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Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein

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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.

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Authors and Affiliations

  1. Dept. of Diagnostic Imaging, Yale University School of Medicine, 333 Cedar St., 06510, New Haven, Connecticut, USA

    J. W. Barton III &  M. S. Keller

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  1. J. W. Barton III
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  2. M. S. Keller
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Barton, J.W., Keller, M.S. Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol 20, 113–114 (1989). https://doi.org/10.1007/BF02010653

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  • DOI: https://doi.org/10.1007/BF02010653

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