Radiologic Manifestations of Sclerosing Cholangitis with Emphasis on MR Cholangiopancreatography
Abstract
Magnetic resonance cholangiopancreatography (MRCP) is a relatively new, noninvasive cholangiographic technique that is comparable with invasive endoscopic retrograde cholangiopancreatography (ERCP) in the detection and characterization of extrahepatic bile duct abnormalities. The role of MRCP in evaluation of the intrahepatic bile ducts, especially in patients with primary or secondary sclerosing cholangitis, is under investigation. The key cholangiographic features of primary sclerosing cholangitis are randomly distributed annular strictures out of proportion to upstream dilatation. As the fibrosing process worsens, strictures increase and the ducts become obliterated, and the peripheral ducts cannot be visualized to the periphery of the liver at ERCP. In addition, the acute angles formed with the central ducts become more obtuse. With further progression, strictures of the central ducts prevent peripheral ductal opacification at ERCP. Cholangiocarcinoma occurs in 10%–15% of patients with primary sclerosing cholangitis; cholangiographic features that suggest cholangiocarcinoma include irregular high-grade ductal narrowing with shouldered margins, rapid progression of strictures, marked ductal dilatation proximal to strictures, and polypoid lesions. Secondary sclerosing and nonsclerosing processes can mimic primary sclerosing cholangitis at cholangiography. These processes include ascending cholangitis, oriental cholangiohepatitis, acquired immunodeficiency syndrome–related cholangitis, chemotherapy-induced cholangitis, ischemic cholangitis after liver transplantation, eosinophilic cholangitis, and metastases.
LEARNING OBJECTIVES FOR TEST 3
After reading this article and taking the test, the reader will be able to:
| •. | Describe the causes and pathophysiology of primary and secondary sclerosing cholangitis. | ||||
| •. | Recognize the cholangiographic manifestations of primary and secondary sclerosing cholangitis. | ||||
| •. | List the advantages and disadvantages of MR cholangiopancreatography. | ||||
Introduction
Noninvasive cholangiography, such as magnetic resonance (MR) cholangiopancreatography (MRCP), may have a role in diagnosis and follow-up of patients with sclerosing cholangitis. Potential advantages of MRCP include (a) visualization of bile ducts not visualized at endoscopic retrograde cholangiopancreatography (ERCP) because of strictures, (b) noninvasive detection of progressive bile duct abnormalities in asymptomatic patients, and (c) early detection of cholangiocarcinoma. These advantages would ultimately result in earlier diagnosis and therapy.
In this article, we present the spectrum of intrahepatic and extrahepatic bile duct abnormalities encountered in patients with primary sclerosing cholangitis (PSC), cholangiocarcinoma, or secondary processes simulating PSC. The emphasis is on MRCP with ERCP correlation.
Primary Sclerosing Cholangitis
PSC is an idiopathic, chronic, fibrosing inflammatory disease of the bile ducts that eventually leads to bile duct obliteration, cholestasis, and biliary cirrhosis. A strong association with inflammatory bowel disease, especially ulcerative colitis, is noted (70% of cases) (,1). Although the cause of PSC is unknown, most experts believe it to be an autoimmune process because PSC may be associated with other autoimmune diseases such as retroperitoneal fibrosis, mediastinal fibrosis, and Sjögren syndrome (,2,,3). The rate of progression is unpredictable, with up to 49% of symptomatic patients eventually developing biliary cirrhosis and liver failure (,4). Treatment is usually palliative and includes medical therapy with orally administered agents such as ursodiol (ursodeoxycholic acid) or endoscopic or percutaneous mechanical dilation of dominant strictures. Currently, orthotopic liver transplantation is the only curative therapy for PSC.
Diagnostic criteria for PSC include (a) typical cholangiographic abnormalities; (b) appropriate clinical, biochemical, and hepatic histologic findings; and (c) the exclusion of secondary causes of sclerosing cholangitis. Cholangiographic findings usually include multifocal, intrahepatic bile duct strictures alternating with normal-caliber ducts, which sometimes produce a beaded appearance (,5). Although patients may be asymptomatic, 75% have progressive fatigue, pruritus, or jaundice (,6). At biochemical analysis, levels of serum bilirubin and alkaline phosphatase are increased in most patients, with a mean increase in the alkaline phosphatase level to three times the upper limit of normal (,6,,7). Because histologic examination of the liver shows nonspecific inflammatory fibrosis of the portal triads and a paucity of bile ducts, histologic analysis is used only for confirmation of a suspected diagnosis of PSC. Before the diagnosis of PSC is established, secondary sclerosing and nonsclerosing processes that mimic PSC at cholangiography must be excluded. These include chronic bacterial cholangitis complicating strictures or stones, parasitic infection of the bile, cholangitis related to acquired immunodeficiency syndrome (AIDS), ischemia due to treatment with floxuridine or hepatic arterial thrombosis complicating liver transplantation, neoplasms including cholangiocarcinoma, metastases, previous bile duct surgery, and congenital biliary anomalies.
