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

Sheetal V Mathai; Victor Kondray; Elias Salloum; Kamlesh Kukreja; Sidhartha Tavri

doi:10.4103/ijri.IJRI_461_18

Indian Journal of Radiology and Imaging, Table of Contents

CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2019; 29(02): 219-222
DOI: 10.4103/ijri.IJRI_461_18

Case Report

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

Sheetal V Mathai

Department of Radiology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Karnataka, India

,

Victor Kondray

Department of Radiology, University Hospitals, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44106, USA

,

Elias Salloum

Department of Radiology, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114, USA

,

Kamlesh Kukreja

Department of Radiology, Texas Children’s Hospital, 6701 Fannin Street, Suite 470, Houston, TX 77030, USA

,

Sidhartha Tavri

Department of Radiology, University Hospitals, Case Western Reserve University School of Medicine, 11100 Euclid Ave, Cleveland, OH 44106, USA

› Author Affiliations
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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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References

References
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