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Indian J Radiol Imaging. 2019 Apr-Jun;29(2):219-222. doi: 10.4103/ijri.IJRI_461_18.

Role of interventional radiology in the diagnosis and management of congenital extrahepatic portosystemic shunts: Two case reports.

Author information

1
Department of Radiology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Karnataka, India.
2
Department of Radiology, University Hospitals, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44106, USA.
3
Department of Radiology, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114, USA.
4
Department of Radiology, Texas Children's Hospital, 6701 Fannin Street, Suite 470, Houston, TX 77030, USA.

Abstract

Congenital extrahepatic portosystemic shunt (CEPS) is a rare splanchnic venous malformation, wherein the portal venous outflow drains into the systemic venous circulation via a pathologic shunt. CEPS exhibits heterogeneous clinical behavior and angiography is the gold standard for evaluation of the portomesenteric communication to systemic vasculature. The potential severity of complications necessitates shunt closure. Here, we present two cases of CEPS. The first patient presented with an asymptomatic hyperammonemia and was found to have a Type 1 CEPS with absence of intrahepatic portal system. The second patient was asymptomatic and was incidentally found to have a Type 2 CEPS on imaging with normal intrahepatic portal system. Both patients were successfully treated with endovascular occlusion of the CEPS.

KEYWORDS:

Congenital extrahepatic portosystemic shunt; endovascular closure; focal nodular hyperplasia; hyperammonemia; portal hypertension

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