March 2006

KUWAIT MEDICAL JOURNAL

Review Article

Mirizzi Syndrome: A Review of the Literature

George J Xeroulis, Ward Davies
Department of Surgery, University of Western Ontario, London, Ontario, Canada
Kuwait Medical Journal 2006, 38 (1): 3-6

INTRODUCTION Mirizzi syndrome is a rare cause of obstructive jaundice. This entity should be considered in the differential diagnosis of all patients with obstructive jaundice. Failure to recognize the condition preoperatively can result in a major bile duct injury, particularly during laparoscopic surgery[1]. The syndrome refers to obstruction of the common hepatic duct by extrinsic compression usually from a gallstone impacted in Hartmanns pouch or the cystic duct. Large gallstones that become impacted in this area produce common hepatic duct obstruction by two mechanisms: mechanical obstruction by direct compression of the common hepatic duct, or they can cause obstruction secondary to repeated bouts of local inflammation. In 1948, Argentinean surgeon Pablo Luis Mirizzi, first described a syndrome of common hepatic duct obstruction in the setting of longstanding cholelithiasis and cholecystitis[2]. The classic description of the disease includes four components: (a) a close parallel course of the cystic duct and the common hepatic duct, (b) an impacted stone in the cystic duct or the neck of the gallbladder, (c) common hepatic duct obstruction secondary to external compression by the cystic duct stone (and the surrounding inflammation), and (d) jaundice, with or without cholangitis. Mirizzis syndrome is a rare complication of cholelithiasis, with an estimated incidence of 0.052.7%[1,3,4]. It presents as a spectrum of disease that varies from extrinsic compression of the common hepatic duct to the presence of a cholecystobiliary fistula. Often, this dangerous alteration to anatomy is not recognized preoperatively, and has the potential to lead to significant morbidity and biliary 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 therapeutic procedure. The original classification, by McSherry et al[5], described two types. Type I referred to compression of the common hepatic duct by a stone impacted in the cystic duct or Hartmanns pouch. Type II referred to erosion of the calculus from the cystic duct into the common hepatic duct, producing a cholecystocholedochal fistula. Csendes et al[6] created a second classification taking into account the extent of fistula. Type I remained the same, external compression of the common hepatic duct due to a stone impacted at the neck of the gallbladder or at the cystic duct. Types II to IV lesion referred to the presence and extent of a cholecystobiliary (cholecystohepatic or cholecystocholedochal) fistula, due to erosion of the anterior or lateral wall of the common hepatic duct by impacted stones. The fistula involved less than one-third of the circumference of the common hepatic duct in type II. Involvement of between one-third and two-thirds of the circumference of the common hepatic duct was called a type III lesion, while destruction of the entire wall of the common hepatic duct was called a type IV lesion. In their original paper, a total of 219 patients were identified with Mirizzis syndrome. The incidence of type I lesions was 11 per cent, type II, 41 per cent, type III, 44 per cent and type IV, four per cent. The majority had obstructive jaundice. The third classification, proposed by Nagakawa and colleagues[7], expanded upon the definition of the Mirizzi syndrome. Type I referred to a stone impacted in the cystic duct or gallbladder neck. Type II was characterized by a fistula of the common duct. Type III was defined by hepatic duct stenosis due to a stone at the confluence of the hepatic and cystic ducts. Type IV was characterized by hepatic duct stenosis as a complication of cholecystitis in the absence of calculi impacted in the cystic duct or gallbladder neck. In one series of 30 patients, the frequency of these four types as described by Nagakawa et al was 14, 2, 6, and 8%, respectively[8].

