INTRODUCTION

Originally described by Kehr and Ruge, Mirizzi Syndrome (MS) isan uncommon complication of gallstone disease. Mirizzi laterreported it in 1948, and attributed the partial or complete biliaryobstruction to a physiological sphincter of the hepatic duct.1,2

We now recognize that there is no physiological sphincter of thehepatic duct.3,4 The syndrome is currently defined as a narrowingof the common hepatic duct produced by direct mechanicalcompression or inflammation resulting from the impaction of acalculus in the neck of the gall bladder or cystic duct. The presenceof four features including a parallel cystic duct, gallstonesimpacted in the neck of the gall bladder or cystic duct, commonhepatic duct obstruction, and recurrent cholangitis, have beennoted.5–8

There are several classifications of MS in the literature, butthe most consistent, proposed by Curet and Csendes,3,9 divides thesyndrome into two types. In type I, there is no additional com-munication between the gall bladder and the biliary ductalsystem, whereas in type II, there is a biliobiliary fistula from thegall bladder to the common hepatic duct with a stone impacted inthe fistula.10,11

Major bile duct injury occurring in patients with MS was rec-ognized in the open cholecystectomy era,12 and a high index of sus-picion is necessary. The common pattern of injury to the bileduct at laparoscopy results from mistaking the common duct for thecystic duct resulting in resection of a portion of the common and

hepatic ducts, and often there is an associated right hepatic arter-ial injury. A similar injury can occur with open cholecystec-tomy,13 but not with the same frequency or extent of hepaticduct resection.14

One percent of patients with a diagnosis of gallstones willhave MS.4,10,15 Painful jaundice or acute cholecystitis are the twocommon forms of presentation.16 Preoperative diagnosis is difficult,even at a specialized unit with supportive radiology and endoscopicretrograde cholangiopancreatography (ERCP) facilities avail-able.15 The risk of injury to the common bile duct (CBD) at thetime of cholecystectomy is increased without a preoperativediagnosis, especially with the laparoscopic approach.17

This study aims to evaluate the frequency, recognition, treatmentand subsequent outcomes of patients with MS from a prospectivestudy of all patients undergoing cholecystectomy since the intro-duction of laparoscopic cholecystectomy in a specialised uppergastrointestinal surgical unit.

METHODS

A prospective data base (Q&A 4.0; Symantec, Cupertino, CA,USA) of all patients having cholecystectomy performed orsupervised by one surgeon was begun in 1990. The databasewas set up to record patient demographics at presentation andpreoperative investigations, including precise ultrasound mea-surements to which are added operative and postoperativedetails as they occur. Review of the database revealed that out of1281 laparoscopic cholecystectomies performed over the 7 yearperiod, nine patients (0.7%) with MS were identified. Patientswere classified as having MS if there was evidence of biliaryobstruction on liver function tests and/or ultrasound, commonhepatic or intrahepatic duct dilatation and a normal CBD attribut-able to stones and not a tumour or stricture.

ANZ J. Surg. (2001) 71, 394–397

ORIGINAL ARTICLE

MIRIZZI SYNDROME: AN EXTRA HAZARD FOR LAPAROSCOPIC SURGERY

J. S. BAGIA, L. NORTH AND D. R. HUNT

St George Hospital, Sydney, New South Wales, Australia

Background: Mirizzi Syndrome (MS) is an important but uncommon complication of gallstones characterized by narrowing of thecommon hepatic duct (CHD) due to mechanical compression or inflammation. This study aimed to assess the impact of preoperativeand intraoperative diagnosis of MS on the performance, safety and efficacy of laparoscopic cholecystectomy.Methods: From a consecutive series of 1281 patients having surgery for gall bladder disease between 1990 and 1998, ninepatients with MS were identified from a prospective database and their clinical progress examined.Results: Five out of the nine patients with MS presented with pain (2/5 were also jaundiced), and four presented with acute chole-cystitis. Liver function tests were abnormal in all patients. Preoperative diagnosis of MS based on ultrasound was made in only twopatients, and in a third on findings of a nasobiliary cholangiogram. In six patients, the diagnosis was intraoperative. In sevenpatients cholecystectomy was completed by laparoscopy. Two patients needed conversion to open cholecystectomy. In twopatients the common bile duct was mistaken for the cystic duct and the error was recognized on relaxation of traction on the gall bladderin one, but in the other a duct injury occurred that was not recognized until the postoperative period.Conclusions: Preoperative diagnosis of MS is difficult, and a high index of suspicion is necessary to avoid serious complications.Once the diagnosis is known, successful laparoscopic management is possible but care should be taken to avoid duct injury.

