AUTHOR AND EDITOR INFORMATION

Section 1 of 12  

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• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
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• Intervention
• Multimedia
• References

INTRODUCTION

Section 2 of 12  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Background

Recurrent pyogenic cholangitis is a disease defined by multiple instances of bacterial biliary tract infection, intrahepatic and extrahepatic biliary pigment stone formation, hepatic abscesses, and dilatation and stricturing of the intrahepatic and extrahepatic bile duct.

Digby published the first detailed English-language report of recurrent pyogenic cholangitis in 1930. He described a syndrome consisting of recurring episodes of suppurative cholangitis accompanied by biliary dilatation and pigment stones in the Chinese populace.

Because recurrent pyogenic cholangitis is endemic to areas of Asia, the terms Oriental cholangitis and Oriental cholangiohepatitis were once commonly used to refer to this syndrome. Recurrent pyogenic cholangitis is being recognized with increasing frequency in Western nations. However, even in these locations, it remains predominantly a disease of Asian immigrants.

Pathophysiology

The exact pathogenesis of recurrent pyogenic cholangitis is not agreed upon. Two contending theories have been offered. The first implicates parasitic infestation of the biliary tract with various helminths as the initiating event. The second theory points to malnutrition as the major predisposing factor.

Parasite infestation theory

Proponents of the parasite infestation theory argue that helminthic organisms such as roundworms (eg, Ascaris lumbricoides) or liver flukes (eg, Clonorchis sinensis) cause the initial injury to the biliary epithelium. The parasites, their ova, and their accompanying debris can also obstruct the biliary duct. These derangements make the biliary tract susceptible to recurring bacterial infections and may help create a nidus for bile pigment stone formation.

Geography dictates the type of parasite encountered. In Asia, the liver fluke C sinensis dominates, with other cases attributed to the Opisthorchis viverrini and Opisthorchis felineus. Indeed, C sinensis infestation is prevalent in Southeast Asia, with estimates of approximately 20 million cases. Humans acquire the parasite by consuming freshwater fish whose flesh is contaminated with the parasite's encysted cercariae. Nearly 20-45% of Asians with recurrent pyogenic cholangitis have evidence of ongoing or historical infestation with one of the aforementioned parasites. The roundworm A lumbricoides is most commonly found outside Asia.

Several factors confound the parasite infestation theory. Most patients with recurrent pyogenic cholangitis have no documented history of parasite infestation. Also, the prevalence of biliary parasite infestation in Asia is no higher in patients with recurrent pyogenic cholangitis than that in patients without recurrent pyogenic cholangitis. Last, areas of China and Japan devoid of Clonorchis infestation have well-defined incidences of recurrent pyogenic cholangitis.

Malnutrition theory

Advocates of the malnutrition theory point to the fact that most patients with recurrent pyogenic cholangitis are from lower socioeconomic groups than those of unaffected individuals, and they tend to consume diets relatively low in protein. These factors, compounded with unsanitary living conditions, lead to multiple instances of bacterial gastroenteritis, bacterial translocation, and portal bacteremia. The end result is bacterial seeding of the biliary tract and recurrent pyogenic cholangitis.

Evidence suggests that a low-protein diet is associated with decreased biliary levels of the beta-glucuronidase inhibitor glucaro-1,4-lactone. Unopposed bacterial beta-glucuronidase deconjugates bilirubin which complexes with calcium to form insoluble calcium bilirubinate. The calcium bilirubinate precipitates to form the nidus for pigment stones, a primary feature of recurrent pyogenic cholangitis.

Common pathway

The common pathophysiologic pathway features recurrent episodes of bacterial cholangitis, ductal obstruction, and dilatation. In some cases, ductal strictures develop. A sort of vicious cycle ensues, with organisms promoting stone formation and ductal obstruction. Obstruction and sluggish bile flow predispose the patient to repeated episodes of cholangitis. Image 1 is a graphic representation of the pathophysiologic mechanisms of recurrent pyogenic cholangitis.

