What is Mirizzi syndrome?

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 or common bile duct, resulting in obstructive jaundice. 1

Epidemiology and Prevalence

• Mirizzi syndrome occurs in less than 1% of patients with gallstones, making it an uncommon but clinically significant complication 1, 2
• The reported frequency ranges from approximately 0.18% to 1% among all cholecystectomy patients 3, 2

Pathophysiology

• The syndrome develops when a gallstone becomes impacted in the cystic duct or Hartmann's pouch (the neck of the gallbladder), creating mechanical compression of the adjacent common hepatic duct 1, 4
• Chronic inflammation from the impacted stone leads to an inflammatory response that can progress to fistula formation between the gallbladder and bile duct in more advanced cases 5, 6
• The condition is classified into types based on severity, with Type I involving only extrinsic compression and Type II involving cholecystobiliary fistula formation 3, 5

Clinical Presentation

• The most common presenting symptoms include:
  • Obstructive jaundice (intermittent or constant) 1, 2
  • Upper abdominal pain 3, 6
  • Fever in some cases 6
• The syndrome is most commonly observed in elderly patients with long-standing gallstone disease 4

Diagnostic Approach

• Ultrasound examination typically reveals a large stone in the cystic duct or gallbladder neck compressing the common hepatic duct 2, 5
• Magnetic resonance cholangiopancreatography (MRCP) is essential for diagnosis and can demonstrate the impacted stone causing bile duct obstruction 2, 4
• Endoscopic retrograde cholangiopancreatography (ERCP) may show a filling defect in the biliary tract at the cystic duct level 5, 4
• Most cases are not identified preoperatively, making intraoperative recognition crucial 2, 1

Treatment and Management

• Open cholecystectomy is the current standard treatment for Mirizzi syndrome, with good short and long-term outcomes 1
• Laparoscopic cholecystectomy may be applicable only in highly selected patients with Type I disease, but carries a high conversion rate (67%) and risk of bile duct injury 3, 5
• Intraoperative cholangiography should be performed when biliary anatomy is unclear to prevent complications 1
• Conversion to open surgery must be considered whenever the surgeon cannot safely manage the procedure laparoscopically 1

Important Clinical Caveats

• Mirizzi syndrome is considered a contraindication for laparoscopic cholecystectomy due to the high risk of common bile duct injury from severe inflammation and adhesions at Calot's triangle 5, 6
• Early recognition during surgery is crucial to avoid bile duct injury, requiring thorough preoperative evaluation 1
• In cases with cholecystobiliary fistula (Type II), partial cholecystectomy with choledochoplasty using a gallbladder flap may be the treatment of choice 5