Key words: bile duct injury, laparoscopic cholecystectomy, Mirizzi syndrome, ultrasound diagnosis.

Correspondence: Dr David R. Hunt, Level 5, Suite 1, St George PrivateMedical Centre, 1 South Street, Kogarah, NSW 2217, Australia.Email: davidrhunt@bigpond.com

Accepted for publication 16 February 2001.

Patient files and radiological investigations were thenreviewed for any changes between the initial assessment andadmission, particularly for those patients admitted acutely while onan elective waiting list. The radiological images were reviewed atthe time of this study to see if any features of MS were present thatmay have been missed in the preoperative assessment.

The data were analysed using one-way A N O V A comparing theMS subset with the group as a whole.

RESULTS

Of the nine patients diagnosed with MS in the 7 year period,seven were female and two were male. Median age was 44 yearswith a range from 20 to 66 years. All had abnormal liver functiontests but only two had a clinically significant elevation of bilirubin.

The MS group was significantly (P < 0.01) younger (mean 44.0 years) than the total laparoscopic cholecystectomy group(mean 53.5 years), but there was no significant differencebetween the MS group and the group with acute cholecystitis atpresentation. There was no significant difference between thethree groups in the number of stones, size of stones, or CBDsize. Gall bladder (GB) wall thickness was significantly greater in the group with acute cholecystitis compared to the totallaparoscopic cholecystectomy group (P < 0.001), as would beexpected, but there was no difference between the MS groupand the other two groups owing to a wide scatter of results.

Five patients presented with a history of recurrent colickyright upper quadrant pain, of whom two were jaundiced at pre-sentation, and four patients presented with acute cholecystitis.

All patients had abdominal ultrasound examinations per-formed as part of their preoperative assessment and the findingswere variable. The GB was thickened and contracted in fivepatients, and distended and relatively thin in the remaining four.There was evidence of cholelithiasis in eight out of the ninecases, with specific mention of a stone impacted in the neck of theGB in four cases, and stones in the cystic duct in one case. Nostones were seen in one case where the GB itself was very con-tracted and fibrotic.

A preoperative diagnosis of MS was suggested on the basis of ultrasound findings in only two of the nine cases. One ofthese had a contracted gall bladder and stones on ultrasound,with a CBD and common hepatic duct (CHD) diameter of 6.5 mmand no distal common duct seen. The other had a distended gallbladder with a large (17 mm) stone in the Hartmans pouch,together with dilatation of the CHD and changes consistentwith acute cholecystitis. In a third patient, the diagnosis of MS wasbased on findings of nasobiliary cholangiogram. On review of the radiology in the six undiagnosed patients, no additionalcases could be confidently identified as MS, although most hadsome of the radiological features commonly seen in MS onultrasound.

Three out of the four patients with a subacute presentationhad preoperative ERCP. In one patient the indication was presen-tation with pain, abnormal liver function tests and a dilatedCBD of 7.1 mm. In another, the ERCP was performed beforethe acute presentation to further assess a periportal cystic structureseen on computed tomography (CT) and ultrasound, which wasfound to be a diverticulum of the second part of duodenum. In thethird patient (a 20-year-old female with HIV infection), ERCP con-firmed the presence of dilatation in the biliary tree shown onscans but failed to identify a cause. A nasobiliary drain wasinserted and a repeat cholangiogram demonstrated a large ovoid

calculus impacted at the junction of the long cystic duct with theCBD, several small stones in the cystic duct and multiple stones inthe gall bladder.

In the nine patients, the laparoscopic approach was successful inseven and two required conversion to open surgery. The laparo-scopic approach was abandoned in one because of abnormalanatomy (Fig. 1). In the other, operative cholangiography showedextrinsic compression of the hepatic duct near the junction ofthe cystic duct with no distal filling; choledochotomy wasperformed laparoscopically and, although several stones wereremoved, the distal CBD could not be cleared. Hepaticojejunos-tomy was performed through a right subcostal incision.

