A 44-year-old Vietnamese woman with a history of recurrent
right upper quadrant pain for the last 5 years presented with
new onset of nausea, vomiting, and right upper quadrant pain.
The patient's physical examination was significant for marked
tenderness to palpation in the right upper quadrant without
hepatomegaly. Laboratory data that included complete blood
count, liver function tests, and electrolytes were all within
Recurrent pyogenic hepatitis (Oriental
cholangiohepatitis), demonstrated by dilatation of common
bile duct, common hepatic duct, and intrahepatic bile ducts
with multiple pigmented stones removed during endoscopic
Initial plain films of the abdomen showed no abnormality.
The liver and spleen were normal in size.
An abdominal ultrasound revealed markedly dilated common
bile duct, common hepatic duct, and intrahepatic bile ducts.
Also, biliary duct obstruction with multiple intrahepatic
bile duct stones in both the left and right lobe of the liver
was identified (figure 1). These findings suggested the
possibility of recurrent pyogenic cholangitis (Oriental
An endoscopic retrograde cholangiopancreatography (ERCP)
(figure 2) demonstrated markedly dilated common bile and
common hepatic ducts with moderate dilatation of the
intrahepatic biliary ducts. Multiple filling defects were
identified throughout the right and left intrahepatic biliary
ducts consistent with intrahepatic ductal calculi.
The patient eventually underwent a sphincterotomy and
removal of multiple pigmented stones, which were soft and of
a muddy clay-like consistency (figure 3). The patient became
asymptomatic after the removal of stones and subsequent
treatment with ciprofloxacin 500 mg (Cipro, Bayer
Corporation, West Haven, CT) po bid for 7 days.
Recurrent pyogenic hepatitis (Oriental cholangiohepatitis)
is characterized by formation of intrahepatic pigmented
stones with recurrent exacerbation and remission of abdominal
pain, frequently associated with jaundice, chills, and fever.
It is a major cause of an acute abdomen in the Far East and
is seen occasionally in Asian immigrants in the United
The cause of recurrent pyogenic hepatitis is unknown, but it
is most likely multifactorial. It is suspected that the
disease is may be secondary to infections with coliform
bacteria or parasites such as
, causing pigmented stone formation by inducing the
precipitation of bilirubin, acting as nidi for stone
formation, or causing biliary strictures that lead to further
The hallmark of the disease is the development of soft
pigmented bilirubinate stones within markedly dilated intra-
and extrahepatic ducts, unlike cholesterol stones often seen
in "Western" biliary stone disease. The pigmented
bilirubinate stones seen in recurrent pyogenic hepatitis
typically have a clay-like consistency and often fill the
ducts with casts.
Clinical presentation is characterized by recurrent
attacks of right upper quadrant pain, fever, chills, and
jaundice. Laboratory findings are polymorphonuclear
leukocytes, elevated levels of alkaline phosphatase, and
excretion of urobilinogen in urine.
The diagnosis rests upon recognizing the appropriate
constellation of clinical signs and biliary and hepatic
abnormalities using the various imaging modalities
Radiologic assessment of the biliary tree is imperative in
patients with high suspicions of possible recurrent pyogenic
hepatitis. Usually, plain radiography is not helpful because
the stones are rarely radiopaque. Radiologic evaluation of
patients with biliary disease can begin with sonography
because it is noninvasive and has high sensitivity for the
detection of cholelithiasis and hepatic ductal dilatation.
Computed tomography can be helpful when sonographic findings
are not definitive or are equivocal. Furthermore, CT is
recommended when imaging guidance is needed for complex
hepatic drainage procedures or surgical procedures, such as a
Direct cholangiography--such as endoscopic retrograde
cholangiography, percutaneous transhepatic cholangiography,
operative cholangiography, and T-tube
cholangiography--demonstrates the full spectrum of ductal
changes and stones in patients with recurrent pyogenic
Treatment of this disease focuses on management of acute
cholangitis, followed by either drainage and removal of
stones using endoscopic, radiologic, or surgical methods, or
hepatic resection for focal disease.
It is frequently the radiologist who initially evaluates the
patient with biliary symptoms and makes a diagnosis of
recurrent pyogenic hepatitis. Therefore, quick and correct
diagnosis is vital because unfamiliarity with the disease
usually leads to a delayed or missed diagnosis, which may
prove fatal in fulminant cases associated with sepsis and