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Recurrent Hyperammonemia After Abernethy Malformation Type 2 Closure: a Case Report

Hui Li1, Zhi Ma1, Ying Xie1

  • 1Department of gastroenterology, Shengjing Hospital Affiliated to China Medical University, Shenyang, Liaoning, China.

Annals of Hepatology
|April 21, 2017
PubMed

Insights

Abernethy malformation, a rare congenital shunt, can cause encephalopathy. This case shows hyperammonemia recurrence after shunt closure, highlighting the need for established Abernethy malformation management strategies.

Area of Science:

  • Hepatology
  • Vascular Surgery
  • Pediatric Gastroenterology

Background:

  • Abernethy malformation is a rare congenital condition characterized by an extrahepatic portosystemic shunt.
  • While often asymptomatic, 15% of patients develop clinical encephalopathy.
  • Type 2 Abernethy malformation involves a complete diversion of portal blood directly into the systemic circulation.

Observation:

  • A patient with type 2 Abernethy malformation presented with hyperammonemia and encephalopathy.
  • Initial treatment involved successful shunt closure via interventional angiography.
  • The patient experienced a recurrence of hyperammonemia three months post-procedure.

Findings:

  • Diagnosis of Abernethy malformation is straightforward.
  • The optimal management strategy for Abernethy malformation remains undetermined.
  • This report details the first documented case of hyperammonemia recurrence following interventional shunt closure.

Implications:

  • Recurrence of hyperammonemia post-intervention necessitates further investigation into long-term management.
  • This case underscores the complexity of treating Abernethy malformation and its associated complications.
  • Further research is crucial to establish effective therapeutic algorithms for Abernethy malformation patients.

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