Conclusion

Parasitic infestation and malnutrition likely coexist in a significant proportion of patients with recurrent pyogenic cholangitis. As living conditions and diets improve in areas where recurrent pyogenic cholangitis is endemic, the incidence of the disease decreases. This has been documented in the study by Lo et al (1997). The decreased incidence may be due to improved eating habits, fewer encounters with parasites, or both. As with many other human diseases, the pathogenesis of recurrent pyogenic cholangitis is probably multifactorial.

Frequency

United States

Recurrent pyogenic cholangitis remains rare in the United States. Most cases are seen in Asian immigrants. Nonetheless, the incidence is increasing.

In 1998, Harris and associates reported the institutional experience with recurrent pyogenic cholangitis at the San Francisco General Hospital from 1984 to 1995. They described 45 patients with the disease during that time. This prevalence amounted to a 100% increase compared with the rate in the years before 1984.

International

This disease is most common in Southeast Asia. The disease is also endemic in areas of China, Korea, Taiwan, and Japan. See also Pathophysiology above.

Mortality/Morbidity

• Mortality: Recurrent pyogenic cholangitis is an indolent disease highlighted by acute infectious episodes and intercurrent periods of quiescence. Death directly attributable to recurrent pyogenic cholangitis is rare.
• Morbidity: Complications of recurrent pyogenic cholangitis can involve any organ in the hepatobiliary system. Remote organs can be affected if organisms access the systemic circulation through portal translocation, the space of Disse, or the peritoneal cavity.

  Complications of recurrent pyogenic cholangitis can be divided into hepatobiliary and remote categories.

  • Hepatobiliary complications include the following: hepatic abscesses, biliary cirrhosis, portal vein thrombosis, bile duct rupture with peritonitis, bile duct rupture with biliary-enteric fistula, bile duct rupture with biliary-parietal fistula, acute pancreatitis, chronic pancreatitis or pancreatic ductal dilatation, cholangiocarcinoma, and hemobilia.
  • Remote complications include the following: peritonitis, intra-abdominal abscesses, systemic sepsis, pulmonary abscesses, and brain abscesses.

Race

Almost all cases of recurrent pyogenic cholangitis have been described in persons of Asian ancestry. In Occidental countries, the disease has been seen almost exclusively in Asian immigrants. Case reports of recurrent pyogenic cholangitis in persons of European ancestry have been published from Australia, New Zealand, Spain, and Belgium.

Sex

The incidence is equal in males and females.

Age

The peak incidence of recurrent pyogenic cholangitis occurs during the third through fifth decades of life.

• It is also frequently discovered in patients older than 60 years.
• The disease is rarely diagnosed in children. Children with recurrent pyogenic cholangitis usually present acutely, with signs and symptoms of systemic sepsis, as Saing and colleagues described (1988).

Anatomy

The most important anatomic features of recurrent pyogenic cholangitis involve the intrahepatic and extrahepatic bile ducts and the liver. The gross and histologic features are discussed below.

Gross anatomic features

The hallmark of recurrent pyogenic cholangitis is dilatation of extrahepatic and intrahepatic ducts that contain bile pigment stones. Ductal strictures can occur but are relatively rare. In the liver, this process most markedly involves the central major lobar and segmental ducts. Smaller peripheral ducts appear sharply truncated or cut off, and the overall picture is like that of a pruned tree. The intrahepatic process is most severe in the left hepatic duct, particularly the left lateral segmental duct. Early in recurrent pyogenic cholangitis, the lateral segment of the left hepatic lobe can be the sole site of disease.

Hepatomegaly with scarring is typical in early recurrent pyogenic cholangitis. Deep parenchymal and subcapsular abscesses may be present, and the Glisson capsule is frequently involved with numerous adhesions to surrounding structures. Following multiple infectious episodes, hepatic atrophy ensues, again mainly affecting the left lateral segment. Hepatic parenchyma in the left lateral segment can be completely destroyed, leaving only dilated ducts surrounded by fibrous scar tissue.

The common hepatic and common bile ducts can be enormously dilated and tortuous. The common bile duct is the most common location where pigment stones are found.