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

Mirizzi Syndrome: A Review of the Literature

March 2006

Table 1: Various Classification Systems of Mirizzis Syndrome McSherry

Type I
Extrinsic compression of Type I the common hepatic duct by stones generally impacted in the cystic duct or in the infundibulum of the gallbladder Presence of cholecystobiliary fistula

Csendes
Extrinsic compression of the Type I common hepatic duct by stones generally impacted in the cystic duct or in the infundibulum of the gallbladder

Nagakawa
Extrinsic compression (stenosis) of the common hepatic duct by stones generally impacted in the cystic duct or in the infundibulum of the gallbladder Fistulization of common hepatic duct from a stone impacted in the cystic duct or in the infundibulum of the gall bladder Common hepatic duct stone at the cystic duct-hepatic duct confluence Common hepatic duct stenosis caused by cholecystitis without stones in the cystic duct or infundibulum of the gallbladder

Type II Presence of cholecystobiliary Type II

fistula with diameter one third of circumference of the common hepatic duct wall

Type II

Type III Presence of cholecystobiliary Type III

fistula with diameter two third of circumference of the common Type IV hepatic duct wall

Type IV Presence of cholecystobiliary

fistula which involves the entire circumference of the common hepatic duct wall

Mirizzi syndrome is part of the differential diagnosis of all patients with obstructive jaundice, and requires a high index of suspicion. Most patients present with jaundice, and right upper quadrant pain[1]. Elevations in the serum concentrations of alkaline phosphatase and bilirubin are present in over 90 per cent of patients[8,9]. The clinical and laboratory findings are similar to patients who present with obstructive jaundice secondary to choledocholithiasis. Once a diagnosis of obstructive jaundice has been made an abdominal ultrasound is often the first imaging test preformed. Imaging generally reveals gallstones, dilated intrahepatic ducts, with a long parallel cystic duct and a contracted gallbladder[10]. The presence of a stone impacted in the gallbladder neck and an abrupt change to a normal width of the common duct below the level of the stone are also very suggestive of Mirrizis syndrome. The sensitivity of ultrasound in detecting Mirizzis syndrome is 23-46%[3,4]. In Csendes series, ultrasound revealed dilated ducts in 81% of patients and raised suspicion of Mirizzis syndrome in only 27% of cases. CT scanning has a similar sensitivity to ultrasound, but can be helpful in diagnosing other causes of obstructive jaundice such as gallbladder cancer, cholangiocarcinoma, or metastatic tumor [11]. CHOLANGIOGRAPHY Direct cholangiography is usually necessary to establish the correct diagnosis and to delineate the hepatic duct anatomy[10]. Pre-operative diagnosis is

essential in avoiding CBD injuries[12,13,14]. If it was unexpectedly encountered at the time of surgery, a cautious approach should be taken. Periductal inflammation and the potential for a cholecystocholedochal fistula make a trial dissection particularly challenging and should only be undertaken by an experienced surgeon. Additional imaging is often needed to obtain details of the biliary anatomy. Intraoperative cholangiogram or closing and obtaining a postoperative ERCP or MRCP should be considered. Cholangiography (intraoperative or ERCP) as well as MRCP will allow for an accurate assessment of anatomy and classification of the type of Mirizzis syndrome (Fig. 1). The possibility of stone retrieval and biliary stenting during ERCP is an added advantage in improving surgical outcome, and stenting also facilitates identification of the CBD during operative dissection [7,15,16]. When ERCP is unsuccessful or difficult, percutaneous transhepatic cholangiography (PTC) is a viable alternative. MRCP and ERCP are equivalent in their ability to diagnosis and to delineate details of biliary strictures, and to detect a cholecystocholedochal fistula [4]. In addition, T2 weighted images can differentiate a neoplastic mass from an inflammatory one which US or CT scan may not be capable of [17]. Early ERCP is preferred when biliary sepsis is the dominant clinical issue and where a beneficial endoscopic therapeutic procedure can be instituted at the same time. By contrast, MRCP is used in the non-septic patient to corroborate the suspicion of malignancy or stones after initial imaging with US or CT scans[4].

March 2006

KUWAIT MEDICAL JOURNAL

l Type I - Cholecystectomy plus common bile duct exploration with T-tube placement. Exploration should be performed only if the CBD is easily exposed. l Type II - Suture of the fistula with absorbable material or choledochoplasty with the remnant gallbladder. l Type III - Choledochoplasty; suture of the fistula is not indicated. l Type IV - Bilio-enteric anastomosis is preferred since the entire wall of the common bile duct has been destroyed. l The approach may vary with the type of fistula present; both the operative mortality and postoperative morbidity increase according to the severity of the lesion [6].

