Mirizzi Syndrome-The Past, Present, and Future

Abstract

Mirizzi syndrome is a complication of gallstone disease caused by an impacted gallstone in the infundibulum of the gallbladder or within the cystic duct, causing chronic inflammation and extrinsic compression of the common hepatic duct or common bile duct. Eventually, mucosal ulceration occurs and progresses to cholecystobiliary fistulation. Numerous systems exist to classify Mirizzi syndrome, with the Csendes classification widely adopted. It describes five types of Mirizzi syndrome according to the presence of a cholecystobiliary fistula and its corresponding severity, and whether a cholecystoenteric fistula is present. The clinical presentation of Mirizzi syndrome is non-specific, and patients typically have a longstanding history of gallstones. It commonly presents with obstructive jaundice, and can mimic gallbladder, biliary, or pancreatic malignancy. Achieving a preoperative diagnosis guides surgical planning and improves treatment outcomes. However, a significant proportion of cases of Mirizzi syndrome are diagnosed intraoperatively, and the presence of dense adhesions and distorted anatomy at Calot's triangle increases the risk of bile duct injury. Cholecystectomy remains the mainstay of treatment for Mirizzi syndrome, and laparoscopic cholecystectomy is increasingly becoming a viable option, especially for less severe stages of cholecystobiliary fistula. Subtotal cholecystectomy is feasible if total cholecystectomy cannot be performed safely. Additional procedures may be required, such as common bile duct exploration, choledochoplasty, and bilioenteric anastomosis. Conclusions: There is currently no consensus for the management of Mirizzi syndrome, as the management options depend on the extent of surgical pathology and availability of surgical expertise. Multidisciplinary collaboration is important to achieve diagnostic accuracy and guide treatment planning to ensure good clinical outcomes.

Keywords: Mirizzi syndrome; bile duct injury; cholecystectomy; choledocholithiasis; cholelithiasis; laparoscopic; subtotal cholecystectomy.

Figure 1

A 72-year-old male was admitted with a clinical presentation suggestive of acute cholangitis. (a) Magnetic resonance imaging demonstrates a hilar stricture (labelled) with suspicion of cholangiocarcinoma. In view of concomitant cholecystitis and the need to evaluate for suspected malignancy, percutaneous gallbladder drainage was performed. (b) A percutaneous cholecystostomy tube is visible, and an endoscopic cholangiogram shows a smooth biliary stricture with brushings that do not show malignant cells. The patient was managed with open cholecystectomy, choledochectomy, and hepaticojejunostomy. The final histology was a benign biliary structure with cholecystitis.

Figure 2

Examples of MRCP findings in Mirizzi syndrome. (a) The first MRCP shows a filling defect (labelled) caudal to the confluence of the hepatic ducts with upstream biliary dilatation. The patient was a 67-year-old female with a background of Mirizzi syndrome treated with laparoscopic subtotal cholecystectomy at another hospital 10 months prior. She was subsequently diagnosed with a retained 19 mm cystic duct stone with a fistula in the CHD (Csendes Type III Mirizzi syndrome) and underwent laparoscopic CBD exploration with primary repair at our institution. (b) The second image illustrates an abrupt cut-off at the level of the CHD with a filling defect (labelled). This was taken from a 70-year-old man whose initial presentation of Mirizzi syndrome was that of acute pyogenic cholangitis. He subsequently underwent ERCP with biliary stenting, followed by laparoscopic CBD exploration, biliary stent removal, and primary closure of the CBD 8 weeks later.

Figure 3

Examples of ERCP findings in Mirizzi syndrome. (a) There is a filling defect (labelled) seen in the CBD. (b) There is a filling defect (labelled) in the CHD. (c) Biliary and pancreatic stenting during ERCP.

Figure 4

Management of Mirizzi syndrome according to Csendes classification. ^ Laparoscopic approach reserved for institutions with the necessary surgical expertise, with a low threshold for conversion to open surgery. ^^ Treatment according to the presence or absence and degree of cholecystobiliary fistula is depicted under types I–IV.

Figure 5

ERCP with cholangioscopy. (a) Endoscopic view of ampulla of Vater. (b) Cholangioscopy image of CBD narrowing with inflammation in a patient with Mirizzi syndrome. (c) CBD stricture due to cholangiocarcinoma in a patient initially suspected to have Mirizzi syndrome. (d) Sampling of biliary stricture using SpyBite forceps. (e) Black pigment of stone seen partly in the CHD and cystic duct. (f) Another view of the stone in (e). (g) The same stone in (e) being fragmented by SpyGlass electrohydraulic lithotripsy. (h) Fragmented stones after lithotripsy.