Ja. Goss et al., ORTHOTOPIC LIVER-TRANSPLANTATION FOR CONGENITAL BILIARY ATRESIA - AN 11-YEAR, SINGLE-CENTER EXPERIENCE, Annals of surgery, 224(3), 1996, pp. 276-284
Objective The authors analyze a single center's 11-year experience wit
h 190 orthotopic liver transplants for congenital biliary atresia. Sum
mary Background Data Hepatic portoenterostomy generally is the initial
treatment for children with congenital biliary atresia. Despite multi
ple modifications of the hepatic portoenterostomy, two thirds of treat
ed patients still develop recurrent cholestasis portal hypertension, c
holangitis, and cirrhosis. Therefore, the only hope of long-term survi
val in the majority of children with congenital biliary atresia is def
initive correction with orthotopic liver transplantation. Methods The
medical records of 190 consecutive patients undergoing orthotopic live
r transplantation for congenital biliary atresia from July 1, 1984 to
February 29, 1996 were reviewed. Results were analyzed via Cox multiva
riate regression analysis to determine the statistical strength of ind
ependent associations between pretransplant covariates and patient aci
d graft survival. Actuarial patient and graft survival was determined
at 1, 2, and 5 years. The type and incidence oi post-transplant compli
cations were determined, as was the quality of long-term graft functio
n. The median follow-up period was 3.21 years. Results The liver graft
s were comprised of 155 whole-organ, 24 reduced-size, and 11 living-do
nor 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 (85%) were United Network for Organ
Sharing (UNOS) status for 1 or 2 at the time of liver transplantation.
One hundred fifty-nine patients (84%) received a single graft, wherea
s 31 patients required 37 retransplants. The 1, 2 and 5 year actuarial
patient survival rates were 83%, 80%, and 78% respectively, whereas g
raft survival rates were 81%, 77%, and 76%, respectively. Cox multivar
iate regression analysis demonstrated that pretransplant total bilirub
in, UNOS status, and graft type significantly predicted patient surviv
al, whereas recipient age, weight, and previous hepatic portoenterosto
my did not. Current median follow-up values of bilirubin and aspartate
aminotransferase levels in the 154 surviving patients were 0.5 mg/dL
and 34 international units/L, respectively. Conclusion Long-term patie
nt survival after orthotopic liver transplantation for congenital bili
ary 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 gr
aft.