The sphincter of Oddi can show signs of trauma due to repeated passage of bile duct stones. The papilla may be hypertrophic and scarred.

Approximately 50-70% of patients with recurrent pyogenic cholangitis have stones in the gallbladder. The gallbladder frequently harbors features of acute or chronic cholecystitis, such as thickening of the wall or pericholecystic fluid.

Histologic features

In the liver, the portal triads can be infiltrated with inflammatory cells. Nests of these cells can extend into the hepatic parenchyma and become associated with a suppurative process. Evidence of thrombophlebitis of portal vein tributaries may be present.

Clinical Details

Clinical signs and symptoms

Clinically active recurrent pyogenic cholangitis is characterized by right upper quadrant abdominal pain, jaundice, and fever with or without accompanying rigors. This clinical constellation is recognized as the Charcot triad. If cholangitis ensues, these signs and symptoms may be augmented by mental status changes and hypotension (Reynolds pentad). Most patients have no history of antecedent episodes. However, up to 30% of patients can describe previous episodes. Many have undergone 1 or more biliary tract operations.

Physical examination

Features of the physical examination include right upper quadrant abdominal tenderness, hepatomegaly, and jaundice. Icterus may be discernible in the sclerae, frenulum of the tongue, tympanic membranes, or the skin. About 25-35% of patients have a palpable gallbladder. This may be caused by acalculous or calculous cholecystitis, gallbladder empyema, or hydrops of the gallbladder.

Bacteriology of recurrent pyogenic cholangitis

The most common organism cultured from the bile of patients with recurrent pyogenic cholangitis is Escherichia coli. The next most common organisms are other enteric gram-negative bacteria, including Klebsiella, Pseudomonas, and Proteus species. Polymicrobial infections are frequently encountered. Anaerobic bacteria are uncommonly recovered. It is hypothesized that transient portal bacteremia introduces organisms into the biliary tree. Under normal circumstances, these are efficiently cleared by endogenous antimicrobial defense mechanisms. However, the ductal epithelial damage, stasis, and obstruction associated with recurrent pyogenic cholangitis allow bacteria to flourish.

Characteristics of bile duct stones

The biliary stones of recurrent pyogenic cholangitis arise within the ducts themselves, as opposed to the gallbladder. The most common location where stones are found is the common bile duct, but these stones are believed to originate from more proximal biliary radicles such as the intrahepatic ducts. In many cases, stones are seen at multiple levels in the biliary tree. The stones are composed primarily of bile pigments and calcium. Stones exhibit a variety of colors (brown, black, orange), consistencies (soft, gravely, muddy, firm), and sizes ( <3 mm to > 3 cm).

Laboratory studies

The most common laboratory findings in recurrent pyogenic cholangitis include the following: increased WBC count, left shift with increased polymorphonuclear leukocytes, increased hepatic acute-phase reactants, increased erythrocyte sedimentation rate, elevated serum alkaline phosphatase level, hyperbilirubinemia, and elevated serum amylase level (particularly if bile duct obstruction is present).

Examination of stool specimens for ova should be requested in cases in which parasitic infestation is suspected.

Diagnosis

The typical clinical constellation, especially with a history of antecedent episodes, in a patient from an endemic area should lead to the inclusion of recurrent pyogenic cholangitis in the differential diagnosis. Other entities in the differential diagnosis include the following: choledocholithiasis with or without concurrent cholangitis, primary sclerosing cholangitis, choledochal cyst (especially types IV and V), and periampullary tumors (including pancreatic and bile duct cancers) with biliary obstruction. Image 2 lists features helpful in differentiating these diseases.

Preferred Examination

The initial screening radiologic study of choice is abdominal ultrasonography. It is highly sensitive for ductal stones and ductal dilatation, and it may be helpful in localizing hepatic abscesses or other complications. All patients should undergo abdominal CT scanning with contrast enhancement. CT scanning allows full assessment of the extent of disease. The merits and inadequacies of these tests are discussed more completely in subsequent sections.