ERCP is the standard of reference for diagnosis of PSC because the clinical, biochemical, and hepatic histologic findings are usually nonspecific. Advantages of ERCP include increased sensitivity to peripheral intrahepatic duct abnormalities and interventional capabilities such as mechanical dilation of obstructing strictures, stent placement, and biopsy. However, in patients with PSC, ERCP is associated with an increased risk of complications such as cholangitis due to bile stasis and perforations due to sacculations and the increased rigidity of the ducts (,8–,10).
MRCP is a relatively new, noninvasive cholangiographic technique that is comparable with ERCP in the detection and localization of bile duct obstruction and choledocholithiasis (,11–,15). By using heavily T2-weighted sequences, the signal of static or slow-moving fluid-filled structures such as the bile and pancreatic ducts is greatly increased, resulting in increased duct-to-background contrast (,,,,Fig 1). Although ERCP is still the standard of reference for imaging the pancreaticobiliary system, MRCP has some advantages over ERCP. Specifically, MRCP (a) is noninvasive; (b) is cheaper; (c) uses no ionizing radiation; (d) requires no anesthesia; (e) is less operator dependent; (f) better demonstrates ducts proximal to an obstruction or tight stenosis; and (g) when combined with conventional T1- and T2-weighted sequences, allows anatomic imaging of extraductal disease. However, MRCP provides less spatial resolution than ERCP, thereby decreasing the sensitivity to peripheral ductal abnormalities (,,,Figs 2, ,,,3). In addition, the peripheral ducts may not be visualized because imaging is performed in the physiologic, nondistended state. The main criticism of MRCP is that appropriate care may be delayed in patients who need therapeutic endoscopic or percutaneous intervention of obstructing bile duct lesions.
At MRCP, the presence of stenoses is usually inferred when there is prestenotic dilatation (,16); however, in the initial stages of stenosis, there is only temporal dilatation due to an increased volume of pancreatic juice and bile after nutrition-induced stimuli. These early stenoses may be undetectable at MRCP. In addition, the extent of a focal, short stricture, especially of the common hepatic duct, may be overestimated at MRCP because the duct downstream from the stricture will be collapsed. ERCP involves the injection of contrast medium, which dilates the ducts so that early strictures can be diagnosed and the full extent of focal strictures can be assessed. However, there may be a discrepancy in ductal caliber between cross-sectional imaging and ERCP because the injection pressure of contrast medium coupled with image magnification may falsely dilate the duct. The upper limit of normal for the caliber of the common bile duct at ERCP and cross-sectional imaging is 8 mm and 6 mm, respectively; normal duct caliber may increase by as much as 6 mm during ERCP (,17–,19).
The role of MRCP in evaluation of intrahepatic ductal abnormalities is currently under investigation. Two small studies have shown good correlation between MRCP and ERCP in diagnosis of sclerosing cholangitis (,11,,20). Mildly dilated peripheral ducts that do not connect to the central ducts may be a characteristic sign at MRCP (,20). However, larger studies are necessary to establish the usefulness of MRCP in evaluation of the intrahepatic bile ducts in PSC.
The cholangiographic findings in PSC depend on the stage of the disease process. Early in the course of the disease, randomly distributed, short (1–2 mm), annular intrahepatic strictures alternating with normal or slightly dilated segments produce a beaded appearance (,Figs 4, ,5). Strictures usually occur at the bifurcation of ducts and are out of proportion to upstream ductal dilatation. The peripheral ducts should extend to the periphery of the liver and form acute angles with the central ducts (,,,Fig 6). As the fibrosing process worsens, strictures increase and the ducts become obliterated, and the peripheral ducts cannot be visualized to the periphery of the liver at ERCP, producing a “pruned tree” appearance. In addition, the acute angles formed with the central ducts become more obtuse (,,,Figs 7, ,,,8). With further progression, strictures of the central ducts prevent peripheral ductal opacification at ERCP (,,,Figs 9, ,10). The key cholangiographic features of PSC are randomly distributed annular strictures out of proportion to upstream dilatation, which probably reflect periductal inflammation and fibrosis that prevent the ducts from dilating. If the ducts in a case simulating PSC are dilated, one should consider other sclerosing ductal processes such as ascending cholangitis or PSC complicated by cholangiocarcinoma.
Other cholangiographic findings include webs, diverticula, and stones. A web is a focal, 1–2-mm-thick area of incomplete circumferential narrowing (,Figs 11, ,12); a diverticulum is a focal, eccentric, saccular dilatation of the bile duct (,Figs 13,–,,,15). These two findings may represent manifestations of the same abnormality because a web may transform into a diverticulum with an increase in intraductal pressure during conventional cholangiography (,21). Diverticula and webs are not pathognomonic for PSC and can be seen in other inflammatory and traumatic processes (,21). Up to 27% of patients with PSC have diverticula (,21). Primary pigmented stones occur in 30% of patients with PSC secondary to bile stasis (,Fig 16).
