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Radiology > GASTROINTESTINAL
Mirizzi Syndrome
Article Last Updated: Dec 29, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Jeffrey W Ross, MD, Staff Physician, Department of Diagnostic Radiology, Medical College of Wisconsin
Jeffrey W Ross is a member of the following medical societies: Radiological Society of North America
Coauthor(s):
Gary S Sudakoff, MD, FSRU, Associate Professor of Radiology and Urology, Medical College of Wisconsin; Chief of Uroradiology, Department of Radiology, Froedtert Memorial Lutheran Hospital;
Gregory B Snyder, MD, Assistant Professor, Department of Interventional Radiology, University of Minnesota Hospital and Clinics
Editors: Neela Lamki, MD, Professor, Department of Radiology, Sultan Qaboos University, Oman; Adjunct Professor, Department of Radiology, Baylor College of Medicine; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Abraham H Dachman, MD, FACR, Professor, Department of Radiology, The University of Chicago School of Medicine; Director of CT, Department of Radiology, The University of Chicago Hospitals; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; John Karani, MBBS, FRCR, Consulting Staff, Department of Radiology, King's College Hospital, London
Author and Editor Disclosure
Synonyms and related keywords:
Mirizzi's syndrome, gallstones, gallstone impaction, Hartmann pouch, compression of the common hepatic duct, obstructive jaundice
Background
In 1948, P. L. Mirizzi described an unusual presentation of gallstones that, when lodged in either the cystic duct or the Hartmann pouch of the gallbladder, externally compressed the common hepatic duct (CHD), causing symptoms of obstructive jaundice (Mirizzi, 1948).
Pathophysiology
Impaction of a large gallstone (or multiple small gallstones) in the Hartmann pouch or cystic duct results in the Mirizzi syndrome in 2 ways: (1) chronic and/or acute inflammatory changes lead to contraction of the gallbladder, which then fuses with and causes secondary stenosis of the CHD, or (2) large impacted stones lead to cholecystocholedochal fistula formation secondary to direct pressure necrosis of the adjacent duct walls. Increasingly, these phenomena are seen not as distinct and separate steps but as part of a continuum (Pemberton, 1997; Hazzan, 1999).
The presence of a long cystic duct in parallel with the CHD or a low insertion of the cystic duct into the common bile duct (CBD) also increases the likelihood of this syndrome. In 1963, Dietrich reported a low CBD insertion in as many as 18% of his patients who underwent surgical cholangiography (Dietrich, 1963; Toscano, 1994).
The process of impaction of a calculus with inflammatory changes that leads to eventual erosion of the involved walls and fistula formation has led some authors to create a classification system for the Mirizzi syndrome.
In 1982, McSherry et al proposed a 2-stage classification based on the results of endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). Type I is simple external compression of the CHD, whereas type II involves the presence of a cholecystocholedochal fistula.
In ensuing years, these 2 categories were further subclassified into the following groups to aid in the surgical treatment of patients:
- Type I - No fistula present
- Type IA - Presence of the cystic duct
- Type IB - Obliteration of the cystic duct
- Types II-IV - Fistula present
- Type II - Defect smaller than 33% of the CBD diameter
- Type III - Defect 33-66% of the CBD diameter
- Type IV - Defect larger than 66% of the CBD diameter
In a large study (219 patients), Csendes et al (1989)reported that 11% of their patients with Mirizzi syndrome had type I lesions, 41% had type II lesions, 44% had type III lesions, and 4% had type IV lesions.
Frequency
United States
Mirizzi syndrome occurs in approximately 0.7-1.4% of all patients undergoing cholecystectomy and in 0.1% of all patients with gallstone disease (Pemberton, 1997; Hazzan, 1999).
Mortality/Morbidity
Preinterventional diagnosis of this rare condition is critical to the patient's prognosis. Chronic inflammation that leads to fibrosis, scarring, edema, and fistula formation can wreak havoc on adjacent biliary structures and cause serious surgical consequences if unnoticed. Therefore, every patient in whom this abnormality is suspected (at initial ultrasonography or CT) must undergo anatomic evaluation with cholangiography before surgical intervention.
Extensive adhesions may make visualization of the biliary anatomy exceptionally difficult, especially within the hepatoduodenal ligament. Consequently, the CBD may be mistaken for the cystic duct, and ligation or permanent injury may occur during surgery (Becker, 1984). Postoperative bile leakage may occur if a fistula is not recognized; rarely, this may result in bile peritonitis.
Race
No racial predilection is known.
