Makindo Medical Notes

🟡 Mirizzi Syndrome is a rare complication of gallstones where an impacted stone in the cystic duct or gallbladder neck compresses the common bile duct (CBD), leading to obstructive jaundice ± fistula formation.

📖 About

• Rare but important cause of obstructive jaundice.
• Accounts for ~0.1% of gallstone disease; often missed until surgery.

🧬 Etiology & Pathophysiology

• Gallstone Impaction: Stone stuck in cystic duct/gallbladder neck → external compression of CBD.
• Chronic Inflammation: Leads to scarring and sometimes fistula formation between gallbladder and CBD.
• Secondary Changes: Long-standing obstruction can cause cholangitis, biliary cirrhosis, or rarely cholangiocarcinoma.

🩺 Clinical Presentation

• Obstructive jaundice (yellow sclera, skin).
• RUQ pain — colicky or constant.
• ± Fever & chills → suggests ascending cholangitis.
• Nausea, vomiting, pruritus.
• Dark urine + pale stools (conjugated hyperbilirubinaemia).

📊 Classification (Csendes)

• Type I: External compression, no fistula.
  • IA: cystic duct intact.
  • IB: cystic duct obliterated.
• Type II: Cholecystocholedochal fistula < 33% CBD diameter.
• Type III: Fistula 33–66% CBD diameter.
• Type IV: Fistula > 66% CBD diameter.

🔍 Investigations

• LFTs: Cholestatic picture (↑ ALP, ↑ bilirubin).
• Ultrasound: Gallstones + proximal biliary dilatation.
• MRCP: Non-invasive gold standard for mapping stones & fistulae.
• ERCP: Diagnostic + therapeutic (stone extraction, stent placement).
• CT Scan: To exclude malignancy and assess complications.

⚕️ Management

• Cholecystectomy: Definitive management. Laparoscopic approach difficult; conversion to open often needed due to distorted anatomy.
• ERCP: Stone removal or CBD stenting in unstable/high-risk patients or as bridge to surgery.
• Bile Duct Repair: For fistulae — may require Roux-en-Y hepaticojejunostomy in advanced cases.
• Antibiotics: If cholangitis present, cover Gram-negative enterics and anaerobes.

💡 Exam Pearls

• Think of Mirizzi when gallstones + obstructive jaundice but no distal CBD stone seen.
• Important cause of difficult cholecystectomy — high risk of CBD injury.
• Differentiate from cholangiocarcinoma (both can cause jaundice + biliary obstruction).