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

Stones within both intrahepatic and extrahepatic ducts

– Hyperdense on CT and low in signal on T2WI MR (with variable T1 signal)

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

Surgical resection of involved liver segments or liver transplant are also options

(Left) Graphic demonstrates marked dilation of the intrahepatic and extrahepatic bile ducts with multiple common bile duct and intrahepatic stones.

(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 . This patient later underwent surgery with a choledochoenterostomy.

(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 . Notice the multiple low signal stones within the left hepatic ducts.

(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 . 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

Intrahepatic and extrahepatic biliary dilatation with multiple biliary calculi

• Location

May be confined to left lobe (often lateral segment) or less commonly, right posterior segment

May involve all biliary ductal segments (and common bile duct) diffusely in later stages of disease

• Size

Stones typically 1-4 cm

• Morphology

Combination of pigment stones and biliary sludge

Fluoroscopic Findings

• ERCP

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

Common duct and intrahepatic duct stones

Nonfilling of biliary ductal segments due to strictures of intrahepatic ducts (missing duct sign)

Vigorous injection of contrast during ERCP with overdistension of biliary tree may cause sepsis

CT Findings

• Dilatation of intrahepatic and extrahepatic biliary ducts

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

Ductal wall hyperenhancement and heterogeneous liver enhancement during acute cholangitis exacerbation

Pneumobilia (may not be related to prior intervention)

• Hepatolithiasis and choledocholithiasis

∼ 90% of stones are hyperdense to liver

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

Atrophy most often affects left lateral segment of liver

– Atrophic segments may be steatotic with heterogeneous enhancement

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

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)

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