Cholangitis is an infection of the biliary tracts and is seen
most commonly in patients with abnormal biliary tracts such as post-Kasai (portoenterostomy)
for biliary atresia. Nearly 60% of patients have one or
more episodes of cholangitis after portoenterostomy, with decreasing
frequency over time.2 Other conditions that cause
functional or mechanical obstruction of the bile ducts such as gallstones,
congenital hepatic fibrosis, choledochal cysts, and primary sclerosing
cholangitis (PSC) also increase the risk of cholangitis. When these
patients present with fever and no obvious other source of infection,
they should be evaluated for possible cholangitis. Cholangitis classically
presents with jaundice, fever, and right upper quadrant pain (the
Charcot triad); however, in children not all symptoms may be present.
In patient status post-Kasai, fever is seen in nearly 100% of
patients with cholangitis, usually with increased bilirubin or acholic
stools and laboratory values showing either leukocytosis or leukopenia.2 Evaluation of
cholangitis should include aspartate aminotransferase, alanine aminotransferase,
alkaline phosphatase, gamma-glutamyl transpeptidase, and blood and
urine cultures, although blood cultures will be positive in less than
50% of cases.2-4 Imaging should also be undertaken
by either ultrasound or CT scan to rule out the presence of abscess,
calculi, ductal dilatation, or other anatomical lesions.3 If
concern persists over the presence of a stone, a magnetic resonance
cholangiogram (MRI) may be helpful in delineating the location, while
endoscopic retrograde cholangiopancreatography (ERCP) provides therapeutic
options in cases of obstruction. Liver biopsy can be used to support
the diagnosis; however, the diagnostic value of the biopsy should
be balanced with the risk of the procedure.