We retrospectively evaluated the utility of Tc-99m DISIDA hepatobiliary scintigraphy and percutaneous needle biopsy in differentiating biliary atresia from intraheaptic cholestasis in 60 consecutive infants. Twenty three patients proved to have biliary atresia and remaining 37 patients had intraheaptic cholestasis such as neonatal heaptitis (34 patients), TPN induced jaundice (2 patients) and Dubin-Johnson syndrome (1 patient). All sixty patients underwent Tc-99m DISIDA hepatobiliary scintigraphy with phenobarbital pretreatment. Of 23 patients with biliary atresia, 22 were correctly interpreted showing 96% sensitivity while of 37 patients with intraheaptic cholestasis, only 12 had intestinal excretion of radionuclide showing 32% specificity. Forty needle biopsies were carried out in 17 patients with biliary atresia and 23 patients with intraheaptic cholestasis. Of 40 biopses, 37 were correctly interpreted as either having biliary atresia or intrahepatic cholestasis showing overall diagnostic accuracy of 93%. Of 3 misdiagnostic cases, the histologic findings of two patients with biliary atresia (aged 43 days and 54 days at the first needle biopsy) essentially were the same as those of neonatal heaptitis but follow-up biopsies showed the findings consistent with biliary atresia. The histologic findings of the 3rd patient (VLBW premie with history of 8 weeks TPN) showed mild ductal proliferation and portal fibrosis being interpreted suspicious biliary atresia, but jaundice resolved gradually. In short, the patients who have intestinal excretion of radionuclide on Tc-99m DISIDA hepatobiliary scintigraphy, biliary atresia can be ruled out. But the patients who do not have intestinal excretion of radionuclide should have further investigation as needle biopsy because of high degree of accuracy of percutaneous needle biopsies in differentiating biliary atresia from intrahepatic cholestasis. We conclude that judicious use of a combination of Tc-99m DISIDA hepatobiliary scintigraphy and percutaneous needle biopsy gives correct diagnosis in 95% or more of infantile cholestasis.
Biliary Atresia* ; Biopsy ; Biopsy, Needle* ; Cholestasis ; Cholestasis, Intrahepatic* ; Diagnosis* ; Fibrosis ; Follow-Up Studies ; Humans ; Infant ; Jaundice ; Jaundice, Chronic Idiopathic ; Needles* ; Phenobarbital ; Radionuclide Imaging* ; Retrospective Studies ; Sensitivity and Specificity

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