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Abdomen » Hepatobiliary
Mirizzi syndrome with dilatation of the common hepatic duct and intrahepatic bile ducts caused by external compression of a stone in the cystic duct
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Author(s) :
Taco Geertsma, MD
 
Presentation 68 year old female with painless jaundice
 
 
 
Caption: Long axis view of the gallbladder and common hepatic duct
Description: There is a small gallbladder with stones.There is a stone in the cystic duct. The common bile duct is slightly dilatated
 
 
 
Caption:  Long axis view of the gallbladder and common hepatic duct
Description: The stones in the gallbladder cause acoustic shadowing. The stone in the cystic duct protrudes in the common hepatic duct, reducing the lumen more than 50%
 
 
 
Caption:  Image of the liver hilum
Description: The arrow points to the large stone protruding in the bile duct
 
 
 
Caption:  Image of the liver hilum at a 90 degree angle to image 3
Description: The arrow points to the stone in the cystic duct that measures 1,2 cm.The intrahepatic bile ducts are slightly dilatated
 
 
 
Caption: Image of the liver
Description: The intrahepatic bile ducts are dilatated
 
 
 
Caption: ERCP
Description: The arrow points to the severe stenosis of the common bile duct caused by external compression of the stone in the cystic duct. The ERCP image corresponds well with the ultrasound image 2
 
Final Diagnosis Mirizzi syndrome with dilatation of the common hepatic duct and intrahepatic bile ducts caused by external compression of a stone in the cystic duct
 
Discussion Mirizzi syndrome is a rare complication of gallstones and occurs in approximately 0.1% of all patients with gallstone disease. Its main clinical symptom is jaundice. Accompanying features can be fever and right upper quadrant pain. Mirizzi syndrome tends to affect older patients

It is named after Pablo Mirizzi, an Argentinean physician

The jaundice is caused by an obstruction of the common hepatic duct. The obstruction is caused by a stone accompanied by inflammation or scarring. The swelling or scarring is caused by one or more stones that are chronically lodged in the cystic duct or Hartmann's pouch (out-pouching of the wall of the gallbladder at the junction of the neck of the gallbladder and the cystic duct) These stones cause inflammation, necrosis, scarring and sometimes fistula formation into the adjacent common hepatic duct. Mirizzi syndrome can be divided into several types depending on the presence or absence of fistula and the size of the fistula.

The diagnostic features on ultrasound are dilatation of the intra and extrahepatic bile ducts to the origin of the cystic duct or the neck of the gallbladder, were a gallstone can be found. It must be differentiated from a bile duct stone. In a case of a bile duct stone, the stone is completely intraluminal. In a case of a Mirizzi syndrome the stone usually only protrudes partially in the duct, or the swelling around the stone is seen impressing the bile duct.

On ERCP the bile duct stone is seen as an intraluminal filling defect and can be removed endoscopically. In a case of a Mirizzi syndrome there is usually an eccentric filling defect caused by the protruding stone and or the inflammation and stenosis. The stone can not be removed endoscopically.

The treatment of choice is surgical excision of the gallbladder, and reconstruction of the common hepatic duct and common bile duct.

Often a stent is placed endoscopically first to alleviate the jaundice and to facilitate the surgical treatment

For more examples of Mirizzi sybdrome see www.ultrasoundcases.info
 
Case References

Kelly MD.Acute mirizzi syndrome.JSLS. 2009 Jan-Mar;13(1):104-9.

Freeman ME, Rose JL, Forsmark CE, Vauthey J. Mirizzi syndrome: A rare cause of obstructive jaundice.Dig Dis. 1999;17(1):44-8. Review.


 
Follow Up Because the stone could not be removed by ERCP a stent was placed in the bile ducts. Because of severe pulmonary problems the treatment will probably remain conservative
 
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