A CASE OF NON-CIRRHOTIC PORTAL-SYSTEMIC ENCEPHALOPATHY TREATED BY COIL EMBOLIZATION OF THE PORTASYSTEMIC SHUNT

Abstract

Introduction: Intrahepatic portal-systemic venous shunts are defined as communication between the portal and the systemic venous circulation, measuring more than 1mm in diameter, and at least partially located inside the liver. The cause of this condition is disputed in many cases. It may be congenital or acquired secondary to portal hypertension. Here we describe a case of portal-systemic encephalopathy due to a spontaneous large-caliber portal-hepatic venous shunt. The encephalopathy was corrected with the treatment of the shunt. Case presentation: A 72-year-old man presented with recurrent episodes of change in mental status. His past medical history was unremarkable without history of hepatitis or cirrhosis. No history of trauma. Liver function was normal except for an elevated ammonia level. Hepatitis panel was negative. AMA, ANA and ASMA were all within normal limits. Abdominal ultra-sonography showed a large caliber portal-hepatic venous shunt in the posterior right lobe. Percutaneous transhepatic portography and hepatic venous angiogram were confirmatory. The treatment with coil embolization was successful, and his encephalopathy resolved postoperatively. Patient has been doing fine on one year follow up without recurrence of his encephalopathy. Discussion: A large intrahepatic portal-hepatic venous shunt is a rare condition. Most of the cases are primary as a congenital fistula or secondary to blunt trauma, hepatic tumor or liver biopsy. Spontaneous portal-hepatic shunt is very rare especially in non-cirrhotic patients. Most of these are asymptomatic discovered incidentally during imaging study done for not related causes. Few cases of portal hypertension had been reported secondary to intrahepatic portal shunt. Non-cirrhotic intrahepatic portal hypertension is difficult to evaluate. Encephalopathy secondary to porto-systemic shunt is seen mostly in patients with concomitant cirrhosis. The dramatic improvement of this patient's encephalopathy after the embolization of the shunt indicate that his encephalopathy was related at least in part to the shunt. Possibilities for treatment of such condition include embolization by interventional radiologist or surgery. The etiology of the shunt in this patient is unclear. Given the age of the patients and the absence of history of cirrhosis ortrauma, the shunt is thought to be spontaneous.