Unusual Case of Mirizzi Syndrome Presenting as Painless Jaundice

BACKGROUND Isolated painless jaundice is an uncommon presenting sign for Mirizzi syndrome, which is typically characterized by symptoms of acute or chronic cholecystitis. We report a rare case of Mirizzi syndrome with an acute onset of painless obstructive jaundice. CASE REPORT A 60-year-old man with an unremarkable prior medical history presented with 1 week of jaundice, dark urine, and acholic stools. His laboratory studies revealed a pattern of cholestasis with marked direct hyperbilirubinemia. Ultrasound and magnetic resonance imaging studies demonstrated intrahepatic ductal dilation and cholelithiasis, including a stone within the cystic duct. Endoscopic retrograde cholangiopancreatography with SpyGlass cholangioscopy confirmed the diagnosis of Mirizzi syndrome. CONCLUSIONS An atypical presentation of Mirizzi syndrome should be suspected in the setting of biliary obstruction without pain. The differential diagnosis is broad and includes choledocholithiasis, ascending cholangitis, and hepatobiliary malignancy. Evaluation should include laboratory studies and biliary tract imaging. Noninvasive biliary tract imaging can help exclude malignancy and confirm ductal dilation but is not sensitive for Mirizzi syndrome. Endoscopic retrograde cholangiopancreatography can serve both diagnostic as well as therapeutic purposes via stone extraction and stent placement. SpyGlass cholangioscopy can also augment management in the form of Electrohydraulic lithotripsy. Although therapeutic biliary endoscopy can be very effective, cholecystectomy remains the definitive treatment for Mirizzi syndrome.