Cholangiocarcinoma
Cholangiocarcinoma is an uncommon neoplasm, representing approximately 0.5%–1% of all cancers and 30% of hepatic primary malignancies. It occurs in 10%–15% of patients with PSC and has a poor prognosis, with a median survival of 7 months and a 5-year survival of 0%–10% (,22–,26). Cholangiocarcinoma has been detected at autopsy or at liver transplantation in 7%–36% of patients with PSC (,25,,27–,29). Clinically, cholangiocarcinoma is suspected when a patient has rapid clinical deterioration such as worsening of jaundice, pruritus, and weight loss in association with a rapid rise in serum bilirubin and alkaline phosphatase levels (,25). The tumor is usually located in the common hepatic duct or at the bifurcation (,,,,Fig 17) and contains a large central core of fibrotic tissue. Up to 70% are Klatskin tumors located at the hilum. Unfortunately, there is no cholangiographic feature specific for cholangiocarcinoma. Cholangiographic features that suggest cholangiocarcinoma include irregular high-grade ductal narrowing with shouldered margins, rapid progression of strictures, marked ductal dilatation proximal to strictures, and polypoid lesions, especially those larger than 1 cm in diameter (,30). An additional limitation of conventional cholangiography is insensitivity in demonstrating the extraductal extent of the tumor (,31). The success of endoscopic brush cytologic biopsy is variable, with sensitivities of 30%–85% (,32–,35). Therefore, it is impossible to exclude cholangiocarcinoma on the basis of a negative brush biopsy result.
Cross-sectional imaging modalities such as computed tomography (CT) and MR imaging in conjunction with ERCP can assist in the work-up of suspected cholangiocarcinoma by demonstrating extraductal abnormalities. In fact, in comparison with ERCP, CT and MR imaging allow better staging of the disease in one-third of cases. Because most cholangiocarcinomas have a fibrous center, they demonstrate delayed accumulation and washout of contrast material, thus producing hyperattenuating lesions at delayed contrast material–enhanced imaging in some cases (,,,,,,,Fig 18) (,36–,38). Because of its increased contrast resolution, dynamic contrast-enhanced MR imaging has been shown to be more sensitive than CT for the diagnosis and staging of cholangiocarcinoma. In addition, MRCP may provide more information than conventional cholangiography about the proximal extent of disease, thereby allowing a more accurate assessment of resectability (,39). At MR imaging, periportal changes manifesting as low signal intensity on T1-weighted images and high signal intensity on T2-weighted images are seen. Such changes are suggestive of cholangiocarcinoma if their extent is greater than 1.5 cm and they enhance after administration of gadolinium contrast material (,,,Fig 19).
Secondary Processes Simulating PSC
Secondary sclerosing processes that resemble PSC at cholangiography include ascending cholangitis as a result of strictures, stones, or bile duct anomalies; oriental cholangiohepatitis; AIDS-related cholangitis; and ischemia related to intraarterial chemotherapy or iatrogenic injury. A variety of nonsclerosing processes can also mimic PSC at cholangiography, including hepatic metastases, cirrhosis, polycystic liver disease, multifocal hepatoma, and cholangiocarcinoma. Therefore, one must consider clinical and laboratory data to achieve an accurate cholangiographic interpretation.
Ascending Cholangitis
Ascending cholangitis usually occurs secondary to bacterial contamination of an obstructed biliary system, usually with gram-negative enteric bacteria. Bile duct strictures, choledocholithiasis, and papillary stenosis are the most common causes of the obstruction (,Fig 20). The role of MRCP or ERCP is to enable diagnosis of the underlying cause of the obstruction, not to enable diagnosis of ascending cholangitis, which is a clinical diagnosis. Patients usually have the classic Charcot triad of abdominal pain, fever, and jaundice. Liver abscess and sepsis can occur when the increased ductal pressure causes necrosis of the duct wall with reflux of infected bile into the portal triads.
Oriental Cholangiohepatitis
Oriental cholangiohepatitis is a chronic parasitic infection of the bile ducts characterized by recurrent attacks of abdominal pain, fever, and jaundice. In endemic areas, such as Southeast Asia, parasites such as Clonorchis sinensis and Ascaris lumbricoides can inhabit the bile ducts and induce ductal injury and strictures (,40,,41). Oriental cholangiohepatitis is uncommon in the United States but represents the third most common cause of acute abdomen in Hong Kong. Cholangiographic findings include multifocal strictures and dilatation of the intrahepatic bile ducts, stones, disproportionate dilatation of the extrahepatic bile duct unrelated to strictures or stones, a right-angle branching pattern, and decreased arborization (,42,,43). In rare instances, cholangiectasis or “bile lakes” can occur from ongoing obstruction and inflammation (,,,,Fig 21) (,44). Complications include primary pigmented stones (80% of cases), hepatic abscess, and cholangiocarcinoma (2.5%–5.0%) (,45–,47).
