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 …

中文翻译：

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小儿门静脉高压症的管理进展

尽管肝外门静脉阻塞 (EHPVO) 和胆道闭锁 (BA) 占大多数病例，但影响肝实质和脉管系统的多种疾病支持儿童门静脉高压 (PH) 的发展。随着新的外科和介入放射学方法加入内镜治疗，儿科发病 PH 的前景在过去几十年中发生了显着变化。尽管取得了这些进展，长期结果仍然没有得到充分表征，PH 继发于不同基础疾病的自然病程和进展风险也是如此。1 通常，复杂 PH 的第一个迹象是胃食管静脉曲张出血，临床表现为呕血。尽管儿童首次静脉曲张出血的估计总体死亡率较低，从 1% 到 3%，在患有肝硬化和高危静脉曲张的儿童中，自发性危及生命的出血事件的发生率接近 20%。2 无论潜在的肝脏疾病如何，内镜干预仍然是控制首次出血和防止进一步出血事件的主要方式。内窥镜束带结扎术也在儿童中取代了硬化疗法，目前在体重超过 10 公斤的儿童中是可行的。就疗效、安全性和标准化程度而言，套扎术被认为优于经典的硬化疗法，尽管在儿童中进行的高质量比较研究很少。同样，关于儿童静脉曲张出血的一级和二级预防的循证治疗算法仍然缺乏，因为前瞻性对照（随机）试验很难在这个年轻、相对罕见和异质性的患者群体中完成。3 不同的中心倾向于遵循他们当地的协议，因为不存在统一接受的指南。尽管高危静脉曲张的预防性内镜治疗（初级预防）有可能降低严重出血事件的发生率，甚至降低相关死亡率，但仅开始内镜干预仍然是常见的做法……