Primary choledocholithiasis after cholecystectomy without hyperbilirubinaemia in a child with sickle cell disease

Children with sickle cell disease (SCD) may have hepatobiliary pathology secondary to chronic haemolysis and intrahepatic sickling during vaso-occlusive crisis. Choledocholithiasis in children with SCD is usually secondary to cholelithiasis but, less commonly, can be due to primary choledocholithiasis. A known history of cholecystectomy may lead to a delay in diagnosis of biliary tract pathology in sickle cell patients presenting with abdominal pain. Normal conjugated bilirubin levels may further stray healthcare providers away from considering biliary disease in these patients. Here, we report an unusual case of primary choledocholithiasis in a child with SCD after cholecystectomy with normal conjugated bilirubin at the time of presentation.