Bacterial cholangitis (BC) is a common complication in patients with biliar
y atresia (BA) and is characterized by fever, acholic stools and positive b
lood cultures. The diagnosis is often empirical because the yield of blood
cultures is low. It is difficult to differentiate BC from other febrile epi
sodes. In order to characterize the clinical and laboratory features of BC
in patients with BA, identify risk factors, and correlate cholangitis with
outcome, 37 patients with BA from 1993 to 1998 who underwent a Kasai operat
ion in our hospital were studied. The follow-up period ranged from 6 to 59
months. A total of 107 febrile episodes were documented in these patients.
The diagnostic criteria for cholangitis were fever, increased jaundice, or
acholic stools. The clinical features, laboratory data, results of bacteria
l. cultures, and outcomes were analyzed retrospectively. A total of 107 feb
rile episodes, including 78 bouts of cholangitis and 29 non-cholangitis inf
ections, were found in 34 patients. Patients with BC had higher postoperati
ve bilirubin levels (P = 0.02) and less frequent use of prophylactic antibi
otics (P = 0.05) than those with non-cholangitis infections. Abnormal white
blood cell counts (>12,000 or (4,000 mm(3)) tended to be present in patien
ts with BC (P = 0.08). There were no statistical differences in the risk fa
ctors and laboratory data between culture-positive (n = 16) and -negative (
n = 62) cholangitis cases. The occurrence of cholangitis significantly redu
ced survival in both patients with good (P = 0.03) and inadequate bile flow
(P = 0.03). All 9 patients who had never had cholangitis survived during t
he follow-up period. Repeated attacks of BC further decreased survival prob
ability. The responsive organisms were mainly enteric bacteria, including E
scherichia coli, Enterobacter cloacae, Klebsiella pneumoniae, Pseudomonas a
eruginosa, Acinetobacter baumanni, and Salmonella typhi. The sensitivity te
sts justified empirical therapy with ceftriaxone. The effectiveness of prop
hylactic trimethoprim-sulfamethoxazole or neomycin warrants further studies
. BC was a highly prevalent postoperative complication in patients with BA,
especially those with inadequate bile drainage. It significantly affected
early mortality. Aggressive and complete treatment with empirical ceftriaxo
ne was appropriate.