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

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Transcatheter Closure of Portal-Systemic Shunt Combining Congenital Double Extrahepatic Inferior Vena Cava with Vascular Plug 5-year-old boy was found to have hyperammonemia; the ammonia level ranged from 92 to 104 ␮mol/L during follow-up. Metabolic screening and a skin biopsy were negative. Physical examination showed no specific finding. Because of abnormal liver echogenicity at sonography, 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). The maximal diameter of the left side IVC was 10.6 mm. Portal vein pressure was 8 mm Hg initially, elevated immediately to 14 mm Hg after test occlusion by an end-hole balloon catheter, and then gradually decreased to 10 mm Hg 1 hour later. Follow-up portal vein venography showed increased portal venous flow (Figure, D). We used a 16-mm Amplatzer vascular plug (AGA Medical Corporation, Golden Valley, Minnesota) to occlude this abnormal shunt. The immediate venography after embolization showed no residual shunt (Figure, E). The patient’s serum ammonia level 1 day and 1 month after embolization decreased to 28 and 19 ␮mol/L, respectively. The Amplatzer vascular plug is safe and efficient in transcatheter closure of portal-systemic shunt.


Shuenn-Nan Chiu, MD Yin-Hsiu Chien, MD Mei-Hwan Wu, MD, PhD Jou-Kou Wang, MD, PhD Department of Pediatrics Shyh-Jye Chen, MD, PhD Department of Radiology National Taiwan University Hospital School of Medicine National Taiwan University Taipei, Taiwan

Figure. A, Three-dimensional computed tomographic angiography demonstrated this complex venous vascular network. B, RIVC venography. C, LIVC venography showed LIVC located left to the spine and connected to PV. D, LIVC venography after test occlusion for 1 hour showed the increase diameter and the ramifications of the portal system. E, Follow-up LIVC angiography after permanent embolization of LIVC showed no residual shunt. MHA ⫽ middle hepatic vein; RHV ⫽ right hepatic vein; PV ⫽ portal vein; RIVC ⫽ right inferior vena cava; RRV ⫽ right renal vein; SMV ⫽ superior mesenteric vein; LHV ⫽ left hepatic vein; LIVC ⫽ left inferior vena cava; SV ⫽ splenic vein; LRV ⫽ left renal vein; IMV ⫽ inferior mesenteric vein.

J Pediatr 2008;153:723 0022-3476/$ - see front matter Copyright © 2008 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2008.04.031


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