An Unusual Cause of Encephalopathy in a Noncirrhotic Adult: Case Report and Review of Literature

Case Presentation: Portosystemic shunts are abnormal connections between the portal vein and the systemic veins bypassing the liver. This is commonly seen in patients with liver cirrhosis secondary to portal hypertension. We present a case of a noncirrhotic intrahepatic Portosystemic shunt with encephalopathy in a female with acute onset confusion and worsening cognition. She was diagnosed to have metabolic encephalopathy due to elevated levels of serum ammonia and abnormal EEG. She was initially medically managed by gastroenterology. Given no clinical improvement and persistent higher levels of serum ammonia, a multidisciplinary meeting was held and endovascular embolization option was decided. The right posterior branch of the portal vein was percutaneously accessed and portogram showed an early draining hepatic vein in concordance with the CT. Selective cannulation of the portal venous branch and venogram demonstrated the intrahepatic portosystemic shunt. A 4 × 14 mm micro nester coil was used to occlude the shunt and post-embolization venography demonstrated absent flow through the shunt. The patient had dramatic improvement in her cognition and speech the day after the procedure, with normalization of serum ammonia. Thus earlier diagnosis and treatment of this unusual cause of encephalopathy in a noncirrhotic patient can be rewarding to the patient, the treating physician, and the interventional radiologist. Understanding the morphology of the shunt and choosing the ideal access is an important part of the treatment.