title: Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein.
abstract: Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.
authors.affiliation: Dept. of Diagnostic Imaging, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: J W
authors.initials: JW
authors.lastname: Barton
authors.forename: M S
authors.initials: MS
authors.lastname: Keller
mesh_terms: D000015:Abnormalities, Multiple; D006528:Carcinoma, Hepatocellular / Q000209:etiology / Q000601:surgery*; D006053:Goldenhar Syndrome / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D008342:Mandibulofacial Dysostosis / Q000150:complications*; D011169:Portal Vein / Q000002:abnormalities*