Portal Hypertension in Children

Portal hypertension (PH) is a common complication in children with both cirrhotic and non-cirrhotic chronic liver diseases. The most important pathogenetic factors for the development of PH are the increased portal vascular resistance and an increased portal blood flow. In such a scenario, the hyperdynamic syndrome is a main feature contributing to PH, characterized by hemodynamic changes including high cardiac output, increased heart rate and total blood volume, and reduced total systemic vascular resistance. Its most threatening manifestation, variceal bleeding, represents a major cause of morbidity in this setting. Although often self-limiting at pediatric age, gastrointestinal bleeding has high morbidity, and is regarded as a frightening event by patients and carers, giving the impression of impending death. PH and its complications are also among the commonest indications to liver transplantation, therefore having relevance in the management of pediatric chronic liver disease. For these reasons, it is important to raise the awareness on the natural history of PH in children, the utility of tools that help preventing and managing acute bleeding, and the signs predicting a poor outcome, thus indicating surgery. Unlike in adults, in the pediatric setting, there is lack of data on pathophysiology, clinical features, and effective management altering the outcome of PH, and therefore children are often managed according to guidelines developed in adults. In this chapter, we discuss the advances made in the management of PH in children, compare it with the larger adult experience, and propose diagnostic and therapeutic algorithms for acute and chronic complications.