AIDS-related Cholangitis
AIDS-related cholangitis is considered an infectious cholangitis. Opportunistic infectious organisms inhabit the duodenum and proximal small intestine, gaining access to the biliary system via the major papilla. In fact, an opportunistic organism, usually Cryptosporidium or cytomegalovirus, can be isolated from the bile ducts in 50% of patients (,48–,51). A spectrum of biliary abnormalities can occur including papillitis, acalculous cholecystitis, and cholangitis. Cholangiographic findings include a stricture of the distal common bile duct (,Fig 22), which is a result of papillitis, or intrahepatic ductal abnormalities simulating PSC (,Fig 23) (,51).
Chemotherapy-induced Cholangitis
In patients with metastatic liver disease from colorectal cancer, hepatic arterial infusion of chemotherapeutic agents such as floxuridine is a common means of palliative treatment. However, hepatic toxic effects can develop because of a high rate of extraction on the first pass through the liver, producing an inflammatory, fibrosing process about the portal triads that simulates PSC at conventional cholangiography (,52). Bile duct strictures occur in up to 15% of treated patients as early as 2 months after therapy (,52). The mechanism of injury is either a direct effect of the treatment or ischemia secondary to thrombosis of the intrahepatic arterial branches (,53,,54). The most common cholangiographic findings are strictures of the bifurcation of the common hepatic duct with sparing of the distal common bile duct (,Fig 24) (,52). Cross-sectional imaging in conjunction with cholangiography is usually necessary to exclude worsening metastatic disease in these patients.
Ischemic Cholangitis after Liver Transplantation
Biliary tree disease, usually in the form of strictures, is the second leading cause of liver failure in transplant recipients. Bile duct strictures may occur because of iatrogenic injury, hepatic arterial thrombosis or stenosis, prolonged preservation time, recurrence of primary liver disease, chronic rejection, cholangitis, or cholangiocarcinoma.
Posttransplantation strictures can be classified into anastomotic and nonanastomotic varieties. Most anastomotic strictures are extrahepatic ductal strictures due to iatrogenic trauma with resultant ischemia and scar formation. If the diagnosis is not made, ascending cholangitis can develop with formation of intrahepatic bile duct strictures that simulate PSC.
Most nonanastomotic strictures are due to ischemia unrelated to iatrogenic bile duct injury (,,,Fig 25). Thrombosis of the hepatic artery occurs in 4%–8% of liver transplants and is the cause of up to 50% of nonanastomotic strictures (,55–,57). Because the parabiliary collateral arteries from the hepatic, gastroduodenal, celiac, and superior mesenteric arteries are absent in the transplanted liver, stenosis or occlusion of the hepatic artery, the sole arterial supply to the transplanted liver, may result in severe ischemia and bile duct or hepatocyte necrosis. Nonanastomotic bile duct strictures and leaks secondary to bile duct necrosis with disruption should suggest the diagnosis of hepatic arterial thrombosis. Doppler US is 92% sensitive for hepatic arterial thrombosis; however, low-flow states, technical errors, or aberrant anatomy may result in a false-positive diagnosis. Conventional arteriography should be performed in equivocal cases. Other causes of nonanastomotic bile duct strictures in a liver transplant, which include prolonged preservation time, bacterial or viral cholangitis, rejection, recurrent PSC, and cholangiocarcinoma, should also be excluded (,58).
Eosinophilic Cholangitis
Eosinophilic infiltration of the hepatobiliary system is usually seen in association with eosinophilic gastroenteritis and can result in radiologic and histologic features compatible with cholangitis, cholecystitis, or chronic hepatitis (,Fig 26) (,59–,63). Most patients respond rapidly to corticosteroid therapy.
Metastatic Disease
In a patient with a known malignancy, elevated results of liver function tests or jaundice may represent metastatic disease to the liver. Diffuse hepatic metastases may simulate PSC at cholangiography, and cross-sectional imaging is necessary to differentiate these entities (,,,Fig 27).
Conclusions
MRCP performed with the single-shot fast spin-echo technique is a promising, noninvasive alternative to more invasive direct cholangiography for evaluating the intrahepatic and extrahepatic bile ducts in patients with sclerosing cholangitis.
Figure 1a. Normal cholangiographic findings. (a) Coronal thick-section MRCP image shows the anteriorly located common hepatic duct and left bile ducts most clearly. Arrowhead = cystic duct. (b, c) Oblique coronal MRCP images obtained at a shallow angle (b) and a steep angle (c) show the more posteriorly located right bile ducts and distal common bile duct most clearly. Arrowhead = cystic duct, arrows = pancreatic duct.
Figure 1b. Normal cholangiographic findings. (a) Coronal thick-section MRCP image shows the anteriorly located common hepatic duct and left bile ducts most clearly. Arrowhead = cystic duct. (b, c) Oblique coronal MRCP images obtained at a shallow angle (b) and a steep angle (c) show the more posteriorly located right bile ducts and distal common bile duct most clearly. Arrowhead = cystic duct, arrows = pancreatic duct.
