KUWAIT MEDICAL JOURNALMarch 2006

Kuwait Medical Journal 2006, 38 (1): 3-6

Review Article

Mirizzi Syndrome: A Review of the Literature

Address correspondence to:George J Xeroulis, Department of Surgery, University of Western Ontario London, Ontario, Canada. E-mail: wardd@rogers.com

George J Xeroulis, Ward Davies

Department of Surgery, University of Western Ontario, London, Ontario, Canada

Mirizzi syndrome is a rare cause of obstructivejaundice. This entity should be considered in thed i ff e rential diagnosis of all patients with obstru c t i v ejaundice. Failure to recognize the conditionpreoperatively can result in a major bile duct injury,particularly during laparoscopic surg e r y[ 1 ]. Thesyndrome refers to obstruction of the commonhepatic duct by extrinsic compression usually froma gallstone impacted in Hartmann’s pouch or thecystic duct. Large gallstones that become impactedin this area produce common hepatic ducto b s t ruction by two mechanisms: mechanicalobstruction by direct compression of the commonhepatic duct, or they can cause obstru c t i o nsecondary to repeated bouts of local inflammation.

In 1948, A rgentinean surgeon Pablo LuisMirizzi, first described a syndrome of commonhepatic duct obstruction in the setting oflongstanding cholelithiasis and cholecystitis[2]. Theclassic description of the disease includes fourcomponents: (a) a close parallel course of the cysticduct and the common hepatic duct, (b) an impactedstone in the cystic duct or the neck of thegallbladder, (c) common hepatic duct obstructionsecondary to external compression by the cysticduct stone (and the surrounding inflammation),and (d) jaundice, with or without cholangitis.

Mirizzi’s syndrome is a rare complication ofcholelithiasis, with an estimated incidence of 0.05-2.7%[1,3,4]. It presents as a spectrum of disease thatvaries from extrinsic compression of the commonhepatic duct to the presence of a cholecystobiliaryfistula. Often, this dangerous alteration to anatomyis not recognized pre o p e r a t i v e l y, and has thepotential to lead to significant morbidity andbiliary injury, particularly in the laparoscopic era.

CLASSIFICATION There are three classifications which have been

proposed to describe variants of Mirizzi syndrome,and to aid in selecting the appropriate therapeuticprocedure. The original classification, by McSherry

et al[5], described two types. Type I referred tocompression of the common hepatic duct by a stoneimpacted in the cystic duct or Hartmann’s pouch.Type II referred to erosion of the calculus from thecystic duct into the common hepatic duct,producing a cholecystocholedochal fistula.

Csendes et al[6] created a second classificationtaking into account the extent of fistula. Type Iremained the same, external compression of thecommon hepatic duct due to a stone impacted atthe neck of the gallbladder or at the cystic duct.Types II to IV lesion referred to the presence andextent of a cholecystobiliary (cholecystohepatic orcholecystocholedochal) fistula, due to erosion ofthe anterior or lateral wall of the common hepaticduct by impacted stones. The fistula involved lessthan one-third of the circumference of the commonhepatic duct in type II. Involvement of betweenone-third and two-thirds of the circumference ofthe common hepatic duct was called a type IIIlesion, while destruction of the entire wall of thecommon hepatic duct was called a type IV lesion.In their original paper, a total of 219 patients wereidentified with Mirizzi’s syndrome. The incidenceof type I lesions was 11 per cent, type II, 41 per cent,type III, 44 per cent and type IV, four per cent. Themajority had obstructive jaundice.

The third classification, proposed by Nagakawaand colleagues[7], expanded upon the definition ofthe Mirizzi syndrome. Type I referred to a stoneimpacted in the cystic duct or gallbladder neck.Type II was characterized by a fistula of thecommon duct. Type III was defined by hepatic ductstenosis due to a stone at the confluence of thehepatic and cystic ducts. Type IV was characterizedby hepatic duct stenosis as a complication ofcholecystitis in the absence of calculi impacted inthe cystic duct or gallbladder neck.

In one series of 30 patients, the frequency ofthese four types as described by Nagakawa et alwas 14, 2, 6, and 8%, respectively[8].

