Recurrent Pyogenic Cholangitis

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 Dilatation of intrahepatic and extrahepatic biliary ducts

– Disproportionate dilatation of extrahepatic and central intrahepatic bile ducts
– Localized intrahepatic biliary ductal dilatation most commonly affects left hepatic lobe
– MRCP nicely illustrates ↓ arborization of peripheral ducts, multiple intrahepatic biliary strictures, and rapidly tapering intrahepatic ducts (arrowhead sign)
– Thickening and hyperenhancement of bile duct walls in setting of acute cholangitis
image Stones within both intrahepatic and extrahepatic ducts

– Hyperdense on CT and low in signal on T2WI MR (with variable T1 signal)
image Lobar or segmental liver parenchymal atrophy associated with sites of greatest biliary dilatation

PATHOLOGY

• Etiology uncertain, but likely related to parasitic (Ascaris, Clonorchis) or bacterial infection of biliary tree

CLINICAL ISSUES

• Almost exclusively occurs in inhabitants of or immigrants from Southeast Asia
• Patients present with Charcot triad (RUQ pain, fever, and jaundice) during bouts of acute cholangitis
• Leukocytosis, ↑ alkaline phosphatase, ↑ bilirubin
• Complications: Liver abscesses, secondary biliary cirrhosis due to chronic infections, and cholangiocarcinoma
• Treatment: Combination of biliary drainage, stone retrieval, treatment of strictures, and antibiotics

image Surgical resection of involved liver segments or liver transplant are also options
image
(Left) Graphic demonstrates marked dilation of the intrahepatic and extrahepatic bile ducts with multiple common bile duct and intrahepatic stones.

image
(Right) ERCP in a patient with recurrent pyogenic cholangitis (RPC) demonstrates massive dilation of the bile ducts, abnormal arborization of the intrahepatic ducts with a pruned appearance, and innumerable intraductal calculi image. This patient later underwent surgery with a choledochoenterostomy.
image
(Left) Coronal MIP MRCP image in a patient from Southeast Asia demonstrates dilatation of the entire biliary system, with focal severe dilatation of the left hepatic lobe ducts image. Notice the multiple low signal stones image within the left hepatic ducts.

image
(Right) Axial T2 FS MR in the same patient demonstrates the localized dilatation of the left hepatic lobe ducts with multiple low T2 signal stones image. Note that the left lobe is mildly atrophic with subtly increased T2 signal. The findings in this case are classic for RPC.

TERMINOLOGY

Abbreviations

• Recurrent pyogenic cholangitis (RPC)

Synonyms

• Oriental cholangitis, oriental cholangiohepatitis, intrahepatic pigment stone disease
• Biliary obstruction syndrome of Chinese/Hong Kong disease

Definitions

• Disease characterized by formation of pigment stones throughout biliary tree, with resultant biliary strictures and repeated bouts of cholangitis

IMAGING

General Features

• Best diagnostic clue

image Intrahepatic and extrahepatic biliary dilatation with multiple biliary calculi
• Location

image May be confined to left lobe (often lateral segment) or less commonly, right posterior segment
image May involve all biliary ductal segments (and common bile duct) diffusely in later stages of disease
• Size

image Stones typically 1-4 cm
• Morphology

image Combination of pigment stones and biliary sludge

Fluoroscopic Findings

• ERCP

image Dilated intrahepatic and extrahepatic bile ducts with disproportionate dilatation of extrahepatic duct

– Rapid tapering of dilated intrahepatic ducts with “arrowhead” configuration, ↓ arborization of peripheral ducts, and short segment bile duct strictures
image Common duct and intrahepatic duct stones
image Nonfilling of biliary ductal segments due to strictures of intrahepatic ducts (missing duct sign)
image Vigorous injection of contrast during ERCP with overdistension of biliary tree may cause sepsis

CT Findings

• Dilatation of intrahepatic and extrahepatic biliary ducts

image Disproportionate dilatation of extrahepatic and central intrahepatic bile ducts: CBD may be markedly enlarged