Figure 1c. Normal cholangiographic findings. (a) Coronal thick-section MRCP image shows the anteriorly located common hepatic duct and left bile ducts most clearly. Arrowhead = cystic duct. (b, c) Oblique coronal MRCP images obtained at a shallow angle (b) and a steep angle (c) show the more posteriorly located right bile ducts and distal common bile duct most clearly. Arrowhead = cystic duct, arrows = pancreatic duct.
Figure 2a. PSC. (2a) ERCP image shows multifocal strictures and irregularity of the right intrahepatic bile ducts. (2b) Coronal thick-section MRCP image shows multiple strictures of the right posterior branches (arrows); however, the strictures of the peripheral ducts are not demonstrated. 
Figure 2b. PSC. (2a) ERCP image shows multifocal strictures and irregularity of the right intrahepatic bile ducts. (2b) Coronal thick-section MRCP image shows multiple strictures of the right posterior branches (arrows); however, the strictures of the peripheral ducts are not demonstrated. 
Figure 3a. (3a) ERCP image shows multifocal strictures of the extrahepatic bile duct and central ducts, which produce a beaded appearance (arrows). The peripheral ducts could not be opacified. (3b) Coronal thick-section MRCP image does not show the strictures of the extrahepatic bile duct as clearly (arrows). However, the peripheral ducts are demonstrated and are seen to also have strictures.
Figure 3b. (3a) ERCP image shows multifocal strictures of the extrahepatic bile duct and central ducts, which produce a beaded appearance (arrows). The peripheral ducts could not be opacified. (3b) Coronal thick-section MRCP image does not show the strictures of the extrahepatic bile duct as clearly (arrows). However, the peripheral ducts are demonstrated and are seen to also have strictures.
Figure 4. PSC. (4) ERCP image shows several strictures of the anterior right hepatic duct alternating with areas of dilatation, thus producing a beaded appearance (arrows). More subtle intrahepatic ductal strictures are noted. 
Figure 5. Coronal thick-section MRCP image shows a high-grade hilar stricture (straight arrow) with proximal dilatation, multifocal intrahepatic strictures and dilatation, and a beaded appearance of the anterior right hepatic duct (curved arrow). In a patient with PSC who develops a focal, high-grade stricture, cholangiocarcinoma should be considered. Bile duct brushings obtained during ERCP showed no tumor in this patient.
Figure 6a. Early PSC in a 45-year-old man with ulcerative colitis and elevated results of liver function tests. (a) Coronal MRCP image shows a stricture at the distal common bile duct (arrowhead), extrahepatic and left intrahepatic ductal dilatation and irregularity, and a subtle stricture at the bifurcation of the posterior right hepatic duct (arrow). The peripheral ducts intersect with the more central ducts at acute angles. (b) Percutaneous cholangiogram shows similar findings. The stricture at the bifurcation of the posterior right hepatic duct is seen more clearly (arrow).
Figure 6b. Early PSC in a 45-year-old man with ulcerative colitis and elevated results of liver function tests. (a) Coronal MRCP image shows a stricture at the distal common bile duct (arrowhead), extrahepatic and left intrahepatic ductal dilatation and irregularity, and a subtle stricture at the bifurcation of the posterior right hepatic duct (arrow). The peripheral ducts intersect with the more central ducts at acute angles. (b) Percutaneous cholangiogram shows similar findings. The stricture at the bifurcation of the posterior right hepatic duct is seen more clearly (arrow).
Figure 7a. PSC in a 24-year-old man with fever. (a) Coronal MRCP image shows multifocal strictures and irregularity of the intrahepatic bile ducts. The left peripheral ducts intersect the central ducts almost at right angles. Note the low and medial insertion of the cystic duct (arrows). (b) ERCP image shows similar findings. 
Figure 7b. PSC in a 24-year-old man with fever. (a) Coronal MRCP image shows multifocal strictures and irregularity of the intrahepatic bile ducts. The left peripheral ducts intersect the central ducts almost at right angles. Note the low and medial insertion of the cystic duct (arrows). (b) ERCP image shows similar findings. 
Figure 8a. PSC in a 67-year-old man with ulcerative colitis and jaundice. (a) Coronal MRCP image does not show the intersegmental bile ducts, an appearance suggestive of strictures. There are random strictures and moderate dilatation of the left intrahepatic bile ducts. Note the choledochojejunostomy (arrow). (b) Percutaneous cholangiogram clearly shows the extent of the PSC because of the ductal distention produced by the contrast material injection.
Figure 8b. PSC in a 67-year-old man with ulcerative colitis and jaundice. (a) Coronal MRCP image does not show the intersegmental bile ducts, an appearance suggestive of strictures. There are random strictures and moderate dilatation of the left intrahepatic bile ducts. Note the choledochojejunostomy (arrow). (b) Percutaneous cholangiogram clearly shows the extent of the PSC because of the ductal distention produced by the contrast material injection.
