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Abernethy Malformation and Gastrointestinal Bleeding: A Case Report and Literature Review

Wenrui Li1, Bin Liu1, Hai Feng1

  • 1Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China.

Vascular and Endovascular Surgery
|September 12, 2024
PubMed

Insights

Congenital extrahepatic portosystemic shunt (CEPS), or Abernethy malformation, is rare. Both surgical and endovascular closures are effective for CEPS, but high shunt flow may require additional treatment beyond coil embolization.

Area of Science:

  • Vascular Surgery
  • Gastroenterology
  • Pediatric Surgery

Background:

  • Congenital extrahepatic portosystemic shunt (CEPS), known as Abernethy malformation (AF), is a rare splanchnic venous system anomaly.
  • Current treatment strategies for CEPS, including surgical, radiological, and transplantation methods, lack comparative data.
  • This study presents a unique case of AF with an unusual shunt between the inferior mesenteric vein and the right ovarian vein.

Observation:

  • A literature review identified 34 AF patients with gastrointestinal (GI) tract bleeding.
  • Type II AF accounted for a significant majority (79%) of these GI bleeding cases.
  • The case report details an atypical shunt connection involving the inferior mesenteric vein and the right ovarian vein.

Findings:

  • Both surgical ligation and endovascular shunt closure demonstrate efficacy and safety in managing AF.
  • Endovascular methods, particularly coil embolization, may be insufficient for complete shunt closure in cases with high shunt flow.
  • The study highlights the diverse clinical presentations of AF, including rare vascular connections.

Implications:

  • Surgical and endovascular approaches are viable treatments for CEPS, offering effective management options.
  • Clinicians should consider the shunt flow dynamics when selecting endovascular treatment, as additional measures may be necessary.
  • Further research comparing different CEPS treatment modalities is warranted to establish optimal therapeutic guidelines.

Keywords:
abernethy malformationcatheter embolizationcongenital extrahepatic portosystemic shunthematocheziainferior mesenteric vein

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