INTRODUCTION

Mirizzi Syndrome: A Review of the Literature March 20064

Mirizzi syndrome is part of the differentialdiagnosis of all patients with obstructive jaundice,and re q u i res a high index of suspicion. Mostpatients present with jaundice, and right upperquadrant pain[1]. Elevations in the serum concentra-tions of alkaline phosphatase and bilirubin arepresent in over 90 per cent of patients[8,9]. Theclinical and laboratory findings are similar topatients who present with obstructive jaundicesecondary to choledocholithiasis. Once a diagnosisof obstructive jaundice has been made anabdominal ultrasound is often the first imaging testpreformed. Imaging generally reveals gallstones,dilated intrahepatic ducts, with a long parallelcystic duct and a contracted gallbladder[10]. Thepresence of a stone impacted in the gallbladderneck and an abrupt change to a normal width of thecommon duct below the level of the stone are alsovery suggestive of Mirrizi’s syndrome. Thesensitivity of ultrasound in detecting Mirizzi’ss y n d rome is 23-46%[ 3 , 4 ]. In Csendes’ series,ultrasound revealed dilated ducts in 81% ofpatients and raised suspicion of Mirizzi’s syndromein only 27% of cases. CT scanning has a similarsensitivity to ultrasound, but can be helpful indiagnosing other causes of obstructive jaundicesuch as gallbladder cancer, cholangiocarcinoma, ormetastatic tumor [11].

CHOLANGIOGRAPHY Direct cholangiography is usually necessary to

establish the correct diagnosis and to delineate thehepatic duct anatomy[10]. Pre-operative diagnosis is

Table 1: Various Classification Systems of Mirizzi’s Syndrome

McSherry Csendes Nagakawa

Extrinsic compression ofthe common hepatic ductby stones generally impac-ted in the cystic duct or inthe infundibulum of thegallbladder

Presence of cholecystobi-liary fistula

Extrinsic compression of thecommon hepatic duct by stonesgenerally impacted in the cysticduct or in the infundibulum ofthe gallbladder

P resence of cholecystobiliaryfistula with diameter one thirdof circumference of the commonhepatic duct wall

P resence of cholecystobiliaryfistula with diameter two thirdof circumference of the commonhepatic duct wall

P resence of cholecystobiliaryfistula which involves the entirecircumference of the commonhepatic duct wall

Extrinsic compression (stenosis) ofthe common hepatic duct by stonesgenerally impacted in the cysticduct or in the infundibulum of thegallbladder

Fistulization of common hepaticduct from a stone impacted in thecystic duct or in the infundibulumof the gall bladder

Common hepatic duct stone at thecystic duct-hepatic duct confluence

Common hepatic duct stenosiscaused by cholecystitis withoutstones in the cystic duct orinfundibulum of the gallbladder

Type I

Type II

Type I

Type II

Type III

Type IV

Type I

Type II

Type III

Type IV

essential in avoiding CBD injuries[12,13,14]. If it wasunexpectedly encountered at the time of surgery, acautious approach should be taken. Periductalinflammation and the potential for a cholecysto-choledochal fistula make a trial dissectionparticularly challenging and should only beundertaken by an experienced surgeon. Additionalimaging is often needed to obtain details of thebiliary anatomy. Intraoperative cholangiogram orclosing and obtaining a postoperative ERCP orMRCP should be considered. Cholangiography(intraoperative or ERCP) as well as MRCP willallow for an accurate assessment of anatomy andclassification of the type of Mirizzi’s syndrome (Fig. 1).

The possibility of stone retrieval and biliarystenting during ERCP is an added advantage ini m p roving surgical outcome, and stenting alsofacilitates identification of the CBD duringoperative dissection[ 7 , 1 5 , 1 6 ]. When ERCP i sunsuccessful or difficult, percutaneous transhepaticcholangiography (PTC) is a viable alternative.

MRCP and ERCP are equivalent in their abilityto diagnosis and to delineate details of biliarystrictures, and to detect a cholecystocholedochalfistula [4]. In addition, T2 weighted images cand i ff e rentiate a neoplastic mass from aninflammatory one which US or CT scan may not becapable of [17]. Early ERCP is preferred when biliarysepsis is the dominant clinical issue and where abeneficial endoscopic therapeutic procedure can beinstituted at the same time. By contrast, MRCP isused in the non-septic patient to corroborate thesuspicion of malignancy or stones after initialimaging with US or CT scans[4].

