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Congenital extrahepatic portosystemic shunt with progressive myelopathy and encephalopathy
  1. Danielle Lux1,
  2. Anna Naito1,
  3. Sreedharan Harikrishnan1,2
  1. 1 Neurology, East Kent Neurology Unit, East Kent University Hospitals NHS Foundation Trust, Canterbury, UK
  2. 2 Medical Education, Kings College London, London, UK
  1. Correspondence to Dr Danielle Lux, Neurology, East Kent Neurology Unit, East Kent University Hospitals NHS Foundation Trust, Canterbury CT9 4AN, UK; d.lux{at}


Portosystemic encephalopathy commonly occurs in patients with portal hypertension caused by end-stage liver disease or portal vein thrombosis. Congenital extrahepatic portosystemic shunt (CEPS) is an underdiagnosed and treatable condition that can cause encephalopathy and various neuropsychiatric symptoms. We report an unusual case of type 2 CEPS in a 29-year-old woman who presented with progressive myelopathy and fluctuating encephalopathy on a background of congenital cardiac disease. Investigations showed hyperammonaemia, and despite no evidence of portal hypertension on ultrasound imaging, CT scan of abdomen showed a shunt between the mesenteric and left internal iliac veins. Patients with unexplained fluctuating or progressive neuropsychiatric symptoms should have their serum ammonia checked. A raised serum ammonia concentration without known portal hypertension should prompt further investigations for extrahepatic shunts.

  • metabolic disease
  • neuropsychiatry
  • myelopathy
  • encephalopathy

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  • Contributors AN and DL contributed equally to this paper and are joint first authors. DL and SH were responsible for the clinical care of the patient. All authors reviewed and approved the final manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed by Tom Britton, London, UK and Robin Lachmann, London, UK.

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