Limitations of Techniques

Ultrasonography is an excellent screening test. However, it is operator dependent and may not demonstrate the full extent of disease. For example, intrahepatic ductal strictures and hepatic lobar atrophy can be missed. CT scanning demonstrates the features of anatomic disease, such as the extent of ductal involvement, the status of the hepatic lobes, and the presence or absence of associated abscesses. On CT scans, isoattenuating features, such as noncalcified stones and biliary sludge, can be missed.

DIFFERENTIALS

Section 3 of 12  

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RADIOGRAPH

Section 4 of 12  

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Findings

Plain abdominal radiographs may be obtained in the workup of patients presenting acutely with abdominal pain or jaundice. The results are nonspecific and add little to no evidence to support a diagnosis of recurrent pyogenic cholangitis.

Degree of Confidence

Plain radiographs provide no reliable information to make or refute the diagnosis of recurrent pyogenic cholangitis. If the diagnosis is suspected, and abdominal sonography or CT scanning must be performed as the next step.

False Positives/Negatives

Because radiography is insensitive and nonspecific, false-positive or false-negative findings are not well described.

CT SCAN

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Findings

Abdominal CT scanning is the most important noninvasive radiologic study in the diagnosis of recurrent pyogenic cholangitis. It provides a wealth of information pertaining to the extent of disease, and is an invaluable adjunct in therapeutic planning.

In 1989, Chan and coworkers used CT to examine 50 patients with recurrent pyogenic cholangitis. Among the patients, 22 had undergone previous surgical procedures including sphincteroplasty or choledochoenterostomy. A wide range of pathologic features was observed; these included intrahepatic ductal dilatation in all (100%), intrahepatic ductal calculi (74%), common bile duct dilatation (68%), pneumobilia (52%), hepatic segmental atrophy (36%), common bile duct calculi (30%), bile duct strictures (22%), and splenomegaly (14%). Unilobar hepatic disease was seen in 28%. The left lateral segment was most commonly involved.

The authors characterized CT findings demonstrable during acute exacerbations of recurrent pyogenic cholangitis. These included hepatic segmental parenchymal enhancement, hepatic abscess formation, enhancement of duct walls and biloma. These investigators concluded that CT is sensitive and allows complete radiologic evaluation of recurrent pyogenic cholangitis. Furthermore, they stated that CT was excellent for confirming sonographic findings, evaluating associated masses, and planning therapeutic procedures (eg, hepatic resection, duct manipulation, stone extraction).

Yoon and colleagues (1999) reported 13 inflammatory hepatic pseudotumors detected with CT scanning. The pseudotumors were 2-7 cm. They appeared as ill-defined, hypoattenuating lesions on nonenhanced scans. With the addition of contrast material, the lesions featured central hypoattenuation with a hyperattenuating or isoattenuating appearance at the periphery in 4 cases. In 9 lesions, multiple hyperattenuating internal septa were noted, as well as a hyperattenuating appearance from the periphery of the mass. All lesions were resected. The histologic findings were consistent with recurrent pyogenic cholangitis.

Findings consistent with portal hypertension, such as splenomegaly and varices, can be demonstrated by CT scan.

Spiral CT data can be reconstructed in a 3-dimensional fashion following the infusion of cholangiographic contrast agents. The reconstruction provides a noninvasive way to specifically image the biliary tree. This technique is called CT cholangiography and has been reported by Klein (1993) and Stockberger (1994). Although the demonstration of stones and strictures is about as good as that with endoscopic retrograde cholangiopancreatography (ERCP), the technique does not yet have the overall accuracy to serve as a front-line replacement for studies such as ERCP or percutaneous transhepatic cholangiography (PTC).

Degree of Confidence

Abdominal CT scanning is currently the most reliable imaging study for ascertaining the complete status of recurrent pyogenic cholangitis. It effectively demonstrates disease in the biliary tree and within the hepatic parenchyma with a high degree of sensitivity and overall accuracy.

Current spiral CT scanners are sensitive for detecting extrahepatic and intrahepatic calculi. More than 90% of intrahepatic stones appear hyperattenuating compared with the nonenhancing hepatic parenchyma.

