Mirizzi Syndrome

Published on 19/07/2015 by admin

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Last modified 22/04/2025

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 Impression on CHD is often concave to the right

image Cholecystocholedochal fistula: Contrast directly passes from biliary tree into GB
• MR: Dilated CHD and intrahepatic ducts with stricture at level of stone (signal void on all pulse sequences)

image Site of CHD narrowing may appear thickened and hyperenhancing due to inflammation on T1WI C+
image GB often thickened and inflamed due to concomitant cholecystitis
• US: Large, immobile gallstone impacted in cystic duct or infundibulum with dilated intrahepatic ducts
• CT: May be difficult to recognize stones

TOP DIFFERENTIAL DIAGNOSES

• Choledocholithiasis
• Cholangiocarcinoma, GB carcinoma, or regional lymphadenopathy
• Benign biliary stricture

PATHOLOGY

• Impaction of stone in cystic duct, infundibulum, or Hartmann pouch compressing bile duct at same level

image Obstruction may be due to direct mass effect or development of stricture in CHD due to inflammation
• Predisposing factors: Long cystic duct running parallel to CHD or low insertion of cystic duct into common bile duct
• Cholecystocholedochal fistula may develop due to chronic inflammation/pressure necrosis, with gallstones eroding from cystic duct into bile duct

CLINICAL ISSUES

• Common symptoms: Fever, jaundice, RUQ pain (symptoms of obstructive jaundice, acute cholecystitis, cholangitis)
• Definitive treatment is surgical, with approach determined by type of Mirizzi syndrome
image
(Left) Graphic of Mirizzi syndrome depicts a large cystic duct stone image causing extrinsic compression of the common hepatic duct (CHD) and dilation of the intrahepatic bile ducts image.

image
(Right) Coronal T2WI MR shows subtle intrahepatic bile duct dilation image and a large gallstone image impacted within the neck of the gallbladder (GB), causing extrinsic narrowing of the common duct.
image
(Left) Coronal CECT shows a cystic duct stone image that causes extrinsic compression of the CHD image. The intrahepatic ducts were dilated, which was better seen on axial sections (not shown).

image
(Right) Coronal CECT demonstrates a large gallstone image in the GB neck compressing the adjacent CHD image, resulting in mild intrahepatic biliary dilatation (not shown). Note the dilated GB with multiple stones image and mild wall thickening.

TERMINOLOGY

Definitions

• Partial or complete obstruction of common hepatic duct (CHD) due to gallstone impaction in cystic duct, infundibulum, or Hartmann pouch of gallbladder (GB)

IMAGING

General Features

• Best diagnostic clue

image Impacted cystic duct stone causing extrinsic mass effect on CHD with resultant dilatation of intrahepatic ducts
• Morphology

image Smooth extrinsic narrowing of CHD

Radiographic Findings

• ERCP

image Extrinsic narrowing of CHD, dilated intrahepatic ducts, and lack of GB opacification

– Extrinsic impression on CHD is often concave to the right due to typical orientation of cystic duct relative to CHD
image Probably best modality for identifying cholecystocholedochal fistula, with contrast seen to directly pass from dilated biliary tree into GB
image Accuracy of ERCP for Mirizzi syndrome: 55-90%

MR Findings

• MRCP or T2WI can nicely demonstrate gallstones, with ≥ 1 stone impacted in GB neck or cystic duct

image Gallstones usually appear as signal voids on all pulse sequences
image Dilated CHD proximal to level of stone, discrete stricture or narrowing at level of stone, and more distal bile duct appearing decompressed
• Site of stricture or narrowing may appear thickened and hyperenhancing due to inflammation on T1WI C+

image May be mistaken for tumor (such as cholangiocarcinoma)
• GB often thickened and inflamed due to concomitant cholecystitis
• MRCP can also demonstrate anatomic variants that might predispose to Mirizzi syndrome, including low insertion of cystic duct and long cystic duct running parallel to CHD

Ultrasonographic Findings

• Large, immobile gallstone impacted in cystic duct or infundibulum with dilated intrahepatic ducts proximal to level of stone
• Most patients have multiple other stones in GB
• Findings of cholecystitis frequent (GB wall thickening, wall hyperemia on color Doppler, pericholecystic fluid, positive sonographic Murphy sign)

image GB may be contracted and thickened due to chronic cholecystitis

CT Findings

• Imaging findings similar to MR or US, with large stone in cystic duct, focal narrowing of CHD at level of stone, and biliary dilatation upstream from level of stone

image More difficult to recognize stones on CT than on MR or US, and may be difficult in some cases to distinguish stone from soft tissue mass or tumor
image If stone is recognized, multiplanar reformats are essential for properly identifying location of stone (within cystic duct rather than CHD) and appreciating mass effect of stone on adjacent common duct
• CT may be helpful for identifying other findings that may suggest tumor, including liver metastases, soft tissue mass in porta hepatis, etc.

