Portal hypertension (PHT) is a condition characterised by an increase in portal vein pressure from a variety of pathological processes, thus resulting in the development of complications, including variceal bleed due to the development of gastro-oesophageal varices, splenomegaly and thrombocytopenia . Aetiologies and pathophysiology differ whether it occurs in the setting of cirrhotic or noncirrhotic conditions [2,3]. Extrahepatic portal vein obstruction (EHPVO) is one of the most common causes of PHT in the paediatric population , but it will not be discussed in this chapter. Most patients with PHT present with an episode of variceal bleed. Diagnosis is established with a physical examination and confirmed through the use of ultrasound (US) or other imaging. Portal vein pressure measurements are rarely done, although hepatic vein wedge pressure measurements are more common after the experience that has been established in adults. A liver biopsy may also contribute to identifying the underlying process that has caused the cirrhotic changes in the liver. In asymptomatic patients, treatment will be oriented at preventing a first bleeding episode. In the setting of acute variceal bleed, pharmaceutical measures and endoscopic variceal ligation (EVL) will most often achieve successful control . While the use of medication therapy and transjugular intrahepatic portosystemic shunt (TIPS) have both been well described in the adult population, there is currently no evidence promoting the use of these modalities in children [1,5]. Selective and, more rarely, nonselective portosystemic shunts are therefore an effective option for the long-term management of patients with variceal bleeding. In the setting of cirrhotic PHT, liver transplant is sometimes required .
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