Mirizzi Syndrome Imaging 

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Mirizzi syndrome is an unusual presentation of obstructive jaundice caused by extrinsic compression of an extrahepatic biliary duct from one or more calculi within the cystic duct or gallbladder. It is a functional hepatic syndrome but can often present with biliary duct dilatation and can mimic other hepatobiliary pathologies such as cholangiocarcinoma.

The clinical presentation varies from no symptoms to severe cholangitis. ^[1] Patients may present with recurrent episodes of jaundice and cholangitis. It can be associated with acute cholecystitis. Fistulae can develop between the gallbladder and the common duct, and the stone may pass into the common duct. ^[2]  Generally, distinguishing between Mirizzi syndrome and other causes of obstructive jaundice is not possible with physical examination alone. ^{[3, 4]}  

(See images of Mirizzi syndrome below.)

Although a number of classification systems have been described, such as acute vs chronic variant, anatomic variant of cystic duct vs no anatomic variant of cystic duct, and obstruction due to gallstones vs obstruction due to inflammation, the most widely used is the Csendes classification system. The Csendes system recognizes 5 major types of Mirizzi syndrome: type I is characterized by “classic” compression stenosis; types II–IV involve formation of a cholecystocholedochal fistula; and type V is defined by the formation of a cholecystoenteric fistula in addition to the presence of types I-IV (see Table 1 below). ^[5] ​

Table 1. Csendes Classification of Mirizzi Syndrome (Open Table in a new window)

Mirizzi type I (10.5–51%) and Mirizzi type II (57%) are the most commonly reported classifications. ^[1]  Other types of Mirizzi yndrome are relatively low in incidence. 

Mirizzi syndrome is characterized by increased occurrence of complications compared with routine surgery for cholecystolithiasis. Bile duct reconstruction becomes more difficult with increases in the size of the cholecystocholedochal fistula. In some cases, difficulty performing bile duct reconstruction makes it necessary to perform complex surgical procedures, such as bile duct resection and choledochojejunostomy by the Roux-en-Y method. ^[6]

Safe and effective surgical therapy is facilitated by accurate preoperative diagnosis. However, such diagnosis is often missed preoperatively, although more advanced cases of disease are easier to detect before surgery. Diagnostic modalities include abdominal ultrasonography, computed tomography (CT) scanning, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP). ^[7]

Ultrasonography is used as a routine initial investigation for biliary disease and can reveal gallstones and cholecystitis, as well as evidence of Mirizzi syndrome such as an atrophic gallbladder and ectatic common hepatic duct with a normal distal CBD, or edematous gallbladder caused by acute cholecystitis. ^[5]

Although no specific radiologic features of Mirizzi syndrome can be recognized on CT, it is useful for detecting the cause and location of biliary obstruction. CT is also useful for differentiating hepatic portal and hepatic infiltration of tumors. In patients with cholecystobiliary fistula, CT scanning is valuable in distinguishing Mirizzi syndrome from neoplasia. ^[8]

MRCP is the preferred imaging modality. It is a noninvasive imaging technique and can delineate the typical characteristics of Mirizzi syndrome. MRCP confirmation is required when ultrasound examination detects a dilated bile duct with evidence of obstructive jaundice or stone impaction in the bile duct. Biliary and pancreatic ducts can also be assessed by MRCP, which can create superior images of inflammation around the gallbladder. Such inflammation is characteristic of Mirizzi syndrome and can be used to distinguish biliary conditions, including cancer. However, MRCP is not efficient at localizing a cholecystocholedochal fistula. ^[5]

Despite its invasiveness, ERCP is considered the gold standard for the diagnosis of Mirizzi syndrome. This technique yields superior visualization of the extrahepatic bile ducts and can clearly show extrinsic compression by impacted gallstones in the CBD, with resulting proximal biliary dilatation. ERCP can also accurately determine the presence and location of fistula and biliary obstruction. ^[5]  ERCP also allows biliary drainage to be performed endoscopically. ^[7]

Less traditional modalities of diagnosis have been reported in the literature. Percutaneous transhepatic cholangiography offers a reasonable option for diagnosis and the relief of obstructive symptoms preoperatively, especially when endoscopic treatment fails. Endoscopic ultrasound (EUS) can also be performed prior to ERCP to further evaluate the bile ducts and pancreas and to determine the cause of biliary strictures. ^[9]

Generally, plain radiography is not useful in diagnosing Mirizzi syndrome. Radiographs may depict radiopaque gallstones that contain sufficient amounts of calcium.

