Transcatheter Closure of Portal-Systemic Shunt Combining Congenital Double Extrahepatic Inferior Vena Cava with Vascular Plug

摘要

A 5-year-old boy was found to have hyperammonemia; -the ammonia level ranged from 92 to 104mumol/L during follow-up. Metabolic screening and a skin biopsy were negative. Physical examination showed no specific finding. Because of abnormal liver echogenicity at sonog-raphy, abdominal ultrafast computed tomography was performed, which showed a double extrahepatic inferior vena cava (IVC) with a congenital extrahepatic portal-systemic shunt and portal vein hypoplasia (Figure, A). For prevention of hepatic encephalopathy by chronic hyperammonemia, catheterization was performed in an attempt to occlude this shunt. Right IVC venography showed right sided IVC receiving blood from bilateral common iliac, bilateral renal, and right and middle hepatic veins. In addition, an abnormal left sided extrahepatic IVC receiving blood from splenic and left hepatic vein was noted by selective left IVC venography (Figure, B and C).