Can We Minimize Wait-List Mortality in
Young Children With Biliary Atresia?
SEE ARTICLE ON PAGE 810
Biliary atresia (BA) remains a major indication for liver
transplantation (LT) in children younger than 2 years
Despite the successful application of Kasai
portoenterostomy in experienced pediatric centers
today, approximately 40%-70% of children still develop
end-stage liver disease and require LT within 2
With an excellent 10-year graft survival rate
of 71%-90% after transplantation, early recognition of
deterioration after failed Kasai and subsequent listing
appears crucial to avoid prolonged waiting time and
death before a graft becomes available.
This is of
particular importance in children without the option of
living donor LT. Thus, identiﬁcation of predictors for
wait-list mortality is crucial in this cohort.
In this issue of Liver Transplantation,vanderDoef
et al. report on outcomes of 711 young children listed
with BA in the Eurotransplant registry.
In this cohort,
the wait-list mortality increased from 4.1% to 7.9%
between 3 and 24 months after registration, whereas
86.9% of candidates received a liver graft within 2
The authors focused on predictors of wait-list
mortality in children listed before the age of 5 years. A
higher Model for End-Stage Liver Disease (MELD)
score and the candidates’ age at the time of listing were
independent risk factors. All children who died while
waiting were younger than 2 years at the time of listing
with a signiﬁcantly higher risk for those between 0 and
0.5 month of age who suffered from an advanced liver
From this cohort, 21% did not survive a 6-month waiting
Although the risk factors for wait-list mortality
described here are not entirely new, this study is the
ﬁrst involving children from 5 countries in Europe. In
contrast, a majority of previous reports included data
from the United States or Japan
a higher Pediatric End-Stage Liver Disease (PELD)
score as a predictor of wait-list mortality.
This is not
surprising because age is already computed in the
PELD score. Results from this European study, there-
fore, paralleled previous ﬁndings in other countries
regarding the impact of disease severity.
ally, the signiﬁcantly higher risk of deterioration prior
to engrafting in very young children has been described
Effective medical treatment to prevent
variceal bleeding or cholangitis combined with aggres-
sive nutritional support prior to LT is very important
to maintain such candidates in a reasonable condition
Accordingly, 1 important task is to further increase
access to the scarce resource of a donor liver. Although
recent series report an increasing number of up to 50%
living donor LTs and 30%-40% whole or split grafts
from deceased donors,
very young children are
exposed to a prolonged waiting time. Furthermore,
transplantation is related to several technical issues in
this population. This is particularly apparent when no
living donor becomes available in children with a
body weight of <5 kg and a graft-to-body weight ratio
of >4%. Such candidates are exposed to a high risk for
large-for-size syndrome, which is related to a high
morbidity with subsequent prolonged ventilation time
and hospital stay.
In this context, Kasahara et al.
recently described a new technique to further reduce
left lateral liver grafts for very small children in order to
achieve primary abdominal closure to decrease the
duration of ventilation and intensive care unit admis-
sion with subsequent prevention of severe infectious
Despite such technical efforts, many livers are not split
because of the lack of a mandatory split policy in many
regions. Although some European countries accept donor
Abbreviations: BA, biliary atresia; LT, liver transplantation;
MELD, Model for End-Stage Liver Disease; PELD, Pediatric
End-Stage Liver Disease.
Address reprint requests to Paolo Muiesan, F.R.C.S., F.E.B.S., The
Liver Unit, Queen Elizabeth Hospital Birmingham, Edgbaston,
Birmingham, United Kingdom B15 2TH. Telephone: 0044 12137
1; FAX: 0044 12141 41833; E-mail: email@example.com
Received April 26, 2018; accepted April 26, 2018.
2018 by the American Association for the Study of Liver
View this article online at wileyonlinelibrary.com.
Potential conflict of interest: Nothing to report.