(See the image below.)

Potential surgical complications of a missed diagnosis obviate direct cholangiography in any patient with suspected Mirizzi syndrome after the initial evaluation with ultrasonography and/or CT. Endoscopic retrograde cholangiopancreatography (ERCP) is the criterion standard, with a mean sensitivity of 76.2%. ^[10]  Percutaneous transhepatic cholangiography (PTC) may also be used for diagnosis, especially if ERCP findings fail to help. ^[9]  Common signs include visible calculus in the expected position of the cystic duct, as well as smooth, lateral, and extrinsically compressed CHD. ^[6]

Typical diagnostic findings of Mirizzi syndrome on CT include the following ^{[11, 12]} :

Dilatation of the biliary system, including the CHD, distal to the level of the gallbladder neck

An impacted calculus in the neck of the gallbladder

A contracted gallbladder

A normal diameter of the CBD below the level of the stone.

Yun et al measured the preoperative diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) and CT for Mirizzi syndrome and found that for combined modality (MRCP and CT), the overall sensitivity was 96% (versus 42% for CT); specificity was 93.5% (CT, 98.5%); positive predictive value was 83.5% (CT, 93%); negative predictive value was 98.5% (CT, 83.5%); and accuracy was 94% (CT, 85%). ^[12]

Typical magnetic resonance cholangiopancreatography (MRCP) findings of Mirizzi syndrome include the following ^{[13, 14, 15, 16]} :

An impacted stone in the gallbladder neck

Compression of the common hepatic duct

Dilatation of the biliary system above the level of impaction

A contracted gallbladder with wall-thickening

Additional sequences should be used to exclude the presence of malignancy.

In one report, successful preoperative diagnosis was achieved by abdominal ultrasonography in only 17% (2/12) of cases, but 83% (10/12) of cases were successfully diagnosed by MRCP. ^[6]

Ultrasonographic findings include the following ^{[4, 9]} :

An impacted calculus in the Hartmann pouch or the cystic duct

Dilatation of the CHD above the level of the impacted stone

Narrowing of the CHD at the level of impaction

Normal caliber of the CBD below the impaction

Hepatobiliary iminodiacetic acid–diisopropyl iminodiacetic acid (HIDA-DISIDA) scintigraphy may be useful in diagnosing Mirizzi syndrome. Three highly sensitive and specific signs noted are a nonvisualized gallbladder, moderate dilatation of the common hepatic duct (CHD), and delayed excretion into the duodenum.

(See the images below.)

Percutaneous transhepatic cholangiography (PTC) findings include the following:

An impacted calculus in the Hartmann pouch or gallbladder neck

Narrowed CHD at the level of impaction

Dilatation of the CHD distal to the level of the impacted calculus

Normal-caliber CBD proximal to the impacted calculus

Kumar A, Senthil G, Prakash A, Behari A, Singh RK, Kapoor VK, et al. Mirizzi’s syndrome: lessons learnt from 169 patients at a single center. Korean J Hepatobiliary Pancreat Surg. 2016 Feb. 20 (1):17-22. [Medline]. [Full Text].

Aldekhayel M, Almohaimeed K, AlShahrani MS, Almweisheer S. Rare case of Mirizzi syndrome associated with cholecystogastric fistula. BMJ Case Rep. 2016 Jan 11. 2016:[Medline]. [Full Text].

Menias CO, Surabhi VR, Prasad SR, Wang HL, Narra VR, Chintapalli KN. Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. 2008 Jul-Aug. 28(4):1115-29. [Medline].

Pelaez-Luna M, Levy MJ, Arora AS, Baron TH, Rajan E. Mirizzi syndrome presenting as painless jaundice: a rare entity diagnosed by EUS. Gastrointest Endosc. 2008 May. 67(6):974-5; discussion 975. [Medline].

Chen H, Siwo EA, Khu M, Tian Y. Current trends in the management of Mirizzi Syndrome: A review of literature. Medicine (Baltimore). 2018 Jan. 97 (4):e9691. [Medline]. [Full Text].

Zhong H, Gong JP. Mirizzi syndrome: experience in diagnosis and treatment of 25 cases. Am Surg. 2012 Jan. 78(1):61-5. [Medline].

[Guideline] Tazuma S, Unno M, Igarashi Y, Inui K, Uchiyama K, Kai M, et al. Evidence-based clinical practice guidelines for cholelithiasis 2016. J Gastroenterol. 2017 Mar. 52 (3):276-300. [Medline]. [Full Text].

