Imaging of portal cavernoma cholangiopathy

Abstract

Portal cavernoma cholangiopathy (PCC) refers to the biliary changes which occur in the setting of extrahepatic portal vein obstruction and secondary portal cavernoma formation. The main radiological findings include the vascular changes in the form of portosystemic collaterals and biliary changes in the form of extrinsic impressions and strictures. Till date, conventional cholangiography has been the gold standard for the diagnosis of PCC. However, it is an invasive procedure and is associated with complications. At present there is a transition towards non-invasive modalities like ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI). The recent advances in these modalities provide an excellent delineation of both the vascular and the biliary changes non-invasively in a short time. The findings of PCC using these newer modalities are not so well described in literature. The findings of PCC also overlap with malignant conditions of biliary tract such as cholangiocarcinoma and compression of biliary tract by malignant adenopathies. In this article we describe the vascular and biliary changes associated with PCC on US, CT and MRI. We also describe the imaging findings using each modality along with their advantages and disadvantages.

Keywords: CBD, common bile duct; CEMR, contrast enhanced MR; CT, computed tomography; EHPVO, extrahepatic portal vein obstruction; ERCP, endoscopic retrograde cholangiopancreatography; MRI, magnetic resonance imaging; PC, periportal collateral; PC, portal cavernoma; PCC, portal cavernoma cholangiopathy; US, ultrasound; extrahepatic portal vein obstruction; imaging; portal cavernoma cholangiopathy.

Figure 1

(a) Direct splenoportography image showing patent splenic vein (long arrow) with attenuated portal vein (short arrow). Multiple periportal and perisplenic collateral channels (thick arrow) are seen. (b) ERCP image showing short segment smooth stricture involving mid CBD (long arrow) and smooth extrinsic impression involving common hepatic duct (short arrow) (likely due to compression by pericholedochal collateral).

Figure 2

(a) and (b): Gray scale and color Doppler ultrasound image at the level of porta showing non-visualization of portal vein (arrow) with multiple periportal collateral (cavernoma) formation. (c) and (d) Gray scale and color Doppler ultrasound image of gallbladder showing wall thickening (arrow) with multiple collaterals along gallbladder wall (gallbladder varices). (e) Spectral Doppler image of one of the collateral in gallbladder wall (arrow) showing continuous venous flow with phasic variation.

Figure 3

Extent of CBD changes on CT (a) MIP image shows CBD and intrahepatic biliary dilatation with biliary changes in the CBD, CHD and IHBRs (arrow). (b) Thick MIP image shows smooth narrowing of CBD and CHD (arrow). (c) Thick MIP image shows smooth narrowing confined to CBD (arrow).

Figure 4

CT imaging of mass-like portal cavernoma (a) Axial CECT image at level of porta showing portal cavernoma (PC) formation with non-visualized portal vein (long arrow). Multiple collaterals are also seen in gastrohepatic region and in perisplenic region (short arrow) with splenomegaly. (b) Axial CECT image showing biliary radical dilatation in right lobe of liver (arrow) due to portal biliopathy.

Figure 5

MR imaging of portal cavernoma (a) Axial T2 WI and (b) Axial T1 WI image showing absence of portal vein flow void with multiple periportal collateral (PC) formation (arrow). (c) Coronal CEMRV image showing similar findings of portal cavernoma formation (arrow).

Figure 6

MR imaging of portal cavernoma (a) Axial CEMR image showing multiple periportal collaterals (cavernoma formation) (long arrow) with proximal biliary dilatation (short arrow) (due to portal cavernoma cholangiopathy). Multiple collaterals are seen in retroperitoneum (thick arrow) with splenomegaly. (b) MRCP image showing bilobar biliary dilatation (short arrows) with obstruction at level of common hepatic duct (long arrow) (due to portal cavernoma cholangiopathy).