Figure 9a. Advanced PSC. (9a) Coronal MRCP image shows strictures and dilatation of the left main hepatic duct; the left peripheral ducts are not seen. Multifocal strictures of the right central hepatic ducts are noted. (9b) Percutaneous transhepatic cholangiogram shows similar findings. Strictures of the central ducts prevent opacification of the peripheral ducts.
Figure 9b. Advanced PSC. (9a) Coronal MRCP image shows strictures and dilatation of the left main hepatic duct; the left peripheral ducts are not seen. Multifocal strictures of the right central hepatic ducts are noted. (9b) Percutaneous transhepatic cholangiogram shows similar findings. Strictures of the central ducts prevent opacification of the peripheral ducts.
Figure 10. Coronal MRCP image shows central ductal strictures and dilatation. At ERCP, the peripheral ducts could not be opacified.
Figure 11. Webs in a patient with PSC. ERCP image shows multiple webs of the common bile duct (arrowheads).
Figure 12. Web in a patient with PSC. Coronal MRCP image shows a web of the common hepatic duct (arrowhead).
Figure 13. Diverticulum. Coronal MRCP image shows a diverticulum of the common bile duct (arrow). 
Figure 14. Diverticula in a patient with PSC. Percutaneous transhepatic cholangiogram shows multiple tiny outpouchings emanating from the mural surface of the extrahepatic and right and left hepatic ducts. 
Figure 15a. Diverticulum in a patient with PSC. (a) Coronal MRCP image shows a diverticulum arising from the anterior right hepatic duct (arrow). The diverticulum contains a filling defect, which is compatible with a calculus. (b) Longitudinal ultrasonographic (US) scan shows the calculus surrounded by bile in the diverticulum. The diverticulum could not be filled at ERCP because of a high-grade stricture at the neck of the diverticulum (not shown).
Figure 15b. Diverticulum in a patient with PSC. (a) Coronal MRCP image shows a diverticulum arising from the anterior right hepatic duct (arrow). The diverticulum contains a filling defect, which is compatible with a calculus. (b) Longitudinal ultrasonographic (US) scan shows the calculus surrounded by bile in the diverticulum. The diverticulum could not be filled at ERCP because of a high-grade stricture at the neck of the diverticulum (not shown).
Figure 16. Stones in a patient with PSC. Coronal MRCP image shows intrahepatic and extrahepatic ductal dilatation due to a stricture at a choledochojejunostomy (bottom arrow). Note the stones in the left hepatic duct (top arrows).
Figure 17a. Cholangiocarcinoma. (a) Coronal single-shot fast spin-echo MR image (echo time, 90 msec) shows ill-defined high signal intensity at the porta hepatis and in the right periductal region (arrowheads). The common bile duct could not be identified. (b) Coronal MRCP image (echo time, 890 msec) shows an obstructing hilar stricture (arrow) with proximal ductal dilatation. (c) Axial fast spoiled gradient-echo MR image obtained 10 minutes after administration of gadolinium contrast material shows periductal enhancement (arrows). Brush cytologic biopsy during ERCP revealed cholangiocarcinoma.
Figure 17b. Cholangiocarcinoma. (a) Coronal single-shot fast spin-echo MR image (echo time, 90 msec) shows ill-defined high signal intensity at the porta hepatis and in the right periductal region (arrowheads). The common bile duct could not be identified. (b) Coronal MRCP image (echo time, 890 msec) shows an obstructing hilar stricture (arrow) with proximal ductal dilatation. (c) Axial fast spoiled gradient-echo MR image obtained 10 minutes after administration of gadolinium contrast material shows periductal enhancement (arrows). Brush cytologic biopsy during ERCP revealed cholangiocarcinoma.
Figure 17c. Cholangiocarcinoma. (a) Coronal single-shot fast spin-echo MR image (echo time, 90 msec) shows ill-defined high signal intensity at the porta hepatis and in the right periductal region (arrowheads). The common bile duct could not be identified. (b) Coronal MRCP image (echo time, 890 msec) shows an obstructing hilar stricture (arrow) with proximal ductal dilatation. (c) Axial fast spoiled gradient-echo MR image obtained 10 minutes after administration of gadolinium contrast material shows periductal enhancement (arrows). Brush cytologic biopsy during ERCP revealed cholangiocarcinoma.
Figure 18a. Cholangiocarcinoma. (a, b) Coronal (a) and axial (b) single-shot fast spin-echo MR images (echo time, 90 msec) show ill-defined high signal intensity in the extrahepatic and proximal intrahepatic periductal areas (arrows). (c-e) Axial fast spoiled gradient-echo MR images obtained 1 minute (c), 3 minutes (d), and 10 minutes (e) after administration of gadolinium contrast material show progressive abnormal enhancement of the porta hepatis (arrows). (f) Percutaneous transhepatic cholangiogram shows an irregular high-grade stenosis with shouldered margins in the common hepatic duct (arrows). Note the multifocal intrahepatic strictures and dilatations, which are compatible with PSC. Brush cytologic biopsy revealed cholangiocarcinoma.
