Portal hypertension (PHT) has no formal definition in children but can arbitrarily be considered as a portomesenteric pressure greater than 10 mmHg. Sustained PHT leads to the development of varices at the sites of the portosystemic watershed, together with splenomegaly. The principal site for the development of varices is the oesophagogastric watershed, but haemorrhoids and rectal varices may become apparent even in children and adolescents. Occasionally, ectopic varices develop around surgical anastomoses such as a Roux loop and can be a cause of obscure gastrointestinal bleeding. The third site of venous overlap is the retroperitoneum and anterior abdominal wall. Spontaneous shunts may develop in the retroperitoneum; they seldom cause clinical symptoms, but they may be problematic at surgery. The most obvious sign of a significant shunt in the anterior abdominal wall is the well-known caput medusae sign, comprising dilated periumbilical veins radiating from the umbilicus. These develop along the obliterated umbilical vein and arise most commonly in cases of long-standing cirrhosis.
KeywordsPortal hypertension Oesophageal varices Portal vein thrombosis Portal systemic shunts Meso-rex shunt
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