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A plethora of diseases affecting liver parenchyma and vasculature underpin development of portal hypertension (PH) in children, although extrahepatic portal vein obstruction (EHPVO) and biliary atresia (BA) represent the majority of cases. With the addition of novel surgical and interventional radiology approaches to endoscopic management, the outlook of paediatric onset PH has markedly evolved during the last decades. Despite these advances, the long-term outcomes remain insufficiently characterised, as are natural history and progression risk of PH secondary to different underlying diseases.1
Typically, the first sign of complicated PH is bleeding from gastro-oesophageal varices, clinically presenting as haematemesis. Although the estimated overall mortality rate of the first variceal haemorrhage in children is low ranging from 1% to 3%, the incidence of spontaneous life-threatening bleeding episodes approaches 20% in children with cirrhosis and high-risk varices.2 Regardless of the underlying liver disease, endoscopic interventions remain the primary modality to control the first bleed and prevent further bleeding episodes. Endoscopic band ligation is replacing sclerotherapy also among children and is currently feasible in children over 10 kg of weight. Band ligation has been thought to outperform classic sclerotherapy as far as efficacy, safety and degree of standardisation are concerned, although high-quality comparative studies are few among children. Similarly, evidence-based treatment algorithms regarding the primary and secondary prophylaxis for variceal haemorrhage in children are still lacking, as prospective controlled (randomised) trials are challenging to accomplish in this young, relatively rare and heterogeneous patient population.3 Different centres tend to follow their local protocols as no uniformly accepted guidelines exist. Although prophylactic endoscopic treatment of high-risk varices (primary prophylaxis) has the potential to reduce the incidence of serious bleeding episodes and even lower the associated mortality, it is still common practice to start endoscopic interventions only …
Contributors Both authors have contributed equally to produce the manuscript including design, drafting and finalising the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Commissioned; externally peer reviewed.