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Non-cirrhotic Extra-Hepatic Porto-Systemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure

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Abstract

Introduction

Encephalopathy secondary to hyperammonemia due to Congenital Extra-hepatic Porto-systemic shunt (CEPS) in the absence of liver cirrhosis is an exceptionally unusual condition. We describe the case of a 54-year-old woman admitted to the Emergency Department complaining of recurrent episodes of confusion and worsening cognitive impairment. At admission, the patient displayed slowing cognitive-motor skills with marked static ataxia and impaired gait. Hyperammonemia was detected in the serum. An abdominal computed tomography (CT) excluded portal hypertension and liver cirrhosis, detecting a congenital extra-hepatic porto-systemic shunt which is a highly unusual vascular malformation. The patient was treated by interventional radiologists with a successful endovascular closure.

Areas Covered

We have performed a review of the last three decades of the literature, starting from the introduction of CT scanning in common clinical practice. Eighteen studies (case reports) described 29 patients with encephalopathy secondary to hyperammonemia due to CEPS in the absence of liver cirrhosis: They underwent treatment similar to our case report of CEPS.

Expert Commentary

Encephalopathy secondary to hyperammonemia in the absence of hepatic dysfunction is an important diagnostic dilemma to many clinicians. An interventional radiologic approach is currently preferred.

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Abbreviations

CEPS:

Congenital extra-hepatic porto-systemic shunt

CT:

Computed tomography

HE:

Hepatic encephalopathy

PSS:

Porto-systemic shunt

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Acknowledgments

The authors are the only ones responsible for the content and writing of the paper.

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Authors and Affiliations

  1. Interventional Radiologist Unit – “Santa Maria” Hospital, Terni, Italy

    Massimiliano Allegritti, Benedetta Enrico & Giovanni Passalacqua

  2. Department of Surgical Science, University of Perugia, Perugia, Italy

    Emanuela Basile, Lara de Vito & Roberto Cirocchi

  3. Department of Pediatric Surgery, Pediatric Autologous Bowel Reconstruction, Rehabilitation and Regenerative Medicine Unit, Meyer Children’s Hospital, Florence, Italy

    Antonino Morabito

  4. Department of Medicine, University of Perugia, Perugia, Italy

    Michela Giustozzi

Authors
  1. Massimiliano Allegritti
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  2. Benedetta Enrico
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  3. Emanuela Basile
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  4. Lara de Vito
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  5. Antonino Morabito
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  6. Roberto Cirocchi
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All authors contributed equally to the manuscript drafting. All authors read and approved the final manuscript.

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Correspondence to Roberto Cirocchi.

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Allegritti, M., Enrico, B., Basile, E. et al. Non-cirrhotic Extra-Hepatic Porto-Systemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure. Dig Dis Sci 65, 946–951 (2020). https://doi.org/10.1007/s10620-019-06024-4

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