Ann Surg 1996 Sep;224(3):276-84; discussion 284-7

Orthotopic liver transplantation for congenital biliary atresia. An 11-year
single-center experience

Goss JA, Shackleton CR, Swenson K, Satou NL, Nuesse BJ, Imagawa DK, Kinkhabwala
MM, Seu P, Markowitz JS, Rudich SM, McDiarmid SV, Busuttil RW

Abstract
OBJECTIVE: The authors analyze a single center's 11-year experience with 190
orthotopic liver transplants for congenital biliary atresia. SUMMARY BACKGROUND
DATA: Hepatic portoenterostomy generally is the initial treatment for children
with congenital biliary atresia. Despite multiple modifications of the hepatic
portoenterostomy, two thirds of treated patients still develop recurrent
cholestasis, portal hypertension, cholangitis, and cirrhosis. Therefore, the
only hope of long-term survival in the majority of children with congenital
biliary atresia is definitive correction with orthotopic liver transplantation.
METHODS: The medical records of 190 consecutive patients undergoing orthotopic
liver transplantation for congenital biliary atresia from July 1, 1984 to
February 29, 1996 were reviewed. Results were analyzed via Cox multivariate
regression analysis to determine the statistical strength of independent
associations between pretransplant covariates and patient and graft survival.
Actuarial patient and graft survival was determined at 1, 2, and 5 years. The
type and incidence of post-transplant complications were determined, as was the
quality of long-term graft function. The median follow-up period was 3.21 years.
RESULTS: The liver grafts were comprised on 155 whole-organ, 24 reduced-size,
and 11 living donor organs. Median pretransplant values for recipient age,
weight, and total bilirubin were 1.4 years, 12.3 kg, and 13.8 mg/dL,
respectively. One hundred sixty-four patients (86%) had undergone prior hepatic
portoenterostomy. Eighty-seven patients (46%) were United Network for Organ
Sharing (UNOS) status 1 or 2 at the time of liver transplantation. The majority
(15/24, 62%) of reduced-size graft recipients were UNOS status I at the time of
transplantation. One hundred fifty-nine patients (84%) received a single graft,
whereas 31 patients required 37 retransplants. The 1, 2, and 5 year actuarial
patient survival rates were 83%, 80% and 78% respectively, whereas graft
survival rates were 81%, 77%, and 76%, respectively. Cox multivariate regression
analysis demonstrated that pretransplant total bilirubin, UNOS status, and graft
type significantly predicted patient survival, whereas recipient age, weight,
and previous hepatic portoenterostomy did not. Current median follow-up values
for total bilirubin and aspartate aminotransferase levels in the 154 surviving
patients were 0.5 mg/dL and 34 international units/L, respectively. CONCLUSION:
Long-term patient survival after orthotopic liver transplantation for congenital
biliary atresia is excellent and is independent of recipient age, weight, or
previous hepatic portoenterostomy. Optimal results are obtained in this patient
population when liver transplantation is performed before marked
hyperbilirubinemia, and when possible, using a living-donor graft