A case of multiple intrahepatic portal-hepatic venous shunts with hepatic coma as the first diagnostic clue

We have reported a rare case of multiple intrahepatic portal-hepatic venous shunts, who developed hepatic coma as the first clinical manifestation.A 60-year-old female was entered to our hospital because of recurrent coma attacks accompanied with hyperammonemia and triphasic wave on EEG. Laboratory data were almost normal except for hyperammonemia and retardation of ICG, and marked increase of heart/liver ratio was found on 201Tl perrectal scintigraphy. Imaging studies using ultrasonography, CT during arterial portography (CT-AP) and MRI suggested multiple intrahepatic portal-hepatic venous shunts, and hepatic venography demonstrated a total of eight macroscopic shunts retrogradely. There was no finding of chronic liver disease on peritoneoscopy or histopathological study of liver specimen. Administration of lactulose and branched chain amino acids has improved serum ammonium level, and she has been completely free of hepatic encephalopathy after medication.