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Mirizzi Syndrome

Definition

  • Mirizzi syndrome is an uncommon condition characterized by extrinsic obstruction of the common hepatic duct due to an impacted gallstone in the gallbladder infundibulum (Hartmann pouch) or the cystic duct.
  • Occurs in 0.3% to 3% of patients undergoing cholecystectomy.

Pathogenesis

  • An impacted stone causes inflammation and compression of the bile duct.
  • Prolonged inflammation may lead to erosion and formation of a cholecystocholedochal fistula (between the gallbladder and common hepatic duct).

Classification

  • Type I: External compression of the common hepatic duct by an impacted stone.
  • Type II: Erosion of the gallstone into the common hepatic duct, creating a fistula.
    • Type IIa: Involvement of less than one-third of the bile duct circumference.
    • Type IIb: Involvement of up to two-thirds.
    • Type IIc: Complete erosion of the bile duct wall.
  • Type V: Presence of a cholecystoenteric fistula (with or without gallstone ileus) alongside any of the other types.

Clinical Features

  • Symptoms of acute cholecystitis (right upper quadrant pain, fever).
  • Jaundice due to bile duct obstruction.
  • Elevated bilirubin and alkaline phosphatase levels.
  • Possible cholangitis if infection develops.

Imaging and Diagnosis

  • Ultrasound:
    • Dilated biliary tree proximal to the gallbladder neck.
    • Impacted gallstone at the gallbladder infundibulum or cystic duct.
    • Abrupt change in common duct caliber below the stone.
  • Computed Tomography (CT):
    • Eccentrically located impacted gallstone.
    • Dilated proximal biliary system with normal downstream ducts.
    • Irregular cavity with surrounding edema near the gallbladder neck.
  • Magnetic Resonance Cholangiopancreatography (MRCP) or Direct Cholangiography (ERCP/PTC):
    • Detailed visualization of the obstruction and any fistula.
  • Preoperative diagnosis is crucial for appropriate surgical planning.

Complications

  • Formation of cholecystobiliary and cholecystoenteric fistulae.
  • Increased risk of gallbladder carcinoma.
  • Development of biliary strictures.

Management

  • Open cholecystectomy is the gold standard treatment.
    • Type I:
      • Standard cholecystectomy may suffice.
      • If inflammation is severe, a partial cholecystectomy may be necessary.
    • Type II:
      • May require biliary reconstruction due to bile duct erosion.
      • Options include:
        • Primary repair of the bile duct.
        • T-tube placement for bile drainage.
        • Roux-en-Y hepaticojejunostomy for biliary-enteric drainage.
  • Laparoscopic approach is generally not recommended due to high conversion and complication rates.
  • Endoscopic interventions:
    • ERCP can aid in diagnosis and may provide biliary decompression.
    • Placement of biliary stents to assist during surgery.

Surgical Considerations

  • Inflammation and fibrosis can distort the anatomy of the triangle of Calot, increasing the risk of bile duct injury.
  • Preoperative imaging is essential to identify anatomical variations and plan surgery.
  • In severe cases, subtotal cholecystectomy may be the safest option.

Cholecystoenteric Fistula

  • Definition: A fistula between the gallbladder and an adjacent hollow organ (usually the duodenum or transverse colon).
  • Etiology: Result of gallbladder perforation due to acute cholecystitis.
  • Clinical Features:
    • Pneumobilia (air in the biliary tree).
    • Cholangitis from bacterial contamination.
    • Gallstone ileus causing small bowel obstruction.
    • Chronic diarrhea in cholecystocolonic fistula.
  • Management:
    • Cholecystectomy with fistula takedown and closure.
    • Enterotomy to remove obstructing stones in gallstone ileus.
    • Surgical approach depends on patient stability and inflammation severity.

Cholecystocholedochal Fistula

  • Definition: A fistula between the gallbladder and the common bile duct.
  • Pathogenesis: Impacted gallstones erode into the bile duct due to prolonged inflammation.
  • Management:
    • Near-total cholecystectomy while preserving bile duct integrity.
    • Biliary reconstruction if necessary.
    • Endoscopic and minimally invasive approaches are emerging but require expertise.

Alternative Diagnoses in Hilar Strictures and Jaundice

  • While cholangiocarcinoma is common, consider:
    • Gallbladder carcinoma:
      • Thickened, irregular gallbladder wall.
      • Infiltration into liver segments IV and V.
      • Obstruction of the mid–bile duct with cystic duct occlusion.
    • Benign focal strictures:
      • Autoimmune cholangitis.
      • Primary sclerosing cholangitis (PSC).
      • Lymphoplasmacytic sclerosing pancreatitis/cholangitis.
      • Granulomatous diseases.

Key Points

  • Early recognition and preoperative diagnosis are essential to reduce surgical complications.
  • Open surgical approach is preferred due to distorted anatomy and high risk of bile duct injury.
  • Minimally invasive techniques are being explored but should be approached cautiously.
  • Awareness of related conditions and alternative diagnoses is important for comprehensive management.

Note: Bolded terms highlight key concepts and terminology for emphasis and easier revision.