What is the management of Mirizzi syndrome?

Management of Mirizzi Syndrome

Open cholecystectomy is the standard treatment for Mirizzi syndrome due to the high risk of bile duct injury with laparoscopic techniques. 1

Definition and Pathophysiology

• Mirizzi syndrome occurs when a gallstone 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
• It affects less than 1% of patients with gallstones and is considered a high-risk condition requiring thorough preoperative evaluation 1, 2
• The condition can progress from external compression to cholecystobiliary fistulation as inflammation becomes chronic 2

Classification

• The Csendes classification is widely used, describing five types of Mirizzi syndrome based on:
  • Presence of cholecystobiliary fistula
  • Severity of the fistula
  • Presence of cholecystoenteric fistula 2
• Common types include:
  • Type I (50%): External compression without fistula
  • Type II (25%): Cholecystobiliary fistula involving less than 1/3 of bile duct circumference
  • Type III (19.4%): Fistula involving up to 2/3 of bile duct circumference
  • Type IV (2.77%): Complete destruction of bile duct wall
  • Type V (2.77%): Any type plus cholecystoenteric fistula 3

Diagnosis

• Clinical presentation is often non-specific with symptoms including:

  • Right upper quadrant abdominal pain (69.4% of cases)
  • Jaundice (16.7% present with pain and jaundice)
  • Abnormal liver function tests (44.4% of patients) 3

• Diagnostic imaging includes:

  • Ultrasonography (first-line imaging but limited sensitivity)
  • MRCP (Magnetic Resonance Cholangiopancreatography) for better visualization
  • ERCP (Endoscopic Retrograde Cholangiopancreatography) - considered the gold standard for diagnosis with 100% sensitivity 3, 4
  • CT scan can help rule out malignancy 2

• Preoperative diagnosis is crucial as it guides surgical planning and improves outcomes, though many cases are diagnosed intraoperatively 2

Surgical Management

• Open cholecystectomy is the standard treatment with good short and long-term outcomes 1

• Laparoscopic approach considerations:

  • Generally not recommended due to high risk of bile duct injury 1
  • May be attempted in select cases of Type I Mirizzi syndrome by experienced surgeons 5
  • High conversion rate to open surgery (30.55%) 3
  • Should be converted to open whenever safe dissection cannot be guaranteed 1

• Surgical options based on type:

  • Type I: Total or subtotal cholecystectomy 2
  • Type II-IV: Subtotal cholecystectomy with repair of bile duct defect, which may include:
    • Direct repair with T-tube placement
    • Choledochoplasty
    • Bilioenteric anastomosis (hepaticojejunostomy) for more extensive defects 3, 2

Intraoperative Considerations

• Intraoperative cholangiography is recommended when biliary anatomy is unclear 1
• Dissection in Calot's triangle should be minimized to avoid bile duct injury 6
• Dense adhesions and distorted anatomy increase risk of complications 2
• Subtotal cholecystectomy is a safer option when total cholecystectomy cannot be performed safely 2

Complications and Follow-up

• Potential complications include:
  • Bile duct injury
  • Residual common bile duct stones
  • Late biliary strictures 6
• Close follow-up is necessary to monitor for late complications 6

Special Considerations

• Multidisciplinary collaboration improves diagnostic accuracy and treatment planning 2
• In rare cases, Mirizzi syndrome may be associated with gallbladder cancer, requiring more extensive surgery 5
• ERCP may be both diagnostic and therapeutic in some cases, allowing for stone removal and stent placement before definitive surgery 4