Figure 18b. Cholangiocarcinoma. (a, b) Coronal (a) and axial (b) single-shot fast spin-echo MR images (echo time, 90 msec) show ill-defined high signal intensity in the extrahepatic and proximal intrahepatic periductal areas (arrows). (c-e) Axial fast spoiled gradient-echo MR images obtained 1 minute (c), 3 minutes (d), and 10 minutes (e) after administration of gadolinium contrast material show progressive abnormal enhancement of the porta hepatis (arrows). (f) Percutaneous transhepatic cholangiogram shows an irregular high-grade stenosis with shouldered margins in the common hepatic duct (arrows). Note the multifocal intrahepatic strictures and dilatations, which are compatible with PSC. Brush cytologic biopsy revealed cholangiocarcinoma.
Figure 18c. Cholangiocarcinoma. (a, b) Coronal (a) and axial (b) single-shot fast spin-echo MR images (echo time, 90 msec) show ill-defined high signal intensity in the extrahepatic and proximal intrahepatic periductal areas (arrows). (c-e) Axial fast spoiled gradient-echo MR images obtained 1 minute (c), 3 minutes (d), and 10 minutes (e) after administration of gadolinium contrast material show progressive abnormal enhancement of the porta hepatis (arrows). (f) Percutaneous transhepatic cholangiogram shows an irregular high-grade stenosis with shouldered margins in the common hepatic duct (arrows). Note the multifocal intrahepatic strictures and dilatations, which are compatible with PSC. Brush cytologic biopsy revealed cholangiocarcinoma.
Figure 18d. Cholangiocarcinoma. (a, b) Coronal (a) and axial (b) single-shot fast spin-echo MR images (echo time, 90 msec) show ill-defined high signal intensity in the extrahepatic and proximal intrahepatic periductal areas (arrows). (c-e) Axial fast spoiled gradient-echo MR images obtained 1 minute (c), 3 minutes (d), and 10 minutes (e) after administration of gadolinium contrast material show progressive abnormal enhancement of the porta hepatis (arrows). (f) Percutaneous transhepatic cholangiogram shows an irregular high-grade stenosis with shouldered margins in the common hepatic duct (arrows). Note the multifocal intrahepatic strictures and dilatations, which are compatible with PSC. Brush cytologic biopsy revealed cholangiocarcinoma.
Figure 18e. Cholangiocarcinoma. (a, b) Coronal (a) and axial (b) single-shot fast spin-echo MR images (echo time, 90 msec) show ill-defined high signal intensity in the extrahepatic and proximal intrahepatic periductal areas (arrows). (c-e) Axial fast spoiled gradient-echo MR images obtained 1 minute (c), 3 minutes (d), and 10 minutes (e) after administration of gadolinium contrast material show progressive abnormal enhancement of the porta hepatis (arrows). (f) Percutaneous transhepatic cholangiogram shows an irregular high-grade stenosis with shouldered margins in the common hepatic duct (arrows). Note the multifocal intrahepatic strictures and dilatations, which are compatible with PSC. Brush cytologic biopsy revealed cholangiocarcinoma.
Figure 18f. Cholangiocarcinoma. (a, b) Coronal (a) and axial (b) single-shot fast spin-echo MR images (echo time, 90 msec) show ill-defined high signal intensity in the extrahepatic and proximal intrahepatic periductal areas (arrows). (c-e) Axial fast spoiled gradient-echo MR images obtained 1 minute (c), 3 minutes (d), and 10 minutes (e) after administration of gadolinium contrast material show progressive abnormal enhancement of the porta hepatis (arrows). (f) Percutaneous transhepatic cholangiogram shows an irregular high-grade stenosis with shouldered margins in the common hepatic duct (arrows). Note the multifocal intrahepatic strictures and dilatations, which are compatible with PSC. Brush cytologic biopsy revealed cholangiocarcinoma.
Figure 19a. Cholangiocarcinoma. (a) Coronal MRCP image shows a stricture at the hepatic hilum with mild intrahepatic ductal dilatation, nonvisualization of the right hepatic duct, and nodularity of the left hepatic duct. The increased signal intensity in the periductal areas (arrows) suggests cholangiocarcinoma or periportal edema. (b) Gadolinium-enhanced axial fat-saturated T1-weighted spin-echo MR image shows enhancement of the periductal areas (arrows), a finding compatible with a tumor.
Figure 19b. Cholangiocarcinoma. (a) Coronal MRCP image shows a stricture at the hepatic hilum with mild intrahepatic ductal dilatation, nonvisualization of the right hepatic duct, and nodularity of the left hepatic duct. The increased signal intensity in the periductal areas (arrows) suggests cholangiocarcinoma or periportal edema. (b) Gadolinium-enhanced axial fat-saturated T1-weighted spin-echo MR image shows enhancement of the periductal areas (arrows), a finding compatible with a tumor.
Figure 20. Ascending cholangitis in a 50-year-old man with fever and jaundice 6 months after cholecystectomy. ERCP image shows a common bile duct stricture (arrow), a result of iatrogenic injury at surgery, and multifocal intrahepatic bile duct strictures and dilatation.