Sex
Males and females are affected equally.
Age
Mirizzi syndrome is more common in the elderly, but any patient with cholelithiasis is at risk (Csendes, 1989).
Anatomy
The fundus is the most distal portion of the gallbladder; the body (corpus), Hartmann pouch (infundibulum), and neck arise proximal to this. From the gallbladder neck the cystic duct runs until it converges with the CHD to form the CBD, which eventually joins the descending duodenum via the ampulla of Vater. The cystic artery, which supplies the gallbladder, usually runs parallel to the cystic duct. The inferior border of the liver, CHD, and cystic duct form the 3 boundaries of the Calot triangle through which the right hepatic artery may be seen.
Clinical Details
Mirizzi syndrome has no consistent or unique clinical features that distinguish it from other more common forms of obstructive jaundice. Symptoms of recurrent cholangitis, jaundice, right upper quadrant pain, and abnormal hepatic serum biochemical findings may or may not be present (Strugnell, 1995; Schafer, 2003).
In one study of 17 patients in whom Mirizzi syndrome was found at surgery, only 10 (59%) had presented with a recent history of jaundice. Twelve (71%) patients had elevated preoperative alkaline phosphatase levels, and 10 (59%) had elevated bilirubin levels (Curet, 1994).
Acute presentations of the syndrome include pancreatitis and cholecystitis; in a patient with persistent jaundice and a right upper quadrant mass, the presence of carcinoma may be mistakenly inferred (Pemberton, 1997).
Some of the literature supports an increased coincidence of gallbladder carcinoma and Mirizzi syndrome (Nishimura, 1999; Redaelli, 1997).
Preferred Examination
Generally, distinguishing between Mirizzi syndrome and other causes of obstructive jaundice is not possible with physical examination alone. Ultrasonography is frequently a first-line diagnostic examination. CT may also be used.
Limitations of Techniques
The Mirizzi syndrome cannot be diagnosed with physical examination alone. Radiologic studies are required.
Biliary Atresia
Cholangiocarcinoma
Cholelithiasis
Other Problems to be Considered
Anxiety disorder, trichotillomania
Alcoholic fatty liver
Ascariasis
Carcinoma of the head of the pancreas
Carcinoma of the papilla
Coccidioidomycosis
Echinococcosis
Macrocytosis
Pancreatitis
Strongyloidiasis
Cholecystitis and biliary colic
Gallbladder tumors
Occasionally, Mirizzi syndrome may be mistaken for malignancy on the basis of the history and/or the physical examination and radiographic findings.
Findings
Generally, plain radiography is not useful in diagnosing Mirizzi syndrome. Radiographs may depict radiopaque gallstones that contain sufficient amounts of calcium.
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. ERCP is probably the criterion standard, and it is also useful in temporarily relieving stenosis of the CHD by means of stent placement. PTC may also be used for diagnosis, especially if ERCP findings fail to help.
Common signs include (1) visible calculus in the expected position of the cystic duct and (2) smooth, lateral, and extrinsically compressed CHD (Pemberton, 1997).
Findings
Typical diagnostic findings of Mirizzi syndrome on CT include (1) dilatation of the biliary system, including the CHD, distal to the level of the gallbladder neck; (2) an impacted calculus in the neck of the gallbladder; (3) a contracted gallbladder; and (4) a normal diameter of the CBD below the level of the stone (Pemberton, 1997; Becker, 1984).
Signs of cholecystitis or pericholecystitis may also be present, but they are nonspecific (Becker, 1984). Some authors maintain that CT should be used primarily to exclude malignancies—namely, liver metastases invading the biliary system and carcinoma of the porta hepatis—from the differential diagnosis (Toscano, 1994).
Findings
Typical magnetic resonance cholangiopancreatography (MRCP) findings of Mirizzi syndrome include (1) an impacted stone in the gallbladder neck, (2) compression of the common hepatic duct, (3) dilatation of the biliary system above the level of impaction, and (4) a contracted gallbladder with wall-thickening.
Additional sequences should be used to exclude the presence of malignancy.
Findings
Ultrasonographic findings include: (1) an impacted calculus in the Hartmann pouch or the cystic duct, (2) dilatation of the CHD above the level of the impacted stone, (3) narrowing of the CHD at the level of impaction, and (4) normal caliber of the CBD below the impaction.
