Type II Abernethy Malformation: A Rare Cause of Hepatic Encephalopathy in Adulthood
  • Inês Gonçalves
    Department of Internal Medicine, Hospital de Braga, Braga, Portugal
  • Daniela Barros
    Department of Radiology, Hospital de Braga, Braga, Portugal
  • Margarida Araújo
    Department of Internal Medicine, Hospital de Braga, Braga, Portugal
  • Ana Isabel Machado
    Department of Internal Medicine, Hospital de Braga, Braga, Portugal
  • Camila Oliveira
    Department of Oncology, Hospital de Braga, Braga, Portugal
  • Luisa Pinto
    Department of Internal Medicine, Hospital de Braga, Braga, Portugal

Keywords

Abernethy malformation, portosystemic shunt, encephalopathy, hyperammonemia

Abstract

Abernethy malformation is a rare congenital extrahepatic portosystemic shunt with variable clinical manifestations, mainly associated with the portosystemic shunt. Hepatic encephalopathy can be the first manifestation. 
We present the case of a 50-year-old woman with hepatic encephalopathy without liver dysfunction. Further evaluation with contrast-enhanced abdominal computed tomography was performed, showing a large-calibre portocaval shunt from the left portal branch, suggestive of type 2 Abernethy malformation. The patient improved with lactulose and rifaximin but maintained rare encephalopathy episodes. She is therefore currently awaiting surgical treatment. 
Despite being a rare cause of hepatic encephalopathy, Abernethy malformation should be considered in patients with unexplained hyperammonaemia. Since it is potentially reversible, and early diagnosis and treatment may improve outcome, raising awareness of this malformation is essential.

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References

  • Kumar P, Bhatia M, Garg A, Jain S, Kumar K. Abernethy malformation: a comprehensive review. Diagn Interv Radiol 2021 Dec 16. doi: 10.5152/dir.2021.20474. Online ahead of print.
  • Mesquita RD, Sousa M, Vilaverde F, Cardoso R. Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature. BJR Case Rep 2018;4(2):20170054.
  • Li H, Ma Z, Xie Y, Tian F. Recurrent hyperammonemia after Abernethy malformation type 2 closure: a case report. Ann Hepatol 2017;16(3):460–464.
  • Uchino T, Matsuda I, Endo F. The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 1999;135(2 Pt 1):254–256.
  • Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010;51(3):322–330.
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    Published: 2022-01-26
    Issue: 2022: Vol 9 No 1 (view)


    How to cite:
    1.
    Gonçalves I, Barros D, Araújo M, Machado AI, Oliveira C, Pinto L. Type II Abernethy Malformation: A Rare Cause of Hepatic Encephalopathy in Adulthood . EJCRIM 2022;9 doi:10.12890/2022_003145.

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