Figure 21a. Oriental cholangiohepatitis. (a, b) Coronal thick-section (a) and maximum-intensity projection (b) MRCP images show a stricture of the extrahepatic bile duct (arrow) and multiple strictures of the central intrahepatic bile ducts, with fusiform dilatation of the peripheral ducts forming bile lakes (cholangiectasis). (c) Percutaneous transhepatic cholangiogram partially shows the peripheral ductal abnormalities. Arrow = extrahepatic bile duct stricture. Analysis of the bile showed C sinensis, and brush cytologic biopsy of the extrahepatic bile duct stricture revealed cholangiocarcinoma.
Figure 21b. Oriental cholangiohepatitis. (a, b) Coronal thick-section (a) and maximum-intensity projection (b) MRCP images show a stricture of the extrahepatic bile duct (arrow) and multiple strictures of the central intrahepatic bile ducts, with fusiform dilatation of the peripheral ducts forming bile lakes (cholangiectasis). (c) Percutaneous transhepatic cholangiogram partially shows the peripheral ductal abnormalities. Arrow = extrahepatic bile duct stricture. Analysis of the bile showed C sinensis, and brush cytologic biopsy of the extrahepatic bile duct stricture revealed cholangiocarcinoma.
Figure 21c. Oriental cholangiohepatitis. (a, b) Coronal thick-section (a) and maximum-intensity projection (b) MRCP images show a stricture of the extrahepatic bile duct (arrow) and multiple strictures of the central intrahepatic bile ducts, with fusiform dilatation of the peripheral ducts forming bile lakes (cholangiectasis). (c) Percutaneous transhepatic cholangiogram partially shows the peripheral ductal abnormalities. Arrow = extrahepatic bile duct stricture. Analysis of the bile showed C sinensis, and brush cytologic biopsy of the extrahepatic bile duct stricture revealed cholangiocarcinoma.
Figure 22. AIDS-related cholangitis in a 25-year-old man with human immunodeficiency virus infection and obstructive jaundice. Coronal MRCP image shows an obstructing stricture of the distal common bile duct (arrow) with proximal ductal dilatation. Cryptosporidium was isolated from the bile ducts. 
Figure 23. AIDS-related cholangitis in a patient with human immunodeficiency virus infection. ERCP image shows strictures and irregularity of the intrahepatic bile ducts. Both cytomegalovirus and Cryptosporidium were found in the bile.
Figure 24. Floxuridine-induced cholangitis in a 64-year-old woman with jaundice who was treated with intraarterial floxuridine for metastatic colon cancer. Coronal MRCP image shows common hepatic duct and hilar strictures with proximal ductal dilatation. CT showed no evidence of hepatic metastases or hilar lymphadenopathy.
Figure 25a. Ischemic cholangitis in a 38-year-old man who developed jaundice 5 days after liver transplantation for PSC. (a) Coronal MRCP image shows random strictures throughout the biliary tree with minimal multifocal intrahepatic ductal dilatation. (b) T-tube cholangiogram shows similar findings. Doppler US showed a tardus-parvus waveform suggestive of a proximal arterial stenosis (not shown), and a hepatic arteriogram showed a high-grade stenosis of the hepatic artery (not shown).
Figure 25b. Ischemic cholangitis in a 38-year-old man who developed jaundice 5 days after liver transplantation for PSC. (a) Coronal MRCP image shows random strictures throughout the biliary tree with minimal multifocal intrahepatic ductal dilatation. (b) T-tube cholangiogram shows similar findings. Doppler US showed a tardus-parvus waveform suggestive of a proximal arterial stenosis (not shown), and a hepatic arteriogram showed a high-grade stenosis of the hepatic artery (not shown).
Figure 26. Eosinophilic cholangitis in a 42-year-old woman with jaundice and eosinophilia. Coronal MRCP image shows subtle intrahepatic ductal irregularity. Note the peripheral ducts intersecting at right angles, which suggest an inflammatory process. The symptoms rapidly resolved after corticosteroid therapy.
Figure 27a. Hepatic metastases in a 65-year-old man with gastric cancer who presented with jaundice. (a) ERCP image shows multiple intrahepatic and extrahepatic ductal strictures, an appearance that mimics PSC. (b) CT scan shows multiple hepatic metastases and lymphadenopathy of the porta hepatis and portacaval region.
Figure 27b. Hepatic metastases in a 65-year-old man with gastric cancer who presented with jaundice. (a) ERCP image shows multiple intrahepatic and extrahepatic ductal strictures, an appearance that mimics PSC. (b) CT scan shows multiple hepatic metastases and lymphadenopathy of the porta hepatis and portacaval region.
Abbreviations: AIDS = acquired immunodeficiency syndrome, ERCP = endoscopic retrograde cholangiopancreatography, MRCP = MR cholangiopancreatography, PSC = primary sclerosing cholangitis See also the article by Vitellas et al (
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