We experienced two cases of
Rotor syndrome in
brothers who were a 13 year-old boy and an 11 year-old boy, respectively. They presented with icteric
scleras for a few months. Their common
laboratory characteristics were as follows Direct
bilirubin was more increased than indirect
bilirubin, but
aminotransferases were normal.
Plasma indocyanine green (ICG) test revealed hepatic excretory defect
plasma ICG concentrations 15 minutes after
intravenous injection were 80.45% and 78.28%, respectively.
99mTc-DISIDA Hepatobiliary scan showed that severely decreased hepatic extraction with mild cardiac
blood pool, markedly delayed
biliary excretion in both intra- & extra- hepatic
bile ducts, delayed visualization of gall
bladder, and markedly delayed intestinal biliary passage.
Needle liver biopsy showed normal hepatic
histology without
pigmentation.