Nuclear Medicine Findings

• Hepatobiliary scan (HIDA): Nonfilling GB, dilated intrahepatic ducts and CHD, and delayed transit of radiotracer into duodenum

Imaging Recommendations

• Best imaging tool

image US, MRCP, ERCP
• Protocol advice

image Add MRCP sequences to depict stones and better delineate ductal anatomy, location of stone, and mass effect on CHD

DIFFERENTIAL DIAGNOSIS

Choledocholithiasis

• Small common duct calculi causing obstruction may not be visualized on US or CT but should be visualized on MRCP 

image Localizing a stone correctly (i.e., cystic duct vs. CHD) may be difficult on CT
• Level of obstruction usually distal to cystic duct (most commonly at distal common bile duct [CBD])

Cholangiocarcinoma

• Small mass in bile duct or focal bile duct wall thickening and hyperenhancement with abrupt irregular narrowing of lumen and proximal biliary dilatation
• Delayed enhancement of tumor on CECT or MR
• Reactive inflammatory wall thickening and enhancement of common duct at level of obstruction in Mirizzi syndrome can rarely be mistaken for cholangiocarcinoma

Gallbladder Carcinoma

• May compress or invade common duct resulting in upstream biliary dilatation
• Cross-sectional imaging shows mass arising from GB with local invasion (rather than obstruction of CHD by stone)
• Association between GB carcinoma and Mirizzi syndrome reported

Benign Biliary Stricture

• Often the sequela of prior cholangitis, choledocholithiasis, or prior intervention
• Level of narrowing most often distal to cystic duct, and no evidence of cystic duct stone obstructing common duct

CHD Obstruction Due to Enlarged Regional Nodes

• Porta hepatis nodes compress CHD
• Distinction with Mirizzi syndrome is usually easily made on MR or CECT

PATHOLOGY

General Features

• Etiology

image Impaction of stone in cystic duct, infundibulum, or Hartmann pouch compressing bile duct at same level

– Hartmann pouch: Bulging of inferior surface of infundibulum
– Portion of cystic duct and CHD run parallel to each other within common sheath
image Obstruction caused by either direct mass effect (extrinsic compression or erosion into bile duct) or secondary development of stricture in CHD due to inflammation from stone 

– May be a single large stone or multiple small stones
– Cholecystocholedochal fistula develops due to chronic inflammation and pressure necrosis, with gallstones eroding from cystic duct into bile duct
– Predisposing factors include long cystic duct running parallel to CHD, tortuous cystic duct, or low insertion of cystic duct into CBD
• Associated abnormalities

image Acute or chronic cholecystitis

– GB often appears atrophic and thickened due to multiple repetitive bouts of acute cholecystitis
image May have cholecystocholedochal fistula
image Reported association with GB carcinoma

– GB cancer may be identified in up to 25% of cases of Mirizzi syndrome
– Some authors recommend intraoperative frozen section of GB

Staging, Grading, & Classification

• Csendes classification (1989)

image Type I (∼ 60% of cases): Extrinsic compression of CHD due to cystic duct stone
image Type II (∼ 25% of cases): Cholecystocholedochal fistula involving < 1/3 circumference of ductal wall
image Type III (∼ 13% of cases): Cholecystocholedochal fistula involving 1/3-2/3 circumference of ductal wall
image Type IV (∼ 3% of cases): Cholecystocholedochal fistula with involvement of entire ductal wall circumference
image Type V was added in 2007: Any Mirizzi type (I-IV) with development of cholecystoenteric fistula
• McSherry classification (1982)

image Type I: Extrinsic compression and obstruction of CHD by stone in cystic duct or Hartmann pouch
image Type II: Presence of cholecystocholedochal fistula

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Fever, jaundice, right upper quadrant (RUQ) pain

– May present initially with symptoms of obstructive jaundice or acute cholecystitis, cholangitis, or pancreatitis
– Symptoms may be fulminant or episodic
image Most patients have longstanding history of gallstone disease
image Very difficult diagnosis based on clinical symptoms, and diagnostic accuracy in pre-ERCP era was as low as 8%

– Over 50% of cases are diagnosed at surgery
• Lab findings: ↑ alkaline phosphatase, ↑ bilirubin

image CA19-9 may be elevated in Mirizzi syndrome even in absence of malignancy

Demographics

• Age

image > 40 years (usually 5th-7th decades of life)
• Gender

image Female predominance (M:F = 1:2)
• Epidemiology

image Reflects prevalence of gallstones
image Seen in 0.7-1.8% of cholecystectomies
image May have higher incidence in populations with increased rates of cholelithiasis (including 2.7% in Native Americans)

