Background: Biliary Atresia (BA) is worldwide the leading cause of
end-stage liver disease in children and the most common indication
for pediatric liver transplantation (LT). National studies from several
European, Asiatic and North American countries report a 48% median
success rate (range 30 1261%) of Kasai Portoenterostomy (KPE) and
a median survival with native liver (SNL) of 37.9% (range 31 1253)
at 10 years after KPE. Young age at diagnosis (<45 days) and center
experience (>5 cases/year) are the main prognostic factors associated
to KPE success. No published data are available concerning BA
epidemiology and outcome in Italy.
Objective: The aim of this study is to describe the outcome of
children with BA treated at a single University Center of Pediatric
Hepatology and LT in Italy.
Methods: Health records of all cases of BA diagnosed at the
Department of Pediatrics of the University of Padova between January
1990 and December 2011 were retrospectively reviewed.
Results: A total of 52 children were diagnosed with BA during the
study period (mean 2.5/year) of which 20 in the last 5 years (mean
4/year). 51 patients underwent KPE at a median age of 66 days
(range 19\u2013115); a significant difference (p<0.001, unpaired t test)
was observed concerning the mean age at KPE between children
diagnosed during the 1990s (mean 49\ub15 days) and after 2000
(mean 72\ub13 days). Median age at last follow up was 5.6 years
(0.7 1222 years). KPE success (i.e. total bilirubin <20 umol/L at
6 months after KPE) was 31%. Among patients with unsuccessful
KPE (n = 34): 64% underwent LT at a median age of 1.8 years (main
indications consisted in end-stage liver disease, hepato-pulmonary
syndrome/HPS, hepatocellular carcinoma); 8% died on the waiting list
for transplant at a median age of 10 months (all due to uncontrolled
variceal bleeding); 14.7% have compensated cirrhosis and are still on
follow up; one patient is on the waiting list for liver transplantation
and one was lost at follow up. Among children with successful KPE
(n = 15): 4 have no clinical or biochemical signs of liver disease;
4 have signs of portal hypertension; 4 show signs of compensated
chronic liver disease; 1 was transplanted for HPS at 9.5 years of age;
1 died for reasons unrelated to BA; one was lost at follow up. Data
of two patients were untraceable. Overall the rate of NLS at 10 years
after KPE was 38%.
Conclusions: Despite advanced age at KPE and relatively low
numbers of new cases per year, both the success rate of KPE
and the 10 years SNL after this intervention are comparable with
current international standards. As demonstrated in other countries,
centralization of surgical care and the launch of a screening program
to anticipate age at referral have the potential to further improve the
outcome of children after KPE. The time to begin an Italian National
Program for the management of BA has definitely come
