Pablo Mirizzi (Fig. 1), a professor of surgery, was born in Cordoba, Argentina, on January 25, 1893, and he died in Cordoba on August 28, 1964.1 His name is connected with the first intraoperative cholangiography, performed in 1931 on a patient named Edwig Bustos de Lara during the removal of common bile duct stones. This type of biliary imaging came during a critical point, when the complications following biliary surgery became extremely serious.2 The problem of extraluminal compression and partial obstruction of common bile duct provoked by the impacted stone still existed in 1905, when it was described by the famous German surgeon Hans Kehr, and it also was described 3 years later in 1908 by another German, Ruge.2

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In 1948 Mirizzi described a condition involving a rare benign cause of obstructive jaundice that was provoked by stone impacted in either the Hartmann pouch or the cystic duct, thereby presenting obstruction of the common hepatic duct (Fig. 2).4

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In modern surgery, the crucial point of the problem and its resolution came in the report of Corlette and Bismuth in 1975,5 in which they presented 24 patients and described two types of cholecystocholedochal fistulas: type I, with a fistula between the gallbladder and the common hepatic bile duct, and type II, in the “trajectory of cystic duct.” They also explained very clearly the pathogenesis of cholecystocholedochal fistula. It starts with a longstanding history of gallstones impacted at the gallbladder neck or cystic duct, as well as inflammation of the gallbladder wall. The inflammatory gallbladder adheres or even fuses to the adjacent bile duct.6 The stone is under intraluminal pressure, protruding into the bile duct and, later, eroding the wall, and in excessive cases the stone is expelled into the bile lumen, causing the signs and symptoms of obstructive jaundice in both type I and type II fistulas.

In 1989 Csendes et al7 classified Mirizzi syndrome (MS) into 4 types (schemes; Fig. 3). MS is uncommon, and it is reported in 0.3% to 3% of patients undergoing cholecystectomy. In the diagnosis of MS, the most common symptoms are jaundice, in 60% to 100% of patients, and abdominal pain, in 50% to 100% of patients.8 We used to do all modern preoperative and intraoperative measurements, such as endoscopic retrograde cholangiopancreatography, transhepatic cholangiography, and magnetic resonance cholangiopancreatography. But, unfortunately, the preoperative diagnosis can be made only in 8% to 62.5% of instances, and intraoperative recognition should be essential (Fig. 4).9

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The treatment of type I MS without cholecystocholedochal fistula is cautiously and precisely done by cholecystectomy. In extreme cases, some authors advise subtotal cholecystectomy, with closure of the remaining gallbladder cuff with the T-tube insertion if necessary for temporary decompression of common bile duct.10 For types II and III MS with cholecystocholedochal fistula, the treatment guidelines are not so strictly defined, but the essential part is to divide the fistula and perform the cholecystectomy. Next, the choledochus must be reconstructed, followed by T-tube insertion, choledochojejunoanastomosis, or hepaticojejunoanastomosis, the latter two being better, in our opinion. In patients at risk of and for those with advanced jaundice, endoscopic papillotomy, or gallstone extraction, stent insertion could be performed. For patients in whom endoscopic treatment failed, percutaneous transhepatic treatment could be tried. In approximately 6% to 27% of patients with MS, carcinoma of the gallbladder turned out to be the final diagnosis. Therefore, the frozen section is obligatory during the cholecystectomy in such patients.

Materials and Methods

We analyzed two groups of patients undergoing cholecystectomy in two surgical units in Serbia: the Surgical Clinic of the KB Center Zvezdara in Belgrade, and the Department of Surgery of the Regional Hospital in Zrenjanin. In the Surgical Clinic of the KB Center Zvezdara in Belgrade, in 2005 we performed 535 cholecystectomies, more than 60% of them laparoscopically, and among these there were registered 4 patients with MS, which means the incidence was 0.75%. In 2009 in Zrenjanin, 332 cholecystetomies were performed, mostly laparoscopically, and among them we found only 2 patients with MS. The incidence here is 0.60%, practically identical to that in Belgrade. All 4 patients in Belgrade were female, and in Zrenjanin there was 1 male and 1 female patient. The operations performed were 3 hepaticojejunoanastomosis procedures and 3 choledochoplasty procedures followed by T-tube insertion (Fig. 5).

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Results

Analyzing the two groups of patients who underwent a cholecystectomy laparoscopically (or, rarely, laparotomically) to detect MS, we found that in Belgrade and Zrenjanin, the incidence of MS was very low (0.63% and 0.75%, respectively). In Zrenjanin, there were 2 hepaticojejunoanastomosis operations performed; in Belgrade, there was 1 hepaticojejunoanastomosis operation and 3 choledochoplasty operations with T-tube insertion performed.

There was no postoperative mortality, nor were there any serious surgical complications, such as biliary fistula or stenosis of the common bile duct.

Discussion

We found in two surgical centers a rather low incidence of MS, accepting that MS exists only in cases with biliar fistula.14 We used the Bismuth definition of MS, practically concerning only patients having more or less apparent cholecystocholedochal or hepatic fistula. We did not find type I MS in our retrospective study.15,16 It can be difficult sometimes to identify intraoperatively the types of MS differentiated by Csendes and other authors, preferring the four- or three-type syndrome classifications. In surgical practice there is only one benchmark for this syndrome: whether or not there exists any kind of biliary fistula,17 either cholecystocholedochal or cholecystohepatic. The fistula diagnosis18,19 is the important step, but the appropriate treatment is the most important part of MS. In the nineteenth and the first half of the twentieth centuries, the gold standard in biliary reconstruction was a very conservative approach—choledochoplasty plus T-tube drainage—but now it is preferable to perform hepaticojejunostomy as the most convenient operation.20 There have been reports about postoperative stenosis after primary choledochoplasty and T-tube repair, and then biliodigestive anastomosis.21 In our series, in Zrenjanin they held that rule and performed 2 biliodigestive anastomoses in 2 patients as a definite solution for the problem, contrary to the Belgrade group, where a biliodigestive anastomosis was performed in only 1 patient, and in the 3 others choledochoplasty with T-tube drainage of choledochus was performed. Of course, it depends on the pathologic substrata width of choledochal defect and pericholedochal tissue, as well as the surgeon's skill.22 The problem of concomitant carcinoma of the gallbladder is always present because the tissue is always destroyed by the chronic inflammation, and it is sometimes very difficult to tell carcinoma of the gallbladder from the benign inflammatory processes. There are authors who found 24% carcinoma rates in patients with MS.23 It must always be kept in mind during the reconstruction that taking a few frozen sections of the tissue could avoid serious misunderstandings and surprises. This must be the guideline for such types of biliary surgery—to always have the option of pathologic examination of surgical substrata available during the operation. In patients with gallbladder carcinoma, one needs to perform a radical surgical treatment, even with liver resection, and therefore the surgeon performing the MS operation needs to be skilled enough to perform larger and more radical resections in the hepatopancreatic region.

Conclusion

The preoperative diagnosis of MS is a challenge. We have accepted the Csendes classification of MS. Open surgery is the gold standard for this condition. MS is still a contraindication and is hazardous for laparoscopic surgery.