Mirrizi Syndrome in a 28-Year-Old Gentleman With Sickle Cell Disease

Abstract

Patient is a 28 year old African gentleman from Ghana with sickle cell disease, anemia, malaria, and chronic indirect bilirubinemia, with one previous sickle cell crisis in the setting of acute malarial infection. who presented to the hospital with 3 days of bilateral upper quadrant abdominal pain associated with nausea and vomiting. Review of systems was positive for dark urine, headache and decreased appetite. On examination, he was afebrile, with a regular heart rate and rhythm and normal respiratory rate. He was jaundiced and had right upper quadrant fullness, tenderness, and guarding. There was no abdominal rigidity. The remainder of the examination was normal. Laboratory evaluation revealed normal kidney function, elevated bilirubin to 31. 7 mg/dl with direct bilirubin of 27 mg/dl,ALP 239 u/L., AST 200 u/L, ALT 94 u/L, hemoglobin 7.1 g/dl,lipase 23 u/L, INR 1.2, and ferritin 122. Ultrasound of the abdomen showed intrahepatic biliary duct dilatation and common biliary duct (CBD) measuring 10mm with enlarged gallbladder and multiple echogenic shadowing stones and gallbladder thickness measuring 5mm. Subsequent magnetic resonance imaging revealed a 1.4 cm stone in the neck of the cystic duct protruding into the CBD with no stones in the CBD. Patient underwent an endoscopic retrograde cholangiopancreatogram with stent placement in the CBD followed by a open cholecystectomy that revealed xanthogranulomatous cholecystitis and pigmented cholelithiasis. The patient's hyperbilirubinemia improved and returned to his baseline indirect predominance. Conclusion: Mirrizzi syndrome should remain in the differential in young patients with sickle cell disease due to increased risk of long standing pigmented gallstones.