Beltrán MA. Mirizzi syndrome: history, current knowledge and proposal of a simplified classification. World J Gastroenterol. 2012 Sep 14. 18 (34):4639-50. [Medline]. [Full Text].

Rayapudi K, Gholami P, Olyaee M. Mirizzi syndrome with endoscopic ultrasound image. Case Rep Gastroenterol. 2013 May. 7 (2):202-7. [Medline].

Yuan H, Yuan T, Sun X, Zheng M. A Minimally Invasive Strategy for Mirizzi Syndrome Type II: Combined Endoscopic With Laparoscopic Approach. Surg Laparosc Endosc Percutan Tech. 2016 Jun. 26 (3):248-52. [Medline].

Patel NB, Oto A, Thomas S. Multidetector CT of emergent biliary pathologic conditions. Radiographics. 2013 Nov-Dec. 33 (7):1867-88. [Medline].

Yun EJ, Choi CS, Yoon DY, Seo YL, Chang SK, Kim JS, et al. Combination of magnetic resonance cholangiopancreatography and computed tomography for preoperative diagnosis of the Mirizzi syndrome. J Comput Assist Tomogr. 2009 Jul-Aug. 33(4):636-40. [Medline].

Oto A, Ernst R, Ghulmiyyah L, Hughes D, Saade G, Chaljub G. The role of MR cholangiopancreatography in the evaluation of pregnant patients with acute pancreaticobiliary disease. Br J Radiol. 2009 Apr. 82(976):279-85. [Medline].

Wani NA, Khan NA, Shah AI, Khan AQ. Post-cholecystectomy Mirizzi’s syndrome: magnetic resonance cholangiopancreatography demonstration. Saudi J Gastroenterol. 2010 Oct-Dec. 16(4):295-8. [Medline]. [Full Text].

Katabathina VS, Dasyam AK, Dasyam N, Hosseinzadeh K. Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics. 2014 May-Jun. 34 (3):565-86. [Medline].

Elhanafy E, Atef E, El Nakeeb A, Hamdy E, Elhemaly M, Sultan AM. Mirizzi Syndrome: How it could be a challenge. Hepatogastroenterology. 2014 Jul-Aug. 61 (133):1182-6. [Medline].

Jeffrey W Ross, MD Consulting Physician, PENRAD Imaging of Colorado Springs

Jeffrey W Ross, MD is a member of the following medical societies: Radiological Society of North America

Disclosure: Nothing to disclose.

Gary S Sudakoff, MD, FSRU Associate Professor of Radiology, Urology and Gastroenterology, Medical College of Wisconsin; Chief of Uroradiology, Department of Radiology, Froedtert Memorial Lutheran Hospital

Gary S Sudakoff, MD, FSRU is a member of the following medical societies: American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, Radiological Society of North America, Sigma Xi, Society of Abdominal Radiology, Society of Radiologists in Ultrasound

Disclosure: Nothing to disclose.

Gregory B Snyder, MD Director of Fellowship for Vascular and Interventional Radiology, University of Minnesota Hospital and Clinics

Disclosure: Nothing to disclose.

Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand

Disclosure: Nothing to disclose.

Abraham H Dachman, MD, FACR Professor, Department of Radiology, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine; Director of CT, Department of Radiology, The University of Chicago Hospitals

Abraham H Dachman, MD, FACR is a member of the following medical societies: Radiological Society of North America

Disclosure: Nothing to disclose.

John Karani, MBBS, FRCR Clinical Director of Radiology and Consultant Radiologist, Department of Radiology, King’s College Hospital, UK

John Karani, MBBS, FRCR is a member of the following medical societies: British Institute of Radiology, Radiological Society of North America, Royal College of Radiologists, Cardiovascular and Interventional Radiological Society of Europe, European Society of Radiology, European Society of Gastrointestinal and Abdominal Radiology, British Society of Interventional Radiology

Disclosure: Nothing to disclose.

Neela Lamki, MD, FACR, FRCPC Professor, Department of Radiology, Sultan Qaboos University, Oman; Adjunct Professor, Department of Radiology, Baylor College of Medicine

Neela Lamki, MD, FACR, FRCPC is a member of the following medical societies: American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Royal College of Physicians and Surgeons of Canada, Texas Medical Association, Texas Radiological Society, Society of Abdominal Radiology, Association of Program Directors in Interventional Radiology

Disclosure: Nothing to disclose.

Mirizzi Syndrome Imaging 

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