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Annals of Hepatology

versão impressa ISSN 1665-2681


LI, Hui; MA, Zhi; XIE, Ying  e  TIAN, Feng. Recurrent Hyperammonemia After Abernethy Malformation Type 2 Closure: a Case Report. Ann. Hepatol. [online]. 2017, vol.16, n.3, pp.460-464. ISSN 1665-2681.

The Abernethy malformation is a rare congenital malformation defined by the presence of an extrahepatic portosystemic shunt. Although most patients are asymptomatic, clinical encephalopathy is present in 15% of cases. We present a patient with type 2 Abernethy malformation, hyperammonemia, and encephalopathy. Shunt closure was performed successfully using interventional angiography; however, hyperammonemia recurred 3 months later. The diagnosis of Abernethy malformation can be made easily, but the ideal patient management strategy has not yet been established. This is the first reported patient with recurrence of hyperammonemia after interventional treatment; we discuss the therapeutic options for Abernethy malformation.

Palavras-chave : Congenital malformation; Encephalopathy; Complications; Shunt occlusion.

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