Fig. 1: Endoscopic retrograde cholangiopancreatography (ERCP) of patient with obstructive jaundice and Mirizzis syndrome. Notice impacted stone in cystic duct causing obstruction of common hepatic duct. Adapted from UptoDate Mirizzi Syndrome James B McGee.

TREATMENT Surgery is the mainstay of therapy of Mirizzi syndrome, the dense inflammatory reaction in Calots triangle, as well as the frequent aberrant biliary anatomy, pose a difficult challenge to the unsuspecting surgeon when dealing with a Mirizzi syndrome. The two principal aims are (a) the safe completion of cholecystectomy without injuring the biliary system and (b) the appr opriate management of the cholecystocholedochal fistula. Meticulous dissection and vigilance are essential in order to avoid inadvertent bile duct injury. If the diagnosis of Mirizzi syndrome is made preoperatively, an operative strategy that minimizes the risk of injury to the biliary tract can be carried out. However, a preoperative diagnosis of Mirizzi syndrome is seldom made because ERCPand direct cholangiography are not widely used. ERCP, direct cholangiography, or magnetic resonance cholangiography should be performed in patients with clinical jaundice and signs and symptoms suggestive of biliary obstruction. A standardized surgical approach has been recommended based on the Csendes classification of the variants of Mirizzi syndrome [6]:

LAPAROSCOPIC SURGERY The Mirizzi syndrome presents a difficult challenge for laparoscopic surgery because the dense adhesions and edematous inflammatory tissue cause distortion of the normal anatomy and increase the risk for biliary injury. While it appears to be feasible, especially for type I anatomy[18,19], the routine use of laparoscopic surgery as the primary treatment of Mirizzi syndrome is controversial[20,21]. It has been suggested, that a prudent approach for type 1 Mirizzi syndrome is to perform a trial laparoscopic dissection, but to have a low threshold to convert to an open procedure. This approach should be undertaken only by experienced laparoscopic surgeons[18,20]. ENDOSCOPIC THERAPY Endoscopic treatment with or without electrohydraulic lithotripsy (EHL) can be effective as a temporizing measure before surgery and can be definitive treatment for unsuitable surgical candidates[9,22,23]. One report described the experience with 14 patients with Mirizzi syndrome treated with EHL[9]. Twelve patients had a single stone and complete clearance was achieved with one treatment session; two had multiple stones and required an additional treatment session. Asymptomatic leakage of contrast medium from the cystic duct into the peritoneal cavity was observed in one patient after removal of a large impacted cystic duct stone. This patient recovered with conservative therapy and suffered no adverse events. In another series of 25 patients with cholangiographic evidence of Mirizzi syndrome, 12 were referred for surgery after preliminary endoscopic therapy and 13 were treated solely with endoscopy[23]. Stones were completely removed in three and nine were treated with long-term stents; complications occurred in four patients [23].

Mirizzi Syndrome: A Review of the Literature

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March 2006

Endoscopic treatment of Mirizzi syndrome should be used as a temporizing measure before surgery. It can serve as a definitive treatment for those patients who are unsuitable surgical candidates when further endoscopic attempts can be made to disimpact and remove the stones. Longterm success appears to be most likely in patients with type II disease who do not have residual gallbladder stones[24]. CONCLUSION Mirizzi syndrome is a rare complication of cholelithiasis and requires a high index of suspicion in the setting of obstructive jaundice. Diagnosis preoperatively may be elusive with bloodwork, US and CT alone. Cholangiography (intraoperative and ERCP) as well as MRCP aids in both the diagnosis and identification of anatomy and may prevent serious biliary injury. Surgery is the mainstay of therapy of Mirizzi syndrome, and requires the safe completion of cholecystectomy without injuring the biliary system and the appropriate management of the cholecystocholedochal fistula. The aberrant anatomy intrinsic to this syndrome presents a difficult challenge to surgeons and the laparoscopic approach should be undertaken with caution and probably left to specialized minimally invasive centres. Endoscopic treatment may be effective as a temporizing measure before surgery and can be definitive treatment for unsuitable surgical candidates. REFERENCES
1. Waisberg J, Corona A, de Abreu IW, Farah JFM, Lupinacci RA, Goffi FS. Benign Obstruction of the Common Hepatic Duct (Mirizzi Syndrome): diagnosis and operative management. Arq Gastroenterol 2005; 42:13-18. Mirizzi, PL. Syndrome del conducto hepatico. J Int de Chir 1948; 8:731-733. Yeh, CN, Jan, YY, Chen, MF. Laparoscopic treatment for Mirizzi syndrome. Surg Endosc 2003; 17:1573-1578. Chan CY, Liau KH, Ho CK, Chew SP. Mirizzi syndrome: a diagnostic and operative challenge. Surgeon 2003;1:273-278. McSherry, CK, Ferstenberg, H, Virshup, M. The Mirizzi syndrome: Suggested classification and surgical treatment. Surg Gastroenterol 1982; 1:219-225.