– ↓ arborization of peripheral ducts and multiple biliary strictures
– Preferential localized dilatation of bile ducts in left hepatic lobe and right posterior hepatic lobe
image Ductal wall hyperenhancement and heterogeneous liver enhancement during acute cholangitis exacerbation
image Pneumobilia (may not be related to prior intervention)
• Hepatolithiasis and choledocholithiasis

image ∼ 90% of stones are hyperdense to liver
image Distribution of dilated ducts unrelated to location of calculi
• May be associated with pyogenic liver abscesses, bilomas, steatosis, segmental atrophy with chronic biliary obstruction, and cholangiocarcinoma

image Atrophy most often affects left lateral segment of liver

– Atrophic segments may be steatotic with heterogeneous enhancement
image Over time, scarring, atrophy, and capsular retraction of liver can produce cirrhotic morphology of liver

MR Findings

• Dilatation of intrahepatic and extrahepatic bile ducts, with disproportionate dilatation of extrahepatic and central intrahepatic ducts

image MRCP nicely illustrates ↓ arborization of peripheral ducts, multiple intrahepatic biliary structures, and rapidly tapering intrahepatic ducts (arrowhead sign)

– Excellent sensitivity for detection of ductal dilatation, strictures, and calculi
– Strictures tend to encompass short segments (< 1 cm) and are easier to see on MRCP compared to CT
– Has ability to show ducts proximal to obstruction (advantage over ERCP)
image Localized intrahepatic biliary ductal dilatation (± biliary stones) most commonly affects left liver lobe, and less commonly, posterior segment of right hepatic lobe

– May produce diffuse ductal dilatation in severe cases due to repeated bouts of inflammation
image May be associated with thickening and hyperenhancement of bile duct walls on T1WI C+ images in setting of acute cholangitis
• Stones with low T2WI signal within both intrahepatic and extrahepatic ducts

image Stones are primarily pigmented stones, and may be diffusely hyperintense or peripherally hyperintense (centrally hypointense) on T1WI
image Sites of dilated bile ducts may not be closely related to the sites of bile duct stones
• Lobar or segmental liver parenchymal atrophy associated with sites of greatest biliary dilatation

image Atrophic liver may be hypo-, iso-, or hyperintense on T1WI and mildly hyperintense on T2WI to normal liver

Ultrasonographic Findings

• Disproportionate dilatation of extrahepatic and central intrahepatic bile ducts
• Hepatolithiasis is seen in ∼ 90% of cases 

image Marked variability in echogenicity and acoustic shadowing of calculi
• Prominent periportal echogenicity
• Focal hypoechoic parenchymal lesions could represent liver abscess, biloma, or cholangiocarcinoma

Nuclear Medicine Findings

• WBC scan

image Positive for cholangitic liver abscesses

Other Modality Findings

• Cholangiography

image Similar to ERCP
image Dilated intra- and extrahepatic ducts with filling defects (stones)
image “Arrowhead” deformity of rapidly tapering intrahepatic ducts

Imaging Recommendations

• Best imaging tool

image MRCP
• Protocol advice

image  

DIFFERENTIAL DIAGNOSIS

Intrahepatic Stones Secondary to Biliary Stricture

• Non-Asian patient without risk factors for RPC
• Strictures due to prior surgery, trauma, or chemotherapy
• Similar clinical presentation to RPC in setting of cholangitis: RUQ pain, fever, chills

Primary Sclerosing Cholangitis

• Multifocal intrahepatic strictures ± strictures of extrahepatic duct with less than expected biliary dilatation

image Thickening and hyperenhancement of CBD and central ducts due to acute inflammation
• Hepatolithiasis can occur proximal to strictures
• Associated with inflammatory bowel disease

Ascending Cholangitis

• Dilated intrahepatic and extrahepatic ducts, most often as result of obstructing stone

image Associated with thickening and hyperenhancement of the bile ducts with heterogeneous liver enhancement
• Stones, sludge, and infectious debris in bile ducts: Hepatolithiasis uncommon, but rarely occurs

Caroli Disease

• Cystic dilatation of right and left intrahepatic ducts with normal caliber extrahepatic bile duct

image More commonly segmental involvement of liver rather than diffuse involvement
• Central dot sign: Portal vein surrounded by bile duct
• May be associated with intrahepatic stones and cholangitis, but less commonly than RPC

Cholangiocarcinoma

• Increased incidence with sclerosing cholangitis, choledochal cysts, recurrent pyogenic cholangitis, clonorchiasis
• Should be suspected in setting of segmental biliary dilatation and segmental liver atrophy
• Hepatolithiasis due to chronic ductal dilatation and stasis

