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Type II Abernethy Malformation in a Patient with Primary Budd-Chiari Syndrome

Peng-Xu Ding1, Xin-Wei Han1, Chao Liu1

  • 1Department of Intervention, First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.

Annals of Hepatology
|May 23, 2019
PubMed

Insights

This report details the first combined case of Abernethy malformation and Budd-Chiari syndrome in China. Treatment involved angioplasty for vena cava obstruction, with monitoring for the vascular malformation.

Area of Science:

  • Vascular Medicine
  • Hepatology
  • Congenital Malformations

Background:

  • Budd-Chiari syndrome (BCS) involves hepatic venous outflow obstruction.
  • Abernethy malformation is a rare congenital vascular anomaly diverting portal blood from the liver.
  • Combined occurrence of these rare vascular diseases is exceptionally uncommon.

Observation:

  • A patient in China presented with a rare combination of Type II Abernethy malformation and BCS.
  • This represents the first reported instance of this dual diagnosis in the region.

Findings:

  • The patient exhibited both hepatic venous outflow obstruction (BCS) and abnormal portal blood flow (Abernethy malformation).
  • Inferior vena cava obstruction, a component of BCS, was successfully treated with percutaneous balloon angioplasty.

Implications:

  • This case highlights the importance of recognizing rare vascular disease combinations.
  • Management involved interventional treatment for BCS and conservative follow-up for Abernethy malformation.
  • Further research may elucidate shared etiologies or management strategies for these rare conditions.

Keywords:
AngiographyCongenital porto-systemic shuntHepatic venous outflow obstructionInferior vena cava

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