Objective To analyze a single center's 13-year experience with 589 ped
iatric orthotopic liver transplants for end-stage liver disease. Summa
ry Background Data Despite advances in medical therapy, liver replacem
ent continues to be the only definitive mode of therapy for children w
ith end-stage liver disease. innovative surgical techniques and improv
ed immunosuppression have broadened the application of liver replaceme
nt for affected children. However, liver transplantation in the child
remains challenging because of the scarcity of donor organs, complex s
urgical technical demands, and the necessity to prevent long-term comp
lications. Methods The medical records of 440 consecutive patients you
nger than 18 years of age undergoing orthotopic liver transplantation
for end-stage liver disease from March 20, 1984, to November 15, 1997,
were reviewed, Results were analyzed using Cox multivariate regressio
n analysis to determine the statistical strength of independent associ
ations between pretransplant covariates and patient and graft survival
. Actuarial patient and graft survival rates were determined at 1, 3,
5, and 10 years. The type and incidence of posttransplant complication
s were determined, as was the quality of long-term allograft function.
The median follow-up period was 4.1 years. Results Biliary atresia wa
s the most common cause (50.4%) of endstage liver disease in this pati
ent population. The median recipient age was 2.4 years; 239 patients (
54%) were younger than 3 years of age and III patients (25%) were youn
ger than 1 year of age. There were 471 whole organs, 29 were ex vivo r
educed size, 33 were living-related donor, and 36 were in situ split-l
iver allografts, Three hundred forty-three (78%) patients underwent a
single allograft, whereas 97 patients required retransplantation; hepa
tic artery thrombosis was the most common indication for retransplanta
tion (55 patients). The 1-, 3-, 5-. and 10-year actuarial patient. sur
vival rates were 82%, 80%, 78%, and 76%, respectively; allograft survi
val rates were 68%, 63%, 60%, and 54%. Long-term liver function remain
s excellent: current median follow-up values for total bilirubin and a
spartate aminotransferase were 0.5 mg/dl and 54 IU/L, respectively. Co
x multivariate regression analysis demonstrated that pretransplant pat
ient age, the era of transplantation, and the number of allografts per
formed significantly and independently predicted patient survival rate
s, whereas allograft type and pretransplant diagnosis did not. Conclus
ions Liver transplantation in the pediatric patient is a durable proce
dure that provides excellent loner-term survival. Although there have
been overall improvements in patient outcome with increased experience
, the effect is most pronounced for patients younger than 1 year of ag
e, Retransplantation, although effective ina meaningful number of pati
ents, continues to carry a progressive decrement in survival with the
number of allografts performed. Use of living-related and in situ spli
t-liver allografts has dramatically reduced waiting times for small ch
ildren ard has improved patient survival.