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Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney

European Journal of Pediatrics volume 157, pages 287–290 (1998)Cite this article

Abstract

Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported – all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed.

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Affiliations

  1. Department of Pediatrics, University of Padova, Via Giustiniani, 3, I-35128 Padova, Italy, e-mail: silvia@child.pedi.unipd.it, Tel.: +39 49 8213505-06, Fax: +39 49 8213509, IT

    G. Guariso, S. Fiorio & L. Zancan

  2. Institute of Pathology, University of Padova, Italy, IT

    G. Altavilla

  3. Department of Paediatric Surgery, University of Padova, Italy, IT

    P. G. Gamba

  4. Institute of Radiology, University of Padova, Italy, IT

    T. Toffolutti & M. Chiesura-Corona

  5. Department of Surgery, University of Padova, Italy, IT

    U. Tedeschi

Authors
  1. G. Guariso
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  2. S. Fiorio
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  3. G. Altavilla
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  4. P. G. Gamba
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  5. T. Toffolutti
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  6. M. Chiesura-Corona
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  7. U. Tedeschi
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  8. L. Zancan
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Additional information

Received: 3 December 1996 / Accepted in revised form: 9 September 1997

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Cite this article

Guariso, G., Fiorio, S., Altavilla, G. et al. Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney. Eur J Pediatr 157, 287–290 (1998). https://doi.org/10.1007/s004310050812

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  • DOI: https://doi.org/10.1007/s004310050812

  • Key words Congenital absence of portal vein
  • Focal nodular hyperplasia of the liver