Purpose: The aim of this study was to assess the long-term survival rate in
children who have undergone orthotopic liver transplantation (OLT) in the
last 13 years.
Methods: The records of 198 consecutive patients under 18 years of age who
underwent 249 OLTs between 1986 and 1998 were reviewed. Actuarial patient s
urvival rates were assessed at 1,3,5, and 10 years in the whole series, in
the last 5 years, and in patients surviving more than 1 year. Age, weight,
and indications were analyzed, as well as type and incidence of posttranspl
ant complications. The median follow-up period was 41 months (0 to 154 mont
hs).
Results: Biliary atresia was the most common indication (41.9%) followed by
alpha-1 antitrypsin deficiency (8.1%), Alagille syndrome (7.6%), and fulmi
nant hepatic failure (6.6%). One hundred forty-six patients (58.6%) were be
low 5 years, and 46 patients were (18.5%) younger than 1 year at operation.
Sixty-eight patients (27.3%) weighed less than 10 kg. One hundred seventy
whole organs and 70 reduced, 5 living-related donor, and 4 split-liver allo
grafts were used. Hepatic artery thrombosis (n = 18), primary nonfunction (
n = 15), and chronic rejection (n = 14)were the most common causes for allo
graft failure. Fourteen patients (7%) had posttransplant lymphoproliferativ
e disorders (PTLD) at a median time of 28 months (4 to 124 months) postoper
ation (3 died). The 1-, 3-, 5-, and 10-year actuarial patient survival rate
s are 80%, 76%, 74%, and 74%, respectively; over the last 5 years it is 88%
at 1 year and 82% at 3 and 5 yea rs. For patients surviving more than 1 ye
ar, 3-, 5-, and 10-year actuarial survival rates are 95%, 93%, and 93%, res
pectively.
Conclusions: (1) Overall results of OLT improve with increasing experience.
(2) Children who survive more than 1 year after OLT have an excellent prog
nosis, although long-term complications of immunosuppression can be expecte
d. Copyright (C) 2000 by W.B. Saunders Company.