Natural History & Prognosis

• Jaundice and cholangitis, if not treated promptly

Treatment

• Stent placement during ERCP may be temporary option prior to surgery but is not definitive therapy

image May obviate need for CBD exploration at surgery
• Definitive treatment is surgical, with approach determined by type of Mirizzi syndrome

image Type I: Partial or total cholecystectomy without CBD exploration

– Although laparoscopic resection is theoretically possible, high rate of conversion to open cholecystectomy
image Type II: Subtotal cholecystectomy, surgical repair of fistula (suture repair, choledochoplasty), and exploration of CBD
image Type III: Subtotal cholecystectomy or biliary reconstruction with biliary-enteric anastomosis
image Type IV: Biliary reconstruction, usually Roux-en-Y hepaticojejunostomy

DIAGNOSTIC CHECKLIST

Consider

• Distinguish Mirizzi syndrome from malignant biliary obstruction including cholangiocarcinoma, GB carcinoma, or extrinsic adenopathy

Image Interpretation Pearls

• Stone impacted in cystic duct causing obstruction of the CHD with upstream intrahepatic biliary dilatation
• Differentiate stones in the GB neck or cystic duct from stones in CHD
image
(Left) Axial CECT shows a dilated GB image with a thickened wall and pericholecystic stranding, suggesting acute cholecystitis.

image
(Right) ERCP in the same patient shows that the GB is obstructed by a large gallstone image (not visualized by CT). In addition, mass effect from the large cystic duct stone on the adjacent CHD causes intrahepatic biliary dilation image, in keeping with Mirizzi syndrome.
image
(Left) MRCP shows marked intrahepatic ductal dilatation and an abrupt transition to a small-caliber common duct image. There is the suggestion of a discrete filling defect image in the CHD at the site of transition.

image
(Right) ERCP in the same patient shows intrahepatic ductal dilatation and an obstructing filling defect within the common duct (CD) image. Note nonfilling of the GB. A cholecystocholedochal fistula and a large stone at the cystic duct-CD junction were shown at surgery, in keeping with Mirizzi syndrome.
image
(Left) Sagittal US of the GB neck demonstrates an impacted stone image with dilation of the adjacent CHD image, compatible with Mirizzi syndrome.

image
(Right) ERCP cholangiogram shows dilated intrahepatic ducts image due to a stricture of the CHD image. Note the presence of multiple gallstones within the GB image. Although not visible on this image, a stone was found impacted in the cystic duct, resulting in Mirizzi syndrome.
image
ERCP of Mirizzi syndrome shows the smooth extrinsic mass effect by a stone compressing the CHD and nonfilling of the gallbladder image.

SELECTED REFERENCES

1. Katabathina, VS, et al. Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics. 2014; 34(3):565–586.

2. Luu, MB, et al. Unusual complications of gallstones. Surg Clin North Am. 2014; 94(2):377–394.

3. Patel, NB, et al. Multidetector CT of emergent biliary pathologic conditions. Radiographics. 2013; 33(7):1867–1888.

 

Cui, Y, et al. Appraisal of diagnosis and surgical approach for Mirizzi syndrome. ANZ J Surg. 2012; 82(10):708–713.

Gupta, M, et al. Mirizzi syndrome: recognition on magnetic resonance cholangiopancreatography. Clin Gastroenterol Hepatol. 2012; 10(12):A32.

Zhong, H, et al. Mirizzi syndrome: experience in diagnosis and treatment of 25 cases. Am Surg. 2012; 78(1):61–65.

Erben, Y, et al. Diagnosis and treatment of Mirizzi syndrome: 23-year Mayo Clinic experience. J Am Coll Surg. 2011; 213(1):114–119. [discussion 120-1].

Antoniou, SA, et al. Laparoscopic treatment of Mirizzi syndrome: a systematic review. Surg Endosc. 2010; 24(1):33–39.

Yun, EJ, et al. Combination of magnetic resonance cholangiopancreatography and computed tomography for preoperative diagnosis of the Mirizzi syndrome. J Comput Assist Tomogr. 2009; 33(4):636–640.

Menias, CO, et al. Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. 2008; 28(4):1115–1129.

Yonetci, N, et al. The incidence of Mirizzi syndrome in patients undergoing endoscopic retrograde cholangiopancreatography. Hepatobiliary Pancreat Dis Int. 2008; 7(5):520–524.

Zaliekas, J, et al. Complications of gallstones: the Mirizzi syndrome, gallstone ileus, gallstone pancreatitis, complications of “lost” gallstones. Surg Clin North Am. 2008; 88(6):1345–1368. [x].

Abou-Saif, A, et al. Complications of gallstone disease: Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. Am J Gastroenterol. 2002; 97(2):249–254.

Gomez, G. Mirizzi syndrome. Curr Treat Options Gastroenterol. 2002; 5(2):95–99.

Haritopoulos, KN, et al. Mirizzi syndrome: a case report and review of the literature. Int Surg. 2002; 87(2):65–68.

Xiaodong, H, et al. Diagnosis and treatment of the Mirizzi syndrome. Chin Med Sci J. 1999; 14(4):246–248.

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