Hereditary hemorrhagic telangiectasia: demonstration of portosystemic venous shunts using power Doppler sonography and sonographically guided percutaneous transhepatic portovenography.

Abstract

Hereditary hemorrhagic telangiectasia is a rare autosomal dominant inherited disease associated with vascular abnormalities, which may occur in any organ. Cases of hereditary hemorrhagic telangiectasia accompanied by intrahepatic portosystemic venous shunts, however, have rarely been described. We report a case of hereditary hemorrhagic telangiectasia in which intrahepatic portosystemic shunts were detected using power Doppler sonography and portovenography with percutaneous transhepatic contrast agent injection. On gray-scale sonography, the common hepatic artery was dilated, and dilated tubular structures mimicking dilated biliary tracts were found. Power Doppler sonography demonstrated the continuity of tortuous vascular channels connecting a branch of the right portal vein to a branch of the right hepatic vein. The dilated vascular channels and tributaries of the right hepatic vein showed a monophasic waveform pattern on spectral analysis. Portovenography showed a tangle of vascular structures connecting with a branch of the right hepatic vein.