Rotor syndrome is a rare, benign familial disorder characterized by chronic fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with normal hepatic histology. In contrast to Dubin-Johnson syndrome, there is no liver pigmentation in Rotor syndrome. A 36-year-old man was admitted due to asymptomatic persistent jaundice. His siblings had jaundice with direct hyperbilirubinemia. Physical examination revealed icteric sclerae without hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with direct bilirubinmia. Hepatic uptake and storage capacity of indocyanine green was markedly reduced, while excretion into bile was slightly suppressed. Markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tract were shown in 99mTc-DISIDA scan. Histology of the liver showed mild steatosis without pigmentation. We report a case with the review of literature.
Adult ; Coloring Agents/*diagnostic use/pharmacokinetics ; Humans ; Hyperbilirubinemia, Hereditary/diagnosis/genetics/radionuclide imaging ; Indocyanine Green/*diagnostic use/pharmacokinetics ; Jaundice, Chronic Idiopathic/*diagnosis/radionuclide imaging ; Liver/radionuclide imaging ; Liver Function Tests ; Male ; Radiopharmaceuticals/*diagnostic use ; Technetium Tc 99m Disofenin/*diagnostic use