Portal hypertension

Portal hypertension (PH) is a clinical syndrome resulting from the combination of increased portal blood flow and increased portal resistance and characterized by an increase in the portosystemic gradient beyond the physiological value of 5 mmHg. PH is a common complication in patients with chronic liver disease, both adults and children, and its major clinical manifestations include oesophageal varices, ascites and splenomegaly with associated hypersplenism. Upper gastrointestinal bleeding (UGIB) is a severe and potentially life-threatening complication among patients with PH, and, although, unlike in adults, in children is often self-limiting, it remains a major cause of morbidity. Complications of PH are principally managed by a conservative strategy based on medical and endoscopic therapy. However, if these fail or the patients develop major neurological or pulmonary complications (e.g. hepatic encephalopathy, hepatopulmonary or portopulmonary syndrome), more aggressive procedures are mandatory including radiological and surgical procedures as well as liver transplantation. Unlike in adults, in the paediatric 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 and adapted to children. The aim of this chapter is reporting the improvements made in the management of PH in children and propose diagnostic and therapeutic algorithms for acute and chronic complications.