Recurrent Pyogenic Cholangitis

Published on 19/07/2015 by admin

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Last modified 19/07/2015

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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

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