Hepatic segmental atrophy, compensatory hypertrophy, capsular distortion, and complications (eg, abscesses, bilomas, cholangiocarcinomas) are reliably imaged with CT. Other parenchymal infectious complications like acute focal pyogenic hepatitis are easily delineated and appear as persistent parenchymal enhancement in affected segments.

CT is good at detecting peripheral cholangiocarcinomas, as reported by Honda and colleagues (1993) but less reliable (40%) for hilar lesions as described by Soyer and coworkers (1995). Most central lesions less than 1.5 cm in diameter will be missed by CT scanning. Features of cholangiocarcinoma on CT include hypoattenuation, lack of encapsulation, central scarring, contrast pooling, and capsular retraction.

False Positives/Negatives

Hyperattenuating stones become more difficult to image after the hepatic parenchyma is enhanced with the administration of contrast material. Pneumobilia in patients presenting with acute exacerbations or in those who have undergone a biliary-enteric anastomosis for drainage can decrease the sensitivity of the examination. However, this can be remedied by adjusting window width.

Upper gastrointestinal preparation with oral contrast agents should be avoided. This approach may cause contrast material to reflux into the common bile duct and confound the findings of the study.

Examiners should be aware that chronic disease can distort the hepatic parenchyma, with atrophy of affected segments or lobes and compensatory hypertrophy of other areas. This can cause rotation of the liver and alteration of normal anatomical relationships of portal triad structures. The most common hepatic segments that display atrophic changes include the lateral segment of the left lobe and posterior segment of the right lobe.

MRI

Section 6 of 12  

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Findings

MRI is being used more frequently than before in the assessment of recurrent pyogenic cholangitis. It can demonstrate all the salient features of the disease, including stones, ductal dilatation and strictures, and hepatic parenchymal disease. Hepatic segmental or lobar atrophy and hypertrophy are easily delineated. Pyogenic hepatitis appears as hepatic parenchymal enhancement on T1-weighted images. Hepatic abscesses and cholangiocarcinomas are both focally hypointense and hyperintense masses on T1- and T2-weighted images, respectively.

T2-weighted images are well suited for depicting ductal pathology because bile-filled structures produce particularly high signal intensities. According to Brinks and Borello (1995), stones are easily detected as intraductal filling defects, irrespective of their chemical composition. In comparison, T1-weighted images are better for demonstrating acute suppurative cholangitis, which appears as ductal wall enhancement.

Magnetic resonance cholangiography (MRC) is a technique that allows 3-dimensional reconstruction and display of the biliary tree. It is most commonly performed by using heavily T2-weighted protocols, which make bile-filled structures appear extremely bright against a hypointense background. Contrast agents are not used. Features of recurrent pyogenic cholangitis, such as ductal dilatation, common bile duct stones, and intrahepatic stones, are detected with sensitivities in the range of 80-95%. MRC accurately depicts sites of obstruction in 85-100% of cases. Reports supporting these rates have been published (Hall-Craggs, 1993; Laubenberger, 1995; Reinhold, 1996).

Park and colleagues (2001) compared MRC with direct conventional cholangiography. A total of 24 patients underwent preoperative MRC before surgery for recurrent pyogenic cholangitis. Eighteen also underwent preoperative conventional cholangiography. The results were verified by the anatomic findings at surgery. MRC was able to depict 100% of hepatic segments with ductal dilatation, 98% of segments with ductal calculi, and 96% of segments with focal strictures. Conventional cholangiography showed 47% of segments with ductal dilatation, 45% with ductal calculi, and 44% with focal ductal strictures. The authors concluded that MRC was superior to conventional cholangiography for the anatomic evaluation of recurrent pyogenic cholangitis since it can depict the entire biliary tree, even in the presence of ductal obstruction or stenosis.

Degree of Confidence

MRI and MRC are highly sensitive and accurate techniques. They can depict ductal and parenchymal pathology as well as any other radiologic study and are likely to become the studies of choice in recurrent pyogenic cholangitis. These techniques do not expose the patient to radiation and do not require the injection of contrast agents.

