Transhepatic embolization of a congenital intrahepatic portosystemic shunt for the treatment of hepatic encephalopathy in a noncirrhotic patient using Amplatzer vascular plug device

Abstract

A 73-year-old male with no history of liver disease was hospitalized for weakness, confusion, ataxia, and new onset hepatic encephalopathy with hyperammonemia. After management with lactulose and rifaximin, his symptoms persisted, and he underwent transjugular liver biopsy. Biopsy showed normal liver, but a portosystemic shunt was incidentally identified on postbiopsy venogram. The patient underwent occlusion of the shunt with two Amplatzer vascular plugs and four Nester coils. Following embolization, the patient’s symptoms resolved completely. Our case reports one of the oldest adults to present with symptoms from a congenital portosystemic shunt. Congenital portosystemic shunts can be considered in patients with new onset hepatic encephalopathy in the absence of underlying liver disease. Prognosis after embolization of congenital portosystemic shunt is great, and embolization may result in full reversal of symptoms.

Congenital intrahepatic portosystemic venous shunt embolization: A two‐case experience

“…In addition, complications like portopulmonary hypertension, reported in 13%‐66% in children, hepatic encephalopathy, and hepatopulmonary syndrome are the most prominent manifestations caused by long‐term shunting . Congenital intrahepatic portosystemic venous shunt may close spontaneously within the first 2 years of life or may remain asymptomatic and undetected for several years . Alternatively, some shunts present in infancy and manifest in neonatal hyperammonemia which has been explained by some authors due to some precipitating factor like constipation .…”

“…Technical strategies to be used are the balloon‐assisted portography for better evaluation of the anatomy and continuous monitorization of the portal venous pressure to avoid potential portal hypertension. The use of ethanol, coils, and Amplatzer Vascular Plugs have all been reported as effective means of shunt closure . Takenaga et al described one case of multiple intrahepatic portosystemic shunts successfully treated with transhepatic embolization …”

“…8 Congenital intrahepatic portosystemic venous shunt may close spontaneously within the first 2 years of life or may remain asymptomatic and undetected for several years. 13 Alternatively, some shunts present in infancy and manifest in neonatal hyperammonemia which has been explained by some authors due to some precipitating factor like constipation. 5 When chronic shunting persists into adulthood, patients most often present with encephalopathy; however, pulmonary arterial hypertension and/or heart failure have both been described in the literature as alternative presentations.…”

“…The use of ethanol, coils, and Amplatzer Vascular Plugs have all been reported as effective means of shunt closure. 13 Takenaga et al described one case of multiple intrahepatic portosystemic shunts successfully treated with transhepatic embolization. 16 Multiple imaging modalities are useful for diagnosis of an intrahepatic portosystemic venous shunt including color Doppler ultrasonography, contrast-enhanced CT, MRI or conventional catheter-directed angiography.…”

Recent advances in hepatic encephalopathy

“…In a case report, a 73-year-old male presented with new-onset HE and was found to have a congenital portosystemic shunt with a normal liver histology. Correction of the shunt led to full reversal of his symptoms 147 . This highlights a surgically correctable cause of acute HE without underlying liver disease.…”