In chronic liver disease, the presentation of portal hypertension is mainly as an abdominal mass due to splenomegaly. This is usually noted in slightly older children (around 8 years of age). Encephalopathy and abdominal distension due to ascitis may also complicate the haematemesis in patients with cirrhosis. Growth retardation is a well-recognized complication of cirrhosis and portal hypertension is a contributory factor to it due to mucosal oedema and lymphatic congestion leading to malabsorption and protein losing enteropathy.

However, in portal venous occlusion, the presentation is usually in younger children (5 years) with acute episode of upper or lower gastro-intestinal bleeding. This may or may not be accompanied by splenic enlargement depending on the blood loss due to the haematemesis or malena. Anorectal varices and haemorrhoids are identified in almost two-thirds of patients with portal venous occlusion.

In patients with Budd-Chiari syndrome, intractable ascitis with hepatomegaly is the usual initial presentation. Jaundice is variable.