In 1995, Soyer and colleagues described the presence of a hyperintense central scar as described on T1-weighted images. This finding and contrast-agent pooling in the mass on late images are more consistent with a cholangiocarcinoma that with another condition. Data published by Fan and coworkers (1993) suggest that MRI is more sensitive than CT in detecting hilar cholangiocarcinomas.

False Positives/Negatives

No normal variants that mimic this disease process have been well described. Various pathologic findings associated with this disease process are better visualized on T1-weighted images than on T2-weighted images or vice versa. These techniques are complementary and should be used together to avoid false-negative results.

ULTRASOUND

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Findings

Abdominal ultrasonography is the initial screening examination of choice. Sonography is noninvasive and does not expose the patient to ionizing radiation. It is also sensitive for biliary tract pathology. The findings can suggest or confirm the diagnosis in up to 97% of cases of recurrent pyogenic cholangitis, according to Ohto and coworkers (1984).

Sonography easily demonstrates dilatation of extrahepatic and proximal intrahepatic ducts. Calculi within the extrahepatic biliary radicles and central intrahepatic ducts are readily depicted as described by Lim and colleagues (1990). The intrahepatic stones are usually hyperechoic compared to the hepatic parenchyma. Sonograms may depict the decreased arborization or truncation of hepatic ducts that typify recurrent pyogenic cholangitis.

Features of hepatic parenchymal disease that can be detected with sonography include increased periportal echogenicity, liver abscesses, and bilomas. An associated cholangiocarcinoma appears as a homogeneous, hyperechoic mass as reported by Zhang and coinvestigators (1996).

Ultrasonography can also be used to help guide interventional procedures such as the drainage of bilomas and hepatic abscesses.

Sonography is the best noninvasive modality for actual imaging of the offending parasites. Flukes are poorly visualized with CT and MRI. With sonography, flukes or clusters of the parasites appear as echogenic foci that are nonshadowing within the bile ducts. Morikawa and associates (1988) were able to capture flukes in motion within peripheral bile ducts on M-mode sonograms. The gallbladder is the best location to visualize parasites. They sink toward the dependent portion of the organ but can be made to float temporarily with a change in position or transdermal agitation of the gallbladder with the transducer. According to Lim and colleagues (1989), flukes in the gallbladder appear fusiform and weakly echogenic and do not produce shadows.

Degree of Confidence

The diagnosis of recurrent pyogenic cholangitis can be made with a high degree of confidence in the vast majority of patients. Sonographic findings should be confirmed by another modality such as CT or MRI. These studies are better at defining the overall extent of hepatic parenchymal and intrahepatic ductal disease.

False Positives/Negatives

Distal intrahepatic ductal and parenchymal disease can be difficult to image with sonography. Patients with acute exacerbations of recurrent pyogenic cholangitis can present with pneumobilia, which obscures details of the ductal system. The presence of ductal air along with a dilated duct packed with stones can appear like an abscess in the acute setting. Also, because the stones in recurrent pyogenic cholangitis are pigment stones, they may be isoechoic and therefore missed by sonographic examination. As always, ultrasonography is highly operator dependent.

NUCLEAR MEDICINE

Section 8 of 12  

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Findings

Radionuclide studies are not typically used in the diagnostic algorithm for recurrent pyogenic cholangitis. Their results are nonspecific and do not yield enough information upon which to base treatment strategy. While the studies may demonstrate some extrahepatic ductal abnormalities well, they are poor choices for delineating the location and extent of intrahepatic disease.

ANGIOGRAPHY

Section 9 of 12  

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Findings

Before the advent of sophisticated CT and MRI techniques, direct contrast cholangiography was considered essential in the diagnosis and extent of disease workup for recurrent pyogenic cholangitis. It can still provide critical information about location and extent of ductal dilatation and strictures, and stones.

Classic cholangiographic findings of recurrent pyogenic cholangitis include ductal dilatation, which is frequently most marked in the extrahepatic duct; dilatation of the proximal central intrahepatic ducts with associated abrupt truncation of more peripheral ducts; and filling defects in contrast agent–filled ducts, which represent the presence of stones.

