Ken-ichi Murakami, Hideo Takamatsu, Hiroyuki Noguchi, Hiroyuki Tahara, Tatsuru Kaji

Department of Pediatric Surgery, Kagoshima University

　　We report a case of a girl with intrahepatic portosystemic venous shunt. She was 13 years old, and was admitted with a dysplasia of the right hip joint. Hepatic dysfunction was pointed out before the operation, and she was transferred to our hospital.
　　Abdominal US showed dilatation of the left portal branch and left hepatic vein, and multiple hyperechoic masses in the liver. We diagnosed it as a focal nodular hypertrophy（FNH）due to irregular distribution of the portal flow. Moreover, she showed mild hyperammonemia and hyper bile-acidemia.
　　She underwent an operation for ligation of the shunt 11 months after the first admission. We found the original vas flowing into the hepatic vein after identification of the SVC and portal vein. We ligated the shunt flow after recognition of minimal elevation of the portal pressure. The intrahepatic nodule was found on intraoperative biopsy to be due to regenerative hypertrophy. After the operation, the right hepatic vein flow got better, with no dilatation of shunt flow or portal flow. She recovered from hyperammonemia and portal hypertension and was kept under observation at the Outpatient Clinic.

Keywords：Portal hypertension, Intrahepatic portosystemic venous shunt, Hyperammonemia, Ligation, MR portography