What is Mirizzi Syndrome?
Mirizzi syndrome is a rare complication of gallstone disease where a stone becomes impacted in the cystic duct or gallbladder neck, causing extrinsic compression of the common hepatic duct and resulting in obstructive jaundice. 1
Epidemiology and Pathophysiology
Mirizzi syndrome occurs in less than 1% of patients with gallstones, with reported frequencies ranging from 0.18% to 0.24% of all cholecystectomies 1, 2, 3
The condition develops when a gallstone becomes impacted in the cystic duct or neck of the gallbladder, creating an inflammatory response that leads to extrinsic compression of the common hepatic duct or common bile duct 1, 4
In more advanced cases, chronic inflammation can erode through the bile duct wall, creating a cholecystobiliary fistula (communication between the gallbladder and bile duct) 4, 5
Clinical Presentation
The most common presenting symptoms are abdominal pain (particularly right upper quadrant pain) and obstructive jaundice 4, 2, 3
Patients typically have prominent jaundice with elevated bilirubin levels 4
Fever may be present but is not universal 6
The clinical presentation is often nonspecific, making preoperative diagnosis challenging 2
Diagnostic Approach
Ultrasound examination typically reveals a large stone in the neck of the gallbladder or cystic duct 4
Endoscopic retrograde cholangiopancreatography (ERCP) is the most useful diagnostic tool, demonstrating a filling defect in the biliary tract at the cystic duct level with 100% sensitivity 4, 3
If a cholecystobiliary fistula is present, ERCP may show an excavated filling defect or blockage of the common duct 4
Magnetic resonance cholangiopancreatography (MRCP) can identify stones impacted in the cystic duct causing obstruction of the common hepatic duct by extrinsic compression 5, 6
CT scanning is also utilized in the diagnostic workup 2
Surgical Management
Open cholecystectomy is the current standard treatment for Mirizzi syndrome, with good short and long-term outcomes. 1
Type I Mirizzi Syndrome (Extrinsic Compression Only)
Laparoscopic cholecystectomy may be applicable in selected patients with type I disease (no fistula present) 2, 3
However, conversion rates from laparoscopic to open approach are high (67% in one series), even for type I cases 2
Five patients who had successful laparoscopic cholecystectomy all had type I disease 2
Type II Mirizzi Syndrome (With Cholecystobiliary Fistula)
When a cholecystobiliary fistula is present, partial cholecystectomy with choledochoplasty using a gallbladder flap is the treatment of choice 4
Alternative approaches include Roux-en-Y cholecysto-choledocho-jejunostomy for complex cases with large fistulous communications 5
Open cholecystectomy remains the standard for type II disease due to the complexity of bile duct reconstruction 2
Critical Surgical Considerations
Mirizzi syndrome is considered a contraindication for laparoscopic cholecystectomy due to the high risk of common bile duct injury 4
Conversion to open surgery should be considered whenever the operating surgeon cannot safely manage the procedure laparoscopically 1
Intraoperative cholangiography may be helpful when biliary anatomy is unclear 1
Severe inflammation and adhesion at Calot's triangle create potentially dangerous conditions during cholecystectomy 6
Early recognition of Mirizzi syndrome during surgery is crucial to avoid bile duct injury 1
Outcomes
There is typically no operative mortality when appropriately managed 2
Morbidity rates are approximately 31%, with complications ranging from minor (Clavien class I) to severe (Clavien class IV) 2
Mean hospitalization ranges from 7 to 9 days 2
Postoperative complications may include residual common bile duct stones 3
Long-term outcomes are generally good with appropriate surgical management 1