Khuroo and coworkers (1993) published findings of direct contrast cholangiography in 227 patients with recurrent pyogenic cholangitis. Baseline cholangiographic abnormalities included biliary calculi and sludge, bile duct dilatation and strictures. Only 21 patients had cholangiographic evidence of prior parasite infestation. A cohort of 55 patients underwent serial cholangiography at intervals of about 18 months. In this subgroup, symptoms and cholangiographic abnormalities resolved in 25 who underwent successful endoscopic sphincterotomy and extraction of biliary calculi. In the remaining patients, recurrent pyogenic cholangitis reoccurred, and the cholangiographic findings worsened over time.

ERCP and PTC are the most common clinical approaches to cholangiography. Both of these are invasive procedures and have low but not negligible complication rates. In addition, Sherman and Lehman (1991) reported inability to cannulate the common bile duct in 5-10% of attempted ERCP studies.

Degree of Confidence

Cholangiography demonstrates ductal disease and the presence of stones with relatively high sensitivity and specificity. This is especially true of the extrahepatic and more centrally located intrahepatic bile ducts. Weaknesses include inability to detect disease distal to strictures and an inherent inability to image the hepatic parenchyma. CT scanning is required to provide information about the extent of hepatic parenchymal disease. This is particularly critical if hepatic resection is planned.

False Positives/Negatives

The predictive value of a positive test is high. The false-positive findings are few. False-negative studies can occur when strictures limit the ability to image distal ductal disease.

INTERVENTION

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Management strategies

Management strategies for recurrent pyogenic cholangitis can be divided into acute and long-term treatment phases (see Image 3). In the acute phase, bacterial cholangitis is eradicated, and the biliary system is decompressed. Intravenous fluid resuscitation and intravenous antibiotics are given. Biliary obstruction is preferably treated with nonsurgical methods. Options include ERCP with sphincterotomy and PTC. If these are ineffective, surgery may be required. Surgical approaches include choledochostomy, removal of stones, sphincteroplasty, and T-tube placement.

Recently, efforts have focused on the application of minimally invasive surgical techniques to the management of recurrent pyogenic cholangitis. Tang and coworkers reviewed their database of recurrent pyogenic cholangitis cases managed by either open (n = 10) or hand-assisted laparoscopic (n = 17) left lateral segmentectomy from 1994-2004. Operative time was longer in the laparoscopic group (232 vs 150 min, P = .007). Inpatient postoperative stay was reduced in the laparoscopic group (8 vs 14 d, P = .019). Total postoperative complications were less in the laparoscopic group, but the difference did not reach statistical significance (P = .129).

The authors stated recurrent pyogenic cholangitis did not recur in either group at 3-year follow-up. The authors concluded that hand-assisted laparoscopic left segmentectomy for recurrent pyogenic cholangitis was as effective as open operation for control of the disease. Furthermore, although operative time is longer, recovery is more rapid and hospital stay curtailed.

The same group detailed their experience with laparoscopic choledochoduodenostomy for the treatment of recurrent pyogenic cholangitis. They performed 12 such procedures in recurrent pyogenic cholangitis patients without intrahepatic strictures between 1995 and 2002. No cases required conversion to open operation. The single major complication was a postoperative bile leak that was successfully managed conservatively. Mean follow-up time was 37.6 months, and instances of cholangitis or sump syndrome were reported. The authors concluded that laparoscopic choledochoduodenostomy is a safe, efficacious drainage procedure in patients with recurrent pyogenic cholangitis.

In the long-term phase, treatment goals include removal of all stones and ductal debris, establishment of durable biliary drainage, correction of associated bile duct strictures, and provision of enterobiliary access for clearance of metachronous bile duct stones. Enterobiliary access is established by construction of a Roux-en-Y hepaticojejunostomy in which the end of the afferent limb is placed in a subcutaneous position, or is formally matured as a stoma. This allows cholangioscopic access via the afferent limb. Biliary strictures can be dilated and stones removed by basketing or electrohydraulic lithotripsy. Good results with these techniques have been published (Fan, 1991; Stain, 1995; Cosenza, 1999; Gott, 1996). Tang and Li (2002) published their experience with hand-assisted laparoscopic resection of atrophic hepatic segments, citing a low recurrence rate after surgical extirpation of segments that harbor stones.