In addition to the problems with ductal anatomy leading toconversion to open surgery, there were problems caused by theadherence of the GB to the CHD in two more patients managed bylaparoscopy. In both cases, traction on the neck of the GB led to theCBD being mistaken for the cystic duct. In one, this was recognizedon relaxation of traction on the neck of the GB but in the other, itled to unrecognized excision of a duct segment with the GB.

Three patients had complications. One patient had residualCBD stones managed by ERCP. Another developed right upperquadrant pain 2 days after cholecystectomy and laparoscopy

MIRIZZI SYNDROME 395

Fig. 1. Intraoperative cholangiogram illustrates abnormal ductanatomy increasing the risk of bile duct injury.

revealed a bile collection (1000 mL) from a leak from the Duct ofLushka on the inferior margin of the GB fossa that was con-trolled with a 3.0 Vicryl suture. The third developed fever andjaundice after surgery and percutaneous transhepatic cholan-giography revealed a complete duct transection requiring hepati-cojejunostomy. Despite a suggestive operative cholangiogram,the injury was not recognized at laparoscopy.

DISCUSSION

In this study, MS occurred in 0.7% of patients on the database inkeeping with a reported frequency of 0.7–1.4% of all patientsundergoing biliary surgery.9,10 However, the majority of ourcases were type I with only one of the nine being classified as typeII. This contrasts with other reports where only 11–30% wereclassified as type I.3,9 This difference is possibly a consequence ofthe different population groups studied. Alternatively, ourpatients presented earlier in their disease. Csendes suggestedthat MS and cholecystobiliary fistulae are different evolvingstages of the same disease process.9 The impacted GB/cysticduct stone initially causing obstructive jaundice as a type Ilesion then goes on to cause pressure necrosis, further acuteinflammation and erodes through to the adjacent bile duct toproduce a cholecystobiliary fistula. Our unit policy of earlyoperation in acute cholecystitis may circumvent progression to typeII pathology.

Ultrasound is the commonest screening investigation inpatients who present with biliary symptoms. Although there arelists of ultrasound features for MS, they have a low predictivevalue.3,11,18,19 Most commonly, a single large stone in the neck ofthe GB raises suspicion, but patients with multiple small stones mayalso develop MS if the stones become impacted in the cysticduct or neck of GB.3,9,11 After ultrasound, cholangiographyrather than CT is required.15 Endoscopic retrograde cholan-giopancreatography is the investigation currently recommended forfurther evaluation of the biliary tree.9–11,15 However, ERCP did notalert us to MS in the three patients who underwent the pro-cedure preoperatively in our series. In one patient, the test wascarried out before the onset of MS, and in another the findings wereinitially misinterpreted. Curet recommended the use of ERCP in thepreoperative assessment of a patient with cholecystitis and jaun-dice.3 Intravenous or percutaneous cholangiography have, in thepast, been advocated, but are no longer appropriate.20,21 Theroles of helical CT with intravenous contrast and magnetic reso-nance cholangiography remain to be evaluated.

Baer acknowledged the increased risk of bile duct injury atsurgery in MS.10 Removal of the entire GB bears a significant riskof ductal injury and partial cholecystectomy; leaving the neck of the GB is prudent in cases where the dissection is difficult.Common bile duct exploration is advocated only when it is tech-nically feasible. Postoperative endoscopic sphincterotomy andstone extraction is a safe alternative.

In type II, the fistulous defect may be large and severalmethods have been suggested for management. Direct suture of thedefect is associated with considerable morbidity and risksbiliary stricture. A gall bladder cuff has been used in primaryclosure with some success,15,20 but Roux-en-Y hepaticojejunostomyhas proved to be a safe procedure.10

Although successful laparoscopic surgery in the treatment of MS has been reported,22 it has also been suggested that MS is a contraindication to laparoscopic cholecystectomy.19 Unex-pected MS anatomy certainly presents a hazard to the CBD

during surgery.17 Difficulties were experienced in four out ofthe nine MS patients in this series. If the CBD is small ornormal in size, it can easily mimic the cystic duct. As seen in Fig. 1, lateral retraction on an instrument near the neck of the GB will elevate the CBD and, after a difficult preliminary dis-section, it may be seized with relief by the unsuspectingsurgeon. The CBD was elevated and dissected inadvertently in twoout of the nine patients in this series, resulting in an excisional ductinjury in one. There were no bile duct injuries in the 1272patients in this series without MS. Intraoperative cholangiographymay help to avert injury to important ductal structures. Basedon preoperative clinical findings, all of the MS patients in thisseries had cholangiography. Most reports have advocated earlyconversion to an open procedure for unexpected anatomy atlaparoscopic cholecystectomy.17 Paul et al. reported a recentcase of MS successfully managed by laparoscopic surgerywhere a transcystic flexible choledochoscope enabled theremoval of impacted stones.22

