Applied Radiology

CASE SUMMARY

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 normal limits.

DIAGNOSIS

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 retrograde cholangiopancreatography

IMAGING FINDINGS

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 cholangiohepatitis).

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.

DISCUSSION

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 States. ¹ 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 Clonorchis sinensis , causing pigmented stone formation by inducing the precipitation of bilirubin, acting as nidi for stone formation, or causing biliary strictures that lead to further biliary stasis. ² 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. ³

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. ⁴ The diagnosis rests upon recognizing the appropriate constellation of clinical signs and biliary and hepatic abnormalities using the various imaging modalities available.

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. ⁴ 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 hepatic resection. ⁴ 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 hepatitis.

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. ⁵ 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 cholangitis.