kasai hepatic portoenterostomy at a single
children’s hospital between January 1986 and December 2000 were
reviewed. Data and follow-up are complete for all patients.
Clinical factors recorded were gender, maternal age, birth weight,
need for phototherapy, gestational age, associated anomalies, age at
presentation, and jaundice duration. All operative reports and operative
complications were reviewed. Stool color change after surgery was
recorded. Portal plate ductules were measured. The incidence of
cholangitis, esophageal varices, portal hypertension, and age at trans-
plantation were recorded.
Patients were divided into 3 groups for analysis: patients who never
underwent transplantation, those who required a transplant in the ﬁrst
year after KHPE, and those who underwent transplantation later in
childhood. Analysis of variance (ANOVA) compared multiple factors
among the 3 groups. Proportional analysis (
and Student’s t test)
compared patients who required a transplant against those who did not.
Statistical signiﬁcance was considered achieved if P was less than .05.
Forty-ﬁve patients were identiﬁed. Twenty-four (53%)
were girls. The mean maternal age was 25 years (range,
17 to 41 years). The mean birth weight was 3.167 kg
(range, 0.50 to 5.47 kg) and the mean gestational age was
From the Division of Pediatric Surgery, Loma Linda University
Children’s Hospital, Loma Linda, CA.
Presented at the 55th Annual Meeting of the Section on Surgery of
the American Academy of Pediatrics, New Orleans, Louisiana, October
31-November 2, 2003.
Address reprint requests to Joanne Baerg MD, Division of Pediatric
Surgery, Loma Linda University Children’s Hospital, Room 21111,
11175 Campus St, Loma Linda, CA 92354.
© 2004 Elsevier Inc. All rights reserved.
800 Journal of Pediatric Surgery, Vol 39, No 6 (June), 2004: pp 800-803