Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

  • Authors:
    • Yabin Hao
    • Xu Hong
    • Xinyan Zhao
  • View Affiliations

  • Published online on: December 4, 2014     https://doi.org/10.3892/ol.2014.2767
  • Pages: 695-700
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Abstract

Abernethy malformation is a rare congenital malformation defined by an extrahepatic portosystemic shunt. The majority of affected patients are young (<18 years of age) and experience various symptoms, including vomiting, jaundice, dyspnea and coma. The current study presents a case of Abernethy malformation in an asymptomatic adult male patient. The patient exhibited congenital absence of the portal vein, congenital heart disease (postoperative ventricular septal defect status), and multiple liver lesions, confirmed to be focal nodular hyperplasia by biopsy. Ultrasonography and magnetic resonance imaging findings revealing the liver lesions, type II congenital absence of the portal vein and the portosystemic shunt are presented. In addition, the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder are investigated by reviewing 101 previously reported cases.
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February-2015
Volume 9 Issue 2

Print ISSN: 1792-1074
Online ISSN:1792-1082

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Spandidos Publications style
Hao Y, Hong X and Zhao X: Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol Lett 9: 695-700, 2015.
APA
Hao, Y., Hong, X., & Zhao, X. (2015). Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncology Letters, 9, 695-700. https://doi.org/10.3892/ol.2014.2767
MLA
Hao, Y., Hong, X., Zhao, X."Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review". Oncology Letters 9.2 (2015): 695-700.
Chicago
Hao, Y., Hong, X., Zhao, X."Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review". Oncology Letters 9, no. 2 (2015): 695-700. https://doi.org/10.3892/ol.2014.2767