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Recurrent Hyperammonemia After Abernethy Malformation Type 2 Closure: a Case Report.
1Department of gastroenterology, Shengjing Hospital Affiliated to China Medical University, Shenyang, Liaoning, China.
Annals of Hepatology
|April 21, 2017
Summary
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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