7.

8.

9.

10.

11. 12.

13. 14.

15. 16.

17.

18.

19.

20.

21.

2. 3. 4. 5.

22.

23. 24.

Csendes, A, Diaz, CJ, Burdiles, P, et al. Mirizzi syndrome and cholecystobiliary fistula: A unifying classification. Br J Surg 1989; 76:1139-1143. Nagakawa, T, Ohta, T, Kayahara, M, et al. A new classification of Mirizzi syndrome from diagnostic and therapeutic viewpoints. Hepatogastroenterology 1997; 44:63-67. Ibrarullah, M, Saxena, R, Sikora, SS, et al. Mirizzis syndrome: Identification and management strategy. Aust N Z J Surg 1993; 63:802-806. Binmoeller, KF, Thonke, F, Soehendra, N. Endoscopic treatment of Mirizzis syndrome. Gastrointest Endosc 1993; 39:532-536. Becker, CD, Hassler, H, Terrier, F. Preoperative diagnosis of the Mirizzi syndrome: Limitations of sonography and computed tomography. Am J Roentgenol 1984; 142:591-596. Berland, LL, Lawson, TL, Stanley, RJ. CT appearance of Mirizzi syndrome. J Comput Assist Tomogr 1984; 8:165-166. Baer, HU, Matthews, JB, Schweizer, WP, et al. Management of the Mirizzi syndrome and the surgical implications of cholecystocholedochal fistula. Br J Surg 1990; 77:743-745. Dewar G, Chung SCS, Li AKC. Operative strategy in Mirizzi syndrome. Surg Gynecol Obstet 1990; 171:157-159. Fan ST, Lau WY, Lee MJR, et al. Cholecysto-hepaticodochal fistula: the value of pre-operative recognition. Br J Surg 1985; 72:743-744. Cotton PB. Endoscopic management of bile duct stones. Gut 1984; 25:587-597. Siegel JH, Yatto RP. Biliary endoprosthesis for the management of retained bile duct stones. Am J Gastroenterol 1984; 79:50-54. Choi BW, Kim MJ, Chung JJ, et al. Rdiologic findings of Mirizzi with emphasis on MRI. Yonsei Med J 2000; 41(1):144-146. Vezakis A, Davides D, Birbas K, et al. Laparoscopic treatment of Mirizzi syndrome. Surg Endosc 2000; 10(1): 1518. Chowbey PK, Sharma A, Mann V, Khullar R, Baijal M, Vashistha A. The management of Mirizzi syndrome in the laparoscopic era. Surg Laparosc Endosc Percutan Tech 2000;10:11-14. Targarona EM, Andrade, E, Balague, C, et al. Mirizzis syndrome. Diagnostic and therapeutic controversies in the laparoscopic era. Surg Endosc 1997; 11:842-845. Sare M, Gurer S, Taskin V, et al. Mirizzis syndrome: Choice of surgical procedure in the laparoscopic era. Surg Laparosc Endosc 1998; 8:63-67. Binnie NR, Nixon SJ, Palmer KR. Mirizzi syndrome managed by endoscopic stenting and laparoscopic cholecystectomy. Br J Surg 1992; 79:647. England RE, Martin, DF. Endoscopic management of Mirizzis syndrome. Gut 1997; 40:272-276. Tsuyuguchi T, Saisho H, Ishihara T, et al. Long-term followup after treatment of Mirizzi syndrome by peroral cholangioscopy. Gastrointest Endosc 2000; 52:6390-644.