Guidelines for diagnosis and follow-up of children and adolescents with portal hypertension

Portal hypertension is a complex syndrome caused by increased resistance to the splachnic venous flow at the portal vein level, with a hyperdynamicsystemic circulation characterized by peripheral vasodilation and high cardiac output. Portal flow can be obstructed at prehepatic ("normal liver"), intrahepatic (as in cirrhosis), or post-hepatic level (as in Budd-Chiari syndrome). In pediatric patients, prehepatic and intrahepatic causesare almost equally distributed (nearly 50% each). Clinical presentation and individual impact are heterogeneous, but in each case, itis the expression of a worsening condition and the need to solve the problem, either by treating its consequences or (ideally) its causes.