KUWAIT MEDICAL JOURNAL 5March 2006

TREATMENT Surgery is the mainstay of therapy of Mirizzi

s y n d rome, the dense inflammatory reaction inCalot’s triangle, as well as the frequent aberrantbiliary anatomy, pose a difficult challenge to theunsuspecting surgeon when dealing with a Mirizzisyndrome. The two principal aims are (a) the safecompletion of cholecystectomy without injuringthe biliary system and (b) the appro p r i a t emanagement of the cholecystocholedochal fistula.Meticulous dissection and vigilance are essential inorder to avoid inadvertent bile duct injury. If thediagnosis of Mirizzi syndrome is made pre o p e r a t i v e l y,an operative strategy that minimizes the risk ofinjury to the biliary tract can be carried out.H o w e v e r, a preoperative diagnosis of Mirizzisyndrome is seldom made because ERCPand directcholangiography are not widely used. ERCP, directcholangiography, or magnetic resonance cholangio-graphy should be performed in patients withclinical jaundice and signs and symptomssuggestive of biliary obstruction.

A s t a n d a rdized surgical approach has beenrecommended based on the Csendes classificationof the variants of Mirizzi syndrome [6]:

l Type I - Cholecystectomy plus common bileduct exploration with T-tube placement.Exploration should be performed only if the CBD iseasily exposed. l Type II - Suture of the fistula with absorbable

material or choledochoplasty with the re m n a n tgallbladder.

l Type III - Choledochoplasty; suture of thefistula is not indicated. l Type IV - Bilio-enteric anastomosis is

preferred since the entire wall of the common bileduct has been destroyed.

l The approach may vary with the type offistula present; both the operative mortality andpostoperative morbidity increase according to theseverity of the lesion [6].

LAPAROSCOPIC SURGERYThe Mirizzi syndrome presents a diff i c u l t

challenge for laparoscopic surgery because thedense adhesions and edematous inflammatorytissue cause distortion of the normal anatomy andincrease the risk for biliary injury. While it appearsto be feasible, especially for type I anatomy[18,19], theroutine use of laparoscopic surgery as the primarytreatment of Mirizzi syndrome is controversial[20,21].It has been suggested, that a prudent approach fortype 1 Mirizzi syndrome is to perform a triallaparoscopic dissection, but to have a low thresholdto convert to an open procedure. This approachshould be undertaken only by experiencedlaparoscopic surgeons[18,20].

ENDOSCOPIC THERAPY Endoscopic treatment with or without

electrohydraulic lithotripsy (EHL) can be effectiveas a temporizing measure before surgery and canbe definitive treatment for unsuitable surg i c a lc a n d i d a t e s[ 9 , 2 2 , 2 3 ]. One report described theexperience with 14 patients with Mirizzi syndrometreated with EHL[9]. Twelve patients had a singlestone and complete clearance was achieved withone treatment session; two had multiple stones andre q u i red an additional treatment session.Asymptomatic leakage of contrast medium fromthe cystic duct into the peritoneal cavity wasobserved in one patient after removal of a largeimpacted cystic duct stone. This patient recoveredwith conservative therapy and suffered no adverseevents. In another series of 25 patients withcholangiographic evidence of Mirizzi syndrome, 12w e re re f e r red for surgery after pre l i m i n a r yendoscopic therapy and 13 were treated solely withendoscopy[23]. Stones were completely removed inthree and nine were treated with long-term stents;complications occurred in four patients[23].

Fig. 1: Endoscopic re t rograde cholangiopancreatography (ERCP) ofpatient with obstructive jaundice and Mirizzi’s syndrome. Noticeimpacted stone in cystic duct causing obstruction of common hepaticduct. Adapted from UptoDate “Mirizzi Syndrome” James B McGee.

Mirizzi Syndrome: A Review of the Literature March 20066

Endoscopic treatment of Mirizzi syndro m eshould be used as a temporizing measure beforesurgery. It can serve as a definitive treatment forthose patients who are unsuitable surg i c a lcandidates when further endoscopic attempts canbe made to disimpact and remove the stones. Long-term success appears to be most likely in patientswith type II disease who do not have residualgallbladder stones[24].

CONCLUSIONMirizzi syndrome is a rare complication of

cholelithiasis and requires a high index of suspicionin the setting of obstructive jaundice. Diagnosispreoperatively may be elusive with bloodwork, USand CT alone. Cholangiography (intraoperativeand ERCP) as well as MRCP aids in both thediagnosis and identification of anatomy and mayp revent serious biliary injury. Surgery is themainstay of therapy of Mirizzi syndrome, andrequires the safe completion of cholecystectomywithout injuring the biliary system and thea p p ropriate management of the cholecysto-choledochal fistula. The aberrant anatomy intrinsicto this syndrome presents a difficult challenge tosurgeons and the laparoscopic approach should beundertaken with caution and probably left tospecialized minimally invasive centres. Endoscopict reatment may be effective as a temporizingm e a s u re before surgery and can be definitivetreatment for unsuitable surgical candidates.

