Texas Children's Hospital and the Department of Pediatrics, Baylor College of Medicine, Houston, TX.
Bambino Gesu Children's Hospital and Tor Vergata Roma University, Rome, Italy.
Hepatology. 2016 Apr;63(4):1368-80. doi: 10.1002/hep.28153. Epub 2015 Oct 23.
Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with extrahepatic portal vein obstruction will experience complications that can be prevented by successful MRB surgery. Therefore, children with extrahepatic portal vein obstruction should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if preoperative and intraoperative evaluation demonstrates favorable anatomy, and if appropriate multidisciplinary care is available for postoperative evaluation and management of shunt thrombosis or stenosis. In contrast, consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of portal hypertension in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized.
There are few pediatric data to indicate the efficacy and safety of pharmacologic or endoscopic therapies as primary prophylaxis or that prevention of a sentinel variceal bleed will ultimately improve survival; therefore, no recommendation for primary prophylaxis with endoscopic variceal ligation, sclerotherapy, or nonspecific beta-blockade in children was proposed.
儿科门静脉高压症和静脉曲张出血的治疗方法仍存在争议,主要是因为严格的临床研究并不能为其提供充分的依据。基础生物学和临床差异使得成人的治疗方法不能直接应用于儿童。2015 年 4 月 11 日至 12 日,该领域的专家召开了第一次贝伐诺儿科卫星会议,讨论并探讨了有关门体分流术(MesoRex bypass,MRB)在肝外门静脉阻塞中的适应证和儿童静脉曲张出血一级预防的现有证据。MRB 达成了共识。绝大多数肝外门静脉阻塞的儿童将经历可以通过成功的 MRB 手术预防的并发症。因此,如果有适当的手术专业知识,如果术前和术中评估显示出有利的解剖结构,如果有适当的多学科护理可用于术后分流血栓或狭窄的评估和管理,那么应该为肝外门静脉阻塞的儿童提供 MRB 用于静脉曲张出血和其他并发症的一级和二级预防。相比之下,对于静脉曲张出血的一级预防并没有达成共识。尽管静脉曲张出血是儿童门静脉高压症的一个令人担忧的并发症,但首次出血似乎很少致命,相关的发病率尚未得到很好的描述。
儿科数据很少表明药物或内镜治疗作为一级预防的疗效和安全性,或者预防首发静脉曲张出血最终会改善生存;因此,不建议对儿童进行内镜下静脉曲张结扎、硬化治疗或非特异性β受体阻滞剂的一级预防。