Findings
A 1992 study revealed that hepatobiliary iminodiacetic acid–diisopropyl iminodiacetic acid (HIDA-DISIDA) scintigraphy is useful in diagnosing Mirizzi syndrome. Three highly sensitive and specific signs noted are (1) a nonvisualized gallbladder, (2) moderate dilatation of the CHD, and (3) delayed excretion into the duodenum (Alarco, 1992).
Findings
PTC findings include (1) an impacted calculus in the Hartmann pouch or gallbladder neck, (2) narrowed CHD at the level of impaction, (3) dilatation of the CHD distal to the level of the impacted calculus, and (4) normal-caliber CBD proximal to the impacted calculus.
Most authors agree that the treatment of choice for Mirizzi syndrome is surgical (Pemberton, 1997; Toscano, 1994; Strugnell, 1995). However, a 1998 study by Seitz et al revealed that electrohydraulic lithotripsy (EHL) was successful in 38 of 38 patients with known Mirizzi syndrome; the only complication was a perforation that was treated conservatively. When surgical treatment is used, the technique depends on the classification of the patient's Mirizzi syndrome, which occasionally is not discovered until the procedure has begun.
Initially, preoperative cholangiography is indicated to determine if the cystic duct is present and to ensure that the CBD is free from calculi. If so, any stones in the cystic duct should then be manipulated back into the gallbladder. The gallbladder should be incised at its fundus (conventional retrograde dissection is contraindicated secondary to risks of injuring the Calot triangle) (Pemberton, 1997), and the gallstone is removed. If a gush of bile into the gallbladder occurs upon removal of the stone, a fistula is present (Baer, 1990). A Foley catheter may be inserted into the gallbladder neck to confirm this (Toscano, 1994).
Mirizzi syndrome type I (ie, compression without a fistula) is generally treated as minimally as possible. Baer et al (1990) advocate partial cholecystectomy, which leaves the neck of the gallbladder in place, because long-standing inflammation and fibrosis always lead to occlusion of the cystic duct. Stenosis of the CBD generally resolves as the inflammation subsides (Pemberton, 1997).
Mirizzi syndrome types II-IV (ie, fistula present) require more complex interventions. Type II defects are generally treated successfully with either cholecystectomy and closure around a T-tube or partial cholecystectomy with in situ T-tube placement (Pemberton, 1997).
Other treatment options for larger defects (ie, Mirizzi syndrome types III and IV) exist, such as the following:
- Incision of the CBD via longitudinal choledochostomy directly over the gallstone, followed by cholecystectomy and subsequent suturing of the remaining gallbladder flaps around a T-tube is one option (Toscano, 1994; McSherry, 1982).
- If repair of the larger fistula is necessary, some researchers have advocated choledochoplasty with a well-vascularized and bile-tolerant gallbladder flap because of its technical ease and effectiveness (Csendes, 1989; Strugnell, 1995).
- Other researchers suggest biliary-enteric bypass via Roux-en-Y choledochojejunostomy or a choledochoduodenostomy, to reduce the mortality and morbidity risk of CBD stricture (Pemberton, 1997; Baer, 1990).
- Laparoscopic stapled cholecystofistulectomy, which avoids contamination of the peritoneal cavity, may be performed in well-equipped centers if a cholecystoenteric fistula is present (Chowbey, 2006).
Special Concerns
- See Intervention for details concerning the different surgical techniques for Mirizzi syndrome types I-IV.
| Media file 1:
This patient presented with acute cholecystitis, as confirmed at imaging. His pain resolved over a few days, but mildly elevated bilirubin levels persisted. Image obtained during endoscopic retrograde cholangiopancreatography shows smooth narrowing of the bile duct (arrow) at the site of insertion of the cystic duct (Mirizzi syndrome). Note the small calculus in the cystic duct. Courtesy of Dr. Ali Nawaz Khan. |
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Media type: X-RAY
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| Media file 2:
Cholescintigraphy (1-h initial images) in 61-year-old man with right upper quadrant pain for 2 days. Note the nonvisualization of gallbladder and small bowel activity. Lower activity is within the urinary bladder. Courtesy of Dr. Arthur Krasnow, Department of Radiology, Medical College of Wisconsin. |
 | View Full Size Image | |
Media type: Image
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| Media file 3:
Cholescintigraphy (3-h delayed images in the anterior projection) of the same patient as in Image 2. Note persistent nonvisualization of the gallbladder and minimal activity within the small bowel. Courtesy of Dr. Arthur Krasnow, Department of Radiology, Medical College of Wisconsin. |
 | View Full Size Image | |
Media type: Image
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Mirizzi Syndrome excerpt Article Last Updated: Dec 29, 2006
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