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Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula

Rohit S Loomba1, Grzegorz W Telega, Todd M Gudausky

  • 1Children's Hospital of Wisconsin/Herma Heart Center, Milwaukee, WI 53226, USA. Rloomba@mcw.edu

Journal of Pediatric Surgery
|May 19, 2012
PubMed

Insights

A child with Abernethy malformation and a unique portal vein-coronary sinus connection developed hepatoblastoma. This is the first documented case of hepatoblastoma in type 2 Abernethy malformation.

Area of Science:

  • Vascular malformations
  • Pediatric oncology

Background:

  • Abernethy malformation is a rare congenital portosystemic shunt.
  • It can be associated with serious complications, including liver tumors.

Observation:

  • A pediatric patient presented with a rare Abernethy malformation variant, featuring an anomalous connection between the portal vein and coronary sinus.
  • The patient was monitored for 30 months.

Findings:

  • The patient was diagnosed with hepatoblastoma, a known complication of Abernethy malformation.
  • This is the first reported instance of hepatoblastoma in a patient with type 2 Abernethy malformation.

Implications:

  • This case highlights the importance of long-term surveillance for Abernethy malformation patients.
  • It expands understanding of Abernethy malformation variants and associated oncological risks.

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