Liver Transplantation for Biliary Atresia
H. Karakayali, S. Sevmis, U
¨
. Özçelik, F. Ozcay, G. Moray, A. Torgay, G. Arslan, and M. Haberal
ABSTRACT
Biliary atresia is the most common indication for liver transplantation (OLT) in children.
We present our experience with OLT as a treatment for end-stage liver disease in children
with biliary atresia. We performed a retrospective review of 20 biliary atresia patients (11
male, 9 female patients; mean age, 21.4 months; range, 6 to 84 months) who had
undergone OLT. Mean preoperative weight and height were 10.1 5.8 kg and 72.5 cm,
respectively. Thirteen recipients were younger than 1 year of age, and 15 weighed less than
10 kg at the time of OLT. Fourteen recipients had undergone a Kasai operation prior to
the OLT. The mean serum total bilirubin level was 22.56 mg/dL before OLT. Eighteen left
lateral segment grafts and two whole grafts were transplanted. The mean recipient
operative time was 9.25 hours. The mean recipient intraoperative blood loss was 1.81 U.
Two hepatic arterial thromboses and one biliary leak occurred soon after surgery. Portal
vein stenoses developed in two recipients at 10 and 12 months after OLT; both were
treated with balloon dilatation. Two biliary stenoses, which occurred at 10 months and 3.5
years after surgery, were treated with balloon dilatation. Two recipients died at 2 and 12
days after OLT because of respiratory distress syndrome and sepsis, respectively. The
remaining 18 (90%) recipients are alive with good graft function. The overall rejection rate
was 31.25%. OLT is an effective treatment for children with biliary atresia and a failed
Kasai procedure. Living related liver grafts represented an excellent organ supply for these
patients.
B
ILIARY ATRESIA is a rare disease characterized by
biliary obstruction of unknown origin, which presents
during the neonatal period. It is the most frequent cause
for surgery in patients presenting with cholestatic jaun-
dice in this age group. The reported worldwide incidence
of biliary atresia varies from 5/100,000 to 32/100,000 live
births; it is highest in Asia and in the Pacific region. Girls
are affected slightly more often than boys. If untreated,
biliary atresia leads to cirrhosis and death within the first
years of life. Once biliary atresia is detected, surgical
intervention (a Kasai portoenterostomy) should be per-
formed as soon as possible. Operations performed earlier
are more likely to be successful. Liver transplantation
(OLT) may be needed later if the Kasai operation fails to
restore biliary flow or if cirrhotic complications occur.
1
The first attempted use of OLT to treat biliary atresia
was made in 1963.
2
Since then, biliary atresia has become
the most common indication for OLT among pediatric
recipients. In this study, we have described current
patient and graft survival rates and causes of death for
pediatric patients with biliary atresia undergoing OLT at
our center. In addition, we examined causes of morbidity
and mortality following OLT for biliary atresia.
MATERIALS AND METHODS
We retrospectively reviewed our experience in pediatric liver
transplant for biliary atresia. Between December 2002 and July
2008, 180 OLTs were performed in 177 recipients including 83
children, 20 (24%) of whom had biliary atresia. We transplanted 18
left lateral segment grafts and two whole grafts. Most donors were
a parent of the recipient (80%) with the mean age of the living
donor being 29.66 8.4 years (range, 23 to 35 years). The two
deceased donors were aged 1 and 7 years. For biliary drainage, a
feeding tube was used in four recipients, and a transhepatic
catheter in eight. In the remaining nine recipients, no tube or stent
was used. Four of the 20 liver grafts had two bile ducts, and 16 had
From the Departments of General Surgery (H.K., S.S., U
¨
.Ö.,
G.M., M.H.), Gastroentology (F.O.), and Anesthesiology (A.T.,
G.A.), Bas ¸ kent University Faculty of Medicine, Ankara, Turkey.
Address reprint requests to Mehmet Haberal, MD, FACS, FICS
(Hon), Bas ¸ kent University Faculty of Medicine, 1. Cad. No: 77
Kat: 4 Bahcelievler, 06490, Ankara, Turkey. E-mail: rektorluk@
baskent-ank.edu.tr
© 2008 by Elsevier Inc. All rights reserved. 0041-1345/08/$–see front matter
360 Park Avenue South, New York, NY 10010-1710 doi:10.1016/j.transproceed.2007.11.015
Transplantation Proceedings, 40, 231–233 (2008) 231