PATHOLOGY

General Features

• Etiology

image Precise etiology is not completely understood, but likely related to parasitic or bacterial infection of biliary tree
image Different theories based on geographic clustering of disease (suggestive of role of epidemiologic factors)

– Parasitic infection

image Parasitic infestation/infection → epithelial damage → fibrosis → stricture → bile stasis → stone formation
image Liver flukes (Clonorchis sinensis, Opisthorchis species, Fasciola hepatica) and Ascaris lumbricoides
– Bacterial infection

image Transient portal bacteremia (from lower GI source) → bacterial infection of biliary tree
image Bacterial production of glucuronidases → hydrolysis of direct bilirubin to unconjugated bilirubin → formation of insoluble calcium bilirubinate
image Lack of host inhibitor of bacterial glucuronidases may play role
image Common cultured bacterial organisms: Escherichia coli, Klebsiella, Pseudomonas, Proteus
image Associated with poor general nutrition
• Associated abnormalities

image Pigment bilirubinate calculi within intra- and extrahepatic ducts, proliferative fibrosis of CBD walls, periductal abscesses
image Secondary biliary cirrhosis and portal hypertension
• Pathogenesis

image Incompletely understood
image De novo stone formation → biliary stricture/obstruction → bile stasis → recurrent cholangitis

Staging, Grading, & Classification

• Classification based on distribution of affected biliary segment

image Isolated to left lobe, particularly lateral segment
image Involving all biliary segments and CBD

Gross Pathologic & Surgical Features

• Dilated bile ducts with brown, mud-like pigment stones and pus
• May have parasitic infection in biliary ducts (liver flukes or Ascaris)

Microscopic Features

• Periductal inflammatory changes
• Infiltration of periportal spaces with inflammatory cells

image Periductal fibrosis, biliary cirrhosis
• Localized segmental hepatic atrophy
• Fatty infiltration of liver

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Charcot triad (RUQ pain, fever ± rigors, and jaundice) during bouts of acute cholangitis
• Other signs/symptoms

image Recurrent abdominal pain without overt cholangitis
image Hypotension, shaking chills
image Pancreatitis due to passage of biliary stones
• Clinical profile

image Southeast Asian patient presenting with fever, RUQ pain, and ↑ WBC
• Lab data

image Leukocytosis, ↑ alkaline phosphatase, ↑ bilirubin
image All patients should be checked for stool ova and parasites
• Diagnosis based on clinical history and imaging findings

Demographics

• Age

image Peak incidence in 3rd and 4th decades of life
• Gender

image M = F
• Ethnicity

image Almost exclusively in inhabitants of or immigrants from Southeast Asia
• Epidemiology

image Endemic in Southeast Asia

– Steady decline in incidence in Asia due to improved standards of living and westernized diet
– Increased incidence in North America as result of migration from endemic regions

Natural History & Prognosis

• Repeated episodes of cholangitis
• Complications

image Liver abscesses are common, with rare development of abscesses in distant sites (lungs, brain)
image Biloma formation: Due to obstruction and severe dilatation of bile duct with leakage of bile
image Portal vein thrombosis: May be secondary to either biliary cirrhosis or periportal inflammation
image Formation of fistulous tract between biliary tree and peritoneum or intestine
image Secondary biliary cirrhosis ± portal hypertension
image Cholangiocarcinoma: Suspect in patient with clinical deterioration and unexplained ↑ in alkaline phosphatase

– Chronic proliferative cholangitis → atypical epithelial hyperplasia → cholangiocarcinoma
– May be hilar or peripheral low-attenuation mass
– Most commonly occurs within segments with severe atrophy or severe stone burden
– Consider if adjacent portal vein narrowed/obliterated
image Inflammatory pseudotumor

– Fibrous stroma and chronic inflammatory infiltrate
– Due to chronic infection, calculi associated bile stasis, biliary degeneration, and necrosis
– Ill-defined, low-attenuation mass on CECT
– Mimics cholangiocarcinoma or abscess
• 10% disease-related mortality; 7% biliary cirrhosis; 3% cholangiocarcinoma

Treatment

• Multidisciplinary approach

image Gastroenterology

– Extrahepatic biliary drainage, stone retrieval, and stent placement
image Interventional radiology

– Percutaneous biliary drainage of more peripherally affected segments
– Basket removal of pigment stones
– Balloon dilation of biliary strictures
– Repeated procedures to clear stones and debris
image Surgery

