A 43-Year-Old Woman With a 7-Month History of Intermittent Abdominal Pain and Weight Loss

Ann S. Fulcher, MD; Aneesa S. Majid, MD; Timothy J. Broderick, MD; Medical College of Virginia of Virginia Commonwealth University, Richmond.

Disclosures

November 09, 2001

Discussion

RPC, often referred to as Oriental cholangiohepatitis, is a progressive, destructive cholangiopathy of unknown cause. Although parasites are generally cultured from the bile and blood of patients with RPC during episodes of acute cholangitis, the precise cause of RPC remains undetermined.

RPC predominately occurs in natives of Southeast Asia,[1] although there have been reports of its occurrence in non-Asians.[9,10] RPC is increasing in frequency in the United States as the number of Asian immigrants also increases.[11] In a series of 276 patients with RPC, Ong[1] noted that RPC occurred with equal frequency in men and women and that the highest incidence occurred in the second through fourth decades of life. The majority of patients in this series presented with recurrent periods of fever, chills, jaundice, or pain. Although the serum alkaline phosphatase and bilirubin levels are often elevated in this setting, some patients -- such as in the case presented here -- demonstrate no biochemical abnormality.[1,12]

An autopsy series of 46 patients with RPC revealed pigmented stones or sludge in the intrahepatic bile ducts in all cases, extrahepatic ductal stones in 60%, gallbladder stones in 30%, and liver abscesses in 80%.[13] Analysis of the stones demonstrated that they were composed primarily of bile pigments, with lesser quantities of cholesterol and calcium. In this particular series, the histologic characteristics of early changes in RPC included acute inflammation of the portal tracts, frequently associated with pylethrombophlebitis; chronic changes were characterized by periductal fibrosis. The periductal fibrosis resulted in the occlusion of portal vein branches in some patients, which in turn led to hepatic lobar or segmental atrophy. Evidence of Clonorchis sinensis and Ascaris lumbricoides was found in 45% and 6.5% of cases, respectively.

Sonographic,[3] CT,[2] MR/MRCP,[14] and direct cholangiographic[15] findings of RPC have been described. Although many of the early imaging reports focused on findings at direct cholangiography, more recent ones focus on the utility of noninvasive means of establishing the diagnosis of RPC. Sonography is useful in depicting the biliary ductal dilatation and the ductal stones that occur in RPC. Lim and colleagues[3] noted that the stones were echogenic in all instances and associated with shadowing in 77% of cases. They also noted that the extrahepatic bile ducts were diffusely dilated both proximal and distal to stones. This latter feature is likely related to the loss of ductal elasticity that occurs with RPC.

Computed tomography (CT) is also useful in diagnosing and determining the extent of RPC. CT findings include dilatation of the intra- and extrahepatic bile ducts; intraductal stones that exceed the attenuation of unenhanced liver; pneumobilia (even in patients without a history of biliary surgery or endoscopic intervention); atrophy of the entire left hepatic lobe or lateral segment of the left hepatic lobe; and enhancement of the duct wall.[2] The latter finding was associated with episodes of acute cholangitis in most instances.

Recently MR and MRCP have been used in the evaluation of RPC,[14] and the combination of conventional abdominal MR and MRCP is particularly advantageous. Specifically, MR provides information about the liver whereas MRCP generates images of the biliary tract that are comparable to those obtained by ERCP or percutaneous transhepatic cholangiography (PTC) without the use of instrumentation or contrast material. In fact, unlike ERCP, MRCP allows for depiction of ducts located proximal to strictures. MRCP readily depicts intraductal stones, biliary ductal dilatation, and strictures. The major advantages of MRCP in the setting of RPC are that this imaging study provides a noninvasive means of establishing the diagnosis and renders high-quality cholangiographic images of the biliary tract useful in planning percutaneous, endoscopic, and surgical interventions.

Over the years, a number of therapeutic options have been employed to treat RPC. Antibiotics are effective in the treatment of the acute stages of cholangitis. However, because RPC is a progressive, destructive cholangiopathy,[15] more definitive treatment is required. Many of the early reported cases of RPC were treated surgically,[1] but with the development of techniques such as PTC and ERCP, stone removal, stricture dilatation, and stent placement became possible by nonsurgical means.[16,17,18] Although ERCP is useful in the removal of extrahepatic bile duct stones,[17,18] the removal of intrahepatic stones is much more problematic.[18]

Because RPC is a complex, progressive disease that involves both the intrahepatic and extrahepatic bile ducts, and because the goals in managing patients with this disorder are complete removal of biliary tract stones and the relief of obstruction, a combination of endoscopic, percutaneous, and surgical techniques is often required.[11,19] Cosenza and coworkers[19] and Stain and associates[20] reported that construction of a hepaticojejunostomy with a cutaneous afferent limb stoma is a safe and effective means of establishing biliary drainage and of providing access for future trans-stomal interventions, such as stone removal and stricture dilatation.

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