In the era of laparoscopic surgery, as preoperative diagnosis ofMS remains difficult, the use of intraoperative cholangiographytogether with a high index of suspicion is necessary to diagnose thecondition and to avoid duct injury. Preoperative diagnosis is notessential for the successful management of these patients andonce the diagnosis is made, successful laparoscopic manage-ment is still possible in the majority of cases.

REFERENCES1. Sutton JP, Sachatello CR. The confluence stone: a hazardous

complication of biliary tract disease. Am. J. Surg. 1967; 113:719–22.

2. Mirizzi PL. Sindrome del condusto hepatico. J. Int. Chir. 1948; 8:731–77.

3. Curet MJ, Douglas ER, Congilosi S. Mirizzi Syndrome in aNative American Population. Am. J. Surg. 1994; 168: 616–21.

4. Blumgart LH (ed.). Surgery of the liver and biliary tract.London: Churchill Livingstone, 1988; 721–52.

5. Ravo B, Epstein H, La Mendola S, Ger R. The Mirizzi Syn-drome: preoperative diagnosis by sonography and transhepaticcholangiography. Am. J. Gastroenterol. 1986; 81: 688–90.

6. Starling JR, Matallana RH. Benign mechanical obstruction ofthe common hepatic duct (Mirizzi syndrome). Surgery. 1980;88: 737–40.

7. Witte CL. Choledochal obstruction by cystic duct stone:Mirizzi syndrome. Am. Surg. 1984; 5: 241–3.

8. Morelli A, Narducci F, Ciccne R. Can Mirizzi Syndrome beclassified into acute and chronic form? Endoscopy. 1978; 10:109–12.

9. Csendes A, Diaz JC, Burchles P et al. Mirizzi syndrome andcholecystobiliary fistula: a unifying classification. Br. J. Surg.1989; 76: 1139–43.

10. Baer HU, Mathews JB, Schweizer WP et al. Management ofMirizzi syndrome and the implications of cholecystochole-dochal fistula. Br. J. Surg. 1990; 77: 743–5.

11. McSherry CK, Ferstenberg H, Virshop M. The Mirizzi Syn-drome: suggested classifications and surgical therapy. Surg.Gastroenterol. 1982; 1: 219–25.

12. Htoo MM. Surgical implications of stone impaction in the gallbladder neck with compression of the common hepatic duct(Mirizzi Syndrome). Clin. Radiol. 1983; 34: 651–5.

13. Andren-Sandberg A, Johansson S, Bengmark S. Accidentallesions of the common bile duct at cholecystectomy: II. Results oftreatment. Ann. Surg. 1985; 201: 452–5.

14. Davidoff AM, Pappas TN, Murray EA et al. Mechanisms ofmajor bile duct injury during laparoscopic cholecystectomy.Ann. Surg. 1992; 215: 196–202.

396 BAGIA ET AL.

MIRIZZI SYNDROME 397

15. Yip AW, Chow WC, Chan J, Lam KH. Mirizzi syndrome withcholecystocholedochal fistula: preoperative diagnosis and man-agement. Surg. 1992; 111: 335–8.

16. Ibrarullah MD, Saxena R, Sadiq SS, Kapoor V, Saraswat VA,Kaushik SP. Mirizzi’s: Identification and Management strategy.Aust. N.Z. J. Surg. 1993; 63: 802–6.

17. Posta CG. Unexpected Mirizzi anatomy – a major hazard to thecommon bile duct during laparoscopic cholecystectomy. Surg.Laparoscopy Endoscopy. 1995; 5: 412–4.

18. Dewbury KG. The features of Mirizzi syndrome on ultrasoundexamination. Br. J. Radiol. 1979; 52: 990–2.

19. Rust KR, Chancy TV, Warren G, Mertesdorf J, Maxwell JG.Mirizzi syndrome: a contraindication to coelioscopic cholecys-tectomy. J. Laparoendosc Surg. 1995; 1: 133–7.