– Biliary drainage with hepaticojejunostomy
– Subcutaneous jejunostomy (biliary access)
– Segmental/lobar liver resection if isolated disease
– Liver transplant in most severe cases
image Medical therapy

– Antibiotic therapy during bouts of acute cholangitis
– Equivocal role of ursodeoxycholic acid to ↓ risk of stone recurrence

DIAGNOSTIC CHECKLIST

Consider

• RPC in patient from Southeast Asia with diffuse or segmental biliary dilatation, multiple biliary stones, and symptoms of cholangitis

Image Interpretation Pearls

• Intra- and extrahepatic bile duct dilatation/stones in Southeast Asian patients
• Massive CBD dilatation
• Rapid tapering of intrahepatic ducts (“arrowhead” sign)
image
(Left) Power Doppler ultrasound shows dilated intrahepatic ducts with intraluminal echogenic calculi image. Note the posterior acoustic shadowing image. In a patient from Southeast Asia, these imaging findings are characteristic of RPC.

image
(Right) Axial T2 FS MR demonstrates localized dilatation of the right posterior segmental bile ducts image with multiple low-signal internal stones. Localized involvement of the right posterior ducts is less common in RPC than localized involvement of the left lobe ducts.
image
(Left) Axial NECT in a recent immigrant from Vietnam presenting with RUQ pain, fever, and chills shows high-attenuation intrahepatic pigment stones image within dilated left lobe bile ducts, and gas image within a hepatic abscess in the right hepatic lobe.

image
(Right) Axial CECT in the same patient shows low-attenuation, branching, dilated bile ducts in the left hepatic lobe containing intrahepatic stones image, and a stone in the right posterior bile duct image.
image
(Left) Axial CECT in a patient with RPC presenting with fever and RUQ pain shows extensive calculi within dilated right and left bile ducts image. Note the absence of peripheral biliary ductal dilatation.

image
(Right) Axial CECT in a patient with RPC shows localized severe dilatation of the left intrahepatic ducts image, with an adjacent heterogeneous, solid mass image within the left lobe that proved to represent a cholangiocarcinoma. RPC is a known risk factor for the development of cholangiocarcinoma.
image
Axial T2WI FSE MR of a 55-year-old Chinese man with right upper quadrant pain and a fever shows dilated right ducts image, a right biliary calculus image, peripheral atrophy, and increased parenchymal T2 signal image, characteristic of RPC.

image
Axial CECT in a 57-year-old woman immigrant from China presenting with RUQ pain, fever, and gram-negative sepsis shows (in the porta hepatis) massive dilatation of the common bile duct, with a large pigment stone image adjacent to a small crescent of low-attenuation bile image.
image
Axial CECT in the same patient illustrates the massive biliary dilatation and large common bile duct stone image. Note that there are no stones in the normal-appearing gallbladder image.
image
Frontal ERCP shows ductal dilation and pneumobilia. Note the presence of large, “soft” stones within the bile duct image, later cleared by a basket sweep of the duct.
image
Axial CECT of recurrent pyogenic cholangitis demonstrates marked intrahepatic biliary dilatation with numerous intrahepatic stones image.
image
Frontal ERCP of the common bile duct in a patient with recurrent pyogenic cholangitis demonstrates a massive filling defect due to stones image.
image
Coronal CECT reformation of recurrent pyogenic cholangitis demonstrates a lack of stones in the gallbladder, but numerous intrahepatic stones image.
image
Coronal MRCP shows marked proximal right biliary ductal dilatation image and multiple large stones within a massively dilated extrahepatic duct image.
image
Axial CECT in an Asian patient with RPC demonstrates predominantly intrahepatic bile duct dilatation image involving the left ductal system, and a filling defect due to pigment calculus image. As the calculi are predominantly composed of pigment, they are not as dense as calculi containing calcium.
image
Axial CECT in the same patient demonstrates a focal abscess image seen in the lateral segment of the left lobe. Abscesses are a common complication in patients with RPC.
image
Coronal CECT in an Asian immigrant shows gross dilation of the intrahepatic and extrahepatic bile ducts.
image
Coronal CECT in the same patient demonstrates multiple large calculi present within the left intrahepatic ducts image, which are dilated out of proportion to the right lobe ducts. The CT findings in this case are classic for recurrent pyogenic cholangitis, consisting of chronic infection and stone formation within the biliary system, usually without gallbladder stones.

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