REFERENCES

1. Waisberg J, Corona A, de Abreu IW, Farah JFM, LupinacciRA, Goffi FS. Benign Obstruction of the Common HepaticDuct (Mirizzi Syndrome): diagnosis and operativemanagement. Arq Gastroenterol 2005; 42:13-18.

2. Mirizzi, PL. Syndrome del conducto hepatico. J Int de Chir1948; 8:731-733.

3. Yeh, CN, Jan, YY, Chen, MF. Laparoscopic treatment forMirizzi syndrome. Surg Endosc 2003; 17:1573-1578.

4. Chan CY, Liau KH, Ho CK, Chew SP. Mirizzi syndrome: adiagnostic and operative challenge. Surgeon 2003;1:273-278.

5. McSherry, CK, Ferstenberg, H, Virshup, M. The Mirizzisyndrome: Suggested classification and surgical treatment.Surg Gastroenterol 1982; 1:219-225.

6. Csendes, A, Diaz, CJ, Burdiles, P, et al. Mirizzi syndromeand cholecystobiliary fistula: A unifying classification. Br JSurg 1989; 76:1139-1143.

7. Nagakawa, T, Ohta, T, Kayahara, M, et al. A n e wclassification of Mirizzi syndrome from diagnostic andtherapeutic viewpoints. Hepatogastro e n t e rology 1997;44:63-67.

8. I b r a rullah, M, Saxena, R, Sikora, SS, et al. Mirizzi’ssyndrome: Identification and management strategy. Aust NZ J Surg 1993; 63:802-806.

9. B i n m o e l l e r, KF, Thonke, F, Soehendra, N. Endoscopictreatment of Mirizzi’s syndrome. Gastrointest Endosc 1993;39:532-536.

10. Becker, CD, Hassler, H, Terrier, F. Preoperative diagnosis ofthe Mirizzi syndrome: Limitations of sonography andcomputed tomography. Am J Roentgenol 1984; 142:591-596.

11. Berland, LL, Lawson, TL, Stanley, RJ. CT appearance ofMirizzi syndrome. J Comput Assist Tomogr 1984; 8:165-166.

12. Baer, HU, Matthews, JB, Schweizer, WP, et al. Managementof the Mirizzi syndrome and the surgical implications ofcholecystocholedochal fistula. Br J Surg 1990; 77:743-745.

13. Dewar G, Chung SCS, Li AKC. Operative strategy inMirizzi syndrome. Surg Gynecol Obstet 1990; 171:157-159.

14. Fan ST, Lau WY, Lee MJR, et al. Cholecysto-hepaticodochalfistula: the value of pre-operative recognition. Br J Surg1985; 72:743-744.

15. Cotton PB. Endoscopic management of bile duct stones.Gut 1984; 25:587-597.

16. Siegel JH, Yatto RP. Biliary endoprosthesis for themanagement of retained bile duct stones. Am JGastroenterol 1984; 79:50-54.

17. Choi BW, Kim MJ, Chung JJ, et al. Rdiologic findings ofMirizzi with emphasis on MRI. Yonsei Med J 2000;41(1):144-146.

18. Vezakis A, Davides D, Birbas K, et al. Laparo s c o p i ctreatment of Mirizzi syndrome. Surg Endosc 2000; 10(1): 15-18.

19. Chowbey PK, Sharma A, Mann V, Khullar R, Baijal M,Vashistha A. The management of Mirizzi syndrome in thel a p a roscopic era. Surg Laparosc Endosc Percutan Te c h2000;10:11-14.

20. Targarona EM, Andrade, E, Balague, C, et al. Mirizzi’ssyndrome. Diagnostic and therapeutic controversies in thelaparoscopic era. Surg Endosc 1997; 11:842-845.

21. Sare M, Gurer S, Taskin V, et al. Mirizzi’s syndrome: Choiceof surgical procedure in the laparoscopic era. Surg LaparoscEndosc 1998; 8:63-67.

22. Binnie NR, Nixon SJ, Palmer KR. Mirizzi syndro m emanaged by endoscopic stenting and laparo s c o p i ccholecystectomy. Br J Surg 1992; 79:647.

23. England RE, Martin, DF. Endoscopic management ofMirizzi’s syndrome. Gut 1997; 40:272-276.

24. Tsuyuguchi T, Saisho H, Ishihara T, et al. Long-term follow-up after treatment of Mirizzi syndrome by pero r a lcholangioscopy. Gastrointest Endosc 2000; 52:6390-644.