Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography: a new etiology of extrahepatic portal hypertension

Vascular disorders of the liver

“…The theory that extrahepatic portal vein obstruction in this age group always results from thrombosis, as opposed to a congenital origin, has not been fully documented and is still debated. 79,80 The prevalence of underlying pro-thrombotic diseases was high in most but not all studies. 81–86 Primary protein C, protein S, or antithrombin deficiency may be overdiagnosed, as these inhibitors are nonspecifically decreased in many affected children with a negative family screening.…”

Pattern of branching of the left portal vein: an anatomo-radiological study

“…Adequate preoperative imaging is essential in advising the surgeon of both the presence of the Rex vein and its patency [14]. Cross-sectional imaging with CT and magnetic resonance can evaluate the extrahepatic portal vein and is sufficient for evaluating the portal inflow and has become reliable at detecting small portal vein branches [29]. In fact, in this study we used a common preoperative imaging method, i.e., CT angiography, to validate its usefulness in defining the vascular anatomy of the left liver.…”

Preoperative imaging of left portal vein at the Rex recess for Rex shunt formation using wedged hepatic vein carbon dioxide portography

“…It can, however, be difficult to image the Rex segment in children because of small size of the intrahepatic portal vein radicals, the presence of multiple collaterals, and poor inflow. In the presence of cavernous transformation, indirect arterioportography and splenoportography are unreliable because of the obstruction to blood flow, reversal of flow, and dilutional effects, and cross-sectional imaging with computed tomography and MRI can evaluate the extrahepatic portal vein [14][15][16][17][18][19]. Direct transhepatic portography is relatively invasive and would be technically challenging in small children and may fail to show the Rex segment if there is no communication between the left and right portal veins.…”