Stent placement

In 1997, Yoon and coworkers reported on the efficacy of expandable metallic stents in the treatment of biliary strictures associated with recurrent pyogenic cholangitis. They placed a total of 26 metallic stents in 23 patients. These stents included 19 Gianturco-Rosch Z stents and 7 Strecker stents. Insertion routes included PTC tracts (n = 16) and T-tube tracts (n = 7); retrograde endoscopic deployment was also used (n = 3).

The initial success rate was 100%. The mean duration of stent patency was 34 months. On average, Gianturco stents remained patent 5 times longer than Strecker stents (50 vs 10 mo; P <.05). Stents placed in intrahepatic ducts (50 mo) tended to remain patent longer than those placed in extrahepatic ducts (18 mo; P = .05). The primary patency rates for the entire group were 92%, 75%, 67%, and 46% at 6, 12, 24, and 36 months, respectively. Causes of stent obstruction included recurrence of stones or reaccumulation of biliary sludge (n = 8), and epithelial hyperplasia (n = 5). The authors concluded that metallic stent placement was not an effective long-term therapy for biliary strictures in recurrent pyogenic cholangitis.

In contrast to these mixed results with stent placement, interventional radiologic techniques have become a mainstay in the therapy of chronic recurrent pyogenic cholangitis. As described in the Clinical Details section, several authors have reported excellent success rates with surgical creation of a jejunal biliary access limb. This allows interventional radiologists to percutaneously basket and remove metachronous intrahepatic stones. As alternative, the stones may be destroyed by electrohydraulic lithotripsy using the access loop.

Medical/Legal Pitfalls

• The only medicolegal pitfalls associated with this condition center on diagnostic delays, both of the primary condition itself and its complications, such as cholangiocarcinoma. These delays should be uncommon given the current diagnostic armamentarium.
• Because recurrent pyogenic cholangitis remains a relatively rare entity in the United States, clinicians and radiologists should maintain a high index of suspicion in a patient with the typical clinical picture who is from an area with endemic disease.

MULTIMEDIA

Section 11 of 12  

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• Multimedia
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Media file 1:  Pathophysiology of recurrent pyogenic cholangitis (RPC).
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Media file 2:  Differential diagnosis of recurrent pyogenic cholangitis (RPC).
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Media file 3:  Management strategies in recurrent pyogenic cholangitis (RPC).
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Media file 4:  Dilated common bile duct in patient with recurrent pyogenic cholangitis (RPC).
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Media file 5:  Preparation to perform bilioenteric anastomosis in Roux-en-Y hepaticojejunostomy.
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Media file 6:  Jejunum being anastomosed to the common bile duct.
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Media file 7:  Completed bilioenteric anastomosis in patient with recurrent pyogenic cholangitis (RPC).
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Media file 8:  Catheter passed via subfascial terminus of hepaticojejunostomy for dilatation of biliary strictures and removal of stones.
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Media file 9:  Close-up image of wire placed in abdominal wall to delineate location of subfascial terminus of Hutson-Russell access loop.
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Media file 10:  Cholangiogram demonstrates disease in extrahepatic and right intrahepatic biliary tree in patient with recurrent pyogenic cholangitis (RPC).
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Media file 11:  Guidewires placed across strictures and into biliary radicles. Stones can be seen as filling defects.
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Media file 12:  Stone extraction in progress. Stones and debris can be seen in access catheter.
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Media file 13:  Some stones and debris have been displaced into jejunum.
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Media file 14:  Stricture dilated. Some residual stones are seen in biliary radicles.
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Media file 15:  Biopsy of liver with pus in bile ducts. Image courtesy of Greg Everson, MD.
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Media file 16:  CT scan of pyogenic liver abscess. Image courtesy of Greg Everson, MD.
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REFERENCES

Section 12 of 12

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

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Article Last Updated: Jun 22, 2006