ORTHOTOPIC LIVER-TRANSPLANTATION FOR INFLAMMATORY MYOFIBROBLASTIC TUMOR OF THE LIVER HILUM

Inflammatory myofibroblastic tumor is a rare gastrointestinal neoplasm . The authors report the case of a 5-year-old girl who initially prese nted with gastric outlet obstruction secondary to an inflammatory myof ibroblastic tumor along the lesser gastric curvature. A subtotal gastr ectomy and wedge resection of a left hepatic lobe nodule were per form ed. Obstructive jaundice developed one month postoperatively. Computer ized tomography, percutaneous transhepatic cholangiography, and select ive celiac and mesenteric arteriography showed a mass that involved th e left hepatic robe, with concomitant high-grade biliary obstruction a nd portal venous encasement. Percutaneous biliary drainage was perform ed. During laparotorny, the tumor was found to be unresectable, and th e patient was referred to the Liver Transplant Service at The Children 's Hospital of Philadelphia. A total hepatectomy with temporary portoc aval shunt was performed, leaving the inferior vena cava in situ. At t he back table, an ex vivo left trisegmentectomy was performed, followe d by reimplantation of the posterior segment of the right hepatic robe . The reimplanted liver segment functioned poorly, and completion hepa tectomy with portocaval shunt was performed 24 hours postoperatively, because of severe coagulopathy, intraabdominal bleeding, and hemodynam ic instability. The patient's condition stabilized, and she was listed for urgent liver transplantation. The anhepatic state was managed wit h intermittent plasmapheresis. She had transplantation 72 hours later, and was discharged from the hospital 3 weeks postoperatively after an uneventful recovery. She remains disease-free and has normal liver fu nction 8 months after transplantation. Copyright (C) 1996 by W.B. Saun ders Company