Introduction
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.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.
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.
Cholescintigraphy (3-h delayed images in the anterior projection) of the same patient as in Image above. 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.
Recent studies
Yun et al studied 52 patients with surgically proven Mirizzi syndrome (n = 13) and cholecystitis without evidence of Mirizzi syndrome (n = 39) who underwent magnetic resonance cholangiopancreatography (MRCP), using single-shot turbo spin echo and 3-dimensional turbo spin echo sequences, and computed tomography (CT) to determine the preoperative diagnostic accuracy of the combined modalities for Mirizzi syndrome. For combined modality, the overall sensitivity was 96% (versus 42% for CT); specificity, 93.5% (CT, 98.5%); positive predictive value, 83.5% (CT, 93%); negative predictive value, 98.5% (CT, 83.5%); and accuracy, 94% (CT, 85%).2
According to Beltran et al, the presence of Mirizzi syndrome should be considered when a cholecystoenteric fistula is encountered during surgery for gallstone disease. The authors retrospectively reviewed the records of 5,673 cholecystectomies performed from 1995 to 2006 and found 327 (5.7%) patients with Mirizzi syndrome and 105 (1.8%) patients with cholecystoenteric fistula. Of the 105 patients with cholecystoenteric fistula, 94 (89.5%) also had Mirizzi syndrome.3
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.4,5
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. Dietrich reported a low CBD insertion in as many as 18% of his patients who underwent surgical cholangiography.6,7
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.
McSherry et al proposed a 2-stage classification based on the results of endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC).8 Type I is simple external compression of the common hepatic duct (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 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.9
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.4,5
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.10 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.9
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.
Presentation
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.11,12,13,14,15
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.16
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.5
Some of the literature supports an increased coincidence of gallbladder carcinoma and Mirizzi syndrome.17,18
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.
Differential Diagnoses
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
More on Mirizzi Syndrome |
 Overview: Mirizzi Syndrome |
| Imaging: Mirizzi Syndrome |
| Follow-up: Mirizzi Syndrome |
| Multimedia: Mirizzi Syndrome |
| References |
| Further Reading |
| Next Page » |
References
Mirizzi PL. Syndrome del conducto hepatico. J Int de Chir. 1948;8:731-77.
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. Jul-Aug 2009;33(4):636-40. [Medline].
Beltran MA, Csendes A, Cruces KS. The relationship of Mirizzi syndrome and cholecystoenteric fistula: validation of a modified classification. World J Surg. Oct 2008;32(10):2237-43. [Medline].
Hazzan D, Golijanin D, Reissman P, et al. Combined endoscopic and surgical management of Mirizzi syndrome. Surg Endosc. Jun 1999;13(6):618-20. [Medline].
Pemberton M, Wells AD. The Mirizzi syndrome. Postgrad Med J. Aug 1997;73(862):487-90. [Medline].
Dietrich KF. [Stenosis of the hepatic duct in lithiasis of the gallbladder neck and cystic duct (Mirizzi syndrome).]. Bruns Beitr Klinischen Chir. Feb 1963;206:9-22. [Medline].
Toscano RL, Taylor PH Jr, Peters J, Edgin R. Mirizzi syndrome. Am Surg. Nov 1994;60(11):889-91. [Medline].
McSherry CK, Ferstenberg H, Virshup M. The Mirizzi syndrome: suggested classification and surgical therapy. Surg Gastroent. 1982;219-25.
Csendes A, Diaz JC, Burdiles P, et al. Mirizzi syndrome and cholecystobiliary fistula: a unifying classification. Br J Surg. Nov 1989;76(11):1139-43. [Medline].
Becker CD, Hassler H, Terrier F. Preoperative diagnosis of the Mirizzi syndrome: limitations of sonography and computed tomography. AJR Am J Roentgenol. Sep 1984;143(3):591-6. [Medline].
Schafer M, Schneiter R, Krahenbuhl L. Incidence and management of Mirizzi syndrome during laparoscopic cholecystectomy. Surg Endosc. Aug 2003;17(8):1186-90; discussion 1191-2.
Strugnell NA, Sali A. Choledochoplasty for cholecystocholedochal fistula (Mirizzi syndrome type II): a case report and literature review. Aust N Z J Surg. Apr 1995;65(4):285-8. [Medline].
Zaliekas J, Munson JL. Complications of gallstones: the Mirizzi syndrome, gallstone ileus, gallstone pancreatitis, complications of "lost" gallstones. Surg Clin North Am. Dec 2008;88(6):1345-68, x. [Medline].
Menias CO, Surabhi VR, Prasad SR, Wang HL, Narra VR, Chintapalli KN. Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. Jul-Aug 2008;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. May 2008;67(6):974-5; discussion 975. [Medline].
Curet MJ, Rosendale DE, Congilosi S. Mirizzi syndrome in a Native American population. Am J Surg. Dec 1994;168(6):616-21. [Medline].
Nishimura A, Shirai Y, Hatakeyama K. High coincidence of Mirizzi syndrome and gallbladder carcinoma. Surgery. Sep 1999;126(3):587-8. [Medline].
Redaelli CA, Buchler MW, Schilling MK, et al. High coincidence of Mirizzi syndrome and gallbladder carcinoma. Surgery. Jan 1997;121(1):58-63. [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. Apr 2009;82(976):279-85. [Medline].
Aydin U, Yazici P, Ozsan I, Ersõz G, Ozütemiz O, Zeytunlu M, et al. Surgical management of Mirizzi syndrome. Turk J Gastroenterol. Dec 2008;19(4):258-63. [Medline].
Seitz U, Bapaye A, Bohnacker S, et al. Advances in therapeutic endoscopic treatment of common bile duct stones. World J Surg. Nov 1998;22(11):1133-44. [Medline].
Baer HU, Matthews JB, Schweizer WP, et al. Management of the Mirizzi syndrome and the surgical implications of cholecystcholedochal fistula. Br J Surg. Jul 1990;77(7):743-5. [Medline].
Chowbey PK, Bandyopadhyay SK, Sharma A. Laparoscopic management of cholecystoenteric fistulas. J Laparoendosc Adv Surg Tech A. Oct 2006;16(5):467-72.
Further Reading
Related eMedicine topics
Gallbladder Disease
Gallbladder Tumors
Gallbladder Cancer
Gallbladder, Carcinoma
Bedside Ultrasonography, Gallbladder Disease
Keywords
Mirizzi syndrome, Mirizzi's syndrome, gallstones, gallstone impaction, Hartmann pouch, compression of the common hepatic duct, obstructive jaundice
