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Case Reports
. 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

Sheetal V Mathai  1 , Victor Kondray  2 , Elias Salloum  3 , Kamlesh Kukreja  4 , Sidhartha Tavri  2
Affiliations
Case Reports

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

Sheetal V Mathai et al. Indian J Radiol Imaging. 2019 Apr-Jun.

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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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1 (A and B)
Figure 1 (A and B)
Axial MRA demonstrating Type 1 portosystemic shunt with portal vein to the IVC (A and B)
Figure 2 (A and B)
Figure 2 (A and B)
SV venogram demonstrating a patent shunt to the IVC (A).Balloon occlusion venogram with hypoplastic portal veins (B)
Figure 3 (A and B)
Figure 3 (A and B)
Balloon occlusion pressure measurements in the proximal shunt demonstrated no significant increased portal pressure (not shown).Venogram prior to Amplatzer plug deployment (A) and fluoroscopic spot image post deployment of Amplatzer plug (B)
Figure 4 (A and B)
Figure 4 (A and B)
Coronal (A) CT image showing an enlarged Type 2C portosystemic shunt between SMV and right IIV. Selective inferior mesenteric vein (IMV) venogram demonstrating the IMV with reversed flow to right IIV shunt, Type IIc (B)
Figure 5 (A and B)
Figure 5 (A and B)
Delayed inferior mesenteric artery (IMA) angiography without balloon occlusion (A) demonstrates venous outflow of the transverse colon through the sigmoid colon drained into an enlarged IMV with patent shunt. IMA angiography with distal balloon occlusion (B) shows colonic drainage can be reversed via shunt
Figure 6 (A and B)
Figure 6 (A and B)
Post coil embolization venogram showing shunt occlusion (A). Six months follow-up coronal CT shows complete resolution of the shunt (B)
See this image and copyright information in PMC

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