20. Bower TC, Nagorney DM. Mirizzi syndrome. HPB Surg. 1988; 1:67–76.

21. Cruz FO, Barriga P, Tocornal J, Burhenne J. Radiology ofMirizzi syndrome: Diagnostic importance of the transhepaticcholangiogram. Gastrointest. Radiol. 1983; 8: 249–53.

22. Paul MG, Burns DG, McGiure AM, Thorfinnson HD,Schonelas H. Laparoscopic Surgery in the treatment of Mirizzisyndrome. J. Laparoendosc. Surg. 1992; 2: 157–63.

ANZ J. Surg. (2001) 71, 397

Neurosurgery in the Tropics: A Practical Approach toCommon Problems. By Jeffrey V. Rosenfeld and David A. K. Watters. Oxford: Macmillan Education, 2000. Illustrated;v + 473 pages. Includes index. ISBN 0-333-68412-5.

This timely book presents management plans for neurosurgical dis-eases and injuries in the developing world. Since many developingcountries lie between the Tropics of Cancer and Capricorn, the title is appropriate and tropical diseases are given proper consid-eration. However, the constraints on the tropical neurosurgeonare largely imposed by a lack of modern equipment and trainedstaff, and by cultural prejudices: in one word, by poverty.

The chief authors are both well-qualified to provide guide-lines in this difficult field. Jeffrey Rosenfeld is a leading Melbourneneurosurgeon, who has made regular neurosurgical visits toPapua New Guinea since 1992; he has also worked and taught inVietnam and China, and has served in Rwanda. David Watters isa general surgeon with much experience in Africa and in PapuaNew Guinea, where he held a professorial chair in surgery untilrecently. Among the six contributing authors, Lawrence Levy is thedoyen of neurosurgeons in Central Africa, and Ken Clezy has along record of surgical achievement in Papua New Guinea.

The authors cover the whole field of neurosurgery as it ispractised in developed countries, and put each clinical problemwithin the perspective of the general surgeon working in adeveloping country with no special training and no access tomodern neuroradiology. Levy reminds us that there may still be aplace for ventriculography and old-style angiography. In addi-tion, the book covers diagnostic problems and operations thatmost Australasian neurosurgeons would see as outside theircapacities; such as snakebite, scoliosis surgery and simple mas-toidectomy. HIV infection and tuberculosis are well-discussedby J. E. Jellis of Lusaka. He provides a convincing decisionalgorithm for the treatment of spinal tuberculosis. It is deeplysaddening to read that in sub-Sahara Africa, some 50% of tuber-culosis victims are now also HIV-positive. Reactions to anti-

tuberculous drugs have increased, costs of streptomycin therapymay be prohibitive and anterolateral spinal decompression may be unavailable. Rehabilitation is discussed, but the authors areaware that this is often a dark side of medicine in the developingworld, and they do not shirk from pointing out that survival with severe physical disability may be an intolerable burden foreveryone concerned. The chief topics are well referenced, withrecommendations for further reading.

It has to be said that the book has blemishes, some of whichcould have been prevented by a good publisher’s reader, if such stillexist. There are several redundancies; thus, a 30-line account ofcervical foraminotomy is reproduced word for word in a laterchapter. In the otherwise excellent section on peripheral nerveinjuries, Seddon’s well-known terminology is given with novelspellings and confusing definitions. A pedant would complainthat Jacksonian epilepsy does not commemorate an Americanneurologist. Some of the authors’ recommendations can bedebated; I myself would not prohibit percutaneous shunt puncturein diagnosing infection or blockage, when the alternative is apossibly unnecessary exploration. But these criticisms are of nogreat moment, and are mentioned only in the hope that therewill soon be another edition.

I warmly recommend this book to any young neurosurgeongoing to work in a tropical country, as a book of reference and still more for its stimulating philosophy. I wish that I could haveread it long ago when I first tried to be useful in a developingcountry. I believe that it will also be very helpful for isolatedgeneral surgeons, to show that many life-saving neurosurgicalprocedures are not insuperably difficult, and to underline thedesirability of establishing a partnership with a neurosurgicalcentre for advice and for referral of selected cases. The bookshould also be studied by overseas aid administrators and byanyone who doubts the need for neurosurgical services in thedeveloping world.

Burnside, South Australia DONALD SIMPSON

BOOK REVIEW